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HCP PORPHYRIA
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PORPHYRIA FACTS:
Coproporphyria (HCP)

Coproporphyria (HCP) is one of the porphyrias, a group of diseases that
involves defects in heme metabolism and that results in excessive secretion of
porphyrins and porphyrin precursors.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Coproporphyria (HCP) manifests with abdominal pain, neuropathies,
constipation, and skin changes.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Constipation in Coproporphyria (HCP) is a part of numerous autonomic
neuropathies commonly associated with the disease.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The presence of colicky abdominal pain is commonly found in Coproporphyria
(HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Coproporphyria (HCP) is an autosomal dominant disease that results from
defects in the enzyme coproporphyrinogen oxidase.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
The coproporphyrinogen in Coproporphyria (HCP) oxidase enzyme speeds the
conversion of coproporphyrinogen to protoporphyrinogen.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
In coproporphyria (HCP) , the porphyrin precursors porphobilinogen and
amino-levulinic acid (ALA) accumulate, as well as the formed porphyrin
coproporphyrin.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
The predominant problem in Coproporphyria (HCP) is neurologic damage that
leads to peripheral and autonomic neuropathies and the psychiatric
manifestations.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Cardiovascular features occur in 70 per cent of HCP cases and include
hypertension.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++

Cardiac arrythmia may present in HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++

Hypertension may occasionally persist during remission and require treatment.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

In Hereditary Coproporphyria (HCP) there may be cardiac arrythmia.

SOURCE:
Columbia Health Systems
Medical Educational
1999
+++++++++

HCP ischaracterized by episodic acute attacks that consist of hypertension.

SOURCE:
Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++

Cardiac arrythmia is often seen in hereditary coproporphyria (HCP).

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++

In Hereditary Coproporphyria (HCP) there may be Hypertension.

SOURCE:
Columbia Health Systems
Medical Educational
1999
+++++++++


Hypertension is often present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

HCP is characterized by episodic acute attacks that consist of tachycardia.

SOURCE:
Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
++++++++++++

In Hereditary Coproporphyria (HCP) there is usually Tachycardia.

SOURCE:
Columbia Health Systems
Medical Educational
1999
+++++++++

Tachycardia often present during HCP onsets.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++

Hypertension is often seen in hereditary coproporphyria (HCP) during the onset
of the acute attack.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++


HCP acute attacks present with tachycardia.

SOURCE:
Jean-Charles Deybach, M.D., Ph.D.
Hôpital L. Mourier and Faculté de Médecine X. Bichat
Université Paris 7, France
++++++++++++


Hypertension can present in HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++
Hereditary coproporphyria (HCP) combines the clinical features of acute and
cutaneous porphyria.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++


The dermopathy resulting from the photosensitivity of HCP includes
vesicular lesions.

SOURCE:
Porphyria Resources
United Medical Services
1996
++++++++++++

Hypertrichosis may occur in expressed cases of HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
++++++++++

In HCP porphyrins absorb visible light strongly.


SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++++++++

Exposure to strong visible light (sunlight) in HCP, may cause pruritis, edema and
so forth.

SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++++++++

Hereditary coproporphyria (HCP) is associated with photosensitivity.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++

HCP has neurological symptoms as well as cutaneous.

SOURCE:
The Porphyrias
A. Kappas et al.
1995, pp 2103-2159
The Metabolic and Molecular Bases of Inherited Disease
vol. 2,
(C.R. Scriver et al., eds.), McGraw Hill
+++++++++++

Hereditary Coproporphyria Clinical manifestations are identical to AIP, except for
cutaneous photosensitivity that occurs in one third of cases.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
++++++++++++++++++++

HCP has photosensitivity in about one third of all cases.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++






Coproporphyria (HCP) manfests cutaneous symptoms and is subject to light
sensitivity."

SOURCE:
The Porphyrias
A. Kappas et al.
1995, pp 2103-2159
The Metabolic and Molecular Bases of Inherited Disease
vol. 2,
(C.R. Scriver et al., eds.), McGraw Hill
++++++++++++

HCP can present as acute muscle pain, fever and weakness.

SOURCE:
Arthritis and Rheumatism
March 1997 Vol. 40 No. 3
page 586
+++++++++++

Most often hcp attacks are accompanied by painful paresthesis and motor

weakness in the limbs.

SOURCE:
Dr. Stanley L. Wiener
"Differential Diagnosis of Acute Pain"
McGraw-Hill 1993
++++++++++++

HCP ischaracterized by episodic acute attacks that consist of various tremors,
seizure, and peripheral neuropathies.

SOURCE:
Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++

Acute peripheral neuropathy is always superimposed upon a pre-existing
chronic nerve fiber lesion.

This therefore shows that there is demonstrating the possibility of more
continuous lesions in hereditary coproporphyria.


SOURCE:
Journal of Neurology
1984
231: 99-101
+++++++++++++++

Upper limbs and proximal muscles are worst affected and truncal muscle
paralysis may lead to respiratory paralysis.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K
+++++++++++++++

Polyneuropathy often present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Upper limbs and proximal muscles are worst affected by PN in HCP, and truncal
muscle paralysis may lead to respiratory paralysis.


SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K
+++++++++++++++

Whole-body paralysis can occur in the acute porphyrias in association with an
acute attack in HCP.



SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++++



Neuropathy can occur in HCP but complicates less than 20 per cent of attacks.


SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++++

HCP can cause episodes of neuromuscular weakness in association with acute
attacks.


SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++

Peripheral neuropathy seems to be associated with latent hereditary

hepatic porphyria (AIP, HCP, and VP), even in patients who have never had
symptoms.

SOURCE:
"Neuropathy in Latent Hereditary Hepatic Porphyria"
Drs. Mustajiki, & Seppalainen
British Medical Journal
May 1975
page 310
++++++++++++++

Acute peripheral neuropathy is always superimposed upon a pre-existing
chronic nerve fiber lesion.

This therefore shows that there is demonstrating the possibility of more
continuous lesions in hereditary coproporphyria."

SOURCE:
Journal of Neurology
1984
231: 99-101
++++++++++++++



In HCP the upper limbs and proximal muscles are worst affected and truncal
muscle paralysis may lead to respiratory paralysis.


SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Extensive fatigue and muscle weakness are usually a part of the clinical
manifestation of HCP.

SOURCE:
Hereditary Copropohyria

Pavel Martasek, MD
Seminars in Liver Disease
Vol. 18 No. 1 1998
+++++++++++++



Muscle weakness can be quite profound in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Acute porphyria including HCP, may present as respiratory failure.

SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
++++++++++++

Respiratory paralysis and bulbar paresis can occur in HCP.

SOURCE:
Lifelines:
Journal of Emergency Medicine
October 1998
123: 437-443

Emergency Treatment of the Porphyric Patient
Kirsch, N,.E., M.D.
+++++++++++++

Severe cases of HCP show fatalities from respiratory paralysis.

One should not underestimate acute attacks.

SOURCE:
Hereditary Copropohyria

Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
+++++++++++++++



Fatalities from respiratory paralysis in HCP suggests that one should not

underestimate acute attacks in HCP or any acute hepatic porphyria.

SOURCE:
Pavel Martasek, MD
"Hereditary Coproporphyria"
page 25
Seminars in LIver Disease
Vol. 18 No. 1998
++++++++++++

Photosensitivity resembling that of PCT occurs in about a third of the cases of
HCP.

SOURCE:
CLINICAL FEATURES OF THE PORPHYRIAS
Dr. Maureen Poh-Fitzpatrick M.D.
Clinics in Bermatology
1998
++++++++++++

There is demonstrating the possibility of more continuous lesions in hereditary
coproporphyria.

SOURCE:
Journal of Neurology
1984
231: 99-101
++++++++++++++

The symptoms of porphyria still cannot be completely explained by our
present understanding of the biochemical defects of porphyria.

SOURCE:
Understanding Porphyria Symptomology
M. Tokomyko MD
International Congress on Porphyria
Paris, France
July 1999
+++++++++++++++

The porphyrias are puzzling since the metabolic defect is confined to
non-neural tissue, and no known diffusible intermediate can cause the observed
neurological symptoms of AIP,. HCP and VP.

SOURCE:
Understanding Porphyria Symptomology
M. Tokomyko MD
International Congress on Porphyria
Paris, France
July 1999
+++++++++++

ANS (altered neurologic state) can occur in HCP patients especially when
hyponatremia is present.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

Sensory loss can be found in 15% of attackswhile cranial nerve involvement
accounts of 9%.

Similiar findings are found among all AIP, VP and HCP patients.

SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Motgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++

Severe hyponatraemia, due to inappropriate secretion of antidiuretic
hormone, complicates some attacks and sometimes presents with
deterioration in the conscious level.

SOURCE:
Porphyria Research Unit
Department of Medicine
Brisbane, Queensland
1999
++++++++++++++

ANS (altered neurologic state) can occur in HCP patients especially when
hyponatremia is present.


SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++




Confusion and mental changes resembling an acute psychosis may be present
during acute attacks but resolve with remission.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K
+++++++++++++

Long-term severe psychiatric illness is very uncommon in HCP.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K
+++++++++++++


In HCP acute attacks of abdominal and neurologic symptoms are precipitated
by the same factors that are important in acute intermittent porphyria (AIP) ,
including certain drugs (eg, barbiturates, sulfonamides) and steroids (especially
progesterone).

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
++++++++++++++

Disorientation may present in HCP patients.

Dr. Kenneth Carlson
Neuropsychiatric Division
++++++++++++++

Seizures are not uncommon in cases of HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

CNS abnormalities include seizures, delirium and coma.

SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
++++++++++++++++

Coma can be observed in HCP patients.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Confusion may be present during acute attacks in HCP patients.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Delirium can present in mental change associated with HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Delirium is often associated with the mental change that is present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

A mental change in HCP patients similar to acute psychosis can be observed in
patients during acute attacks.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++++

Mental changes resembling an acute psychosis may be present during acute
attacks in HCP patients but resolve with remission.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Mental confusion can occur in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++



In HCP porphyrins absorb visible light strongly.

SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++

Exposure to strong visible light (sunlight) may cause pruritis, edema and so
forth.

The most damaging wavelength is 405 nm, which is the peak of the absorption
spectra of porphyrins.

The porphyrins absorb visible light strongly in Hereditary Coproporphyria
(HCP).

SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++

Exposure to strong visible light (sunlight) may cause pruritis, edema and so
forth in HCP patients.

SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++

Pruritis is often seen in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

The most damaging wavelength for HCP patients is 405 nm, which is the peak
of the absorption spectra of porphyrins.

This wavelength is not blocked by window glass.


SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++

HCP has neurological symptoms as well as cutaneous.

SOURCE:
The Porphyrias
A. Kappas et al.
1995, pp 2103-2159
+++++++++++

Clinical manifestations are identical to AIP, except for cutaneous photosensitivity
that occurs in one third of cases.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison’s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

The dermopathy resulting from the photosensitivity of HCP includes blistering.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++++

The dermopathy resulting from the photosensitivity of HCP includes urticaria.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++++

Some Hereditary Coproporphyria (HCP) patients develop skin photosensitivity.
and must avoid sunlight.

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

HCP patients with photosensitivity must avoid sunlight.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++

HCP has cutaneous symptoms.

SOURCE:
The Porphyrias
A. Kappas et al.
1995, pp 2103-2159
The Metabolic and Molecular Bases of Inherited Disease
vol. 2,
(C.R. Scriver et al., eds.), McGraw Hill
++++++++++++++

HCP is a form of hepatic porphyria that is similar to AIP, except that some
patients develop skin photosensitivity.

SOURCE:

Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++

The treatment of the cutaneous manifestations of HCP may be difficult.

SOURCE:
MEDIC'S HANDBOOK
1995
+++++++++++++++

The use of beta carotene for treatment of the cutaneous manifestations in HCP
is not considered beneficial.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++


Beta carotene therapy which is used in protoporphyria is not effective in HCP.

SOURCE:
MEDIC'S HANDBOOK
1995
+++++++++++++++

In HCP 20 - 30% of known cases include skin problems.

SOURCE:
Porphyria Resources
United Medical Services
1996
++++++++++++

Alopecia may occur in the more severely expressed cases of HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++


There is the possibility of continuous lesions in hereditary coproporphyria.

SOURCE:
Journal of Neurology
1984
231: 99-101
+++++++++++++++




Tachycardia or a fast pulse is often seen in hereditary coproporphyria (HCP).

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++


Hypertension may persist to some extent between attacks.

SOURCE:
Porphyria Research Unit
Department of Medicine
Brisbane, Queensland
1999
+++++++++++++
In Coproporphyria (HCP), skin disease also is present but not as commonly as
the neurovisceral symptoms.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The etiology of the skin aspects of Coproporphyria (HCP), may be the deposition
of formed porphyrins in the skin that react with sunlight and lead to skin damage.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Although Coproporphyria (HCP), patients with acute neurovisceral attacks
always have elevations of porphobilinogen and ALA, researchers still are
unclear about how this leads to the symptomatic disease because most patients
with the genetic defect have excessive porphyrin secretion but no symptoms.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Coproporphyria (HCP) is 20 times less common than acute intermittent porphyria
(AIP). in the United States.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
There are far fewer HCP patients than AIP.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++
Researchers feel that Coproporphyria (HCP) is a less severe disease than acute
AIP, but deaths have been reported in improperly
treated cases.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Researchers feel that women with Coproporphyria (HCP) tend to be
symptomatic more than men are, but the data are sparse.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Most patients with Coproporphyria
(HCP) porphyria become symptomatic
at age 18-40 years. Attacks are rate before puberty or after age 40 years.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Coproporphyria (HCP) displays both neurovisceral and skin manifestations.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Neurovisceral symptoms of Coproporphyria (HCP) includes peripheral
neuropathy.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Seizures are commonly associated with
active Coproporphyria (HCP).
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++
Delirium can present in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Hereditary coproporphyria (HCP) combines the clinical features of acute and
cutaneous porphyria.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Hereditary Coproporphyria (HCP) : clinical manifestations are identical to AIP,
except for cutaneous photosensitivity that occurs in one third of cases.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison’s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

During acute attacks, it is impossible to distinguish clinically and laboratorially
HCP from AIIP.

Treatment is the same of AIP.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison’s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
+++++++++++++


Hereditary Coproporphyria (HCP is also known as Porphyria Hepatica.

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

Hereditary Coproporphyria (HCP) is also known as Coproporphyria

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

Hereditary Coproporphyria (HCP) is an autosomal dominant form of hepatic
porphyria.

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

Hereditary Coproporphyria (HCP) is very similar to Acute Intermittent Porphyria
(AIP), although it is usually a less severe disease.

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

The classic mental, neurological and abdominal symptoms are often observed
in HCP patients, but there appear to be atypical clinical features and
laboratory findings that may lead to underdiagnosis.

SOURCE:
Hereditary coproporphyria
Downey, D. C.
British Journal of Clinical Practice
1994 48:97-9
++++++++++++

HCP can present as acute muscle pain, fever and weakness.

SOURCE:
Arthritis and Rheumatism
March 1997 Vol. 40 No. 3
page 586
+++++++++++



In HCP many carriers are completely asymptomatic, while others complain of
intermittent or chronic low-grade symptoms without avcute exacerbations.

SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++

Laboratory features of HCP include Reduced coproporphyinogen oxidase
in erythrocytes; and, Elevated fecal coproporphyrin ± protoporphyrin between
attacks .

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++++

Hypertrichosis may occur in expressed cases of HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
++++++++++

During acute attacks, whether abdominal pain is mild or severe, in most cases of
HCP, the recurring abdominal discomfort has caused patients to seek
examination.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++

HCP is a milder disease than AIP.

Nevertheless one should not underestimate acute attacks.

SOURCE:
Hereditary Copropohyria
Seminars in Liver Disease
Vol. 18 No. 1 1998
+++++++++++++++

Reliable assays for the deficient enzyme in HCP are not generally available.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++

Abdominal pain is often very severe in HCP.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++
In hereditary coproporphyria (HCP), excretion may return to normal or near
normal within a week or so after the onset of symptoms.

SOURCE:
Front line tests for the investigation of suspected porphyria.
A C Deacon, G H Elder.
Journal of Clinical Pathology.
July 2001
v54 i7 p500.
++++++++++

Diminished activity of Cpg oxidase in hepatocytes is definitive for a diagnosis
of HCP.

SOURCE:
Columbia Health Systems
Medical Educational
1999
+++++++++



If the HCP enzyme is measured, red blood cells should not be used.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++

HCP is less common than the two other acute porphyrias.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++

The HCP enzyme is found in mitochondria which are not present in red blood
cells.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++

Hereditary coproporphyria (HCP) is similar to acute intermittent porphyria (AIP),
although it is less common.

SOURCE:

The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++

Acute porphyria including HCP, may present as respiratory failure.

SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
++++++++++++



Profused sweating may occur with manifestation of HCP.


SOURCE:
Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++++++++++

In HCP there are gastrointestinal symptoms.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

Hereditary coproporphyria (HCP) is similar to acute intermittent porphyria,
although it is less common, is often milder, and occasionally is associated with
photosensitivity.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++

HCP is reportedly more often latent than is AIP.

The number of reported cases are fewer as well.

SOURCE:
D. Montgomery Bissell
Hepatic Porphyrias
Chapter 31
Diseases of the Liver
6th Edition / Lippincott
+++++++++++


Gastrointestinal symptoms often present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

There is no racial predominance in HCP.

SOURCE:

"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++

Hereditary coproporphyria (pronounce) is a defect of hepatic
coproporphyrinogen oxidase.

Large amounts of coproporphyrin III are excreted in the feces.

SOURCE:
The Porphyrias
Medical School
University of Utah
++++++++++++

The genetic location of HCP can be found at Chromosome 3q12.

SOURCE:

The Neuromuscular Disease Center
Washington University School of Medicine
St. Louis, MO.
++++++++++++

Hereditary Coproporphyria (HCP) is an autosomal dominant porphyria.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Hereditary Coproporphyria Clinical manifestations are identical to AIP, except for
cutaneous photosensitivity that occurs in one third of cases.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
++++++++++++++++++++



Extensive fatigue is usually a part of the clinical manifestation of HCP.

SOURCE:
Hereditary Copropohyria
Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
+++++++++++++



During acute attacks, it is impossible to distinguish clinically and laboratorially
HCP from AIP.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
++++++++++++++++++++

Treatment for HCP is the same as that of AIP.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
++++++++++++++++++++

Extreme fatigue is often seen in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

HCP is autosomal Dominant .

SOURCE:
The Neuromuscular Disease Center
Washington University School of Medicine
St. Louis, MO.
++++++++++++


Clinical featuresof nervous system involvement in hepatic porphyria can be quite
different in HCP from those observed in the classical acute attack of hepatic
poprhyria.

SOURCE:
Hereditary Coprophyria
C. Casali et. al.
Journal of Neurology
1984
+++++++++++

Abdominal pain is often very severe in HCP.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

Abdominal pain is generally present in the onset of HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

The symptoms of porphyria still cannot be completely explained by our
present understanding of the biochemical defects of porphyria.

SOURCE:
Understanding Porphyria Symptomology
M. Tokomyko MD
International Congress on Porphyria
Paris, France
July 1999
+++++++++++++++

In all cases of porphyria including HCP, there is an identifiable abnormality of
the enzymes.Medical science as we enter the millenium can still not explain the
neurological symptoms experienced in porphyria.

SOURCE:
Understanding Porphyria Symptomology
M. Tokomyko MD
International Congress on Porphyria
Paris, France
July 1999
+++++++++++++++

The porphyrias are puzzling since the metabolic defect is confined to
non-neural tissue, and no known diffusible intermediate can cause the observed
neurological symptoms of AIP,. HCP and VP.

SOURCE:
Understanding Porphyria Symptomology
M. Tokomyko MD
International Congress on Porphyria
Paris, France
July 1999
+++++++++++

Acute porphyria symptoms in HCP can be quite variable.

SOURCE:
Susceptibility to Environmental Chemicals
The International Congress on Hazardous Waste
Atlanta Georgeia
1995
Dr. William E. Morton M.D.
+++++++++++++


Porphyria symptoms can be ranging from severe acute attacks to continuing
low-grade smoldering symptoms to asymptomatic latency.

SOURCE:
Susceptibility to Environmental Chemicals
The International Congress on Hazardous Waste
Atlanta Georgeia
1995
Dr. William E. Morton M.D.
+++++++++++++

HCP is a milder disease than AIP.

SOURCE:
Hereditary Copropohyria
Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
+++++++++++++++

In hereditary coproporphyria (HCP), excretion may return to normal or near
normal within a week or so after the onset of symptoms.

If screening tests are negative and clinical suspicion of acute porphyria remains,
it is essential to determine urinary PBG and ALA by a quantitative method and to
analyse porphyrins in plasma and, if plasma fluorescence scanning excludes
VP, faeces.

If all these investigations are normal, acute porphyria is excluded as the cause
of the symptoms

SOURCE:
Front line tests for the investigation of suspected porphyria.
A C Deacon, G H Elder.
Journal of Clinical Pathology.
July 2001
v54 i7 p500.
++++++++++++++

Severe cases of HCP show fatalities from respiratory paralysis.

One should not underestimate acute attacks.

SOURCE:
Hereditary Copropohyria
Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
+++++++++++++++

In AIP, HCP and VP, many carriers are completely asymptomatic, while others
complain of intermittent or chronic low-grade symptoms without avcute
exacerbations."

SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++

Hereditary coproporphyria (HCP) is a defect of hepatic coproporphyrinogen
oxidase.

SOURCE:
The Porphyrias
Medical School
University of Utah
++++++++++

Large amounts of coproporphyrin III are excreted in the feces. of HCP patients.

SOURCE:
The Porphyrias
Medical School
University of Utah
++++++++++


Extensive fatigue and muscle weakness are usually a part of the clinical
manifestation of HCP.

SOURCE:

Hereditary Copropohyria
Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
+++++++++++



Fatalities from respiratory paralysis in HCP suggests that one should not
underestimate acute attacks in HCP or any acute hepatic porphyria.

SOURCE:
Pavel Martasek, MD
"Hereditary Coproporphyria"
page 25
Seminars in LIver Disease
Vol. 18 No. 1998
++++++++++++



HCP ischaracterized by episodic acute attacks that consist of various
neuro-psychiatric symptoms and signs, such as abdominal pain, vomiting,
constipation, hypertension, profused sweating, tremors, seizure, peripheral
neuropathies, and tachycardia associated with increased excretion of
porphyrins and porphyrin precursors.


SOURCE:
Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++


Severe abdominal pain without signs of perittonism is seen in almost all attacks
of HCP.


SOURCE:
Acute Porphyrias: Pathogenesis of Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++


Respiratory paralysis and bulbar paresis can occur in HCP.

SOURCE:
Lifelines:
Journal of Emergency Medicine
October 1998
123: 437-443
Emergency Treatment of the Porphyric Patient
Kirsch, N,.E., M.D.
+++++++++++++

Respiratory paralysis and bulbar paresis in HCP can be most life threatening
and medical personnel should be on aleart for signs of associated
breathing difficulty or inability to speak.

SOURCE:
Lifelines:
Journal of Emergency Medicine
October 1998
123: 437-443
Emergency Treatment of the Porphyric Patient
Kirsch, N,.E., M.D.
++++++++++++


Sensory loss can be found in 15% of attackswhile cranial nerve involvement
accounts of 9%.

Similiar findings are found among all AIP, VP and HCP patients.

SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Motgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++

In hereditary coproporphyria (HCP), excretion may return to normal or near
normal within a week or so after the onset of symptoms.

SOURCE:

Front line tests for the investigation of suspected porphyria.
A C Deacon, G H Elder.
Journal of Clinical Pathology.
July 2001
54 i7 p500.
++++++++++

HCP is a milder disease than AIP.

SOURCE:
Hereditary Copropohyria
Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
++++++++++++

HCP show fatalities from respiratory paralysis.

One should not underestimate acute attacks.

SOURCE:
Hereditary Copropohyria
Pavel Martasek, MD
Seminars in Livefr Disease
Vol. 18 No. 1 1998
++++++++++++++




Although HCP is rare, there is evidence that a relationship between porphyria
and epsilepsy exists.

SOURCE:
Hereditary Coproporphyria
Journal of Neurology
1984
231: 99-101
++++++++++++

In HCP 4 out of 6 report cases of epsilepsy.

Therefore clinical presentation of porphyria syndrome accompanied by the
epsilepsy seems to be particularly frequent in HCP.

It is well known in HCP that the children are more prone to seizure activity.

SOURCE:
Hereditary Coproporphyria
Journal of Neurology
1984
231: 99-101
++++++++++++

Hyponatremia often present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

A severe hyponatremia can develop from inappropriate release of
antidiuretic hormone, gastrointestinal loss and possibly renal loss.

Other associated problems in some patients may be seen with increased serum
binding globulin, increased cholesterol, and increased amylase.

SOURCE:
Lifelines:
Journal of Emergency Medicine
October 1998
123: 437-443
Emergency Treatment of the Porphyric Patient
Kirsch, N,.E., M.D.
+++++++++++++++

Convulsions and ANS can present during attacks of HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

Severe hyponatraemia, due to inappropriate secretion of antidiuretic
hormone, complicates some attacks and sometimes presents as convulsions or
deterioration in the conscious level.

SOURCE:
Porphyria Research Unit
Department of Medicine
Brisbane, Queensland
1999
++++++++++++++

ANS (altered neurologic state) can occur in HCP patients especially when
hyponatremia is present.


SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++


Dysuria is a painful urination found in HCP acute attacks.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++


Dysuria is a manifesation often found during acute attacks of HCP porphyria.

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
+++++++++++++

There can be Bladder dysfunction and dysuria in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

Dysuria and bladder dysfunction have been described in HCP.

Urinary retension may require catherization.

SOURCE:
"The Inherited Porphyrias"
Anderson, Karl E, McGovern, Margaret.,
Astrin, Kenneth & Desnick, Robert J.
Emery and RImon's Principles and Practices of Medical Genetics
3rd Edition
Churchill, New York, 1996
+++++++++++

Bladder dysfunction is often noted during the onset of acute attacks of
HCP .

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++++


Constipation and vomiting may also be present in the onset of HCP.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

Management of HCP patients is the same as for acute intermittent porphyria and
VP patients.


SOURCE:
MEDIC'S HANDBOOK
1995
++++++++++

Clinical manifestations are identical to AIP, except for cutaneous photosensitivity
that occurs in one third of cases.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison’s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

Acute attacks in HCP porphyric patients are mainly pharmacologically induced.

SOURCE:
Acute Porphyrias
J.W. Stevens et. al.
Anesthesia & Analgesia
Volume 82 No. 2
February 1996
++++++++++++

It is important for anesthetists and doctors to know which drugs to avoid in
HCP to prevent the triggering of an acute attack.

SOURCE:
Acute Porphyrias
J.W. Stevens et. al.
Anesthesia & Analgesia
Volume 82 No. 2
February 1996
++++++++++++

Severe abdominal pain HCP without signs of perittonism is seen in almost all
attacks.

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++

Elevated urine porphyrins and decreased levels of coproporphyrinogen oxidase
confirmed the correct diagnosis of hereditary coproporphyria in patients
misdiagnosed as MS.

SOURCE:
Hereditary coproporphyria: an imitator of multiple sclerosis.
Macy JA, Gilroy J, Perrin JC.
Department of PM&R,
William Beaumont Hospital,
Royal Oak, MI 48072.
Archives of Physical Medicine and Rehabilatation
1991 Aug;72(9):703-4
++++++++++++


The diagnosis of HCP is based on finding increased amounts of ALA, PBG, and
coproporphyrin in urine and excess coproporphyrin in feces.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
++++++++++++

In HCP acute attacks of abdominal and neurologic symptoms are precipitated
by the same factors that are important in acute intermittent porphyria (AIP) ,
including certain drugs (eg, barbiturates, sulfonamides) and steroids (especially
progesterone).

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
++++++++++++++

Coproporphyrin excretion is increased in HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++++

Slight peripheral neuropathy seems to be associated with latent HCP even in
patients who have never had symptoms.

SOURCE:
"Neuropathy in Latent Hereditary Hepatic Porphyria"
Drs. Mustajiki, & Seppalainen
British Medical Journal
May 1975
page 310
+++++++++

Treatment of HCP acute attacks is the same as that for acute intermittent
porphyria.

SOURCE:

The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++++



The majority of HCP patients are assymptomatic.

SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
+++++++++++

Diminished activity of CPG oxidase in hepatocytes is definitive for a diagnosis
of HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++

Treatment of HCP acute attacks is the same as that for acute intermittent
porphyria.


SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
++++++++++++


Porphyria hepatica II is another name for Hereditary coproporphyria.
(HCP).

SOURCE:
Lister Hill National Center for Biomedical CommunicationsNational Library of
Medicine
National Institutes of Health
Department of Health & Human Services
+++++++++++++++

The diagnosis of HCP is based on finding increased amounts of ALA, PBG, and
coproporphyrin in urine and excess coproporphyrin in feces.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++

A predominant or exclusive presence of fecal coproporphyrin is more
suggestive of HCP than of variegate porphyria in which fecal coproporphyrin and
protoporphyrin concentrations are usually about equal.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++]


Urinary ALA, PBG, and uroporphyrin may be increased during acute attacks of
HCP.

These normalize between attacks more commonly than in acute intermittent
porphyria.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++

Coproporphyrinogen oxidase deficiency can be demonstrated in cells other than
erythrocytes, but this is not recommended for routine diagnosis of HCP.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++

Treatment of acute attacks is the same as that for acute intermittent porphyria.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++


Hereditary Coproporphyria (HCP)is caused by an enzyme deficiency.

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

The diagnosis of Hereditary Coproporphyria (HCP) is established by finding
excess coproporphyrin in urine and stool (other types of porphyrins show little or
no increase).

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

Urinary ALA and PBG are increased during acute attacks of Hereditary
Coproporphyria (HCP), but may become normal on recovery.

SOURCE:
Dr. Alex Razlova
Hemotology
+++++++++++

HCP patients are at a high risk of HCC (hepatocellular carcinoma).

SOURCE:
Occurrence of hepatocellular carcinoma in a case of hereditary coproporphyria.
Andant C, Puy H, Deybach JC, Soule JC, Nordmann Y.
Am J Gastroenterol.
1997 Aug;92(8):1389-90.
++++++++++++

Signs and symptoms of HCP include gastrointestinal symptoms.

SOURCE:

The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Hereditary coproporphyria results from low levels of the enzyme responsible for
the sixth step in heme production.

SOURCE:
Hereditary coproporphyria
Disease Resource Book
Alliance Medical Systems
+++++++++++

CNS in HCP abnormalities include seizures, delirium and coma.

SOURCE:

The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
+++++++++++++

3q12 is the genetic location for HCP.

This means that the CPO gene is located on the long arm of chromosome 3 at
position 12.

SOURCE:
CPO
Lister Hill National Center for Biomedical Communications
National Library of Medicine
National Institutes of Health
Department of Health & Human Services
++++++++++++

HCP is a form of hepatic porphyria that is similar to AIP, except that some
patients develop skin photosensitivity.

SOURCE:

Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++

During acute attacks, it is impossible to distinguish HCP from AIP.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
University Medical School, Brazil
+++++++++++++

Treatment is the same of AIP.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
University Medical School, Brazil
+++++++++++++

Precautions and treatment for acute attacks of HCP are as described for AIP.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++

The first line of treatment for HCP is intravenous infusion of glucose.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++

Management of HCP and VP patients is the same as for acute intermittent
porphyria.

SOURCE:
MEDIC'S HANDBOOK
1995
+++++++++++++++

During acute attacks, it is impossible to distinguish clinically and laboratorially
HCP from AIIP.

Treatment is the same as that of AIP.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison’s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

Avoid foods such as red grapes, cabbage, brussel sprouts, red plums, all
which may contain chemicals that in large amounts can stimulate heme and
porphyrin synthesis.

SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++

Hereditary coproporphyria (HCP) is a more latent disease.

SOURCE:
Anderson KE, Bloomer JR,
Bolwell B, Lichtin AE.
Department of Dermatology,
Columbia University College of
Physicians and Surgeons, New
York, New York, USA
+++++++++++++

Constipation may present in HCP patients.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

Alopecia may occur in the more severely expressed cases of HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++


Coma can occur in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Neuropathy can occur in HCP but complicates less than 20 per cent of attacks.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K
+++++++++++++++

Seizures are not uncommon in cases of HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

CNS abnormalities include seizures, delirium and coma.

SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
++++++++++++++++

Delirium can present in mental change associated with HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Confusion and mental changes resembling an acute psychosis may be present
during acute attacks in HCP patients but resolve with remission.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Mental confusion can occur in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
Mental changes in Coproporphyria (HCP) include delirium.
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++
Coma has been observed in HCP patients.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++
Coma may occur in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Some HCP have been observed in coma.
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++++++++
Coproporphyria (HCP) symptoms consist
of autonomic neuropathies including
vomiting, and hypertension.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Depression may present in Coproporphyria (HCP).
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++
The sequence of events in attacks usually is
abdominal pain then psychiatric symptoms
(eg, hysteria), then peripheral neuropathies
in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Hysteria may present in HCP.
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++
Coproporphyria (HCP) patients can
have very severe abdominal pain that
lasts for several days.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Pain of short duration (minutes) or
chronic abdominal pain does not
develop in coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
The pain found in Coproporphyria (HCP)
often is epigastric and is colicky in nature.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The exact mechanism by which the porphyrin
precursors lead to symptoms in Coproporphyria
(HCP) is unknown.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Patients often are free of pain between
attacks in Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Constipation in Coproporphyria (HCP) is
common and can be very severe.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Nausea and vomiting frequently are present in
Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Skin manifestations in Coproporphyria (HCP) are varied.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The skin disease of Coproporphyria (HCP) is similar to porphyria cutanea tarda.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
With long-term (not acute) sun exposure,
Coproporphyria (HCP) patients can develop
vesicals and bullae.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
If Coproporphyria (HCP) patients are symptomatic with coproporphyria, they
tend to have neurovisceral symptoms
rather than skin symptoms.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Neurovisceral symptoms are more common than skin
problems in HCP.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++
Blisters form in sun-exposed areas and can
evolve into chronic scarred areas of fragile
skin in Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Coproporphyria (HCP) patients also may develop excessive hair growth in
sun-exposed areas.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Neurologic manifestations in Coproporphyria
(HCP) are similar to those indentified in AIP.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++
HCP Patients with coproporphyria can have central
nervous system signs, including seizures.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
There are often mental status
changes in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Cortical blindness may present in HCP.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++
There may be cortical blindness in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Coma can present in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Coproporphyria (HCP) patients often experience peripheral neuropathies that
predominantly are motor neuropathies and can mimic Guillain-Barré syndrome.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The PN weakness in Coproporphyria (HCP) usually starts in the lower limbs and
ascends, but neuropathies occur in any nerve distribution.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Pain may be experienced in a variety of
locations in HCP.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++


Hereditary coproporphyria is a less common form of acute attack porphyria,and
is also autosomally dominant.

SOURCE:
Dr. Poh-Fitzpatrick, professor,
Department of Dermatology
Columbia University, New York
Dermatology Times,
Jun96, Vol. 17 Issue 6, p14, 2p
+++++++++++++++++

Hereditary Coprorporphyria is another name for HCP.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++



Coproporphyria is the full name for HCP.

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++++

Hereditary coproporphyria (HCP) is a genetic disorder of heme and porphyria
biosynthesis.

SOURCE:
Harderoporphyria
Nordmann, Yves et. al.
Journal of CLinical Investigation
Vol. 72 1139-1149
September 1983
+++++++++++++

Hereditary coproporphyria (HCP) is a defect of hepatic coproporphyrinogen
oxidase.

Large amounts of coproporphyrin III are excreted in the feces.

SOURCE:
The Porphyrias
Medical School
University of Utah
+++++++++++++


Hereditary coproporphyria is a rare type of porphyria that can have acute
(severe but usually not long-lasting) symptoms along with symptoms that affect
the skin.

SOURCE:
Lister Hill National Center for
Biomedical Communications
National Institutes of Health
Department of Health & Human Services
+++++++++++
Hereditary coproporphyria (HCP) combines the clinical features of acute and
cutaneous porphyria.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++

Coproporphyrinogen oxidase deficiency is another name for hereditary
coproporphyria (HCP).

SOURCE:

Disease Resource Book
Alliance Medical Systems
+++++++++++

Coproporphyrinogen oxidase deficiency is another name used for hereditary
coproporphyria porphyria [HCP].

SOURCE:
Lister Hill National Center for
Biomedical Communications
National Institutes of Health
Department of Health & Human Services
++++++++++


HCP is a form of hepatic porphyria that is similar to AIP, except that some
patients develop skin photosensitivity.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++

Coproporphyria is the full name for HCP.

SOURCE:
Dr. Robert Johnson M.D.
++++++++++++

HCP is an autosomal dominant disorder resulting from a deficiency of
coproporphyrinogen oxidase.

SOURCE:

The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++

Porphyria hepatica II is an alternative name for hereditary coproporphyria.

SOURCE:
Disease Resource Book
Alliance Medical Systems
++++++++++++

The CPO gene produces an enzyme called coproporphyrinogen oxidase.

SOURCE:
CPO
Lister Hill National Center for Biomedical Communications
National Library of Medicine
National Institutes of Health
Department of Health & Human Services
++++++++++++++++

CPX deficiency is another name for hereditary coproporphyria (HCP).

SOURCE:
Disease Resource Book
Alliance Medical Systems
++++++++++++

Coproporphyrinogen oxidase deficiency is another name used for hereditary
coproporphyria porphyria [HCP].

SOURCE:
Lister Hill National Center for Biomedical Communications
National Library of Medicine
National Institutes of Health
Department of Health & Human Services
++++++++++++++

CPO deficiency is another name for the hepatic porphyia known as HCP.

SOURCE:
Lister Hill National Center for
Biomedical Communications
National Library of Medicine
National Institutes of Health
Department of Health & Human Services
+++++++++++++

Hereditary coproporphyria is a defect of hepatic coproporphyrinogen oxidase.

Large amounts of coproporphyrin III are excreted in the feces."

SOURCE:
The Porphyrias
Medical School
University of Utah
++++++++++++
The deficient enzyme is coproporphyrinogen oxidase.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++
The enzyme defect for HCP is Coproporphyrinogen 3 oxidase.

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++

The genetic location for HCP is Chromosome 3q12; Dominant

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++

Coproporphyria is identifiable by the enzyme defect of coproporphyrinogen
oxidase.

The inheritance pattern for HCP if coded AR.

The genetic location of HCP is 3q.

SOURCE:
Porphyria Genetics
Dr. Robert Huskey
+++++++++++++
Heme is the portion of hemoglobin that carries oxygen in the blood from the
lungs to the rest of the body.

Each of the steps in heme production is controlled by a separate gene.

Coproporphyrinogen oxidase removes carbon and oxygen from

coproporphyrinogen III (the product of the fifth step in the production of heme) to
form protoporphyrinogen IX in mitochondria.

Two additional enzymes must modify protoporphyrinogen IX before it becomes
heme.

SOURCE:
CPO
Lister Hill National Center for Biomedical Communications
National Library of Medicine
National Institutes of Health
Department of Health & Human Services
++++++++++++++++
Coproporphyrinuria may occur in cholestasis, and poisoning with heavy metals
(eg lead, mercury) or certain chemicals (eg hexachlorbenzene).

SOURCE:
Australia Porphyria PAthology Laboratory
++++++++++++
Hereditary Corpropohyria (HCP) is due to decreased coproporphyrinogen
oxidase activity which causes increased excretion of fecal coproporphyrin.

SOURCE:
Medic Handbook
1995
+++++++++++++++

In Hereditary Corpropohyria (HCP) urine coproporphyrin may or may not be
increased.

SOURCE:
Medic Handbook
1995
+++++++++++++

In acute attacks of Hereditary Corpropohyria (HCP) there can be produced
sweating, bulbar paralysis, respiratory paralysis, coma, seizures and ocular
problems assoicated with the porphyria.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
Diagnostic criteria are poorly-defined in the porphyrias.

SOURCE:
The Porphyrias
Dr. Ed R. Friedlander
Pathology Department
University of Health Sciences
College of Osteopathic Medicine
+++++++++++
HCP is inherited as autosomal dominant.

SOURCE:
Harderoporphyria
Nordmann, Yves et. al.
Journal of Clinical Investigation
Vol. 72 1139-1149
September 1983
++++++++++
HCP is autosomal dominant in inheritance.

SOURCE:

Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++
(HCP) Hereditary coproporphyria is a rarely diagnosed disease and is one of the
acute porphyrias.

SOURCE:
Hereditary coproporphyria
Downey, D. C.
British Journal of Clinical Practice
1994 48:97-9
+++++++++++

Signs and symptoms of HCP include gastrointestinal symptoms.

SOURCE:

The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++++++

HCP is marked with gastrointestinal symptoms including vomiting, constipation
and abdominal pain which can be very severe.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++

The diagnosis of HCP is established by finding excess coproporphyrin
(especially coproporphyrin type III) in urine and stool.

Other types of porphyrins show little or no increase.

Urinary ALA and PBG are increased during acute attacks but may become
normal on recovery.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
+++++++++++++

Signs and symptoms of HCP include gastrointestinal symptoms.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

HCP includes gastrointestinal symptoms.

Abdominal pain is often very severe in HCP.

Constipation and vomiting may often present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++

Cardiovacular

Cardiovascular features occur in 70 per cent of HCP cases and include
hypertension and tachycardia.

SOURCE:

The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

Urinary findings

During acute episodes of HCP, the production and urinary excretion of
delta-aminolevulinic acid (ALA) and porphobilinogen (PBG) are increased.

SOURCE:
Porphyria Resources
United Medical Services
1996
++++++++++++++

Coproporphyrin and porphobilinogen excretion in urine are markedly increased
during acute attacks of hereditary coproporphyria, increase of urinary
uroporphyrin may be found, and increased fecal coproporphyrin III is described.

SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++

During acute episodes of HCP the production and urinary excretion of
delta-aminolevulinic acid (ALA) and porphobilinogen (PBG) are increased.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++++++


In hereditary coproporphyria (HCP), excretion may return to normal or near
normal within a week or so after the onset of symptoms.

SOURCE:
Front line tests for the investigation of suspected porphyria.
A C Deacon, G H Elder.
Journal of Clinical Pathology.
July 2001
v54 i7 p500.
++++++++++++



Clinical manifestations

Clinical manifestations are identical to AIP, except for cutaneous photosensitivity
that occurs in one third of cases.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison's Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

Most ommon symptom

The initial and commonest manifestation of HCP is abdominal pain, which can
be diffuse or localized, colicky.

SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
+++++++++++

Hereditary coproporphyria is often misdiagnosed when small, clinically
unimportant elevations of coproporphyribn are found.

SOURCE:
The Porphyrias
Pierach, Dr. Claus A.
Conn's Current Therapy
1998
+++++++++++

HCP as all acute porphyria symptoms, can be quite variable.

Acute porphyria symptoms can be ranging from severe acute attacks to
continuing low-grade smoldering symptoms to asymptomatic latency."

SOURCE:
Susceptibility to Environmental Chemicals
The International Congress on Hazardous Waste
Atlanta Georgeia
1995
Dr. William E. Morton M.D.
+++++++++++++++


Clinical features of HCP include acute attacks & neuropathy, as well as Skin
photosensitivity.

SOURCE:
Dr. Robert Johnson M.D.
++++++++++++

Sensory loss

Sensory loss in HCP can be found in 15% of acute attacks.

Similiar findings are found among all AIP, VP and HCP patients.

SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
++++++++++++++

About one-sixth of HCP experience regular sensory loss associated with their
HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++

Cranial nerve involvement

Cranial nerve involvement in HCP patients has been found to be about 9%.

Similiar findings are found among all AIP, VP and HCP patients.

SOURCE:
"The Porphyrias"
Diseases of the Liver
6th Edition 1987
Dr. Montgomery Bissell MD
University of California-San Francisco
*Member of the APF medical advisory board
+++++++++++++
Hereditary coproporphyria (HCP) combines the clinical features of acute and
cutaneous porphyria.

HCP is less common than the two other acute porphyrias.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++++

Hereditary coproporphyria (HCP) is the least common of the autosomal
dominant acute hepatic porphyrias.

SOURCE:
Characterization of mutations
in the CPO gene in British
patients demonstrates
absence of genotype-phenotype
correlation and identifies
relationship between
hereditary coproporphyria
and harderoporphyria.
Elder GH. et. al.
Centre Francais des Porphyries
Universite Paris VII
Hopital Louis Mourier
Colombes Cedex, France.
American Journal of Human Genetics
2001 May;68(5):1130-8.
+++++++++++++++

Mutations

At least 34 mutations that cause hereditary coproporphyria and the
more severe form of the disorder, harderoporphyria, have been identified in the
CPO gene.

SOURCE:
CPO
Lister Hill National Center for Biomedical Communications
National Library of Medicine
National Institutes of Health
Department of Health & Human Services
+++++++++++++++++

The molecular relationship between these Harderoporphyria and HCP
has not been defined.

SOURCE:
Characterization of mutations in the CPO gene in British patients demonstrates
absence of genotype-phenotype correlation and identifies relationship between
hereditary coproporphyria and harderoporphyria.
Elder GH. et. al.
Centre Francais des Porphyries
Universite Paris VII
Hopital Louis Mourier
Colombes Cedex, France.
American Journal of Human Genetics
2001 May;68(5):1130-8
+++++++++++++

HCP is more heterogeneous than other acute porphyrias.

SOURCE:
Characterization of mutations in the
CPO gene in British patients
demonstrates absence of
genotype-phenotype correlation
and identifies relationship between
hereditary coproporphyria and
harderoporphyria.
Elder GH. et. al.
Centre Francais des Porphyries
Universite Paris VII
Hopital Louis Mourier
Colombes Cedex, France.
American Journal of Human Genetics
2001 May;68(5):1130-8.
++++++++++++++++

Photosensitivity


HCP Clinical manifestations are identical to AIP,with the addiiton of cutaneous
photosensitivity that occurs in one third of cases.

SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
University Medical School, Brazil
++++++++++++++
In an acute attack of HCP, urine when passed becomes dark on
standing, due to porphobilinogen (PBG) polymerising to uroporphyrin
and a brownish-red pigment porphobilin.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++++

During acute attacks of HCP , it is impossible to distinguish clinically
and laboratorially HCP from AIIP.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison's Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

Symptoms of HCP are the same as those of AIP with the addition
of cutaneous features.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison's Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++

Atypical presentation

HCP can have atypical clinical presentations depending on the organ or tissue
that is being primarily affected.

It is believed that many of the vague modern illnesses are actually
manifestations of unrecognized porphyria.

SOURCE:
Porphyria: a new perspective.
Downey, D. 1996.
Medical Hypotheses 46:378-82..
++++++++++++++++



The classic mental, neurological and abdominal symptoms are often observed
in HCP patients, but there appear to be atypical clinical features and
laboratory findings that may lead to underdiagnosis.

SOURCE:
Hereditary coproporphyria
Downey, D. C.
British Journal of Clinical Practice
1994 48:97-9
+++++++++++++++++

Laboratory features of HCP include Reduced coproporphyinogen oxidase i
n erythrocytes; and, Elevated fecal coproporphyrin ± protoporphyrin between
attacks .

SOURCE:
Dr. Robert Johnson M.D.
++++++++++++++++


HCP resembles two other forms of inherited porphyria, AIP and VP.

SOURCE:
Harderoporphyria
Nordmann, Yves et. al.
Journal of Clinical Investigation
Vol. 72 1139-1149
September 1983
+++++++++++++

Homozygous cases


A few homozygous cases of HCP have been described.

SOURCE:

The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++++


Profused sweating

Profused sweating may occur with manifestation of HCP.


SOURCE:

Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++++++++

Blladder dysfunciton

Bladder dysfunction is often noted during the onset of acute attacks of HCP .

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++

Nausea

Nausea may present in HCP patients.

SOURCE:

The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++

Vomiting

Vomiting may present in HCP patients.

SOURCE:

The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++++

Hypertrichosis

Hypertrichosis may occur in expressed cases of HCP.

SOURCE:
Porphyria Resources
United Medical Services
1996
+++++++++++++

Pyrexia

Fever (pyrexia) may accompany acute episodes of acute hepatic porphyria.

SOURCE:
Porphyria Resources
United Medical Services
1996
++++++++++++++


HCP ischaracterized by episodic acute attacks that consist of various
neuro-psychiatric symptoms.

SOURCE:
Journal of Photodermatololy, Photoimmunology and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++++++++++

HCP has neurological symptoms as well as cutaneous.

SOURCE:
The Porphyrias
A. Kappas et al.
1995, pp 2103-2159
The Metabolic and Molecular Bases of Inherited Disease
vol. 2,
(C.R. Scriver et al., eds.), McGraw Hill
+++++++++++++++

Long-term severe psychiatric illness in HCP is very uncommon.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++++

Treatment

Treatment for HCP is the same of AIP.

SOURCE:

The Porphyrias
Meyer, Urs A.
Harrison's Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++++++

HCP is an Acute disease which has been induced by anabolic steroid
methandrostenolone.

HCP remains an inherited form of porphyria.

SOURCE:
Anderson KE, Bloomer JR,
Bolwell B, Lichtin AE.
Department of Dermatology,
Columbia University College of
Physicians and Surgeons, New
York, New York, USA
++++++++++++++
The management of Hereditary Corpropohyria (HCP) is the same as for AIP and
VP.

SOURCE:
Medic Handbook
1995
+++++++++++
Te first line of treatment for HCP is iv infusion of glucose.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++

Latency

Hereditary coproporphyria (HCP) is a more latent disease.

SOURCE:
Anderson KE, Bloomer JR,
Bolwell B, Lichtin AE.
Department of Dermatology,
Columbia University College of
Physicians and Surgeons, New
York, New York, USA
++++++++++++++++

Associated medical conditions

Among the conditions that researchers have found to be associated with
biochemically confirmed HCP are: fibromyalgia, CFS, MS,
lupus, MCS and others.

SOURCE:
Porphyria: a new perspective.
Downey, D. 1996.
Medical Hypotheses 46:378-82..
+++++++++++++++

Death

The acute porphyrias including AIP, VP and HCP may give rise to acute,
potentially fatal crises.

SOURCE:
The little imitator
Porphyria: a Neuropsychiatric Disorder
Helen Crimlisk
Neurology, Neurosurgery & Psychiatry
1997
62; 319-328
++++++++++++++

Supporting evidence for the diagnosis of MS in HCP patients is of a
demyelinating disease included three oligoclonal bands in the patient's cerebral
spinal fluid, a prolonged visual evoked response bilaterally, abnormal sensory
evoked potentials, and an area of increased signal in theposterior cervical cord
suggestive of demyelination that was demonstrated on magnetic resonance
imaging (MRI).

SOURCE:
Hereditary coproporphyria: an imitator of multiple sclerosis.
Macy JA, Gilroy J, Perrin JC.
Department of PM&R,
William Beaumont Hospital,
Royal Oak, MI 48072.
Archives of Physical Medicine and Rehabilatation
1991 Aug;72(9):703-4
++++++++++++

Hereditary coproporphyria: an imitator of multiple sclerosis.

SOURCE:
Macy JA, Gilroy J, Perrin JC.
Department of PM&R,
William Beaumont Hospital,
Royal Oak, MI 48072.
Archives of Physical Medicine and Rehabilatation
1991 Aug;72(9):703-4



Undiagnosis

HCP is considered the least common of the acute porphyrias.

However, HCP may be more prevalent than was once thought because
disordered heme metabolism and differing tissue sensitivity may lead to atypical
clinicalpresentations; thus, many cases may go unrecognized or are perhaps
defined as another entity, such as fibromyalgia."

SOURCE:
Acute porphyric disorders.
Moore, A. W., & J. M. Coke
Oral Surgury; Oral Medicine;
Oral Pathology; Oral Radiology
& Endrocrinology 2000, 90:257-62.
+++++++++++++++++

Severity

HCP is a milder disease than AIP.

Nevertheless one should not underestimate acute attacks.

SOURCE:
Hereditary Copropohyria
Seminars in Liver Disease
Vol. 18 No. 1 1998
++++++++++++++
During acute attacks, it is impossible to distinguish clinically and laboratorially
HCP from AIIP.

Treatment is the same of AIP.

SOURCE:

The Porphyrias
Meyer, Urs A.
Harrison's Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++
Precautions and treatment for acute attacks are as described for AIP.

The first line of treatment is intravenous infusion of glucose.

SOURCE:

Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++
Diffuse pain, especially in the upper body, can be observed in Coproporphyria
(HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Coproporphyria (HCP) Patients also can develop autonomic neuropathies.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
A major autonomic neuropathy in Coproporphyria (HCP)
os that of hypertension.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Tachycardia is often present in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Many HCP patients experienced a rapid heart beat
during attacks.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++
Psychiatric manifestations may develop
in Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Coproporphyria (HCP) patients can have a
wide variety of psychiatric symptoms.
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++
Coproporphyria (HCP) patients usually have
concurrent neurologic or abdominal symptoms.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Tachycardia develops in 30-80% of
Coproporphyria (HCP) patients.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
A fast racing heart pulse may be experienced during
episodes of HCP.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++
Fever can be present in some Coproporphyria
(HCP) patients.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
HCP patients may exoperience pyrexia (fever).
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++++++
Hypertension develops in 50% of Coproporphyria
(HCP) cases and may persist between attacks.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Neurologic manifestations are commonly
associated with Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Typically, the neuropathy Coproporphyria
(HCP) of is a motor neuropathy that is more
predominant in the lower limbs.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Areflexia is observed during the
examination ofCoproporphyria (HCP)
patients; however, any nerve can be involved.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Cranial neuropathies are observed,
and theCoproporphyria (HCP) patient
may have cortical blindness.
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Abdominal manifestations are commonly associated with Coproporphyria (HCP)
Despite the intense pain, the findings
on abdominal examination often are
nonspecific in Coproporphyria (HCP)
Skin manifestations can be varied in
Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Coproporphyria (HCP) patients can have
blisters, chronic erosion, and areas of
excessive hair growth.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Hypertrichosis is commonly found in HCP patients.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++++
Skin damage develops in sun-exposed
areas of the skin in Coproporphyria (HCP)
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Abdominal films sometimes demonstrate
an ileus in Coproporphyria (HCP),
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Findings on cranial CT scans are normal
in Coproporphyria (HCP),
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Brain MRI scans occasionally show signs
of increased edema in Coproporphyria (HCP),
patients with very severe episodes of the disease.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Like AIP, Coproporphyria (HCP) is due to a
combination of a genetic enzyme defect and
acquired causes that become symptomatic in rare cases.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
In patients with Coproporphyria (HCP), the function of coproporphyrinogen
oxidase is only 40-60% of normal.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Many Coproporphyria (HCP), patients with defects
in coproporphyrinogen oxidase never have any symptoms.
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
The classic inducers of Coproporphyria (HCP), porphyria are chemicals or
situations that boost heme synthesis.
This includes fasting and many medications.
SOURCE:
Hereditary Coproporphyria Porphyria
Francisco Talavera, PharmD, PhD
++++++++++++++++++++
Although extensive lists of safe and unsafe
drugs exist, many of these are based on
anecdotes or laboratory evidence rather
than meeting strict criteria.
SOURCE:
Hereditary Coproporphyria Porphyria
Francisco Talavera, PharmD, PhD
++++++++++++++++++++
In general, drugs that lead to increased activity
of the hepatic P450 system (eg, phenobarbital,
sulfonamides, estrogens, alcohol) are associated
with Coproporphyria (HCP), porphyria.
SOURCE:
Hereditary Coproporphyria Porphyria
Francisco Talavera, PharmD, PhD
++++++++++++++++++++
Fasting can trigger an attack of Coproporphyria (HCP),
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Many Coproporphyria (HCP), attacks will occur,
however, without any obvious provocation
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Stool coproporphyrins are necessary in the diagnosis of
Coproporphyria (HCP),
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Physicians establish the diagnosis of
Coproporphyria (HCP) by demonstrating
excess secretion of coproporphyrins in the stool.
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Stool coproporphyrins in Coproporphyria (HCP),
are markedly elevated, usually 10-200 times
greater than control samples.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
Urine coproporphyrins are indicative of a HCP
diagonosis only partially.
Urine porphyrins vary, but usually the
urine coproporphyrins also are markedly
elevated in Coproporphyria (HCP).
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Coproporphyria (HCP), has estremely high
elevations of urine coproporphrins during
acute attacks of the disease.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
If Coproporphyria (HCP), patients are having
neurovisceral symptoms, the urine
porphobilinogen is elevated.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Mild elevations of urine coproporphyrins
(eg, as high as 2 times the reference range)
are common and nonspecific in Coproporphyria (HCP),
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Fasting, subtle liver disease, or normal variations
are the most common causes of elevated urine
coproporphyrins.
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Coproporphyria (HCP), patients who truly have
symptoms due to coproporphyria have marked
elevations of urine, and especially stool,
coproporphyrins.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
As with AIP, Coproporphyria (HCP), patients who
are experiencing neurovisceral symptoms due to
coproporphyria have elevations in urine porphobilinogen.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The most common diagnostic error in
proporphyria is to label patients with mild elevations of urine coproporphyrins as
having porphyria.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Hyponatremia (syndrome of inappropriate
secretion of antidiuretic hormone [SIADH])
and mild leukocytosis are other nonspecific
signs during Coproporphyria (HCP), a attack.
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Although coproporphyria is caused by a
defective enzyme, there is little use in
measuring the activity of coproporphyrinogen oxidase.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++
The vast majority of patients who have the
defective enzyme do not have any symptoms
of the disease.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
Furthermore, the only available clinical assay has
been withdrawn due to problems with high rates of
false-positive results.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++
The diagnosis of a porphyria
attack rests on demonstration of
excessive excretion of porphyrins
and porphyrin precursors.
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++
Coproporphyria (HCP), patients should
receive a high-carbohydrate diet during the attack.
SOURCE:
Sheryl WIlson MNS, RD
++++++++++++++++++++
Intravenous glucose should be administered
if Coproporphyria (HCP), patients cannot eat.
SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Between attacks, Coproporphyria (HCP),
patients should eat a constant balanced diet
rather than one that is extremely rich in glucose.
SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Imaging studies are not helpful in
Coproporphyria (HCP),
SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++




Coproporphyria (HCP) is one of the porphyrias,
a group of diseases that involves defects in heme
metabolism and that results in excessive secretion
of porphyrins and porphyrin precursors.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++


Coproporphyria (HCP) manifests with abdominal pain,
neuropathies, constipation, and skin changes.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Constipation in Coproporphyria (HCP) is a part
of numerous autonomic neuropathies commonly
associated with the disease.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

The presence of colicky abdominal pain is
commonly found in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++



Coproporphyria (HCP) is an autosomal dominant
disease that results from defects in the enzyme
coproporphyrinogen oxidase.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

The coproporphyrinogen in Coproporphyria
(HCP) oxidase enzyme speeds the conversion
of coproporphyrinogen to protoporphyrinogen.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

In coproporphyria (HCP) , the porphyrin precursors
porphobilinogen and amino-levulinic acid (ALA)
accumulate, as well as the formed porphyrin
coproporphyrin.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++



The predominant problem in Coproporphyria
(HCP) is neurologic damage that leads to
peripheral and autonomic neuropathies and
the psychiatric manifestations.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

In Coproporphyria (HCP), skin disease also
is present but not as commonly as the
neurovisceral symptoms.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


The etiology of the skin aspects of

Coproporphyria (HCP), may be the deposition

of formed porphyrins in the skin that react with

sunlight and lead to skin damage.



SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++




Although Coproporphyria (HCP), patients

with acute neurovisceral attacks always have

elevations of porphobilinogen and ALA,

researchers still are unclear about how this

leads to the symptomatic disease because

most patients with the genetic defect have

excessive porphyrin secretion but no symptoms.



SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++







Coproporphyria (HCP) is 20 times less
common than acute intermittent porphyria
(AIP). in the United States.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

There are far fewer HCP patients than AIP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++
Researchers feel that Coproporphyria (HCP)

is a less severe disease than acute AIP,

but deaths have been reported in improperly

treated cases.



SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Researchers feel that women with

Coproporphyria (HCP) tend to be symptomatic

more than men are, but the data are sparse.



SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++








Most patients with Coproporphyria

(HCP) porphyria become symptomatic

at age 18-40 years. Attacks are rate

before puberty or after age 40 years.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++
Coproporphyria (HCP) displays both

neurovisceral and skin manifestations.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Neurovisceral symptoms of Coproporphyria
(HCP) includes peripheral neuropathy.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++



Seizures are commonly associated with
active Coproporphyria (HCP).

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++

Delirium can present in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Mental changes in Coproporphyria (HCP) include delirium.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++


Coma has been observed in HCP patients.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

Coma may occur in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Some HCP have been observed in coma.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++++++++


Coproporphyria (HCP) symptoms consist
of autonomic neuropathies including
vomiting, and hypertension.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++



Depression may present in Coproporphyria (HCP).

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++

The sequence of events in attacks usually is
abdominal pain then psychiatric symptoms
(eg, hysteria), then peripheral neuropathies
in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Hysteria may present in HCP.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++


Coproporphyria (HCP) patients can
have very severe abdominal pain that
lasts for several days.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Pain of short duration (minutes) or
chronic abdominal pain does not
develop in coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

The pain found in Coproporphyria (HCP)
often is epigastric and is colicky in nature.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

The exact mechanism by which the porphyrin
precursors lead to symptoms in Coproporphyria
(HCP) is unknown.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++



Patients often are free of pain between
attacks in Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Constipation in Coproporphyria (HCP) is
common and can be very severe.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Nausea and vomiting frequently are present in
Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Skin manifestations in Coproporphyria (HCP) are varied.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

The skin disease of Coproporphyria
(HCP) is similar to porphyria cutanea tarda.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++


With long-term (not acute) sun exposure,
Coproporphyria (HCP) patients can develop
vesicals and bullae.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

If Coproporphyria (HCP) patients are symptomatic with coproporphyria, they
tend to have neurovisceral symptoms
rather than skin symptoms.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

Neurovisceral symptoms are more common than skin
problems in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++

Blisters form in sun-exposed areas and can
evolve into chronic scarred areas of fragile
skin in Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Coproporphyria (HCP) patients also may develop excessive hair growth in
sun-exposed areas.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Neurologic manifestations in Coproporphyria
(HCP) are similar to those indentified in AIP.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++

HCP Patients with coproporphyria can have central
nervous system signs, including seizures.


SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


There are often mental status
changes in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Cortical blindness may present in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++


There may be cortical blindness in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

Coma can present in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Coproporphyria (HCP) patients often experience
peripheral neuropathies that predominantly are
motor neuropathies and can mimic
Guillain-Barré syndrome.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


The PN weakness in Coproporphyria (HCP) usually
starts in the lower limbs and ascends, but neuropathies
occur in any nerve distribution.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

Pain may be experienced in a variety of
locations in HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++

Diffuse pain, especially in the upper body, can be observed
in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Coproporphyria (HCP) Patients also can develop autonomic neuropathies.
SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


A major autonomic neuropathy in Coproporphyria (HCP)
os that of hypertension.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Tachycardia is often present in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Many HCP patients experienced a rapid heart beat
during attacks.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++

Psychiatric manifestations may develop
in Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++



Coproporphyria (HCP) patients can have a
wide variety of psychiatric symptoms.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++++++++

Coproporphyria (HCP) patients usually have
concurrent neurologic or abdominal symptoms.


SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

Tachycardia develops in 30-80% of
Coproporphyria (HCP) patients.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

A fast racing heart pulse may be experienced during
episodes of HCP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++

Fever can be present in some Coproporphyria
(HCP) patients.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

HCP patients may exoperience pyrexia (fever).

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++++++
Hypertension develops in 50% of Coproporphyria
(HCP) cases and may persist between attacks.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

Neurologic manifestations are commonly
associated with Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Typically, the neuropathy Coproporphyria
(HCP) of is a motor neuropathy that is more
predominant in the lower limbs.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Areflexia is observed during the
examination ofCoproporphyria (HCP)
patients; however, any nerve can be involved.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Cranial neuropathies are observed,
and theCoproporphyria (HCP) patient
may have cortical blindness.

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++


Abdominal manifestations are commonly associated with Coproporphyria (HCP)

Despite the intense pain, the findings
on abdominal examination often are
nonspecific in Coproporphyria (HCP)

Skin manifestations can be varied in
Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Coproporphyria (HCP) patients can have
blisters, chronic erosion, and areas of
excessive hair growth.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Hypertrichosis is commonly found in HCP patients.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++++

Skin damage develops in sun-exposed
areas of the skin in Coproporphyria (HCP)

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++

Abdominal films sometimes demonstrate
an ileus in Coproporphyria (HCP),

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Findings on cranial CT scans are normal
in Coproporphyria (HCP),

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++


Brain MRI scans occasionally show signs
of increased edema in Coproporphyria (HCP),
patients with very severe episodes of the disease.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

Like AIP, Coproporphyria (HCP) is due to a
combination of a genetic enzyme defect and
acquired causes that become symptomatic in rare cases.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

In patients with Coproporphyria (HCP), the function of coproporphyrinogen
oxidase is only 40-60% of normal.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

Many Coproporphyria (HCP), patients with defects
in coproporphyrinogen oxidase never have any symptoms.

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++

The classic inducers of Coproporphyria (HCP), porphyria are chemicals or
situations that boost heme synthesis.

This includes fasting and many medications.

SOURCE:
Hereditary Coproporphyria Porphyria
Francisco Talavera, PharmD, PhD
++++++++++++++++++++


Although extensive lists of safe and unsafe

drugs exist, many of these are based on

anecdotes or laboratory evidence rather

than meeting strict criteria.

SOURCE:
Hereditary Coproporphyria Porphyria
Francisco Talavera, PharmD, PhD
++++++++++++++++++++



In general, drugs that lead to increased activity

of the hepatic P450 system (eg, phenobarbital,

sulfonamides, estrogens, alcohol) are associated

with Coproporphyria (HCP), porphyria.

SOURCE:
Hereditary Coproporphyria Porphyria
Francisco Talavera, PharmD, PhD
++++++++++++++++++++


Fasting can trigger an attack of Coproporphyria (HCP),

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++


Many Coproporphyria (HCP), attacks will occur,

however, without any obvious provocation

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++

Stool coproporphyrins are necessary in the diagnosis of
Coproporphyria (HCP),

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Physicians establish the diagnosis of
Coproporphyria (HCP) by demonstrating
excess secretion of coproporphyrins in the stool.

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++


Stool coproporphyrins in Coproporphyria (HCP),
are markedly elevated, usually 10-200 times
greater than control samples.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++


Urine coproporphyrins are indicative of a HCP
diagonosis only partially.


Urine porphyrins vary, but usually the
urine coproporphyrins also are markedly
elevated in Coproporphyria (HCP).

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++


Coproporphyria (HCP), has estremely high
elevations of urine coproporphrins during
acute attacks of the disease.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

If Coproporphyria (HCP), patients are having
neurovisceral symptoms, the urine
porphobilinogen is elevated.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++


Mild elevations of urine coproporphyrins
(eg, as high as 2 times the reference range)
are common and nonspecific in Coproporphyria (HCP),

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Fasting, subtle liver disease, or normal variations
are the most common causes of elevated urine
coproporphyrins.

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++

Coproporphyria (HCP), patients who truly have
symptoms due to coproporphyria have marked
elevations of urine, and especially stool,
coproporphyrins.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++



As with AIP, Coproporphyria (HCP), patients who
are experiencing neurovisceral symptoms due to
coproporphyria have elevations in urine porphobilinogen.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


The most common diagnostic error in
proporphyria is to label patients with mild elevations of urine coproporphyrins as
having porphyria.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++


Hyponatremia (syndrome of inappropriate
secretion of antidiuretic hormone [SIADH])
and mild leukocytosis are other nonspecific
signs during Coproporphyria (HCP), a attack.

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++

Although coproporphyria is caused by a
defective enzyme, there is little use in
measuring the activity of coproporphyrinogen oxidase.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++

The vast majority of patients who have the
defective enzyme do not have any symptoms
of the disease.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++


Furthermore, the only available clinical assay has
been withdrawn due to problems with high rates of
false-positive results.

SOURCE:
Hereditary Coproporphyria Porphyria
Emmanuel C Besa, MD,
Department of Internal Medicine
Division of Hematology and Oncology
Drexel University College of Medicine
++++++++++++++++++

The diagnosis of a porphyria
attack rests on demonstration of
excessive excretion of porphyrins
and porphyrin precursors.

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++


Coproporphyria (HCP), patients should

receive a high-carbohydrate diet during the attack.

SOURCE:
Sheryl WIlson MNS, RD

++++++++++++++++++++



Intravenous glucose should be administered

if Coproporphyria (HCP), patients cannot eat.

SOURCE:
Hereditary Coproporphyria Porphyria
Clarence Sarkodee-Adoo, MD,
Department of Bone Marrow Transplantation
City of Hope Samaritan
++++++++++++++++++++


Between attacks, Coproporphyria (HCP),

patients should eat a constant balanced diet

rather than one that is extremely rich in glucose.

SOURCE:
Hereditary Coproporphyria Porphyria
Rajalaxmi McKenna, MD, FACP,
Southwest Medical Consultants, SC,
Department of Medicine,
Good Samaritan Hospital,
Advocate Health Systems
+++++++++++++++++++++



Imaging studies are not helpful in
Coproporphyria (HCP),

SOURCE:
Hereditary Coproporphyria Porphyria
Marcel E Conrad, MD
Distinguished Professor of Medicine
University of South Alabama
Mobile Infirmary Medical Center
+++++++++++++++++++++++++


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PORPHYRIA FACTS is a medical education website dedicated to helping you focus your research on the inherited metabolic diseases known as the "Porphyrias".

PORPHYRIA FACTS is for individuals seeking information on Porphyria. The specific focus is on education, and research in the porphyrias.

PORPHYRIA FACTS present medical citations from medical professionals and others qualified and knowledgeable in the porphyrias.


PORPHYRIA FACTS takes no responsibility for medical information that is discussed here. You are encouraged to always seek medical advice before trying any new protocols. Open communication with your physician is important in developing effective treatment protocols.