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PORPHYRIA FACTS:
UROLOGICAL ASPECTS OF PORPHYRIA
Dysuria and bladder dysfunction have been described in AIP.
Urinary retension in AIP 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
+++++++++++
Urinary retention can be a feature of Acute Intermittent Porphyria (AIP).
SOURCE:
Columbia Health Care
1998
++++++++++
Urine color
The terms "porphyrin" and "porphyria" are derived from the Greek word
"porphyrus" meaning purple.
The urine in some forms of porphyria often is purple.
SOURCE:
Dr. Robert Johnson M.D
Internal Medicine
+++++++++
Seventy-two percent of porphyria patients experience red or dark urine.
SOURCE:
United Health Services
Medical Education Department
1999
++++++++++
The high level of porphyrins in porphyria patients is responsible for
discoloration of urine such as port-colored urine.
SOURCE:
Painful porphyrins
Julie L. McDowell
Diseases and disorders
American Chemical Society
2001
++++++++++++
Porphyria originally referred to the red to purple color of the urine of patients
affected by acute intermittent porphyria.
Other forms of porphyria produce urine that varies from pink to red to brown.
SOURCE:
Porphyria: Don't Forget to Look At the Skin
Dr. Michael W. Rich
Professor of Medicine
Internal Medicine
Northeastern Ohio Universities
College of Medicine, Rootstown
Summa Health System, Akron.
+++++++++
Did types of porphyria often produce different colors of urine.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++
In an acute attack, 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.
++++++++++++
Urine from some porphyria patients may be reddish in color due to the presence
of excess porphyrins and related substances, and the urine may darken after
standing in the light.
Excretion of rose-colored urine is consistent with the diagnosis of an acute
porphyria.
SOURCE:
"Acute intermittent porphyria in childhood.
A neglected diagnosis?"
Barclay, N. (1974).
Archives of Disturbed Children
49(5): 404-6.
++++++++++++++
Bladder dysfunction is often noted during the onset of acute attacks of
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
+++++++++++
Urinary retention
Urinary retention in AIP may require catherization.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
++++++++++++
Urinary retention often present is the acute porphyrias.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Urinary retention can be a feature of Acute Porphyrias.
SOURCE:
Columbia Health Care
1998
++++++++++++
URINE COLORATION
Discoloration of urine
The terminology "discoloration of urine" refers to any urine that appears different
from the usual straw-yellow color, or that is cloudy, dark, or blood-tinged.
Any changes in urine color, or the presence of an abnormal urine color that
cannot be linked to the consumption of a food or drug, should be reported to
your doctor.
SOURCE:
Andrew Koren, MD, Department of Nephrology
NYU-Mount Sinai Medical Center
New York, NY.
+++++++++
It is often the color change in urine that piques a physicians
diagnostic evaluation to include testing for the porphyrias.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
This is particularly important if it happens for longer than a day or two, or if there
are repeated episodes, especially with other
symptoms presenting such as in the acute porphyrias or with the
cutaneous symptoms with the rest of the porphyrias.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Some dyes used in candy may be excreted in the urine, and a wide variety of
drugs can discolor the urine.
Cloudy, murky, or turbid urine is characteristic of a urinary tract infection.
In cases of UTI's the urine may have an offensive smell or may be caused by
the presence of bacteria, mucus, white blood cells or red blood cells, epithelial
cells, fat, or phosphates.
Dark brown and clear urine is characteristic of a liver disorder such as acute
viral hepatitis or cirrhosis or some forms of porphyria.
SOURCE:
Andrew Koren, MD, Department of Nephrology
NYU-Mount Sinai Medical Center
New York, NY.
+++++++++
Pink, red, or smoky brown urine can be a side effect of a medication or caused
by the recent consumption of beets, blackberries or certain food colorings.
It is also characteristic of porphyria which can also cause urine to take on these
colors.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Dark yellow or orange urine can be caused by recent use of laxatives or
consumption of B complex vitamins or carotene.
SOURCE:
Andrew Koren, MD, Department of Nephrology
NYU-Mount Sinai Medical Center
New York, NY.
+++++++++
A cranberry, wine or purple hue is often indicative of acute porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
Orange urine is often caused by Pyridium rifampin, and warfarin.
Green or blue urine is due to the effect of artificial color in food or drug.
It may also result from medications including amitriptyline, indomethacin, and
doxorubicin.
SOURCE:
Andrew Koren, MD, Department of Nephrology
NYU-Mount Sinai Medical Center
New York, NY.
+++++++++
Diagnostic tests that may be performed due to urine color change include, blood
tests, liver function tests, and a urinalysis.
In knowledgeable labs a Watson-Schwartz tests will be performed.
If porphyria is suspected and has not been previously diagnosed, a 24 hour
urine test will be commenced immediately during a porphyria episode.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++
After seeing your health care provider, if a diagnosis was made by your health
care provider related to abnormal urine color, you may want to note that
diagnosis in your personal medical record.
It is important to get photocopies of all test results and charting notes stating a
diagnosis.
SOURCE:
Andrew Koren, MD, Department of Nephrology
NYU-Mount Sinai Medical Center
New York, NY.
+++++++++
Porphyria patients may occasionally notice discolored urine, either dark brown
or pink to red ("port-wine" colored).
This coloration is not due to blood.
Any change in urine color is most likely to occur during an illness reaction, and
the dark color may be more obvious after the urine has been left standing for a
few hours exposed to light or heat
SOURCE:
Evaluating Porphyrin Metabolism
Dr. Grace Ziem, MD
1996
+++++++
DYSURIA (PAINFUL URINATION)
Dysuria is a manifesation often found during acute attacks of porphyria."
SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
+++++++++++
Dysuria is the experiencing of painful or difficult urination.
SOURCE:
Dept. of Medical Oncology
University of Newcastle upon Tyne
++++++++++++++
Dysuria often is present during acute attacks of porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++
Dysuria is a manifesation often found during acute attacks of porphyria.
SOURCE:
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
+++++++++++
Dysuria and bladder dysfunction have been described in AIP.
Urinary retension in AIP 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
+++++++++++
Dysuria is having painful urination.
Dysuria is commonly associated with acute porphyria during acute attacks.
SOURCE:
Acute Porphyrias: Pathogenesis of Neurological Manifestations
Urs. A Meyers M.D. et. al.
1998
++++++++
Dysuria and bladder dysfunction have been described in AIP.
Urinary retension in AIP 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
++++++++++
Dysuria and bladder dysfunction have been described in AIP.
Urinary retension in AIP 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 - DYSURIA - FLUID RETENTION
Bladder dysfunction have been described in acute porphyria.
Urinary retention in 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 porphyria.
SOURCE:
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++++
Various neurological diseases and conditions can adversely affect lower urinary
tract (bladder and urinary sphincters) function leading to inability to urinate,
urinary incontinence, recurrent urinary tract infections, urinary stones, and even
kidney failure.
This is referred to as neurogenic bladder dysfunction.
Conditions that cause neurogenic bladder can include the acute porphyrias.
Managing the urinary bladder aggressively and correctly is of utmost
importance when these conditions exist since mismanagement can lead to the
serious complications.
Often acute porphyria patients will experience UTI's (urinary tract infections).
Having a UTI in turn will often trigger an acute episode of porphyria if the
infection is left untreated.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
Dysuria (painful urination) is a manifesation often found during acute attacks of
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
++++++++++++
Most patients can be managed with oral medications alone or in combination
with clean intermittent self catheterization (CISC) if a patient can not void.
When porphyria patients are hospitalized and have voiding disorder and bladder
distention, catherization is often employed.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
Some patients require surgical bladder augmentation or urinary diversion.
SOURCE:
Martin Morrison PA
Urology
++++++++
Voiding disorders are usually a result of neurologic conditions, such as acute
porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
Dysuria has been described in acute porphyrias.
Urinary retention 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
+++++++++++++++
Incontinence and urinary retention can cause social embarrassment and added
morbidity, such as infections, stones, or renal injury.
SOURCE:
Martin Morrison PA
Urology
++++++++
The sympathetic nervous system regulates the process of urine storage in the
bladder.
In contrast, the parasympathetic nervous system controls bladder contractions
and the passage of urine.
SOURCE:
Martin Morrison PA
Urology
++++++++
Parasympathetic nerve impulses travel from S2-S4 ventral gray matter via the
pelvic nerves to the ganglia near the bladder wall.
Postganglionic nerve impulses then travel to the smooth muscle cholinergic
receptors to produce bladder contraction.
Sympathetic efferent nerve fibers originate from the lateral gray column of the
spinal cord from T11-L2.
The sympathetic system has a long postganglionic chain that runs with the
hypogastric nerve to synapse with alpha-receptors and beta-receptors in the
bladder wall and bladder neck or internal sphincter.
Beta-receptors are responsible for mediating relaxation of the bladder with filling.
Alpha-receptors are responsible for tonically contracting the internal sphincter
during bladder filling.
The somatic efferent nerve fibers originate from the pudendal nucleus of S2-S4
and supply the external periurethral sphincter.
The external sphincter is under voluntary control and normally contracts in
response to coughing or the Valsalva maneuver or when a person actively tries
to prevent or halt urine flow.
Three areas of the central nervous system (CNS) (ie, the sacral micturition
center, the pontine micturition center, the cerebral cortex) control bladder
function.
The sacral micturition center is located at the S2-S4 levels and is responsible for
bladder contraction.
The pontine micturition center may play a role in coordinating relaxation of the
external sphincter with bladder contractions while the cerebral cortex plays an
inhibitory role in relation to the sacral micturition center.
SOURCE:
Martin Morrison PA
Urology
++++++++
Bladder disorders are reported in 40-90% of patients with MS.
Many porphyria patients experience co-existing MS.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
Lesions of the peripheral nerves or the sacral micturition center cause detrusor
areflexia that manifests as distended bladder with overflow incontinence.
SOURCE:
Martin Morrison PA
Urology
++++++++
Dysuria is a painful urination associated with acute attacks of porphyria.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++
Bladder dysfunction is often noted during the onset of acute attacks of 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
++++++++++
Dysuria and bladder dysfunction have been described
in AIP.
Urinary retension in AIP 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
+++++++++++
Urinary retention in AIP may require catherization."
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++
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