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PORPHYRIA FACTS:
AIP - ASSOCIATED CO-EXISTING CONDITIONS
In AIP there may be alterations in carbohydrate, lipid, water, and electrolyte
metabolism in addition to clinically inapparent endocrine abnormalities.
SOURCE:
Acute intermittent porphyria:
clinical and selected research aspects.
Tschudy DP, Valsamis M, Magnussen CR.
Annals of Internal Medicine
1975 Dec;
83(6):851-64.
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Cardiovascular
Studies by Kauppinen and Mustajoki showed that AIP patients taking
hydrochlorothiazide, beta-blockers, and nitrates remained free of the commonly
associated cardiovascular symptoms associated with AIP.
SOURCE:
Treatment of Hypertension in a Patient With Acute Intermittent Porphyria?
Bruce Gardner, MD
Associate Clinical Professor of Family Medicine
University of Washington, Seattle
Attending Physician, Family Medicine
Swedish Hospital and Medical Center, Seattle
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Severe hypertension and tachycardia in AIP can lead to both cardiac arrythmia
and renal complications in AIP.
SOURCE:
Robert Johnson MD
Internal Medicine
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Cortical blindness
Patients with acute intermittent porphyria have presented with acute cortical
blindness, for which deleterious vasospasm in both posterior cerebral arteries
has been inferred.
SOURCE:
Transient Cortical Blindness and Bioccipital Brain Lesions
in Acute Intermittent Porphyria
Hugo Kupferschmidt, MD; et. al.
Annals of Internal Medicine
American College of Physicians
15 October 1995
123:598-600.
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In AIP there is optic neuritis or cortica blindness can be found during acute
attacks.
SOURCE:
Psoriasis, Ichthyosis, and Porphyria
Drs. Griffith Steiner, & Robert Arffa
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In AIP the cranial nerves manifestations in may lead to optic nerve atrophy.
SOURCE:
Metabolic Disorders
Porphyrias: Clinical Manifestations, Diagnosis and Treatment
Bernardo Haddock Lobo Goulart & Samanta Teixeira Basto
University Medical School, Brazil
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Creatinine
Women with AIP have significantly higher mean serum creatinine levels.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
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Diabetes
Diabetes II is commonly associated with AIP.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++
Diabetic metabolism may prevent the development of HCC in patients with AIP.
SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea UniversityUmea, Sweden
Lakartidningen.
2001 Dec 19;98(51-52):5874-6.
++++++++++++++
In AIP attacks there is an escalating metabolic chain reaction leading
to heme deficiency and increased levels of porphyrin precursors.
This reaction is inhibited by treatment.
In a population-based study none of the patients showed AIP symptoms after
the onset of their diabetes.
SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea University
Lakartidningen.
2001 Dec 19;98(51-52):5874-6
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Hypercholesteremia
Hypercholesterolaemia is found in human hepatic porphyria mostly during acute
attacks, often falling to near normal levels during remission.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
+++++++++++
High cholesterol is commonly found as a co-existing conditions with AIP
patients.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
In Acute Intermittent Porphyria (AIP) many patients have hypercholesterolemia.
SOURCE:
The Porphyrias
Medical Handbook
1997
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Hypercholesterolaemia is commonly associated with AIP.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
++++++++++
Acute intermittent porphyria can present with hypercholesterolemia.
SOURCE:
Abnormal thyroid function and hypercholesterolemia in acute intermittent
porphyria.
Shiue JW, et. al.
Taiwan Yi Xue Hui Za Zhi.
1989 Jul;88(7):729-31.
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High cholesterol is commonly associated with AIP.
SOURCE:
Prevalence of hyperlipidemia in persons with hypertension and/or diabetes
mellitus
Assmann G & Schulte H.
American Heart Journal
1988; 116: 1713 24.
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Cholesterol is manufactured in the liver for normal body functions including the
production of hormones, bile acid, and Vitamin D.
Cholesterol is transported in the blood to be used by all parts of the body.
High cholesterol is commonly found in patients with acute intermittent porphyria.
SOURCE:
Poune Saberi, M.D., M.P.H.
Family Practice and Community Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
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Hyponatremia
A severe hyponatremia can develop in AIP from inappropriate release of
antidiuretic hormone, gastrointestinal loss and possibly renal loss.
SOURCE:
Acute Intermittent Porphyria
Guide to Disease
Columbia Health Systems
1996
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Hyponatremia is commonly found in AIP patients during acute attacks.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
Hypertension
Hypertension is found in 56% of AIP patients.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
++++++++++
Hypertension is found in 16% of latent AIP patients.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
++++++++++
Hypertension is more common in acute AIP patients than in latent AIP patients.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
++++++++++
Hypertension is more common in patients with manifest AIP than in those with
latent AIP.
SOURCE:
Hypertension in patients with acute intermittent porphyria.
Journal of Internal Medicine
1994 Aug;236(2):169-75
Andersson C, Lithner F.
Primary Health Care Centre,
Arjeplog, Sweden.
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Hypertension is often found present during acute attacks of porphyria in AIP
patients.
SOURCE:
Porphyria Resources
United Medical Services
1996
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High blood pressure readings duiring acute attacks are commonly found in AIP
patients.
Tachycardia is also present.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
AIP is associated with hypertension during acute attacks.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
++++++++++
Hypertension is rarely found in AIP during periods of remission.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
++++++++++
Increased hypertension in AIP patients has been suggested to be due to the
presence of xcessive amounts of porphyrin metabolities causing cytotoxic
or vasopastic renal vascular lesions, thereby inducing raised blood pressure.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
+++++++++++
Hypertension and renal disease can present in patients with AIP.
SOURCE:
Journal of Internal Medicine
1994 Aug;236(2):169-75
Andersson C, Lithner F.
Primary Health Care Centre,
Arjeplog, Sweden.
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Hypothalamic dysfunction
Central nervous dysfunction in AIP can be seen as well as hypothalamic
dysfunction.
SOURCE:
Acute Intermittent Porphyria
Guide to Disease
Columbia Health Systems
1996
+++++++++++
Hypothyroidism
Hypothyroidism is often a co-existing condition in persons with AIP.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
Kidneys
Renal disease may be due to AIP.
SOURCE:
Journal of Internal Medicine
1994 Aug;236(2):169-75
Andersson C, Lithner F.
Primary Health Care Centre,
Arjeplog, Sweden.
+++++++++++
Liver
Hepatic dysfunctions in AIP are common.
SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
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Liver cancer
Hepatic carcinoma may develop in older patients with acute intermittent
porphyria (AIP).
SOURCE:
Acute intermittent porphyria
Brekke OL. et. al.
Medisinsk avdeling Nordland Sentralsykehus 8092 Bodo.
Tidsskr Norwegian Laegeforen
2002 Apr 30;122(11):1102-5
+++++++++++
Liver cancer (HCC) is often found in AIP patients.
AIP patients are at a much higher increase risk for HCC than the general
population.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++
In a study of AIP patients with hepatocellular carcinoma (HCC) none had
diabetes.
This implies that diabetic metabolism may prevent the development of HCC in
patients with AIP.
SOURCE:
Diabetic metabolism protective in severe acute intermittent porphyria
Andersson C, Lithner F.
Institute of Medicine
Umea UniversityUmea, Sweden
Lakartidningen.
2001 Dec 19;98(51-52):5874-6.
++++++++++++++
Lupus (SLE)
Lupus (SLE) has been found to be a co-existing conditions in AIP patients.
SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++
PN
Distal neuropathy has been assessed in patients with acute intermittent
porphyria (AIP).
The chronic neurological signs were symmetrical and similar to those in Type 1
diabetic patients.
SOURCE:
Neuropathy of the lower legs and feet in acute intermittent porphyria.
Wikberg A, Andersson C, Lithner F.
Institutionen for omvardnad, Universitetet, Umea
Lakartidningen.
2001 Sep 19;98(38):4038-41
+++++++++++++
Significant PN impairment has been found concerning perception, extensor
digitorum brevis test, lower leg pain, ankle and knee tendon reflexes but not
concerning dry feet, loss of forefoot arch and hammer toes, when comparing
patients with manifest vs. latent AIP.
The neurological signs were more severe in the diabetic patients.
SOURCE:
Neuropathy of the lower legs and feet in acute intermittent porphyria.
Wikberg A, Andersson C, Lithner F.
Institutionen for omvardnad, Universitetet, Umea
Lakartidningen.
2001 Sep 19;98(38):4038-41
+++++++++++++
AIP patients have more permanent quadriplegia and renal failure after severe
attacks.
SOURCE:
Neuropathy of the lower legs and feet in acute intermittent porphyria.
Wikberg A, Andersson C, Lithner F.
Institutionen for omvardnad, Universitetet, Umea
Lakartidningen.
2001 Sep 19;98(38):4038-41
+++++++++++++
Patients with manifest AIP have significantly more signs of distal chronic,
symmetrical neuropathy of axonal type than had patients with latent AIP.
SOURCE:
Neuropathy of the lower legs and feet in acute intermittent porphyria.
Wikberg A, Andersson C, Lithner F.
Institutionen for omvardnad, Universitetet, Umea
Lakartidningen.
2001 Sep 19;98(38):4038-41
+++++++++++++
Overall more serious neurological lesions (PN) appear to develop after severe
attacks.
SOURCE:
Neuropathy of the lower legs and feet in acute intermittent porphyria.
Wikberg A, Andersson C, Lithner F.
Institutionen for omvardnad, Universitetet, Umea
Lakartidningen.
2001 Sep 19;98(38):4038-41
+++++++++++++
Septicenua
Septic complications, such as pneumonia, septicemia, and urinary infection, are
frequent in 50% of AIP patients.
SOURCE:
Porphyric crisis: experience of 30 episodes (AIP)
Medicina
Buenas Aires
999;59(1):23-7
Morales Ortega X, et. al.
Departmento of Medicine
Occidente Hospital San Juan de Dios,
University of Chile, Santiago, Chile.
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Uremia
Uraemia is cited as the cause of death in 9.1% of AIP patients.
SOURCE:
Hypertension in patients with AIP
C. Andersson et. al.
Journal of Internal Medicine 1994
236: 169-175
+++++++++++
It should be remembered that AIP patients can develop other diseases, and
symptoms may not always be due to porphyria.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
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