[The hepatic porphyrias: experience with 105 cases].
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| Abstract |
Hepatic porphyria is a rare metabolic syndrome caused by abnormal enzyme activity in heme biosynthesis. Between 1974 and 1991; 105 patients have met criteria for diagnosis of hepatic porphyria based on typical clinical findings and/or laboratory abnormalities. According to type, 42% had porphyria cutanea tarda, 21% porphyria variegate, 15% protoporphyria, 6.7% acute intermittent porphyria, 6.7% coproporphyria and 1.9% porphyria due to porphobilinogen deficit. A proper classification was not established in 6.7% of patients. Porphyria cutanea tarda was more common in males (70%) and porphyria variegata, in females (90%). A family history of the disease was present in 33% of patients; 20% of patients were of European descent and 4% of Mapuche descent. Diagnosis was usually established in the third decade, somewhat later in porphyria cutanea tarda (45 years of age) and very early in protoporphyria. 10% of patients were asymptomatic and 29 patients developed at least one porphyric crisis. These were related to pregnancy in 6 patients, to hormone administration in 7, to antibiotics in 5. No cause was established in 21 cases. Severe crisis were successfully treated with Hematin. Venipuncture was used to treat 50% of patients with porphyria cutanea tarda with 95% success. Thus, hepatic porphyria is recognized with increasing frequency and can be treated successfully in most cases.
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| Authors | R Armas, C Wolff, P Krause, P Chaná, A Parraguez, J Soto |
| Journal | Revista medica de Chile (Rev Med Chil) Vol. 120 Issue 3 Pg. 259-66 (Mar 1992) ISSN: 0034-9887 [Print] Chile |
| Vernacular Title | Las porfirias hepáticas: experiencia con 105 casos. |
| PMID | 1342477 (Publication Type: English Abstract, Journal Article, Research Support, Non-U.S. Gov't, Review) |
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