Acute porphyria in Trinidad - abstract

Prompted by reports of acute porphyria in continental Africa and India, and in African populations in the U.S.A., a prospective study was undertaken to determine prevalence and characteristics of acute porphyria amongst emergency admissions to the medical wards of a West Indian general hospital over a 30-month period. The population surveyed was about 500,000 persons mainly from African and Indian (Asian) descent. Criteria for diagnosis were suggestive clinical features, past medical history and the presence of excess of urinary porphobilinogen as determined by the Watson Schwartz test. Six unrelated cases of typical acute porphyria were identified from among 25,000 admissions. Sixty controls matched for age, sex and ethnic origin had a negative Watson Schwartz test. Patients ranged in age from 13 to 26 years and none had a family history of porphyria-type illness. Ethnic origin was not a distinctive feature and there was considerable morbidity due to non-recognition that acute porphyria is more common in West Indians than hitherto suspected and there should be greater efforts to diagnose the disease (AU)

Coexistence of porphyria cutanea tarda and systemic lupus erythematosus

The association of porphyria cutanea tarda with a well-documented case of systemic lupus erythematosus is reported. Renal histopathology is presented. Both conditions have been quiescent during the past year of follow-up (AU)

Acute intermittent porphyria in a 17-year-old Trinidadian girl

We report the first case of acute intermittent porphyria in Trinidad and Tobago. This condition is most commonly described in people of European origin and is considered to be rare in blacks. This girl is of African descent and has no family history of acute intermittent porphyria (AU)

Report on a case of porphyria in Surinam

A male adult Amerindian was after several disgnostic pitfalls recognized as a patient with acute intermittent porphyria. Because of an autosomal congenital transmitted error of metabolism a deficiency of the enzyme uroporphyrinogen I-synthetase (UPG I S) exists resulting in increased levels of delta-aminolevulinic acid (ALA) and porphobilogen(PBG). The Chinese powder containing pyrazolon together with an infectious disease might have been the provocative factors by inducing the enzyme delta-aminolevulinic acid-synthetase(ALA-S). Luminal is leading the long list of drugs to be avoided because of their precitating effect. General practioners, internists as well as surgeons, neurologists, psychiatrists, dermatologists and gynecologists should be aware of the different ways in which the porphyria group of diseases may present. The Watson-Schwartz test is a simple screening method to detect PBG in freshly voided urine when suspecting acute intermittent porphyria. (Au).

Tetanus and prophyria

Two cases of tetanus with positive tests for excessive porphyrins and prophobilinogen are presented. The arguments are presented for this being a false positive diagnostic error as well as the arguments for this being a rare coincidence. A third equivocating view is also presented. Investigators are encouraged to follow this series of events in their own cases (AU)