Acute porphyria in Trinidad - abstract

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)