Acute Intermittent Porphyria in a Kumhar community of Western Rajasthan.

ABSTRACT

OBJECTIVE: To know the current magnitude of the occurrence of Acute Intermittent Porphyria in Kumhar community of a part of Western Rajasthan. MATERIAL AND METHODS: This is a cross sectional study of rural community of Kumhars of Bikaner district of Western Rajasthan. The households of kumhar community were approached. Besides recording other information and examination details, their urine samples were subjected to Watson-Schwartz Test. RESULT: 1237 subjects out of a total of 2385 kumhar population distributed among 20 randomly selected villages of Bikaner district could be studied. The prevalence of AIP was estimated to be 1.16%. (CI=0.012 +/- 0.0005), showing higher prevalence than the earlier reported studies. The cases exhibited varied symptomatology. Overall preponderance was in favor of females (21) and majority of cases (38.9%) were found in the age group of 20-29 years. Average age of manifestation was 24.5 +/- 4.8 years. A follow up was also done to know the disease consequences, if any. CONCLUSIONS: The study finds higher prevalence of the disease and as such warrants a need for generating awareness among the families of cases in the specific caste group in the study area for early detection and better management of the disease.