Autosomal recessive polycystic kidney disease in 15 Arab children

The study includes 15 children (8 males, 7 females) with autosomal recessive polycystic kidney disease (ARPKD) whose ages at diagnosis ranged from 2 days to 7 years (median 10 months). Eleven (73.3%) patients were hypertensive on admission and 1 developed hypertension 4 months later; 5 patients became normotensive after receiving treatment for 18-36 months (mean 23.2 months). Patients were followed for a period of 1-48 months (mean 20.9 months). Glomerular filtration rate remained normal in 7 patients, improved in 4 and deteriorated in 1. Two patients died soon after diagnosis and 1 was lost to follow-up and is assumed dead. Of the 4 patients less than 6 months old at the time of diagnosis, only 1 is alive compared with 10 of 11 presenting after 6 months of age. The cumulative chance of survival from the time of diagnosis was 85% at 3 months and 77% at 6 months. The study highlights the reversible nature of hypertension in ARPKD. Survival is better in patients older than 6 months at the time of diagnosis and those surviving 6 months follow-up.