AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE IN 15 ARAB CHILDREN

The study includes 15 children (8 males, 7 females) with autosomal rec essive polycystic kidney disease (ARPKD) whose ages at diagnosis range d from 2 days to 7 years (median 10 months). Eleven (73.3%) patients w ere hypertensive on admission and 1 developed hypertension 4 months la ter; 5 patients became normotensive after receiving treatment for 18-3 6 months (mean 23.2 months). Patients were followed for a period of 1- 48 months (mean 20.9 months). Glomerular filtration rate remained norm al in 7 patients, improved in 4 and deteriorated in 1. Two patients di ed soon after diagnosis and 1 was lost to follow-up and is assumed dea d. 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 ag e. 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 f ollow-up.