Abstract
The study includes 15 children (8 males, 7 females) with autosomal recessive polycystic kidney disease (APRKD) 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.
References
Resnick J, Vernier RL (1987) Renal cystic disease and renal dysplasia. In: Holliday MA, Barratt TM, Vernier RL (eds) Pediatric nephrology, 2nd edn. Williams and Wilkins, London, pp 371–383
Kaplan BS, Fay J, Shah V, Dillon MJ, Barratt TM (1989) Autosomal recessive polycystic kidney disease. Pediatr Nephrol 3:43–49
Cole BR, Conley SB, Stapleton FB (1987) Polycystic kidney disease in the first year of life. J Pediatr 111:693–699
Swiet M de, Fayers P, Shinebourne EA (1980) Systolic blood pressure in population of infants in the first year of life: the Brompton study. Pediatrics 65:1028–1035
Schwartz GJ, Feld LG, Langford DJ (1984) A simple estimate of glomerular filtration rate in full-term infants during the first year of life. J Pediatr 104:849–854
Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259
Nicholson JF, Pesce MA (1992) Laboratory medicine and reference tables. In: Behrman RE, Kliegman RM, Nelson WE, Vaughan VC (eds) Nelson textbook of pediatrics, 14th edn. Saunders, Philadelphia, pp 1797–1849
Gang DL, Herrin JT (1986) Infantile polycystic kidney disease of the liver and kidneys. Clin Nephrol 25:28–36
Kaariainen H, Koskimies O, Norio R (1988) Dominant and recessive polycystic kidney disease in children: evaluation of clinical features and laboratory data. Pediatr Nephrol 2:296–302
Rahill WJ, Rubin MI (1972) Hypertension in infantile polycystic renal disease. Clin Pediatr (Phila) 11:232–235
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mattoo, T.K., Khatani, Y. & Ashraf, B. Autosomal recessive polycystic kidney disease in 15 Arab children. Pediatr Nephrol 8, 85–87 (1994). https://doi.org/10.1007/BF00868276
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00868276