Abstract
A 10 months - old ma le baby was admitted to hospital with the complaints of weakness, irritability loss of weight, aııd it was obtained from the history of patient that he was admitted hospital several times with the diagnosis of acute gastroenteritis and dehydratation since the newborn period. Hypokalemic tubular disease was considered because of the determination in physical examination and in laboratory studies of high serum level of aldosteron and renin, hypokalemia, hypochloremia, metabolic alkalosis, and growth retardation. It was diagnosed as Hyperprostaglandin E syndrome since the complaints started from the newborn period, the functions of platelets were normal, serum Mg level was normal, and since a slight juxtaglomerular apparatus hyperplasia accompanying an interstitial calcification was observed in kidney biopsy. This case was reported as it is a rare syndrome and as there was a satisfactory response to KC1 and indomethacine medication.