Clinical analysis of 9 children with Pseudo-Bartter syndrome

Abstract

Objective To analyze the clinical characteristics of children's Pseudo-Bartter syndrome(PBS) in order to enhance physician's understanding of the disease. Methods Nine children with PBS who were admitted into Beijing Children's Hospital from Nov.2008 to Sep.2013 were selected as research subjects.A retrospective study was carried out with the clinical data and the outcome of treatment. Results 1.Clinical characteristics: there were 9 cases in this group including 5 male and 4 female.The patients' age ranged from 4 months to 8 years 8 months.The most common cause of children's PBS was gastrointestinal symptoms(such as diarrhea and vomiting) induced by respiratory tract infection(7/9 cases). Six patients had no striking clinical manifestations, and hypokalemia was found in the treatment of primary disease.2.Laboratory tests: All of the children in this group had hypokalemia and metabolic alkalosis in varying degrees.The activation of renin, angiotensin and aldosterone system increased.3.Therapy: all children were treated by giving potassium supplemental treatment or indomethacin therapy [1 mg/(kg·d), 3 times orally]. After treatment, all cases achieved clinical improvement and normal blood electrolytes.All patients' blood electrolytes remained normal for 5 to 7 days after stopping treatment. Conclusions 1.In China, the most common cause of children's PBS is gastrointestinal symptoms(such as diarrhea and vomiting) induced by respiratory tract infection.2.Except for clinical manifestations related to causes, patients have no significant clinical manifestations.Hypokalemia can be found in the treatment of primary disease.3.The biochemical results show low blood potassium chloride with metabolic alkalosis.In PBS renin, angiotensin and aldosterone concentration in blood are all elevated.4.Treatment of children's PBS mainly includes etiolo-gical treatment and electrolyte supplement therapy.The treatment effectiveness is good after etiological treatment and potassium supplement treatment.In the condition of controlling etiology and potassium supplementation, electrolytes mas return to normal in 2-4 days. Key words: Pseudo-Bartter syndrome; Hypokalemia; Metabolic alkalosis