Clinical analysis on 18 children with neonatal bartter syndrome / 中国医师杂志

ABSTRACT

Objective To analyze the clinical characteristics of Neonatal Bartter syndrome in order to enhance understanding of the disease.Methods Eighteen children with Neonatal Bartter syndrome who were admitted into our hospital from November 2006 to July 2013 were selected as research subjects.A ret-rospective study was done on the clinical data and the outcome of treatment.Results ⑴Clinical character-istics This group included 13 males and 5 females.The onset age ranged from birth to 1 years 3 months (4.01 ±4.49years).Six cases got the disease after birth.Amniotic fluid which lead to premature birth and low birth weight may happened in cases.The most common clinical symptom was malnutrition (89%).⑵Laboratory tests and Renal ultrasound All of the children showed hypokalemia and metabolic alkalosis in some degree,renin and angiotensin increased.In some cases urinary calcium /creatinine ratio were in-creased and urinary specific gravity,showed a low proportion of urine.Some cases'renal ultrasound exami-nation revealed nephrocalcinosis.⑶ TherapyAll of the patients in this group were given intravenous and /or oral potassium chloride treatment.For drug treatment,10 cases were given single application of indom-ethacin [1 ~3mg/(kg·d),points 3 times per day oral]treatment.Conclusions The incidence of Neo-natal Bartter syndrome was early in infant onset as early as after birth,even the fetal period.Some patients may appear premature birth and /or low birth weigh because of amniotic fluid.The main clinical manifesta-tions include malnutrition,no weight increasing,retardation,serious dehydration and even life-threatening. The characteristic appearance such as the forehead,small jaw,eyes are the important characteristics of neo-natal Bartter syndrome.Patients often appears Hypokalemia,hyponatremia and Hypochloremia.Urinary po-tassium of this type increased.Most children's urinary sodium and chloride increased significantly.