bartter syndrome

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In 90% of cases, neonatal Bartter syndrome is seen between 24 and 30 weeks of gestation with excess amniotic fluid(polyhydramnios). After birth, the infant is seen to urinate and drink excessively (polyuria, and polydipsia, respectively). Life-threatening dehydration may result if the infant does not receive adequate fluids. About 85% of infants dispose of excess amounts of calcium in the urine (hypercalciuria) and kidneys (nephrocalcinosis), which may lead to kidney stones. In rare occasions, the infant may progress to renal failure. Patients with classic Bartter syndrome may have symptoms in the first two years of life, but they are usually diagnosed at school age or later. Like infants with the neonatal subtype, patients with classic Bartter syndrome also have polyuria, polydipsia, and a tendency to dehydration, but normal or just slightly increased urinary calcium excretion without the tendency to develop kidney stones. These patients also have vomiting and growth retardation. Kidney function is also normal if the disease is treated, [2] but occasionally patients proceed to end-stage renal failure. Bartter's syndrome consists of hypokalaemia, alkalosis, normal to low blood pressures, and elevated plasma renin and aldosterone. Numerous causes of this syndrome probably exist. Diagnostic pointers include high urinary potassium and chloride despite low serum values, increased plasma renin, hyperplasia of the juxtaglomerular apparatus on renal biopsy, and careful exclusion of diuretic abuse. Excess production of renal prostaglandins is often found. Magnesium wasting may also occur.

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A description of Bartter Syndrome

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In 90% of cases, neonatal Bartter syndrome is seen between 24 and 30 weeks of gestation with excess amniotic fluid(polyhydamnios!" #fte bith, the infant is seen to uinate and dink excessi$ely (polyuia, and polydipsia, especti$ely!" %ife&theatening dehydation may esult if the infant does not ecei$e ade'uate fluids" #bout ()% of infants dispose of excess amounts of calcium in the uine (hypecalciuia! and kidneys (nephocalcinosis!, which may lead to kidney stones" In ae occasions, the infant may pogess to enal failue"*atients with classic Bartter syndrome may ha$e symptoms in the fist two yeas of life, but they ae usually diagnosed at school age o late" %ike infants with the neonatal subtype, patients with classic +atte syndome also ha$e polyuia, polydipsia, and a tendency to dehydation, but nomal o ,ust slightly inceased uinay calcium excetion without the tendency to de$elop kidney stones" -hese patients also ha$e $omiting and gowth etadation" .idney function is also nomal if the disease is teated,/20 but occasionally patients poceed to end&stage enal failue" +atte1s syndome consists of hypokalaemia, alkalosis, nomal to low blood pessues, and ele$ated plasma enin and aldosteone" 2umeous causes of this syndome pobably exist" 3iagnostic pointes include high uinay potassium and chloide despite low seum $alues, inceased plasma enin, hypeplasia of the ,uxtaglomeula appaatus on enal biopsy, and caeful exclusion of diuetic abuse" 4xcess poduction of enal postaglandins is often found" 5agnesium wasting may also occu"