CONGENITAL HYPOKALEMIA WITH HYPERCALCIURIA IN PRETERM INFANTS - A HYPERPROSTAGLANDINURIC TUBULAR SYNDROME DIFFERENT FROM BARTTER SYNDROME
CONGENITAL HYPOKALEMIA WITH HYPERCALCIURIA IN PRETERM INFANTS - A HYPERPROSTAGLANDINURIC TUBULAR SYNDROME DIFFERENT FROM BARTTER SYNDROME
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DOI：
10.1016/s0022-3476(85)80395-4
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发表时间：
1985-01-01
影响因子：
5.1
通讯作者：
SCHARER, K
中科院分区：
文献类型：
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作者：
SEYBERTH, HW;RASCHER, W;SCHARER, K
先天性低钾小管疾病具有许多类似巴特综合征的特征。其他特点包括产前发病羊水过多和早产；不能茁壮成长;发作性发热、呕吐、腹泻、肾脏电解质和水分流失；高钙尿;肾钙质沉着症;和骨量减少。与Bartter综合征不同的是，在氯离子的管状再吸收方面没有缺陷。尿中前列腺素E2和7 - α的水平。-羟基-5,11-二酮四丙前列腺酸-1,16-二酸选择性升高，表明肾脏和全身PGE2产生明显刺激。吲哚美辛对PGE2活性的慢性抑制纠正了大多数异常，并且在停用吲哚美辛后，疾病立即失代偿。我们的结论是，这些早产儿有不同种类的低钾血症小管疾病，而不是Bartter综合征的变体，因为肾脏和全身性高前列腺素症在致病链中排名较高，抑制PGE2多动与受影响儿童的发育（可能还有预后）显著改善有关。
A congenital hypokalemic tubular disorder is described with many features resembling Bartter syndrome. Additional features include prenatal onset with polyhydramnios and premature labor; failure to thrive; episodes of fever, vomiting, diarrhea, and renal electrolyte and water wastage; hypercalciuria; nephrocalcinosis; and osteopenia. Unlike Bartter syndrome, there is no defect in tubular reabsorption of chloride. Urinary levels of prostaglandin E2 and 7.alpha.-hydroxy-5,11-diketotetranorprosta-1,16-dioic acid are selectively elevated, indicating marked stimulation of renal and systemic PGE2 production. Chronic suppression of PGE2 activity by indomethacin corrects most of the abnormalities, and there is an immediate decompensation of the disease on indomethacin withdrawal. We conclude that these preterm infants have a distinct variety of hypokalemic tubular disorders rather than a variant of Bartter syndrome, because renal and systemic hyperprostaglandinism ranks high in the pathogenic chain of events, and the suppression of PGE2 hyperactivity is associated with significant improvement in the development (and probably in the prognosis) of the affected children.