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[新生儿暂时性假性甲状旁腺功能减退症]

[Transitory pseudohypoparathyroidism in the newborn infant].

作者信息

Schaumberger E, Hohenauer L, Sommer R

出版信息

Klin Padiatr. 1986 Jul-Aug;198(4):349-52. doi: 10.1055/s-2008-1033886.

Abstract

This is a report on two young infants suffering from transitory pseudohypoparathyroidism. Therapy with vitamin D in high doses was not helpful but metabolic changes were promptly corrected by the administration of 1.25-(OH)2-D3 (Rocaltrol). The typical pretreatment findings were: hypocalcemia, hyperphosphatemia, reduced clearance of phosphate. Parathormone in serum was normal. Treatment with 0.25 and 0.5 micro-Gram 1.25-(OH)2-D3 respectively resulted in prompt correction of serum calcium- and serum-phosphate-changes. The observation of the urinary-cAMP-excretion during the course of the disease enables one to differentiate between the permanent and the transient form of the disease: values reaching 5-10 nmol/mg Creatinine indicate the time when treatment can be discontinued in the transitory form.

摘要

这是一份关于两名患有暂时性假性甲状旁腺功能减退症的幼儿的报告。高剂量维生素D治疗无效,但给予1,25-(OH)₂-D₃(罗钙全)后代谢变化迅速得到纠正。典型的治疗前表现为:低钙血症、高磷血症、磷酸盐清除率降低。血清甲状旁腺激素正常。分别用0.25微克和0.5微克的1,25-(OH)₂-D₃治疗可迅速纠正血清钙和血清磷的变化。在疾病过程中观察尿环磷腺苷(cAMP)排泄量有助于区分疾病的永久性和暂时性形式:尿cAMP排泄量达到5 - 10纳摩尔/毫克肌酐表明暂时性疾病可停止治疗的时间。

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