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[严重心脏功能不全与Ib型假性甲状旁腺功能减退症]

[Severe cardiac insufficiency and type Ib pseudohypoparathyroidism].

作者信息

Massing J L, Weber E, Baille N, Dusselier L, Zakari I

机构信息

Service de cardiologie, hôpital Sainte-Blandine, Metz.

出版信息

Arch Mal Coeur Vaiss. 2000 Jul;93(7):869-73.

Abstract

The authors report the case of a 28 year old woman with acute left ventricular failure associated with severe hypocalcaemia (1.7 mmol/l) without chronic renal failure or hypoproteinaemia. The echocardiographic appearances were those of dilated and globally hypokinetic cardiomyopathy with a severely depressed left ventricular ejection fraction (23%). Haemodynamic improvement was only obtained by the association of calcium supplements and Vitamin D derivatives (Un-Alfa) to conventional treatment. A low serum calcium associated with hyperphosphotaemia, hypocalciuria, hypophosphaturia and, above all, a high parathormone concentration, provided the diagnosis of a sporadic form of type Ib pseudohypoparathyroidism. Secondary cardiac failure to the hypocalcaemia is mainly observed in children and young adults in the context of chronic renal failure or true hypoparathyroidism. Pseudohypoparathyroidism is a very rare condition and systolic LV dysfunction for which hypocalcaemia is responsible, would seem to be totally reversible after calcium supplementation.

摘要

作者报告了一例28岁女性患者,该患者患有急性左心室衰竭,伴有严重低钙血症(1.7 mmol/L),无慢性肾衰竭或低蛋白血症。超声心动图表现为扩张型和整体运动减弱型心肌病,左心室射血分数严重降低(23%)。通过将钙剂和维生素D衍生物(骨化三醇)与传统治疗相结合,才实现了血流动力学改善。低血清钙伴有高磷血症、低钙尿症、低磷尿症,最重要的是甲状旁腺激素浓度升高,从而诊断为散发型Ib型假性甲状旁腺功能减退症。低钙血症继发的心力衰竭主要见于儿童和年轻人,常伴有慢性肾衰竭或真性甲状旁腺功能减退症。假性甲状旁腺功能减退症是一种非常罕见的疾病,由低钙血症导致的收缩期左心室功能障碍在补充钙剂后似乎完全可逆。

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