Donghi Valentina, Mora Stefano, Zamproni Ilaria, Chiumello Giuseppe, Weber Giovanna
Department of Pediatrics, Vita-Salute San Raffaele University, Via Olgettina 60, 20132 Milan, Italy.
Cases J. 2009 May 28;2:6734. doi: 10.1186/1757-1626-2-6734.
Pseudohypoparathyroidism refers to a heterogeneous group of disorders characterized by parathyroid hormone (PTH) resistance. Pseudohypoparathyroidism is an uncommon sporadic or inherited genetic disorder subdivided into several distinct entities (type Ia, Ib, Ic, type II). We report cases of four children (aged 8 to 13 years) in the winter season 2007-'08. The present work highlights the variable mode of presentation of pseudohypoparathyroidism and the difficulty of an early diagnosis. We stress the importance of a complete biochemical investigation of the calcium-phosphate metabolism to recognize typical biochemical alterations associated with this condition (hypocalcaemia, hyperphosphataemia with increased phosphate tubular reabsorption and elevated PTH levels) in spite of a phenotypic aspect that often lacks the presence of all the peculiar clinical features of Albright hereditary osteodistrophy.
假性甲状旁腺功能减退症是一组以甲状旁腺激素(PTH)抵抗为特征的异质性疾病。假性甲状旁腺功能减退症是一种罕见的散发性或遗传性疾病,可细分为几个不同的类型(Ia型、Ib型、Ic型、II型)。我们报告了2007 - 2008年冬季4名儿童(年龄在8至13岁)的病例。本研究强调了假性甲状旁腺功能减退症的多种表现形式以及早期诊断的困难。我们强调,尽管在表型方面往往缺乏奥尔布赖特遗传性骨营养不良所有独特的临床特征,但对钙磷代谢进行全面的生化检查对于识别与该疾病相关的典型生化改变(低钙血症、高磷血症伴肾小管对磷的重吸收增加和PTH水平升高)非常重要。