假性甲状旁腺功能减退性甲状旁腺功能亢进症六例临床分析

Clinical Analysis of Six Cases of Pseudohy-poparathyroidism with Secondary Hyperparathy-roidism

作者报告了６例假性甲状旁腺功能减退性甲状旁腺功能亢进症。对所有具有低血钙、高血磷、高碱性磷酸酶、血ＰＴＨ增高或正常及骨Ｘ线表现符合甲状旁腺功能亢进的病人，在认真除外慢性肾功能不全后，不论有无先天性畸形，均应考虑假性甲状旁腺功能减退性甲状旁腺功能亢进症。

AbstractSix cases of pseudohypoparathyroidism with sec-ondary hyperparathyroidism were reported.Therewere 3 males and 3 females with the age ranged from14 ～ 20 years. All had the clinical manifestations ofhypocalcemia and all except one,complained of bonepain. Two had characteristic somatic features of pseu-do-hypoparathyroidism,The serum calcium level waxreduced and serum phosphate and alkaline phosphataselevels were elevated in all.Serum PTH vvas measuredin 3 cases,2had elevated PTH level and 1 normal.X -ray or bone biopsy showed classic hyperparathy-roidism changes or osteoporosis in all patients. Pseudohypoparathyroidism with secondary hyper-parathyroidism is characterized by the bone changes. It is a rare condition and is itself a distinct clincal enti-ty of renal resistance and skeletal responsiveness toPTH in contrast to classic pseudohypoparathyroidismin which both bone and kidney are resistant.In anypatient with hypocalcemia, hyperphosphatemia,ele-vated alkline phosphatase levels,elevated or normal serum PTH level and X-ray evidence of hyper-parathyroidism bone disease,after ruling out chronicrenal insufficiency, the diagnosis of pseudohy-poparathyroidism with secondary hyperparathyroidismshould be considered.