特发性甲状旁腺功能减退症的某些特殊问题(附8例报告)

特发性甲状旁腺功能减退症(特发性甲旁减)国内报道较多,一般临床表现、诊断治疗已有详尽阐述。本文报道本症8例,其临床表现、生化检查及治疗均有其特殊之处;如合并Klinefelter氏综合征,先有角质下脓疱病后发生本症,由于严重癫痫用大量抗癫痫药诱发全身剥胶性皮炎、CPK及LDH明显升高等国内尚未见报告。本组血钙最低为2.4mg/dl,血磷最高达11.7mg/dl,亦为文献中之极限值。治疗方面3例作了甲状旁腺移植术;1例为狗甲状旁腺埋藏,2例为带血管甲状分腺移植。 癫痫及癔症样发作可为四旁减之表现。故遇上述病人应除外甲旁减之可能。本文对临床常用甲状旁腺功能如磷廓清、肾小管磷重吸收率及钙耐量试验进行了评价,认为临床表现生化结果均典型病人,巳无必要再行上述试验。亚临床期甲旁减是否能赖上述试验而获早期诊断有待观察。 本文最后对甲状旁腺移植进行了讨论,认为带血管移植为治疗甲旁减之有效措施。1例狗甲状旁腺埋藏,效果奇特,其机制值得进一步探讨。

Some Special Problems concerning Idiopathic Hypoparatbyroidism

There have been more than 100 cases of idiopathic hypoparathyroidism(IHP) reported in our country. The 8 cases reported in this paper showed some special features in the clinical manifestations, the biochemical parameters, and the response to treatment.Clinical, one case was complicated with Klinefelter's syndrome. Another case suffered from subcorneal pustulosis prior to the onset of idiopathic hypo-parathyroidism. Two cases were misdiagnosed as epilepsy and admitted to the department of neurology. Generalized exfoliative dermatitis was induced in one case by large doses of antiepileptic agents to control the convulsive seizures.Biochemically, both CPK and LDH were elevated in one case. Serum calcium was as low as 2.4 mg/dl in one case, and serum phosphrus was as high as 11.7 mg/d1 in another. These phenomena were rarely encounted in IHP.Therapeutically, transplantation of homogenous parathyroid gland with blood vessels was the method of choice. Two cases were treated in this way with good results. A third case received a dog's parathyroid gland, which also showed good results. The mechanism of heterogenous parathyroid transplantation needs further investigation.In addition, the diagnosis and differential diagnosis of IHP were discussed.