小儿原发性甲状旁腺功能减退症61例分析

目的　探讨小儿原发性甲状旁腺功能减退症的临床特点及治疗。方法　对北京协和医院 1973～2 0 0 2年收治的 6 1例小儿原发性甲状旁腺功能减退症病例 (发病年龄≤ 14岁 ,男 4 1例 ,女 2 0例 )进行回顾性分析。结果　 6 1例主要临床表现为手足抽搐和癫发作 ,出现癫者为 39例 (6 3 9% ) ,颅内钙化者 5 1例(79 6 % )。颅内钙化是癫发生的预测因子 (OR =3 6 0 4 ,P =0 0 0 6 ) ,而病程长短影响颅内钙化的发生 (OR =2 982 ,P =0 0 11)。治疗包括钙剂和维生素D或其衍生物。结论　小儿原发性甲状旁腺功能减退症以癫和颅内钙化多见 ,维生素D制剂及其衍生物和钙剂治疗效果好。对小儿手足抽搐、癫发作应检查血钙磷水平 ,以免漏诊。

Clinical characteristics and therapy of primary hypoparathyroidism in childhood:a retrospective analysis of 61 cases

Objective To study the clinical characteristics and therapy of primary hypoparathyroidism in childhood.Methods The clinical data of 61 cases of primary hypoparathyroidism in childhood in our hospital during 1973 to 2002 were collected and analyzed retrospectively.Results The predominant manifestations were tetanys of the distal extremities and seizures.Seizures occurred in 63 9% of the cases.Intracerebral calcifications were detected by CT scanning in 79 6% of the cases.Patients were treated with calcium supplement and vitamin D or its metabolites.Conclusion Seizures and intracerebral calcifications are common in children with primary hypoparathyroidism.Calcium supplement combined with vitamin D or its metabolites is an effective therapy.Children with seizures and tetanys should be examined for their serum calcium and phosphate in order to avoid misdiagnosis.