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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。
Objective To investigate the clinical features and treatment of children with primary hypoparathyroidism. Methods A retrospective analysis was performed on 61 cases of children with primary hypoparathyroidism (age ≤ 14 years, 41 males and 20 females) admitted to Peking Union Medical College Hospital from 1973 to 2002. Results The main clinical manifestations of 61 cases were hand-foot convulsions and epileptic seizures. There were 39 cases (63.9%) with epilepsy and 51 cases (79.6%) with intracranial calcification. Intracranial calcification was a predictor of epilepsy (OR = 3600, P = 0 0 06), and duration of disease affected intracranial calcification (OR = 2 982, P = 0 0 11). Treatments include calcium and vitamin D or their derivatives. Conclusion Primary hypoparathyroidism in children with epilepsy and intracranial calcification more common, vitamin D preparations and derivatives and calcium treatment effect. Tetanus in children with limbs, epileptic seizures should check serum calcium and phosphorus levels, so as to avoid missed diagnosis.