【摘要】 目的探讨特发性甲状旁腺功能低下(IHP)的临床特征及预后。方法对14例儿童起病的IHP的临床、生化、放射学检查特征及超过3年的随访进行了回顾性的分析。结果其中男性8例,女性6例,年龄14.6±6.2岁,发病到确诊时间为3.4±3.2年,随访时间6.4±2.9年;临床表现最常见为手足抽搐,其次为癫痫发作,病程约3~14年,平均7.9年;颅内钙化灶最常位于基底节。治疗前血清总钙1.3±0.23mmoL/L、血无机磷浓度2.73±0.67mmol/L、甲状旁腺素浓度7.45±1.16pg/mL。经过骨化三醇和钙剂治疗后监测到血清总钙明显上升到1.89±0.23mmol/L(与治疗前相比有统计学差异,F=24.75,P<0.05),血无机磷降至1.92±0.40mmol/L(与治疗前相比有统计学差异,F=12.39,P<0.05)。40%的合并癫痫患者使用丙戊酸作为抗癫痫药物。结论 IHP在儿科较为少见,完整的病史资料、查体及血清总钙、血无机磷及甲状旁腺素浓度有确诊价值,其头颅CT具有特征性表现,治疗推荐使用骨化三醇及钙剂,合并癫痫时,应注意避免选择影响钙磷代谢的抗癫痫药物。更多还原

【Abstract】 Objective To explore the clinical characteristics and prognosis of idiopathic hypoparathyroidism(IHP).Methods Retrospective analysis was conducted among 14 patients with IHP in the aspects of clinical features,biological and radiological examination results,and over 3 years’ follow-up.Results There were 8 males and 6 females with mean age of 14.6 ± 6.2 years,mean duration from onset to diagnosis of 3.4±3.2 years,and mean follow-up of 6.4 ±2.9 years.The most common clinical manifestation was carpopedal spasm,followed by seizures with duration of 3-14 years(mean duration of 7.9 years).Intracranial calcification foci were most commonly seen in basal ganglia.Before treatment the levels of serum total calcium,blood inorganic phosphate and parathormone were 1.3 ± 0.23mmol/L,2.73 ±0.67mmol/L and 7.45 ± 1.16pg/mL,respectively.After treatment with calcitriol and calcium,the serum total calcium rose to 1.89 ± 0.23 mmol/L and blood inorganic phosphorus level fell to 1.92 ± 0.40 mmol/L(F value was 24.75 and 12.39,respectively,both P <0.05).Valproic acid was taken as antiepileptic drug in almost 40%of patients.Conclusion IHP is rarely seen in pediatrics,and complete medical history,physical examination and concentrations of serum total calcium,blood inorganic phosphorus and parathormone have definite diagnostic value.Cranial CT has special manifestations.Calcitriol and calcium are recommended for treatment.If combined with epilepsy,patients should pay attention to avoid choosing antiepileptic drugs affecting calcium phosphorus metabolism.更多还原