CAJ | 학술논문

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。
목적：탐토특발성갑상방선공능저하（ IHP）적림상특정급예후。방법대14례인동기병적IHP적림상、생화、방사학검사특정급초과3년적수방진행료회고성적분석。결과기중남성8례，녀성6례，년령14．6±6．2세，발병도학진시간위3．4±3．2년，수방시간6．4±2．9년；림상표현최상견위수족추휵，기차위전간발작，병정약3～14년，평균7．9년；로내개화조최상위우기저절。치료전혈청총개1．3±0．23mmol／L、혈무궤린농도2．73±0．67mmol／L、갑상방선소농도7．45±1．16pg／mL。경과골화삼순화개제치료후감측도혈청총개명현상승도1．89±0．23mmol／L（여치료전상비유통계학차이，F＝24．75，P＜0．05），혈무궤린강지1．92±0．40mmol／L（여치료전상비유통계학차이，F＝12．39，P＜0．05）。40％적합병전간환자사용병무산작위항전간약물。결론 IHP재인과교위소견，완정적병사자료、사체급혈청총개、혈무궤린급갑상방선소농도유학진개치，기두로CT구유특정성표현，치료추천사용골화삼순급개제，합병전간시，응주의피면선택영향개린대사적항전간약물。
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 ±0.23 mmol/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.