CAJ | 학술논문

目的探讨儿童颅咽管瘤的临床特点及其内分泌改变情况,提高儿科医师对本病的认识.方法回顾性分析首都医科大学附属北京儿童医院2004年1月至2012年12月收治的颅咽管瘤患儿14例,对其临床资料进行分析.对所有患儿进行随访,分析术后症状改善情况、内分泌检查结果及用药情况等.结果临床表现主要包括头痛(7/14例,50.0％)、生长迟缓(4/14例,28.6％)、呕吐(4/14例,28.6％)、多饮多尿(3/14例,21.4％)、视力减低症(3/14例,21.4％)等.未行手术治疗者3例,经辅助检查后提示尿崩症3例、生长激素缺乏症2例、中枢性甲状腺功能减低症1例.余11例均行手术治疗,术后均出现不同程度的内分泌改变.11例患儿经手术切除肿瘤,术后出现多饮多尿5例、电解质紊乱5例、癫痫2例.成功随访9例,随访时间为术后5个月～10年[(3.29±3.52)年].症状好转7例,另2例病情加重.结论对于临床以颅内压增高症状、内分泌改变及视力、视野改变就诊的患儿,应考虑颅咽管瘤的可能.术后常见并发症为内分泌改变、电解质紊乱和癫痫.对术后内分泌改变者需根据内分泌改变情况进行长期激素替代治疗.
목적 탐토인동로인관류적림상특점급기내분비개변정황,제고인과의사대본병적인식.방법 회고성분석수도의과대학부속북경인동의원2004년1월지2012년12월수치적로인관류환인14례,대기림상자료진행분석.대소유환인진행수방,분석술후증상개선정황、내분비검사결과급용약정황등.결과 림상표현주요포괄두통(7/14례,50.0％)、생장지완(4/14례,28.6％)、구토(4/14례,28.6％)、다음다뇨(3/14례,21.4％)、시력감저증(3/14례,21.4％)등.미행수술치료자3례,경보조검사후제시뇨붕증3례、생장격소결핍증2례、중추성갑상선공능감저증1례.여11례균행수술치료,술후균출현불동정도적내분비개변.11례환인경수술절제종류,술후출현다음다뇨5례、전해질문란5례、전간2례.성공수방9례,수방시간위술후5개월～10년[(3.29±3.52)년].증상호전7례,령2례병정가중.결론 대우림상이로내압증고증상、내분비개변급시력、시야개변취진적환인,응고필로인관류적가능.술후상견병발증위내분비개변、전해질문란화전간.대술후내분비개변자수근거내분비개변정황진행장기격소체대치료.
Objective To analyze the clinical characteristics and the endocrine changes in children with craniopharyngioma,and to improve the pediatrician understanding of the disease.Methods The study subjects consisted of 14 children with craniopharyngioma admitted to the Department of Endocrinology,Beijing Children's Hospital Affiliated to Capital Medical University from Jan.2004 to Dec.2012.All the patients were followed up to analyze the clinical symptoms improvement,endocrine test results and medication,et al.Results The main clinical manifestations were headache (7/14 cases,50.0％),growth retardation(4/14 cases,28.6％),vomiting (4/14 cases,28.6％),polydipsia/ polyuria (3/14 cases,21.4％) and vision diminution (3/14 cases,21.4％).Three patients didn' t undergo the surgery,and 3 cases with diabetes insipidus and 2 cases with growth hormone deficiency,and 1 case with central hypothyroidism by laboratory test.The rest 11 children received surgery and all patients had changes in endocrine after it.Five cases got polydipsia and polyuria,other 5 cases had electrolyte disturbances,and 2 cases had epilepsy.Nine patients were followed up,and the follow-up duration ranged from 5 months to 10 years [(3.29 ± 3.52) years] after surgery.Seven patients got better and 2 patients got worse.Conclusions For clinical symptoms of increased intracranial pressure,changes in endocrine,the vision and visual field,the possibility of craniopharyngioma should be taken into account.Surgery is the main treatment,but it can lead to the damage of hypothalamus and pituitary gland.Changes in endocrine,electrolyte disturbances and epilepsy are the common complications.According to the level of endocrine,longterm hormone replacement therapy for some postoperative patients should be continued.