以胸腔积液为首发表现的儿童Gorham-Stout综合征1例报道  

Child Gorham-Stout syndrome with pleural effusion as initial manifestation:a case report

作　　者：刘静月[1] 付文龙[1] 田代印[1] 代继宏[1] Liu Jingyue;Fu Wenlong;Tian Daiyin;Dai Jihong(The First Respiratory Department,Children's Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Chongqing Key Laboratory of Pediatrics)

机构地区：[1]重庆医科大学附属儿童医院呼吸一科,儿童发育疾病研究教育部重点实验室,国家儿童健康与疾病临床医学研究中心,儿童发育重大疾病国家国际科技合作基地,儿科学重庆市重点实验室,重庆400014

出　　处：《重庆医科大学学报》2020年第6期813-816,共4页Journal of Chongqing Medical University

摘　　要：Gorham-Stout综合征是临床上罕见的以骨质溶解为特征的骨病,可发生于全身任何骨骼,临床表现无特异性,需结合影像学、组织病理学等综合诊断。此例患儿发病年龄早,以胸腔积液为首发表现,经过实验室及影像学检查提示为双侧乳糜胸,伴有肋骨病变,经过病理学检查及多学科会诊后明确诊断,患儿经过胸腔穿刺引流、胸腔注射阿奇霉素、西罗莫司口服等治疗,仍有双侧顽固性胸腔积液,提示患儿预后不佳。此病目前尚无确切的治疗方法,临床上主要以内科药物治疗、外科手术治疗、放射治疗等为主,疗效有待进一步观察。Gorham-Stout syndrome is a rare clinical osteopathy characterized with osteolysis,which can occur in any bone of the whole body.It has no specificity in clinical manifestations,therefore,the general diagnosis should combine with imaging and histopathology.In this case,the child had a early onset age,with initial manifestation of pleural effusion.According to laboratory and imaging examinations,it was suggested bilateral chylothorax,accompanying rib lesions.The disease was finally diagnosed through pathology examination and multidisciplinary consultation.After treatment of thoracic drainage,chest injection of azithromycin,oral administration of sirolimus and so on,the child still had bilateral refractory pleural effusion,suggesting poor prognosis.There is no precise treatment for this disease at present,and it is mainly treated with internal medicine,surgery,radiotherapy and other methods in clinical practice,with uncertain efficacy.

关 键 词：Gorham-Stout综合征 胸腔积液 乳糜胸 骨质溶解 儿童 

分 类 号：R72[医药卫生—儿科]