ROHHAD综合征——快速肥胖伴低通气、下丘脑功能障碍及自主神经功能失调综合征  

ROHHAD syndrome: rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation syndrome

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作  者:张静怡 何阳 胡克[1] Zhang Jingyi;He Yang;Hu Ke(Second Department of Respiratory and Critical Care Medicine,Renmin Hospital of Wuhan University,Wuhan 430060,China)

机构地区:[1]武汉大学人民医院呼吸与危重症医学二科,武汉430060

出  处:《国际呼吸杂志》2022年第21期1675-1680,共6页International Journal of Respiration

摘  要:ROHHAD综合征是一种以快速发生肥胖,伴低通气、下丘脑功能障碍以及自主神经功能失调为特征的罕见疾病,病因尚未阐明且存在一定争议,新近俄罗斯学者报道了1例与新型冠状病毒相关的类ROHHAD综合征。一半以上此综合征患者存在神经内分泌肿瘤,合并疾病多且病死率高,预后不良。本文系统地介绍了该综合征的病因、临床表现、诊断以及当前治疗。Rapid-onset obesity with hypoventilation,hypothalamic dysfunction,autonomic dysregulation(ROHHAD)syndrome is a rare disease with unknown and debated etiology,characterized by rapid-onset obesity with hypoventilation,hypothalamic dysfunction,autonomic dysregulation.Recently,Russian scholars reported a case of COVID-19-associated ROHHAD-like syndrome.Neuroendocrine tumors can be associated in more than half of the cases.This rare condition has a severe outcome because of high morbidity and mortality.In this article provides a comprehensive description of the etiopathogenetic theories of the disease,clinical presentation,diagnostic workup and treatment possibilities.

关 键 词:肥胖症 低通气 下丘脑功能障碍 自主神经功能失调 

分 类 号:R597[医药卫生—内科学]

 

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