儿童迟发型先天性中枢性低通气综合征2例  

Two children with late-onset congenital central hypoventilation syndrome

作　　者：仇书要 杨李强 钟建文 罗向前 刘大波 QIU Shuyao;YANG Liqiang;ZHONG Jianwen;LUO Xiangqian;LIU Dabo(Pediatric Otolaryngology Department of Shenzhen Hospital,Southern Medical University,Shenzhen,518000,China)

机构地区：[1]南方医科大学深圳医院儿童耳鼻咽喉科,广东深圳518000

出　　处：《临床耳鼻咽喉头颈外科杂志》2023年第8期656-657,666,共3页Journal of Clinical Otorhinolaryngology Head And Neck Surgery

摘　　要：报道2例确诊为迟发型先天性中枢性低通气综合征患儿,其中1例为男性,出生后无异常,1岁6个月时出现呼吸衰竭表现,住院后予吸氧和无创辅助通气治疗,CO_(2)潴留不能纠正,气管插管呼吸机通气1个月,未能成功拔管,逐行气管切开术,患儿睡眠时需要呼吸机辅助呼吸,清醒时经气管套管自主呼吸。另1例为女性,出生后无异常,11个月时出现呼吸衰竭表现,患儿睡眠时需要经鼻面罩无创辅助通气,白天自主呼吸,活动正常。2例患儿随访2年后生长发育正常。Two children with late-onset congenital central hypoventilation syndrome were reported,one of whom was male and had no abnormal manifestations after birth,respiratory failure occurs at the age of 1 year and 6 months.After being hospitalized,he was treated with oxygen inhalation and non-invasive ventilation,but carbon dioxide retention could not be corrected.After one month of tracheal intubation,he was failure to wean from ventilator,so tracheostomy was performed.He needs a ventilator to help breath while sleeping,and can breath autonomously during the day without ventilator.The other case was a female,with no abnormalities after birth.At the age of 11 months,she developed respiratory failure.During sleep,the child needs non-invasive assisted ventilation through a nasal mask,and during the day,she breathed autonomously.Two patients were followed up forever 2 years and their growth and development were normal.

关 键 词：中枢性低通气 先天性 儿童 无创辅助通气 

分 类 号：R725.6[医药卫生—儿科]