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婴儿神经内分泌细胞增生症
儿科
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李医师![](data:image/png;base64,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)
![](data:image/png;base64,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)
成都市锦江区妇幼保健院
主诉 病史
患儿女,10个月,因“咳嗽2个月,气促1个月”入院。2个月前出现咳嗽,无痰,伴低热1d,当地医院按“肺炎”,予抗感染治疗后,体温恢复正常,但仍有咳嗽,并逐渐出现气促。肺部CT示双肺磨玻璃影,按“间质性肺炎”,予甲波尼龙治疗无缓解。
查体 复查
查体:安静时呼吸急促,呼吸音粗糙,左侧可闻及少许爆裂音。心音有力,无杂音。
辅助检查:血气分析pH7.41,PC02 28.8mmHg,P02 74.2mmHg,Sa0295.2%,BE-6.0mmol/L。肺功能示小气道阻塞性通气功能障碍,左肺通气功能降低。
诊断 治疗
诊断:咳嗽查因;轻度低氧血症
处理:予吸氧、雾化等对症支持治疗,并给予孟鲁司特4ms/d,小剂量红霉素3~5mS/(kg·d)口服抗炎,甲波尼龙2mg/(kg·d)静点,治疗2周后,改为甲泼尼龙片2ms/(kg·d)口服(激素总疗程1个月)。治疗20d后,患儿仍有气促,50次/min,肺内听诊可闻及爆裂音,胸部CT示肺透光度不均匀,右肺中叶和左肺舌叶仍可见磨玻璃影,以及区域性过度通气(图2)。1个月后患儿呼吸急促未见好转,但血氧饱和度提高,精神状态及生命体征明显好转即出院。
回顾性诊断:婴儿神经内分泌细胞增生症
随访 处理
随访:出院后随访4年。患儿仅口服孟鲁司特治疗1年,气促逐渐好转;第2年气促减轻;第4年气促症状消失。期间患儿曾有过1次喘息(冬季感冒后),平素活动耐受尚可,无明显喘息及气促。
讨论:本病诊断的金标准为肺活检,但其母亲考虑到患儿年龄较小,行肺活检对患儿创伤较大,故拒绝接受此检查。本病出院时并未能获得正确诊断,提示临床医生长期咳嗽激素无效不能缓解应考虑该病。
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