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为探讨郎格罕细胞组织细胞增生症(LCH)患儿肺部病变临床特点 ,对20例郎格罕细胞组织细胞增生症患儿进行分析 ,其中男12例 ,女8例 ;就诊年龄4个月～6岁6个月。结果显示20例患儿均具有间质性肺损害 ,但出现呼吸道症状者仅占57%。有阵发性咳嗽11例 ,气促、呼吸衰竭1例 ,反复呼吸道感染1例 ,无咯血、气胸、紫绀者 ;肺部体征有呼吸音粗糙6例 ,肺部罗音1例 ;活动期患儿可有血气和肺功能异常 ;X线异常多出现在呼吸道症状之前 ,其中双肺纹理粗多11例 ,肺门影增粗模糊2例 ,网点状阴影10例 ,片状影10例 ,肺囊泡5例 ,毛玻璃样1例 ,肺气肿1例 ,胸膜增厚1例 ,胸腺肿大2例。提示胸部高分辨率CT扫描有利于早期发现LCH的肺部改变 ;早期、全身、强化疗有利于肺部病变的恢复
To investigate the clinical features of lung lesions in children with Langerhans cell histiocytosis (LCH), 20 children with Langerhans cell histiocytosis were analyzed, including 12 males and 8 females; the age of diagnosis was 4 Month ~ 6 years old 6 months. The results showed that all 20 children had interstitial lung damage, but only 57% had respiratory symptoms. There are 11 cases of paroxysmal cough, shortness of breath, respiratory failure in 1 case, recurrent respiratory tract infection in 1 case, no hemoptysis, pneumothorax, cyanosis; pulmonary signs of rough breathing in 6 cases, pulmonary rales in 1 case; active Children may have abnormal blood gas and pulmonary function; X-ray abnormalities appear in the respiratory symptoms before, including coarse lungs in 11 cases of coarse, thickening of the hilar ambiguity in 2 cases, 10 dot-like shadow, flaky shadow in 10 cases, lung Vesicle in 5 cases, ground glass in 1 case, emphysema in 1 case, pleural thickening in 1 case, thymus enlargement in 2 cases. Tip high-resolution chest CT scan is conducive to early detection of LCH lung changes; early, systemic, intensive chemotherapy is conducive to the recovery of lung lesions