小儿特发性间质性肺炎18例临床分析

Clinical analysis of 18 cases of idiopathic interstitial pneumonia in children

目的探讨儿童特发性间质性肺炎(IIP)的病因、临床特点、疗效及随访结果。方法采用临床流行病学调查方法 ,对2003年7月至2008年7月于新疆医科大学第一附属医院儿科临床诊断为IIP的18例患儿资料进行分析。将其中部分血清学检测指标与同期来院体检35名儿童(对照组)的血样本资料进行比较。结果 18例均未查明与患病有直接关系的确切病因,仅发现与发病有关的背景因素;患儿呈现进行性呼吸困难而致活动受限,干咳、气短,多无发热,有消瘦或体重下降、乏力、纳差,双肺底部可闻及细小湿性啰音(Velcro啰音),伴有不同程度的紫绀、杵状指为其主要表现;入院前多被诊断为支气管肺炎、哮喘或肺结核等;与对照组相比,多数患儿有血白细胞和乳酸脱氢酶(LDH)、C-反应蛋白(CRP)升高,血沉(ESR)增快,血清肿瘤坏死因子-α(TNF-α)、白介素-8(IL-8)异常,α-抗胰蛋白酶(α-AT)减低等特点,胸片表现双肺广泛浸润性改变,(HRCT)以双下肺叶弥漫性网状、蜂窝样、磨玻璃状改变为主,肺功能(>8岁)为限制性通气障碍,血气分析和心电监护仪显示持续性低氧血症;给予糖皮质激素冲击联合丙种球蛋白静滴,阿奇霉素口服、糖皮质激素吸入等治疗及随访,症状缓解12例,死亡3例,无效自动出院失访3例。结论 IIP病因多不明确,病程迁延而危重,症状多持续或反复,易于误诊或漏诊,依据临床表现和实验室以及必要的影像学检查是早期诊断关键,尽管治疗比较困难,综合治疗仍能使部分患儿症状得到控制,肺组织活检在儿科实施尚有难度。

Objective In order to reduce misdiagnosis of idiopathic interstitial pneumonia （IIP） in children we summarized the etiology, clinical characteristics, treatment and follow-up results of 18 cases. Method The clinical epidemic investigations were used in our present study. A retrospective analysis was made on the pediatric patient＇s clinical materials, who were diagnosed with liP. Some of the serum indexes were compared with those of 35 children who came to our hospital for physical examination. Results The etiology was unknown in these patients; we just found some background factors. The main symptoms were progressive dyspnea and dry cough; most of patients usually had no fever, but had weight loss, fatigue and poor appetite. Fine crackles could be heard on the base of bilateral lungs, accompanied with cyanosis and clubbed fingers. Prior to being admitted to the hospital, the patients were misdiaguosed with bronehopneumonia, asthma or pulmonary tuberculosis etc. Blood test showed that WBC was elevated, ESR was accelerated, CRP and LDH, TNF- α and IL-8 levels were increased, but α-AT decreased. X ray film indicated diffused infiltrating change and HRCT characterized by reticulate, honey comb and frosted change. Lung function test （〉8 years） showed restrictive ventilation function impairment; blood gas analysis and monitoring showed persistent hypoxemia. The patients received systemic corticosteroid pulse therapy combined with intravenous gamma immunoglobulin, azithromycin taken orally, inhaled Flutieasone aerosol therapy and follow. Twelve eases clinically resolved, 3 cases died; there was no improvement in 3 case and they gave up treatment. Conclusion The etiology is uncertain in this cohort, and childhood liP is easily misdiaguosed and under-diagnosed. The duration and degree of disease is prolonged. Most patients have persistency or recurrent symptoms. The key point of early diagnosis is clinical manifestations, laboratory test results and imaging studies. Although the treatment effect is not ideal in this cohort, combination therapy can improve symptoms in some patients. Pulmonary biopsy is very difficult to perform in the clinic.