Dr. von Haunersches Kinderspital, University of Munich, Member of the German Center for Lung Research, Lindwurmstr, 4a, D-80337 Munich, Germany.
Orphanet J Rare Dis. 2013 Aug 8;8:121. doi: 10.1186/1750-1172-8-121.
Hypersensitivity pneumonitis (HP) also called exogenous allergic alveolitis = extrinsic allergic alveolitis in children is an uncommon condition and may not be recognized and treated appropriately.To assess current means of diagnosis and therapy and compare this to recommendations, we used the Surveillance Unit for Rare Paediatric Disorders (ESPED) to identify incident cases of HP in Germany during 2005/6. In addition, cases of HP reported for reference from all over Germany to our center in the consecutive year were included.Twenty-three children with confirmed pediatric HP were identified. All (age 9.4 y (4.4-15.1) presented with dyspnoea at rest or with exercise, mean FVC was 39% of predicted, seven of the 23 children already had a chronic disease state at presentation. IgG against bird was elevated in 20, and against fungi in 15. Bronchoalveolar lavage was done in 18 subjects (41% lymphocytes, CD4/CD8 1.99), and lung biopsy in 6. Except 2, all children were treated with prolonged courses of systemic steroids. Outcome was not favourable in all cases.Late diagnosis in up to a quarter of the children with HP and inappropriate steroid treatment must be overcome to improve management of HP. Inclusion of children with HP into international, web-based registry studies will help to study and follow up such rare lung diseases.
过敏性肺炎（HP）也称为儿童外源性过敏性肺泡炎=外源性过敏性肺泡炎，是一种不常见的疾病，可能无法得到正确的识别和治疗。为了评估当前的诊断和治疗方法，并将其与建议进行比较，我们使用罕见儿科疾病监测单位（ESPED）在 2005/6 年期间在德国确定了过敏性肺炎的新发病例。此外，还包括来自德国各地向我们中心报告的过敏性肺炎病例。共确定了 23 例确诊的儿童 HP 病例。所有（年龄 9.4 岁（4.4-15.1 岁）在休息或运动时出现呼吸困难，平均 FVC 为预测值的 39%，23 例中有 7 例在就诊时已有慢性疾病状态。20 例 IgG 针对鸟类升高，15 例针对真菌升高。18 例（41%淋巴细胞，CD4/CD8 1.99）进行了支气管肺泡灌洗，6 例进行了肺活检。除 2 例外，所有儿童均接受了长期全身类固醇治疗。并非所有病例的结局均良好。过敏性肺炎患儿中有四分之一可能出现迟诊，且类固醇治疗不当，必须加以克服，以改善过敏性肺炎的管理。将患有 HP 的儿童纳入国际、基于网络的登记研究将有助于研究和随访此类罕见肺部疾病。
