Ricketti Peter A, Unkle David W, King Katherine A, Cleri Dennis J, Ricketti Anthony J
Department of Internal Medicine, Rutgers University, New Jersey Medical School, Newark, New Jersey, USA.
Allergy Asthma Proc. 2015 Jan-Feb;36(1):82-6. doi: 10.2500/aap.2015.36.3784.
Wet cough, wheeze, and sputum in an adolescent with evidence for bronchiectasis is an uncommon presentation. The differential diagnosis includes cystic fibrosis (CF), immunodeficiency disorders, complement deficiency, allergic bronchopulmonary aspergillosis, alpha-1 antitrypsin disease, repeated aspiration pneumonia, foreign body, bronchial carcinoid, unresolved right middle lobe pneumonia, and primary ciliary dyskinesia (PCD). The likely diagnosis proceeds from the more to less common in patients with these symptoms. The location of disease on computed tomography scanning, nasal and bronchial exhaled nitric oxide, identification of ultrastructural defects on electron microscopy, and specific genetic mutation help separate CF and PCD. Although differentiating these conditions is vital, the chronic management of the bronchiectasis usually includes clearance mechanisms, bronchodilators, regular exercise, appropriate vaccinations, and judicious antibiotics for airway infections.
一名患有支气管扩张症的青少年出现湿性咳嗽、喘息和咳痰是一种不常见的表现。鉴别诊断包括囊性纤维化(CF)、免疫缺陷疾病、补体缺陷、变应性支气管肺曲霉病、α-1抗胰蛋白酶病、反复吸入性肺炎、异物、支气管类癌、未愈的右中叶肺炎和原发性纤毛运动障碍(PCD)。对于有这些症状的患者,可能的诊断是从较常见的情况到较不常见的情况依次进行排查。计算机断层扫描上疾病的位置、鼻腔和支气管呼出一氧化氮、电子显微镜下超微结构缺陷的鉴定以及特定基因突变有助于区分CF和PCD。虽然区分这些情况至关重要,但支气管扩张症的慢性管理通常包括清除机制、支气管扩张剂、规律运动、适当接种疫苗以及针对气道感染合理使用抗生素。