Visiting Clinical Professor of Pediatrics, University of California San Diego, Rady Children's Hospital, San Diego, California.
Associate Professor of Pediatrics, University of California San Diego, Rady Children's Hospital, San Diego, California.
Pediatr Pulmonol. 2019 Apr;54(4):457-462. doi: 10.1002/ppul.24226. Epub 2019 Jan 4.
Diffuse panbronchiolitis (DPB) is a rare progressive and eventually fatal pulmonary disease first identified in Japan and initially seen predominantly in Southeast Asia. Macrolide antibiotics rapidly reverse symptoms and pathology, and their use increased the 5 and 10-year survival from 50 and 30 percent, respectively, to over 90%. Review of 181 case reports from previous publications found patients with DPB commonly had their pulmonary symptoms preceded by rhinosinusitis, frequently by many years. Long delays in diagnosis for many years were common. The review further identified DPB in all ethnic groups and multiple areas outside of Southeast Asia. Although diagnosis was most commonly made in adults, 13% of the diagnoses were made in children and nine of the adult cases described onset in childhood. Few cases of relapse were reported, but extended periods of monitoring after treatment were not generally present.
弥漫性泛细支气管炎（DPB）是一种罕见的进行性、最终致命的肺部疾病，最初在日本发现，最初主要见于东南亚。大环内酯类抗生素可迅速逆转症状和病理，其使用使 5 年和 10 年生存率分别从 50%和 30%提高到 90%以上。对以往文献中 181 例病例报告的回顾发现，DPB 患者的肺部症状常先于鼻窦炎，常早于多年。许多患者多年来都存在长期的诊断延误。该综述进一步发现 DPB 存在于所有种族和东南亚以外的多个地区。尽管诊断最常见于成年人，但有 13%的诊断发生在儿童中，有 9 例成人病例描述的发病年龄在儿童期。报告的复发病例很少，但治疗后一般没有进行长时间的监测。
