骨化性气管支气管病：对自孩童开始就患喘息的非吸烟者和非过敏患者的一份罕见的病例报告
2016/06/20
摘要
背景：骨化性气管支气管病（TBPO）是一种少见的良性疾病，特征是在气管支气管树的粘膜下层生有1-3毫米骨化或软骨化结节。气管后膜壁通常特别薄。
软骨和骨结节可以引起慢性炎症和黏膜上皮化生、阻塞和气道阻塞。这种疾病往往是无临床症状或症状不典型，其患病率通常被低估，诊断的平均年龄为50岁。
病例陈述：我们报告的病例是一个49岁的、不抽烟的男性。他是个铁匠，无家可归，出生在罗马尼亚，从小就被诊断出哮喘。他被送诊到我们呼吸科的时候出现低烧和大汗、咳嗽、脓痰，高分辨CT扫描显示主支气管出现不规则毛玻璃样阴影。纤维支气管镜检查显示一直到肺段支气管入口都存在黏膜不规则。组织学检查显示结节的软骨骨化性特征，符合骨化性气管支气管病（TBPO）。支气管肺泡灌洗液中的微生物检测也显示了铜绿假单胞菌感染。
结论：骨化性气管支气管病（TBPO）是一种罕见的疾病，特点是喘息、咳嗽、咯血、和肺部反复感染，在成年期典型发病。在本报告中，症状开始于童年，虽然被他们误诊为哮喘。即使儿童期发病还未见文献报道，它很可能在最初几年出现了小的变化，在成年后变得更加明显。TBPO通常未累积肺段支气管和亚段支气管，可以解释我们的病人从小就气喘和干咳。
（杨冬 审校）
Multidiscip Respir Med. 2016 Apr 19;11:16. doi: 10.1186/s40248-016-0050-7. eCollection 2016.
Tracheobronchopathia Osteochondroplastica: a rare case report of a non-smoker and non-atopic patient, with a long history of wheezing since childhood.
Fois AG1, Arcadu A1, Santoru L1, Trisolini R2, Marras V3, Ginesu GC4, Canu S1, Cordero L1, Diana G1, Pirina P1.
Author information
Abstract
BACKGROUND:Tracheobronchopathia Osteochondroplastica (TBPO) is an uncommon and benign condition characterized by osseous or metaplastic cartilaginous 1-3 mm nodules in the submucosa of the tracheo-bronchial tree. Posterior membranous wall of trachea is typically spared. Ecchondrosis and exostosis nodules can cause chronic inflammation and mucosal metaplasia, stiffness and airway obstruction. The prevalence of this disease, often asymptomatic or associated with nonspecific symptoms, is underestimated, and the mean age at diagnosis is 50 years.
CASE PRESENTATION:We report a case of a 49 year old male, non-smoker. He was a smith, homeless, born in Romania and reported a diagnosis of asthma since childhood. He was admitted to our Respiratory Unit presenting low-grade fever with profuse sweating, cough, purulent sputum, and ground-glass opacity with irregularity in main bronchi detected by High-Resolution Computed Tomography (HRCT) scan. Fibrobronchoscopy revealed the presence of mucosal irregularities up to the segmental bronchi entrance. Histological examination showed nodules of osseouscartilaginous nature, consistent with TBPO. Microbiological tests of Bronchoalveolar Lavage fluid also revealed an infection by Pseudomonas Aeruginosa.
CONCLUSION:TBPO is a rare disease characterized by wheezing, cough, hemoptysis, and recurrent pulmonary infections, with typical onset during adulthood. In the case reported, the symptoms began in childhood, although they had been misinterpreted as asthma. Even if childhood-onset is not reported in literature, it is likely that small changes occur in the first few years of life and become more evident in adulthood. The involvement of segmental and sub-segmental bronchi, usually spared in TBPO, could explain the presence of wheezing and non-productive cough reported by our patient since childhood.
KEYWORDS:Rare diseases; Respiratory sounds; Trachealdisease;Tracheobronchopathia Osteochondroplastica
Multidiscip Respir Med. 2016 Apr 19;11:16. doi: 10.1186/s40248-016-0050-7. eCollection 2016.
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胃动力障碍与咳嗽变异型哮喘患者的咳嗽相关的生活质量的损害相关
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