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黄甲综合征中的快速进展性支气管扩张和肺纤维化,可能与选择性IgM缺乏有关。

Rapidly Progressive Bronchiectasis and Pulmonary Fibrosis in Yellow Nail Syndrome with Possible Association with Selective IgM Deficiency.

作者信息

Abu-Abaa Mohammad, Hassan Mohamed, Oudah Bashar, Al-Ameri Noor

机构信息

Internal Medicine, Capital Health Regional Medical Center, Trenton, NJ, USA.

Eisenhower Health, Rancho Mirage, CA, USA.

出版信息

Eur J Case Rep Intern Med. 2022 Sep 27;9(9):003577. doi: 10.12890/2022_003577. eCollection 2022.

Abstract

UNLABELLED

A 67-year-old man with a history of IgM deficiency and pulmonary fibrosis and bronchiectasis was admitted for management of worsening symptoms. Investigations revealed exudative pleural effusion with rapidly progressive bronchiectasis. Although a potential trigger of bronchiectasis and pulmonary fibrosis was not identified despite extensive work-up by several physicians in the past, a bedside observation of yellow dystrophic nails on all extremities revealed the diagnosis. This case report helps to remind clinicians of a rare medical disorder of still uncertain aetiology and no available cure. This case is consistent with a few previous case reports that suggest a potential association with selective immunoglobulin deficiency.

LEARNING POINTS

Yellow nail syndrome is a rare condition characterized by the classic triad of respiratory, nail and lymphatic involvement.Diagnosis remains clinical with no confirmatory tests available; the absence of yellow nails does not preclude the diagnosis.An association with selective IgM deficiency has been infrequently reported.

摘要

未标注

一名67岁男性,有IgM缺乏症、肺纤维化和支气管扩张病史,因症状恶化入院治疗。检查发现有渗出性胸腔积液以及快速进展的支气管扩张。尽管过去几位医生进行了广泛检查,但未确定支气管扩张和肺纤维化的潜在触发因素,然而对患者四肢黄色营养不良指甲的床边观察揭示了诊断结果。本病例报告有助于提醒临床医生注意一种病因仍不确定且无法治愈的罕见医学病症。该病例与之前一些病例报告一致,提示可能与选择性免疫球蛋白缺乏有关。

学习要点

黄甲综合征是一种罕见病症,其特征为呼吸、指甲和淋巴系统受累的典型三联征。诊断仍依靠临床判断,尚无确诊检查;没有黄色指甲并不排除诊断。与选择性IgM缺乏的关联鲜有报道。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e57/9586509/85a519ae4a62/3577_Fig1.jpg

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