A family case Williams – Campbell syndrome in clinical practice

Q4 Medicine

Pulmonologiya Pub Date : 2023-09-29 DOI:10.18093/0869-0189-2023-33-5-713-721

E. V. Stezhkina, N. A. Belykh, V. S. Arsenina, M. V. Chaplanov, Yu. V. Deeva, A. A. Burceva

引用次数: 0

Abstract

Currently, among all pathologies of the respiratory system, bronchiectasis receives special attention. This is due to its increasing incidence and the improvement of diagnostic methods. Williams – Campbell syndrome is a congenital form of bronchiectasis characterized by a defect or complete absence of bronchial wall cartilage in the subsegmental bronchi. In view of this, medical personnel should be familiar with the basics of differential diagnosis and treatment, as well as the prognosis of this disease. The aim of this paper is to present a clinical family case of Williams – Campbell syndrome. The father of the family was diagnosed with WCS at the age of 3 years by bronchography, and his daughter has all clinical signs of this disease today. To date, the question remains as how to diagnose WCS and monitor the patient’s health in the future. Conclusion. Physicians of all specialties should be informed about rare diseases.

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临床实践中的一例威廉姆斯-坎贝尔综合征家庭病例

目前，在呼吸系统的所有病理中，支气管扩张受到了特别的关注。这是由于其发病率的增加和诊断方法的改进。Williams - Campbell综合征是一种先天性支气管扩张形式，其特征是亚节段支气管壁软骨缺损或完全缺失。鉴于此，医务人员应熟悉鉴别诊断和治疗的基础知识，以及本病的预后。本文的目的是提出一个威廉姆斯-坎贝尔综合征的临床家庭病例。该家庭的父亲在3岁时通过支气管造影诊断为WCS，他的女儿今天具有该病的所有临床症状。迄今为止，问题仍然是如何诊断WCS并在未来监测患者的健康状况。结论。所有专业的医生都应该了解罕见病。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pulmonologiya Medicine-Pulmonary and Respiratory Medicine

CiteScore

1.40

自引率

0.00%

发文量

期刊介绍： The aim of this journal is to state a scientific position of the Russian Respiratory Society (RRS) on diagnosis and treatment of respiratory diseases based on recent evidence-based clinical trial publications and international consensuses. The most important tasks of the journal are: -improvement proficiency qualifications of respiratory specialists; -education in pulmonology; -prompt publication of original studies on diagnosis and treatment of respiratory diseases; -sharing clinical experience and information about pulmonology service organization in different regions of Russia; -information on current protocols, standards and recommendations of international respiratory societies; -discussion and consequent publication Russian consensus documents and announcement of RRS activities; -publication and comments of regulatory documents of Russian Ministry of Health; -historical review of Russian pulmonology development. The scientific concept of the journal includes publication of current evidence-based studies on respiratory medicine and their discussion with the participation of Russian and foreign experts and development of national consensus documents on respiratory medicine. Russian and foreign respiratory specialists including pneumologists, TB specialists, thoracic surgeons, allergists, clinical immunologists, pediatricians, oncologists, physiologists, and therapeutists are invited to publish article in the journal.