Rare lung diseases: Williams – Campbell syndrome

Williams – Campbell syndrome (WCS) is a rare developmental defect characterized by deficiency or complete absence of cartilage mainly in the subsegmental bronchi. Inferiority of the cartilaginous apparatus creates favorable conditions for the development of recurrent lower respiratory tract infection and, as a consequence, the formation of bronchiectasis, which ultimately leads to the appearance and progression of respiratory failure, as well as to a decrease in the quality of life of patients. Insufficient awareness of this disease among doctors leads to later diagnosis, and complex conservative therapy in the absence of specific treatment for WCS entails a positive effect only in the short term. The aim. Demonstration of a clinical case of a rare respiratory disease – WCS in a 35-year-old man. Conclusion. This case illustrates the complexities of diagnosing and treating WCS and highlights the importance of raising awareness among healthcare workers regarding this disease.

Authors

Institutions

Publication Details

Journal
PULMONOLOGIYA
Published
2025-08-08
DOI
https://doi.org/10.18093/0869-0189-2025-35-4-608-612
Primary Topic
Tracheal and airway disorders
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Rare lung diseases: Williams – Campbell syndrome

E. Stezhkina, Oleg Uryasev, А. В. Шаханов, L. V. Korshunova et al.
PULMONOLOGIYA
Tracheal and airway disorders
article

Rare lung diseases: Williams – Campbell syndrome

E. Stezhkina, Oleg Uryasev, А. В. Шаханов, L. V. Korshunova, A. E. Chervyakov, Yu. B. Uryaseva
article en

Abstract

Williams – Campbell syndrome (WCS) is a rare developmental defect characterized by deficiency or complete absence of cartilage mainly in the subsegmental bronchi. Inferiority of the cartilaginous apparatus creates favorable conditions for the development of recurrent lower respiratory tract infection and, as a consequence, the formation of bronchiectasis, which ultimately leads to the appearance and progression of respiratory failure, as well as to a decrease in the quality of life of patients. Insufficient awareness of this disease among doctors leads to later diagnosis, and complex conservative therapy in the absence of specific treatment for WCS entails a positive effect only in the short term. The aim. Demonstration of a clinical case of a rare respiratory disease – WCS in a 35-year-old man. Conclusion. This case illustrates the complexities of diagnosing and treating WCS and highlights the importance of raising awareness among healthcare workers regarding this disease.

PULMONOLOGIYAVol. 35(4)
Ryazan State Medical University named after Academician I.P. Pavlov (RU), Regional State Budget Health Care Institution "Regional Clinical Hospital" (RU)
Openalex Percentile: Top 69%
Tracheal and airway disorders
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.