Vanishing Lung Syndrome in a Cystic Fibrosis Patient

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F17%3A10373780" target="_blank" >RIV/00216208:11130/17:10373780 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00064203:_____/17:10373780

Výsledek na webu

<a href="https://doi.org/10.1016/j.arbres.2017.01.015" target="_blank" >https://doi.org/10.1016/j.arbres.2017.01.015</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.arbres.2017.01.015" target="_blank" >10.1016/j.arbres.2017.01.015</a>

Jazyk výsledku

angličtina

Název v původním jazyce

Vanishing Lung Syndrome in a Cystic Fibrosis Patient

Popis výsledku v původním jazyce

Vanishing lung syndrome is a rare condition characterized by unilateral or bilateral asymmetric upper lobe involvement with the formation of multiple large bullae replacing normal pulmonary architecture and compressing surrounding parenchyma. In this paper, we report the case of a male cystic fibrosis (CF) patient with severe sinopulmonary disease, malnutrition, diabetes mellitus and asthenozoospermia. He was diagnosed with CF at 3 years of age due to malnutrition and recurrent respiratory infections. His sweat chloride concentration was 101 mmol/L and CFTR gene mutations were F508del and 2935del11. At the age of 30, he was evaluated for a lung transplant and chest imaging studies were performed. Previous chest X-rays had showed slowly progressing changes in the upper areas of both lung fields over the past 3 years. In patients with CF, the typical CT picture is bronchiectasis, bronchial wall thickening, mucus plugging, atelectasis, consolidation and air trapping.2 Emphysematous and bullous changes are not consistent with CF lung disease. Our patient was a never-smoker and had normal levels of serum α1-antitrypsin, with no symptoms of other diseases that may be complicated by vanishing lung syndrome or giant bullae (e.g. sarcoidosis, granulomatosis with polyangiitis or Marfan and Ehlers-Danlos syndromes).

Název v anglickém jazyce

Vanishing Lung Syndrome in a Cystic Fibrosis Patient

Popis výsledku anglicky

Vanishing lung syndrome is a rare condition characterized by unilateral or bilateral asymmetric upper lobe involvement with the formation of multiple large bullae replacing normal pulmonary architecture and compressing surrounding parenchyma. In this paper, we report the case of a male cystic fibrosis (CF) patient with severe sinopulmonary disease, malnutrition, diabetes mellitus and asthenozoospermia. He was diagnosed with CF at 3 years of age due to malnutrition and recurrent respiratory infections. His sweat chloride concentration was 101 mmol/L and CFTR gene mutations were F508del and 2935del11. At the age of 30, he was evaluated for a lung transplant and chest imaging studies were performed. Previous chest X-rays had showed slowly progressing changes in the upper areas of both lung fields over the past 3 years. In patients with CF, the typical CT picture is bronchiectasis, bronchial wall thickening, mucus plugging, atelectasis, consolidation and air trapping.2 Emphysematous and bullous changes are not consistent with CF lung disease. Our patient was a never-smoker and had normal levels of serum α1-antitrypsin, with no symptoms of other diseases that may be complicated by vanishing lung syndrome or giant bullae (e.g. sarcoidosis, granulomatosis with polyangiitis or Marfan and Ehlers-Danlos syndromes).

Druh

J_SC - Článek v periodiku v databázi SCOPUS

CEP obor

—

OECD FORD obor

30203 - Respiratory systems

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2017

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Archivos de Bronconeumología

ISSN

0300-2896

e-ISSN

—

Svazek periodika

53

Číslo periodika v rámci svazku

8

Stát vydavatele periodika

ES - Španělské království

Počet stran výsledku

1

Strana od-do

451

Kód UT WoS článku

000406648200011

EID výsledku v databázi Scopus

2-s2.0-85025432376