Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data.
BMC Pulm Med
; 20(1): 99, 2020 Apr 20.
Article
em En
| MEDLINE
| ID: mdl-32312280
ABSTRACT
BACKGROUND:
The tracheobronchomalacia is a life-threatening complication of mucopolysaccharidosis (MPS) without known effective, optimal treatment. The severe expiratory collapse of the trachea and bronchi is one of causes of the high rate of deaths in the course of airway impairment in MPSII patients. CASE PRESENTATION Due to the adynamic tracheobronchomalacia despite of enzymatic treatment (ERT) in our MPSII patient, a life-saving tracheal bifurcated type-Y endoprosthesis (a self-expanding, metal stent for the prosthesis of tracheal and bronchial stenosis) was implanted. In the followed months, the breathing efficiency improved, but then gradual worsening, progression of bronchi occlusion at the stent border resulted in patient's death.CONCLUSION:
The Y-stent implantation appears to be a short-term, life-saving solution without satisfactory long-term effects due to the progress of peripheral bronchomalacia and increased tissue proliferation and granulation, that arises during the illness' course.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Insuficiência Respiratória
/
Doenças da Traqueia
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Broncopatias
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Mucopolissacaridose II
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Stents Metálicos Autoexpansíveis
Limite:
Adult
/
Humans
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article