Double-Lung Transplantation in a Patient with Pulmonary Type B Niemann-Pick Disease: A Valid Treatment Option

Double-Lung Transplantation in a Patient with Pulmonary Type B Niemann-Pick Disease: A Valid Treatment Option

Niemann-Pick disease is a rare autosomal recessive disease characterized by an abnormal intracellular lipid accumulation. Type B is later in onset and a less severe form of the disease, so affected people may survive in adulthood. Storage of sphingomyelin in pulmonary macrophages can lead to interst...

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Bibliographic Details
Published in:Case reports in transplantation Vol. 2022; pp. 5428381 - 5
Main Authors: Mora, Víctor Manuel, Osorio, Joy Selene, Iturbe, David, Tello, Sandra, Guzmán, Yedra, Sánchez, Laura, Gómez, José Javier, Cifrián, José Manuel
Format: Journal Article
Language:English
Published: United States Hindawi 2022
Hindawi Limited
Subjects:
Biopsy
Brain diseases
Case Report
Case reports
Convulsions & seizures
Dyspnea
Genetic disorders
Hospitals
Lipids
Liver
Lung diseases
Lung transplants
Lymphatic system
Medical imaging
Metabolic disorders
Mutation
Nervous system
Patients
Pulmonary arteries
Steroids
Surgery
Tuberculosis
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Description
Summary:Niemann-Pick disease is a rare autosomal recessive disease characterized by an abnormal intracellular lipid accumulation. Type B is later in onset and a less severe form of the disease, so affected people may survive in adulthood. Storage of sphingomyelin in pulmonary macrophages can lead to interstitial lung disease. There are very few published cases of lung transplantation in patients with Niemann-Pick disease, all of them described in the last 2 years. We present here one case of a 57-year-old man successfully treated with a double-lung transplant.
Bibliography:Academic Editor: Federica Meloni
ISSN:2090-6943
2090-6951
DOI:10.1155/2022/5428381