Double-Lung Transplantation in a Patient with Pulmonary Type B Niemann-Pick Disease : A Valid Treatment Option
Copyright © 2022 Víctor Manuel Cuesta Mora et al..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:2022 |
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| Enthalten in: |
Case reports in transplantation - 2022(2022) vom: 23., Seite 5428381 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Mora, Víctor Manuel Cuesta [VerfasserIn] |
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| Anmerkungen: |
Date Revised 16.07.2022 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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| doi: |
10.1155/2022/5428381 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM340597305 |
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| 520 | |a 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 | ||
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| 700 | 1 | |a Gómez, José Javier Román |e verfasserin |4 aut | |
| 700 | 1 | |a Cifrián, José Manuel Martínez |e verfasserin |4 aut | |
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