Neurofibromatosis Type‐1 Lung Disease Complicated by Pleuroparenchymal Fibroelastosis

Neurofibromatosis type-1 is a rare autosomal dominant disease, due to the loss of the NF1 tumour suppressor gene. Here we present a case of a 28-year-old man with neurofibromatosis type-1 lung disease and pleuroparenchymal fibroelastosis leading to recurrent pneumothoraxes requiring intervention.

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Publication Details

Journal
Respirology Case Reports
Published
2025-02-28
DOI
https://doi.org/10.1002/rcr2.70137
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
Field-Weighted Citation Impact
0.00
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article

Neurofibromatosis Type‐1 Lung Disease Complicated by Pleuroparenchymal Fibroelastosis

Nicole Goh, Abhir Nainani, Hugh Buzacott
Respirology Case Reports
Neurofibromatosis and Schwannoma Cases
article

Neurofibromatosis Type‐1 Lung Disease Complicated by Pleuroparenchymal Fibroelastosis

Nicole Goh, Abhir Nainani, Hugh Buzacott
article en

Abstract

Neurofibromatosis type-1 is a rare autosomal dominant disease, due to the loss of the NF1 tumour suppressor gene. Here we present a case of a 28-year-old man with neurofibromatosis type-1 lung disease and pleuroparenchymal fibroelastosis leading to recurrent pneumothoraxes requiring intervention.

Respirology Case ReportsVol. 13(3)
Austin Health (AU)
Openalex Percentile: Top 93%
Neurofibromatosis and Schwannoma Cases
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Neurofibromatosis Type‐1 Lung Disease Complicated by Pleuroparenchymal Fibroelastosis — Nicole Goh, Abhir Nainani, et al. · Respirology Case Reports (2025) | TGRS Research Map | TGRS