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Pleuroparenchymal fibroelastosis in an 8‐year old treated for neuroblastoma
Author(s) -
Gerstle Karyn,
Tanager Kevin,
Hegde Satyanarayan,
Chung Jonathan H.,
Husain Aliya N.,
Applebaum Mark A.,
Dyamenahalli Umesh
Publication year - 2020
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.24638
Subject(s) - medicine , endocardial fibroelastosis , lung , interstitial lung disease , lung transplantation , respiratory system , chemotherapy , disease , respiratory disease , pediatrics
Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.