Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma.
Pediatr Pulmonol
; 55(3): E1-E4, 2020 03.
Article
in En
| MEDLINE
| ID: mdl-31944579
ABSTRACT
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pleura
/
Lung Diseases, Interstitial
/
Parenchymal Tissue
Type of study:
Etiology_studies
Limits:
Child, preschool
/
Female
/
Humans
Language:
En
Journal:
Pediatr Pulmonol
Journal subject:
PEDIATRIA
Year:
2020
Document type:
Article