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Cor pulmonale in a case of infantile Gaucher's disease

Infantile Gaucher's disease presenting as cor pulmonale is rarely reported in pediatric literature. We report a 3.3 year old boy with infantile Gaucher's disease who presented to us as interstitial lung disease, pulmonary hypertension along with features of cor pulmonale. The high resoluti...

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Detalles Bibliográficos
Autores principales: Patra, Soumya, Chatterjee, Sayan, Singhla, Shilpi, Patra, Bijoy, Maheswari, Anu, Seth, Anju
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3125004/
https://www.ncbi.nlm.nih.gov/pubmed/21731876
http://dx.doi.org/10.4103/2230-8210.81948
Descripción
Sumario:Infantile Gaucher's disease presenting as cor pulmonale is rarely reported in pediatric literature. We report a 3.3 year old boy with infantile Gaucher's disease who presented to us as interstitial lung disease, pulmonary hypertension along with features of cor pulmonale. The high resolution CT findings were typical of interstitial and airspace disease. Cor pulmonale in this patient was a result of severe pulmonary hypertension.