Original contributionPulmonary pathology in Gaucher's disease
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2021, Pediatric Clinics of North AmericaAllogeneic hematopoietic stem cell transplantation for treating severe lung involvement in Gaucher disease
2020, Molecular Genetics and Metabolism ReportsCitation Excerpt :Results of a 10-year follow-up in non-splenectomized patients were also significant: increase in hemoglobin levels and platelet count, decrease in liver and spleen volumes, improvement in DXA Z-scores, and reduction in bone crises [3]. Pulmonary involvement in Gaucher disease (GD) includes interstitial lung disease, alveolar/lobar consolidation, pulmonary hypertension, and hepatopulmonary syndrome [4]. It is less common than visceral, hematologic, and bone manifestations.
Respiratory complications of metabolic disease in the paediatric population: A review of presentation, diagnosis and therapeutic options
2019, Paediatric Respiratory ReviewsLessons from lung transplantation: Cause for redefining the pathophysiology of pulmonary hypertension in gaucher disease
2019, Respiratory Medicine Case ReportsCitation Excerpt :PH in GD1 has been classified into WHO Group 5, for unclear or multifactorial mechanisms [11]. The classic pathophysiologic explanation for PH in GD1 is that Gaucher cells deposit in the pulmonary parenchyma and capillaries, thus obstructing blood flow [8,12]. However, severe PH in GD1 has been described with little-to-no evidence of Gaucher cells within the lungs [2,13].
Histological characterisation of visceral changes in a patient with type 2 Gaucher disease treated with enzyme replacement therapy
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