| Literature DB >> 29354164 |
Ufuk Demirci1, Ahmet Çizmecioglu2, Ismet Aydogdu3.
Abstract
Gaucher's disease (GD) is a rare disease characterized by a β-glucocerebroside accumulation in the reticulo-endothelial system. Patients may refer to the clinic with complaints of bone pain, hepatosplenomegaly, anemia, thrombocytopenia, growth retardation, interstitial pulmonary disease, pulmonary hypertension, and skeletal disorders. Skeletal system involvement is observed commonly in Gaucher patients and a significant cause of morbidity. Our patient was followed for several years as a glass child - osteogenesis imperfecta and he had joint deformities due to skeletal fractures. We wanted to present this case to raise awareness of GD's skeletal involvement and effects of late diagnosis.Entities:
Keywords: Gaucher Disease; fracture; glass-child; osteogenesis imperfecta; β-glucocerebroside
Year: 2017 PMID: 29354164 PMCID: PMC5762226 DOI: 10.11138/ccmbm/2017.14.3.336
Source DB: PubMed Journal: Clin Cases Miner Bone Metab ISSN: 1724-8914