T Cell Numbers Relate to Bone Involvement in Gaucher Disease

T cell numbers relate to bone involvement in Gaucher disease.

The major elements of bone pathology in Gaucher disease are a failure of osteoclast and osteoblast function, resulting in osteopenia and also osteonecrosis. T lymphocytes have recently been found to be involved in the regulation of osteoblast/osteoclast activity in vitro. In the present report the peripheral blood T major lymphocyte subsets were investigated in a group of genotyped type 1 Gauch...

Pulmonary involvement in Gaucher disease

http://dx.doi.org/10.1590/0100-3984.2016.0070 Leonor Garbin Savarese1, Henrique Simão Trad2, Edwaldo Edner Joviliano1, Valdair Francisco Muglia1, Jorge Elias Junior1 1. Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil. 2. CEDIRP – Central de Diagnóstico Ribeirão Preto, Ribeirão Preto, SP, Brazil. Mailing address: Dra. Leonor Garbin Sava...

Bone involvement in sickle cell disease.

Bone involvement is the commonest clinical manifestation of sickle cell disease both in the acute setting such as painful vaso-occlusive crises, and as a source of chronic, progressive disability such as avascular necrosis. Management of these problems is often difficult because of the diagnostic imprecision of most laboratory and imaging investigations and because of the lack of evidence for m...

Involvement of hepcidin in iron metabolism dysregulation in Gaucher disease

Gaucher disease (GD) is an inherited deficiency of glucocerebrosidase leading to accumulation of glucosylceramide in tissues such as the spleen, liver, and bone marrow. The resulting lipid-laden macrophages lead to the appearance of "Gaucher cells". Anemia associated with an unexplained hyperferritinemia is a frequent finding in GD, but whether this pathogenesis is related to an iron metabolism...

Bone Involvement in Neuroblastoma