Gaucher's disease in a patient presenting with hip and abdominal pain
Rev. Assoc. Med. Bras. (1992, Impr.)
; 63(12): 1025-1027, Dec. 2017. graf
Article
in En
| LILACS
| ID: biblio-896327
Responsible library:
BR1.1
ABSTRACT
Summary Gaucher's disease is characterized by glucocerebroside accumulation in the cells of the reticuloendothelial system. There are three subtypes. The most common is type 1, known as the non-neuropathic form. Pancytopenia, hepatosplenomegaly and bone lesions occur as a result of glucocerebroside accumulation in the liver, lung, spleen and bone marrow in these patients. Findings associated with liver, spleen or bone involvement may be seen at radiological analysis. Improvement in extraskeletal system findings is seen with enzyme replacement therapy. Support therapy is added in patients developing infection, anemia or pain. We describe a case of hepatosplenomegaly, splenic infarction, splenic nodules and femur fracture determined at radiological imaging in a patient under monitoring due to Gaucher's disease.
Key words
Full text:
1
Index:
LILACS
Main subject:
Pain
/
Femur Head
/
Gaucher Disease
Type of study:
Diagnostic_studies
/
Etiology_studies
Limits:
Female
/
Humans
Language:
En
Journal:
Rev. Assoc. Med. Bras. (1992, Impr.)
Journal subject:
EducaÆo em Sa£de
/
GestÆo do Conhecimento para a Pesquisa em Sa£de
/
MEDICINA
Year:
2017
Type:
Article