Neurovisceral Glucocerebroside Storage (Gaucher's Disease) in a Dog
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چکیده
منابع مشابه
Neurovisceral glucocerebroside storage (Gaucher's disease) in a dog.
Absruact. An 8-month-old Sydney Silky dog that was ataxic and hyperkinetic was found to have a neurovisceral storage disease. Typical Gaucher cells were seen in the liver, lymph nodes and cerebellum, but not in the spleen. Ultrastructurally, the storage bodies in Gaucher cells contained tubular structures, and many neurones contained laminated cytosomes, accumulations of a ‘wispy’ material and ...
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A 51 year old man presented in 1969 with slowly progressive cerebellar ataxia of unknown origin. He was admitted to hospital aged 68 after a fall, and a ruptured spleen was removed at laparotomy. Histological analysis of the spleen suggested Niemann-Pick disease, which was subsequently confirmed. He deteriorated and died of bronchopneumonia shortly afterwards: subdural haemorrhage with storage ...
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متن کاملGauchers disease presenting with portal hypertension.
Gauchers disease is a rare lysosomal storage disorder characterized by abnormal accumulation of lipid-laden macrophages in different organs. Though hepatosplenomegaly is commonly found, symptomatic presentation with portal hypertension is rare. We report a child with liver cirrhosis and bleeding esophageal varices who was diagnosed with Gaucher's disease.
متن کاملSelective effects of glucocerebroside (Gaucher's storage material) on macrophage cultures.
Although the enzymatic lesion in Gaucher's disease is well established, little is known concerning the pathogenic mechanisms involved in the clinical manifestations of the disease. In order to obtain insight into this unexplored aspect of Gaucher's disease, we examined the effects of glucocerebroside (GL(1)) at the cellular level in monolayers of cultured murine macrophages. The addition of GL(...
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ژورنال
عنوان ژورنال: Veterinary Pathology
سال: 1973
ISSN: 0300-9858,1544-2217
DOI: 10.1177/030098587301000302