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Lipid storage disease.

机译：脂质贮积病。

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An assessment is made of 8 cases (6 published) of cholesterol ester storage disease (CLSD) in fox-terriers to identify the potential use of fox-terriers as models for human disease. All the cases showed a retarded state of development with poorly developed skeletal muscle, enlarged yellowish liver with friable parenchyma and enlarged spleen. Lesions of the eyes were present in all cases. Lipid-containing macrophages were found predominantly in the liver, spleen, lymph nodes, intestinal mucosa and bone marrow. A comparison of 2 CLSD cases with 2 control dogs showed considerably higher cholesterol concentrations in liver samples of those with CLSD. It is suggested that the longer progression of CLSD from onset to death in the fox-terrier cases compared with that of human patients may indicate different degrees of cholesterol esterase deficiency in dogs and human beings. It was concluded that the clinical and morphological findings in 5 of the cases involving CLSD in fox-terriers closely resemble those of the human disease known as Wolman's disease and therefore animals suffering from CLSD could be used for improvement of therapeutic strategies in man.
机译：对狐狸梗中的8例胆固醇酯贮积病（CLSD）进行了评估（6篇发表），以确定狐狸梗作为人类疾病模型的潜在用途。所有病例均表现出发育迟缓的状态，骨骼肌发育不佳，肝黄色泛滥，实质薄弱，脾脏肿大。在所有情况下都存在眼部病变。含脂质的巨噬细胞主要存在于肝脏，脾脏，淋巴结，肠粘膜和骨髓中。将2例CLSD病例与2只对照犬进行比较后，发现CLSD病例的肝脏样本中的胆固醇浓度明显较高。有人认为，与人类患者相比，狐狸梗犬从发病到死亡的CLSD病程更长，可能表明狗和人体内胆固醇酯酶缺乏程度不同。结论是，狐狸梗中5例涉及CLSD的病例的临床和形态学发现与被称为沃尔曼氏病的人类疾病极为相似，因此患有CLSD的动物可用于改善人类的治疗策略。

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