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猫体内假定的原发性和继发性肝铜蓄积

Presumed primary and secondary hepatic copper accumulation in cats.

作者信息

Hurwitz Brandi M, Center Sharon A, Randolph John F, McDonough Sean P, Warner Karen L, Hazelwood Kanda S, Chiapella Ann M, Mazzei Michael J, Leavey Kathy, Acquaviva Anthony E, Lindsay Mary M, Sanders Leslie, Pintar Jason

机构信息

Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853.

出版信息

J Am Vet Med Assoc. 2014 Jan 1;244(1):68-77. doi: 10.2460/javma.244.1.68.

Abstract

OBJECTIVE

To determine signalments, clinical features, clinicopathologic variables, imaging findings, treatments, and survival time of cats with presumed primary copper-associated hepatopathy (PCH) and to determine quantitative measures and histologic characteristics of the accumulation and distribution of copper in liver samples of cats with presumed PCH, extrahepatic bile duct obstruction, chronic nonsuppurative cholangitis-cholangiohepatitis, and miscellaneous other hepatobiliary disorders and liver samples of cats without hepatobiliary disease.

DESIGN

Retrospective cross-sectional study.

ANIMALS

100 cats with hepatobiliary disease (PCH [n = 11], extrahepatic bile duct obstruction [14], cholangitis-cholangiohepatitis [37], and miscellaneous hepatobiliary disorders [38]) and 14 cats without hepatobiliary disease.

PROCEDURES

From 1980 to 2013, cats with and without hepatobiliary disease confirmed by liver biopsy and measurement of hepatic copper concentrations were identified. Clinical, clinicopathologic, and imaging data were compared between cats with and without PCH.

RESULTS

Cats with PCH were typically young (median age, 2.0 years); clinicopathologic and imaging characteristics were similar to those of cats with other liver disorders. Copper-specific staining patterns and quantification of copper in liver samples confirmed PCH (on the basis of detection of > 700 μg/g of liver sample dry weight). Six cats with PCH underwent successful treatment with chelation (penicillamine; n = 5), antioxidants (5), low doses of elemental zinc (2), and feeding of hepatic support or high-protein, low-carbohydrate diets, and other hepatic support treatments. One cat that received penicillamine developed hemolytic anemia, which resolved after discontinuation of administration. Three cats with high hepatic copper concentrations developed hepatocellular neoplasia.

CONCLUSIONS AND CLINICAL RELEVANCE

Results suggested that copper accumulates in livers of cats as primary and secondary processes. Long-term management of cats with PCH was possible.

摘要

目的

确定疑似原发性铜相关肝病(PCH)猫的信号特征、临床特征、临床病理变量、影像学表现、治疗方法和生存时间,并确定疑似PCH、肝外胆管阻塞、慢性非化脓性胆管炎 - 胆管肝炎以及其他各种肝胆疾病的猫肝脏样本中铜的积累和分布的定量测量及组织学特征,以及无肝胆疾病猫的肝脏样本情况。

设计

回顾性横断面研究。

动物

100只患有肝胆疾病的猫(PCH [n = 11]、肝外胆管阻塞 [14]、胆管炎 - 胆管肝炎 [37] 和其他各种肝胆疾病 [38])以及14只无肝胆疾病的猫。

步骤

在1980年至2013年期间,通过肝活检和肝铜浓度测量确定患有和未患有肝胆疾病的猫。比较患有和未患有PCH的猫的临床、临床病理和影像学数据。

结果

患有PCH的猫通常较年轻(中位年龄,2.岁);临床病理和影像学特征与其他肝脏疾病的猫相似。肝脏样本中铜特异性染色模式和铜定量证实了PCH(基于检测到肝样本干重>700μg/g)。6只患有PCH的猫通过螯合治疗(青霉胺;n = 5)、抗氧化剂(5)、低剂量元素锌(2)以及给予肝脏支持或高蛋白、低碳水化合物饮食和其他肝脏支持治疗获得成功治疗。1只接受青霉胺治疗的猫出现溶血性贫血,停药后缓解。3只肝铜浓度高的猫发生肝细胞肿瘤。

结论及临床意义

结果表明铜在猫肝脏中以原发性和继发性过程积累。对患有PCH的猫进行长期管理是可行的。

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