Hoffmann G, van den Ingh T S G A M, Bode P, Rothuizen J
Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Netherlands.
J Vet Intern Med. 2006 Jul-Aug;20(4):856-61. doi: 10.1892/0891-6640(2006)20[856:cchilr]2.0.co;2.
This study summarizes the clinical and pathologic findings in 15 Labrador Retrievers with copper-associated chronic hepatitis (CACH). Our hypothesis was that this form of hepatitis is caused by a defect in hepatic copper metabolism, which most likely originates from a genetic defect. Affected Labradors consisted of 11 female and 4 male Labrador Retrievers. Eight family members of 2 of these patients were examined prospectively, as were 6 unrelated healthy Labrador Retrievers. All dogs were registered at the breed club. The average age at clinical presentation was 7 years (range, 2.5-10.5 years). All dogs were presented for anorexia, which was associated with vomiting in 8 patients. The diagnosis of CACH was based on histologic examination of liver biopsy specimens in all dogs, including semiquantitation of copper. A disproportionate increase in alanine aminotransferase (ALT) activity relative to alkaline phosphatase (ALP) activity, as well as the centrolobular localization of copper and the association of copper accumulation with hepatic lesions, suggested a primary copper storage disease rather than primary cholestatic liver disease causing copper accumulation. Mean hepatic copper concentration measured in related Labradors was 1,317 microg/g dry weight liver (range, 402-2,576 microg/g). Mean hepatic copper concentration of unrelated normal Labradors was 233 microg/g dry weight liver (range, 120-304 microg/g). Our findings support the hypothesis that a hereditary form of hepatitis occurs in Labrador retrievers and is caused by a defect in hepatic copper metabolism.
本研究总结了15只患有铜相关性慢性肝炎（CACH）的拉布拉多寻回犬的临床和病理表现。我们的假设是，这种肝炎形式是由肝脏铜代谢缺陷引起的，而这很可能源于遗传缺陷。受影响的拉布拉多犬包括11只雌性和4只雄性。对其中2只病犬的8个家庭成员进行了前瞻性检查，同时也检查了6只无亲缘关系的健康拉布拉多犬。所有犬只均在品种俱乐部登记。临床发病时的平均年龄为7岁（范围为2.5 - 10.5岁）。所有犬只均因厌食就诊，其中8只伴有呕吐。所有犬只的CACH诊断均基于肝活检标本的组织学检查，包括铜的半定量分析。丙氨酸转氨酶（ALT）活性相对于碱性磷酸酶（ALP）活性不成比例地升高，以及铜在中央小叶的定位和铜蓄积与肝脏病变的关联，提示这是一种原发性铜贮积病，而非导致铜蓄积的原发性胆汁淤积性肝病。相关拉布拉多犬肝脏铜的平均浓度为1317微克/克干重肝脏（范围为402 - 2576微克/克）。无亲缘关系的正常拉布拉多犬肝脏铜的平均浓度为233微克/克干重肝脏（范围为120 - 304微克/克）。我们的研究结果支持这样的假设，即拉布拉多寻回犬中存在一种遗传性肝炎，其由肝脏铜代谢缺陷引起。
