HEMOCHROMATOSIS IN SALERS CATTLE
HEMOCHROMATOSIS IN SALERS CATTLE
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DOI：
10.1111/j.1939-1676.1994.tb03206.x
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发表时间：
1994-03-01
影响因子：
2.6
通讯作者：
PINO, MV
中科院分区：
文献类型：
--
作者：
HOUSE, JK;SMITH, BP;PINO, MV
对来自不同牧群的两头2岁的Salers牛进行了生长迟缓和腹泻的评估。肝酶活性升高和磺基溴邻苯二甲酸(BSP)半衰期延长(T1/2)提示肝病患者肝功能受损。肝活检的组织病理学检查显示肝细胞、库普弗细胞和小动脉内有明显的含铁血黄素沉积的小结节状硬化。转铁蛋白饱和度(TS)和肝脏铁含量显著升高，与血色素沉着症的诊断一致。由于病情恶化，两只动物都被安乐死。尸检结果包括肝脏肿大和含铁血黄素在肝脏、淋巴结、胰腺、脾、甲状腺、肾脏、脑和其他腺体组织中积聚。对第二个牛群(血清铁、总铁结合力[TIBC]、不饱和铁结合力[UIBC]和TS)的持续监测发现，一头小母牛在下一代被怀疑患有血色素沉着病。该动物的肝脏活检显示出与前两只动物相同的组织病理学变化，肝脏铁含量也有类似的增加(8,700ppm，正常范围为45至300ppm)。这3头受影响的牛都是品系育种计划的产物，拥有共同的祖先。缺乏膳食铁负荷与组织病理学和代谢结果相结合，与原发性血色素沉着症的诊断一致。报道的疾病类似于人类的特发性血色素沉着症，在这种疾病中，铁代谢存在遗传缺陷。
Two 2‐year‐old Salers cattle from different herds raised on pasture were evaluated for retarded growth and diarrhea. Increase of liver enzyme activities and prolonged sulfobromophothalein (BSP) half life (T1/2) indicated liver disease with impaired liver function. Histopathologic examination of liver biopsies revealed a micronodular cirrhosis with marked deposition of hemosiderin in hepatocytes, Kupffer cells, and arterioles. Transferrin saturation (TS) and liver iron content were markedly increased, consistent with a diagnosis of hemochromatosis. Both animals were euthanatized due to deterioration in their condition. Necropsy findings included hepatomegaly and hemosiderin accumulation in the liver, lymph nodes, pancreas, spleen, thyroid, kidney, brain and other glandular tissue. Continued surveillance of the second herd (serum iron, total iron binding capacity [TIBC], unsaturated iron binding capacity [UIBC], and TS), identified a heifer as a hemochromatosis suspect in a subsequent generation. Liver biopsies from that animal revealed the same histopathologic changes as the previous 2 animals, and similar increases in liver iron content (8,700 ppm, normal range 45 to 300 ppm). The 3 affected cattle were all products of line breeding programs and shared a common ancestor. The absence of dietary iron loading in conjunction with the histopathologic and metabolic findings were consistent with a diagnosis of primary hemochromatosis. The reported disease is similar to idiopathic hemochromatosis in human beings in which there is a hereditary defect in iron metabolism.