Pathogenesis and development of genetic diagnosis for combined immunodeficiency in Japanese Black beef cattle

免疫缺陷综合征定义为7头雄性和1头雌性日本黑牛犊。受影响的牛犊在10日龄至80日龄期间发病。所有的小牛都有腹泻、肺率增加和轻度吸气困难，并在2-4周的时间里变得越来越严重。犊牛出现淋巴细胞减少和低蛋白血症，血清IgG1、IgG2、IgM和IgA浓度极低。尸检时，淋巴结、脾、胸腺和Peyer‘s斑块明显小于正常。弥漫性肺不张和弥漫性出血坏死性溃疡是由前胃毛霉菌感染和皱胃引起的。胸腺皮质和髓质中淋巴细胞减少，皮髓分化很少或没有分化。日本黑小牛的这种免疫缺陷综合征的临床和病理结果与人和阿拉伯马的严重联合免疫缺陷非常相似。

Immunodeficiency syndrome was defined in 7 male and 1 female Japanese Black beef calves. Affected calves became ill from 10 to 80 days of age. All calves had diarrhea, increased pulmonary rate, and mild inspiratory dyspnea, and become progressively worse over a period of 2-4 weeks. The calves had lymphopenia and hypoproteinemia with extremely low concentration of serum IgG1, IgG2, IgM and IgA. At necropsy, lymph nodes, spleen, thymus, and Peyer's patches were appreciably smaller than normal. Disseminated pulmonary atelectasis and diffuse hemorrhagic and necrotizing ulcer caused by the infection of Mucorales in the forestomach and abomasum were present. Depletion of lymphocytes in the cortex and medulla with little or no corticomedullary differentiation were in thymus. Clinical and pathologic findings of this immunodeficiency syndrome in Japanese Black calves were closely similar to severe combined immunodeficiency of humans and Arabian horses.