Studdert M J
Aust Vet J. 1978 Sep;54(9):411-7. doi: 10.1111/j.1751-0813.1978.tb05562.x.
Set in a context of immunodeficiency diseases in general this paper provides a brief, illustrated review of a primary, severe, combined immunodeficiency (PSCID) disease of Arabian foals. Affected foals are clinically normal at birth but beginning at about 10 days of age they develop a range of clinical signs particularly bronchopneumonia and diarrhoea with which adenoviruses are peculiarly associated. Despite intensive therapy foals invariably die by about 3 months of age. Affected foals are profoundly lymphopagenic (greater than 1000 lymphcoytes per mm3). There is thymic and lymph node hypoplasia and all lymphoid tissues are profoundly depleted of both T and B lymphocytes. The depletion of both T and B lymphocytes suggests that the primary defect is at the level of bone marrow stem cells which are the precursor cells for both lymphocyte populations. PSCID of Arabian foals is inherited as a simple, autosomal, recessive gene. Some 2 to 3% of all such foals may be born with PSCID, this frequency corresponds to a gene frequency of about 30% in parents. The syndrome is, therefore, an important cause of economic wastage. It also represents the only occurrence of the syndrome in an animal species other than man and as such has considerable comparative interest.
本文以一般免疫缺陷疾病为背景，简要且配有插图地回顾了阿拉伯马驹的一种原发性、严重、联合免疫缺陷（PSCID）疾病。患病马驹出生时临床症状正常，但大约在10日龄时开始出现一系列临床症状，特别是支气管肺炎和腹泻，腺病毒与这些症状有特殊关联。尽管进行了强化治疗，马驹通常在3个月龄左右死亡。患病马驹淋巴细胞生成严重（每立方毫米大于1000个淋巴细胞）。存在胸腺和淋巴结发育不全，所有淋巴组织中的T淋巴细胞和B淋巴细胞都严重减少。T淋巴细胞和B淋巴细胞均减少表明主要缺陷在于骨髓干细胞水平，而骨髓干细胞是这两种淋巴细胞群体的前体细胞。阿拉伯马驹的PSCID作为一种简单的常染色体隐性基因遗传。所有此类马驹中约有2%至3%可能出生时患有PSCID，这一频率对应于父母中约30%的基因频率。因此，该综合征是经济损失的一个重要原因。它也是该综合征在人类以外的动物物种中唯一出现的情况，因此具有相当大的比较研究价值。
