Clinical, morphologic, and biochemical characteristics of Chediak-Higashi syndrome in fifty-six Japanese black cattle.
Clinical, morphologic, and biochemical characteristics of Chediak-Higashi syndrome in fifty-six Japanese black cattle.
复制标题
五十六头日本黑牛 Chediak-Higashi 综合征的临床、形态和生化特征。
DOI：
10.2460/ajvr.1997.58.11.1221
复制
发表时间：
1997
影响因子：
1
通讯作者：
Hiromitu Otsuka
中科院分区：
文献类型：
--
作者：
Hiroyuki Ogawa;Chien;Hitoshi Kagamizono;Kozo Soki;Yuziro Inoue;Hiroto Akatsuka;Shun;Takeru Wada;Masahisa Ikeya;Susumu Makimura;Kazuyuki Uchida;R. Yamaguchi;Hiromitu Otsuka
目的
研究日本黑牛Chediak-Higashi综合征（C-HS）的特征。
动物
200头牛中有56头患有出血性疾病和白细胞中的巨大颗粒。
程序
进行临床观察、全血细胞计数、止血筛选试验、血小板聚集测定、电子显微镜、血小板成分分析和检眼镜检查。
结果
受影响的日本黑牛出血倾向增加，白细胞中有异常颗粒。感染易感性未增加。皮肤白化病在6头新生小牛中很明显，但在大多数受影响的牛中不明显。在所有受影响的牛，绒毡层底部是苍白的，非绒毡层底部几乎没有色素。电子显微镜下观察到血小板中致密颗粒的数量显著减少。在功能上，胶原诱导的血小板聚集显著减少。
结论
这种出血性疾病被诊断为C-HS。与人类和赫里福德牛的C-HS相比，日本黑牛C-HS的感染易感性、白化病和死亡率的临床表现为中度。
临床相关性
由于常染色体隐性遗传模式的记录和隐性纯合子可以很容易地检测到，C-HS在日本黑牛可以控制。
OBJECTIVE
To characterize Chediak-Higashi syndrome (C-HS) in Japanese Black cattle.
ANIMALS
56 of 200 cattle with a bleeding disorder and giant granules in leukocytes.
PROCEDURE
Clinical observation, CBC, hemostatic screening test, platelet aggregometry, electron microscopy, platelet constituent analysis, and ophthalmoscopic examination were done.
RESULTS
Affected Japanese Black cattle had increased bleeding tendency and abnormal granules in their leukocytes. Susceptibility to infection was not increased. Cutaneous albinism was evident in 6 new-born calves, but not in most affected cattle. In all affected cattle, the tapetal fundus was pale and the nontapetal fundus was almost devoid of pigment. By electron microscopy, a remarkable decrease in the number of dense granules in platelets was observed. Functionally, collagen-induced platelet aggregation was markedly reduced.
CONCLUSIONS
This bleeding disorder was diagnosed as C-HS. With regard to susceptibility to infection, albinism, and mortality, clinical manifestations of C-HS in Japanese Black cattle were moderate, compared with C-HS in human beings and Hereford cattle.
CLINICAL RELEVANCE
Because an autosomal recessive mode of inheritance was documented and recessive homozygotes could be easily detected, C-HS in Japanese Black cattle can be controlled.