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大鼠中与骨肾综合征相关的遗传性多囊肾病

Hereditary polycystic kidney disease associated with osteorenal syndrome in rats.

作者信息

Kaspareit-Rittinghausen J, Rapp K, Deerberg F, Wcislo A, Messow C

机构信息

Department of Pathology, Veterinary School, Hannover, Federal Republic of Germany.

出版信息

Vet Pathol. 1989 May;26(3):195-201. doi: 10.1177/030098588902600302.

Abstract

Hereditary polycystic kidney disease (PKD) was seen in Han:SPRD rats and identified as an autosomal, incomplete dominant trait. Homozygous animals died at 3 to 4 weeks of age, while heterozygous males were severely ill or moribund within about 6 months. Heterozygous females developed PKD to a lesser extent than males and survived longer. Renal secondary hyperparathyroidism and osteodystrophia fibrosa were seen in most males but were absent in females. In male rats metastatic calcification was found in kidneys, lungs, glandular and forestomach, blood vessels, and heart. In a few uremic males ulcerative enteritis occurred in cecum and colon. Blood urea nitrogen values were significantly increased in both sexes.

摘要

遗传性多囊肾病(PKD)在Han:SPRD大鼠中被发现,并被确定为一种常染色体不完全显性性状。纯合子动物在3至4周龄时死亡,而异合子雄性在约6个月内病情严重或濒死。异合子雌性患PKD的程度比雄性轻,存活时间更长。大多数雄性出现肾性继发性甲状旁腺功能亢进和纤维性骨营养不良,而雌性则无此症状。在雄性大鼠中,在肾脏、肺、腺胃和前胃、血管及心脏发现转移性钙化。少数尿毒症雄性大鼠的盲肠和结肠出现溃疡性肠炎。两性的血尿素氮值均显著升高。

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