Last R D, Hill J M, Roach M, Kaldenberg T
Vetdiagnostix--Veterinary Pathology Services, Cascades, South Africa.
J S Afr Vet Assoc. 2006 Dec;77(4):210-4. doi: 10.4102/jsava.v77i4.379.
Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease) described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.
两只13周龄的雄性金毛寻回犬幼犬同胞被诊断患有先天性肝内胆管囊肿，累及肝内胆管和节段性胆管，同时伴有双侧多囊肾病。两只幼犬肝脏的超声检查显示节段性囊性病变与胆管相连。组织病理学显示肝脏存在囊性扩张的胆管增生和发育异常，并伴有不同程度的门脉纤维化，而在肾脏中，集合管呈放射状排列、圆柱形扩张，延伸至髓质和皮质。这种病理变化与人类、犬类和大鼠中描述的先天性大胆管和节段性胆管扩张（卡罗利病）相符。在人类中，卡罗利病具有常染色体隐性遗传模式，而在大鼠中，MEK5/ERK级联反应的激活引发了该物种卡罗利病的胆管发育异常。然而，犬类卡罗利病的确切遗传模式和发病机制尚不清楚。此前关于犬类先天性肝囊性疾病的报道描述了不同品种中类似卡罗利病的病变，但据信这些是金毛寻回犬品种中首次报道的病例。
