Johnstone A C, Jones B R, Thompson J C, Hancock W S
Batchelar Agriculture Centre, Palmerston North, New Zealand.
J Comp Pathol. 1990 Feb;102(2):125-37. doi: 10.1016/s0021-9975(08)80118-1.
The gross and histological features of congenital lipoprotein lipase deficiency are described in eight cats. The main histological features could be directly related to the presence of the chylomicronaemia. They consisted of lipid accumulation within clear vacuoles or ceroid accumulation within residual bodies in parenchymatous organs such as the liver, spleen, lymph nodes, kidney and adrenal gland. Xanthomata were seen in various sites, probably arising either from frank haemorrhage or the leakage of lipid-rich plasma perivascularly. As in human lipoprotein lipase deficiency there was no evidence of the formation of atherosclerotic plaques. Focal degenerative changes were, however, present within arteries and this may indicate blood vessel weakness and explain the tendency to haemorrhage and xanthomata/granulomata formation. The degeneration and fibrous replacement of glomeruli and nephrons possibly arises from pressure necrosis of adjacent xanthomata and alterations in renal blood flow.
本文描述了8只患有先天性脂蛋白脂肪酶缺乏症猫的大体和组织学特征。主要组织学特征与乳糜微粒血症的存在直接相关。其表现为实质器官（如肝脏、脾脏、淋巴结、肾脏和肾上腺）中透明空泡内的脂质蓄积或残余小体内的类蜡质蓄积。在不同部位可见黄瘤，可能是由明显出血或富含脂质的血浆血管周围渗漏引起的。与人类脂蛋白脂肪酶缺乏症一样，没有动脉粥样硬化斑块形成的证据。然而，动脉内存在局灶性退行性改变，这可能表明血管脆弱，并解释了出血倾向以及黄瘤/肉芽肿形成的原因。肾小球和肾单位的变性和纤维性替代可能源于相邻黄瘤的压迫性坏死以及肾血流的改变。
