Rozengurt N
Department of Pathology and Infectious Diseases, Royal Veterinary College, London, UK.
J Comp Pathol. 1994 Apr;110(3):295-301. doi: 10.1016/s0021-9975(08)80282-4.
Endocardial fibroelastosis (EFE) is a pathological disorder characterized by a diffuse and elastic thickening of the endocardium. It is often thought to be congenital as most victims are infants who die of congestive heart failure before reaching one year of age. A naturally occurring animal model of EFE was proposed more than a decade ago on the basis of one pair of adult pure-bred Burmese cats which produced offspring consistently affected with EFE. In the present study a case of EFE is described which occurred in a closed colony of experimental SPF cats. A 5-week-old kitten which was found dead showed hydrothorax, hydropericardium and oedematous lungs at necropsy. Histological examination of the heart revealed left atrio-ventricular dilation with severe, diffuse endocardial thickening which did not extend into the myocardium. A retrospective study was carried out on stored tissues of two further kittens and one adult male cat from the colony which had died during a period of 2 years. The hearts of all the animals showed endocardial thickening in the left atria and ventricle but not in the right chambers. Over a period of 12 years of existence of this colony considerable inbreeding had occurred and it is concluded that an inherited factor was responsible for the disorder.
心内膜弹力纤维增生症(EFE)是一种以心内膜弥漫性弹性增厚为特征的病理紊乱。通常认为它是先天性的,因为大多数受害者是在一岁前死于充血性心力衰竭的婴儿。十多年前,基于一对成年纯种缅甸猫能持续产出患EFE后代,提出了一种EFE的自然发生动物模型。在本研究中,描述了一例发生在实验用无特定病原体(SPF)封闭猫群中的EFE病例。一只5周龄死亡小猫尸检时发现有胸腔积液、心包积液和肺部水肿。心脏组织学检查显示左房室扩张,伴有严重的、弥漫性的心内膜增厚,且未延伸至心肌。对该猫群中另外两只小猫和一只成年雄猫在两年内死亡时留存的组织进行了回顾性研究。所有动物的心脏均显示左心房和心室的心内膜增厚,而右心房和心室未出现增厚。在这个猫群存在的12年里发生了大量近亲繁殖,得出结论认为该紊乱是由遗传因素导致的。