Ferrari A H, Miyagui T, Rodrigues F R, Borges C B, do Carmo C C
Departamento de Patologia, Universidade Federal Fluminense (UFF), Hospital Universitário Antônio Pedro (HUAP).
Arq Gastroenterol. 1998 Jan-Mar;35(1):49-53.
Acute liver insufficiency, followed by heart disease, pneumopathy and infectious syndrome is reported in a 36 year old brown alcoholic man in which the necropsy revealed anatomopathological changes suggesting sickle cell trait. Acute ischemic lesions were noted in the heart, kidneys, central nervous system and liver. In the absence of other causes explaining the ischemic lesions, they were attributed to circulatory changes related to sickle cell trait. Hepatic failure was consequent to functional disturbances derived from the occlusion of sinusoids and necrosis of hepatocytes. The factor responsible for sickling, which caused recent ischemic lesions, may have been the respiratory insufficiency secondary to lung disease. Chronic ischemic lesions have been seen in the heart and may be related to previous sickling episodes. Subsequent to necropsy findings, screening of family members revealed a daughter with sickle cell trait.
据报道,一名36岁的棕色人种酗酒男子出现急性肝功能不全,随后并发心脏病、肺病和感染综合征,尸检显示解剖病理学变化提示镰状细胞性状。心脏、肾脏、中枢神经系统和肝脏均发现急性缺血性病变。在没有其他原因解释这些缺血性病变的情况下,它们被归因于与镰状细胞性状相关的循环变化。肝功能衰竭是由于肝血窦阻塞和肝细胞坏死引起的功能障碍所致。导致镰状化并引起近期缺血性病变的因素可能是继发于肺部疾病的呼吸功能不全。心脏中可见慢性缺血性病变,可能与先前的镰状化发作有关。尸检结果出来后,对家庭成员进行筛查发现一名女儿有镰状细胞性状。