纤维囊性病与克山病的病因学,脏器病理学的共同点

克山病(Keshan Disease、简称 KSD)与纤维囊性病(Cystic Fibrosis 简称 CF)有其共同特点,如心肌呈散在性灶性坏死和纤维变,发病年龄多为胎儿或学龄前儿童等。而肝脏和胰腺损害为纤维囊性病的特征性病变。本文作者对克山病尸检材料作了进一步研究。在已确诊的1700例克山病病例中发现595例(35%)有纤维囊性病的胰腺改变,均为胎儿和学龄前儿童。其中850例(50%)表现出不同程度的灶性胆汁性肝硬变,85例(5%)发展成严重的小叶性肝硬化。纤维囊性病和克山病的另一共同点是易感个体的硒元素缺乏,易感个体多为胎龄17周至6个月的胎儿或是早期幼儿。克山病和纤维囊性病缺硒的表现是有区别的,但在基本的细胞水平及组织水平的病理生理学上,二者即便不是完全一致,已是十分相近的。

Common Denominatorrs in The Etiology and Pathology of Visceral Lesions of Cystic Fibrosis and Keshan Dlsease

The common denominator of a unique disseminated multifocalmilliary myocardial hyaline necrosis and fibrosis in Keshan Disease (KSD)and Cystic Fibrosis (CF) and a commonality of the affected age groups of fetusesand preschool children led to the review of existing KSD autopsy material tosearch for pancreatic and hepatic lesions considered pathognomonic for CF.Pancreatic lesions considered pathognomonic for CF were found in 595 or 35per cent of 1700 documented cases of KSD.The pancreatic lesions were limited totissues of fetuses and preschool children.Varying degrees of focal biliary cirrhosis were identified in 850 or 50 percent of the KSD autopsies and 85 or 5 per cent developed severe lobular cirrhosis.The common denominator in CF and KSD appears to be a selenium deficiencyin age susceptable humans either prenatally at or around 22 weeks of fetallife or early post natal life.The natural history of the apparent selenium defici-ency differs between KSD and CF,however,the basic pathophysiology at thetissue and cellular levels seems to be similar if not identical.