Lüftner J, Scheibner K, Wöckel W
Zentralbl Allg Pathol. 1977;121(1-2):195-200.
In 25 cases of autoptically proven mucoviscidosis 3 cases were detected with hepatic infestation. In two cases there was a focal biliary fibrosis (one male baby of 3 months, one female baby of 8 months), the third case was a 7 1/2 years old boy with a multilobulary biliary cirrhosis. The morphologie picture of these lesions is described, and their frequency, etiology and pathogenesis are discussed. Secondary alterations of the liver are for more frequent in this disorder than real liver infestations of mucoviscidosis, possible implications of this fact are shortly mentioned, too.
在25例经尸检证实的黏液黏稠病中,发现3例有肝脏受累。2例有局灶性胆管纤维化(1例3个月大的男婴,1例8个月大的女婴),第3例是一名7岁半的男孩,患有多叶性胆汁性肝硬化。描述了这些病变的形态学表现,并讨论了它们的发生率、病因和发病机制。在这种疾病中,肝脏的继发性改变比黏液黏稠病真正的肝脏受累更为常见,也简要提及了这一事实的可能影响。