Sokal E M, Van Hoof F, Alberti D, de Ville de Goyet J, de Barsy T, Otte J B
Department of Paediatrics, Université Catholique de Louvain, Hôpital St. Luc, Brussels, Belgium.
Eur J Pediatr. 1992 Mar;151(3):200-3. doi: 10.1007/BF01954384.
Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectionosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.
由于早期进行性肝硬化，原位肝移植（OLT）已被提议用于治疗IV型糖原贮积症患者。报告显示，OLT后其他器官无疾病进展，甚至有一例患者心脏支链淀粉浸润消退。我们描述了一名15个月大的儿童，因IV型糖原贮积症接受了肝移植。OLT前无肝外疾病的临床体征。9个月后，患者出现进行性心功能不全，死于心力衰竭。由于大量支链淀粉沉积、心肌细胞肌原纤维减少以及排除了其他心力衰竭原因，死亡归因于支链淀粉沉着症。我们的观察结果与匹兹堡的经验不同，提示OLT后心脏支链淀粉沉着症可能进展。
