[A case of sudden death of a patient with glycogen storage disease type 1--delayed aftereffect of a traffic accident].
A case of sudden death of a 22-year-old man with glycogen storage disease type 1 is reported. Autopsy revealed striking hepatomegaly with round edges. The liver was yellowish and weighed 2840 g. Histologic findings showed excessive deposition of PAS-positive material in the hepatocytes and in the proximal renal tubular epithelium cells, which means the accumulation of glycogen. A mass surrounding splenic artery was seen in the hilus of the spleen. There was about 2000 ml of fresh blood and clots in the abdominal cavity due to bleeding from a ruptured mass. The mass was composed of a hematoma with connective tissue surrounding it and was a pseudoaneurysm. The mass was considered to have been formed from bleeding due to injury to a branch of a splenic artery. Consideration of these findings led to the conclusion that death was caused by loss of blood from rupture of the mass and that the mass was formed due to injury of the branch of the splenic artery in a traffic accident 2 years earlier but not the pathogenesis of glycogen storage disease.
Hatake
K
K
Department of Legal Medicine, Hyogo College of Medicine, Nishinomiya, Japan.
Kubota
A
A
Taniguchi
T
T
Ouchi
H
H
Minami
T
T
Hishida
S
S
D000063:Accidents, Traffic; D000328:Adult; D000783:Aneurysm / Q000209:etiology; D003645:Death, Sudden / Q000209:etiology*; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006406:Hematoma / Q000209:etiology; D006801:Humans; D008297:Male; D012422:Rupture, Spontaneous; D013157:Splenic Artery / Q000293:injuries