잠시만 기다려 주세요. 로딩중입니다.

Wilson씨 병의 1 부검례

An Autopsy Case of Wilson´s Disease

부산의대잡지 1968년 8권 1호 p.243 ~ 251
김순호, 김익형, 안도철, 박영림,
소속 상세정보
김순호 (  ) - 부산대학교 의과대학 병리학교실
김익형 (  ) - 부산대학교 의과대학 병리학교실
안도철 (  ) - 부산대학교 의과대학 병리학교실
박영림 (  ) - 부산대학교 의과대학 병리학교실

Abstract


A case of Wilson´s disease identified by postmortem examination in the body of a 11 year-old Korean boy is to be reported.
The patient admitted to the Pusan Charity Hospital in April, 1962, because of frequent convulsions and aphasia. His family history was obscure except for the fact that his father died of unknown disease when he was 3 year old. Five days before admission, he developed headache and pain in the left leg associated with convulsions and aphasia. During the convulsive state his body became spastic.
On admission he developed rhythmical tremor in the extremities and muscular rigidity in the left leg but his consciousness was clear. No Kayser-Fleischer ring was recognized at the cornea. There was no hepatosplenomegaly. The neurological examination revealed positive Kernig´s sign on both sides and Babinsky´s sign on the left side. The tremor and rigidity appeared repeatedly in every two to three minutes and usually lasted for several minutes. On the seventeen hospital day the patient died with convulsion
Clinically this case was suspected as cerebral paragonimiasis and treated as such with emetin chloride and sepamin.
The liver weighed 310 gm. and measured 17 by 11 by 8 cm. The surface of this organ was very nodular. The nodules were nearly uniform in size, varying from 0.5 to 1.0 cm. and were distributed evenly on the entire surface. They were rather light grayish brown in color, while the intervening fibrous tissue was brown. On the cut surface of the liver the nodules were found again to be distributed evenly throughout. The nodular tissue was rather friable and the intervening fibrous thissue was firm. Histologically, the liver lobules were divided into two to three psedolobules by broad bands of a proliferating fibrous tissue originating from the portal areas and interlobular fibrous tissue. Within the fibrous bands there were found many proliferating bile ducts and considerable numbers of small round cells. Some of the liver cells composing the pseudolobules contained fat droplets, golden-yellow pigments in their cytoplasm. -Some of the nuclei of the liver cells revealed glycongen degeneration. In places, there were noted enlarged Kupffer cells.
The brain weighed 975 gm. Grossly, there were found softening, necrosis and cavity formation in both lenticular nuclei. Gray brown discoloration was seen about the necrotic lesions. Histologically, the most characteristic feature of the lesion was marked proliferation of astrocytes. Alzheimer type I and II cells and Opalski cells were found here and there in the degenerative lesions. In the lesions of the cerebral cortex and white matter, particularly of the subcortical areas, found in the frontal and parietal
lobes and :n the thalamic structures, there were noted a mild hyperplasia of astrocytes and a few Alzheimer type II and I cells and Opalski cells. No inflammatory infiltrate were observed throughout the brain.
Unfortunately, we could not determine tissue copper and serum cerulopasmin.

키워드

원문 및 링크아웃 정보

등재저널 정보