We reported a case of Wilson's disease, which was histologically confirmed by liver biopsy in a 15 year-old boy and made a comprehensive analysis on the sum of 22 cases reported in Korean literature. Although Wilson's disease is still rare, it should be included in the differential diagnosis of chronic liver diseases particularly in children of 5 to 15 years old. For the clinical diagnosis of Wilson's disease, changes in serum ceruloplasmin, plasma copper and urine copper values are most important as well as Kayser-Fleischer ring and family hestory. The serum level of GOT, when elevated, were always higher than GPT. This atypical transaminase profile may be a clue for diagnosis of hepatic diseases by a metabolic derangement. Among various histologic changes of the liver in Wilson's disease, what have diagnostic importance are anisonucleosis, nuclear glycogenosis and Mallory body, all of which may appear in any stages of the disease. In 3 cases only neurologic symptoms such as dysarthria and athetosis were found. Hemolytic anemia was another rare complication.
Child ; Male ; Female ; Humans ; Diagnosis, Differential ; Biopsy

No abstract available.
DNA, Mitochondrial* ; Essential Tremor* ; Humans

Authors studied how Claude Bernard, the first founder of experimental medicine, contributed significantly to establishment of modernism and influenced European modern culture. Authors first studied his views on modernity, comparing with Descartes and Magendie, and on the similarity between "Experimental medicine" and the European literature in the 19th century. Bernard was not exclusively against vitalism, but the dogmatic misuse of vitalism. His objective thinking could be a useful model for the authors, who considered science to be an origin of modernity in literature of naturalism. Especially, Emile Zola was strongly influenced by Bernard's "An introduction to the study of Experimental medicine" and published "Experimental novel," a manifesto of naturalism. Although Bernard's experimental methodology and determinism deeply influenced modern European culture, the relationship between his Experimental medicine and modernism have not been fully investigated yet. His experimental medicine also needs to be discussed from the ecological viewpoints. His anthropo-centrism was unique since he emphasized any human theory could not surpass the principle of nature. Conventional anthropo-centrism claims that human beings are superior enough to own and govern the nature. And Bernard's the necessary determinism contains the ecological principle that all life forms and inanimate objects are organically related and intertwined to each other, irrespectively of their usefulness for the human beings. Although there were some ethical debates related to his medical experiments on living bodies of animal, his strict principle to perform experiments only after animal or human body died was worth considering as an effort to sustain ecological viewpoints. He was also unique in terms of being realistic and candid about his situation which was limited by the 19th century's scientific and medical development. In conclusion, the significance of convergence of literature and medical science in Experimental medicine and the importance of Bernard's ecological viewpoints, need to be further studied in the field of medical history.
Animals ; Ecology ; Human Body ; Humans ; Photosensitizing Agents ; Silanes ; Thinking ; Vitalism

Primary hepatic sarcoma is a rare tumor, with fewer than 60 previously reported cases. It is thought to arise from hepatic connective tissue or vascular channels. The tumor is located usually in the intrahepatic area. Pedunculated or bulging lesion is also reported. We report a case of primary leiomyosarcoma of the liver occuring in a 62-year-old Korean woman. A pedunculated, 21 16 cm sized mass is located in the left lateral segment of the liver. Microscopically, the tumor is hypercellular and composed of elongated, partly pleomorphic spindle cells with blunt-ended nuclei, which are focally reactive for muscle specific actin. Mitotic figures are 5/10 high power fields (HPFs). No other primary site is recognized on clinical study.
Actins ; Connective Tissue ; Female ; Humans ; Leiomyosarcoma* ; Liver ; Middle Aged ; Sarcoma

To elucidate the histologic pattern of alcoholic liver disease (ALD) in Korea, liver biopsies from 173 chronic alcoholics with clinical liver diseases were classified according to the pathologic parameters. One hundred and seventeen cases, the sum of 91 of 116 serum HBsAg negative and 26 of 57 HBsAg positive patients, had the histologic evidence of ALD. Fatty change(23.9%), alcoholic fibrosis (AF)(23.1%) and cirrhosis (23.1%), comprised the three major ALDs, and only 8.5% of cases fit the criteria of alcoholic hepatitis. Chronic sclerosing hyaline disease (CSHD), chronic active alcoholic hepatitis (CAAH) and AF, where non-cirrhotic fibrosis is the predominant change, comprised 44.5% of ALD. Both features of ALD and HBV liver disease (HBV-LD) were found in 17 cases that included 8 AF and 7 cirrhosis. These 17 patients tended to consume less alcohol than patients with other types of pure ALD except alcoholic heaptitis. Patients with the serum HBsAg positive ALD (37.4years) were about 8 years younger than those with the serum HBsAg negative ALD (45.1years). More or less fatty change and foamy degeneration were seen in 77.4% and 31.6% of ALD respectively. Mallory bodies, megamitochondria, iron deposition and perihepatocellular fibrosis were found in 20.5%, 29.9%, 42.7% and 77.8%, respectively. These findings indicate that non-cirrhotic chronic ALD such as CSHD, CAAH and AF are the important histologic patterns of ALD in Korea, and that chronic alcohol consumption and HBV may act synergistically in developing liver disease.
Biopsy

No Abstract Available.
Lupus Erythematosus, Systemic*

No Abstract Available.
Mucinoses*

While most dermal melanocytoses are congenital or have an onset in early childhood, there is an acquired group with a late onset. We describe a case of aldolescent onset, unilateral, macular dermal melanocytosis on the hand. A 16-year-old girl presented with a 10-month history of gray-bluish pigmentation on the right palm and the volar side of the right fourth finger. The light microscopic studies showed spindle-shaped dermal melanocytes. The S-100 protein staining of these cells was positive and CD68 staining was negative. Dermal melanocytosis of the macular type, as observed in our case, is very rare.
Adolescent ; Female ; Fingers ; Hand* ; Humans ; Melanocytes ; Pigmentation ; S100 Proteins

Bullous pemphigoid(BP) and herpes gestationis(HG) are subepidermal bullous diseases which show clinical and immunological similarities. Both diseases show immune deposits along the basement membrane zone and their autoantibodies bind a common antigenic site within the non-collagenous stretch of the 180 kDa BPAG2 ectodomain. Besides its association with pregnancy, HG has some characteristic features that distinguish it from BP. The serum of patients with HG often contains an IgG that avidly fixes complement, and showes IgG1 subclass predominance. We report here two cases of non-pregnant young women presenting clinical and histological features of bullous pemphigoid or herpes gestationis. The immunopathology, IgG subtyping and immunoblotting studies showed that the autoantibodies in the patients were the characteristic ones of herpes gestationis. The patients might be a subtype of BP that have characteristics of autoantibodies of HG patients.
Autoantibodies* ; Basement Membrane ; Complement System Proteins ; Female ; Humans ; Immunoblotting ; Immunoglobulin G ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy

No abstract available.
Hypoglycemia*