No abstract available.

A case of Epidermolysis Bullosa Dystrophica is reported with review of pathogenesis with literatures. Epidermolysis Bulloaa Dystrophica is a rare genetic disease which is characterized by the formation of blister upon the minor trauma or friction to the skin. A 7-year old boy was referred to our dept. in Aug. 1976 because of a severe bullous eruption primarily on his hands and feet. On admission tense bullae were noted on the dorsa of hands and feet which is usually serous but may be hermorhagic tendency. Soon thereafter tbis bullous lesion tend to break down into painful shallow denudation which may leave depigmented scars and milia after healing. Family history was negative for skin disorders.
Blister ; Child ; Cicatrix ; Epidermolysis Bullosa Dystrophica* ; Epidermolysis Bullosa* ; Foot ; Friction ; Hand ; Humans ; Male ; Skin ; Transcutaneous Electric Nerve Stimulation

No abstract available.
Fatigue*

We report a case of morphea profunda in a 21-year-old male who had diffuse brown sclerotic plaques on his extremities. Laboratory findings showed peripheral eosinophilia and an increased titers for anti-DNA and anti-nuclear antibodies. Histopathologic findings showed diffuse fibrosis and a thickening of the lower dermis and subcutaneous tissue. He has been treated with hydroxychloroquine 400mg per day and the sclerosis of the skin improved.
Antibodies ; Dermis ; Eosinophilia ; Extremities ; Fibrosis ; Humans ; Hydroxychloroquine ; Male ; Scleroderma, Localized* ; Sclerosis ; Skin ; Subcutaneous Tissue ; Young Adult

Shaken baby syndrome refers to the group of nonaccidental injuries occuring in infants and children as a consequence of violent shaking. The characteristic injuries include subdural and subarachnoid hemorrhages, retinal hemorrhages, traction-typed metaphyseal fracture of long bone and fractures of the ribs. General physical findings may include bruising and burns, but sometimes no extracranial injuries are detected. Affected children are nearly always under 2 years of age or younger. Common symptoms include lethargy, irritability, seizures, increased or decreased muscle tone, poor feeding, impaired consciousness, vomiting and apnea. We experienced a case of shaken baby syndrome in a 3-month-old girl. Although the history of trauma(shaking) is little known, our findings of clinical and radiographic features corresponded to shaken baby syndrome. (J Korean Pediatr Soc 2000;43:851-855)
Apnea ; Burns ; Child ; Consciousness ; Female ; Humans ; Infant ; Lethargy ; Muscle Hypotonia ; Retinal Hemorrhage ; Ribs ; Seizures ; Shaken Baby Syndrome* ; Subarachnoid Hemorrhage ; Vomiting

To investigate the diagnostic accuracy of ERCP in pancreatobiliary disease, the authors analysed the 1,244 cases of ERCP. The success rate of ERCP was 91.5%. The diagnostic accuracy of ERCP was 93.6% and complications occurred in 2.1%. Complications included pancreatitis,cholangitis and drug reactions. Among the biliary tract disease diagnosed, by ERCP, biliary tract stone was the most common finding. Compared with operative diagnosis of biliary tract stones,the sensitivity of ERCP diagnosis was 92.7% while that of sonographic diagnosis was 81.4%. The specificity of ERCP diagnosis was 94.4% while that of sonagraphic diagnosis was 80.9%. ln the diagnosis of common bile duct stones, ERCP was more accurrate than sonographic diagnosis. The results show that ERCP was a safe and effective diagnostic tool in the assessment of pancreatobiliary tract disease.
Biliary Tract ; Biliary Tract Diseases ; Cholangiopancreatography, Endoscopic Retrograde* ; Common Bile Duct ; Diagnosis ; Sensitivity and Specificity ; Ultrasonography

To investigate the diagnostic accuracy of ERCP in pancreatobiliary disease, the authors analysed the 1,244 cases of ERCP. The success rate of ERCP was 91.5%. The diagnostic accuracy of ERCP was 93.6% and complications occurred in 2.1%. Complications included pancreatitis,cholangitis and drug reactions. Among the biliary tract disease diagnosed, by ERCP, biliary tract stone was the most common finding. Compared with operative diagnosis of biliary tract stones,the sensitivity of ERCP diagnosis was 92.7% while that of sonographic diagnosis was 81.4%. The specificity of ERCP diagnosis was 94.4% while that of sonagraphic diagnosis was 80.9%. ln the diagnosis of common bile duct stones, ERCP was more accurrate than sonographic diagnosis. The results show that ERCP was a safe and effective diagnostic tool in the assessment of pancreatobiliary tract disease.
Biliary Tract ; Biliary Tract Diseases ; Cholangiopancreatography, Endoscopic Retrograde* ; Common Bile Duct ; Diagnosis ; Sensitivity and Specificity ; Ultrasonography

We report an autopsy case of the brain stem glioma that extended extensively in the brain stem itself and cephalad. This 18-year-old boy first presented with dizziness, vomiting and left side weakness with left facial palsy. Brain MRI revealed a diffusely infiltrative tumor involving whole medulla, pons and lower midbrain. A total of 4000 R was given with some alleviation of respiratory difficulty. He died one year after the onset. Autopsy revealed the tumor involving pons, a portion of medulla oblongata, and cerebellum. The tumor showed diffusely infiltrative pattern and extended along the periventricular area to the thalamus and corpus callosum. The cut surface was grayish white and solid. It also showed areas of myxoid degeneration and necrosis probably related to radiation therapy. Microscopically the tumor was a cellular and pleomorphic glioma that showed some astrocytic differentiation. It was diffuse without geographic necrosis.

This study was undertaken to document the effect of transforming growth factor-beta2 (TGF-beta2(TGF-beta2) and basic fibroblast growth factor (bFGF) on the proliferation of pig retinal pigment epithelial cells (RPE). Whereas bFGF increased the proliferation, TGF-beta2 showed the inhibitory effect on the proliferation The inhibitory effect of TGF-beta2 disappeared in RPE subcultured with 10ng/ml of bFGF. Both TGF-beta2- and bFGF-specific antisense oligonucleotides blocked the autocrine effect of the growth factors. PLC-71 -specific antisense oligonucleotide inhibited the effect of TGF-beta2 and bFGF. Genistein inhibited the effect of TGF-beta2 and bFGF in dose-dependent man, ner. The data suggest the involvement. of in PLC-/1 and tyrosine kinase in signalling.
Epithelial Cells* ; Fibroblast Growth Factor 2 ; Genistein ; Intercellular Signaling Peptides and Proteins ; Oligonucleotides, Antisense ; Protein-Tyrosine Kinases ; Retinaldehyde* ; Transforming Growth Factor beta2*

BACKGROUND: A and B transferase are glycosyltransferase that transfer N-acetylgalactosamine and D- galactose to H antigen, respectively and lead to the expression of A and B phenotypes in ABO blood group system. Reduced or no activities of serum A and B transferase were observed in some A and B subgroup individuals. Determining the activities of serum A and B transferase can be useful in discriminating rare A and B subgroups. MATERIALS AND METHODS: ABO typing, saliva test, adsorption elution test and serum transferase assay were performed on samples from 12 individuals showing ABO discrepancy or weakened cell typing reactions which were referred to the Seoul National University Hospital to confirm their ABO blood types. Serum transferase activity was assayed by determining the ability of serum to convert group 0 RBCs into A or B cells. RESULTS: Determination of serum ABO transferase activity was useful in the identification of Ael (3 cases), B. (2 cases), Bm (1 case), Am (1 case), Bx (1 case), 0 with weakened anti-A or anti-B (3 cases), and A without anti-B due to hypogammaglobulinemia (1 case). CONCLUSION: Determining serum A and B glycosyltransferase activity was proven to be a simple and useful tool for the classification of several ABO subgroups.(Korean J Blood Transfusion 10(1): 27-33, 1999)
ABO Blood-Group System ; Adsorption ; Agammaglobulinemia ; B-Lymphocytes ; Blood Transfusion ; Classification* ; Galactose ; Phenotype ; Saliva ; Seoul ; Transferases