A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

A 7 day old female baby suffered from perinatal asphyxia and trauma at birth. Subsequently, she had a violeceous subcutaneous plaque on back. The clinical setting, the skin manifestation and the histologic findings of the lesion were diagnostic of subcutaneous fat necrosis of the newborn. Histologic findings revealed fat necrosis with infiltration of inflammatory cells, foreign body giant cells and several fat cells contain needle shaped cleft in radial arrangement. Four weeks latter, the skin lesion was healed spontaneously.
Adipocytes ; Asphyxia ; Fat Necrosis ; Female ; Giant Cells, Foreign-Body ; Humans ; Infant, Newborn* ; Necrosis* ; Needles ; Parturition ; Skin ; Skin Manifestations ; Subcutaneous Fat*

No abstract available.
Nocardia Infections*

To investigate the imbalance of the cytokine production profile of T cells from atopic asthmatics, we measured concentrations of IL-4, IL-5 and IFN-y by ELISA method in the culture supernatants of peripheral blood mononuclear cells(PBMCs) and Derrnato-phagoides pteronyssinus(Der p) J-stimulated PBMCs from Der p-sensitized atopic asthmatics, Der p-sensitized healthy atopits, non-atopic asthmatics and healthy non-atopics. The suppressive effect of IFN-y on cytokine production of Der p J-stimulated PBMCs was also examined. The PBMCs from atopics showed higher IL-4 and IL-5 production in response to PHA +TPA and higher IFN-gamma production in response to Der p Jq compared with non-atopits. The Der p J-stimulated PBMCs from atopics showed a tendency of increased IL-5 production in response to Der p J and higher IL-4 and IL-5 production in response to PHA+TPA compared with non-atopics. IL-5 production of Der p J-stimulated PBMCs from atopics was suppressed by IFN It is suggested that an imbalance in IL-4, IL-5 and IFN-y production is a feature of the atopic state. The TH2 characteristics of allergen-stimulated PBMCs could be regulated by IFN-y.
Enzyme-Linked Immunosorbent Assay ; Interleukin-4 ; Interleukin-5 ; T-Lymphocytes

BACKGROUND: Familial aggregation of coronary risk factors is well known, which are hypertension, hyperlipidemia, diabetes mellitus, and obesity. However inter-spouse relation of the risk factors has remained controversial. As spouses are not blood-related, any risk factor relation that exist probably may not reflect genetic factors, but the environmental factors to which the couple is exposed. Moreover, the effect of assortative marriage should be considered. This study is designed to identify inter-spouse relation of coronary risk factors and to document the effects of marriage duration to spouse concordance. METHODS: From May 1995 to March. 1999, we enrolled 1,859 couples who had visited the Health Promotion Center at Seoul National University Hospital. We analysed inter-spouse correlation of body mass index, blood pressure, glucose, serum lipid factors, uric acid, smoking, alcohol drinking, and stress known as coronary risk factors. The correlation coefficients were compared according to marriage duration. RESULTS: There was statistically significant spouse concordance for all risk factors with the exception of smoking. The correlation coefficients of blood pressure, total cholesterol, and triglyceride increased with marriage duration but troughed at 30-39 years of marriage. Those of body mass index and glucose peaked at early and late marriage duration. Those of HDL-cholesterol and uric acid was relatively fixed throughout marriage. CONCLUSION: The presence of significant inter-spouse correlation for most of the coronary risk factors reflect the influence of assortative marriage and environmental factors on spouse concordance. If a spouse has many cardiovascular risk factors, partner should be evaluated for risk factors.
Alcohol Drinking ; Blood Pressure ; Body Mass Index ; Cardiovascular Diseases ; Cholesterol ; Diabetes Mellitus ; Family Characteristics ; Glucose ; Health Promotion ; Humans ; Hyperlipidemias ; Hypertension ; Marriage* ; Obesity ; Risk Factors* ; Seoul ; Smoke ; Smoking ; Spouses* ; Triglycerides ; Uric Acid

No abstract available.
Humans ; Infant, Newborn*

No abstract available.
Anencephaly* ; Humans

Invasive infection of the Streptococcus bovis group in a neonate is rare. In cases reported to date, the pathogen of neonatal S. bovis infections is usually Streptococcus gallolyticus subsp. pasteurianus (S. bovis biotype II/2). Streptococcus lutetiensis (S. bovis biotype II/1) was identified using 16S rRNA and tuf gene sequence analysis of the isolates from blood and cerebrospinal fluid (CSF) of a fever-presenting 28-day-old male. Blood culture analysis was performed using automatic equipment (VITEK 2) and identified Streptococcus infantarius supsp. infantarius, yet we were unable to get accurate results from the CSF culture. The fever subsided on the second day of hospitalization, and the patient was discharged without neurologic complication after 14 days of antibiotic therapy. In this case, we were able to accurately identify the pathogen using molecular genetic methods. To our knowledge, this is the first case of late onset neonatal bacteremia and meningitis caused by S. lutetiensis.
Bacteremia* ; Cerebrospinal Fluid ; Fever ; Hospitalization ; Humans ; Infant, Newborn ; Male ; Meningitis* ; Molecular Biology ; Sequence Analysis ; Streptococcus bovis ; Streptococcus*

T cell lymphoblastic lymphoma is characterized by immature lymphoid cells that are indistinguishable from the lymphoblasts and prolymphocytes of acute lymphoblastic leukemia. Several characteristic clinical features of lymphoblastic lymphoma, with include a high male-to-female ratio, a relatively high incidence in older children and young adults, the frequent presence of mediastinal involvement at the time of diagnosis. Also, this disease is rapidly progressive, and early dissemination to the bone marrow, blood, and central nervous system leads to the evolution of a picture resembling a acute lymphoblastic leukemia. We have experienced a rare case of congenital T-cell lymphoblastic lymphoma in which 1/365 year old female newborn had generalized multiple irregular protruding mass on her body. On bone marrow biopsy, CSF analysis, ultrasonogram and whole body MRI studies. We found metastasized tumor mass to, orbit, abdominal cavity, bulva, skin, and lower extremities. Histopathologically, specimen from mass on the right thigh showed diffuse infiltration of poorly differentiated and immature lymphoid cells in the skelectal muscle and subcutaneous soft tissue. By immunophenotyping studies using anti T cell and B cell monoclonal antibody, these cells were reactive with UCHL-1, MB2, but unreactive with MB1, L26. Based on these findings, the lesion was diagnosed as lymphoblastic lymphoma of the T-cell type, which occurred congenitally. Most T cell lymphoblastic lymphoma are noted, but congenital case was not reported. So we report it with a brief review of literature.
Abdominal Cavity ; Biopsy ; Bone Marrow ; Central Nervous System ; Child ; Diagnosis ; Female ; Humans ; Immunophenotyping ; Incidence ; Infant, Newborn ; Lower Extremity ; Lymphocytes ; Magnetic Resonance Imaging ; Orbit ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Skin ; T-Lymphocytes ; Thigh ; Ultrasonography ; Young Adult

PURPOSE: Ginseng has been reported to reduce blood glucose levels in diabetic animals and patients, and it is also reported to slow the aging process by acting as an anti-atherosclerotic agent or as an anti-oxidant. This study was designed to investigate whether ginseng and irbesartan can prevent the development of diabetic nephropathy in streptozotocin-induced diabetic rats. METHODS: Diabetes was induced in 7 week-old male Sprague-Dawley rats by intravenous injection of 60 mg/kg streptozotocin. Ginseng(1 g/kg/day) or irbesartan (20 mg/kg/day) was given to diabetic rats for 25 weeks. Blood glucose and body weight were checked weekly and urinary albumin excretion was evaluated every 6 weeks. At the end of the experiment, the kidneys were weighed and sliced for microscopic examination. Glomerular size and hyaline deposition were measured on light microscopy(on Masson' trichrome stain and PAS stain) and thickness of glomerular basement membrane(GBM) on electron microscopy. Renal histologic findings of ginseng or irbesartan treated rats were compared with those of normal control and diabetic control groups. RESULTS: The weight gain in diabetic rats was significantly reduced, and the final body weight of diabetic rats was lower than that of normal control rats. There was no significant difference in body weights between the diabetic control, ginseng, and irbesartan treated groups. Mean levels of blood glucose were significantly increased in diabetic rats compared to normal rats, but there was no significant difference in blood glucose among the three groups of diabetic rats. Urinary albumin excretion was increased in the diabetic groups compared to the normal control group, and it was significantly decreased in the irbesartan treated group compared to the diabetic control group at 13th week of treatment. At the end of the experiment, the kindeys of the diabetic rats were examined and showed significantly enlarged than those of the normal rats, and the ratio of kidney weight to body weight was decreased in the ginseng treated group compared to the diabetic control and irbesartan treated group. There was no significant difference in the size of glomerulus, the thickness of GBM, and glomerular hyaline deposition among the three diabetic groups. CONCLUSION: There was no significant hypoglycemic effect of ginseng in streptozotocin-induced diabetic rats. Renal hypertrophy was relatively milder in the ginseng-treated group, but there was no difference in findings of renal histology between the treatment groups.
Aging ; Animals ; Blood Glucose ; Body Weight ; Diabetic Nephropathies* ; Humans ; Hyalin ; Hypertrophy ; Hypoglycemic Agents ; Injections, Intravenous ; Kidney ; Male ; Microscopy, Electron ; Panax* ; Rats ; Rats, Sprague-Dawley ; Streptozocin ; Weight Gain