Sclerema neonatorum is an uncommon and fatal disease which encountered in premature & debilitated newborn infants. Recently we experienced a case of sclerema neonatorum in premature infant with congenital heart disease which was confirmed by autopsy. We present a case with a brief review of literatures.
Autopsy ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Infant, Premature ; Sclerema Neonatorum*

A case of pycnodysostosis in 12 years old girl was presented with a brief review ofliterature. This patient was admitted to our pediatric dept. of N.P.H. with chief complaints of loss of appetite and respiratory difficulty. She was characterized by a peculiar face with anterior bossing, exophthalmos, opened anterior fontanel and coronal suture and dwarfism. So bone series was performed and showed character of pycnodysostosis such as opened anterior fontanel and coronal suture, receding mandibular angle, inoreased bone density with narrowing bone marrow and acrcosteolytica on distal phalanx. But she died on the 25 th hospital day due to chronic renal failure.
Appetite ; Bone Density ; Bone Marrow ; Child ; Cranial Fontanelles ; Dwarfism ; Exophthalmos ; Female ; Humans ; Kidney Failure, Chronic ; Pycnodysostosis* ; Sutures

This study was conducted to investigate the association of the occurrence of pediatric disease with environmental, seasonal and atmospheric factors. The data were collected at 5 pediatric clinics in Seoul and the Department of Pediatrics of Yongin Severance Hospital from May 1986 to April 1987. The results were as follows: 1. Vacation periods had a great influence upon the occurrence of pediatric diseases. 2. The majority of pediatric diseases occurred mainly in spring and autumn, not in summer and winter. 3. The higher the average relative humidity was, the less diseases occurred; and the higher the maximum change of daily temperature, the more diseases occurred. 4. In summer, the pattern of diseases varied along with the environmental factors(eg., toilet).
Gyeonggi-do ; Humidity ; Pediatrics ; Seasons* ; Seoul

Tremendous efforts have been made to develop better vaccines and diagnostic markers for the effective control of tuberculosis. Recently, we reported that the Triton X-100 soluble protein (TSP) of Mycobacterium tuberculosis induced strong T-cell proliferation and IFN-gamma production in humans, and also conferred a significant level of protection against tuberculosis in a mouse model. In this study, the TSP was prepared by Triton X-100 extraction of Mycobacterium tuberculosis bacilli, which was followed by Triton X-114 phase partitioning. Western blot analysis using sera of 177 active pulmonary tuberculosis patients and 323 healthy individuals revealed that the TSP contained a immunodominant 40-kDa antigen specifically reacting with some sera from pulmonary tuberculosis patients. The 40-kDa antigen was purified by ion-exchange chromatography, and partially characterized by two-dimensional gel electrophoresis and N-terminal sequencing. Results of this study suggest that 40-kDa molecule of the TSP antigen from the cell suface of Mycobacterium tuberculosis can be used as a serodiagnostic marker as well as a potential vaccine candidate against tuberculosis.
Animals ; Blotting, Western ; Chromatography, Ion Exchange ; Electrophoresis, Gel, Two-Dimensional ; Humans ; Mice ; Mycobacterium tuberculosis* ; Mycobacterium* ; Neptune* ; Octoxynol* ; T-Lymphocytes ; Tuberculosis ; Tuberculosis, Pulmonary ; Vaccines

Sclerosing epithelioid fibrosarcoma(SEF) is an uncommon tumor of the deep soft tissue. It was first described in 1995 by Meis-Kindblom et al. Histologically, this tumor is characterized by uniform, small, round to ovoid epithelioid cells with clear cytoplasm, and the cells are arranged in distinct nests and cords. On immunohistochemistry, the most consistently positive marker is vimentin, although other antigens(cytokeratin, epithelial membrane antigen, S100 protein and neuron specific enolase) have been recorded as being inconsistently positive. We report here on a case of a 46-year-old woman who presented with back pain. The radiologic findings revealed a right renal mass and multiple bone metastases. The patient underwent radical nephrectomy and the pathologic finding was primary SEF of the kidney.
Back Pain ; Cytoplasm ; Epithelioid Cells ; Female ; Fibrosarcoma* ; Humans ; Immunohistochemistry ; Kidney* ; Middle Aged ; Mucin-1 ; Neoplasm Metastasis ; Nephrectomy ; Neurons ; Vimentin

Primary urethral carcinoma accounts for less than 1% of the malignancies in males and adenocarcinoma accounts for 5% of all urethral carcinomas. A 36-year-old man with a history suggestive of urethral stricture was scheduled to undergo retrograde urethrography and visual urethrotomy. On urethroscopic examination, an abnormal polypoid mass was found in the proximal bulbous urethra, so transurethral resection biopsy was added to the urethrotomy. Histopathologic examination demonstrated urethral adenocarcinoma with no invasion to the corpus spongiosum and cavernosa on the magnetic resonance imaging. There was no evidence of metastasis and the search for the primary focus of adenocarcinoma revealed that the tumor originated from the urethra. The patient underwent segmental urethral resection and there has been no recurrence for 1-year follow-up.
Adenocarcinoma* ; Adult ; Biopsy ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Neoplasm Metastasis ; Recurrence ; Urethra ; Urethral Stricture

In clinical therapy, the current goal of dental implants is to enhance quantity and quality of osseointegration. Surface roughness and oxide structure are considered to influence the behavior of adherent cells. The purpose of this study is to evaluate the effect of different surface treatment on cellular response. The attachment and proliferation of osteoblast-like cell on sandblasted, sandblasted and etched, thermal oxidated surfaces have been compared. Sandblasting was done with Al2O3 particles(grain size of 50micrometer), etching was processed with NH4OH : H2O2 : H2O(1:1:5) at 90degrees C for 1 minute. Thermal oxidation was followed sandblasting and etching at 400degrees C, 600degrees C, 800degrees C for 2 hours. Measurement of surface roughness after the different treatment did not show any differences of Ra value between terated surfaces. Cell attachment and proliferation were increased during experiment period, but no difference was observed. SEM evaluation revealed a similar pattern of osteoblastlike cells, well attached with dendritic extension and producing numerous matrix vesicles on cell surface. The results of this study showed that oxide layer alteration by thermal oxidation did not affect the attachment and proliferation of osteoblast-like cells. This suggests the possibility that the cellular responses are further influenced by surface roughness than titaniun oxide structure. This study was supported by a grant(HMP-98-G-2-035-B) of the HAN(highly advanced National) Projected, Ministry of Health & Welfare, R.O.K
Dental Implants ; Osseointegration ; Osteoblasts

BACKGROUND: This study was done to analyze the treatment outcomes of adult lymphoblastic lymphoma patients according to the risk groups and the treatment regimens. METHODS: The analysis was performed on twenty patients histologically diagnosed as lymphoblastic lymphoma at Seoul National University Hospital. The high-risk group was defined as patients with Ann Arbor stage IV with bone marrow, central nervous system (CNS) involvement or initial serum lactate dehydrogenase level more than 1.5 times upper normal limits. Twelve patients received dose-intensive Stanford/Northern California Oncology Group (NCOG) regimen consisted of four phase of induction, CNS prophylaxis, consolidation, and maintenance. Eight patients received conventional dose regimen, either six courses of vincristine, bleomycin, cyclophosphamide, doxorubicin, prednisolone, procarbazine (COPBLAM-V) or cyclophosphamide, vincristine, doxorubicin, prednisolone, L-asparaginase (CHOP/L-ASP) with CNS prophylaxis. We analyzed the response rate and the survival rate according to the risk groups and treatment regimens. RESULTS: The overall response rate was 90% (75% complete response). In low-risk group, the complete response (CR) rate was 100% irrespective of treatment regimen. In high-risk group, conventional dose regimen did not produce CR. Four of the six high-risk patients receiving dose-intensive regimen achieved CR. The 5-year overall survival (OS) rate was 52% in total patients. The 5-year OS rate were 77% and 30% for low- and high-risk group, respectively (P=0.242). In low-risk group, conventional dose regimen showed similar survival outcomes compared with that of dose intensive regimen. Toxicity profile was more favorable in the patients with conventional dose regimens. CONCLUSION: For low-risk patients, conventional dose regimen showed similar effect in comparison with dose-intensive regimen. However, for high-risk patients, CR was observed only with dose-intensive regimen. Multi-center clinical trials are necessary to confirm our observation.
Adult ; Male ; Female ; Humans