Nezelof's syndrome(combined immunodeficiency with immunoglobulin) is a hereditary primary immunodeficiency characterized by recurrent chronic pulmonary infections, oral and cutaneous candidiasis, failure to thrive, chronic diarrhea, skin infection, urinary tract infection, gram-negative sepsis, severe progressive varicella infection, lymphopenia, diminished lymphoid tissue, abnormal structure of the thymus, and presence of normal or increased levels of one or more of the major immunoglobulin classes, but with impaired antibody synthesis. We experienced a case of Nezelof's syndrome in 5-month-old boy who complained cough, poor feeding, vomiting and diarrhea in first admission day and discharged with recovered general condition in 38th admission day and then died of recurrent bacterial and fungal infections and malnutrition in 15-month-old age. He had no thymic shadow in chest X-ray and immunologic abnormalities including decreased lymphocyte count, decreased T cell count and function, normal number of B cell count and immunoglobulins with funtional impairment in antibody synthesis. We report a case of Nezelof's syndrome with brief review of related literatures.
Candidiasis, Cutaneous ; Cell Count ; Chickenpox ; Cough ; Diarrhea ; Failure to Thrive ; Humans ; Immunoglobulin Isotypes ; Immunoglobulins ; Infant ; Lymphocyte Count ; Lymphoid Tissue ; Lymphopenia ; Male ; Malnutrition ; Sepsis ; Skin ; Thorax ; Thymus Gland ; Urinary Tract Infections ; Vomiting

PURPOSE: Adiponectin is an adipocyte-derived plasma protein with various metabolic effects that include increasing insulin sensitivity, antiatherogenic, and antiinflammatory properties. The purpose of this study was to investigate the association of cord plasma adiponectin levels with body size, ponderal index, and gender in newborns and also age and body mass index in their mothers. METHODS: The cord blood was obtained from 99 healthy newborns (male 46, female 53, gestational age of 32-41 weeks) and the concentrations of adiponectin were analyzed by a radioimmunoassay kit. Anthropometric parameters of the newborns including birth weight and length were measured. Maternal weight and height were identified, and their body mass index was calculated. RESULTS: The cord plasma adiponectin levels of the newborns whose gestational age was longer than 39 weeks were significantly higher compared with those of gestational age shorter than 39 weeks (15.0+/-9.9 vs 8.4+/-8.9 microgram/mL, P=0.001). The cord plasma adiponectin concentrations were positively correlated with gestational age and length at birth of the newborns. There was no correlation between cord plasma adiponectin levels and sex, birth weight or ponderal index of the newborns. Any significant correlation was not found between cord plasma adiponectin levels and maternal age or body mass index. CONCLUSION: These findings indicate that cord plasma adiponectin concentrations are positively associated with gestational age and length at birth of neonates. However there is no correlation between cord plasma adiponectin levels and maternal age or body mass index.
Adiponectin* ; Birth Weight* ; Body Mass Index ; Body Size ; Female ; Fetal Blood* ; Gestational Age ; Humans ; Infant, Newborn* ; Insulin Resistance ; Maternal Age ; Mothers ; Parturition* ; Plasma ; Radioimmunoassay

. This study aims to compare the marginal fitness of two types of implant-supported fixed dental prosthesis, i.e., cementless fixation (CL.F) system and cement-retained type. MATERIALS AND METHODS. In each group, ten specimens were assessed. Each specimen comprised implant lab analog, titanium abutment fabricated with a 2-degree tapered axial wall, and zirconia crown. The crown of the CL.F system was retained by frictional force between abutment and relined composite resin. In the cement-retained type, zinc oxide eugenolcement was used to set crown and abutment. All specimens were sterilized with ethylene oxide, immersed in Prevotella intermedia culture in a 50 mL tube, and incubated with rotation. After 48 h, the specimens were washed thoroughly before separating the crown and abutment. The bacteria that penetrated into the crownabutment interface were collected by washing with 500 μL of sterile saline. The bacterial cell number was quantified using the agar plate count technique. The BacTiter-Glo Microbial Cell Viability Assay Kit was used tomeasure bacterial adenosine triphosphate (ATP)-bioluminescence, which reflects the bacterial viability. The t-test was performed, and the significance level was set at 5%. RESULTS. The number of penetrating bacterial cells assessed by colony-forming units was approximately 33% lower in the CL.F system than in the cement-retained type (P<.05). ATP-bioluminescence was approximately 41% lower in the CL.F system than in the cementretained type (P<.05). CONCLUSION. The CL.F system is more resistant to bacterial penetration into theabutment-crown interface than the cement-retained type, thereby indicating a precise marginal fit.

Papillary adenoma of the lung is a very rare tumor in humans. Papillary adenoma is benign, and morphologically distinctive neoplasm.Since Fanton et al. firstly described a bronchial tumor showing Clara cell features,only a few cases having features of both Clara cells and type II pneumocytes have been reported.The immunohistochemical and ultrastructural findings hint at an origin from type II pneumocytes or Clara cells.Noguchi et al. demonstrated the presence of surfactant apoprotein in cytoplasm of tumor cells in a case of papillary adenoma indicating type II pneumocyte-like differenciation of the tumor cells. The tumor was encounted in a asymptomatic patient in a mass-survey chest X-ray examination. The chest X-ray films showed the tumor as well dermacated small lesion. We reports a case of papillary adenoma of the lung with pulmomary sequestration with review of articles.
Adenoma* ; Apoproteins ; Bronchopulmonary Sequestration* ; Cytoplasm ; Humans ; Lung Neoplasms ; Lung* ; Pneumocytes ; Thorax ; X-Ray Film

Fairly specific and important duodenoscopic findings for diagnosing mucinous ductal ectasia (MDE) of pancreas include a prominant ampulla, patulous ampullary orifice and extrusion of mucin through the ampulla. However, we have experienced two cases of mucinous ductal ectasia without these pathognomonic appearance of ampulla. Initially, two cases were admitted for evaluation of pancreatic multicystic lesions, and MDE was incidentally found. Their pancreatographic findings showed normal main duct and cystic dilatation confined to the branch ducts. On gross findings of surgical specimens, a duct dilatation with gelatinous mucin was localized at the uncinate process of the pancreas. Microscopically, the ductal epithelia containing mucin were lined with well-differentiated tall columnar cells showing focal hyperplastic change. We report these unusual cases of MDE with review of the literatures.
Dilatation ; Dilatation, Pathologic* ; Gelatin ; Mucins* ; Pancreas*

BACKGROUNDS AND STUDY AIMS: Multiple biliary papillomatosis (MBP) is an uncommon disease. This study was undertaken to review cases with respect to describing clinical features and characteristical cholangiographic findings, and to define the role of cholangioscopy in the diagnosis and treatment of this disorder. PATIENATS AND METHODS: from 1990 to 1997, nine patients (mean age; 57.3 years, 6 men and 3 women) were diagnosed as having MBP arnong 5,361 cases of endoscopic retrograde cholangiography (ERC) conducted at our center. A retrospective analyses of the cholangiographic and cholangioscopic findings as well as clinieal features were performed.
Cholangiography ; Diagnosis ; Humans ; Male ; Papilloma* ; Retrospective Studies