Primary oxalosis is a rare genetic disorder caused by a deficiency of the peroxisomal enzyme alanine-glyoxylate aminotransferase(type I) and D-glyceric dehydrogenase(type II). It is characterized by the triad of radiopaque urolithiasis, nephrocalcinosis, and latered renal function. We report a case of primary oxalosis in a 10-year-old Korean girl. She presented with chronic renal failure at 9 years of age. Ultrasonographic examination revealed bilateral, multiple renal and ureteral stones. Removed stones were chemically analysed to be composed of calcium oxalate. She underwent renal transplantation after prolonged period of dialysis. Removed kidneys were firm and gritty. Cut sections showed numerous tiny yellow sandy stones and a large staghorn stone. Specimen X-ray also exhibited disseminated fine granular and often coalescent radiopaque materials throughout the cortex and medulla. Microscopically numerous varying-sized crystals were noted in the kidney in globular or rhomboid appearance. The crystals were semitranslucent and doubly refractile under the polarized light. Diffuse interstitial fibrosis and chronic inflammation as well as glomerular obsolescence were also noted. The oxalate deposit was diffuse and marked, and was thought to be intimately related to the parenchymal damage and fibrosis.

OBJECTIVES: This study was performed to estimate the prevalence and possible risk factors of the metabolic syndrome in steel-mill workers, and to evaluate the relation between gamma-glutamyltransferase (gamma-GTP) and the metabolic syndrome. METHODS: The study subjects comprised 1,604 male steel-mill workers. The indices of metabolic syndrome, such as BMI, triglyceride, HDL-cholesterol, blood pressure, fasting glucose, and gamma-GTP were analyzed in each subject. We collected information about demographic characteristics, behavioral patterns, such as alcohol drinking, smoking, and exercise, and family medical history through a self-administered questionnaire. Statistical analysis was done by using the chi-square test, Mantel-Haenszel trend test and logistic regression model. RESULTS: The crude and age-adjusted prevalences of the metabolic syndrome were 21.3% and 15.5%, respectively, in the steel-mill workers. Age (OR: 1.063, 95% CI: 1.033-1.094), alcohol drinking (OR: 1.657, 95% CI: 1.175-2.337) and smoking (OR: 1.359, 95% CI: 1.017-1.816) were risk factors for the metabolic syndrome. Meanwhile, shift work showed a significant relation with hypertension (OR: 1.329, 95% CI: 1.038-1.700), but not with other metabolic syndrome components. However, tenure, exercise and family medical history were not significant factors for the metabolic syndrome in this study. The risk ratio of the metabolic syndrome (OR: 3.345, 95 % CI: 2.534-4.416) and its components, such as obesity, hypertriglyceridemia, hypertension and hyperglycemia, were higher in the high gamma-GTP group (> or =63 IU/ liter) than in the controls (<63 IU/liter). CONCLUSIONS: These results confirm the need for health education to control the metabolic syndrome by improving behavioral patterns, such as alcohol drinking and smoking, in factory workers. In addition, it is suggested that gamma-GTP might be a useful candidate in screening for the metabolic syndrome.
Alcohol Drinking ; Blood Pressure ; Fasting ; gamma-Glutamyltransferase ; Glucose ; Health Education ; Humans ; Hyperglycemia ; Hypertension ; Hypertriglyceridemia ; Logistic Models ; Male ; Mass Screening ; Obesity ; Odds Ratio ; Prevalence ; Questionnaires ; Risk Factors* ; Smoke ; Smoking ; Triglycerides

PURPOSE: This study was conducted to determine the factors which affect the resilience and quality of life for cancer patients with radiotheraphy. METHODS: Collecting data was conducted by self-administered questionnaire that 205 cancer patients with radiation therapy in one university hospital participated from 15th to 31st May 2015. The data analyzed by SPSS v18 and AMOS v18. RESULTS: This research found that the side-effect of treatments, social support, self esteem, depression, uncertainty and resilience are verified to be the variables having not only direct but indirect influence on the quality of life of patients receiving radiotheraphy. The side-effect of treatments was found to have direct influence on the quality of life of patients receiving was, and social support was found to have indirect influence on resilience through uncertainty and self esteem, and resilience was found to have direct influence on the quality of life. for cancer patients. CONCLUSION: It's necessary to come up with the approaches to develop the realistic practice guideline in order to raise the quality of life of patients receiving radiotheraphy, and to lower social support, self esteem, uncertainty in order to have higher resilience.
Depression ; Humans ; Quality of Life* ; Radiotherapy* ; Self Concept ; Uncertainty

Carcinoid tumors are the most common endocrine tumors of gastrointestinal tract. Argentaffin cells are the origin of carcinoids. These cells belong to the amino precursor uptake and decarboxylation (APUD) system. These cells give the tumor its most distinguishing feature: the ability to produce biogenic amines and polypeptide hormones that, in turn, give rise to the dramatic carcinoid syndrome. We treated a case of multi-centric carcinoid tumor of the duodenum in a 63-year-old male patient. He was admitted to the hospital because of epigastric pain. On the gastrofiberscopic examination, 1.0 x1.5 cm and 0.7 x0.8 cm sized two polyps (Yamada type II, I) were noticed on the duodenal bulb. The biopsy specimen showed carcinoid tumors of different histologic types. The level of 24-hour urine 5-HIAA of this case was normal. He was treated with subtotal gastrectomy with Billroth-II anastomosis. We report this case with literature review.
Biogenic Amines ; Biopsy ; Carcinoid Tumor* ; Decarboxylation ; Duodenum ; Enterochromaffin Cells ; Gastrectomy ; Gastrointestinal Tract ; Humans ; Hydroxyindoleacetic Acid ; Male ; Middle Aged ; Peptide Hormones ; Polyps

No abstract available.
Pregnancy* ; Stomach Neoplasms*

PURPOSE: The treatment of infected revision total hip arthroplasty (THA) is very challenging due to retained revision prosthesis, poor bone stock and soft tissue condition derived from previous revision surgeries, and comorbidities. The purpose of this study was to investigate the effectiveness and short-term outcomes of aggressive debridement and use of antibiotic-loaded cement beads with retention of the prosthesis for acute delayed or late infection of revision THAs. MATERIALS AND METHODS: Ten consecutive patients with symptoms or signs of less than one-week evolution and well-fixed prostheses, were treated with this procedure and a postoperative course of organism-specific antibiotics for a minimum of 6 weeks. All hips presented with acute delayed or late infection of revision THAs. Patients with a mean age of 68.1 years (range, 59-78 years) underwent an average of 1.9 previous revision THAs (1-4) before the index surgery. The minimal follow-up was 2 years with a mean of 46.2 months (range, 24-64 months). RESULTS: There were 8 cures (80.0%) and 2 failures with no mortality during the study period. The 2 failures involved the same and resistant bacteria implicated in the primary infection (methicillin-resistant Staphylococcus aureus and Prevotella oralis, respectively). The mean Harris hip score was 65.2 (range, 26-83) and the mean visual analogue scale was 2.6 (range, 1-4) at final follow-up. CONCLUSION: With a favorable success rate and no mortality, our procedure may be considered a safe and effective alternative for the treatment of acute delayed or late infection of revision THAs with well-fixed prostheses.
Anti-Bacterial Agents ; Arthroplasty, Replacement, Hip* ; Bacteria ; Comorbidity ; Debridement ; Follow-Up Studies ; Hip ; Humans ; Mortality ; Prevotella ; Prostheses and Implants* ; Staphylococcus aureus

Authors report a case of cystic tuberculosis of left olecranon in a 44 year-old woman, which mimicked the bone tumor at initial presentation. She complained mild discomfort in the left elbow over a month. On examination there were no local redness, swelling, tenderness and deformity. There was no limitation of left elbow motion. After open curettage of the lesion, bone tuberculosis was confirmed by histological study. Postoperative triple chemotherapy of 12 months (Rifamcpicin, ethambutal, INH) could cure the disease.
Congenital Abnormalities ; Curettage ; Elbow ; Female ; Humans ; Olecranon Process ; Tuberculosis ; Tuberculosis, Osteoarticular

In order to obtain novel genes for craniofacial development of human, molecular cloning and sequencing were performed and followed by in situ hybridization in tissue sections. Subtracted cDNA library of craniofacial tissue from 8 weeks old human embryo was made by the subtraction with cDNA of RHEK cells. A total of 231 clones were obtained and their partial sequence data disclosed that 214 clones were nonredundant in Genebank search. We have done in situ hybridization screening on the craniofacial sections of a 10 weeks old human fetus, and found significant positive reaction in 30 clones. Depending on the cell type of similar developmental origin, the positive reactions could be divided into four groups: first group showed an intense positive reaction in neural tube, ganglion, and a part of peripheral nerve tissue, second group relatively diffuse positive reaction in neural tube, cartilage, epithelium, and muscle, third group localized positive reaction in nerve, and muscle, and fourth group positive reaction in almost all kinds of cells of craniofacial tissues. Although every clone showed different expression patterns in the craniofacial development, some of them showed intense mRNA expressions in the characteristic cell type. Because this study also aimed to test a screening methods to find out novel genes related to craniofacial development by the subtracted cDNA library and in situ hybridization, the intense positive reaction of a certain clone by in situ hybridization may indicate its role in the developmental processes. We presumed that 30 clones selected in this study are possibly important new genes for the development of human craniofacial structure.
Cartilage ; Clone Cells ; Cloning, Molecular* ; DNA, Complementary ; Embryonic Structures* ; Epithelium ; Fetus ; Ganglion Cysts ; Gene Library ; Humans* ; In Situ Hybridization ; Mass Screening ; Neural Tube ; Peripheral Nerves ; RNA, Messenger

Callosal disconnection results in the functional independence of each hemisphere and usually produces characteristic signs including alien hand, left-sided apraxia, left agraphia and left tactile anomia. Our two patients; a 75-year-old right-handed woman with hypertension and a 71-year-old right-handed woman with diabetes mellitus, showed impairments in the identification of body parts with their left hands in addition to characteristic symptoms such as left ideomotor apraxia, agraphia, tactile anomia, and right alien hand signs with groping and grasping. Brain MRIs of these patients upon admission demonstrated infarcts in the medial portion of the left frontal lobes and in the corpus callosum supplied by the left anterior cerebral artery. The impairment of body parts cognition in our patients can be attributed to the failure of the patient's left hand to communicate with the body schema stored in the left hemisphere.
Aged ; Agraphia ; Anomia ; Anterior Cerebral Artery ; Apraxia, Ideomotor ; Apraxias ; Body Image ; Brain ; Cognition* ; Corpus Callosum ; Diabetes Mellitus ; Emigrants and Immigrants ; Extremities* ; Female ; Frontal Lobe ; Hand ; Hand Strength ; Human Body ; Humans ; Hypertension ; Magnetic Resonance Imaging

Benign endobronchial tumors are rare diseases with an incidenced of between 1 and 5% of all lung tumors. An endobronchial fibroepithelial polyp is an extremely rare form of benign bronchial tumor. Clinically, an endobronchial fibroepithelial polyp causes an airway obstruction and obstructive pneumonitis as does other endobronchial tumors. Therefore, it is important to differentiate an endobronchial fibroepithelial polyp pathologically from other benign endobronchial tumors and bronchogenic carcinomas. Here, we report a case of an endobronchial fibroepithelial polyp, in a 25-year-old man who had suffered from chest discomfort upon deep breathing with a brief review of the relevant literature.
Adult ; Airway Obstruction ; Carcinoma, Bronchogenic ; Humans ; Lung ; Pneumonia ; Polyps* ; Rare Diseases ; Respiration ; Thorax