Congenital adrenal hyperplasia(CAH) results from an inherited defect in enzymatic steps required to synthesize cortisol from cholesterol. 21-hydroxylase deficiency accounts for 95% cases of CAH. We have analyzed CYP21 genes of CAH by PCR direct sequencing. Our results shows three cases of CAH owing to multiple mutations of CYP21 gene; first case, IVS2AS, A/G, -13, Ile172Asn; second case, IVS2AS, A/G, -13, Ile236Asn, Val237Glu, Met239Lys; third case, Ile172Asn, C to G at 1590nt, Val281Leu, Arg484Pro, G to A at 2697nt. Mutations such as Ile236Asn, Val237Glu, Met239Lys, and Arg484Pro are first noted in Korea.
Adrenal Hyperplasia, Congenital* ; Cholesterol ; Hydrocortisone ; Korea ; Polymerase Chain Reaction ; Steroid 21-Hydroxylase

Background: Alopecia areata (AA) is a T cell-mediated autoimmune disease. In patients with chronic AA, hair loss occurs because of the insidious destruction of the stem cells in the hair bulge and hair-bulb matrix along with the attack of the cytotoxic T cells and infiltration of the T-helper 17 cells. Cyclosporine (CsA) inhibits the action of calcineurin in hair stem cells and subsequently induces the inhibition of the nuclear factor of activated T cells c1 and expression of cyclin-dependent kinase 4, thereby stimulating hair regrowth.
Objective
The purpose of this study was to evaluate the long-term therapeutic outcomes of low-dose systemic CsA treatment in patients with chronic AA, relative to their initial severity of AA. Methods: A total of 98 outpatients with chronic AA, who were treated with low-dose systemic CsA, were included in the study. Results: Among the 98 patients, 72 (73.5%) had more than 50% hair regrowth, while 30 patients (30.6%) had more than 90% hair regrowth after 18 months of CsA treatment. Patients with a lower initial severity of alopecia tool (SALT) score demonstrated better treatment outcomes than those with a higher initial SALT score. Conclusion Patients with chronic AA who were treated with low-dose, systemic CsA experienced significant hair regrowth with fewer side effects. Therefore, the use of low-dose, systemic CsA is recommended in the treatment of patients with chronic AA.

We report a case of severe(gradeV) unilateral vesicoureteral reflux(VUR) without any renal damage in a 6-month-old boy through the early sibling screening test for VUR, whose old brother had reflux nephropathy. The early detection of VUR aroused us to take special precautions to prevent urinary tract infection in this patient. We believe that this approach was helpful to reduce the risk of renal damage in this patient and are reporting the case with a brief review of related literatures. It is our firm suggestion that screening for vesicoureteral reflux should be carried out in every child who has siblings with reflux nephropathy.
Child ; Humans ; Infant ; Male ; Mass Screening* ; Siblings* ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

No abstract available.
Soccer*

Central neurocytoma is a rare, well-differentiated neuronal tumor and is usually located in the lateral or third ventricle of young adults. Its overall prognosis is excellent with a low proliferative index. The majority of previously reported malignant variants rarely did recurred after tumor removal and regarded as benign tumor despite of histopathological malignant feature. Nevertheless, we experienced a case of malignant variant of the central neurocytoma with high proliferative index(Ki-67 labeling index >30%), which showed recurrence immediately after surgery and died within 3 months POD. Here, we describe the case with a review of the literatures.
Humans ; Neurocytoma* ; Neurons ; Prognosis ; Recurrence ; Third Ventricle ; Young Adult

BACKGROUND: Acute renal failure(ARF) is characterized by rapid decline in glomerular filtration rate and retension of nitrogenous waste products. This syndrome occurs in approximately 5 percent all hospital admissions and up to 30 percent of admissions to intensive care units. ARF is diagnosed when screening of hospital patients reveals a recent increase in serum BUN(blood urea nitrogen) and creatinine. The mortality rate for ARF is approximate 40-60% and has changed little in past three decades. This lack of improvement in outcome, despite significant advances in medicine. The reasons of high mortality rate is not certain. This study intend to identify prognostic risk factors influencing survivals. METHODS: We retrospectively analyzed 60 patients with ARF during 2 years period from Oct. 1996 to Oct. 1998 at chosun university hospital. Multiple factors which may influence mortality were evaluated. RESULTS: 1)Of the 60 patients, 34 were male and 26 were female. The mean age was 55.8+/-15.9 years. 2)The cause of ARF is Drug, toxin, dehydration, infection, trauma, surgery, urinary tract obstruction, HFRS, rhabdomyolysis and bleeding. 3)Underline disease is observed in 52 case. DM, Hypertension, Malignancy, Pulmonary disease, Liver disease, Renal disease 4)The mortality rate is 31.7%. The major cause of deaths is DIC, infection and hepatic failure. 5)Based on the unpaired t-test, chi-squre analysis, albumin, total bilirubin, hemoglobin, thrombocytopenia, APACHE II score, serum sodium, urine creatinine, number of multiple organ failure, cause of acute renal failure, pH, vital sign on admission, sepsis, DIC, oilguria, and hemodialysis were significant factors between survivors and nonsurvivors(p< 0.05). 6)APACHE II score on admission is good prognostic factor for patients with acute renal failure (p<0.001). CONCLUSION: This results suggest that the evaluation of patients symptom, sign, laboratory data, APACHE II score is important for patients with acute renal failure. the discriminant score by multiple analysis and APACHE II score could relatively predict the mortality of ARF patients. however further evaluation and clinical apply of prognostic factors is required to confirm these results.
Acute Kidney Injury* ; APACHE ; Bilirubin ; Cause of Death ; Creatinine ; Dacarbazine ; Dehydration ; Female ; Glomerular Filtration Rate ; Hemorrhage ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Hydrogen-Ion Concentration ; Hypertension ; Intensive Care Units ; Liver Diseases ; Liver Failure ; Lung Diseases ; Male ; Mass Screening ; Mortality ; Multiple Organ Failure ; Nitrogen ; Renal Dialysis ; Retrospective Studies ; Rhabdomyolysis ; Risk Factors* ; Sepsis ; Sodium ; Survivors ; Thrombocytopenia ; Urea ; Urinary Tract ; Vital Signs ; Waste Products

OBJECTIVES: To evaluate sleep characteristics and factors associated with sleep disturbance in schizophrenia patients with concurrent active psychotic symptoms and insomnia. METHODS: Schizophrenia patients with insomnia and active psychotic symptoms (n = 63) were recruited from communitybased mental rehabilitative facilities. Sleep scales such as the Korean version of the Insomnia Severity Index (ISI-K) and the Korean Version of the Pittsburgh Sleep Quality Index (PSQI-K) were evaluated and those with ISI-K >15 were included in the study. Psychotic, anxiety and depressive symptoms were rated with the Brief Psychotic Rating Scale (BPRS), the Korean Version of the Anxiety Sensitivity Index (K-ASI), and the Korean Version of the Beck Depression Inventory-I (K-BDI), respectively. Pearson correlation analyses were performed between the sociodemographic data, ISI-K and PSQI-K. Multiple linear regression analysis was conducted to investigate the factors which affected the ISI-K and PSQI-K. RESULTS: The mean ISI-K and PSQI-K scores were 18.1 ± 2.6 and 12.0 ± 2.2, respectively. Pearson correlation analysis showed a negative correlation between age of onset and ISI-K score and positive correlations between BRPS and PSQI-K scores and between K-ASI and both ISI-K and PSQI-K scores. Multiple regression analyses for both ISI-K and PSQI-K with K-ASI, age of onset, and BPRS as covariates revealed K-ASI as the only significant remaining factor. CONCLUSION: Our study suggests that anxiety symptoms are associated with insomnia symptoms in schizophrenia patients regardless of depressive or psychotic symptoms.
Age of Onset ; Anxiety ; Community Mental Health Services ; Depression ; Humans ; Linear Models ; Mental Health Services* ; Mental Health* ; Schizophrenia* ; Sleep Initiation and Maintenance Disorders* ; Weights and Measures

No Abstract Available.
Endothelium, Corneal* ; Lidocaine*

Alopecia areata is a chronic organ-specific autoimmune disease and it could be associated with other autoimmune diseases. We, herein, report a case of alopecia areata in a patient with a thymoma without myasthenia gravis. Multiple hairless patches rapidly developed 6 weeks before the first visit on the patient who had been newly diagnosed with thymoma 2 weeks before the hairless patches occurred, and thymectomy was done 2 weeks before visiting dermatologic department. She had no symptoms associated with myasthenia gravis, and there were no abnormal findings on neurologic exams and acetylcholine receptor autoantibody was not detected in serum. Scalp biopsy showed numerous lymphocytic inflammations around hair follicles and in immunohistochemical staining, the aggregation of CD4+ and CD8+ T cells was observed around hair follicles and FoxP3+ T lymphocytes were rarely observed around hair follicles. The patient refused any treatment and her hairless patches were completely recovered 3 months after thymectomy, without being recurred 3 years after thymectomy. On the basis of both clinical manifestations and histologic findings, we concluded that alopecia areata in the patient had developed in association with thymoma and was recovered rapidly after thymectomy.

PURPOSE: The purpose of this sudy was to compare the fracture resistance of the IPS Empress ceramic crown with 1.0mm width rounded shoulder, which is usually recommended in all ceramic crown, and 0.5mm width chamfer finish lines on the maxillary central incisor. MATERIAL AND METHOD: After 15 metal dies were made for each group, the IPS Empress all ceramic crowns were fabricated and cemented with resin cement(Bistite resin cement, Tokuyama Soda Co. LTD., Japan) on the metal die. The cemented crowns were mounted on the positioning jig and the universal testing machine(Zwick Z020, Zwick Co, Germany)was used to measure the fracture strength, with loading on the incisal edge. And also, three-dimensional finite element stress analysis was used to measure the stress distribution with the various types of the finish lines(1.0mm width rounded shoulder, 0.5mm width chamfer), the loading site(incisal edge, incisal 1/3), and the type of loading(concentration loading, distribution loading). RESULTS AND CONCLUSION: 1. In the fracture resistance experiment according to the finish line, the mean fracture strength of rounded shoulder(876N) and the mean fracture strength of chamfer(882N) did not show any significant difference between each other(p<0.05). 2. The stress distribution of all ceramic crown in three dimensional finite element analysis showed concentration aspect at loading point and cervical area of labial surface. 3. In metal die, there were no differences in stress distribution between finish lines, but in natural teeth model, chamfer finish line showed higher stress than rounded shoulder finish line. 4. When force was loaded on the incisal edge, the stress was concentrated on the incisal edge and the cervical area of labial surface. When force was loaded on the incisal 1/3, the stress concentrated on the cervical area of labial surface and the cingulum area. 5. Generally, natural teeth model showed higher and various stress than the metal die.