PURPOSE: The effects of gonadotropin-releasing hormone agonist (GnRHa) treatment on body mass index (BMI) are controversial in girls with central precocious puberty (CPP). We therefore evaluated auxological parameters during GnRHa therapy in patients with CPP, specifically focusing on changes in BMI. METHODS: Seventy-seven girls with idiopathic CPP who underwent GnRHa therapy were retrospectively recruited. We investigated BMI changes during the treatment period after stratifying them according to baseline BMI status as follows: normal (BMI percentile of <85th) and overweight groups (BMI percentile of ≥85th). RESULTS: The incidence of overweight/obesity (40.3%/23.4%) was very high in the girls with CPP. In the overall study population, no significant BMI change was observed during the GnRHa treatment period. However, when stratified according to baseline BMI status, the normal-weight group showed a significant increase in BMI-standard deviation score (SDS), whereas the overweight group showed no change in BMI-SDS. Baseline BMI-SDS was an independent predictor of changes in BMI during the GnRHa treatment period. Changes in weight-SDS were similar, but changes in height-SDS were significantly greater in the overweight group than in the normal-weight group, which explains the observed difference in BMI-SDS. CONCLUSION: Our results demonstrate that the difference in the pattern of BMI changes among our CPP patients suggests that delayed puberty induced by GnRHa treatment may have different effects on linear growth according to baseline body composition. This study underscores the importance of individualized lifestyle intervention in CPP children.
Body Composition ; Body Mass Index* ; Child ; Female* ; Gonadotropin-Releasing Hormone* ; Humans ; Incidence ; Life Style ; Obesity ; Overweight* ; Puberty, Delayed ; Puberty, Precocious* ; Retrospective Studies

Nephronophthisis-related ciliopathy (NPHP-RC) is a common genetic cause of end-stage renal failure during childhood and adolescence and exhibits an autosomal recessive pattern of inheritance. Genetic diagnosis is quite limited owing to genetic heterogeneity in NPHP-RC. We designed a novel approach involving the step-wise screening of Sanger sequencing and targeted exome sequencing for the genetic diagnosis of 55 patients with NPHP-RC. First, five NPHP-RC genes were analyzed by Sanger sequencing in phenotypically classified patients. Known pathogenic mutations were identified in 12 patients (21.8%); homozygous deletions of NPHP1 in 4 juvenile nephronophthisis patients, IQCB1/NPHP5 mutations in 3 Senior–Løken syndrome patients, a CEP290/NPHP6 mutation in 1 Joubert syndrome patient, and TMEM67/MKS3 mutations in 4 Joubert syndrome patients with liver involvement. In the remaining undiagnosed patients, we applied targeted exome sequencing of 34 ciliopathy-related genes to detect known pathogenic mutations in 7 (16.3%) of 43 patients. Another 18 likely damaging heterozygous variants were identified in 13 NPHP-RC genes in 18 patients. In this study, we report a variety of pathogenic and candidate mutations identified in 55 patients with NPHP-RC in Korea using a step-wise application of two genetic tests. These results support the clinical utility of targeted exome sequencing to resolve the issue of allelic and genetic heterogeneity in NPHP-RC.
Adolescent ; Diagnosis* ; Exome* ; Genetic Heterogeneity* ; Humans ; Kidney Failure, Chronic ; Korea ; Liver ; Mass Screening ; Wills

PURPOSE: Hypertensive disorders of pregnancy increase morbidity and mortality of fetus and neonates. Recently some studies revealed that antihypertensive agents affected the neonatal outcomes. The aim of this study was to investigate the prognosis of preterm infants delivered from the mothers with hypertensive disorders who were treated with antihypertensive agents and magnesium sulfate. METHODS: This retrospective study was conducted on preterm infants who were delivered from normotensive mother (control, n=436) and antihypertensive drugs +/- magnesium sulfate treated mother (study, n=150) between January 2009 and December 2013. Study group were divided into two groups based on whether they received antihypertensive drugs only (n=110) and additional magnesium sulfate (n=40). We compared the characteristics of mothers and neonatal outcomes. RESULTS: Study group had shorter gestational age (32.2+/-3.5 weeks vs. 33.7+/-3.0 weeks, P=0.000) and lower birth weight (1,810.5 +/- 689.2 g, 2,212.1 +/- 604.9 g, P=0.000), and higher rate of small for gestational age infants (22% vs 11%, P=0.000). One minute and 5 minutes Apgar score were lower, but duration of hospital days, oxygen supplement and mechanical ventilation were longer in study group. Respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus, retinopathy of prematurity, intraventricular hemorrhage occurred more in study group than control. The group treated with magnesium sulfate together with antihypertensive agent had lower 1 minute and 5 minutes Apgar score than the group taken antihypertensive agent only. CONCLUSIONS: Mothers with hypertensive disorders have increased the risk of preterm delivery, low birth weight, and high neonatal morbidity rate. Therefore it is important to predict and manage possible complication. Moreover, if magnesium sulfate is taken, careful neonatal monitoring is needed because of possible low Apgar score.
Antihypertensive Agents* ; Apgar Score ; Birth Weight ; Bronchopulmonary Dysplasia ; Ductus Arteriosus, Patent ; Fetus ; Gestational Age ; Hemorrhage ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature* ; Magnesium Sulfate ; Mortality ; Mothers* ; Oxygen ; Pregnancy* ; Prognosis ; Respiration, Artificial ; Retinopathy of Prematurity ; Retrospective Studies

BACKGROUND/AIMS: To investigate the rate of detection of monosodium urate (MSU) crystals in the synovial fluid (SF) of patients with acute gouty arthritis and factors associated with false-negative results. METHODS: A total of 179 patients with acute gouty arthritis who had undergone SF crystal examination were identified from the data warehouse of two university hospitals. Clinical and laboratory data were obtained from the medical records. RESULTS: The overall rate of detection of MSU crystals was 78.8%. In univariate analyses, the only significant differences between the variables of crystal-negative and crystal-positive patients were a lower C-reactive protein level (p = 0.040) and fewer patients undergoing emergent surgery in the crystal-positive group (p = 4.5 x 10(-6)). In logistic regression analyses, MSU crystal-negative results were significantly associated with the interval from arthritis onset to crystal examination (p = 0.042), and this was the most significant risk factor for arthroscopic surgery (p = 2.1 x 10(-4)). Seventeen patients who underwent arthroscopic surgery had a significantly longer hospital stay (p = 0.007) and a significant delay in gout treatment (p = 8.74 x 10(-5)). The distribution of crystal-negative patients differed significantly between the SF samples that were evaluated by both the laboratory medicine and the rheumatology departments (p = 1.2 x 10(-14)), and the kappa value was 0.108. CONCLUSIONS: Although several clinical features were associated with detection failure, SF MSU crystal identification was critically dependent on the observer. Considering the impact on the treatment outcomes, implementation of a quality control program is essential.
Acute Disease ; Aged ; Arthritis, Gouty/diagnosis/*metabolism/*surgery ; Arthroscopy ; Biological Markers/metabolism ; Crystallization ; False Negative Reactions ; Female ; Hospitals, University ; Humans ; Length of Stay ; Logistic Models ; Male ; Microscopy, Polarization ; Middle Aged ; Observer Variation ; Predictive Value of Tests ; Reproducibility of Results ; Republic of Korea ; Retrospective Studies ; Synovial Fluid/*metabolism ; Time Factors ; Time-to-Treatment ; Treatment Outcome ; Uric Acid/*metabolism

PURPOSE: Topical calcineurin inhibitor is recently developed topical immunomodulator, and preliminary studies showed its effectiveness in the treatment of atopic dermatitis (AD). However, some side effects including transient irritation can influence the patient compliance. So, there are some needs to improve the patient compliance. The purpose of this study was to evaluate the efficacy, safety and patient compliance with using topical tacrolimus 0.1% to treat AD when the correct information about topical tacrolimus are properly given to patients. METHODS: We examined the medical recordings, clinical severity scoring of total 194 AD patients at 9 general hospitals in Seoul, Korea from September 2010 to August 2011. We offered an infosheet of topical tacrolimus 0.1% and the patients applied it twice a day for 2 weeks. And we measured the efficacy of the topical tacrolimus 0.1% with SCORing atopic dermatitis (SCORAD) index, patient's global assessment (PGA), and investigator's global assessment (IGA). RESULTS: Topical tacrolimus 0.1% effectively controlled AD with a reduction of the SCORAD index from baseline 31.9 to 20.2 at 2 weeks of application. In IGA results showed 98% got improvement and in PGA, results showed 96% got improvement after treatment. Although 42.3% of the patients complained of adverse effects, these were all transient. The effect of information on topical tacrolimus 0.1% showed 34% patients could predict the side effect, 35% patients could feel safety to use, and 18% patients experienced side effect but could maintain topical calcineurin inhibitor. CONCLUSION: Topical tacrolimus 0.1% may be an effective treatment modality for AD when patients show good compliance for applying the ointment. And properly given, the correct information may increase the patient compliance.
Calcineurin ; Compliance ; Dermatitis, Atopic ; Hospitals, General ; Humans ; Immunoglobulin A ; Korea ; Medical Records ; Patient Compliance ; Prostaglandins A ; Tacrolimus

BACKGROUND: Simian virus 40 (SV40), a polyomavirus, was discovered as a contaminant of a human polio vaccine in the 1960s. It is known that malignant mesothelioma (MM) is associated with SV40, and that the virus works as a cofactor to the carcinogenetic effects of asbestos. However, the reports about the correlation between SV40 and MM have not been consistent. The purpose of this study is to identify SV40 in MM tissue in Korea through detection of SV40 protein and DNA. METHODS: We analyzed 62 cases of available paraffin-blocks enrolled through the Korean Malignant Mesothelioma Surveillance System and performed immunohistochemistry for SV40 protein and real-time polymerase chain reaction (PCR) for SV40 DNA. RESULTS: Of 62 total cases, 40 had disease involving the pleura (64.5%), and 29 (46.8%) were found to be of the epithelioid subtype. Immunostaining demonstrated that all examined tissues were negative for SV40 protein. Sufficient DNA was extracted for real-time PCR analysis from 36 cases. Quantitative PCR of these samples showed no increase in SV40 transcript compared to the negative controls. CONCLUSIONS: SV40 is not associated with the development of MM in Korea.
Asbestos ; DNA ; Humans ; Immunohistochemistry ; Korea ; Mesothelioma ; Pleura ; Poliomyelitis ; Polymerase Chain Reaction ; Polyomavirus ; Real-Time Polymerase Chain Reaction ; Simian virus 40 ; Viruses

Kimura's disease is a rare inflammatory disorder of unknown etiology primarily seen in young Asian males. It is characterized by painless subcutaneous masses, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E (IgE) levels. Clinically, the subcutaneous nodules occur predominantly in the head and neck. However, we report the case of a five-year-old boy presenting with an asymptomatic solitary brown pigmented nodule on the left buttock diagnosed with Kimura's disease. Here we describe the diagnosis and treatment of this unusual case.
Asian Continental Ancestry Group ; Buttocks ; Child, Preschool ; Eosinophilia ; Head ; Humans ; Immunoglobulin E ; Immunoglobulins ; Male ; Neck

Thymidine phosphorylase (TP) has shown to be up-regulated in several cancers and to play a role in angiogenesis and invasion. Most studies regarding TP have focused on cancer cells. Recently, evidences suggest that TP in cancer-infiltrating inflammatory cells (CIICs) also affect the cancer cell behavior. To evaluate the significance of TP expression of CIICs in gastric cancer, we assessed TP expression of cancer cells and CIICs separately using immunohistochemical assay on 116 paraffin-embedded tissue samples from stomach cancer patients and investigated their clinical significance. When subjects were divided into 4 groups according to the TP expression: cancer/matrix (+/+), C/M (+/-), C/M (-/+), and C/M (-/-), intratumoral microvessel density scores were higher in the C/M (+/-) group than in the C/M (-/-) group (p=0.02). For lymph node metastasis and survival, there were no significant differences among the 4 groups. However, there were significant differences in survival (p=0.035) and LN metastasis (p=0.023) between the two groups divided by TP expression of CIICs alone irrespective of TP expression of cancer cells. Taken together, this study suggested the TP expression in CIICs could affect lymph node metastasis and patients' survival in gastric cancer.
Adult ; Aged ; Female ; Humans ; Immunohistochemistry ; Inflammation/*enzymology/pathology ; Kaplan-Meiers Estimate ; Lymphatic Metastasis ; Lymphocytes, Tumor-Infiltrating/*enzymology/pathology ; Male ; Microcirculation/pathology ; Middle Aged ; Neovascularization, Pathologic ; Prognosis ; Stomach Neoplasms/blood supply/*enzymology/mortality/pathology ; Thymidine Phosphorylase/*metabolism

BACKGROUND AND OBJECTIVES: Percutaneous mitral balloon valvuloplotomy (PMV) is a safe and effective procedure for the treatment of mitral stenosis (MS); however, its long-term results according to the severity of MS remain unknown. The aim of this study was to compare the long-term results of PMV between moderate and severe MS. SUBJECTS AND METHODS: The immediate and long-term outcomes of 786 patients (198 male, mean age; 43 years) who underwent PMV at a single referral center, between 1988 and 2000, were analyzed. The clinical and echocardiographic data were compared between moderate (n=357) and severe MS (n=429). RESULTS: Optimal results were gained in 618 (79%) patients, including 80 and 76% moderate and severe MS cases, respectively (p=0.13). Acute complications occurred in 85 patients (11%), but without significant difference between the two groups. After a mean follow-up period of 94 months (range, 6 to 210 month), the 5 year event-free survival rate was better in moderate (88%) than in severe MS (79%), but the 10 year event-free survival rates were no different (65% vs. 65%). Independent predictors for better prognosis were a lower echo score (< or =8)(p=0.017, HR=0.67), a larger mitral valve area after the PMV (> or =1.7 cm2)(p=0.002, HR=0.65), less mitral regurgitation ( Atrial Pressure ; Balloon Valvuloplasty ; Disease-Free Survival ; Echocardiography ; Follow-Up Studies ; Humans ; Male ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Stenosis* ; Prognosis ; Referral and Consultation

This study was to investigate the effect of the education about general nutrition knowledge on the acceptance of TV food advertisements in adolescents. Twenty hours of nutrition related education in home economics classes were done during 3 months, to conveniently selected 784 first grade middle school students living in large and small cities and rural areas of Chungnam Province. The education effects were evaluated through questionnaire surveys before and after the education and their comparative analysis. Average TV watching time was 2.92 +/- 1.66 hours per day, which was about one hour longer on weekends. Nearly half of the subjects watched TV mainly after 9 PM. After nutrition education, nutrition knowledge scores of total subjects were significantly increased from 7.73 +/- 2.16 to 10.25 +/- 2.51 with a full score of 16. Food attitude and food habit scores also significantly increased from 32.45 +/- 4.65 (full score 50) to 33.93 +/- 4.68 and from 36.20 +/- 5.70 to 37.29 +/- 5.87, respectively. The general acceptance of TV food advertisements scored 25.25 +/- 4.44 (full score 40) before education and 26.90 +/- 4.55 after education, which was significantly raised by the education. Acceptance scores of TV food advertisements showed significant positive relationships with the scores of nutrition knowledge, food attitudes, and food habits. In addition, TV watching time had negative relationships with nutrition knowledge, food attitudes, and food habits scores, but not with acceptance score of TV food advertisements. The above results suggested that school-based nutrition education improved slightly but significantly the attitude of accepting TV food advertisements maybe through increasing nutrition knowledge and making food attitudes and habits better. Nutrition education focused on the evaluation and acceptance of food advertisements are further needed to improve the TV food advertisement acceptance of adolescents.
Adolescent ; Chungcheongnam-do ; Education* ; Food Habits ; Humans ; Surveys and Questionnaires