A nineteen year-old boy with progressive enlargement of the joints and distal extremities, clubbing, coarse facial features and hyperhidrosis was investigated. In physical examination, thickening of the scalp with furrowing (cutis verticis gyrata) and greasy thickening of skin (pachyderma) was prominent. His endocrine profile was normal. Radiological studies demonstrated bilateral symmetrical periosteal new bone formation with acroosteolysis and incidental microadenoma of pituitay gland. After extensive investigation to exclude systemic and endocrine causes, the patient was diagnosed as pachydermoperiostosis (PDP). PDP is a rare syndrome manifested clinically by finger clubbing, extremity enlargement, hypertrophic skin changes, and periosteal bone formation. The pathogenesis of the disorder has not been clarified though few endocrine abnormalities were seen. To aware of these clinical phenotype would help to differentiate PDP from acromegaly.
Acro-Osteolysis ; Acromegaly ; Extremities ; Fingers ; Humans ; Hyperhidrosis ; Joints ; Male* ; Osteoarthropathy, Primary Hypertrophic* ; Osteogenesis ; Phenotype ; Physical Examination ; Scalp ; Skin ; Young Adult*

We aimed to compare the immune response induced by natural infection with 2009 pandemic influenza A/H1N1 (pH1N1) virus and by monovalent pH1N1 vaccination in children and adolescents. This cross-sectional clinical study was conducted at 3 hospitals in Korea from February to May 2010. A total of 266 healthy subjects aged from 6 months to 18 yr were tested for the presence of the antibody against pH1N1 using hemagglutination inhibition (HI) test. Information about pH1N1 vaccination and laboratory-confirmed pH1N1 infection history was obtained. The overall rate of HI titers of > or = 1:40 against pH1N1 was 38.7%, and the geometric mean titer (GMT) was 20.5. Immunogenicity of pH1N1 vaccination only was reflected by a 41.1% of seroprotection rate and a GMT of 22.5. Immunogenicity of natural infection only was reflected by a 61.0% of seroprotection rate and a GMT of 40.0. GMT was significantly higher in the subjects of natural infection group than in the subjects of pH1N1 vaccination group (P < 0.001). The immune responses induced by natural pH1N1 infection exceed those induced by pH1N1 vaccinations.
Adolescent ; Antibodies, Neutralizing/blood ; Antibody Formation ; Child ; Child, Preschool ; Cross-Sectional Studies ; Hemagglutination Inhibition Tests ; Humans ; Infant ; Influenza A Virus, H1N1 Subtype/*immunology/metabolism ; Influenza, Human/epidemiology/*immunology/prevention & control ; Pandemics ; Vaccination

OBJECT: Object of this study is to study which factors affect the prognosis in spontaneous thalamic hemorrhage treated with conservative method, stereotactic surgery and extraventricular drainage. MATERIAL AND METHODS: We analyzed 127 patients with spontaneous thalamic hemorrhage for evaluating the factors affecting prognosis. Various factors such as age, sex, location of hematoma, Glasgow coma scale(GCS) on admission, intraventricular hemorrhage(IVH), ventricular dilatation, CT classification, hematoma diameter, 4th ventricle dilatation, and treatment modality. RESULTS: The results were as follows: 1) Of the 40 patients with GCS of 3 to 8, 15 patients(37.5%) died. Of the 34 patients with GCS of 13-15, 33 patients(97%) survived 2) The 99 patients had intraventricular hemorrhage and 25 patients(25.2%) died. The 28 patients had no intraventricular hemorrhage and they all survived. 3) Of the 24 patients in whom the cerebrocaudate index(CCI) was more than 0.25, 10 patients(41.6%) died and only 2 patients had good prognosis. The 35 patients had no ventricular dilatation and all 35 patients survived 4) Of the 23 patients extending to hypothalamus or midbrain with ventricular hemorrhage, 11 patients(47.8%) died and 12 patients(52.1%) survived. 5) Of the 10 patients in whom hematoma diameter was more than 45mm, 4 patients(40%) died and 6 patients(60%) had a poor prognosis. 6) The 31 patients had hemorrhagic dilatation of the fourth ventricle and 16 patients(51.6%) died. Of the 29 patients with ventricular hemorrhage and no dilatation, 6 patients(20.6%) died. CONCLUSION: Multiple logistic regression analysis showed prognostic factors: GCS score(p<1/20.0001), extending to hypotahalamus or midbrain with ventricular hemorrhage(p<1/20.0001), thalamic hematoma with IVH(p<1/20.0001), dilated 4th ventricle hemorrhage(p<1/20.0012), hematoma diameter(p<1/20.0001), CCI(p<1/20.0001).
Classification ; Coma ; Dilatation ; Drainage ; Fourth Ventricle ; Hematoma ; Hemorrhage* ; Humans ; Hypothalamus ; Logistic Models ; Mesencephalon ; Prognosis

PURPOSE: The purpose of this study was to construct reference data for birth length of full-term and preterm Korean infants by sex and to define a sex-specific birth length cut-off to identify small for gestational age (SGA).METHODS: Data were collected from the 4th Korean National Health and Nutrition Examination Survey (2007–2009), comprising 843 children with birth length data and birth history.RESULTS: References for the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles of birth length and weight were created using singleton neonates of gestational age (GA) 36–37 weeks and 38–41 weeks by sex. The birth length cutoff value for SGA (<10th percentile) was 48 cm in both male and female neonates, and the ≤3rd percentile cutoff was 47 cm in males and 46 cm in females born at a GA of 38–41 weeks.CONCLUSION: New Korean reference data were created for birth length and differed from those of other ethnicities. Further research on short-term and long-term health outcomes of SGA infants based on the new reference data is needed.
Asian Continental Ancestry Group ; Child ; Female ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Male ; Nutrition Surveys ; Parturition ; Reproductive History

PURPOSE: The objective of this study is to estimate the trend in age at menarche in the Korean female and evaluate the relationship between age at menarche and adult body mass index (BMI), which is a indicator of later-life health. METHODS: We conducted a cross-sectional analysis of a nationally representative sample (self-reported age at menarche and measured height and weight) of 11,065 females aged 15 and older. Data were obtained from the Fourth Korea National Health and Nutrition Examination Survey (KNHANES IV, 2007-2009). RESULTS: We found a statistically significant decline in age at menarche in successive birth groups, indicating a 0.726 year decrease per decade. The age at menarche in 1990-1994 year birth group was 12.60 years, which showed a significant decreased from 3.11 years in the 1980-1984 birth group. We also found a significant negative association between age at menarche and current BMI. A one-year decrease in age at menarche was associated with mean BMI increase of 0.109 kg/m2 (95% confidence interval [CI], 0.069 to 0.150) after adjustment for age. In multivariate logistic regression, the odds ratios of obesity in females with early menarche (<12 years) was 1.845 fold (95% CI, 1.441 to 2.361). CONCLUSION: We found that age at menarche is still falling in the Korean female. We also found that early menarche is a risk factor for obesity in adults.
Adult ; Aged ; Body Mass Index ; Cross-Sectional Studies ; Female ; Humans ; Korea ; Logistic Models ; Menarche ; Nutrition Surveys ; Obesity ; Odds Ratio ; Parturition ; Risk Factors

PURPOSE: The objective of this study is to estimate the trend in age at menarche in the Korean female and evaluate the relationship between age at menarche and adult body mass index (BMI), which is a indicator of later-life health. METHODS: We conducted a cross-sectional analysis of a nationally representative sample (self-reported age at menarche and measured height and weight) of 11,065 females aged 15 and older. Data were obtained from the Fourth Korea National Health and Nutrition Examination Survey (KNHANES IV, 2007-2009). RESULTS: We found a statistically significant decline in age at menarche in successive birth groups, indicating a 0.726 year decrease per decade. The age at menarche in 1990-1994 year birth group was 12.60 years, which showed a significant decreased from 3.11 years in the 1980-1984 birth group. We also found a significant negative association between age at menarche and current BMI. A one-year decrease in age at menarche was associated with mean BMI increase of 0.109 kg/m2 (95% confidence interval [CI], 0.069 to 0.150) after adjustment for age. In multivariate logistic regression, the odds ratios of obesity in females with early menarche (<12 years) was 1.845 fold (95% CI, 1.441 to 2.361). CONCLUSION: We found that age at menarche is still falling in the Korean female. We also found that early menarche is a risk factor for obesity in adults.
Adult ; Aged ; Body Mass Index ; Cross-Sectional Studies ; Female ; Humans ; Korea ; Logistic Models ; Menarche ; Nutrition Surveys ; Obesity ; Odds Ratio ; Parturition ; Risk Factors

Hepatic sinusoidal obstruction syndrome (SOS) is a life-threatening syndrome that generally occurs as a complication after hematopoietic stem cell transplantation or, less commonly, after conventional chemotherapy. Regarding SOS in rhabdomyosarcoma patients who received conventional chemotherapy, the doses of chemotherapeutic agents are associated with the development of SOS. Several cases of SOS in rhabdomyosarcoma patients after receiving chemotherapy with escalated doses of cyclophosphamide have been reported. Here, we report on a 9-year-old female with rhabdomyosarcoma who developed severe SOS after receiving chemotherapy consisting of vincristine, actinomycin-D, and a moderate dose of cyclophosphamide. She was treated successfully with defibrotide without sequelae to the liver.
Child* ; Cyclophosphamide* ; Drug Therapy ; Female ; Hematopoietic Stem Cell Transplantation ; Hepatic Veno-Occlusive Disease* ; Humans ; Liver ; Rhabdomyosarcoma* ; Vincristine*

BACKGROUND: The appropriate procedures and equipment for the pretransfusion test are fundamental to a safe blood transfusion. The present study aimed to assess the current status of procedures and equipment for pretransfusion tests at small- and medium-sized medical institutions, as well as to use this basic raw data to better manage blood transfusions at these institutions. METHODS: Offline and online questionnaire surveys were performed at institutions that used between 24 and 1,000 units of blood products in 2014. A total of 338 institutions participated, and the survey results were subsequently analyzed. RESULTS: Among 307 institutions where on-site ABO blood typing was performed, 15.0%, 2.1%, and 43.5% did not conduct ABO serum typing, RhD typing, and irregular antibody screening tests, respectively, and 12.8% only conducted the saline phase for crossmatching. Moreover, among 338 institutions, only 66.7% of blood banks had centrifuges, 84.5% had 37℃ incubators, 41.1% had slide view boxes; in addition, 66.1% and 18.6% had refrigerators and deep freezers, respectively, for blood storage. CONCLUSION: Certain small- and medium-sized institutions did not have the essential equipment required to operate as blood banks. Moreover, they also needed to improve their testing procedures. To address these issues, the initiation of systematic training programs and the employment of institutional strategies are necessary to enhance testing procedures and equipment, respectively.
Blood Banks ; Blood Grouping and Crossmatching ; Blood Transfusion ; Education ; Employment ; Incubators ; Korea* ; Mass Screening

BACKGROUND: Organophosphate insecticides poisoning is one of the most common toxicologic emergencies in Korea. There have been few reports of organophosphate intoxication via parenteral route, although many reports on organophosphate intoxication by oral ingestion were present in the literature. This study aimed to validate the clinical characteristics of organophosphage intoxication according to the route of administration to the body. METHOD: Data were collected retrospectively by the review of the medical records from 49 patients with organophosphate intoxication. Severity of intoxication was classified by the Namba's Classification. Collected data were analysed and compared on the clinical features and laboratory findings between the patient intoxicated by inhalation or contact(parenteral group, n=23) and the other oral ingestion(enteral group, n=26). RESULTS: Severity class by clinical features was higher in enteral group than parenteral group. Severity class by serum cholinestetrase level was not positively correlated with severity class by clinical manifestations. Cholinesterase level tended to overestimate the severity of intoxication in parenteral group. Ventilator therapy and admission to intensive care unit were more frequently needed in enteral group than parenteral group in case that the severity class by clinical features was equal. CONCLUSION: In patients with organophosphate intoxication by parenteral route, serum cholinesterase level of the patient had disparity with clinical severity of intoxication. Considering this disparity, clinical severity should be considered as a more important indicator for treatment of organophosphate intoxication including atropinization, rather than serum cholinesterase level in patients intoxicated by parenteral route.
Cholinesterases* ; Classification ; Eating ; Emergencies ; Humans ; Inhalation ; Insecticides* ; Intensive Care Units ; Korea ; Medical Records ; Plasma* ; Poisoning ; Retrospective Studies ; Ventilators, Mechanical

PURPOSE: To obtain normative data on bone mineral density of each region of interest (ROI) measured by Hologic model dual-energy x-ray absorptiometry (DXA) of children and adolescents. METHODS: Cross sectional results from 723 healthy Korean children and adolescents (10-20 years of age) in the Korean National Health and Nutrition Examination Surveys were analyzed. We used age- and sex-specific reference data for bone mineral density (BMD) of the lumbar spine, femur neck, and total body except head from Hologic DXA device as recommended by the International Society for Clinical Densitometry. RESULTS: The bone mineral density of each ROI increased with age in both boys and girls. Maximal increase in the lumbar BMD occurred between ages 11 and 12 in girls and between ages 12 and 14 in boys. However, the increases of BMD in each ROI were different. The plateaus of the lumbar spine and whole body except head BMD in girls occurred at ages 15 and 17, respectively. The plateaus of BMD in each ROI occurred at age 17 in boys. CONCLUSION: Most of the skeletal mass, including lumbar spine and total body except head, is reached before the end of the second decade. This study provides reference values for bone density of each ROI measured with DXA for children and adolescents.
Absorptiometry, Photon ; Adolescent ; Bone Density ; Child ; Femur Neck ; Head ; Humans ; Korea ; Reference Values ; Sex Characteristics ; Spine