No abstract available.
Stevens-Johnson Syndrome*

No abstract available.

No abstract available.
Kasabach-Merritt Syndrome*

Slipped capital femoral epiphysis is a rare hip disorder that mainly occurs in pubertal children. Although the exact cause of this disorder is unknown, it is known to be associated with obesity, trauma, delayed sexual development, delayed bone maturation, chronic renal failure, genetic diseases, endocrine disorders (growth hormone deficiency, hypothyroidism, hypogonadism), growth hormone therapy, and gonadotropin releasing hormone agonist (GnRH agonist) therapy. We report 2 cases of slipped capital femoral epiphysis in adolescent females who were receiving growth hormone therapy. The first case is of a 16 year-old-girl with chronic renal failure and renal osteodystrophy. The second case is of an 11 year-old-girl with idiopathic precocious puberty who had received GnRH agonist and growth hormone therapy. Unilateral or bilateral slipped capital femoral epiphysis developed at 1 year 3 months after treatment in both the cases. The chief complaints were pain in the hip joint and lower extremities. Growth hormone and/or GnRH agonist therapy was stopped, and in situ screw fixations of the involved hip epiphyses were performed.
Adolescent ; Child ; Endocrine System Diseases ; Epiphyses ; Female ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Hip ; Hip Joint ; Humans ; Hypothyroidism ; Kidney Failure, Chronic ; Lower Extremity ; Obesity ; Puberty, Precocious ; Renal Osteodystrophy ; Sexual Development ; Slipped Capital Femoral Epiphyses

Human-derived materials are a crucial element of research in the life sciences. The Korea Biobank Network (KBN) portal is a shared open platform that provides the nationʼs most extensive disease resources, possessed by Human Bioresource Unit Banks of the KBN, to the public, including those in the fields of industry, academia, and research.This platform was developed to increase the efficient use of national disease resources. In the KBN portal, the current status of disease resources collected in Korea can be checked online. Human bioresources and clinical information are provided to consumers through systematic search and efficient distribution programs. Additionally, by simultaneously operating the KBN Distribution Support Center, we are working to support the rapid and convenient distribution of human resources in response to the needs of consumers. To effectively utilize the open human bioresource sharing platform, it is necessary to introduce an integrated clinical information management system. Currently, the KBN is in the process of establishing standard terminology for data and applying a common data model for the integrated management of various clinical information held by the KBN. We provide communications through the KBN portal, which is interconnected with the distribution support center, regional biobanks, and consumers. In conclusion, the KBN portal will provide open innovation by creating a business or service model by delivering shared open data and internalizing external innovative capabilities.

No abstract available.
Humans ; Infant, Newborn* ; Intracranial Hemorrhages* ; Vitamin K* ; Vitamins*

OBJECTIVE: Adult onset Still s disease is an acute systemic inflammatory disorder. There are no pathognomonic symptoms or specific laboratory abnormalities. In recent reports, serum ferritin concentration is increased in active disease phase and decreased after defervescence. Our purpose was to determine the clinical significance of serum ferritin as an indicator for disease activity. METHODS: Seven patients who were diagnosed as adult onset Still s disease at Samsung Medical Center between October 1994 and March 1997, were reviewed. In these patients we checked leukocyte count, ESR, CRP and serum ferritin concentrations at the time of diagnosis and during follow-up periods and recorded febrile events during follow-up periods. RESULTS: At the time of diagnosis and during febrile periods, the concentrations of ferritin were extremely high(927ng/ml to 96,650ng/ml normal 10-290.8 ng/ml). The values were unrelated to other manifestations of the disease or laboratory findings. The ferritin concentrations decreased rapidly after adequate treatment. Eleven febrile reattacks happened in 7 patients. Serum ferritin concentrations were increased in 8 febrile attacks, while leukocyte count, ESR, and CRP were increased in 5, 5, 6 febrile attacks respectively, There were 10 events of increased serum ferritin concentrations in 7 patients during follow-up periods and 8 events were related with fever. The increases of other laboratory tests were similar. CONCLUSIONS: In all patients, serum ferritin concentrations were increased at the time of diagnosis and closely related to fever. During follow-up periods, serum ferritin concentrations are helpful in monitoring disease activity and guiding decisions about treatment.
Adult* ; Diagnosis ; Ferritins* ; Fever ; Follow-Up Studies ; Humans ; Leukocyte Count ; Still's Disease, Adult-Onset*

PURPOSE: This study aimed to test the mediating effect of uncertainty appraisal and coping style in the relation between uncertainty and anxiety in hospitalized pregnant women diagnosed with preterm labor. METHODS: The participants were 105 pregnant women diagnosed with preterm labor in hospitals in Korea. Data were collected from July to October 2017. The measurements included the Uncertainty in Illness Scale, Uncertainty Appraisal Scale, Coping Style Scale, and State Anxiety Inventory. Data were analyzed using descriptive statistics, an independent t-test, correlation, and multiple regression following the Baron and Kenny method and Sobel test for mediation. RESULTS: The mean score for anxiety was 2.29 out of 4.00 points and for uncertainty it was 2.46 out of 5.00 points. There were significant correlations among uncertainty, uncertainty danger appraisal, uncertainty opportunity appraisal, problem-focused coping, emotion-focused coping, and anxiety. Uncertainty danger appraisal (β=.64, p < .001) had a complete mediating effect in the relation between uncertainty and anxiety (Z=4.54, p < .001). Uncertainty opportunity appraisal (β=−.45, p < .001) had a complete mediating effect in the relation between uncertainty and anxiety (Z=3.28, p < .001). Emotion-focused coping (β=−.23, p=.021) had a partial mediating effect in the relation between uncertainty and anxiety (Z=2.02, p=.044). CONCLUSION: Nursing intervention programs focusing on managing uncertainty appraisal and improving emotion-focused coping are highly recommended to decrease anxiety in hospitalized pregnant women diagnosed with preterm labor.
Anxiety* ; Female ; Humans ; Korea ; Methods ; Negotiating* ; Nursing ; Obstetric Labor, Premature* ; Pregnancy ; Pregnant Women* ; Uncertainty*

In Behcet's disease(BD), there is a marked increase in vascular complication. Venous thrombosis is a major feature of the disease, although arterial thrombosis is rarely described. In Behcet's disease, thrombosis occurs in 20 to 30% of patients. We present two cases of Behcet's disease admitted to our hospital whose chief complaint was progressive increases in swelling and pain in their legs. In the first case, routine coagulation tests and sero-immunological tests were within normal limits, however, protein C and S activity were significantly decreased in the patient. So these findings suggest that auto-immune acquired protein S deficiency may be involved in the pathogenesis of thrombotic events in BD.
Humans ; Leg ; Protein C ; Protein S Deficiency ; Thrombosis ; Venous Thrombosis*

PURPOSE: Assessment of the diameters of the aortic arches in the human fetus may be helpful in the prenatal diagnosis of aortic arch anomalies. The purpose of this study is to construct valuable reference ranges of fetal aortic arch for various fetal growth parameters in an unselected Korean population. METHODS: A prospective, cross-sectional study was performed in 98 normal singleton fetuses between 20 and 38 weeks of gestation. The transverse aortic arch diameter (TAD) and distal aorta isthmus diameter (AID) were measured in the longitudinal view during systole. The bi-parietal diameter (BPD), head circumference (HC), abdominal circumference (AC) and femur length (FL) were obtained at the same time. Ultrasound examinations were performed with a 2.0-6.0 MHz convex probe by abdominal ultrasound. RESULTS: TAD and AID as a function of gestational age (GA) was expressed by the regression equation TAD= -1.246+0.168 X GA, and AID = -1.341+0.180 X GA; TAD and AID are expressed in millimeters and GA in weeks. TAD and AID as a function of BPD was expressed by the regression equation TAD= -1.147+0.664 X BPD, and AID = -1.152+0.696 X BPD. TAD and AID as a function of HC was expressed by the regression equation TAD = -1.187+0.182 X HC, and AID = -1.350+0.198 X HC. TAD and AID as a function of AC was expressed by the regression equation TAD = -0.528+0.173 X AC, and AID = -0.533+0.183 X AC. TAD and AID as a function of FL was expressed by the regression equation TAD = -0.519+0.799 X FL, and AID = -0.601+0.860 X FL; BPD, HC, AC and FL are expressed in centimeters. Correlations were found to be highly statistically significant in relation to GA, BPD, HC, AC, and FL respectively (P<0.0001 in all). Normal mean of TAD and AID per GA, BPD, HC, AC and FL and 95% prediction limits were also defined, respectively. CONCLUSION: New reference ranges of fetal aortic arch related to other fetal bio-parameters representing fetal growth were obtained successfully. The normative date will be helpful in the prenatal accurate diagnosis of aortic anomalies and abnormal growth of fetal aortic arches in Korea.
Aorta ; Aorta, Thoracic ; Cross-Sectional Studies ; Echocardiography ; Femur ; Fetal Development ; Fetus ; Gestational Age ; Head ; Humans ; Korea ; Pregnancy ; Prenatal Diagnosis ; Prospective Studies ; Reference Values ; Systole