PURPOSE: Routine screening for toxoplasmosis, rubella, cytomegalovirus (CMV), and herpes simplex virus (TORCH) in intrauterine growth restriction (IUGR) and small for gestational age (SGA) neonates has become a common practice. However, the incidence of TORCH varies across countries, and the cost of TORCH testing may be disadvantageous compared to disease-specific screening. To evaluate the efficacy of TORCH screening, the medical charts of IUGR or SGA neonates born in a single institution in Bucheon, Korea from 2011 to 2015 were reviewed. METHODS: The clinical data of the 126 IUGR or SGA neonates were gathered, including gestational age, Apgar scores, neonatal sonographic findings, chromosome study, morbidities, developmental follow-up, and growth catch-up. Maternal factors including underlying maternal disease and fetal sonography were collected, and placental findings were recorded when available. TORCH screening was done using serum IgM, CMV urine culture, quantification of CMV DNA with real-time polymerase chain reaction, and rapid plasma reagin qualitative test for syphilis. Tests were repeated only for those with positive results. RESULTS: Of the 119 TORCH screenings, only one was positive for toxoplasmosis IgM. This result was deemed false positive due to negative IgM on repeated testing and the absence of clinical symptoms. CONCLUSION: Considering the incidence and risk of TORCH in Korea, the financial burden of TORCH screening, and the single positive TORCH finding in our study, we suggest disease-specific screening based on maternal history and the clinical symptoms of the neonate. Regarding CMV, which may present asymptomatically, universal screening may be appropriate upon cost-benefit analysis.
Cost-Benefit Analysis ; Cytomegalovirus* ; DNA ; Fetal Growth Retardation ; Follow-Up Studies ; Gestational Age* ; Gyeonggi-do ; Herpes Simplex* ; Humans ; Immunoglobulin M ; Incidence ; Infant, Newborn* ; Korea* ; Mass Screening* ; Plasma ; Real-Time Polymerase Chain Reaction ; Rubella* ; Simplexvirus ; Syphilis ; Toxoplasmosis ; Ultrasonography

PURPOSE: The aim of this study was to evaluate the efficacy of pamidronate therapy in children and adolescents with secondary osteoporosis. METHODS: Nine patients (7 males, 2 females, 13.2 +/- 2.5 years, 10.1-17.4 years) with secondary osteoporosis who had a history of severe bone pain and/or fracture were enrolled. Intravenous pamidronate 1.5 mg/kg (0.5 mg/kg for 3 consecutive days) was given every 6 to 8 weeks for 0.86 +/- 0.15 years (6 or 8 cycles). Bone mineral density (BMD) in lumbar spine and femoral neck and their Z-scores were measured before treatment, after the fourth and last cycle (sixth or eighth cycle). RESULTS: Underlying diseases were as follows; neurofibromatosis type 1 (n = 2), epilepsy with/without cerebral palsy (N=2), autoimmune disease treated with steroid (n = 2), hematologic malignancy (n = 3). Bone pain was relieved in most of the patients after the first cycle of treatment, and no more fracture occurred thereafter. There was a significant increase in BMD Z-score of the lumbar spine and femoral neck after the last cycle of therapy, compared to baseline values (from -3.91 +/- 1.79 to 1.86 +/- 1.18, in L1-4 and -3.71 +/- 1.83 to -2.53 +/- 1.77 for femoral neck; P = 0.008 and 0.011, respectively). However, there was no significant change in BMD Z-scores between the fourth cycle and the last cycle. Fever developed in 7 out of 9 patients (77.8%), which was relieved by antipyretics. Total serum levels of calcium and phosphorus were significantly decreased (calcium, P = 0.008; phosphorus, P = 0.015) after pamidronate therapy, and three of them experienced symptomatic hypocalcemia during the first cycle. The growth velocity was normal during follow-up periods (mean, 4.47 +/- 1.69 years; range, 1.05 to 6.77 years). CONCLUSION: In conclusion, pamidronate can be administered to the patients with secondary osteoporosis, relieving the symptoms and signs effectively and safely. However, its side effects should be monitored during treatment.
Adolescent ; Antipyretics ; Autoimmune Diseases ; Bone Density ; Calcium ; Cerebral Palsy ; Child ; Diphosphonates ; Epilepsy ; Female ; Femur Neck ; Fever ; Follow-Up Studies ; Hematologic Neoplasms ; Humans ; Hypocalcemia ; Male ; Neurofibromatosis 1 ; Osteoporosis ; Phosphorus ; Spine

PURPOSE: Short sleep duration is associated with obesity. Urinary 6-sulfatoxymelatonin (6-OHMS), the principal metabolite of melatonin, is closely related with sleep. We evaluated the difference in urinary 6-OHMS levels between obese girls and normal weight girls, and the relationship of urinary 6-OHMS with other hormones regulating body weight and metabolism. METHODS: A total of 79 girls (6.3 to 12.4 years) were included in this study, of whom 34 were obese; 15, overweight; and 30, normal-weight. We examined their pubertal status and bone age. Fasting serum levels of total ghrelin, leptin, insulin, and first morning urinary 6-OHMS were measured. Homeostatic model assessment-insulin resistance (HOMA-IR) was calculated from the fasting insulin and glucose levels. RESULTS: There was no significant difference in the creatinine adjusted 6-OHMS levels between the obese girls and the control group. Urinary 6-OHMS did not show any correlations with body mass index (BMI), BMI percentile, total ghrelin, leptin, and HOMA-IR. Negative correlations were found between urinary 6-OHMS levels and chronological and bone ages. CONCLUSION: Our results suggest that melatonin production is not reduced consistently in obese girls.
Body Mass Index ; Body Weight ; Child ; Creatinine ; Fasting ; Ghrelin ; Glucose ; Humans ; Insulin ; Leptin ; Melatonin ; Obesity

Hypoglycemia is one of the severe complications of diabetes. To prevent hypoglycemia, an emphasis is placed on maintaining an appropriate balance between nutrition, activity, and treatment, which can be achieved by the repetition of self-trials based on self-monitoring. Clinicians routinely focus on patients’ contribution, including timely intake of an adequate amount of carbohydrates, physical activity, antidiabetic medication, and abstinence from alcohol. Recently, many guidelines have highlighted the importance of clinicians’ factors and recommend individualized treatments according to lifestyle patterns and specific needs following the de-intensification of treatment. The optimal value of hemoglobin A1c (HbA1c) levels for blood glucose level regulation remains controversial among countries, but it generally does not exceed 8.0%. In populations that are at a risk of hypoglycemia, such as the older adults, it is advisable to adjust the target blood glucose level to less than 8.0%. Meanwhile, a blood glucose level of 7.0%–7.5% is generally recommended for healthy older adults. If the expected lifetime is shorter than 10 years or in patients with chronic kidney disease and severe cardiovascular disease, the HbA1c level target can be increased to 7.5%–8.0%. For even shorter lifetime expectancy, the target can be adjusted up to 8.0%–9.0%. To prevent hypoglycemia, the target blood glucose level needs to be adjusted, particularly in older adult patients. Ultimately, it is important to identify the maximum blood glucose levels that do not cause hypoglycemia and the minimum blood glucose levels that do not cause hyperglycemia-associated complications.

Hypoglycemia is one of the severe complications of diabetes. To prevent hypoglycemia, an emphasis is placed on maintaining an appropriate balance between nutrition, activity, and treatment, which can be achieved by the repetition of self-trials based on self-monitoring. Clinicians routinely focus on patients’ contribution, including timely intake of an adequate amount of carbohydrates, physical activity, antidiabetic medication, and abstinence from alcohol. Recently, many guidelines have highlighted the importance of clinicians’ factors and recommend individualized treatments according to lifestyle patterns and specific needs following the de-intensification of treatment. The optimal value of hemoglobin A1c (HbA1c) levels for blood glucose level regulation remains controversial among countries, but it generally does not exceed 8.0%. In populations that are at a risk of hypoglycemia, such as the older adults, it is advisable to adjust the target blood glucose level to less than 8.0%. Meanwhile, a blood glucose level of 7.0%–7.5% is generally recommended for healthy older adults. If the expected lifetime is shorter than 10 years or in patients with chronic kidney disease and severe cardiovascular disease, the HbA1c level target can be increased to 7.5%–8.0%. For even shorter lifetime expectancy, the target can be adjusted up to 8.0%–9.0%. To prevent hypoglycemia, the target blood glucose level needs to be adjusted, particularly in older adult patients. Ultimately, it is important to identify the maximum blood glucose levels that do not cause hypoglycemia and the minimum blood glucose levels that do not cause hyperglycemia-associated complications.

Background: There are many models for predicting diabetes mellitus (DM), but their clinical implication remains vague. Therefore, we aimed to create various DM prediction models using easily accessible health screening test parameters. Methods: Two sets of variables were used to develop eight DM prediction models. One set comprised 62 easily accessible examination results of commonly used variables from a tertiary university hospital. The second set comprised 27 of the 62 variables included in the national routine health checkups. Gradient boosting and random forest algorithms were used to develop the models. Internal validation was performed using the stratified 10-fold cross-validation method. Results: The area under the receiver operating characteristic curve (ROC-AUC) for the 62-variable DM model making 12-month predictions for subjects without diabetes was the largest (0.928) among those of the eight DM prediction models. The ROC-AUC dropped by more than 0.04 when training with the simplified 27-variable set but still showed fairly good performance with ROC-AUCs between 0.842 and 0.880. The accuracy was up to 11.5% higher (from 0.807 to 0.714) when fasting glucose was included. Conclusion We created easily applicable diabetes prediction models that deliver good performance using parameters commonly assessed during tertiary university hospital and national routine health checkups. We plan to perform prospective external validation, hoping that the developed DM prediction models will be widely used in clinical practice.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian ducts, and is characterized by the triad of uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis. Generally it is diagnosed at puberty after menarche due to recurrent pelvic pain or abdominal mass. We report 2 cases of female newborns whose fetal ultrasonography (USG) showed unilateral renal agenesis and were diagnosed with HWWS by postnatal evaluation. Both cases were female newborns who were born at term by vaginal delivery. They had no perinatal problems except suspicious findings of unilateral renal agenesis from fetal USG. Abdominal ultrasonography and pelvic MRI were performed after birth, and they were diagnosed with HWWS. The potential complications of this syndrome such as pyosalpinx, pelvic adhesions and increased risk of abortion or infertility can occur, but without complication, the prognosis is very good with simple surgical treatment. If renal agenesis is detected in a fetus or a newborn, possible anomalies of urogenital tract such as HWWS should be considered; and postnatal evaluation should be performed, as a simple surgical treatment before menarche can prevent unnecessary complications of disease.
Congenital Abnormalities ; Female ; Fetus ; Humans ; Infant, Newborn ; Infertility ; Kidney ; Kidney Diseases ; Menarche ; Mullerian Ducts ; Parturition ; Pelvic Pain ; Prognosis ; Puberty ; Ultrasonography, Prenatal ; Urogenital Abnormalities ; Wolffian Ducts

BACKGROUND: While many studies have shown the good efficacy and safety of exenatide in patients with diabetes, limited information is available about exenatide in clinical practice in Korean populations. Therefore, this retrospective cohort study was designed to analyze the effects of exenatide on blood glucose level and body weight in Korean patients with type 2 diabetes mellitus. METHODS: We reviewed the records of the patients with diabetes who visited Seoul St. Mary's Hospital and for whom exenatide was prescribed from June 2009 to October 2011. After excluding subjects based on their race/ethnicity, medical history, whether or not they changed more than 2 kinds of oral hypoglycemic agents with exenatide treatment, loss to follow-up, or whether they stopped exenatide therapy within 6 months, a total of 52 subjects were included in the final analysis. RESULTS: The mean glycated hemoglobin (HbA1c) level and weight remarkably decreased from 8.5+/-1.7% to 6.7+/-1.0% (P<0.001) and from 82.3+/-15.8 kg to 78.6+/-16.3 kg (P<0.001), respectively. The multiple regression analysis indicated that the reduction in HbA1c level was significantly associated with a shorter duration of diabetes, a higher baseline HbA1c level, and greater weight reduction, whereas weight loss had no significant correlation with other factors. No severe adverse events were observed. CONCLUSION: These results suggest that a 6-month exenatide injection therapy significantly improved patients' HbA1c levels and body weights without causing serious adverse effects in Korean patients with type 2 diabetes.
Blood Glucose ; Body Weight ; Cohort Studies ; Diabetes Mellitus, Type 2 ; Follow-Up Studies ; Glucagon-Like Peptide 1 ; Hemoglobins ; Humans ; Hypoglycemic Agents ; Peptides ; Retrospective Studies ; Treatment Outcome ; Venoms ; Weight Loss

Obesity and its related factors are known to suppress the secretion of growth hormone (GH). We aimed to evaluate the influence of body mass index (BMI) on the peak GH response to provocative testing in short children without GH deficiency. We conducted a retrospective review of medical records of 88 children (2-15 yr old) whose height was less than 3 percentile for one's age and sex, with normal results (peak GH level > 10 ng/mL) of GH provocative testing with clonidine and dopamine. Peak stimulated GH level, height, weight, pubertal status and serum IGF-1 level were measured. Univariate analysis showed that the BMI standard deviation score (SDS) correlated negatively with the natural log (ln) of the peak stimulated GH level (ln peak GH). BMI SDS did not correlate significantly with sex, age, pubertal status, or ln IGF-1 level. BMI SDS correlated negatively with ln peak GH level induced by clonidine but not by dopamine. In stepwise multivariate regression analysis, BMI SDS was the only significant predictor of ln peak GH level in the combination of tests and the clonidine test, but not in the dopamine test. In children without GH deficiency, BMI SDS correlates negatively with the peak GH level. BMI SDS should be included in the analysis of the results of GH provocation tests, especially tests with clonidine.
Adolescent ; Body Height ; *Body Mass Index ; Body Weight ; Child ; Child, Preschool ; Clonidine/therapeutic use ; Dopamine/therapeutic use ; Dwarfism/drug therapy ; Female ; Human Growth Hormone/*analysis ; Humans ; Insulin-Like Growth Factor I/analysis ; Male ; Regression Analysis ; Retrospective Studies

PURPOSE: This study aimed to evaluate the prognosis of necrotizing enterocolitis (NEC) according to the extent of involvement, among very low birth weight infants. Furthermore, the predictive factors for extent of involvement were evaluated. METHODS: Medical records of all newborns with surgically treated NEC admitted to the neonatal intensive care unit of Seoul National University Children's Hospital between 2005 and 2013 were reviewed. Infants were grouped according to the extent of involvement of NEC: isolated segment involvement (ISI, n=31) and multi-segment involvement (MSI, n=17). We evaluated the clinical characteristics, outcomes, and pre-operative factors according to symptoms, laboratory and radiologic findings. RESULTS: The incidence of small for gestational age was significantly higher in the MSI than ISI group (12.9% vs. 41.2%, P=0.036). The length of resected bowel was significantly longer (1.7 cm vs. 8 cm, P=0.010), and the incidence of short bowel syndrome (SBS) (0% vs. 23.1%, P=0.023) and mortality (3.2% vs. 23.5%, P=0.047) were significantly higher in the MSI than ISI group. However, there was no significant difference between the two groups in terms of high-output stoma, time of full enteral feeding, extrauterine growth retardation, changes of z-score of body weight between admission and discharge and reoperation. Portal vein gas detected by ultrasonography was the only statistically significant predictive factor of extent of involvement (odds ratio=13.237, P=0.029). CONCLUSION: SBS and mortality were higher in MSI NEC compared to ISI NEC. However, there was no difference in the time of full enteral feeding and growth between the two groups. Portal vein gas detected by ultrasonography maybe a predictive factor of extent of NEC.
Body Weight ; Enteral Nutrition ; Enterocolitis, Necrotizing* ; Gestational Age ; Humans ; Incidence ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Medical Records ; Mortality ; Portal Vein ; Prognosis* ; Reoperation ; Seoul ; Short Bowel Syndrome ; Ultrasonography