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

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.

Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from –4.09±0.83 to –3.68±0.43 after 1 year of hGH treatment, and to –3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

BACKGROUND: We evaluated the efficacy and safety of acarbose add-on therapy in Korean patients with type 2 diabetes mellitus (T2DM) who are inadequately controlled with metformin and sitagliptin. METHODS: A total of 165 subjects were randomized to metformin and sitagliptin (Met+Sita, n=65), metformin, sitagliptin, and acarbose (Met+Sita+Acarb, n=66) and sitagliptin and acarbose (Sita+Acarb, exploratory assessment, n=34) therapy in five institutions in Korea. After 16 weeks of acarbose add-on or metformin-switch therapy, a triple combination therapy was maintained from week 16 to 24. RESULTS: The add-on of acarbose (Met+Sita+Acarb group) demonstrated a 0.44%±0.08% (P<0.001 vs. baseline) decrease in glycosylated hemoglobin (HbA1c) at week 16, while changes in HbA1c were insignificant in the Met+Sita group (−0.09%±0.10%, P=0.113). After 8 weeks of triple combination therapy, HbA1c levels were comparable between Met+Sita and Met+Sita+Acarb group (7.66%±0.13% vs. 7.47%±0.12%, P=0.321). Acarbose add-on therapy demonstrated suppressed glucagon secretion (area under the curve of glucagon, 4,726.17±415.80 ng·min/L vs. 3,314.38±191.63 ng·min/L, P=0.004) in the absence of excess insulin secretion during the meal tolerance tests at week 16 versus baseline. The incidence of adverse or serious adverse events was similar between two groups. CONCLUSION: In conclusion, a 16-week acarbose add-on therapy to metformin and sitagliptin, effectively lowered HbA1c without significant adverse events. Acarbose might be a good choice as a third-line therapy in addition to metformin and sitagliptin in Korean subjects with T2DM who have predominant postprandial hyperglycemia and a high carbohydrate intake.
Acarbose ; Diabetes Mellitus, Type 2 ; Drug Therapy, Combination ; Glucagon ; Hemoglobin A, Glycosylated ; Humans ; Hyperglycemia ; Incidence ; Insulin ; Korea ; Meals ; Metformin ; Sitagliptin Phosphate

BACKGROUND/AIMS: This study evaluated the relationship between thyroid nodules and metabolic syndrome (MS) and its components in apparently healthy Koreans. METHODS: We reviewed the records of 3,298 subjects with no noticeable symptoms who underwent thyroid ultrasound imaging as part of a routine check-up between July 2009 and June 2010; of these, 1,308 were excluded based upon predefined criteria. Among the remaining 1,990 patients, we examined the association between MS and its components and the incidence of thyroid nodules. RESULTS: Of the 1,990 subjects included in this study, 38.4% (n = 764) had thyroid nodules and 12.7% (n = 253) had MS. Female sex, older age, higher body mass index, larger waist circumference, higher glycated hemoglobin level, lower thyroid stimulating hormone level, and presence of MS were all closely related with the presence of thyroid nodules (all p < 0.05). Furthermore, the relevant number of MS components showed a positive linear correlation with the occurrence of thyroid nodules (p < 0.001). Evidence of MS alone was not independently associated with thyroid nodules after adjusting for sex and age in a multivariate binary logistic regression analysis; however, glycated hemoglobin for females and waist circumference for males, as well as both age and thyroid stimulating hormone for all patients, were identified as independent predictors for the existence of thyroid nodules (all p < 0.05). CONCLUSIONS: This study suggests a positive relationship between the components of MS and thyroid nodules in an ostensibly healthy Korean population. Our data support the idea that the recent increase in thyroid nodules is partly due to increases in both MS and obesity.
Adult ; Biomarkers/blood ; Body Mass Index ; Chi-Square Distribution ; Cross-Sectional Studies ; Diabetes Mellitus/diagnosis/epidemiology ; Female ; Hemoglobin A, Glycosylated/analysis ; Humans ; Incidence ; Linear Models ; Logistic Models ; Male ; Metabolic Syndrome X/blood/diagnosis/*epidemiology ; Middle Aged ; Multivariate Analysis ; Obesity/diagnosis/epidemiology ; Odds Ratio ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Thyroid Nodule/blood/diagnostic imaging/*epidemiology ; Thyrotropin/blood ; Waist Circumference

Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré syndrome (GBS) are both rare post-infectious neurological disorders. The co-existence of these conditions has often been reported despite of low incidence. We describe a 20-year-old male, who presented with acute flaccid paralysis and encephalopathy. The patient showed reversible MRI lesions suggesting ADEM. This case showed anti-GT1a IgG and anti-GM1 IgM antibodies positivity. We suggest that certain immunogenicity within central and peripheral nervous system may share a common autoimmune process during the disease course.
Antibodies* ; Brain Diseases ; Encephalomyelitis, Acute Disseminated* ; Gangliosides ; Guillain-Barre Syndrome* ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Incidence ; Magnetic Resonance Imaging ; Male ; Nervous System Diseases ; Paralysis ; Peripheral Nervous System ; Young Adult

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

Necrotizing enterocolitis (NEC) is a major gastrointestinal disorder in premature infants associated with high morbidity and mortality rates. When NEC is clinically suspected, radiological and laboratory studies should be performed to confirm the diagnosis and to aid in the management of patients. As the clinical manifestations of NEC are usually nonspecific, diagnoses are often made using abdominal radiographic findings, such as pneumatosis intestinalis. Clinicians typically consider the presence of pneumatosis intestinalis on radiographs as the definite evidence of stage II NEC. Here, we report 3 cases of preterm infants who had radiographic findings of pneumatosis intestinalis but did not have any other associated laboratory and clinical evidence of NEC, except bloody stools. The infants' systemic manifestations were mild or absent, and all of them completely recovered within 2-3 days, as demonstrated by the resolution of pneumatosis intestinalis on abdominal radiographs. The combination of hematochezia and intestinal pneumatosis in preterm infants strongly suggests the diagnosis of NEC. In our cases, there was no laboratory evidence of inflammation or platelet consumption, and the clinical course was benign without any sings of surgical abdomen. Additionally, our patients had barium-induced colitis or milk protein allergy, which are other possible causes of pneumatosis intestinalis. Because pneumatosis intestinalis can result from causes other than NEC, it is important to consider clinical, laboratory, and radiological findings to confirm the diagnosis of NEC.
Abdomen ; Blood Platelets ; Colitis ; Diagnosis ; Enterocolitis, Necrotizing* ; Gastrointestinal Hemorrhage ; Humans ; Hypersensitivity ; Infant, Newborn ; Infant, Premature* ; Inflammation ; Milk Proteins ; Mortality

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