No abstract available.
Obesity*

No abstract available.
Diabetes Mellitus* ; Obesity*

No abstract available.
Adipocytes* ; Homeostasis*

Adolescent* ; Humans ; Insulin Resistance* ; Insulin* ; Polycystic Ovary Syndrome*

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*

Congenital lipoid adrenal hyperplasia, the most severe form of congenital adrenal hyperplasia, is caused by mutations in the steroidogenic acute regulatory protien (StAR). It is characterized by failure of synthesis of all three classes of adrenal steroids and massive accumulation of lipids and cholesterol in the adrenal cortex. The computed tomography (CT) unequivocally delineated massively enlarged adrenal glands of fat-tissue attenuation, enabling early diagnosis and replacement therapy. We report a case of congenital lipoid adrenal hyperplasia, in which CT established that lipoid deposition at the adrenal cortex disappeared after the adrenal hormone replacement therapy.
Adrenal Cortex ; Adrenal Glands ; Adrenal Hyperplasia, Congenital ; Cholesterol ; Early Diagnosis* ; Hormone Replacement Therapy ; Hyperplasia* ; Steroids

Neonatal diabetes mellitus (NDM) is defined as hyperglycemia that presents clinical symptoms within the first month of life and requires insulin therapy to maintain euglycemia. The diabetic condition may be transient, permanent, or transient with recurrence later in life. NDM is a rare clinical disorder. Three cases of NDM have been reported in Korea so far. We experienced two cases of NDM in twin brothers who were born with small for gestational ages. Their HLA typings were DR9 and DR14, and insulin autoantibody, islet cell antibody, GAD-reactive autoantibody, and insulin receptor antibody were not found. The genetic analysis with polymorphic DNA markers for chromosome 6 indicated paternal uniparental isodisomy.
Chromosomes, Human, Pair 6 ; Diabetes Mellitus* ; Genetic Markers ; Gestational Age ; Histocompatibility Testing ; Humans ; Hyperglycemia ; Insulin ; Islets of Langerhans ; Korea ; Receptor, Insulin ; Recurrence ; Siblings* ; Twins* ; Uniparental Disomy

Diabetes is a rapidly increasing heath care problem all over the world due to increased prevalence during past decade. Diabetic nephropathy develops in 25-30% of patients with type 1 diabetes and is the leading cause of end stage renal disease. Diabetic nephropathy is characterized by persistent proteinuria, decline in renal function, hypertension and increased cardiovascular morbidity and mortality. Early detection of diabetic nephropathy risk is an important goal because early diagnosis and treatment prevent advanced renal damage and other diabetic complications. Increased urinary albumin excretion rate is widely accepted as the first clinical sign of diabetic nephropathy. However, reduced glomerular filtration or hypertension could be the first manifestation in some diabetic patients. We need improved markers and predictors of diabetic nephropathy risk. We report a case of diabetic nephropathy and decreased glomerular filtration rate (GFR) without microalbuminuria occcured in type 1 diabetic patient.
Diabetes Complications ; Diabetic Nephropathies* ; Early Diagnosis ; Filtration ; Glomerular Filtration Rate ; Humans ; Hypertension ; Kidney Failure, Chronic ; Mortality ; Prevalence ; Proteinuria

PURPOSE:Idiopathic ketotic hypoglycemia (IKH) is an important cause of hypoglycemia in children. The purpose of this study was to determine the prevalence of ketotic hypoglycemia and to describe the clinical manifestation. METHODS:We conducted a retrospective chart review of children presenting hypoglycemia to the emergency department (ED) of Ewha Womans University Hospital between January 1995 and June 2004. Information recorded for subject included age, gender, weight, time of presentation, presenting symptoms, and laboratory data. RESULTS:Eighty two children were identified for hypoglycemia during the study period. IKH was the ultimate diagnosis for 66 patients (80.5%). Other diagnoses included hyperinsulinism (2.4%), drug adverse event (1.2%), sepsis (1.2%), and other disorder. The mean age for presentation of IKH was 37.9+/-18.6 months. Thirty eight boys and twenty eight girls were identified. The median time of presentation was 9:48 a.m. Of the 66 subjects, body weight of 46.9% patients was below 25th percentile for age. The average blood glucose was 41.4+/-14.2 mg/dL. Symptoms at presentation included lethargy (24.2%), mental change (16.7%) and vomiting (16.7%). 49 patients (74.2%) were described as having a concomitant illness. CONCLUSION: IKH was responsible for 80.4% of cases of hypoglycemia in pediatric ED and it had usually concomitant intercurrent illness which resulting in poor oral intake. Awareness of IKH, and its presenting characteristics, will be helpful in the ED when taking care of hypoglycemic children.
Blood Glucose ; Body Weight ; Child ; Diagnosis ; Emergency Service, Hospital ; Female ; Humans ; Hyperinsulinism ; Hypoglycemia* ; Lethargy ; Prevalence* ; Retrospective Studies ; Sepsis ; Vomiting

PURPOSE:It is well known that childhood obesity associated with high morbidity of hyperlipidemia, hypertension, fatty liver and type 2 diabetes mellitus. Recently investigators have revealed that hyperinsulinemia in obese children is related with not only insulin resistance but also hyperlipidemia and fatty liver. We investigated the relationship between fasting insulin level and comorbidities of childhood obesity. Also we determined the prevalence of asymptomatic fatty liver and presumed nonalcoholic steatohepatitis and to assess the correlation of fatty liver with degree of obesity and serum insulin level. METHODS:We measured fasting serum insulin in 300 obese children (obesity index greater than 20 percent), from 8 to 15 years old age and blood pressure, fasting serum glucose and lipid profiles were also checked. Fatty liver was diagnosed by liver function test and abdominal ultrasonography. The correlations of insulin with BMI, blood pressure and lipid profiles were analyzed by linear regression. The prevalences of hypertension, hyperlipidemia and fatty liver were compared according to fasting insulin level. RESULTS:Mean obesity index of obese children was 35.26 (37.01 in male, 31.61 in female). Mean body mass index of obese children was 26.15 kg/m2 (26.72 kg/m2 in male, 24.98 kg/m2 in female). Among obese children, 87 children had hypercholesterolemia (>or=200 mg/dL, 28.9%), 60 children had hypertriglyceridemia (>or=150 mg/dL, 20.0%), 128 children had fatty liver (42.7%) and 139 children had hyperinsulinemia (46.6%). Serum insulin level had positive correlation with body mass index (r=0.274, P>0.01), systolic blood pressure (r=0.291, P<0.01), serum triglyceride (r=0.339, P<0.01) and not with serum total cholesterol. The prevalences of hypertriglyceridemia and fatty liver were higher in obese children with hyperinsulinemia than with normal insulin level (P<0.01). The severity of fatty liver was positively related to BMI, obesity index, triglyceride, insulin, HOMA. Fatty liver with elevated ALT was found in 40 subjects (13.3%). CONCLUSION: In this study, fasting insulin level had closely related with dyslipidemia and fatty liver in obese children. Our results suggest that monitoring of insulin level is helpful to assess the morbidities and should become a part of routine care of obese children.
Adolescent ; Blood Glucose ; Blood Pressure ; Body Mass Index ; Child* ; Cholesterol ; Comorbidity* ; Diabetes Mellitus, Type 2 ; Dyslipidemias ; Fasting* ; Fatty Liver ; Humans ; Hypercholesterolemia ; Hyperinsulinism ; Hyperlipidemias ; Hypertension ; Hypertriglyceridemia ; Insulin Resistance ; Insulin* ; Linear Models ; Liver Function Tests ; Male ; Obesity ; Pediatric Obesity* ; Prevalence ; Research Personnel ; Triglycerides ; Ultrasonography