Journal Detail Information

1

Cite

Share

Pachydermoperiostosis in a 19 Year-old Boy Presenting as an Acromegaly-like Syndrome.

Jung Sub LIM ; Jun A LEE ; Dong Ho KIM ; Kyung Jin LIM ; Dae Geun JEUN

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):213-219.

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*

2

Cite

Share

A Case of Congenital Lipoid Adrenal Hyperplasia: Early Diagnosis by Using Computed Tomography.

Jung Hyun KWON ; Ji Young KIM ; Hae Soon KIM ; Sun Wha LEE ; Han Wook YOO

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):208-212.

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

3

Cite

Share

Two Cases of Transient Neonatal Diabetes Mellitus in Twin Brothers.

Jin Hyung CHO ; Eon Woo SHIN ; Seung YANG ; Eun Young PARK ; Kwang Nam KIM ; Dae Yeol LEE ; Phil Soo OH

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):204-207.

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

4

Cite

Share

A Case of Diabetic Nephropathy without Microalbuminuria in Type 1 Diabetes.

Na Young LEE ; Im Jeong CHOI ; Gil Hyun KIM ; Jin Hwa JUNG ; Sung Mi KIM ; Mi Young JEON

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):199-203.

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

5

Cite

Share

Prevalence and Clinical Features of Ketotic Hypoglycemia in Childhood.

Ji Young KIM ; Ji Sun SHIN ; Hae Soon KIM ; Seung Joo LEE

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):194-198.

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

6

Cite

Share

Fasting Insulin Level in Obese Children: Relationship to Comorbidities of Childhood Obesity.

Soo Young KIM ; Dong Han SHIN ; Hyung Jin KIM ; Baik Lin EUN ; Sang Hee PARK ; Sang Hoon CHA ; Kee Hyoung LEE

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):186-193.

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

7

Cite

Share

Plasma Ghrelin Levels and Its Relationship with Obesity in Obese Children.

Dae Sun JO ; Jae Un LEE ; Sun Young KIM ; Sun Jun KIM ; Chang Won KANG ; Pyoung Han HWANG ; Dae Yeol LEE

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):179-185.

Background:Ghrelin is a new endogenous ligand for the growth hormone secretagogue receptor. It activates the release of growth hormone from the pituitary and it also participates in the regualtion of energy homeostasis. The aims of the study were to characterize the changes in plasma ghrelin levels in obese subjects compared with lean control or overweight subjects, and their relationship to various parameters in obese subjects. METHODS:In this study, 121 elementary school children were divided into 3 groups according to their body mass index (BMI). The lean control subjects consisted of 28 children who had less than 85 percentile of BMI. The overweight subjects consisted of 22 children who had 85-95 percentile of BMI. The obese subjects consisted of 71 children who had over 95 percentile of BMI. All subjects in 3 groups were evaluated according to their age, height, weight, obesity index, plasma ghrelin, serum lipid, glucose and insulin levels. Leu72Met mutation of prepro-ghrelin gene was directly detected by digesting the PCR fragments with Bsrl. RESULTS:Among antropometric data, body weight, BMI and obesity index were higher in obesity and overweight subjects than those of lean control subjects (P<0.05). The plasma ghrelin levels were significantly lower in overweight and obese subjects (P<0.05). In addition, serum triglyceride and LDL cholesterol levels were significantly higher in these groups compared to the control subjects (P<0.05). The concentrations of plasma ghrelin were significantly negatively correlated with BMI, obesity index, serum triglyceride, LDL cholesterol and insulin in all the children. However, there was no significant relationship between plasma ghrelin levels and any various parameters in obese subjects. Leu72Met mutation was detected in about 30% of obese children. However, we could not find any differences between lean control and obese children. CONCLUSION: We proved a significantly lower plasma ghrelin levels in overweight and obese subjects. Further studies are now needed to establish the role of ghrelin in the pathogenesis of human obesity.
Body Mass Index ; Body Weight ; Child* ; Cholesterol, LDL ; Ghrelin* ; Glucose ; Growth Hormone ; Homeostasis ; Humans ; Insulin ; Obesity* ; Overweight ; Plasma* ; Polymerase Chain Reaction ; Receptors, Ghrelin ; Triglycerides

8

Cite

Share

Fatty Acids Composition in Breast Milk and Its Relationship with Infant Body Weight Gain.

Yu Sok HAN ; Mi Jung PARK ; Sung Han KIM ; Jang Hyuk AHN ; Hyun Sok JIN

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):173-178.

PURPOSE:Recent studies proposed that polyunsaturated fatty acids (PUFA) of the omega6 series (linoleic acid, LA) compared to the omega 3 series (linolenic acid, LNA) may be a potent promoters of adipogenesis during the gestation/lactation period. Increased ratio of LA/LNA may stimulate adipose tissue development during gestation/lactation period and may subsequently lead to chilhood obesity. The purpose of this study is to evaluate fatty acids composition of the breast milk in Korean lactating women and to analyze the relationship between LA/LNA and the body weight gain of the infants. METHODS:Fifty eight healthy postpartum lactating women and their healthy 58 breast feeding infants were studied. At 11.3+/-3.9 days of postpartum, breast milk were taken and analyzed by gas-liquid chromatography with one-step methylation method. RESULTS:The total fat contents in breast milk were 3.2+/-1.3%. The total fat contents in breast milk were not related to maternal BMI or Kaup index of infants. The percentage of PUFA was 24.9+/-6.0%. Among these PUFA, the percentage of LA and LNA was 20.3+/-5.8% and 2.2+/-0.8%, respectively. LA/ LNA ratio was 10.2+/-2.5. There were no significant correlations between LA/LNA ratio and infant weight gain during the first 2 months of life. CONCLUSION: Short term weight gain of infant was not related to the LA/LNA ratio in the breast milk. Further studies on the long term effects on fat percent and body weight of infants would be needed.
Adipogenesis ; Adipose Tissue ; alpha-Linolenic Acid ; Body Weight* ; Breast Feeding ; Breast* ; Chromatography, Gas ; Fatty Acids* ; Fatty Acids, Unsaturated ; Female ; Humans ; Infant* ; Linoleic Acid ; Methylation ; Milk, Human* ; Obesity ; Postpartum Period ; Weight Gain

9

Cite

Share

Clinical Manifestations of Chronic Autoimmune Thyroiditis in Children and Adolescents.

Ju Yeon HAM ; Hong Kuk KIM ; Jin Soon HWANG

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):168-172.

PURPOSE:Chronic autoimmune thyroiditis is the most common thyroid disease in children and adolescents, and also is the most common cause of hypothyroidism in children and adolescents. We studied the clinical manifestations and natural courses of chronic autoimmune thyroiditis in children and adolescents. METHODS:We analyzed retrospectively twenty one children and adolescents who were diagnosed as chronic autoimmune thyroiditis before 15 years of age. The diagnoses were made from July 1997 to February 2004 at Ajou Univesity Hospital. We reviewed the clinical manifestations and thyroid functions of these patients. RESULTS:Nineteen patients were female and two were male. The mean age at diagnosis was 9.95+/-3.1 years, the mean duration of follow up was 23.52+/-22.98 months. At diagnosis, the most common chief complaint was goiter (15 patients/21 patients) and the others were short stature, easy fatigue, cardiac murmur and weight loss. Eighteen patients had goiters at diagnosis. Four had familial history of thyroid disease, Three of them had maternal history of thyroid disease and one had paternal history of thyroid disease. At diagnosis, Seven patients were euthyroid and four patients were hyperthyroid, eighteen patients had antithyroglobulin antibody, seventeen patients had antimicrosomal antibody, fourteen patients had both of them. At last follow-up, eight patients were euthyroid and ten patients were hypothyroid, three patients were hyperthyroid. CONCLUSION: Chronic autoimmune thyroiditis in children and adolescents can have the various important influences on the metabolism including the growth. The clinical manifestations and thyroid functions should be monitored in children and adolescents with chronic autoimmune thyroiditis.
Adolescent* ; Child* ; Diagnosis ; Fatigue ; Female ; Follow-Up Studies ; Goiter ; Heart Murmurs ; Humans ; Hypothyroidism ; Male ; Metabolism ; Retrospective Studies ; Thyroid Diseases ; Thyroid Gland ; Thyroiditis, Autoimmune* ; Weight Loss

10

Cite

Share

Growth and Metabolic Consequences in Children Born with Small for Gestational Age at Term Gestation.

Shin Yun BYUN ; Jae Ho YOO ; Choong Ho SHIN ; Sei Won YANG

Journal of Korean Society of Pediatric Endocrinology.2004;9(2):159-167.

PURPOSE:The purpose of this study was to assess the growth status and to evaluate the factors affecting the development of metabolic syndrome in children born with small for gestational age (SGA) at term gestation. METHODS:We retrospectively reviewed medical records of 73 (36 boys, 37 girls) children born with SGA at term gestation who were visited for short stature and metabolic problems at Seoul National University Children' Hospital between 1994 and 2003. We assessed several clinical parameters as follows:birth weight, height standard deviation score (SDS), weight SDS, weight for height, bone age (BA), chronologic age (CA), predicted adult height (PAH) and midparental height (MPH). We evaluated the factors affecting height SDS according to pubertal status. We also evaluated the factors affecting development of type 2 diabetes mellitus, dyslipidemia or obesity. RESULTS:Pubertal children had greater height SDS, weight SDS and difference between BA and CA than prepubertal children, respectively (P<0.05). Height SDS positively correlated with difference between BA and CA (r=0.43, P<0.01), but BA advancement (BA>CA) was not observed in prepubertal children. BA advancement was observed in all pubertal children except one. The children who had greater weight SDS than height SDS had significantly lower height SDS and delayed BA, respectively (P<0.05). The children who had metabolic problems had higher weight SDS (P<0.05). CONCLUSION: This study suggests that rapid skeletal maturation may develope during transition from prepuberty to puberty and catch-up growth may contribute to the development of metabolic syndrome in children born with SGA, but further study will be required.
Adolescent ; Adult ; Child* ; Diabetes Mellitus, Type 2 ; Dyslipidemias ; Gestational Age* ; Humans ; Medical Records ; Obesity ; Pregnancy* ; Puberty ; Retrospective Studies ; Seoul

DISPLAY OPTIONS

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share