PURPOSE: Leptin is a hormone, encoded by ob gene in adipocytes and regulates food intake and energy expenditure. The serum concentration of leptin is known to be proportional to the amount of body fat but the regulation of leptin for growth and development in childhood is not clear. The purpose of this study is to evaluate the correlation of serum leptin levels to gender, pubertal development and sex steroid in children. METHODS: Serum leptin levels were measured by RIA in 145 Korean children of both sexes, age 8-16yr, and were compared according to body mass index, gender and pubertal stage. The correlations of leptin with testosterone and estradiol were also analized. RESULTS: A positive relationship was observed for leptin levels with body mass index(in male:r=0.63, P<0.001, in female:r=0.80, P<0.001). The mean leptin level of girls was higher than that of boys(7.50+/-.83ng/mL vs 4.11+/-.72ng/mL, P<0.05) in lean children but there was no significant difference in obese group. An analysis according to the pubertal development showed an increase of leptin level in girls while leptin decreased in boys at overt puberty. Serum leptin levels of boys in puberty correlated inversely with testosterone(r=-0.52, P<0.001) but leptin of girls did not have significant correlation with estradiol. CONCLUSION: Serum leptin level increases in girls and decreases in boys as the pubertal development proceeds. The relationships between leptin level and pubertal developement show gender difference and it might be explained in part by suppressive effects of androgen in male.
Adipocytes ; Adipose Tissue ; Adolescent ; Body Mass Index ; Child* ; Eating ; Energy Metabolism ; Estradiol ; Female ; Growth and Development ; Humans ; Leptin* ; Male ; Puberty ; Testosterone

PURPOSE: To evaluate leptin values in placental cord blood of newborns with normal intruterine growth after 30 weeks of gestation and to study the relationship of leptin with gestational age, birth size, ponderal index, BMI, placental weight, gender difference, Insulin-like Growth Factor-I(IGF-I) and Insulin-like Growth Factor binding protein-3(IGFBP-3). METHODS: Ninety healthy newborns(49 males and 41 females) with normal intrauterine growth were studied. They were classified into 4 groups according to the gestational age(GA):group I(30-33 weeks GA, n=17), group II(34-36 weeks GA, n= 24), group III(37-39 weeks GA, n=32) and group IV(40-42 weeks GA, n=17). At the time of delivery, newborn infant' weight, length, head circumference and placental weight were measured and BMI, ponderal index were calculated. A venous cord blood sample was collected at the time of delivery and serum leptin(RIA), IGF-I (IRMA) and IGFBP-3(IRMA) were measured. RESULTS: Cord blood leptin correlated positively with gestational age(r=0.730, P>0.01), body weight(r=0.686, P<0.01), length(r=0.419, P<0.01), head circumference(r= 0.498, P<0.01), BMI(r=0.750, P<0.01), ponderal index(r=0.704, P<0.01), placental weight(r=0.536, P<0.01), IGF-I(r=307, P<0.01) and IGFBP-3(r=0.736, P<0.01). Multiple linear regression analysis showed BMI, IGFBP-3 and sex(female) were independent predictors of leptin values. Leptin values increased progressively throughout gestation. Mean leptin values of group II(3.90+/-.28ng/mL) were significantly higher (P<0.01) than those of group I(1.92+/-.09ng/mL) and also values of group III(5.37+/-.52ng/mL) were higher than those of group II(P<0.05). But there was no significant difference between group III and group IV(6.45+/-.36ng/mL). Mean leptin values in females(5.07+/-.54ng/mL, n=41) were significantly higher than in males(4.07+/-.76 ng/mL, n=49) but there was no inter-gender difference in body weight, BMI, ponderal index, IGF-I, IGFBP-3. In addition, mean leptin values in females(7.45+/-.07 ng/mL) were significantly higher than in males(5.02+/-.09ng/mL) after 40 weeks of gestation. CONCLUSION: BMI, IGFBP-3 and sex(female) were independent predictors of leptin values. Leptin values increased significantly after 34 weeks of gestation and females had higher leptin values than males, especially in fullterm newborns. Leptin values in newborns had higher correlations with IGFBP-3 than with IGF-I as gestational age increased.
Body Weight ; Female ; Fetal Blood* ; Gestational Age ; Head ; Humans ; Infant, Newborn* ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Leptin* ; Linear Models ; Male ; Parturition ; Pregnancy*

PURPOSE: Exercise is frequently recommended in the management of diabetes mellitus(DM) and can improve blood control by increasing insulin sensitivity and psychological benefits. Exercise can result in good glycemic control in type 2 DM, when combined with diet and drug therapy. However, in type 1 DM, the expected improvement in glycemic control with exercise have not been clearly established. Effects of exercise on glycemic control in patients with type 1 DM were investigated. METHODS: 20 patients with of type 1 DM, who were no retinopathy, neuropathy, nephropathy and cardiac disorders, were enrolled and exercised for 30min. with 50% of individualized maximum VO2. Blood sugar concentration were measured before, immediate and 15min after exercise. The results were evaluated with HbA1c, C-peptide and DM duration. RESULTS: Blood sugar concentration were significantly decreased from pre-exercise 198+/-9.7mg/dL to immediate 145+/-7.1mg/dL and at 15min. Post-exercise 134+/-4.0mg/dL(P<0.05). In case with HbA1c<10%, there was significantly decreased in blood sugar level from pre-exercise 176+/-9.7mg/dL to immediate 123+/-63.2mg/dL and at 15min post-exercise 113+/-1.9mg/dL(P<0.05). In case with HbA1c>10% there was also significantly decreased in blood sugar levels 222+/-64.7mg/dL, 169+/-6.1mg/dL, 157+/-1.2mg/dL respectively(P<0.05). Group with moderate decreased blood sugar(40-99mg/dL) was 8 patients(42.1%), group with mild decreased blood sugar(<40mg/dL) was 7 patients(36.8%) and group with decreased more than 100mg/dL was 4 patients(21.5%). There was no correlation in degree of decreased blood sugar concentration among HbA1c, C-peptide, DM duration. CONCLUSION: Proper exercise in type 1 diabetic children can decrease blood sugar level significantly and make good glycemic control and can decrease DM microvascular complications in addition to motivating physically active lifestyle.
Blood Glucose ; C-Peptide ; Child ; Diabetes Mellitus ; Diabetes Mellitus, Type 1* ; Diet ; Drug Therapy ; Humans ; Insulin Resistance ; Life Style

PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Age of Onset ; Congenital Hyperinsulinism* ; Diabetes Mellitus ; Early Diagnosis ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Infant, Newborn ; Male ; Medical Records ; Pancreas ; Pancreatectomy* ; Seizures

PURPOSE: The reported clinical manifestations in Turner syndrome were different by karyotypes, countries and races. So, We evaluated the physical findings & medical problems in patient with Turner syndrome according to karyotype. METHODS: We examined prospectively 53 cases that diagnosed as Turner syndrome by chromosome study at the Pediatric Endocrine Clinic, YUMC from May 1999 to Aug 1999. According to the karyotype, they were divided into 3 groups:45, X(22 cases, 41.5%), mosaicism(18 cases, 34%) and structural aberration group(13 cases, 24.5%), and 53 cases were reevaluated by prepared protocol of 22 abnormal physical findings and 4 medical problems. Chi-square test was used for statistic analysis. RESULTS: 1)In case of 45, X group, they had short stature(100%), cubitus valgus(95.5%), widely spaced nipples(86.4%), shield chest(72.7%), low posterior hair line(68.2%), in case of mosaicism group, they had short stature(100%), cubitus valgus(72.2%), high arched palate(66.7%), widely spaced nipples(61.1%), short neck(61.1%) and in case of structural aberration group, they had short stature(100%), high arched palate (92.3%), widely spaced nipples(92.3%), low posterior hair line(76.9%), shield chest (76.9%), in frequency order. Short stature was the most common finding in Turner syndrome. The incidence of high arched palate was significantly higher in structural aberration group(P<0.05). 2)In case of 45, X group, they had otitis media(63.6%), thyroid problem(4.5%) and no renal problem and cardiac problem, in case of mosaicism group, they had otitis media(38.9%), thyroid problem(5.6%) and no renal problem and cardiac porblem and in case of structural aberration group, they had otitis media(61.5%), thyroid problem(15.4%), renal problem(7.7%) and cardiac problem(7.7%) in frequency order, they had no statistical difference in among three groups. CONCLUSION: In case of 45, X group, the incidence of cubitus valgus, webbed neck were more common findings and of high arched palate, micrognathia were less common findings compared to other group. In case of mosaicism group, the incidence of micrognathia, epicanthal fold were more common findings and wide spaced nipples, antimongoloid fissure were less common findings compared to other group. In case of structural aberration group, the incidence of high arched palate is most common finding among 3 group(P<0.05) and pigmented nevi and epicanthal fold were less common findings compared to other group. The incidence of medical problems such as otitis media and thyroid problem were than other foreign country reports.
Continental Population Groups ; Hair ; Humans ; Incidence ; Karyotype* ; Mosaicism ; Neck ; Nevus, Pigmented ; Nipples ; Otitis ; Otitis Media ; Palate ; Prospective Studies ; Thorax ; Thyroid Gland ; Turner Syndrome*

PURPOSE: The growth hormone receptor(GHR) is essential for the actions of growth hormone on postnatal growth and metabolism. GHR transcripts are characterized by the presence of disparate 5'untranslated exons. In contrast to L1 transcript, factors regulating the expression of the GC rich L2 transcript have remained unidentified. The purpose of this study is in order to characterize the mechanisms regulating expression of the L2 transcript in the murine GHR gene METHODS: Transient transfection experiments including deletional analysis and co-transfection assay were performed to find a region containing promoter activity in the L2 5'flanking sequence using BNCL2(mouse liver) cells, CV-1(African green monkey kidney) cells, HRP.1 trophoblasts and Drosophila Schneider(SL2) cells. Sequencing analysis was performed to find the region contained consensus binding sites for transcription factors. Standard gel shift(Electrophoretic mobility shift assay, EMSA) and supershift analysis using liver nuclear extracts was performed to establish proteins(transcription factors) bound this regulatory element. RESULTS: The 5'flanking region of the L2 untranslated region(UTR) exhibited promoter activity in BNCL2(mouse liver), CV-1(monkey kidney) cells and HRP.1 trophoblasts. Deletional analyses indicated the presence of a Sp binding site important for transcription of the L2 UTR and localized the major regulatory region within 75 bp of the 5'transcription start site. Sequencing analyses revealed the region contained consensus binding sites for the Sp family of transcription factors. EMSA and supershift EMSA revealed that in mouse liver nuclear extracts that Spl and Sp3 bound to this cis-element. Functional studies in Drosophila SL2 cells and BNCL2(mouse liver) cells established the ability of Sp3 and Sp1 to stimulate transcriptional activity via this cis-element. Functional studies in Drosophila SL2 cells demonstrated a functional interaction between Sp3 and Sp1 at this DNA-binding site. CONCLUSION: Sp family transcription factors play a role in regulation of L2 transcript gene expression in the 5'flanking region of the murine GHR gene.
Animals ; Binding Sites ; Cercopithecus aethiops ; Consensus ; Drosophila ; Electrophoretic Mobility Shift Assay ; Exons ; Gene Expression ; Growth Hormone* ; Humans ; Liver ; Metabolism ; Mice ; Receptors, Somatotropin* ; Regulatory Sequences, Nucleic Acid ; Transcription Factors* ; Transfection ; Trophoblasts

Thyroid cancer is not only an infrequent cancer, but also is the most common malignancy of the endocrine system. When thyroid cancer occurs in childhood and adolescence, it is an unusual but interesting malignancy. Thyroid cancer in childhood and adolescence has a slightly lower preference for female than for male subjects, as compared to adult patients. With regard to the pathogenesis the only factor definitely known to promote development of thyroid cancer in the pediatric age group is irradiation. The evidence demonstrates that thyroid carcinoma in the pediatric age group is a biologically self-reliant and more hostile nature than in adults but paradoxically the prognosis is good. Enhanced survival resulting from advances in therapy in patients with Hodgkin's disease is associated with long-term morbidity, including the potential for the development of a second solid malignancy. Second malignant neoplasms(SMNs) have become a principal consideration in evaluating the prolonged consequences of strategy in pediatric oncology. Thyroid carcinoma has proven to be a common SMN. We have experienced 2 cases of papillary thyroid cancer following radiation therapy for Hodgkin's lymphoma and a papillary thyroid cancer with pulmonary metastases.
Adolescent* ; Adult ; Endocrine System ; Female ; Hodgkin Disease ; Humans ; Male ; Neoplasm Metastasis ; Prognosis ; Thyroid Neoplasms*

Small ovarian cysts(<0.7 cm in diameter) are common in prepubertal children. Occasionally larger cysts occur in young girls with isosexual incomplete precocious puberty in the absence of LH and FSH elevation. The mechanism of production of these autonomous functioning cysts is unknown. A 3 year 6 month-old girl was referred to our department of pediatrics because of severe vaginal bleeding. On examination, she was tall and had breast enlargement of Tanner stage II. On hormonal study, FSH(0.38mIU/ml) was suppressed and estradiol(62.2pg/ml) was markedly elevated, and GnRH stimulation failed to evoke an increase in gonadotropin. Bone age was advanced. Pelvic ultrasonography showed both huge ovarian cysts. Brain MRI showed no abnormalities. We underwent both ovarian cystectomy and histological examination showed follicular cyst in left ovary and luteal cyst in right ovary. After ovarian cystectomy vaginal bleeding stopped and hormonal abnormalities were normalized. We report a case of autonomous ovarian cysts presenting severe vaginal bleeding treated with ovarian cystectomy.
Brain ; Breast ; Child ; Cystectomy ; Female ; Follicular Cyst ; Gonadotropin-Releasing Hormone ; Gonadotropins ; Humans ; Infant ; Magnetic Resonance Imaging ; Ovarian Cysts* ; Ovary ; Pediatrics ; Puberty, Precocious ; Ultrasonography ; Uterine Hemorrhage*

PURPOSE: Prader-Willi Syndrome(PWS) is caused by absence of paternal contributions of the chromosome region 15q11-q13. To detact this region, high resolutional cytogenetic analysis, FISH with probe at PWS critical region or microsatellite polymorphism can be used. The gene for the small nuclear ribonucleoprotein polypeptide N(SNRPN) is not expressed in patients with PWS. We conducted molecular analysis with RT-PCR with SNRPN primers to find out more useful diagnostic tool in PWS. METHODS: Four patients with obesity and other characteristics of PWS were studied. The exprssion of SNRPN and control gene were studed by RT-PCR from peripheral lymphocytes. RESULTS :The SNRPN expression in reverse transcribed RNA from blood were easily detected in normal control but not in patients with suspected Parder-Willi Syndrome. CONCLUSION: We conclude that SNRPN expression study is a useful diagnostic method for detection of Prader-Willi Syndrome.
Cytogenetic Analysis ; Humans ; Lymphocytes ; Microsatellite Repeats ; Obesity ; Pathology, Molecular* ; Prader-Willi Syndrome* ; Ribonucleoproteins, Small Nuclear ; RNA ; snRNP Core Proteins*

PURPOSE: Thyroid disease is the most common endocrine disease in childhood. Thyroid hormone has critical effects on growth and development, especially in childhood. We survey the prevalence, sex and age distribution, symptoms and thyroid function states of thyroid diseases in childhood. METHODS: Three hundred ninety one children who were diagnosed as having thyroid disease at department of pediatrics, St. Mary's hospital from Jan. 1987 to Dec. 1998 enrolled in this study. RESULTS: 1) The ratio between male and female patients was 1: 5.3. Age distribution was puberty, school age and infancy in their order of frequency. 2) The results of thyroid function tests showed normal function in 210 cases (53.7%), increased function in 95 cases(24.3%), and decreased function in 86 cases (22%). 3) Simple goiter, Graves disease, and chronic lymphocytic thyroiditis were the most common diseases in euthyroid, hyperthyroid, and acquired hypothyroid state, respectively. 4) The most common sign and symptom was goiter in euthyroid(100%), hyperthyroid(98.8%) and acquired hypothyroid state(96.2%). Forty of 68 cases(58.8%) with congenital hypothyroidism were detected by neonatal screening. CONCLUSION: Simple goiter, chronic lymphocytic thyroiditis and Graves disease were common acquired thyroid diseases in childhood, and goiter is the most common clinical manifestation in acquired thyroid disease. Recently, increasing number of congenital hypothyroidism was detected by neonatal screening test.
Adolescent ; Age Distribution ; Child ; Congenital Hypothyroidism ; Endocrine System Diseases ; Female ; Goiter ; Graves Disease ; Growth and Development ; Hashimoto Disease ; Humans ; Infant, Newborn ; Male ; Neonatal Screening ; Pediatrics ; Prevalence ; Puberty ; Thyroid Diseases* ; Thyroid Function Tests ; Thyroid Gland*