No abstract available.
Turner Syndrome*

No abstract available.
Thyroid Gland* ; Thyroid Nodule*

PURPOSE:The clinical course in adult autoimmune thyroiditis varies: both transient and intermittent hypothyroidism occur during the course of disease and spontaneous recoveries from the hypothyroid state has been described. To determine whether these kinds of variations occur in the course of juvenile chronic lymphocytic thyroiditis and whether the size of thyroid gland, or immunologic measurement such as thyroid antibodies correlated with the course or outcome of juvenile chronic lymphocytic thyroiditis. METHODS:Fifty six patients (54 girls) who attended the clinic between 1986 and 1996 were followed during 36 months prospectively. The clinical diagnosis of juvenile chronic lymphocytic thyroiditis was confirmed by Fisher et al.(1975) criteria in all patients. Assessment of thyroid function was based on clinical diagnosis and on the levels of thyroid stimulating hormone and free thyroxine. Physical examination, thyroid function test and antithyroid antibody test were evaluated with 6 months intervals. RESULTS:Initially, 27 patients were euthyroid, 19 compensatory hypothyroidism and 10 overt hypothyroidism. At the end of follow up, 31 patients were euthyroid, 11 compensatory hypothyroidism and 14 overt hypothyroidism. On one or more occasions, 92.7% of the patients had positive results for thyroid antibodies. Overt hypothyroidism at the end of follow up correlated with the initial thyroid states and with detection of thyroglobulin antibodies. CONCLUSIONS:The best predictors of the final hypothyroid state were initial hypothyroidism and detection of thyroglobulin antibodies.
Adult ; Antibodies ; Diagnosis ; Follow-Up Studies ; Hashimoto Disease* ; Humans ; Hypothyroidism ; Physical Examination ; Prospective Studies ; Thyroglobulin ; Thyroid Function Tests ; Thyroid Gland ; Thyroiditis, Autoimmune ; Thyrotropin ; Thyroxine

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*

No abstract available.
Child* ; Fetal Growth Retardation* ; Growth Hormone* ; Humans

No abstract available.
Humans ; Infant ; Infant Mortality*

Prader-Willi(PW)syndrome is characterized by obesity, hypotonia, mental retardation, hypogonadism, short stature, excessive eating and characteristic facial appearance. Diabetes mellitus has been considered a component of PW syndrome. Recently this syndrome is caused by the absence of paternally derived genes normally located on chromosome segment 15 q11-q13 or may be the result of maternal uniparental disomy with the absence of paternally derived 15 q11-q13 region. The developement of probes containing segments of DNA from chromosome region 15 q11-q13 provides the oppotunity to confirm the diagnosis of PW syndrome by fluorescence in situ hybridization(FISH). We experienced a 15-year-old boy of PW syndrome with diabetes mellitus, who revealed mental retardation, hypogonadism, obesity and microdeletion of chromosome 15 q11-q13 comfirmed by FISH.
Adolescent ; Chromosomes, Human, Pair 15* ; Diabetes Mellitus ; Diagnosis ; DNA ; Eating ; Fluorescence ; Humans ; Hypogonadism ; Intellectual Disability ; Male ; Muscle Hypotonia ; Obesity ; Prader-Willi Syndrome* ; Uniparental Disomy

Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.
Adrenalectomy ; Adrenocortical Carcinoma* ; Adult ; Child ; Cushing Syndrome ; Feminization ; Hair ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Rare Diseases ; Virilism

PURPOSE:Differential diagnosis of sexual precocity has been aided by new imaging techniques and endocrine evaluation. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS:A total 95 children(87 girls and 8 boys) who were diagnosed with sexual precocity at St. Mary's Hospital, Catholic University Medical College from 1984 to 1996, were studied. All underwent standard anthropometric measures. They were assinged to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: 1) The most common cause of sexual precocity was premature thelarche, 50 cases(52.6%) of all children. 2) Central precocious puberty was noted in 7 girls and 3 boys(female to male ratio 2.3:1). The causes were not identified in 6 cases(85.7%) in girls, but the causes were not found in 1 cases(33.3%) in boys. The idiopathic central precocious puberty occurs at least two fold more frequently in girls than in boys. 3) Incomplete precocious puberty was noted in 29 girls and 5 boys(female to male ratio 5.8:1). In the causes of 29 girls with incomplete precocious puberty, congenital adrenal hyperplasia were found in 23 cases, autonomous functional ovarian cyst in 5 cases and McCune-Albright syndrome in 1 case. In the causes of 5 boys with incomplete precocious puberty, virilizing adrenal cortical tumor were found in 3 cases, congenital adrenal hyperplasia in 1 case, and hepatoblastoma in 1 case. CONCLUSION: Sexual precocity occurs at least ten fold more frequently in girls than in boys. Premature thelarche is the most common form of sexual precocity.
Adrenal Hyperplasia, Congenital ; Child ; Diagnosis ; Female ; Fibrous Dysplasia, Polyostotic ; Hepatoblastoma ; Humans ; Incidence* ; Male ; Ovarian Cysts ; Puberty, Precocious ; Ultrasonography

PURPOSE: The need for continuing Growth Hormone(GH) replacement after adolescence in patients with childhood-onset GH deficiency has been recognized. The purpose of this study was to evaluate the abnormalities of lipid profiles in young adults with childhood-onset hypopituitarism who discontinued GH therapy after the completion of height growth. METHODS: Nine male patients(mean age:22.4+/-3.3 years) with childhood-onset hypopituiatarism in whom GH treatment had been discontinued after final height was achieved were included. Their body mass index(BMI) and serum levels of total cholesterol, triglyceride(TG), high-density lipoprotein(HDL) cholesterol, and low-density lipoprotein(LDL) cholesterol were measured. The relationships of duration after GH discontinuation, age, and BMI to lipid profiles were anaylzed. RESULTS: BMI increased significantly from 21.8+/-1.9 kg/m2 before GH discontinuation to 23.0+/-3.0 kg/m2 after GH discontinuation(P<0.05). Serum levels of total cholesterol, TG, HDL cholesterol, and LDL cholesterol were 217.3+/-33.4, 237.8+/-128.2, 42.1+/-7.1, and 127.4+/-27.2 mg/dL, respectively, 3.1+/-2.0 years after GH discontinuation. Percentage of patients who had total cholesterol > or = 200 mg/dL, TG > or = 150 mg/ dL, LDL cholesterol > or = 140 mg/dL, and HDL cholesterol < or = 40 mg/dL were 77.8%, 88.9%, 44.4%, and 33.3%, respectively. All subjects had some abnormalities of lipid profiles. A significant positive correlation was found between duration after GH discontinuation and serum levels of total cholesterol and TG(r=0.84, P<0.01; r=0.83, P<0.01). A significant positive correlation was also found between age and serum levels of total cholesterol and TG(r=0.86, P<0.01; r=0.81, P<0.01). There were no correlations between BMI and serum lipid levels. CONCLUSION: Most of young adult patients with childhood-onset hypopituitarism had abnormal lipid profiles by 1-5 years after discontinuation of GH treatment. These data suggest that continuous GH treatment after completion of height growth is necessary.
Adolescent ; Adult* ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Growth Hormone* ; Humans ; Hypopituitarism* ; Male ; Young Adult