No abstract available.
Turner Syndrome*

No abstract available.
Child* ; Diabetes Mellitus* ; Humans

The McCune-Albright Syndrome is characterized by polyostotic fibrous dysplasia, cafe-au-lait colored patches of the skin and endocrinological abnormalities, including precocious puberty. Affected patients progress from GnRH-independent puberty to GnRH-dependent puberty. GnRH analogues are ineffective in GnRH-independent precocious puberty. Three year and 2 month old girl with breast development(SMR B3) and irregular vaginal bleeding were seen & diagnosed as incomplete sexual precocity. Decapeptyl treatment was started for the purpose of regression of breast development & vaginal bleeding with no effect. After 10 months, cafe-au-lait skin lesion & polyostotic fibrous dysplasia were noted and diagnosed as McCune-Albright syndrome. Breast development regressed to SMR B2 and vaginal bleeding was controlled with ketoconazole. As our experience, ketoconazole treatment might be effective to delay the progression of sexual development in patients with precocious puberty in McCune-Albright Syndrome.
Adolescent ; Breast ; Female ; Fibrous Dysplasia, Polyostotic* ; Gonadotropin-Releasing Hormone ; Humans ; Infant ; Ketoconazole* ; Puberty ; Puberty, Precocious ; Sexual Development ; Skin ; Triptorelin Pamoate ; Uterine Hemorrhage

PURPOSE: This study is designed to find out the clinical characteristics, growth status, and response to growth hormone treatment in children with organic growth hormone deficiency(GHD) after treatment of brain tumors. METHODS: Fifty-three children with organic GHD were evaluated for pituitary function, serum insulin-like growth factor-1(IGF-1), and insulin-like growth factor binding protein-3(IGFBP-3) concentrations. We also observed their growth status and corresponding change with or without growth hormone treatment. RESULTS: The causes of organic GHD were craniopharyngioma(47%), germinoma (19%), and medulloblastoma(17%), and 18 children(35%), diagnosed with brain tumors, presented with symptoms suggesting hormonal deficit. Initial height was -2.5+/-.2 SDS in craniopharyngioma, -1.7+/-.1 SDS in germinoma, and -2.1+/-.6 SDS in medulloblastoma, and children with craniopharyngioma showed the highest obesity rate, at 21.4+/-9.3%. After treatment for brain tumors, children with craniopharyngioma had the lowest values of peak GH, IGF-1, and IGFBP-3 concentrations, which were 1.1+/-.3 ng/mL, 74.1+/-6.6 ng/mL(-1.7+/-.2 SDS), and 1.9+/-.0 mg/L(-2.0+/-.1 SDS) respectively. The numbers of deficient hormones increased from 2.4+/-.1 to 3.2+/-.2 after treatment of brain tumors(P<0.05). Nine children showed normal or accelerated growth velocity(growth velocity 7.0+/-.8 cm/yr) without GH replacement and they had higher body mass index(BMI), IGF-1 concentrations, and IGFBP-3 SDS(P<0.05) compared to the others(growth velocity 1.9+/-.9 cm/yr). Height SDS increased every year during the first three years of GH treatment(P<0.05), 0.5+/-.4 SDS(n=20) for the first year, 0.4+/-.4 SDS(n=14) for the second, and 0.3+/-.5 SDS(n=11) for the third, and it increased by 1.1+/-.9 SDS(n=11) in total. CONCLUSION: The numbers of deficient pituitary hormones were increased after operation, irradiation, and/or chemotherapy. Children with GHD showed good response to GH replacement. Some children grew normally in spite of growth hormone deficiency, and their BMI, serum levels of IGF-1 and IGFBP-3 SDS were increased compared to those of the decreased growth group. This study suggests that further studies are needed to determine the mechanism of growth with low GH concentrations.
Brain Neoplasms* ; Brain* ; Child* ; Craniopharyngioma ; Drug Therapy ; Germinoma ; Growth Hormone* ; Humans ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Medulloblastoma ; Obesity ; Pituitary Hormones

PURPOSE: Adults with GH deficiency(GHD) have abnormal body composition, reduced physical performance, altered lipid metabolism, increased cardiovascular diseases, and reduced quality of life. Administration of GH to these patients reduce clinical abnormalities to normal ranges. Therefore, patients with childhood-onset GHD might need to continue GH replacement after the attainment of final height. Recently studies have shown that a high proportion of patients with childhood-onset GHD are no longer GHD when retested at young adult. METHODS: GH secretion was reevaluated with insulin and clonidine after completion of GH treatment in 29 young adult patients(21.3+/-2.8 yrs, 17 men, 12 women) with childhood-onset GHD diagnosed at a mean age of 11.4+/-3.5 yr. The mean duration of GH treatment was 3.7+/-3.0 yrs. Eleven(11 men) with idiopathic patients presented in 2(18%) isolated GHD and 9(82%) in multiple pituitary hormonal deficiencies. Eighteen(6 men, 12 women) with organic patients presented in 4(22%) isolated GHD and 14(88%) in multiple pituitary hormomal deficiencies, which was caused from craniopharyngioma, germinoma & other lesions. Blood sampling were done as usual method for checking LH, FSH and TSH concentration after injection of gonadotropin releasing hormone & thyrotropin releasing hormone. Serum cortisol levels were also checked after insulin injection and all hormonal concentrations were measured with radioimmunoassay method. Total cholesterol, high density lipoprotein (HDL)-cholesterol, low density lipoprotein(LDL)-cholesterol concentrations were measured by standard techniques. Bone density was measured in the level of lumbar spine and femur with DEXA. M-mode, two-dimensional and pulsed Doppler echocardiographic studies were performed. Quality of life was assessed from Beck depression inventory questionnaire with age-matched control. RESULTS: All patients with idiopathic and organic GHD were confirmed as GHD through combined pituitary function retesting at young adult. The additional pituitary hormonal deficiencies were increased in numbers. Their total cholesterol and triglyceride levels were increased especially in patients with organic GHD. There were no specific abnormal findings in echocardiographic findings compared to normal reference. Bone density with DEXA showed osteopenia(T score <-1) was found in 20/24(83%) and osteoporosis(T score <-2.5) in 8/24(33%) in young adult GHD. Quality of life was evaluated with BDI questionnaire and showed mild depression in 32% and moderate to severe depression in 11%. CONCLUSION: 82% of patients with idiopathic and 88% of organic GHD have additional pituitary hormonal deficiencies in childhood, showing multiple pituitary hormonal deficiencies rather than isolated GHD and has GHD permanently in all young adults with idiopathic and organic GHD and that is a little different findings from other foreign reports and needs to follow up in future.
Adult ; Body Composition ; Bone Density ; Cardiovascular Diseases ; Cholesterol ; Clonidine ; Craniopharyngioma ; Depression ; Echocardiography ; Femur ; Germinoma ; Gonadotropin-Releasing Hormone ; Humans ; Hydrocortisone ; Insulin ; Lipid Metabolism ; Lipoproteins ; Male ; Quality of Life ; Surveys and Questionnaires ; Radioimmunoassay ; Reference Values ; Spine ; Thyrotropin-Releasing Hormone ; Triglycerides ; Young Adult*

A case-control study was conducted to investigate the relationship between blood cadmium, blood zinc and cadmium/zinc ratio and hypertension. Eighty-three hypertensive and seventy-seven normotensive study subjects matched for age and sex were selected from the workers who had no history of job-related cadmium exposure, in Ulsan city and it's vincinity, Korea. The blood cadmium in hypertensive group was 2.90 etag/mL, which was significantly higher than that of control group, 1.99 etag/mL(P<0.01). After stratifing for smoking and age variables, the relationship was still remained. The blood cadmium/zinc ratio in hypertensive group was 2.46, which was significantly higher than that of control group, 1.65(P<0.01). After stratifing for smoking and age variables, the relationship was still remained. There was no significant difference in blood zinc between hypertensive and control group. On multiple logistic regression analysis, the blood cadmium/zinc ratio is highly significant than blood cadmium. In conclusion, there is the possible relationship between blood cadmium level which has been known to be within normal limits and hypertension. But, further cohort studies to define the effect of cadmium on human hypertension are required.
Blood Pressure* ; Cadmium* ; Case-Control Studies ; Cohort Studies ; Humans ; Hypertension ; Korea ; Logistic Models ; Smoke ; Smoking ; Ulsan ; Zinc*

PURPOSE: Growth hormone(GH) has not only growth promoting effect but also various metabolic effects. We evaluated GH effects by anthrometric data, biochmical data, electrolytes and simple CT in patients with Prader-Willi syndrome. METHODS: Nine children with Prader-Willi syndrome(PWS) were studied. The children were treated with GH(0.6U/kg/week) for 6 months. Before and after therapy we measured height, weight, waist, hip, and thigh. Blood sampling for eletrolytes, HgA1C, lipid profiles and other biochemistry were done in all patients before and after therapy. We also compared fat distribution with scan. RESULTS: Height standard deviation (SD) score increased from -0.7 to -0.5 and weight SD score decreased from 5.3 to 4.9. Body mass index(BMI) decreased from 28.2kg/m2 to 27.2kg/m2. But the changes in height, weight and BMI were not significant statistically. The waist/hip ratio decreased from 1.04 to 0.97(P<0.05), Thigh circumference had been decreased from 58.2+/-21.7cm to 49.9+/-6.9cm insignificantly. The visceral fat were decreased from 7,613+/-1,760 to 5,022+/-1,533 after GH therapy, and thigh muscle mass was increased from 6,358+/-1,616 to 7,175+/-2,155 (P<0.05). Total cholesterol and triglyceride decreased and HDL cholesterol increased after therapy although they were insignificant statistically. There were no differences in electrolytes, HgA1C, other biochemistry(Ca, P, protein, albumin, BUN, Cr) before and after therapy. CONCLUSION: In children with PWS, waist/hip ratio and fat mass were reduced and muscle mass was increased after GH therapy. There was tendency that total cholesterol and triglyceride decreased and HDL cholesterol increased after therapy. We confirmed that GH therapy had not only growth promoting effect but also metabolic effect on lipid and protein metabolism in children with PWS.
Biochemistry ; Child ; Cholesterol ; Cholesterol, HDL ; Electrolytes ; Growth Hormone* ; Hip ; Humans ; Intra-Abdominal Fat ; Metabolism ; Prader-Willi Syndrome* ; Thigh ; Triglycerides

Pupose:Recently childhood obesity is increasing and has been accompanied by an increase in the prevalence of type 2 diabetes mellitus(DM) among children and adolescents. We evaluated insulin resistance and impaired glucose tolerance(IGT) in 48 obese children and adolescents. METHODS:All subjects underwent a two-hour oral glucose tolerance test(1.75 g of glucose per kg of body weight) and then glucose and insulin levels were measured. Insulin resistance was estimated by homeostatic model assessment, and betacell function was estimated by calculating the ratio between the changes in the insulin level and glucose level during the first 30 minutes after the ingestion of glucose. RESULTS:IGT was detected in 4.2% of the 24 obese children(4-10 years of age) and 20.8% of the 24 obese adolescents(11 to 18 years of age). Type 2 DM was identified in 8.3% in children and 29.2% in adolescent obesity. Insulin levels were markedly elevated after the glucose tolerance test in subjects with IGT but a little decreased in cases with DM. Those changes of insulin levels were not significant statistically. The insulinogenic index was decreased in IGT and DM group of childhood and adolescent obesity compared to normal glucose tolerance(NGT) but it was not significant statistically. Insulin resistance, which was evaluated with fasting insulin, QUICKI and HOMA-IR, was increased in childhood obesity compared with adolescent obesity and the HOMA-IR of adolescent IGT group was significantly higher compared with NGT group(P<0.05). CONCLUSION: IGT is highly prevalent among children and adolescents with obesity. IGT was associated with insulin resistance and hyperinsulinemia, while beta-cell function was still relatively preserved. Overt type 2 diabetes was linked to insulin resistance and to beta cell failure.
Adolescent* ; Child* ; Eating ; Fasting ; Glucose Tolerance Test ; Glucose* ; Humans ; Hyperinsulinism ; Insulin Resistance* ; Insulin* ; Obesity* ; Pediatric Obesity ; Prevalence*

Pupose:Recently childhood obesity is increasing and has been accompanied by an increase in the prevalence of type 2 diabetes mellitus(DM) among children and adolescents. We evaluated insulin resistance and impaired glucose tolerance(IGT) in 48 obese children and adolescents. METHODS:All subjects underwent a two-hour oral glucose tolerance test(1.75 g of glucose per kg of body weight) and then glucose and insulin levels were measured. Insulin resistance was estimated by homeostatic model assessment, and betacell function was estimated by calculating the ratio between the changes in the insulin level and glucose level during the first 30 minutes after the ingestion of glucose. RESULTS:IGT was detected in 4.2% of the 24 obese children(4-10 years of age) and 20.8% of the 24 obese adolescents(11 to 18 years of age). Type 2 DM was identified in 8.3% in children and 29.2% in adolescent obesity. Insulin levels were markedly elevated after the glucose tolerance test in subjects with IGT but a little decreased in cases with DM. Those changes of insulin levels were not significant statistically. The insulinogenic index was decreased in IGT and DM group of childhood and adolescent obesity compared to normal glucose tolerance(NGT) but it was not significant statistically. Insulin resistance, which was evaluated with fasting insulin, QUICKI and HOMA-IR, was increased in childhood obesity compared with adolescent obesity and the HOMA-IR of adolescent IGT group was significantly higher compared with NGT group(P<0.05). CONCLUSION: IGT is highly prevalent among children and adolescents with obesity. IGT was associated with insulin resistance and hyperinsulinemia, while beta-cell function was still relatively preserved. Overt type 2 diabetes was linked to insulin resistance and to beta cell failure.
Adolescent* ; Child* ; Eating ; Fasting ; Glucose Tolerance Test ; Glucose* ; Humans ; Hyperinsulinism ; Insulin Resistance* ; Insulin* ; Obesity* ; Pediatric Obesity ; Prevalence*

No abstract available.
Humans ; Pseudohypoparathyroidism*