Multicystic ovary was first described by Silver in 1958 as a rare case feature of acquired primary hypothyroidism. In 1980, Lindsay reported four girls with hypothyroidism associated with multicystic ovary diagnosed by pelvic ultrasonography. The mechanism of ovarian cyst formation and pseudoprecocious puberty in severe hypothyroidism in childhood are unknown. Increased ovarian sensitivity to GnRH and increased TSH level which acts on FSH receptor is one of the mechanism explained. We report a case of a girl with primary hypothyroidism presented with pseudoprecocious puberty and giant follicular ovarian cyst with torsion.
Adolescent ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Hypothyroidism* ; Ovarian Cysts* ; Ovary ; Puberty ; Receptors, FSH ; Silver ; Ultrasonography

We experienced two cases of congenital adrenal hyperplasia(CAH), complicated by true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis in a boy and a girl. The boy was diagnosed as CAH at 8 years of age, when he showed pseudoprecocity with adrenal rest tumor. The girl was diagnosed at neonate, when she showed ambiguous genitalia and salt-losing symptom. They developed precocious puberty during steroid treatment. Clinically they showed precocity, advanced bone age, and decreased predicted adult height, even though they showed partial gonadotrophin response to luteinizing hormone releasing hormone (LHRH) stimulation test. They were treated with LHRH analogue(leuprolide depot) resulting no further progression of precocity.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor ; Adult ; Axis, Cervical Vertebra ; Disorders of Sex Development ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Infant, Newborn ; Male ; Puberty, Precocious*

PURPOSE:The prevalence of childhood obesity has been increased rapidly, associated with high morbidity of dyslipidemia, cardiovascular risk factors and insulin resistance/hyperinsulinemia. Hyperinsulinemia in obese children may depend on either increased insulin secretion or decreased insulin clearance. We investigate whether insulin secretion and clearance are different between obese and normal weight children. METHODS:The body mass index(BMI), fasting serum glucose, insulin and C-peptide levels were measured in 101 children(52 obese children and 49 non-obese controls) in Ansan area, from 6 to 14 years old. Fasting serum C-peptide/insulin ratio, insulin/glucose ratio, homeostasis model assessment(HOMA) were calculated and compared between obese and control group. RESULTS:The fasting serum insulin and C-peptide levels were significantly higher in obese group than in controls. The C-peptide/insulin ratio was significantly lower and insulin/glucose ratio was significantly higher in obese group than in controls. There were positive correlations between BMI and fasting serum insulin level, fasting insulin/glucose ratio and HOMA. The prevalence of hyperinsulinemia is higher in obese adolescents than in controls. CONCLUSION: Our findings suggest that increased insulin secretion and decreased insulin clearance may contribute to hyperinsulinemia in obese children.
Adolescent ; Blood Glucose* ; C-Peptide* ; Child* ; Dyslipidemias ; Fasting* ; Gyeonggi-do ; Homeostasis ; Humans ; Hyperinsulinism ; Insulin* ; Obesity* ; Pediatric Obesity ; Prevalence ; Risk Factors

PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

PURPOSE:Insulin-like growth factor-I(IGF-I) is an essential anabolic factor for postnatal rat brain development and IGF-I expression is highly abundant during the first 21 days, critical growth period. Hypoxic-ischemic brain insults occurring during the perinatal period result in neuronal necrosis and permanent brain damage. To investigate the regulation of the action of IGF-I in response to such a hypoxic insult, we examined the gene expression of IGF-I and IGFBP-5 during the first 72 hr after hypoxic-ischemic injury in immature rat brain. METHODS:Ligation of the right carotid artery of 7-day-old rats was followed by 2 hour exposure to 8% oxygen to produce severe hypoxic brain damage. Using reverse transcriptase-polymerase chain reaction(RT-PCR), the expression of IGF-I mRNA and IGFBP-5 mRNA was determined in both hypoxic and control brains at post 1, 4, 12, 24, 48 hr and 72 hr after hypoxic-ischemic insult. RESULTS:The IGF-I mRNA and IGFBP-5 mRNA expression of hypoxic brain were not different from those of controls at 1 hr of recovery but IGF-I mRNA expression was decreased rapidly at post 4 hr, this decrease more pronounced at 12 hr of recovery. IGF-I mRNA and IGFBP-5 mRNA expression were increased at 48 hr and 24 hr of recovery, respectively and both IGF-I mRNA and IGFBP-5 mRNA expression showed similar level of controls at 72 hr of recovery. CONCLUSION: Out findings suggest that IGF-I play a important role in both neuronal loss and repair process following hypoxic-ischemic brain injury and IGFBP-5 is also strongly involved in the repair of damaged brain tissue by mediating IGF-I action. (J Korean Soc Pediatr Endocrinol 2003;8:56-63)
Animals ; Brain Injuries* ; Brain* ; Carotid Arteries ; Gene Expression ; Hypoxia, Brain ; Insulin-Like Growth Factor Binding Protein 5 ; Insulin-Like Growth Factor I ; Necrosis ; Negotiating ; Neurons ; Oxygen ; Rats* ; RNA, Messenger*

PURPOSE:Cardiovascular morbidity has recently been demonstrated to potentially reduce life expectancy in growth hormone deficiency(GHD). The aim of this study was to evaluate cardiovascular abnormalities and atherosclerotic changes in adults with childhood-onset GHD in whom GH treatment had been stopped at the achievement of final height. METHODS:Nine patients with childhood-onset GHD(7 idiopathic and 2 organic), with an age of 24.0+/-.0 year, were studied. Clinical characteristics of subjects were determined and blood pressure, body mass index(BMI), and serum concentrations of lipids were measured. Structural and functional evaluation of cardiovascular system was performed by M-mode echocardiography and linear phase array imaging transducer. RESULTS:BMI of patients was 27.3+/-.7 mg/m2, and four patients(44%) were overweight(BMI 25-30 mg/m2), but none was obese(BMI >30 mg/m2). The percentage of patients who had total cholesterol > or = 200 mg/dL, triglyceride > or = 150 mg/dL, LDL cholesterol > or = 140 mg/dL, and HDL cholesterol < or = 40 mg/dL were 56%, 44%, 33 %, and 44%, respectively. Interventricular septum thickness(IVST), left ventricular posterior wall thickness(LVPWT), left ventricular end-diastolic internal diameter (LVID), left ventricular mass index(LVMI) of patients were 6.4+/-2.1 mm(SDS -1.5+/-1.1), 6.3+/-1.2 mm(SDS -2.1+/-0.8), 44.9+/-4.3 mm(SDS -1.0+/-0.9), and 85.4+/-94.8 g/m2 (SDS -0.5+/-6.8), respectively. The number of patients whose IVST, LVPWT, LVID, and LVMI were decreased(<-2SD) were 4(44%), 5(56%), 1(11%), and 6(67%), respectively. Carotid artery intima-media thickness(IMT) was 0.86+/-0.22 mm, and it was increased(>2SD) in 3 patients(33%). Three out of four patients with IVST lower than -2SD had increased carotid artery IMT, whereas none of five patients with IVST higher than -2SD had increased carotid artery IMT. There were no differences in echocardiographic findings between groups according to sex, age, duration of disease, duration after GH discontinuation, BMI, and severity of dyslipidemia. CONCLUSION: Decreases in IVST, LVPWT, and LVMI, and an increase in carotid artery IMT were observed in a significant number of patients with childhood-onset GHD. These findings support the need of GH replacement after completion of growth and careful evaluation of cardiovascular changes in patients with childhood-onset GHD.
Adult* ; Blood Pressure ; Cardiovascular Abnormalities* ; Cardiovascular System ; Carotid Arteries ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Dyslipidemias ; Echocardiography ; Growth Hormone* ; Humans ; Life Expectancy ; Transducers ; Triglycerides

PURPOSE:Craniopharyngioma is one of the most common causes of organic growth hormone deficiency leading to pituitary hormonal insufficiency. However, some growth hormone(GH)-deficient children with craniopharyngioma may grow normally or even show accelerated growth. This study was designed to evaluate several factors associated with growth of patients with craniopharyngioma. METHODS:Forty children operated on for craniopharyngioma were evaluated for their pituitary function, serum insulin like growth factor-I(IGF-I), serum insulin like growth factor binding protein-3(IGFBP-3) and serum prolactin levels. We also observed their growth status and corresponding changes with or without GH treatment. RESULTS:Among 40 patients, one had normal pituitary hormonal status and one had isolated GHD(GH deficiency). The other patients showed multiple pituitary hormone deficiency including GH(98%), LH, FSH(75%), TSH(65%), ACTH(62%), and ADH(38%) deficiencies. Patients with GHD were categorized into 2 groups. Group 1 consisted of children who showed normal growth, thus had not received GH treatment(n=14) and Group 2, those who showed subnormal growth(n=25). Patients in Group 2 were subdivided into Group 2A, when the patients had not received GH treatment in spite of subnormal growth(n=9) and Group 2B, when GH treatment had been added later on(n=16). There were no differences in the age at diagnosis of GHD, initial height standard deviation score(Ht SDS), body mass index(BMI), peak GH concentration between Group 1 and Group 2. Height velocities in Group 1, 2A, and 2B were 8.1+/-.2 cm/yr, 2.4+/-.2 cm/yr, 2.7+/-.2 cm/yr during the first year of endocrinologic follow-up, 7.1+/-.8 cm/yr, 1.2+/-.1 cm/yr, 7.6+/-.7 cm/yr during the second year, 5.9+/-.0 cm/yr, 2.8+/-.9 cm/yr, 7.3+/-.7 cm/yr during the third year, respectively. BMI changes during the first year of endocrinologic follow-up and postoperative prolactin levels were not significantly different between Group 1 and Group 2A. Postoperative IGF-I and IGFBP-3 levels in Group 1 were significantly higher than those in Group 2A(P<0.05). Both IGFBP-3 and prolactin levels correlated significantly with height velocity in Group 1 and 2A(P=0.004 r=0.64 and P= 0.035 r=0.74 , respectively). CONCLUSION: In this study, growth in children with craniopharyngioma was likely to be associated with IGF-I, IGFBP-3 and prolactin levels. Further studies are needed to unravel other growth promoting factors related to GH independent growth.
Child* ; Craniopharyngioma* ; Diagnosis ; Follow-Up Studies ; Growth Hormone ; Humans ; Insulin ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Prolactin

PURPOSE:Improved adult final height(FH) is a major goal in the treatment of children with short stature due to growth hormone deficiency(GHD). The purpose of this study was to evaluate final height in idiopathic and organic GHD children after long-term growth hormone(GH) treatment. METHODS:Twenty five(16 males and 9 females) patients with GHD(14 idiopathic and 11 organic GHD) were included. GHD was diagnosed by two or more GH provocation tests(peak GH level <10 ng/mL). All subjects had multiple pituitary hormone deficiencies, and aged 10.7+/-.5(5.5-14) years at the start of GH treatment. The patients were treated with GH 0.45-0.7 IU/kg/week in 3-7 divided doses for 6.9+/-.8(5.2-10) years. Treatment was ended when growth velocity reached lower than 2 cm/year and/or bone age reached 16 years. Standard auxologic measurements were performed at the start of GH treatment and at every 6 month after initial GH treatment. RESULTS:FH was 166.9+/-.8 cm, which was not significantly lower than target height(167.1+/-.9 cm) and predicted adult height(169.1+/-5 cm). FH SDS was significantly improved to -0.8+/-.5 compared with -3.4+/-.0 of height SDS at the start of GH treatment. The largest height increment was observed in the first year of GH treatment, with a gradual decrease in the following years. There was no difference in FH and FH SDS between idiopathic and organic GHD. Unwanted serious adverse events were not observed in all patients during GH therapy. CONCLUSION: Early diagnosis and continuous treatment with optimal doses of GH to near adult height improve the outcome in children with short stature due to idiopathic and organic GHD.
Adult ; Child* ; Early Diagnosis ; Growth Hormone* ; Humans ; Male

No abstract available.
Adrenal Hyperplasia, Congenital*

No abstract available.
Diagnosis* ; Thyroid Gland* ; Thyroid Nodule*