No abstract available.
Hypogonadism*

No abstract available.

No abstract available.
Humans ; Infant* ; Measles*

Seckel syndrome is a rare, autosomal recessive disorder of severe growth retardation and distinct craniofacial, orodental, and skeletal anomalies. We report hereby the first two Korean cases of typical Seckel syndrome who had characteristic symptoms of intrauterine growth retardation, small head, large eyes, sharp facial features (beaked nose, dysplastic ears and narrow face) with underdeveloped chin, dwarfism, severe mental retardation, and other malformation. We report two cases of Seckel syndrome with a brief review of related literatures.
Chin ; Dwarfism ; Ear ; Fetal Growth Retardation ; Head ; Intellectual Disability ; Nose

No abstract available.

No abstract available.
Tachycardia, Supraventricular*

The use of recombinant DNA technology to produce human growth hormone has resulted in a marked increase in availability of Growth Hormone(GH) to treat short stature due to GH deficiency and other conditions, such as Turner syndrome, familial short stature, chronic renal insufficiency and intrauterine growth retardation (IUGR). But, the GH therapy may result in the adverse events such as sodium and water retention, pseudotumor cerebri, slipped capital femoral epiphysis, growth of nevi, recurrence of tumor. We experienced a case of severe hypertension associated with GH therapy in a 14-year-old male who presented high blood pressure up to 190/100 mmHg and normalized at 2-3 weeks after discontinuation of GH. Therefore, we think that the blood pressure should be carefully monitored during GH therapy.
Adolescent ; Blood Pressure ; DNA, Recombinant ; Fetal Growth Retardation ; Growth Hormone* ; Human Growth Hormone ; Humans ; Hypertension* ; Male ; Nevus ; Noonan Syndrome ; Pseudotumor Cerebri ; Recurrence ; Renal Insufficiency, Chronic ; Slipped Capital Femoral Epiphyses ; Sodium

PURPOSE: The prevalence and severity of childhood obesity are increasing rapidly worldwide. Spontaneous and stimulated growth hormone (GH) secretion are impaired in obesity. However, despite the low GH levels, normal or increased insulin-like growth factor-I (IGF-I) levels have been observed in obese subjects. Growth velocity is commonly normal or increased in obese children. As for the possible mechanisms underlying these observations, overnutrition, chronic hyperinsulinemia and increased free IGF-I have been suggested. To explain the possible mechanisms by which obese children are taller than normal weight children, we have compared height, leptin, insulin, IGF-I and IGF binding protein-3 (IGFBP-3) with body mass index and studied the relationship among these parameters in obese and control group. METHODS: Auxological and endocrine evaluation were performed in 33 obese children (18 boys and 15 girls) and 47 non-obese children (24 boys and 23 girls) at Hanyang University Hospital from Jan. 1999 to Dec. 2000. Obesity was defined as a body mass index (BMI) greater than the 95th percentile for age and sex. Fasing blood samples were taken for the measurement of serum leptin, insulin, IGF-I and IGFBP-3 by radioimmunoassay. RESULTS: The serum concentrations of leptin, insulin, IGF-I and IGFBP-3 were significantly higher in obese children than those in non-obese children. The serum concentrations of leptin (r=0.751, P=0.000), insulin (r=0.746, P=0.000) and IGF-I (SDS) (r=0.747, P=0.000) showed positive correlation to BMI. And the serum concentrations of IGFBP-3 showed positive correlation to BMI with low correlation coefficient respectively (r=0.275, P=0.015). The serum concentration of insulin correlated to that of IGF-I (SDS) positively (r=0.585, P=0.000). CONCLUSION: This study suggest that increased sensitivity of GHR modulated by chronic hyperinsulinemia and increased circulating IGF-I produced by accumulated adipose tissue may enhance the growth in obese children.
Adipose Tissue ; Body Mass Index ; Child* ; Growth Hormone ; Humans ; Hyperinsulinism ; Insulin* ; Insulin-Like Growth Factor Binding Protein 3* ; Insulin-Like Growth Factor I* ; Leptin* ; Obesity ; Overnutrition ; Pediatric Obesity ; Prevalence ; Radioimmunoassay

No abstract available.
Child* ; Growth Hormone* ; Humans ; Insulin* ; Insulin-Like Growth Factor I*

Some sexual differentiation disorders are associated with gonadal neoplasia and increased incidence of testicular tumors has been discribed in the patients with, XY gonadal dysgenesis. The incidence of testicular tumors in infants and children are rare, representing only 1% of all pediatric solid tumors. In general, gonadal stromal tumors are one of the most characteristic endocrine tumors of the testis, endocrine activity occurs in at least 10-20%, among them Leydig cell tumors and Sertoli cell tumors are clinically important. Although the exact pathogenesis is unknown, endocrine activity due to estrogen secretion can be manifested clinically with gynecomastia or precocious puberty. We experienced and reported a child who visited for sexual precocity and had XY gonadal dysgenesis with Sertoli cell tumor.
Child ; Disorders of Sex Development ; Estrogens ; Gonadal Dysgenesis ; Gonads ; Gynecomastia ; Humans ; Incidence ; Infant ; Leydig Cell Tumor ; Male ; Puberty, Precocious ; Sertoli Cell Tumor* ; Testicular Neoplasms ; Testis