No abstract available.

No abstract available.
Hypogonadism*

No abstract available.
Humans ; Infant* ; Measles*

No abstract available.
Tachycardia, Supraventricular*

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.

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

BACKGROUND: Urticaria pigmentosa(UP) is primarily a disease of children. There have been no clinical studies of UP in Korea. OBJECTIVE: This study was performed to find the clinical characteristics of UP in Korean child-hood patients. METHOD: Twenty-nine cases of urticaria pigmentosa confirmed clinically and histopathologically were analyzed. RESULTS: The maculopapular type was the most common with the usual age of onset before the age of 6 months, and the lesions tended to be distributed in the central portion of the body. Darier sign was positive in 92% of the patients(24/26). No systemic involvements were detected in any of the patients with minimal associated symptoms. Seven patients of the maculopapular type and one patient of the multiple nodular type followed up for more than 2 years showed a tendency to improve or clear by the age of 6 years. CONCLUSION: Neonatal or infantile-onset patients of UP in Korean pediatric population were considered to have a benign clinical course and to require no aggressive therapy.
Age of Onset ; Child* ; Clinical Study* ; Humans ; Korea ; Methods ; Urticaria Pigmentosa* ; Urticaria*

In air leak syndrome, a significant portion of the volume delivered during a positive pressure breath can be lost through the leak. HFOV can achieve adequate ventilation at lower peak and/or mean intrapulmonary pressure than conventional mechanical ventilation (CMV) and has been an effective treatment of already established air leak syndrome. We report a 1-day-old male infant with severe respiratory failure from pneumothorax and pneumomediastinum, who was refractory to CMV with chest tube drainage. HFOV was applied to this patient for 114 hours, and improvement of oxygenation and ventilation as well as significant reduction of pneumothorax followed.
Chest Tubes ; Drainage ; Humans ; Infant ; Male ; Mediastinal Emphysema ; Oxygen ; Pneumothorax ; Respiration, Artificial ; Respiratory Insufficiency ; Ventilation*

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