No abstract available.
Hypogonadism*

No abstract available.

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

PURPOSE:The pathogenesis of short stature in growth hormone(GH) deficiency is believed to be based on the growth failure of growth plate chondrocytes by reduced growth hormone dependent insulin-like growth factor- I (IGF- I ) level in serum. Therefore, author studied the growth promoting effect of IGF- I purified from human serum on the chondrocytes, cultured from rat rib cartilage. METHODS:Rat rib cartilage were treated with type II collagenase and hyaluronidase and were cultured in Ham's F-12 culture media containing 10% fetal calf serum. Growth promoting effect of IGF- I was measured by MTT dye by adding 20ng/ml IGF- I purified by protein-diol 120 column(YMC Co, Japan) from human serum, to 1*104 cultured chondrocytes separated into each of 96 well culture vessel. RESULTS: 1) When elution time of biotin labeled IGF- I by protein pak 300sw column was compared to elution time of standard molecular weight, IGF- I exists as large complex of 150Kd and small complex of 50Kd with free 7Kd form in serum before acid treatment. After acid treatment, IGF- I exists as small complex of 50Kd with free 7Kd form. 2) IGF- I purified from blood samples, as compared to genetic engineering product standard IGF- I , showed good parallelism in competition inhibition curve by purity analysis utilizing IGF- I antibody, and thus it is assumed that complex protein as inhibiting factor for purified IGF- I does not exist. Furthermore, complex protein was not present on the Western ligand method using biotin-labeled IGF- I ligand after purified IGF- I transferred to nitrocellulose paper following SDS-PAGE electrophoresis. 3)IGF- I of 20ng/ml showed 30% growth promoting effect, when rat rib chondrocyte culture with Dulbeco's modified Eagles medium(DMEM) is considered to show maximum growth promoting effect, while with pure culture medium, DMEM, showing minimum effect. CONCLUSIONS:The results of this study suggest that IGF- I purified by this method assumes the role of growth promoting effect on the chondrocytes, and that the described method of radioimmuno assay of IGF- I also could effectively remove inhibiting protein complex, therefore allowing more accurate assay.
Animals ; Biotin ; Cartilage ; Chondrocytes* ; Collagenases ; Collodion ; Culture Media ; Eagles ; Electrophoresis ; Electrophoresis, Polyacrylamide Gel ; Genetic Engineering ; Growth Hormone ; Growth Plate ; Humans* ; Hyaluronoglucosaminidase ; Molecular Weight ; Rats* ; Ribs* ; Staphylococcal Protein A

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

Nowadays, to evaluate growth hormone(GH) deficiency-suspicious short stature, we usually use more than two kinds of provocative tests using various pharmacologic agents such as clonodine, L-dopa, insulin, etc. However, the importance of physiologic natural secretion of GH was recently approved. In the past, diagnosis of GH neurosecretory dysfunction was made by studying the 24-hour spantaneous GH secretion profile. But, because it is very clinically difficult, and so we measured and evaluated the mean GH concentrations of blood samples, obtained every 30 minutes during the first 3 hours' nocturnal sleep, instead of that. At the department of Pediatrics, Hanyang University Hospital from November, 1992 to February, 1994, we selected 34 GH deficiency-suspected children on the base of their growth data and bone age, etc. After GH stimulation with clonidine(100-150 ug/m2) and L-dopa(200-250 mg/m2), we measured their peak GH values by the immunoradiometric assay(IRMA) kit. And, we measured the mean GH concentrations of blood samples, obtained every 30 minutes during the first 3 hours noctumal sleep(22:OOPM-1:OOAM). RESULTS: 1. We analyzed the relationship between the mean of 3-hours' physiologic night-time surge of GH(X) and the phamacologically stimulated peak GH level(Y), except for the two neurosecretory dysfunction suspicious children. And so, for the 32 children, the relationship was Y=1.806X+ 3.177, r= 0.794, p<0.01. However, for all the tested children(34), the relationship is Y=1.709X+ 4.06, r=0.737, p<0.01. 2. We analyzed the relationship between the peak of 3-hours' physiologic night-time surge of GH(X) and the phamacologicaUy stimulated peak GH level(Y), except for the two neurosecretory dysfunction suspieious children. And so, for the 32 children, the relationship was Y=0.683X+ 3.686, r=737, p<0.01. CONCLUSION: For all the tested children(34), the relationship between the mean 3hr physiologic GH night time surge and stimulated peak GH value is Y=1.709X+4.06, r=0.737, p<0.01. The percentage of GH neurosecretory dysfunction is 5.8%(2/34). And, except for the two childrren, the relationship is more good, Y=1.806X+3.177, r=0,794, p<0.01. On the above relationship, the 3-hour GH night time-surge test might to give us some helpful information for the diagnosis of neurosecretory dysfunction.
Child* ; Diagnosis ; Growth Hormone* ; Humans ; Insulin ; Levodopa ; Pediatrics