No abstract available.
Rhinitis*

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: Leptin is a hormone involved in the regulation of energy balance. Serum leptin levels are correlated with body fat. It provide information to hypothalamus on the amount of energy stored in the adipose tissue. Certain endocrine disease presents obesity in childhood, such as growth hormone deficiency, Prader- Willi syndrome and Turner syndrome. The purpose of this study is to evaluate leptin levels in obese children and to know whether it is a useful marker to differentiate the underlying cause of obesity. METHODS: One hundred sixty six obese children were included in this study. Height, weight, HTSDS, WTSDS, adjusted WTSDS to height age and BMI were measured. Serum leptin levels were measured. RESULTS :Leptin levels in simple obesity, growth hormone deficiency, Prader-Willi syndrome, Turner syndrome and control were 12.3+/-6.3ng/ml, 6.4+/-2.0ng/ml, 19.9+/-11.2ng/ml, 8.9+/-5.3ng/ml, 5.7+/-3.7ng/ml respectively. Leptin levels were significantly high in obese children, especially in Prader-Willi syndrome, simple obesity and Turner syndrome. Leptin concentration were correlated with BMI and WTSDS. CONCLUSION: Leptin can be used as an indicator of obesity but, not suitable as a differential diagnostic factor for obesity.
Adipose Tissue ; Child ; Endocrine System Diseases ; Growth Hormone ; Humans ; Hypothalamus ; Leptin* ; Obesity* ; Pediatric Obesity* ; Prader-Willi Syndrome ; Turner Syndrome

PURPOSE:The recombinant human growth hormone is widely used to improve short stature but it was reported that GH therapy might accelerate sexual maturation especially short girl. We tried to find out the effects of GH on pubertal progression in female rat. METHODS:In animal experiment, forty female Sprague-Dawley rats were treated with rhGH(1U/kg). GH was given subcutaneously daily for 5weeks since 25day of life. We measured body weight and length twice a week, vaginal opening and epithelial change were observed daily. We checked the serum estrogen and insulin-like growth factor- I concentration weekly. The results were compared to 40 control female rats. RESULTS: 1) Body weight increased significantly in GH treated group(P<0.05). 2) Serum IGF- I concentration increased significantly after GH treatment(P<0.05). 3) Vaginal opening occurred on postnatal 34.3+/-1.9day in GH treated group and 35.4+/-2.2day in control group(p>0.1). First diestrous phase of puberty was noted on postnatal 36.8+/-1.9day and 38.3+/-3.0day, respectively(p>0.1). 4) The difference of serum E2 concentration of two group was insignificant. CONCLUSIONS:GH administration in female rats increased body weight and length without accelerating of pubertal maturation compared to control group. So GH treatment will give a good favor on final height.
Adolescent ; Animal Experimentation ; Animals ; Body Weight ; Estrogens ; Female ; Growth Hormone* ; Human Growth Hormone ; Humans ; Puberty ; Rats ; Rats, Sprague-Dawley ; Sexual Maturation

In order to determine the beneficial or detrimental effects of motion on fracture healing, rabbits femora were fractured surgically and each fracture was immobilized with a apecially designed external skeletal flxation device, which permitted a controlled spontaneous angulatory motion. in the first control group no motion was permitted; in the second group 5 degrees, and in the third group 10 degrees of angulatory motion was permitted. At 7th post-operative week, the bony union rates were 79%, 93%, and 54% In the first, second and third groups, respectively. Radiographically, the fractures were united most sufficiently in the second group. The ultimate bending load was signlficantly greater In the second group than the control but no significant differences were observed between the other groups. The ultimate bending stress and the modulus of elasticity were significantly greater ln the second group than the control and the third group, but no signlficant dlfferences were observed between the control and the third group. Fracture healing was significantly enhanced in the second group as compared to the control and the third group. It is suggested that a certain amount of motion at the fracture site enhances fracture healing, while excessive motion hinders it. Such enhancement seems to be related with the motion itself, and not wlth the muscle or jolnt function, since the rabbits Included in this study permitted the spontaneous full weight bearing which also permitted near normal muscle and joint function.
Elastic Modulus ; Fracture Healing ; Joints ; Rabbits ; Weight-Bearing

PURPOSE: Short stature is the most constant finding in Turner syndrome. Short stature in Turner syndrome has lately received considerable attention, mostly because of recent attemp to improve growth by hormonal treatments; growth hormone, oxandrolone, estrogen. The aim of this study was to find out whether growth promoting treatment would improve final height in girls with Turner syndrome. METHODS:Seventy-one girls with the clinical chracteristics Turner syndrome verified by karyotype analysis were entered into this study. The following selection criteria for final adult height were used; Chronological age of more than 14years old, bone age of more than 15years old and growth velocity of less than 0.5cm per 6months. Analysis was performed by means of multiple regression analysis between descriptive data; modality of treatment with oxandrolone and/or estrigen, parental height, karyotype and final adult height. RESULTS: 1) The final adult height of untreated Turner syndrome was 138.9+/-3.9cm. 2)The final adult height in 29 GH treated Turner girls was 143.9+/-6.5cm, significant higher value than 42 GH untreated Turner girls height, 139.8+/-5.2cm(p<0.01). 3) The final height in GH only group and combined group were 141.2+/-6.0cm, 146.2+/-6.2cm, respectively. The combined therapy was more effective than GH therapy(p<0.01). 4) The final height in 32 patients with karyotype of 45,X was 141.6+/-5.6cm, and that of 31 structural aberration group was 140.3+/-6.2cm. There was no significant difference between two groups. But in mosaicism, only numeric abnormalities, the final height 145.9+/-6.1cm was much more higher than other two groups(p<0.05). 5) The final adult height in Turner syndrome was in good correlation with target height. Final adult height(cm)= 1.01*Target height(cm)- 4.97 r=0.51, p<0.05. 6) There was positive correlation between final adult height and height SDS at start GH treatment and negative correlation with age at start GH treatment. The delta height (final height - height at start treatment) correlate with GH treatment duration. CONCLUSIONS:The final adult height in Turner syndrome in a given ethinic or national population varies in the same way as adult height in normal women. Growth hormone therapy may increase final height in Turner syndrome irrespective of ethinic or national difference. Further growth was observed in GH combined with estrogen or oxandrolone.
Adult* ; Estrogens ; Female ; Growth Hormone ; Humans ; Karyotype ; Mosaicism ; Oxandrolone ; Parents ; Patient Selection ; Turner Syndrome*

PURPOSE:Growth hormone stimulates longitudinal growth, and it also exerts various effects on the metabolism of carbohydrates, lipids and proteins, indirectly regulating fuel metabolism. A hallmark is the stimulation of lipolysis, suppression of glucose oxidation, and development of insulin resistance. These metabolic effects subsequently affect body composition and atherogenic risk factors. There have recently been numerous reports concerning the metabolic effects of growth hormone, but the results are conflicting and exact cellular mechanism of action is yet unknown. The aim of this study is to assess the effect of biosythetic growth hormone replacement on carbohydrate and lipid metabolism and its subsequent effect on atherogenic risk in growth hormone deficient and idiopathic short stature. METHODS:We studied 111 idiopathic short stature patients and 12 children diagnosed with growth hormone deficiency by growth hormone provocation tests. Subjects were divided into three groups on the basis of duration of treatment; those who had been receiving GH replacement for 6, 12 and 18 months. Growth hormone was adminstered in a subcutaneous dose of 0.1 units per kg 6 times a week, and levels of blood sugar, total cholesterol, triglycerides, HDL-cholesterol, free fatty acid were measured before and after treatment in each group and changes in the atherogenic index (calculated as total cholesterol/HDL-cholesterol) were compared. RESULTS: 1)Blood sugar levels showed no significant change after GH therapy in both idiopathic short stature and growth hormone deficient groups. 2)Levels of total cholesterol showed a signficant decrease 6, 12 and 18 months after GH therapy in the idiopathic short stature patients, but no signficant change was noted in the growth hormone deficient patients. 3)GrowthhormonetherapyshowednosignificanteffectoneitherHDL-cholesterol or atherogenic index in both idiopathic short stature and growth-hormone deficient children. 4)There was no significant change in triglyceride and free fatty levels in children with idiopathic short stature after growth hormone therapy. CONCLUSIONS:Growth hormone administration significantly lowered total cholesterol levels in idiopathic short stature children, but failed to have a significant effect on atherogenic risk. Despite the increasingly widespread use of growth hormone in the treatment of hypopituitarism and non-GHD short stature, data concerning the metabolic effects of growth hormone are conflicting and the precise underlying mechanism is yet to be revealed, making further research necessary to determine the long term consequences of growth hormone replacement.
Blood Glucose ; Body Composition ; Carbohydrates ; Child ; Cholesterol ; Glucose ; Growth Hormone* ; Humans ; Hypopituitarism ; Insulin Resistance ; Lipid Metabolism* ; Lipolysis ; Metabolism ; Risk Factors ; Triglycerides

PURPOSE: Prader-Willi Syndrome(PWS) is caused by absence of paternal contributions of the chromosome region 15q11-q13. To detact this region, high resolutional cytogenetic analysis, FISH with probe at PWS critical region or microsatellite polymorphism can be used. The gene for the small nuclear ribonucleoprotein polypeptide N(SNRPN) is not expressed in patients with PWS. We conducted molecular analysis with RT-PCR with SNRPN primers to find out more useful diagnostic tool in PWS. METHODS: Four patients with obesity and other characteristics of PWS were studied. The exprssion of SNRPN and control gene were studed by RT-PCR from peripheral lymphocytes. RESULTS :The SNRPN expression in reverse transcribed RNA from blood were easily detected in normal control but not in patients with suspected Parder-Willi Syndrome. CONCLUSION: We conclude that SNRPN expression study is a useful diagnostic method for detection of Prader-Willi Syndrome.
Cytogenetic Analysis ; Humans ; Lymphocytes ; Microsatellite Repeats ; Obesity ; Pathology, Molecular* ; Prader-Willi Syndrome* ; Ribonucleoproteins, Small Nuclear ; RNA ; snRNP Core Proteins*

Hyperinsulinemic hypoglycemia is a relatively rare disease in childhood period except neonate, but hypoglycemia due to delicate imbalance between glucose production & consumption is evoked easily and left permanent damage to brain at these period. The definition is that serum insulin level is above 10microU/ml when blood sugar level is below the 40mg/dl and so I/G ratio is higher than 0.4. The clinical manifestations are irrtability, frequent feeding and seizures etc. and there is no specific pancreatic pathology in most cases. We experienced 2 cases of hyperinsulinemic hypoglycemia with pancreatic hyperplasia and pancreatic adenoma each other. The diagnosis was made on clinical manifestations, laboratory results, radiologic and pathologic findings. We reported these cases with brief review of literature.
Adenoma ; Blood Glucose ; Brain ; Diagnosis ; Glucose ; Humans ; Hyperplasia ; Hypoglycemia* ; Infant, Newborn ; Insulin ; Pathology ; Rare Diseases ; Seizures

Pituitary adenoma should be differentiated diagnostically from pituitary hyperplasia, which can be classified by primary, secondary & tertially caused by ectopic tumors. Two cases with marked pituitary enlargement secondary to primary hypothyroidism were reported. The volume of the sellar turcica correlates with circulating TSH level. The subsequent regression with thryoxine therapy indicated hyperplasia rather than adenoma. This observation emphasizes the importance of diagnosing and treating primary hypothyroidism prior to considering surgery for possible pituitary adenoma. A brief review of related literatures was also made.
Adenoma ; Hyperplasia* ; Hypothyroidism* ; Pituitary Neoplasms*