Isolated ACTH deficiency is a rare cause of secondary adrenocortical insufficiency. The clinical presentation can be similar to that of primary adrenal insufficiency, but most of them may be nonspecific. A female patient of 25 months of age, complainig short stature, showed hypocortisolemia without ACTH & cortisol stimulation by insulin-induced hypoglycemia test. All the other hormone state was normal. Left hand AP view revealed delayed bone age(3 month) compared with chronological age. No radiologic abnormality was found in sella MRI and adrenal CT. Here we report a case of isolated ACTH deficiency presented by short stature.
Addison Disease ; Adrenocorticotropic Hormone ; Female ; Hand ; Humans ; Hydrocortisone ; Hypoglycemia ; Magnetic Resonance Imaging

PURPOSE: Congenital hypothyroidism(CH) is not uncommon disorder, leading to retardation of mental development and growth, if not treated early. The aim of this study is to determine the factors influencing IQ of children with CH, diagnosed after 1 month of life. METHODS : Thirteen children with CH were included. They had intelligence test by KEDI-WISC(Korean Educational Development Institute-Wechsler Intelligence Scale for Children) and their medical records were reviewed. Their T4, TSH, height, age at diagnosis were investigated retrospectively. To evaluate the influence of T4, TSH, height, age at diagnosis on IQ, children were divided into three groups ; athyroid(n=8), sublingual(n=3), inborn errors of thyroid hormone synthesis(n=2) according to the result of thyroid scan. Results : In athyroid group, IQ closely correlated to VIQ and PIQ and had close relationship to T4 at diagnosis(.p=0.0086, r=0.8427), but no relation to TSH. There was no difference in height, T4 TSH, and IQ between athyroid and sublingual group. CONCLUSION : The results suggest that intellectual function in children with CH, diagnosed after 1 month of life depends on serum level of T4 at diagnosis. Further study is mandatory to elucidate the relationship between final IQ and factors, including thyroid function, age at diagnosis, adequacy of treatment, etc.
Child* ; Congenital Hypothyroidism* ; Diagnosis ; Growth and Development ; Humans ; Intelligence ; Intelligence Tests ; Medical Records ; Retrospective Studies ; Thyroid Gland

PURPOSE:Neopterin is a marker of activation of the T-lymphocyte/monocyte axis. We measured serum neopterin concentration to investigate whether serum neopterin levels are increased in children with Graves' disease and whether serum neopterin measurement can be used as a marker of disease activity in Graves disease. METHODS:Twenty children with Graves' disease(3 boys and 17 girls) and 15 healthy children(7 boys and 8 girls) are enrolled in this study. Serum neopterin concentrations are measured by radioimmunoassay. RESULTS:Neopterin concentration in children with Graves' disease(1.59+/-1.25ng/ml) is not higher than that of healthy children(1.51+/-0.73ng/ml). Neopterin concentration is not influenced by thyroid function and remission state. CONCLUSION: Serum neopterin level in children with Graves' disease can not be used as a marker of activity.
Axis, Cervical Vertebra ; Child* ; Graves Disease* ; Humans ; Neopterin* ; Radioimmunoassay ; Thyroid Gland

PURPOSE:Precocious puberty is the development of secondary sexual characteristics before the age of 8 years in girls and 9.5 years in boys. It is usually associated with premature, rapid skeletal maturation and closure of the epiphyseal plates, resulting in short stature compared with genetic height potential and can produce significant psychological distress for patients. We examined effects of treatment with long-acting gonadotropin-releasing hormone(GnRH) agonist on somatic and skeletal growth in patients with central precocious puberty(CPP). MATERIALS & METHODS:Two male and seven female patients were diagnosed as having central precocious puberty(CPP) on the basis of onset age of secondary sexual characteristics, bone age, results of GnRH stimulation test and levels of sex hormones. They were treated with Triptorelin or Leuprorelin acetate(80-100ug/kg, IM every 4 weeks) for 1 year. The patients have been analyzed in terms of changes in auxological parameters including height velocity(HV), HV SDS CA, height SDS CA, height SDS BA and predicted adult height(PAH) SDS before and 1 year after treatment with GnRH agonist. RESULTS:The growth velocity a year after treatment was decreased to 4.1+/-0.9 from 7.5+/-1.2cm/year(P<0.01) and the height velocity standard deviation score(SDS) for chronologic age decreased to -1.6+/-0.4 from 2.8+/-0.8(P<0.01). The height SDS for chronologic age was increased to 2.0+/-0.7 from 3.8+/-1.0 a year after treatment (P<0.01). However, no significant difference were observed in height SDS for bone age(-1.9+/-0.2 from -2.1+/-0.3)(p>0.05) and predicted adult height SDS(-2.2+/-0.5 from -2.3+/-0.4)(p>0.05) one year after treatment. CONCLUSION: We observed a remarkable growth deceleration a year after treatment with GnRH agonist in CPP patients. However, the results of this study shows no benefit of GnRH agonist treatment in improving predicted adult height. It is still not clear whether GnRH agonist treatment will eventually help the patients with CPP achieve a final adult height within the range of their genetic target height or not. Further extensive long-term study using strict selection criteria for GnRH agonist treatment is required to address this issue.
Adolescent ; Adult ; Age of Onset ; Deceleration ; Female ; Gonadal Steroid Hormones ; Gonadotropin-Releasing Hormone* ; Growth Plate ; Humans ; Leuprolide ; Male ; Patient Selection ; Puberty ; Puberty, Precocious* ; Triptorelin Pamoate

PURPOSE:Bone mineral mass increases with age, weight and pubertal development. Several factors influence the process of bone mineralization, and evaluation of bone mineral density(BMD) in children gives important clue to the different mechanisms of bone mass accumulation. To investigate the abnormality in bone mineralization, the normal range of bone mineral content in healthy children should be understood. The pattern, time and velocity in the decrease of bone mineral contents depend on disease entities. Because the ratio of trabecular and cortical bones and turn-over rates differ in each bone, it is needed to make normal data for trabecular and cortical bones respectively in children. METHODS:In 75 children(40 boys) with normal growth and development, BMD was measured with XR26 Bone densitometry using DEXA. The BMD of trabecular bone was obtained at lumbar spine at 2-4 level, and that of cortical bone was measured at femur neck. RESULTS:The BMD of trabecular bone increased with age without sexual difference. But the BMD of cortical bone was higher in male significantly at 8-9 and 10-11 yr of age. In both male and female, the BMD of trabecular and cortical bones correlated positively with age, bone age, height, weight, and body surface area. CONCLUSION: With this data, the changes in BMD affected by several diseases could be assessed.
Body Surface Area ; Bone Density* ; Calcification, Physiologic ; Child* ; Densitometry ; Female ; Femur Neck ; Growth and Development* ; Humans ; Male ; Reference Values ; Spine

PURPOSE:Growth hormone(GH) therapy is very effective for the treatment of short stature, but it is unconvenient that GH should be injected daily because of short half-life. Sustained-release forms of GH preparation is needed for better compliance. This study aimed to measure peak pattern and duration of release of hGH from solid microparticles using sodium hyaluronate. METHODS:In group 1, hGH(EutropinTM) 285microg/kg was injected subcutaneously to 2 Jindo dogs everyday for 7 days. In group 2, hGH solution(EutropinTM) was continuously infused subcutaneously for 12 hours a day for the first 2 days via mini pump(minimed co.) and then for 24 hours a day thereafter until 7th day with the rate of 11.9microg/kg/hr. In group 3, dose of 2mg/kg hGH in sustained-release formulation using sodium hyaluronate, was injected subcutaneously to 3 Jindo dogs. In group 4, two dose levels of 1mg/kg and 2mg/kg hGH in sustained-release formulation using sodium hyaluronate, were injected subcutaneously to each group of 4 Beagle dogs. To evaluate side reactions from continuous injection of sodium hyaluronate, sustained release form of hGH 2mg/kg was injected to 4 Beagle dogs once a week for 4 weeks and compared to 4 control Beagle dogs. Blood samples were withdrawn half- hourly for 6 hour and 2-4 times a day thereafter in Jindo dogs and at 6hr, 12hr, 22hr in the first day and twice a day(at 9:00, 16:00 O'clock) for the following 6 days. RESULTS:In group 1, peak GH conc. of 122+/-27ng/ml was observed at 1 hour after hGH(EutropinTM) 285microg/kg injection and 1/2 of peak GH conc. at 4 hour. and decreased to 2ng/ml at 24 hour. GH AUC(Area under curve) was 670(ng/ml.hr). In group 2, initial steady state GH conc. of 25ng/ml occurred after 6 hour, however, GH conc. decreased gradually to 16ng/ml at the 7th day. GH AUC based on th initial steady state GH conc. was 600(ng/ml.hr). In group 3(Jindo dogs), GH conc. was peaked at 12 hour and 1/2 of peak GH conc at 30-46 hour and decreased to baseline at 70 hour. GH AUC was 2173(ng/ml.hr). In group 4(Beagle dog), peak GH concentrations of 56+/-7ng/ml and 108+/-12ng/ml were observed at 12 hour for the doses of 1mg/ kg and 2mg/kg, respectively and 1/2 of peak GH conc at 48 hour and decreased to baseline at 80 hour. GH AUC was 3560(ng/ml.h) for 2mg/kg treated dogs. Serum IGF-1 was increased to peak levels of 520ng/ml, and 580ng/ml for the doses of 1mg/kg 2mg/kg, respectively, and persisted above the baseline till 120 hour. There was no specific side reaction during experimental period. CONCLUSION: Sustained-release form of hGH with sodium hyaluronate released GH for 70-80 hour with the peak level lower than that resulted from the conventional aqueous formulation of the equivalent dose, and higher concentration IGF-I maintained for 120 hour after injection above baseline. More extensive study is needed to permit for new therapeutic application.
Animals* ; Area Under Curve ; Compliance ; Dogs ; Growth Hormone* ; Half-Life ; Hyaluronic Acid ; Insulin-Like Growth Factor I ; Polymers

PURPOSE:Growth retardation is a serious clinical problem in children with chronic renal failure(CRF). Dialysis and renal transplantation do not provide an improvement in growth velocity. Possible causes of growth retardation are nutritional deficiency, electrolyte imbalance, uremia, renal asteodystrophy and chronic anemia. However, catch-up growth cannot be achieved after correcting these factors. There is no concordance about disturbances of growth hormone(GH)-insulin-like growth factor-I (IGF-I) axis. in CRF. This study was designed to evaluate the growth status, IGF-I, GH and the effect of GH in CRF. METHODS:Twelve children with CRF(five were treated conservative, seven were transplanted) were included. IGF-I, stimulated GH, 24 hour integrated concentration of GH (IC-GH)were measured. Six were given rhGH(0.1U/kg/day) for average one year. RESULTS: 1)Growth velocity(GV) was 3.2+/-0.8cm/yr(conservative therapy:3.3+/-0.7, transplanted:2.9+/-0.8). Height standard deveation score(SDS) was -2.4+/-1.3cm/yr(conservative therapy group:-3.3+/-1.4, transplanted group:-1.3+/-0.4). Bone age lagged 2.1+/-13yr behind chronological age. 2) IGF-I concentrations were normal. 3)Stimulated GH levels were normal(16.6+/-3.3ng/ml) except one patient. Twenty- four hour IC-GH were less than 3.2ng/ml in 4 patients. 4)After GH therapy, GV increased 3.3+/-0.7cm/yr to 5.4+/-0.8cm/yr and Ht SDS increased -3.3+/-1.4 to -2.9+/-1.5 in the conservatively treated group. GV increased 2.9+/-0.8cm/yr to 5.5+/-1.8cm/yr and Ht SDS increased -1.3+/-0.4 to -0.8+/-0.5 in the transplanted group. CONCLUSION: Stimulated GH was normal but spontaneous secretion of GH was decreased in some patients with CRF. This neurosecretory dysfunction may be one causative factor in CRF. For these patients GH replacement therapy will be effective in promoting growth.
Anemia ; Axis, Cervical Vertebra ; Child* ; Dialysis ; Growth Hormone* ; Humans ; Insulin-Like Growth Factor I ; Kidney Failure, Chronic* ; Kidney Transplantation ; Malnutrition ; Uremia

PURPOSE:Hypochondroplasia is a skeletal dysplasia characterized by poor childhood growth and an inadequate pubertal growth spurt. Final height attainment of hypochondroplasia has been reported to range between 120 and 152cm. Increased availability of growth hormone with the introduction of recombinant human growth hormone has allowed for clinical trials in a number of growth hormone sufficient children with growth problems. The purpose of this study was to assess the growth promoting effect of human growth hormone in children with hypochondroplasia. METHODS:Five patients with hypochondroplasia diagnosed by clinical and radiological findings between 1993 and 1997 at our hospital was aged 3 and 1/2 -11 and 1/2 years. Each patients continuously received human growth hormone 0.6-0.7U/Kg/week, intramuscularly or subcutaneously in 6-7 divided dose for 2 years. Standard auxologic assessment was carried out every 3 month interval in the first year after commencement of therapy and then same assessment was 6 monthly. Bone age was assessed 6 monthly using Gleurich-Pyle method. RESULTS:Mean height velocity of pretreatment and year 1 and 2 of GH treatment were 3.9+/-0.7, 6.5+/-1.8 and 5.7+/-1.5cm/year, respectively. Mean height standard deviation score for chronological age of pretreatment and year 1 and 2 of GH treatment were -2.7+/-0.3, -2.4+/-0.3 and -2.2+/-0.4, respectively. The increase in the height velocity diminishes over the subsequent year. The increment of bone age after GH treatment were same as the increments of chronological age. CONCLUSION: Short-term GH therapy increases the height velocity of children with hypochondroplasia, but the effect of GH therapy on final height remains unknown.
Child* ; Growth Hormone* ; Human Growth Hormone ; Humans

PURPOSE:Insuline-like growth factor I(IGF-I) is polypeptide mitogen and mediate growth effect of growth hormone(GH). It's serum level is regulated by GH. The aim of this study is to evaluate whether -2 standard deviation of IGF-I level in normal short stature after insuline and L-dopa stimulation test has any diagnostic significance in GH deficiency. METHOD:We included 64 children with GH deficiency(complete GH deiciency 18 cases, partial GH deficiency 46 cases). Their height was below 10 percentile of korean children's standard growth chart. Control group was 175 children whose test results were normal after insuline and L-dopa stimulation test. Serum growth hormone level was measured by IRMA(immunoradiometric assay) with "Daiichi" kit(Japan) and serum IGF-I level was measured with 125I RIA kit (U.S.A). RESULTS: 1)Serum IGF-I level in normal stimulation test result group was increased with the age and the level was higher in female than that of male. 2)Using the cut-off value of -2SD of IGF-I level in control group, sensitivity was 17.2%, specificity was 98.86%, positive predictive value was 84.62%, negative predictive value was 76.55%, and test accuracy was 76.99%. Sensitivity and test accuracy was 44.44% and 93.26% in th complete GH deficiency, respectively. 3)Serum IGF-I level was significantly correlated with peak GH level with insuline stimulation test in control and GH deficiency group(Y=0.018889X+11.32 r= 0.23930 P=0.0014, Y=0.008592X+4.189 r=0.28141 P=0.0267). But serum IGF-I level was was not correlated with peak GH level with L-dopa stimulation test(Y= 0.005609X+13.88 r=0.06625 P=0.3823, Y=0.008293X+2.98 r=0.20895 P=0.1031). CONCLUSION: Serum IGF-I level in GH deficiency group was lower than that of control group and had wide variation of normal range. Based upon above results IGF-I level has limited clinical value in the diagnosis of GH deficiency.
Child ; Diagnosis ; Female ; Growth Charts ; Growth Hormone* ; Humans ; Insulin ; Insulin-Like Growth Factor I* ; Levodopa ; Male ; Reference Values ; Sensitivity and Specificity

PURPOSE:Insulin-like growth factors, IGF- I and IGF-II, are proteins that promote cellular growth and differentiation of the various organs including the kidney. These peptides circulate in serum bound to specific carrier proteins, called IGF binding proteins(IGFBPs). The IGFs are produced in most organs but liver is believed to be the principal source of circulating IGF-I. We studied the correlation of serum IGF-I and IGFBP-2 pattern with aging. METHODS:Sera were collected from 320 healthy population who were grouped according to age. IGF-I was seperated from IGFBPs by Sephadex G-50 acid chromatography. We measured serum IGF-I and IGFBP-2 by using radioimmuno-assay (RIA) and immunoradiometric assay (IRMA) respectively. RESULTS:Serum IGF-I levels were quite low in early childhood, rising slowly and reaching a peak during puberty and a significant decline(P<00.01) during adulthood. The age-dependent pattern of serum IGFBP-2 levels shows a pattern opposite to that of IGF-I which are high at birth, decline by late puberty and increase again with aging. CONCLUSION: Our results demonstrate the alteration of serum IGF-I and IGFBP- 2 pattern with aging. These data suggests that when these tests are performed in the clinic, their interpretation should be based upon age specific criteria.
Adolescent ; Aging* ; Carrier Proteins ; Chromatography ; Humans ; Immunoradiometric Assay ; Insulin-Like Growth Factor Binding Protein 2 ; Insulin-Like Growth Factor Binding Proteins ; Insulin-Like Growth Factor I ; Insulin-Like Growth Factor II ; Intercellular Signaling Peptides and Proteins ; Kidney ; Liver ; Parturition ; Peptides ; Puberty