Pathophysiology of Precocious Puberty / 대한소아내분비학회지

No abstract available.

Psychosocial Adaptation in Children with Growth Hormone Deficiency / 대한소아내분비학회지

PURPOSE: Emotional and behavioral disturbances have been reported in severe short stature children. The purpose of this study was to examine psychosocial problems in children with Growth hormone deficiency(GHD), comparing with their normal height siblings. METHODS: Twenty three children with GHD(17 boys and 6 girls, mean age:13.1+/-2.8 yrs) were included. Their normal height siblings(8 boys and 2 girls, mean age:12.3+/-2.9 yrs) were included as control group. Parents of all participating children completed the Child Behavior Checklist. Patients 10 years and older completed the Youth Self Report. RESULTS: 1)Internalizing problem scores(somatic complaints, anxiety, depression, social incompetence) were higher in GHD compared to control group(49.0+/-15.0 vs 43.2+/-6.2, P<0.05). Externalizing problem scores and social competences were not different between GHD and control group. 2) Total problem scores in GHD were not different by sex. 3)Total problem scores were not different between idiopathic GHD and organic GHD. 4)Attention problems(r=0.45, P<0.05), delinquent behavior(r=0.49, P=0.01) and aggressive behavior(r=0.51, P<0.01) increased by age. 5)Height SDS negatively correlated with social problem(r=-0.47, P<0.05) and delinquent behavior(r=-0.61, P<0.01). Height SDS also negatively correlated with somatic complaints(r=-0.75, P<0.01), anxiety and depression(r=-0.66, P=0.01). CONCLUSION: Internalized problems such as somatic complaints, anxiety and depression were higher in GHD than control group. Behavioral problem scores were increased by age and behavioral problem scores were correlated with height deficit. When we treat GHD children, appropriate medical and psychological counseling should be included.

Clinical Manifestations according to Karyotype in Turner Syndrome / 대한소아내분비학회지

PURPOSE: The reported clinical manifestations in Turner syndrome were different by karyotypes, countries and races. So, We evaluated the physical findings & medical problems in patient with Turner syndrome according to karyotype. METHODS: We examined prospectively 53 cases that diagnosed as Turner syndrome by chromosome study at the Pediatric Endocrine Clinic, YUMC from May 1999 to Aug 1999. According to the karyotype, they were divided into 3 groups:45, X(22 cases, 41.5%), mosaicism(18 cases, 34%) and structural aberration group(13 cases, 24.5%), and 53 cases were reevaluated by prepared protocol of 22 abnormal physical findings and 4 medical problems. Chi-square test was used for statistic analysis. RESULTS: 1)In case of 45, X group, they had short stature(100%), cubitus valgus(95.5%), widely spaced nipples(86.4%), shield chest(72.7%), low posterior hair line(68.2%), in case of mosaicism group, they had short stature(100%), cubitus valgus(72.2%), high arched palate(66.7%), widely spaced nipples(61.1%), short neck(61.1%) and in case of structural aberration group, they had short stature(100%), high arched palate (92.3%), widely spaced nipples(92.3%), low posterior hair line(76.9%), shield chest (76.9%), in frequency order. Short stature was the most common finding in Turner syndrome. The incidence of high arched palate was significantly higher in structural aberration group(P<0.05). 2)In case of 45, X group, they had otitis media(63.6%), thyroid problem(4.5%) and no renal problem and cardiac problem, in case of mosaicism group, they had otitis media(38.9%), thyroid problem(5.6%) and no renal problem and cardiac porblem and in case of structural aberration group, they had otitis media(61.5%), thyroid problem(15.4%), renal problem(7.7%) and cardiac problem(7.7%) in frequency order, they had no statistical difference in among three groups. CONCLUSION: In case of 45, X group, the incidence of cubitus valgus, webbed neck were more common findings and of high arched palate, micrognathia were less common findings compared to other group. In case of mosaicism group, the incidence of micrognathia, epicanthal fold were more common findings and wide spaced nipples, antimongoloid fissure were less common findings compared to other group. In case of structural aberration group, the incidence of high arched palate is most common finding among 3 group(P<0.05) and pigmented nevi and epicanthal fold were less common findings compared to other group. The incidence of medical problems such as otitis media and thyroid problem were than other foreign country reports.

Effects of Exercise on Glycemic Control in Patients with Type 1 Diabetes Mellitus / 대한소아내분비학회지

PURPOSE: Exercise is frequently recommended in the management of diabetes mellitus(DM) and can improve blood control by increasing insulin sensitivity and psychological benefits. Exercise can result in good glycemic control in type 2 DM, when combined with diet and drug therapy. However, in type 1 DM, the expected improvement in glycemic control with exercise have not been clearly established. Effects of exercise on glycemic control in patients with type 1 DM were investigated. METHODS: 20 patients with of type 1 DM, who were no retinopathy, neuropathy, nephropathy and cardiac disorders, were enrolled and exercised for 30min. with 50% of individualized maximum VO2. Blood sugar concentration were measured before, immediate and 15min after exercise. The results were evaluated with HbA1c, C-peptide and DM duration. RESULTS: Blood sugar concentration were significantly decreased from pre-exercise 198+/-9.7mg/dL to immediate 145+/-7.1mg/dL and at 15min. Post-exercise 134+/-4.0mg/dL(P10% there was also significantly decreased in blood sugar levels 222+/-64.7mg/dL, 169+/-6.1mg/dL, 157+/-1.2mg/dL respectively(P<0.05). Group with moderate decreased blood sugar(40-99mg/dL) was 8 patients(42.1%), group with mild decreased blood sugar(<40mg/dL) was 7 patients(36.8%) and group with decreased more than 100mg/dL was 4 patients(21.5%). There was no correlation in degree of decreased blood sugar concentration among HbA1c, C-peptide, DM duration. CONCLUSION: Proper exercise in type 1 diabetic children can decrease blood sugar level significantly and make good glycemic control and can decrease DM microvascular complications in addition to motivating physically active lifestyle.

Prevalence of Autoimmune Antibodies in Type I Diabetic Children and Their Siblings / 대한소아내분비학회지

Background:Insulin dependent diabetes mellitus(IDDM) is known to be a disease characterized by a deficiency of insulin caused by destruction of the pancreatic beta-cells. It has been suggested that the clinical and immunological characteristics of IDDM in Korean are different from those of Caucasian. This study was undertaken to investigate the clinical characteristics and the prevalence of autoimmune markers in type I diabetic children and their prediabetic siblings in Korea. METHODS:Insulin autoantibody(IAA), antiglutamic acid decarboxylase(Anti-GAD) antibody, thyroid autoantibodies such as antithyroid antibody(ATA) and antimicrosomal antibody(AMA), and rheumatoid facter(RF) in 54 type I diabetic children have been measured. Diabetic autoimmune antibodies were also measured in 48 siblings. RESULTS: 1)Clinical characteristics of type I diabetic children were that age of onset was 8.6+/-4.4 years, duration of diabetes was 4.1+/-3.3 years. C-peptide at onset of diabetes was fasting 0.7+/-0.5ng/ml, and postprandial 1.2+/-0.5ng/ml, and HbA1c was 12.5+/-4.3%. 2)The positivity of IAA and anti-GAD antibody of type I diabetic children was 74% and 50% respectively. ATA and AMA positivity of type I diabetic children was 3.7% and 5.6%. however RF was not detected at all. Among the diabetic siblings, 48 persons for anti-GAD antibody, 21 for IAA, 27 for ICA were measured but 1 case was positive for IAA. 3)Clinical characteristics of type I diabetic children were not specific different between IAA and anti-GAD antibody positivity. But the mean age of onset of type I diabetic children was younger in case of both positivity of IAA and anti-GAD antibody than both negativity(7.8 vs 11.4 years old, P<0.05). 4)A case in whose brothers are diagnosed as IDDM has shown that autoantibody of elder brother was positive in both IAA and anti-GAD antibody, and younger brother was also strongly positive in IAA. Another case in whose sisters were IDDM, has shown that, while elder sister was positive in IAA, younger sister strongly positive in both IAA and anti-GAD antibody. 5)In a case of identical twin brother, the elder is type I diabetic child and the younger is normal, elder brother's onset of age was 6 years and 8 months old, and titer of anti-GAD antibody was measured as strong positive. Both ICA and anti- GAD antibody were negative in normal younger brother. First phase insulin release in IV GTT and the insulin levels in oral GTT showed reduction from the normal level in normal brother, and repeat check up showed normal ranges but on-going study is needed under observation. CONCLUSION: The prevalence of autoantibody positivity of type I diabetic children of Korea in this study were IAA 74%, and anti-GAD antibody 50%. Cases with both IAA and anti-GAD antibody positive were shown to be earlier onset. Though titers of auto-antibody in IDDM twins, brothers and sisters were strongly positive, auto-antibodies in siblings of IDDM patients were detected only one case with IAA positive(0.47%). We suggest that the pathogenesis of IDDM in Korean is different from foreign countries in terms of prevalence of autoimmune antibodies and more numbers of diabetic siblings should be tested for further study.

Response of Growth Hormone Treatment to Final Height in Children with Growth Hormone Deficiency and Familial Short Stature / 대한소아내분비학회지

PURPOSE: A number of studies have been published on the effect of growth hormone therapy over 1-3 years in children with growth hormone deficiency(GHD) & Familial short stature(FSS). So far final height data are seldomly available. Final heights of GH treated children with GHD & FSS were evaluated. METHODS: 10 Children with GHD and 69 children with with FSS were enrolled for the study. They were treated with GH 0.1IU/kg/daily in 10 GHD and 20 children with FSS. They were grown up and reached adult height. 49 children with FSS were not treated at all. Facors influencing final height were investigated. RESULTS: 1) All patients with GHD(Idiopathic 8 cases, Organic 2 cases) had additional gonadotropin deficiency and had multiple pituitary hormone deficiency. 2) At start of GH treatment boys of idiopathic GHD were 9.8 years old and 12.4 years old in girls and their mean height was 114.8cm(-2.8SDS), 123.0cm(-2.9 SDS)in boys and girls respectively. Boy with orgnaic GHD was 11.1 years and 6.7 years old in girl. Their height were 126.0cm(-1.5SDS) and 104cm(-1.2SDS) respectively. 3) Mean final height of idiopathic GHD was 167.6cm(-0.5SDS) in male and 161.0 cm(0.7SDS) and that of organic GHD was 173.0cm(0.5SDS) in male and 157cm (0SDS) in girl. 4) Mean Final height in untreated children with FSS was 159.8+/-.2cm(-1.6 SDS)in male and 149.6+/-.3cm(-1.4SDS) in female. Mean final height of GH treated in FSS was 162.5+/-.1cm(-1.5SDS) in male and 152.0+/-.4cm(-1.2SDS) in female But there was no statiscally difference between untreated and treated children in final height. 5) The age of onset of menarche was 12.74+/-.78 years old in GH treated group (n=12) and 12.45+/-.16 years old in untreated group(n=34). CONCLUSION: The GH administration in patients with GHD has been confirmed for growth promotion. but in case of FSS there was no significant difference between treated and untreated group. More further studies are needed for the confirmation of the efficacy of GH therapy in patients with FSS.

Two Cases with Prolonged TSH Elevation in Congenital Hypothyroidism / 대한소아내분비학회지

It has been proven that the newborn screening programs for congenital hypothyroidism are very important for its early detection and the early treatment of congenital It has been proven that the newborn screening programs for congenital hypothyroidism are very important for its early detection and the early treatment of congenital hypothyroidism can ameliorate and possibly reverse its consequences. Treatment with L-thyroxine is started in case of suspicious hypothyroidism at the first visit. Serum concentration of total and free thyroxine become normal within 1week after start of therapy but TSH values become normal from 2 months to 6 months after L-thyroxine therapy. The possible explanations for prolonged TSH elevation in congenital hypothyroidism are poor compliance for therapy, an inadequate dose of L-thyroxine, elevated threshold for thyrotropin suppression and two novel mutations in the thyrotropin (TSH) receptor gene in a child with resistance to TSH. Authors have experienced two cases of prolonged TSH elevation with normal T3 and T4 levels till 18months and 27 months of age after optimal L-thyroxine therapy and literature were reviewed.

Diagnostic Significance of Excercise-Induced Urinary Albumin Measurement in Children with Type 1(Insulin Dependent) Diabetic Children / 대한소아내분비학회지

PURPOSE:Urine examination for microalbuminuria during the clinical silent years can help to identify those patients most likely to progress to overt nephropathy. The normalization of the AER(Albumin Excretion Rate) by alteration of glomerular hemodynamics may be the key to preventing or delaying overt nephropathy. The prognostic and therapeutic implications have led to attempts to identify patients with microalbuminuria as early as possible.Because diagnosis of microalbuminuria has required a 12 or 24 hour urine collection,there has been interested in developing tests that might serve as a first-stage screen for microalbuminuria. Most reports have dealt with the usefulness of either the albumin concentration or the albumin/creatinine ratio in a timed or randomly collected urine specimen. Exercise induced albumin excretion is increased compared to resting and could unmask latent glomerular damage. The purpose of this study is to evaluate diagostic significance of urinary albumin measurement after exercise in children with insulin dependent diabetes. METHODS:Fifty-seven patients with IDDM collected their overnight urine from 22:00 to 6:00 in following morning. Exercised method for urine examination was 100M running and collected 2 hour urine after exercise. The AER and Albumin/ creatinine ratio(ACR) were measured in the urine and compared with before and after exercise. RESULTS:Mean ages of diabetic children was 13.7 yrs old and duration of diabetes was 4.1+/-2.7yrs, HbA1c 8.6+/-1.9%,Systolic BP 107+/-10mmHg and Diastolic BP 67+/-8mmHg. The exercise induced AER was increased compared to before exercise(10.3+/-10.5 vs 18.1+/-16.0 mg/24hr). The exercise induced ACR also increased too(1.4+/-1.5 mg/g.Cr vs 4.1+/-3.6mg/g.Cr)(P<0.01). Two(3.5%) children with IDDM was positive for microalbuminuria in terms of AER before exercise and 8 children(14%) after exercise. No one positive for microalbuminuria in terms of Alb/cr before and after exercise. The charateristics of 8 children with microalbuminuria after exercise was no specific difference between positive and negative microalbuminuria. There was specific correlation between microalbuminuria and HbA1c(P<0.05) but sex, ages, duration of diabetes & blood pressure were not correlated with incidence of microalbuminuria. CONCLUSION: The mean timed urinary albumin excretion after exercise was significantly higher than before exercise. Measurement of timed urinary albumin excretion after exercise will be helpful for detecting microalbuminuria earlier.