No abstract available.
Turner Syndrome*

PURPOSE:Chronic autoimmune thyroiditis is the most common thyroid disease in children and adolescents, and also is the most common cause of hypothyroidism in children and adolescents. We studied the clinical manifestations and natural courses of chronic autoimmune thyroiditis in children and adolescents. METHODS:We analyzed retrospectively twenty one children and adolescents who were diagnosed as chronic autoimmune thyroiditis before 15 years of age. The diagnoses were made from July 1997 to February 2004 at Ajou Univesity Hospital. We reviewed the clinical manifestations and thyroid functions of these patients. RESULTS:Nineteen patients were female and two were male. The mean age at diagnosis was 9.95+/-3.1 years, the mean duration of follow up was 23.52+/-22.98 months. At diagnosis, the most common chief complaint was goiter (15 patients/21 patients) and the others were short stature, easy fatigue, cardiac murmur and weight loss. Eighteen patients had goiters at diagnosis. Four had familial history of thyroid disease, Three of them had maternal history of thyroid disease and one had paternal history of thyroid disease. At diagnosis, Seven patients were euthyroid and four patients were hyperthyroid, eighteen patients had antithyroglobulin antibody, seventeen patients had antimicrosomal antibody, fourteen patients had both of them. At last follow-up, eight patients were euthyroid and ten patients were hypothyroid, three patients were hyperthyroid. CONCLUSION: Chronic autoimmune thyroiditis in children and adolescents can have the various important influences on the metabolism including the growth. The clinical manifestations and thyroid functions should be monitored in children and adolescents with chronic autoimmune thyroiditis.
Adolescent* ; Child* ; Diagnosis ; Fatigue ; Female ; Follow-Up Studies ; Goiter ; Heart Murmurs ; Humans ; Hypothyroidism ; Male ; Metabolism ; Retrospective Studies ; Thyroid Diseases ; Thyroid Gland ; Thyroiditis, Autoimmune* ; Weight Loss

PURPOSE:Mutations of the PROP1 (Prophet of PIT1) gene lead to combined pituitary hormone deficiency (CPHD), which is characterized by a deficiency of growth hormone (GH), thyroid stimulating hormone (TSH), luteinizing hormone (LH)/follicle stimulating hormone (FSH), prolactin (PRL) and adrenocorticotropic hormone (ACTH). We studied the PROP1 mutations in Korean children with hypopituitarism. METHODS:Twelve patients with congenital hypopituitarism were recruited from the Ajou University Hospital, Korea. The pituitary phenotype ranged from isolated growth hormone deficiency (IGHD) to CPHD. Clinical data, including endocrine and neuroradiological data were obtained from the medical records, and the DNA was collected and screened for mutations within PROP1 using polymerase chain reaction (PCR). The PCR products were sequenced directly. RESULTS:Nine patients had abnormal pituitary gland and three patients showed normal pituitary gland in magnetic resonance imaging (MRI). Endocrinologically, seven patients had IGHD and five had CPHD. Three of CPHD had GHD and hypogonadotrophic hypogonadism and the other two had GHD, central hypothyroidism, and ACTH deficiency. We identified no mutations in the PROP1 gene. However, three known polymorphisms were identified: The 27T->C (Ala9Ala) and the associated IVS1+3A->G in exon 1 were found in 25% of patient alleles sequenced. The 424G->A (Ala142Thr) in exon 3 change was identified in 4% of those sequenced. CONCLUSION:This study corresponds to the previous reports that PROP1 mutations are rare in sporadic cases of CPHD. The low mutation frequency in Korean patients may be due to ethnic-specificity or other candidate genes causing this disease. It is necessary to study PROP1 gene and the involved genes in more Korean patients.
Adrenocorticotropic Hormone ; Alleles ; Child ; DNA ; Exons ; Growth Hormone ; Humans ; Hypogonadism ; Hypopituitarism ; Hypothyroidism ; Korea ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Medical Records ; Mutation Rate ; Phenotype ; Pituitary Gland ; Polymerase Chain Reaction ; Prolactin ; Thyrotropin

PURPOSE: Antithyroid drug treatment is usually recommended as the initial treatment for children with Graves' disease. However, achieving remission with medical therapy usually requires many years of treatment, the risk for adverse reactions is relatively high and relapse is common after discontinuation of medication. There is therefore a debate about how Graves' disease should be treated in children. The aim of this study was to identify predictors of remission during 2 years from quitting of antithyroid drug. METHODS: We retrospectively studied children who had been treated for Graves' disease with antithyroid medication. We compared the children who achieved remission after two years with those who had persistent disease, to determine the variables associated with remission. RESULTS: 41 patients were enrolled, of whom five (12.2%) achieved remission and the rest (87.8%) could not achieve remission until the last visit. There were no significant differences in T3, free T4, antimicrosomal antibody (AMA), or thyroid stimulating hormone (TSH)-binding inhibitory immunoglobulin (TBII) between the two groups at presentation. However, the remission group had a significantly higher titer of TSH (P = 0.04) and a significantly lower titer of antithyroglobulin antibody (ATA) (P = 0.01) than the non-remission group at presentation. The non-remission group had higher ATA concentrations 3 months after treatment with antithyroid medication than the remission group did (P = 0.02). CONCLUSION: TSH and ATA concentrations at presentation can be used to stratify patients according to the likelihood of remission during 2 years from quitting of antithyroid drug.
Child ; Male ; Female ; Humans

Purpose : Shortness is the most frequent and quite disturbing characteristics of patients with Turner syndrome. The aim of this study was to evaluate the factors affecting final adult height(FAH) in these patients. METHODS : The study group was comprised of 19 patients who were diagnosed as Turner syndrome and attained FAH. We analyzed the influences of various factors on FAH in GH treated group with those in GH untreated group. Results : Nineteen patients were enrolled; thirteen received GH treatment and six did not. The mean duration of GH treatment was 24.3 months(range : 9 to 50 months), and the mean dosage of GH was 0.98+/-0.35IU/kg/wk in GH treated group. The mean growth velocity during GH treatment was 5.6+/-1.8 cm/yr, which was significantly higher than that during pretreatment period(P<0.05). In GH treated group, the mean chronological age, bone age, mean height, and height SD score at GH therapy were 13.7+/-1.7yr, 11.3+/-1.9yr, 129.7+/-7.9cm, and -4.1+/-1.1, respectively, which were not statistically different from those at diagnosis of GH untreated group. In GH treated group, the mean FAH and FAH SD score were 144.8+/-5.0cm, and -3.2+/-0.9, respectively, which showed no significant difference compared with those of GH untreated group. Analyzing the factor affecting FAH in all Turner girls of both groups together, parental height, chronological age, bone age, and bone age delay at diagnosis(or at the initiation of GH therapy) were not related to FAH. Height and height SD score at diagnosis(or at the initiation of therapy) were positively related to FAH(P<0.05, r=0.72). CONCLUSION : The results suggest that GH treatment dose not improve FAH in patients with Turner syndrome, despite increased growth velocity during GH treatment, which might come from intermittern GH therapy. This should be remained to be clarified with more Turner patients who attained FAH.
Adult* ; Diagnosis ; Female ; Growth Hormone ; Humans ; Parents ; Turner Syndrome*

Maturity-onset diabetes of the young (MODY) is a heterogenous form of diabetes characterized by the early onset of diabetes, autosomal dominant inheritance, and impaired insulin secretion. MODY is mostly caused by mutations of the hepatocyte nuclear factor 1-alpha (HNF1-alpha) and glucokinase genes in Caucasians. However most Korean, Japanese, and Chinese patients with MODY do not express known MODY genes. The cause of MODY in Asians has not yet been elucidated clearly. This review focuses on studies on Asian patients with MODY.
Asian Continental Ancestry Group ; Diabetes Mellitus, Type 2 ; Glucokinase ; Hepatocyte Nuclear Factor 1-alpha ; Humans ; Insulin ; Wills

Type 2 diabetes mellitus in children and adolescents has been increasing for last 10 years. The increase in frequency of type 2 diabetes appears to parallel the increase in prevalence and severity of obesity in children and adolescents. The metabolic syndrome, cluster of potent risk factors for atherosclerotic cardiovascular disease and type 2 diabetes, consists of insulin resistance, obesity, hypertension and hyperlipidemia. The atherosclerotic cardiovascular disease are rarely seen in the young, but the pathologic processes and risk factors are associated its development have been shown to begin during childhood. In pediatrician it is important to recognize early and treat aggressively for prevention of future cardiovascular disease in children and adolescents with metabolic syndrome.
Adolescent ; Cardiovascular Diseases ; Child ; Diabetes Mellitus, Type 2* ; Humans ; Hyperlipidemias ; Hypertension ; Insulin Resistance ; Obesity ; Pathologic Processes ; Prevalence ; Risk Factors

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: Type I diabetes mellitus(DM) is an autoimmune disease which decreases insulin secretion of pancreatic beta-cell. The honeymoon period in type I DM is known to be related to the partial recovery of C-peptide and preservation of pancreatic beta-cell function. The aim of this study was to determine factors influencing the onset of honeyrnoon period in children with type I DM. METHODS: The study group was composed of 50 patients with type I DM. The honeymoon period was defined as a period requiring less than 0.5U/kg/day to maintain near-normal blood glucose control without urine glucose for consecutive days. The factors for three study were age at diagnosis, sex, initial 24-hr urine C-peptide, initial HbA C, diabetic ketoacidosis and maximum insulin dose for near-normal blood glucose control before honeymoon. RESULTS: The group with honeymoon period received significantly smaller doses of insulin near-normal blood glucose control, compared to the group without honeymoon period, before honeymoon(P<0.01). Other factors had no significant connection with the development of honeymoon period. CONCLUSION: The developrnent of honeymoon period in type I DM had a significant relationship with maximum insulin dose before the onset of honeymoon period for near-normal blood glucose control. This needs remains to be further study.
Autoimmune Diseases ; Blood Glucose ; C-Peptide ; Child* ; Diabetes Mellitus* ; Diabetes Mellitus, Type 1 ; Diabetic Ketoacidosis ; Diagnosis ; Glucose ; Humans ; Insulin

This study was designed to assess the iron nutritional status and anemia of high school students. 383 female subjects in Ulsan Metropolitan city were evaluated using a questionnaire, and a measurement of hematological indices. The average height and weight of the respondents were 161.24+/-4.90 cm and 53.12+/-6.37 kg, repectively. The average BMI(body mass index) was 20.43+/-2.26 which was in the normal ramge. The average hemoglobin(Hb) concentration of the subjects was 13.14+/-0.97g/dl, and the average hematocrit(Hct) level was 40.84+/-17.40%. Transferrin saturation{TS(%)} was 20.86+/-10.32%, and the ferritin by Hct(<36%), 27.2%by TS(<14%),26.6% by ferritin(<12 ng/ml). As for clinical symptoms, the greatest number of respondents reported that they experienced 'decreased ability to concectrate'. Mean daily intakes of iron were 14.89+/-4.48 mg and heme iron intakes were 5.04+/-2.13 mg, which was 29.6% of total iron intake. The total iron binding capacity(TIBC) was negatively correlated with Hb concentration(r= -0.222, p<0.01). Serum ferritin was positively correlated with Hb concentration(r= - 0.323, p<0.05) and negatively correlated with TIBC(r= -0.367, p<0.01). TS(%) was positively correlated with Hb concentration(r= 0.402, p<0.01) and positively correlated with serum ferritin(r=0.413, p<0.01). As for the correlation between blood biochemistry and clinical symptoms related to anemia, the Hb concentration was negatively correlated with 'shortening of breath when going upstairs(p>0.05)' and 'cold hands and feet' significantly(r= -0.109, p<0.05). The level of Mean corpuscular volume(MCV) was negatively correlated with 'feel dizzy when standing up', 'tired out easily', and 'decrease ability to concentrate' significantly(p<0.05). In particular, the level of Fe was negatively correlated with 'shortening of breath when going upstairs' and 'feeling blue' significantly(p<0.01). These results suggest That the prevalence of iron deficiency of female high school students is very high, therefore guidelin......
Anemia* ; Biochemistry ; Surveys and Questionnaires ; Female* ; Ferritins ; Hand ; Heme ; Humans ; Iron* ; Nutritional Status ; Prevalence ; Transferrin ; Ulsan*