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: 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

No abstract available.
Humans ; Infant, Newborn* ; Mothers*

Human puberty is a complex, coordinated biological process with multiple levels of regulations. The timing of puberty varies greatly in children and is influenced by both environmental and genetic factors. The key genes of pubertal onset, KISS1, GPR54, GNRH1 and GNRHR, may be major causal factors underlying gonadotropin-releasing hormone-dependent precocious puberty (GDPP). Two gain-of-function mutations in KISS1 and GPR54 have been identified recently as genetic causes of GDPP. GNRH1 and GNRHR are also gene candidates for GDPP; however no mutations have been identified in these genes. Presently potential genetic causes like LIN28B continues to appear; many areas of research await exploration in this context. In this review, I focus primarily on the genetic causes of GDPP.
Biological Processes ; Child ; Gonadotropins ; Humans ; Puberty ; Puberty, Precocious ; Social Control, Formal

No abstract available.
Hepatitis B* ; Hepatitis*

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*

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

The authors performed prospectively the transcranial Doppler monitoring of bilateral anterior and middle cerebral arteries in 15 patients with ruptured cerebral aneurysm. The entry criteria for the study were confined to the patients who were admitted within 3 days after bleeding and had clinical grades of I, II, or III. The mean frequency shifts of bilateral anterior and middle cerebral arteries were increased immediately after ictus and showed continous further elevation between the 3rd and 8th rupture days. After that, they normalized slowly. The increase of frequency shift preceded clinical ischemic symptoms and an early steep increase of frequency shift was correlated to a high chance for suffering delayed ischemic deficits. The thick clots in subarachnoid cisterns shown on CT scans taken within the third rupture day were correlated well to the severe increase of frequency shifts. The aggressive treatment was done on asymptomatic patients who showed relatively rapid increase of frequency shifts, and they had shown no or trasient mild ischemic symptoms.
Hemorrhage ; Humans ; Intracranial Aneurysm* ; Middle Cerebral Artery ; Prospective Studies ; Rupture ; Tomography, X-Ray Computed