We have experienced a 14 year old female patient who had suffered from headache, dizziness, exertional dyspnea and chest pain during 6 months. She was diagnosed as primary pulmonary hypertension by ultrasonogram and cardiac cathererization. On the cardiac catheterization, there was elevated pulmonary artery pressure and normal pulmonary wedge pressure. We report this case with related literature review.
Adolescent ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Dizziness ; Dyspnea ; Female ; Headache ; Humans ; Hypertension, Pulmonary* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Ultrasonography

The purpose of this study was to obtain the angulation and inclination of FACC of all teeth to FH plane. Study models of 31 persons with normal occlusion were selected and mounted on the semiadjustable articulator for this study. Using TARG. with a little modified method, the angulation and inclination of FACC of all teeth to FH plane were measured and then the measurements were analyzed statistically. The obtained results were as follows. Mean, standard deviation, maximum value, and minimum value of the angulation and inclination of FACC of upper and lower teeth were obtained. The FACC in both upper and lower arch was progressively lingually-inclined from anterior teeth to posterior teeth. In the angulations of FACC of upper teeth, central and lateral incisor showed similar value. Yet, the FACC of the rest was progressively distally-angulated -from the canine to posterior teeth. The FACC in lower arch was progressively mesially-angulated from anterior teeth to posterior teeth. The angulation and inclination of FACC of any tooth in both upper and lower teeth correlated strongly and positively with the angulation and inclination of FACC of adjacent tooth.
Dental Articulators ; Dental Occlusion ; Humans ; Incisor ; Tooth*

In this article, we introduce a new method of tooth positioner fabrication using modified T.A.R.G. to measure the inclination and angulation of individual teeth. In finishing stage of orthodontic treatment with a fixed appliance, we anticipate that tooth positioner fabricated using the described construction method provides the movement of individual tooth into a desired position; corrected inclination and angulation of teeth, extrusion, intrusion, rotation and so on.
Tooth*

A hyperosmolar hyperglycemic state (HHS) is a life-threatening complication rarely seen in children and adolescents with type 1 diabetes mellitus (T1DM). However, early diagnosis and proper treatment are vital to reduce the high morbidity and mortality rates associated with HHS. We describe a male patient who presented with polydipsia, polyuria, and a drowsy mental status. His initial biochemistry results demonstrated severe hyperglycemia (1,456 mg/dL), hyperosmolarity of 359 mOsm/kg (effective osmolarity, 323 mOsm/kg), and mild acidosis (venous pH, 7.327). The patient was diagnosed with HHS and T1DM based on the presence of hyperosmolarity, hyperglycemia, and positivity for antiglutamic acid antibodies. Intensive intravenous fluid and regular insulin (0.025 units/kg/hr) were administered. After hydration and insulin treatment, the patient's mental status and serum glucose and sodium levels improved, and no neurological complications were observed. In summary, most cases of HHS are observed in adult patients with type 2 diabetes. However, occurrences in children and adolescents with T1DM have also been reported. Therefore, HHS should be considered in the differential diagnosis of hyperglycemic emergencies.

No abstract available.
Female ; Ovary* ; Teratoma*

Graves' disease (GD) accounts for 10%-15% of thyroid disorders in children and adolescents. The use of antithyroid drugs as the initial treatment option in GD is well accepted. An average two years remission is achieved in about 30% of children treated with antithyroid drugs. However, the optimal treatment duration and the predictive marker of remission after antithyroid drug therapy are still controversial. Additionally, 131I therapy and surgery are considered the option for treatment in children and adolescents with GD. We review the treatment options for pediatric GD and the possible determinants of remission and relapse on antithyroid drug treatment in children and adolescents.
Adolescent* ; Antithyroid Agents ; Child* ; Drug Therapy ; Graves Disease* ; Humans ; Hyperthyroidism ; Recurrence ; Thyroid Gland

PURPOSE:This study was performed to elucidate that status epilepticus (SE) induces long- term neuronal damages in an immature brain and to evaluate that topiramate (TPM) has a protective effect. METHODS:We investigated the changes in a subtype expression of glutamate and gamma- amino butyric acid (GABA) receptors, and the structural integrity due to cell losses in the mouse pup hippocampus after SE using an immunoblot and confocal microscopy. RESULTS:SE induced significant cell losses with structural changes in the hippocampus 1 month later. SE up-regulated the glutamate receptor1 (GluR1) expression with an increased ratio of GluR1 to glutamate recptor2 (GluR2), leading to the formation of Ca2+ permeable alpha- amino-3-hydroxy-5-methyl-4-isoxazoleepropionic acid (AMPA) receptors for the enhanced neurotoxicity. TPM prevented the SE-induced GluR1 expression. The expression of GABAA receptors was highly increased 1 month after SE, whereas that of GABAB receptors was not changed. The TPM treatment attenuated SE-induced upregulation of GABAA receptors. SE induced significant cell losses and disruption of structural integrity in the hippocampus CA1 and CA3 regions, but the TPM treatment for 1 month in developing brains reduced the SE- induced hippocampal damage. CONCLUSION:TPM has a neuroprotective action, which might be mediated by the modulation of GluR1 and GABAA receptors.
Animals ; Brain* ; Butyric Acid ; Glutamic Acid ; Hippocampus ; Mice* ; Microscopy, Confocal ; Neurons ; Receptors, GABA ; Status Epilepticus ; Up-Regulation

Congenital lipoid adrenal hyperplasia is the rarest type among salt losing types of congenital adrenal hyperplasia. The defect of this disorder is in the cholesterol side chain cleavage enzyme(P450SCC)which converts cholesterol to pregnenolone. W experienced a case of 20,22 desmolse deficiency in a 21-day old phenotypically female who was admitted to our hospital due to lethargy and dark skin pigmentation. The characteristic findings were decreased serum cortisol, aldosterone, testosterone, increased ACTH. The ACTH and hCG stimulation test were performed and there were no response. The sex chromosomal analysis showed made XY. One year later after hormone therapy, growth and development are normal.
Adrenal Hyperplasia, Congenital ; Adrenocorticotropic Hormone ; Aldosterone ; Cholesterol ; Female ; Growth and Development ; Humans ; Hydrocortisone ; Hyperplasia* ; Lethargy ; Pregnenolone ; Skin Pigmentation ; Testosterone

Congenital hypogonadotropic hypogonadism (CHH) is characterized by complete or partial failure of pubertal development because of inadequate secretion of gonadotropic hormones. CHH consists of hypogonadotropic hypogonadism with anosmia or hyposmia, Kallmann syndrome, and the normosmic variation normosmic idiopathic hypogonadotropic hypogonadism. CHH is one of the few treatable diseases of male infertility, although men with primary testicular dysfunction have irreversibly diminished spermatogenic capacity. The approach to CHH treatment is determined by goals such as developing virilization or inducing fertility. This review focuses on the current knowledge of therapeutic modalities for inducing puberty and fertility in men with CHH.

Type 1 diabetes mellitus (T1DM) is characterized by autoimmune destruction of pancreatic beta-cells in genetically predisposed individuals, eventually resulting in severe insulin deficiency. It is the most common form of diabetes in children and adolescents. Genetic susceptibility plays a crucial role in development of T1DM. The human leukocyte antigen complex plays a key role in the pathogenesis of T1DM. Furthermore, genome-wide association studies and linkage analysis have recently made a significant contribution to current knowledge relative to the impact of genetics on T1DM development and progression. This review focuses on current knowledge of genetics as a pathogenesis for T1DM. It also discusses mechanisms by which genes influence the risk of developing T1DM as well as the clinical and research applications of genetic risk scores in T1DM.
Adolescent ; Child ; Diabetes Mellitus, Type 1 ; Genetic Predisposition to Disease ; Genetics ; Genome-Wide Association Study ; Humans ; Insulin ; Leukocytes