Development of waist circumference percentiles for Japanese children and an examination of their screening utility for childhood metabolic syndrome: a population-based cross-sectional study.

Background: In Japan, waist circumference (WC) percentiles to screen for childhood metabolic syndrome (MetS) are unavailable. The objectives of this study were to develop WC and WC-to-height ratio (WC/Ht) percentile curves by age and sex for Japanese children, and to test their utility in screening for MetS in children with obesity who are otherwise healthy.

Methods: The WC and WC/Ht percentiles were developed using the LMS method of summarizing growth standards, which monitors changing skewness (L), medians (M), and coefficients of variation (S) in childhood distributions.

[Causes and pathophysiology of pediatric obesity and its diagnostic criteria].

Recently, obesity has been listed as important health problems not only in adults but also in children. Prevalence of pediatric obesity has been rising in the last half century in most of the developed countries including Japan. In addition to changes in lifestyle, which are shown to have contributed greatly to promote weight gain in recent years, patients with some syndromes or diseases may have obesity as one of the manifestations of the disorders especially in childhood.

Rate of trabecular bone area in 3-6-year-old children and relation to intrauterine growth.

Background: The aim of the present study was to investigate bone mass using rate of trabecular bone area (RTBA) in Japanese children to determine its relationship with indices of growth such as height and weight from before birth through childhood.

Methods:   A cross-sectional study was conducted. The sample consisted of Japanese boys (n= 716) and girls (n= 586) aged 3-6 years.

Expression of 11beta-hydroxysteroid dehydrogenase 2 contributes to glucocorticoid resistance in lymphoblastic leukemia cells.

Synthetic glucocorticoids (GCs) form a crucial first-line treatment for childhood acute lymphoblastic leukemia (ALL). However prolonged GC therapy frequently leads to GC-resistance with an unclear molecular mechanism. 11β-hydroxysteroid dehydrogenase (11β-HSD) 2 inactivates GCs within cells.

Abdominal obesity is associated with cardiovascular risk in Japanese children and adolescents.

Background: Metabolic syndrome is listed as a risk for atherosclerosis. However, changes in that risk during childhood and adolescence have not been well-documented. It is also unclear whether individuals with abdominal obesity, but with as yet undiagnosed metabolic syndrome, have cardiovascular risks.

Importance of C-reactive protein level in predicting non-response to additional intravenous immunoglobulin treatment in children with Kawasaki disease: a retrospective study.

Background: Intravenous immunoglobulin (IVIG) therapy in the acute stage of Kawasaki disease (KD; mucocutaneous lymph node syndrome) is the treatment of first choice for preventing the development of coronary artery lesions (CALs). Failure of initial treatment with IVIG remains the most consistent risk factor for CALs. However, there are few reports on nonresponders to additional IVIG therapy in KD.

Prevalence of congenital heart disease assessed by echocardiography in 2067 consecutive newborns.

Aim: There are discrepancies in the reported prevalence of congenital heart disease (CHD). This study prospectively evaluated the prevalence of CHD in consecutive newborns using echocardiographic screening.

Methods: A cohort screening study was conducted in an unselected series of all live-birth newborns.

Hereditary spherocytosis coexisting with UDP-glucuronosyltransferase deficiency highly suggestive of Crigler-Najjar syndrome type II.

Patients with co-existing hereditary spherocytosis (HS) and UDP-glucuronosyltransferase 1A1 (UGT1A1) deficiency as Gilbert's syndrome (GS) have been reported, and previous studies have demonstrated an increased risk for developing gallstones in patients with co-inheritance of GS and HS. We experienced an interesting case of HS showing persistent jaundice after splenectomy, and upon further evaluation, the 25-year-old female patient was found to have HS combined with UGT1A1 deficiency. Sequence analysis of the UGT1A1 gene revealed that she was a compound heterozygote with p.

Age and sex differences in fat distribution in non-obese Japanese children.

Objective: To assess fat distribution in non-obese Japanese children and adolescents.

Design: 130 non-obese Japanese children (73 boys and 57 girls) from Kikugawa, Hamamatsu were included. The visceral fat area (VFA) and subcutaneous fat area (SFA) were measured by computed tomography (CT) and calculated (in cm(2)).

Initial Treatment of Pediatric Graves' Disease with Methimazole: A Retrospective Follow-up Study.

Antithyroid drugs are widely used in the therapy of Graves' disease (GD), and methimazole (MMI) is preferred for treatment of pediatric GD. The recommended initial dosage of MMI is 0.5-1.

Case of polyhydramnios complicated by Opitz G/BBB syndrome.

Opitz G/BBB syndrome is a congenital disorder characterized by midline defects, such as hypertelorism, cleft lip and/or palate, hypospadias, and by dysphagia often caused by laryngo-tracheo-esophageal abnormalities. We experienced a case of polyhydramnios in a male dichorionic diamniotic (DD) twin, who was diagnosed with Opitz G/BBB syndrome after birth. It is suggested that severe dysphagia was causatively associated with the development of polyhydramnios.

Dynamics of endogenous glucocorticoid secretion and its metabolism in Kawasaki disease.

Objective: Kawasaki disease (KD) is a severe inflammatory disease that occurs in childhood. Recently, the initial corticosteroid therapy for KD has been reconsidered because its efficacy is controversial. The aim of this study was to evaluate the dynamic change in endogenous glucocorticoid levels and their relation with 11beta-hydroxysteroid dehydrogenase (11beta-HSD) activity in the acute phase of KD.

Delayed enhancement cardiac magnetic resonance imaging in propionic acidemia.

We report on cardiomyopathy in propionic acidemia (PA) diagnosed using delayed-enhancement cardiac magnetic imaging (DECM) in a 27-year-old woman. DECM demonstrated hyperenhancement at the lateral wall of the left ventricle, suggesting myocardial changes occurring after a metabolic crisis, which improved during the convalescent period. DECM was useful for the diagnosis of cardiomyopathy in PA.

Effect of cibenzoline on biventricular pressure gradients in a pediatric patient with hypertrophic obstructive cardiomyopathy.

This report presents the case of an 8-year-old male with biventricular hypertrophic obstructive cardiomyopathy who achieved a successful reduction of the pressure gradients by treatment with cibenzoline. Ultrasound echocardiography showed marked intraventricular septal hypertrophy and left midventricular and right ventricular outflow obstructions. A cardiac catheterization revealed left midventricular and right outflow obstructions associated with pressure gradients of 100 and 35 mmHg, respectively.

Postnatal changes in adrenal size in very low-birth-weight infants: sonographic evaluation for the prediction of late-onset glucocorticoid-responsive circulatory collapse.

We investigated the postnatal pattern of changes in adrenal size in very low-birth-weight (VLBW) infants and its relation to late-onset glucocorticoid-responsive circulatory collapse (LGCC) that may be associated with adrenal insufficiency. In 36 VLBW infants born at <33 weeks' gestation, ultrasound examinations of postnatal changes in adrenal size during the first 3 weeks of life were performed. VLBW infants were classified into three groups: group A (N = 6), the actual adrenal area was greater than or equal to the predicted value at birth and unchanged at 3 weeks; group B (N = 24), the actual adrenal area was greater than or equal to the predicted value and decreased at 3 weeks; and group C (N = 6), the actual adrenal area was less than the predicted value and unchanged at 3 weeks.

Cytochrome P450 oxidoreductase deficiency with Antley-Bixler syndrome: steroidogenic capacities.

For patients with cytochrome P450 oxidoreductase deficiency (PORD), steroid replacement is recommended at times of stress. However, it is unknown how hormones respond to actual physical stress in these patients. We report a female infant with PORD accompanied by the Antley-Bixler syndrome phenotype.

Differential regulation of 11beta-hydroxysteroid dehydrogenase-1 by dexamethasone in glucocorticoid-sensitive and -resistant childhood lymphoblastic leukemia.

Glucocorticoid therapy forms a crucial first-line treatment for childhood acute lymphoblastic leukemia (ALL). However, glucocorticoid resistance is a therapeutic problem with an unclear molecular mechanism. 11beta-Hydroxysteroid dehydrogenase-1 (11beta-HSD1) is expressed in glucocorticoid target tissue, where it regenerates active glucocorticoids from inert 11keto-glucocorticoids, amplifying intracellular glucocorticoid levels.

Glucocorticoid regulation of the promoter of 11beta-hydroxysteroid dehydrogenase type 1 is indirect and requires CCAAT/enhancer-binding protein-beta.

11beta-Hydroxysteroid dehydrogenase type 1 (11beta-HSD1) converts inert 11keto-glucocorticoids to active 11beta-hydroxy forms, thereby amplifying intracellular glucocorticoid action. Up-regulation of 11beta-HSD1 in adipose tissue and liver is of pathogenic importance in metabolic syndrome. However, the mechanisms controlling 11beta-HSD1 transcription are poorly understood.