Cranemcleod8859

4607C>T (p. Ala1537Val) in ABCC8 related to maturity-onset diabetes of the young (MODY) 12. This is the first reported case of HTG induced AP with DKA in a patient with MODY. In addition, we reviewed the literatures for pediatric cases of HTG with DKA. In patients with DKA, timely awareness of severe HTG related to insulin deficiency is crucial for improving the consequence of AP. We recommend considering AP in all DKA patients presenting with severe HTG to ensure early and proper management.

Metabolic and cardiovascular disease prevention starting in childhood is critical for reducing morbidity in later life. This study aimed to investigate the association of novel biomarkers with metabolic syndrome and vascular function/structure indices of early atherosclerosis in children.

This is a prospective study of 78 children (8-16 years old) grouped by the presence or not of metabolic syndrome (MS). The serum biomarkers studied were fibroblast growth factor 21 (FGF21), leptin, adiponectin and insulin-like growth factor binding protein-1. Endothelial function and carotid atherosclerosis were assessed with brachial artery flow-mediated dilation and carotid intima-media thickness, respectively.

Children with MS (n=12) had higher levels of FGF21 (median [interquartile range] 128 [76-189] vs. 60 [20-98] pg/ml, p=0.003) and leptin (18.1 [11-34.8] vs. 7.5 [1.9-16.5] ng/ml, p=0.003), and lower levels of insulin-like growth factor binding protein-1 (1.5 [1.2-2.1] vs. 2.3 [1.5-6] ng/ml, p=0.028) compared to children without MS. Flow-mediated dilation was inversely correlated with FGF21 (Spearman's rho -0.24; p=0.035) and leptin (rho -0.24; p=0.002) in all children. The best cut-off value of FGF21 levels for MS diagnosis was above 121.3 pg/ml (sensitivity/specificity 58/86%). Only FGF21 was significantly associated with the presence of MS after adjustment for body-mass-index, age and gender (odds ratio per 10 pg/ml increase 1.10 [95% CI 1.01-1.22]; p=0.043).

Increased FGF21 levels were associated with the presence of MS and worse endothelial function in children. Larger studies are needed to evaluate the potential value of FGF21 as a biomarker that could predict future metabolic/cardiovascular disease at an early stage.

Increased FGF21 levels were associated with the presence of MS and worse endothelial function in children. Larger studies are needed to evaluate the potential value of FGF21 as a biomarker that could predict future metabolic/cardiovascular disease at an early stage.Sphingosine kinase is a lipid kinase that phosphorylates sphingosine to generate sphingosine 1-phosphate (S1P). S1P regulates pancreatic islet β-cell endoplasmic reticulum stress and proliferation. 10074-G5 chemical structure Type 1 and type 2 diabetes share some key pathogenic processes. In this study, we investigated whether secretion of insulin and production of S1P is altered in alloxan and glucose-treated cells from the rat pancreatic β-cell line RIN-5F. RIN-5F cells were treated with 2 mM alloxan and 20 mM glucose for 6 h or 24 h before being evaluated by ELISA and western blotting. Insulin secretion and expression was higher in RIN-5F cells treated with glucose compared to control cells. In contrast, alloxan treatment did not affect insulin secretion and expression in RIN-5F cells. Interestingly, compared with normal control levels, S1P/EDG-5 was increased in both alloxan and glucose treated pancreatic β cell than normal control. MAPK-ERK inhibition strongly decreased the expression of insulin and S1P in glucose- or alloxan-treated RIN-5F cells. We observe that production of S1P is increased in both diabetic cell models. In addition, MAPK-ERK signaling regulates secretion of insulin and S1P expression in pancreatic β-cells. Based on the literature and our findings, S1P may be a promising agent for the treatment of insulin-related disorders.

There are few reports on the therapeutic effects of gonadotropin-releasing hormone agonists in boys with central precocious puberty, and no studies have been reported in Korea. We aimed to assess the significance of clinical factors and the effects of gonadotropin-releasing hormone agonist treatment on final adult height in boys diagnosed with central precocious puberty.

We retrospectively evaluated the medical records of 18 boys treated for idiopathic central precocious puberty between 2007 and 2018 at Chosun University Hospital. Gestational age, birth weight, and parental height were assessed at the initial visit. Chronological age, bone age, bone age/chronological age ratio, height and height standard deviation scores, predicted adult height, body mass index, and hormone levels were assessed during the treatment period.

At the time of diagnosis, the chronological age was 9.9±0.6 years, the bone age was 11.6±1.0 years, and the bone age/chronological age ratio was 1.20±0.1. The bone age/chronological age ratio decreased significantly to 1.12±0.1 at the end of treatment (P<0.05). The luteinizing hormone/follicular stimulating hormone ratio was 3.4±1.2, 0.6±0.4, 0.6±1.0 at the start of treatment, after 1 year of treatment, and at the end of treatment, respectively. After gonadotropin-releasing hormone agonist treatment, the final adult height reached 172.0±4.8 cm into the range of target height of 171.0±4.0 cm.

In boys with central precocious puberty, gonadotropin-releasing hormone agonist treatment improved growth potential.

In boys with central precocious puberty, gonadotropin-releasing hormone agonist treatment improved growth potential.

Antithyroid drugs (ATDs) are primarily used as an initial treatment in pediatric patients with Graves' disease (GD). We aimed to investigate the long-term outcomes in pediatric GD patients receiving ATDs.

Retrospective data from a single center were collected from April 2003 to July 2020. A total of 98 children and adolescents aged 2-16 years diagnosed with GD and receiving ATDs were enrolled. We investigated the factors correlated with remission by comparing children who achieved remission after 5 years and those with persistent disease.

The study included 76 (77.6%) girls and 22 (22.4%) boys. During the 5-year follow-up period, 18 children (18.3%) maintained remission, ATDs could not be discontinued in 74 (75.5%) patients, and relapse occurred in 6 (6.2%) patients. The remission group had significantly lower thyroid-stimulating hormone-binding inhibitory immunoglobulin (TBII) levels at diagnosis (P=0.002) and 3 months (P=0.002), 1 year (P=0.002), 2 years (P≤0.001), 3 years (P≤0.001), 4 years (P≤0.001), and 5 years (P≤0.