Sudden death and critical illness due to carbohydrate metabolic disorders / 中国实用儿科杂志

Carbohydrates are the important energy source of body,including glucose,galactose,fructose and glycogen. Congenital enzymes defects will cause carbohydrates metabolic disorders. Most of the carbohydrate metabolic disorders could lead to hypoglycemia. Most patients presented with chronic disease course. But some patients with serious diseases,such as glycogen storage disease type I,fructose-1,6-bisphosphate deficiency,presented as acute onset with critical illness,resulting in hypoglycemia and multiple organ damage(encephalopathy,cardiomyopathy,hepatopathy and myopathy). Most of the carbohydrate metabolic disorders have good prognosis if the prompt diagnosis and proper intervention are available. Sudden death occurred in some severe cases. Post-morterm study by metabolic autopsy is important to conform the diagnosis and directive genetic counseling.

Correlation between serum microRNA-122 and insulin resistance in obese children / 中国当代儿科杂志

OBJECTIVE@#To study the relationship between serum microRNA-122 (miR-122) and insulin resistance in obese children.@*METHODS@#Forty-seven children with severely obesity aged 7-14 years and 45 age- and gender matched healthy children with normal weight (control group) were enrolled. The levels of height, weight, waistline, hip circumference, fasting blood glucose (FBG), fasting insulin (FINS), triglyceride (TG), total cholesterol (TC), free fatty acid (FFA), interleukin-6 (IL-6) and miR-122 in the two groups were measured. Body mass index (BMI), waist-hip ratio (WHR) and insulin resistance index (HOMA-IR) were calculated.@*RESULTS@#Compared with the control group, the height, weight, BMI, WHR, FINS, HOMA-IR, TG, FFA, IL-6, and miR-122 levels in the obese group were significantly increased (P<0.05). MiR-122 levels in the obese group were positively correlated with FINS, HOMA-IR and IL-6 levels (r=0.408, 0.442, and 0.464 respectively, P<0.05). The changes of miR-122 have a linear regression relationship with IL-6 (b'=0.318, P<0.05).@*CONCLUSIONS@#The elevated serum miR-122 levels may be correlated with insulin resistance in obese children. The mechanism needs to be further studied.

Protective effect of vitamin A on residual pancreatic β cell function in children with type 1 diabetes mellitus / 中国当代儿科杂志

OBJECTIVE@#To study the protective effect of vitamin A on residual pancreatic β cell function in children with type 1 diabetes mellitus (T1DM) and its mechanism.@*METHODS@#A total of 46 children with T1DM (with a course of disease of 0.5-1 year) were randomly divided into an intervention group and a non-intervention group (n=23 each). The children in both groups were given insulin treatment, and those in the intervention group were also given vitamin A at a daily dose of 1 500-2 000 IU. A total of 25 healthy children were enrolled as the control group. The daily dose of insulin was calculated for the children with T1DM, and the serum levels of glycosylated hemoglobin (HbA1C), stimulated C-peptide, vitamin A, and interleukin-17 (IL-17) were measured before intervention and 3 months after intervention.@*RESULTS@#Before vitamin A intervention, the intervention group and the non-intervention group had a significantly lower serum level of vitamin A and a significantly higher level of IL-17 than the control group (P<0.01). After 3 months of intervention, the intervention group had significantly lower serum IL-17 level and insulin dose and a significantly higher level of stimulated C-peptide than the non-intervention group (P<0.05).@*CONCLUSIONS@#Vitamin A may protect residual pancreatic β cell function, possibly by improving the abnormal secretion of IL-17 in children with T1DM.

Status of penis and testicular development and effects of overweight/obesity on them in boys in the Zhengzhou area / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To evaluate the current status of penis and testicular development in boys and the effects of overweight/obesity on their development in the Zhengzhou area of Henan Province.</p><p><b>METHODS</b>Height, weight, waist circumference, hip circumference, penis length and testicular volume were measured in 3 546 4 to 12-year-old boys. The penis length and testicular volume were compared between the overweight/obesity and normal weight groups.</p><p><b>RESULTS</b>Before 9 years of age, the testicular volume was progressively smaller, and after 9 years old, it gradually increased. By the age of 11, it increased rapidly. The penis length increased gradually between 4 and 11 years of age, and after the age of 11 it increased rapidly. Phimosis was found in 144 cases (4.01%) and cryptorchidism was found in 18 cases (0.51%). A total of 639 (18.02%) boys were overweight or obese among 3 546 boys. At the ages of 6 and 7 years, the testicular volume in the overweight/obesity group was greater than in the normal control group (P<0.05). The penis length in the overweight/obesity group was significantly shorter than in the normal control group (P<0.05) by the age of 11 years. The correlation analysis showed that the testicular volume at the ages of 4 and 5 years was positively correlated with height, weight, BMI, waist circumference and hip circumference in overweight/obese boys. The penis length at the ages of 7 and 8 years was negatively correlated with weight, waist circumference and hip circumference. By the age of 12 years, the penis length was positively correlated with the height.</p><p><b>CONCLUSIONS</b>The development of penis and testicles in boys in the Zhengzhou area is in line with the level of sex development of Chinese boys. Overweight/obesity adversely affects the development of penis and testicles.</p>

Association of cytotoxic T lymphocyte-associated antigen-4+49A/G polymorphism with childhood type 1 diabetes mellitus: a meta-analysis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To evaluate the association of cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) + 49A/G polymorphism with type 1 diabetes mellitus (T1DM) in children.</p><p><b>METHODS</b>Papers about the association of CTLA4+49A/G polymorphism with T1DM in children were collected by searching PubMed, EBSCO, CBM, CNKI, and Wanfang Data. A meta-analysis was performed to examine differences in the genotypes (AG, GG, and GG+AG) and G allele at position 49 of the CTLA-4 gene between a childhood T1DM group and a control group.</p><p><b>RESULTS</b>A total of 10 papers involving 1084 T1DM children and 1338 healthy children were included. The Meta-analysis was performed to evaluate the association of the genotypes (AG, GG, and GG+AG) and the G allele at position 49 of the CTLA-4 gene with T1DM using a fixed effect model according to the heterogeneity test results of all studies. The pooled OR values (95% CI) were 1.13 (0.97-1.33), 1.42 (1.16-1.75), 1.20 (1.03-1.40), and 1.21 (1.09-1.33), suggesting a significant difference in genotypes (AG, GG, and GG+AG) and the G allele at position 49 of the CTLA-4 gene between the two groups.</p><p><b>CONCLUSIONS</b>CTLA-4 +49A/G polymorphism is associated with T1DM in children.</p>

Effects of Astragalus membranaceus on cytokine secretion of peripheral dendritic cells in children with Henoch-Schonlein purpura in the acute phase / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To explore the effects of Astragalus membranaceus (AM) on the cytokines secretion of peripheral dendritic cells (DC), including interleukin-10, -12, and -18 (IL-10, IL-12 and IL-18), in children with Henoch-Schonlein purpura (HSP) in the acute phase; and to study the immunological regulation mechanism of AM.</p><p><b>METHODS</b>Peripheral blood mononuclear cells (PBMC) were obtained from 28 children with acute HSP by density gradient centrifugation, and each sample was divided into two parts, one untreated and one treated with AM. All cells were developed to mature DC through treating with recombinant human granulocyte macrophage colony stimulating factor (GM-CSF), interleukin-4 (IL-4) and tumor necrosis factor-alpha (TNF-alpha). Expression of CD83 in the surface of mature DC was detected by flow cytometry, and levels of IL-10, IL-12 and IL-18 in the supernatant were measured by ELISA.</p><p><b>RESULTS</b>The supernatant level of IL-12 was higher [(141.58 +/- 100.19) ng/L vs (96.18 +/- 76.65) ng/L, t = 3.90, P<0.01], while levels of IL-10 and IL-18 were lower (t = 2.70, P<0.05; t = 4.07, P<0.01) in AM treated PBMCs than those in the untreated ones.</p><p><b>CONCLUSION</b>AM can correct the immunologic dysfunction of HSP children through increasing the IL-12, and decreasing the IL-10 and IL-18 secretions of PBMCs.</p>