Pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL) is an extremely rare autosomal dominant cerebral small vessel disease. Since the discovery of the pathogenic gene COL4A1, several PADMAL pedigrees have been reported in recent years. The pathogenesis of PADMAL is due to the increased formation of type Ⅳ collagen caused by mutation of the 3′ untranslated region of COL4A1. Abnormal accumulation of collagen in the extracellular matrix leads to thickening of the basement membrane and narrowing of the lumen. Patients present with recurrent lacunar infarction at early stage, and cognitive impairment may occur as the disease progresses. The main imaging changes of PADMAL are multiple lacunar infarcts and bilateral hemispherical white matter lesions. Under electron microscope, the lumen of small blood vessels in the skin is obviously narrowed and the basement membrane is thickened. Currently, there are no effective treatments or interventions for PADMAL.

Objective: To explore the relationship between premenarchal dietary carbohydrate and dietary fiber intakes and age at menarche in Chinese girls. Methods: Based on dietary and menarcheal information on 750 girls from the Chinese Health and Nutrition Survey, multivariate linear regression models and logistic models were used to analyze the influence of dietary carbohydrate and dietary fiber intakes before menarche onset and age at menarche. Carbohydrate intake was replaced by engergg ratio carbohydrate for a sensitivity analysis. Results: Adjusting for residence, per capita household income, body mass index standard deviation score, and energy intake, higher intake and higher energy ratio of carbohydrate were associated with later age at menarche(P<0.01). Compared to girls at the lowest tertile of carbohydrate intake level, those at the highest tertile had a 0.35 years(2.8%) delay in age at menarche, while 55% decreased[OR(95%CI)=0.45(0.30-0.69)]. Dietary fiber intake was not associated with age at menarche in any model(P>0.2). Conclusion Girls with higher premenarcheal carbohydrate intake experienced menarche later, while dietary fiber intake was not associated with age at menarche.

Objective: To examine the prospective association between peripubertal diet quality and pubertal timing. Methods: Multivariate regression analyses were performed using data obtained from 1 588 SCCNG (Southwest China Childhood Nutrition and Growth) study participants, which included dietary data, anthropometric parameters, and information about potential confounders during peri puberty, two and four years before the onset of menarche or voice break. Dietary intake in peri puberty was assessed using a validated food frequency questionnaire. Diet quality was determined using the Chinese Children Dietary Index (CCDI), which measures adherence to current dietary recommendations. Age at menarche or voice break (M/VB) were used as pubertal markers. Results: The CCDI score ranged from 58.0 to 132.5 for girls and from 48.3 to 129.9 for boys. The pubertal markers consistently indicated that girls and boys with a higher peripubertal diet quality entered puberty later than their counterparts with lower CCDI scores (higher vs. lower CCDI tertiles: age at M was 13.1 years (11.2-13.3) and 12.5 years (12.0-13.9)(P<0.01); VB was 13.8 years (12.1-14.8) and 13.4 years (12.5-15.1)（P=0.03), after adjusting for paternal education level, energy intake, and pre pubertal body fat. Conclusion Children with a higher diet quality during peri puberty entered puberty at a later age. Dietary guidance and intervention are needed for better dietary quality among children during pubertal transition.

Objective: To explore the prospective effect of dietary intake of total fat and fatty acids on menarcheal timing among girls,and to provide a theoretical basis for preventing the early puberty development of Chinese children. Methods: Using the data from 1997-2015 China Health and Nutrition Survey (CHNS), 1 240 girls aged 6-13 with menarche information, baseline dietary survey data and at least one follow up assessment were selected. Cox regression analysis was performed to examine the prospective effect of dietary intake of total fat and fatty acids before menarche on age at menarche. Results: The mean baseline age of the participants was (8.3±1.8). After adjustment for year of birth, residence, household income, dietary energy intake and body mass index Z score at baseline, girls in the highest quartile of intake of total fat and polyunsaturated fatty acid (PUFA) had a 30% and 34% higher probability of experiencing menarche at an earlier age than those in the lowest quartile [ HR(HR 95%CI )=1.30 (1.01~1.68)，1.34(1.05~1.70)]. After adjusting for the confounders, there were no correlations between the intake of saturated fatty acid (SFA) and monounsaturated fatty acid (MUFA) and the onset of menarche [ HR(HR 95%CI )=1.24(0.98~1.58),1.25(0.97~ 1.62 )]( P >0.05). Conclusion Higher dietary intake of total fat and PUFA before menarche may lead to earlier age at menarche and no correlation between intake of SFA and MUFA before menarche with age at menarche is found among Chinese girls.

Objective: To investigate the prospective effects of the consumption of protein and animal foods before menarche on the age at menarche among Chinese girls. Methods: This paper was based on the data collected in the China Health and Nutrition Survey(CHNS) from 1997 to 2015. A total of 683 girls aged 6 and over who had completed information on age at menarche, height, weight, per capita annual household income, maternal education level and participated in at least one complete dietary survey within 1 to 4 years before menarche were included. Urban-rural stratified multivariable linear regression model was used to examine the effects of protein and animal foods intake before menarche on Chinese girls age at menarche in urban and rural areas. Results: After adjusted for total energy intake, body mass index standard deviation score and per capita annual household income, the consumption of meat before menarche was negatively associated with the age at menarche among rural Chinese girls(B=-0.003, P=0.00), but not among urban Chinese girls(B=0.002, P>0.05). Total protein, dairy, eggs and aquatic products intake before menarche were not associated with Chinese girls age at menarche in urban and rural areas(B=0.002, -0.001, 0.003, 0.000; 0.001, 0.001, -0.001, -0.003, P>0.05). Conclusion Higher intake of meat before menarche might lead to earlier menarche onset in rural Chinese girls. The consumption of total protein, dairy, eggs, and aquatic products before menarche did not affect the age at menarche in Chinese girls.

Objective To explore the situation of sedentary behavior and how it correlated to overweight/obesity among children and adolescents in Chengdu,China.Methods A total of 2 211 children and adolescents aged 7-15 years old were recruited in Chengdu city by using the cluster random sampling method.This was a cross-sectional study which aimed to collect data on anthropometry including height and weight and information on basic characters and sedentary behavior by using a self-designed questionnaire.Results According to IOTF criteria,the reporting rate of factor as ‘watching TV ≥2 hours per day on weekend’ showed statistically significant differences among normal,overweight and obese groups (34.80％,4.21％ and 1.92％ respectively,P=0.016 0) in boys.The reporting rates of ‘doing homework ≥2 hours per day’ were 12.17％,0.89％ and 0.49％ respectively (P=0.036 0) and statistically different among the three groups,in girls.Similar results were obtained in boys based on the WGOC criteria.However,there was no statistical difference seen on sedentary behaviors among the three groups in girls according to WGOC criteria.After stratified by age groups,rates on ‘ watching TV on weekend’ were statistically different among normal,overweight and obese group in 13-15 age group in girls (P=0.035 1).Physical activity level (PAL) was positively associated with WHR among both boys and girls when grouped according to PAL (Schofield).However,it was positively associated with WHR only seen in boys according to Chinese Nutrition Society (CNS) criteria.Conclusion The reporting rates of sedentary behavior in obese children were higher than that in overweight/normal ones.However,the correlation of sedentary behaviors on overweight/obesity called for further studies.

Objective To develop Chinese Children Dietary Index,the overall diet quality of children and adolescents; the association between overall diet quality and overweight/obesity among children in Chengdu.Methods Chinese Children Dietary Index was formulated based on the current Chinese Dietary Guidelines and Food Guide Pagoda for children and adolescents,consisting of 12components.1 997 children and adolescents aged 7-15 years in Chengdu were recruited and their diet quality was evaluated according to Chinese Food Guide Pagoda.Differences in percent body fat (PBF),fat mass index (FMI),fat-free mass index (FFMI) between 3 categories as low,moderate,and high diet quality,were tested.Results The mean Chinese Children Dietary Index score for our population was 59.36 ± 11.50.The diet quality of 13-15 years old adolescents was the worst.7-9years old children with moderate diet quality had highest PBF (P=0.04),while 10-12 years old children with higher diet quality had the lowest FFMI (P=0.002).Conclusion The overall diet quality of children and adolescents in Chengdu could be reflected by Chinese Children Dietary Index.There was association between overall diet quality and overweight/obesity among children and adolescents.However,this association warrants more in depth evaluation in future studies.

Based on the data from China Health and Nutrition Survey (CHNS), 840 girls aged 6-15 years who had information about menarche and participated in dietary survey at least once within 1-5 years before onset of menarche between 1989 and 2015 were included in the study. The median age at baseline of the 840 participants was 10 (9, 11) years. The median age at menarche of the participants was 13 (12, 14) years. After adjusting for the age of birth, place of residence, body mass index Z-score, physical activity level and annual household income per capita, the average age of menarche was advanced by 0.036 (95% CI: -0.068, -0.004) years for every 10 g increase in daily fat intake before menarche. The average age at menarche was 0.008 (95% CI: -0.014, -0.001) years earlier for every 1% increase in the daily energy supply ratio of fat before menarche.

Based on the data from China Health and Nutrition Survey (CHNS), 840 girls aged 6-15 years who had information about menarche and participated in dietary survey at least once within 1-5 years before onset of menarche between 1989 and 2015 were included in the study. The median age at baseline of the 840 participants was 10 (9, 11) years. The median age at menarche of the participants was 13 (12, 14) years. After adjusting for the age of birth, place of residence, body mass index Z-score, physical activity level and annual household income per capita, the average age of menarche was advanced by 0.036 (95% CI: -0.068, -0.004) years for every 10 g increase in daily fat intake before menarche. The average age at menarche was 0.008 (95% CI: -0.014, -0.001) years earlier for every 1% increase in the daily energy supply ratio of fat before menarche.

Objective To examine the association of pre?pregnancy body mass and weight gain during pregnancy with macrosomia. Methods From January 2015 to December 2015, a total of 20 477 pregnant women were recruited by probabilistic proportional scale sampling with simple randomization in Sichuan, Yunnan and Guizhou Provinces. Basic information of pregnant women, weight gain during pregnancy and weight of newborn were collected. A multiple logistic regression model was used to assess the association between the pre?pregnancy body mass and gestational weight gain indicators with macrosomia. Results 20 321 mother?infant were included in the final analysis. 20 321 pregnant women were (30.09 ± 4.10) years old and delivered at (39.20 ± 1.29) weeks, among which 12 341 (60.73%) cases were cesarean delivery. The birth weight of 20 321 infants were (3 292.26 ± 431.67) grams, and 970 (4.77%) were macrosomia. The multiple logistic regression model showed that after adjusting for the age of women, compared to the normal weight group in the pre?pregnancy, the overweight and obesity group elevated the risk of macrosomia, with OR (95%CI) about 1.99 (95%CI: 1.69-2.35) and 4.05 (95%CI: 3.05-5.39), respectively. After adjusting for the age, the pre?pregnancy BMI, delivery weeks, delivery mode and infant's gender, compared to the weight?gain appropriate group, higher weight gain rate in the mid?pregnancy and excessive total gestational weight gain elevated the risk of macrosomia, with OR (95%CI) about 1.99 (95%CI:1.66-2.39) and 1.80 (95%CI: 1.55-2.08), respectively. Conclusion The overweight before pregnancy, obesity before pregnancy, the rate of weight gain in the second trimester and the high total weight gain during pregnancy could increase the risk of macrosomia.