"Hot Cross Bun" Sign in a Patient with Glutamic Acid Decarboxylase 65-KDa Isoform Associated Cerebellar Ataxia: Case Report and Review of the Literature.

The "hot cross bun" sign (HCBs) is a cruciform hyperintensity on T2-weighted imaging within the pons initially found in patients diagnosed as multiple system atrophy. However, recent findings have broadened the disease spectrum presented with HCBs. Here is a case report at an academic medical center. Cerebral magnetic resonance imaging (MRI), electroneuromyography, serum, and CSF analysis were performed. Literature is comprehensively reviewed. We investigated a woman presented with blurred speech and cerebellar ataxia. Her MRI showed the vertical line of HCBs 2 weeks after disease onset and gradually enhanced, presenting as an intact HCBs in a year. Glutamic acid decarboxylase 65-kDa isoform (GAD65) antibody IgG was detected in serum and CSF. The patient was diagnosed as GAD65 associated cerebellar ataxia and treated with corticosteroid and rituximab. We found 6 previously reported autoimmune cerebellar ataxia patients with HCBs. Anti-KLHL-11, anti-Homer-3, anti-Ri, and anti-Amphiphysin were associated. All patients had cerebellar ataxia with other neurological symptoms. Five patients were diagnosed with tumor. First-line immunotherapy including corticosteroid, intravenous immunoglobulin, and plasma exchange for most patients was unsatisfied. This case highlights the importance of considering GAD65 IgG evaluation in patients with progressive cerebellar syndrome and HCBs. Early diagnosis and therapy are challenging but imperative. Further studies are required in regard to therapeutic management.

Favorable Outcomes in a Case of Non-paraneoplastic DNER Ataxia Treated with Immunotherapy.

Anti-DNER antibody is associated with paraneoplastic cerebellar degeneration (PCD) and Hodgkin's disease (HD). However, recent studies reported cases absence of HD and that non-tumor anti-DNER antibody-associated ataxia was not well characterized. We present a case of acute cerebellar ataxia and nystagmus with detected anti-DNER antibody. Brain magnetic resonance imaging (MRI) showed cerebellar atrophy. High titer of anti-DNER antibody was detected in CSF and serum. Positron emission tomography (PET) scanning was unremarkable at a 10-month follow up. The patient improved significantly after immunosuppressive therapy with intravenous steroids, immunoglobulin followed by rituximab. Our study suggest that the presence of such anti-neuronal antibodies might not come along with malignancy and that early onset non-tumor patients are more likely to have a better outcome after immunotherapy. Early diagnosis and timely immunosuppressive therapy may prove beneficial for these patients.

Neuronal intranuclear inclusion disease mimicking progressive supranuclear palsy.

BACKGROUND: Given the variable nature of clinical manifestations, neuronal intranuclear inclusion disease (NIID) is regarded as a heterogeneous disease which is challenging to diagnose early. To the present, progressive supranuclear palsy (PSP)-like symptoms have never been listed in the performance of NIID. CASE PRESENTATION: A 58-year-old man presented with progressive Parkinsonism and postural instability for 3 years. Initially, he was considered as probable PSP due to vertical supranuclear gaze palsy, postural instability, and hummingbird sign. No high-intensity signal on diffusion-weighted imaging (DWI) was revealed. Eventually, the diagnosis was revised to NIID by Notch 2 N-terminal like C (NOTCH2NLC) GGC repeat expansions and skin biopsy showing intranuclear eosinophilic inclusions in the vesicles and ductal epithelial cells of sweat glands. CONCLUSION: Even if the typical high-intensity along the corticomedullary junction (CMJ) on DWI is lacking, clinicians should be alert to the possibility of NIID when PSP-like symptoms develop. This case report offers new features of NIID and expands its clinical spectrum.

[Sugar-sweetened beverage intakes and age of menarche in Chinese girls in 2004-2015].

OBJECTIVE: To investigate the prospective association between sugar-sweetened beverage intakes and age at menarche among Chinese girls. METHODS: Based on China Health and Nutrition Survey(CHNS) from 2004 to 2015, 293 girls aged 7-16 years with both data on sugar-sweetened beverage intake 1-5 years before menarche and age of menarche were included in the present study. Multivariate linear regression analysis and Logistic regression analysis were used to explore the influence of the sugar-sweetened beverage consumption on menarcheal age of Chinese girls. RESULTS: The median age at menarche of girls was 12 years old. The consumption of sugar-sweetened beverages(SSBs) was 0.5(0.2, 1.0) L/week for sweetened soft drinks, 0.5(0.2, 0.9) L/week for sweetened fruit drinks and 0.6(0.3, 1.3) L/week for total. After adjusting age at baseline, residency, maternal educational, level, energy intake at baseline and body mass index standard deviation score, no significant association was found between sweetened soft drinks, sweetened fruit drinks, total sugar-sweetened beverage intake and age at menarche. CONCLUSION: The intake of sugary beverages may not be prospectively related to the age of menarche among Chinese girls.

High quality of an absolute phase reconstruction for coherent digital holography with an enhanced anti-speckle deep neural unwrapping network.

It is always a challenge how to overcome speckle noise interference in the phase reconstruction for coherent digital holography (CDH) and its application, as this issue has not been solved well so far. In this paper, we are proposing an enhanced anti-speckle deep neural unwrapping network (E-ASDNUN) approach to achieve high quality of absolute phase reconstruction for CDH. The method designs a special network-based noise filter and embeds it into a deep neural unwrapping network to enhance anti-noise capacity in the image feature recognition and extraction process. The numerical simulation and experimental test on the phase unwrapping reconstruction and the image quality evaluation under the noise circumstances show that the E-ASDNUN approach is very effective against the speckle noise in realizing the high quality of absolute phase reconstruction. Meanwhile, it also demonstrates much better robustness than the typical U-net neural network and the traditional phase unwrapping algorithms in reconstructing high wrapping densities and high noise levels of phase images. The E-ASDNUN approach is also examined and confirmed by measuring the same phase object using a commercial white light interferometry as a reference. The result is perfectly consistent with that obtained by the E-ASDNUN approach.

Development of the Chinese preschooler dietary index: a tool to assess overall diet quality.

BACKGROUND: Diet quality in early childhood has a long-term impact on health outcomes. However, there are scarce dietary indexes for Chinese preschool children, and the existing indexes had limited validity and reliability. This study thus aimed to develop a dietary index for preschool children based on the Chinese Dietary Guideline and Chinese Dietary Reference Intakes and to assess their overall diet quality using the China Health and Nutrition Survey (CHNS). METHODS: The Chinese Preschooler Dietary Index (CPDI) included 11 components, covering 9 food group components and two nutrient components. The total scores of CPDI ranged from 0 to 90, with a higher score indicating greater diet quality. This study assessed the diet quality of 1742 preschoolers aged two to five years old from CHNS using the CPDI. Dietary intake data were obtained using three-day 24-h diet recalls, and sociodemographic information was also collected. Cochran-Mantel-Haensel (CMH) test was used to explore the association between demographic and CPDI total scores. The principal component analysis, correlation analysis and Cronbach's alpha were used to evaluate the relative reliability and validity of the CPDI. Finally, a stepwise multiple regression analysis was performed to explore potential influencing factors of CPDI. RESULTS: Among the 1742 CHNS preschool children, more than 70% resided in rural areas and 41.2% of the sample were raised in a low-income family. The mean CPDI score of the preschoolers was 38.8 ± 12.9. Higher diet scores were correlated with higher energy and nutrient intake. Children with higher age (ß = 0.93, SE = 0.26, P = 0.0003), raised in a home with higher household income (ß = 3.11, SE = 0.27, P < 0.0001) or living in urban areas (ß = -4.44, SE = 0.66, P < 0.0001) were associated with higher CPDI scores. CONCLUSIONS: The CPDI is useful in evaluating the diet quality of preschool children. Based on the CPDI, the diet quality of Chinese preschoolers needs to be improved, especially in rural areas.

Smek1 deficiency exacerbates experimental autoimmune encephalomyelitis by activating proinflammatory microglia and suppressing the IDO1-AhR pathway.

BACKGROUND: Experimental autoimmune encephalomyelitis (EAE) is an animal disease model of multiple sclerosis (MS) that involves the immune system and central nervous system (CNS). However, it is unclear how genetic predispositions promote neuroinflammation in MS and EAE. Here, we investigated how partial loss-of-function of suppressor of MEK1 (SMEK1), a regulatory subunit of protein phosphatase 4, facilitates the onset of MS and EAE. METHODS: C57BL/6 mice were immunized with myelin oligodendrocyte glycoprotein 35-55 (MOG35-55) to establish the EAE model. Clinical signs were recorded and pathogenesis was investigated after immunization. CNS tissues were analyzed by immunostaining, quantitative polymerase chain reaction (qPCR), western blot analysis, and enzyme-linked immunosorbent assay (ELISA). Single-cell analysis was carried out in the cortices and hippocampus. Splenic and lymph node cells were evaluated with flow cytometry, qPCR, and western blot analysis. RESULTS: Here, we showed that partial Smek1 deficiency caused more severe symptoms in the EAE model than in controls by activating myeloid cells and that Smek1 was required for maintaining immunosuppressive function by modulating the indoleamine 2,3-dioxygenase (IDO1)-aryl hydrocarbon receptor (AhR) pathway. Single-cell sequencing and an in vitro study showed that Smek1-deficient microglia and macrophages were preactivated at steady state. After MOG35-55 immunization, microglia and macrophages underwent hyperactivation and produced increased IL-1ß in Smek1-/+ mice at the peak stage. Moreover, dysfunction of the IDO1-AhR pathway resulted from the reduction of interferon γ (IFN-γ), enhanced antigen presentation ability, and inhibition of anti-inflammatory processes in Smek1-/+ EAE mice. CONCLUSIONS: The present study suggests a protective role of Smek1 in autoimmune demyelination pathogenesis via immune suppression and inflammation regulation in both the immune system and the central nervous system. Our findings provide an instructive basis for the roles of Smek1 in EAE and broaden the understanding of the genetic factors involved in the pathogenesis of autoimmune demyelination.

The overall diet quality in childhood is prospectively associated with the timing of puberty.

PURPOSE: The influences of nutrition in childhood on puberty onset could have sustained consequences for health and wellbeing later in life. The aim of this study was to investigate the prospective association of diet quality prior to puberty with the timing of puberty onset. METHODS: We considered data from 3983 SCCNG (Southwest China Childhood Nutrition and Growth) study participants with dietary data, anthropometric measurement, and information on potential confounders at their baseline assessment (mean age: 7.1 years for girls and 7.3 years for boys; mean length of follow-up was 4.2 years). Cox proportional hazard regression estimating hazard ratios (HRs) and 95% confidence intervals (CIs) were used to examine the relationship between diet quality and puberty onset. Dietary intake at baseline 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 (theoretical range: 0-160 points). Age at Tanner stage 2 for breast/genital development (B2/G2), menarche or voice break (M/VB) were used as pubertal markers. RESULTS: The CCDI score ranged from 56.2 to 136.3 for girls and 46.1-131.5 for boys. Pubertal markers consistently indicate that girls and boys with higher diet quality were more likely to enter their puberty later than their counterparts with lower CCDI scores (higher vs. lower CCDI tertiles: adjusted HR for age at B2: 0.85 (95% CI, 0.81-0.94), p for trend = 0.02; G2: 0.86 (95% CI,0.80-0.96), p for trend = 0.02; M: 0.86 (95% CI,0.80-0.95), p for trend = 0.02; VB: 0.86 (95% CI,0.79-0.98), p for trend = 0.03), after adjustment for paternal education level, baseline energy intake, and pre-pubertal body fat. CONCLUSIONS: Our data suggested a later puberty onset and later timing of progressed puberty stages in children with a high diet quality, which were independent of pre-pubertal body fat.

Beyond protein intake: does dietary fat intake in the year preceding pregnancy and during pregnancy have an impact on gestational diabetes mellitus?

PURPOSE: Studies regarding the association between dietary fat intake and gestational diabetes mellitus (GDM) are limited and provide conflicting findings. Thus, the study aims to examine the association of dietary fat intake in the year preceding pregnancy and during pregnancy with the risk of GDM, taking the relevance of dietary protein intake on GDM into consideration. METHODS: A prospective study was conducted in 6299 singleton pregnancies, using the data from the Nutrition in Pregnancy and Growth in Southwest China (NPGSC). A validated food frequency questionnaire was used to assess dietary fat intake in the year preceding pregnancy and during the first and second trimesters of pregnancy. Logistic regression analysis was used to assess the prospective associations of dietary fat intake and the type and source of dietary fats in different time windows with GDM risk. RESULTS: Higher intake of total fat [OR (95% CI): 2.21 (1.19-4.20), P = 0.02] during 12-22 weeks of gestation was associated with higher GDM risk. However, adjustment for animal protein intake greatly attenuated this association [OR (95% CI): 1.81 (0.93, 3.64), P = 0.11]. Total fat intake neither in the year preceding pregnancy nor during the early pregnancy was associated with GDM risk. Moreover, insignificant associations were observed between intakes of vegetable fat, animal fat, cholesterol, saturated fatty acid, monounsaturated fatty acid and polyunsaturated fatty acid one year before pregnancy and during the first and second trimesters and GDM risk. CONCLUSION: Our study indicated that dietary fat intake one year before pregnancy and across the two pregnancy trimesters preceding the diagnosis of GDM has no relevance on GDM risk among Chinese women, particularly those with normal BMI, low, or normal calorie intake.

Correction to: A De Novo Frameshift Mutation in TNFAIP3 Impairs A20 Deubiquitination Function to Cause Neuropsychiatric Systemic Lupus Erythematosus.

Figure 4a in Manuscript ID#JOCI-D-19-00318 has been revised due to the replacement of immunoblot lane of ß-catenin by Zo-1 in NHA group.

Nutrition in Pregnancy and Growth in Southwest China (NPGSC) cohort: Design, implementation, and characteristics.

BACKGROUND: Pregnancy and birth cohorts addressing maternal nutrition and its impact on health outcomes have been rare in China, especially in Southwest China. OBJECTIVES: To describe the design, implementation, baseline characteristics, and initial results of the Nutrition in Pregnancy and Growth in Southwest China (NPGSC) cohort. POPULATION: Pregnant women with their children in Southwest China. DESIGN: NPGSC participants have been prospectively recruited since 2014. Pregnant women were invited to participate in the study at their first routine ultrasound examination in gestational weeks 9-11. Data were assessed three times during pregnancy (9-11, 20-22, and 33-35 gestation weeks), and eight times in infants and toddlers. METHODS: Pre-pregnancy body weight and height were self-reported; gestational weight gain was measured at regular intervals. Both food frequency questionnaires (FFQ) and 24-hour dietary recalls were used to collect dietary intakes during pregnancy, and FFQ for diet before pregnancy. Information on pregnancy outcomes was extracted from the medical birth registry. Anthropometry of children in the first 3 years of life was measured by trained investigators. Other child outcomes, including feeding practices (self-reported by mothers) and cognitive development (assessed by the Chinese version of Ages and Stages Questionnaire), were recorded. PRELIMINARY RESULTS: Between 2014 and 2018, 12 989 pregnant women were enrolled, and 2296 children completed the 3 years follow-up. Among them, 115 pregnancies ended in stillbirth. Mean maternal pre-pregnancy BMI was 21.1 kg/m2 and mean gestational weight gain was 13.5 kg 18.6% of mothers developed gestational diabetes and 1.5% of mothers were diagnosed with preeclampsia. Mean birthweight and birth length of children were, respectively, 3329 g and 49.4 cm. CONCLUSION: We built a prospective cohort in Southwest China, which can provide valuable data to investigate the relevance of nutrition for the health of mothers and children.

WDFY4 Is Involved in Symptoms of Systemic Lupus Erythematosus by Modulating B Cell Fate via Noncanonical Autophagy.

Genome-wide association studies have recently illuminated that WDFY4 is genetically associated with systemic lupus erythematosus (SLE) susceptibility in various ethnic groups. Despite strong genetic evidence suggesting a role of WDFY4 in SLE pathogenesis, its functional relevance is largely unknown. In this study, we generated Wdfy4 B lymphocyte conditional knockout (Wdfy4-CKO) mice and found that loss of Wdfy4 led to a decrease in number of total B cells and several subpopulations of B cells in the periphery and a defect in the transition from the pro- to pre-B cell stage in bone marrow. Also, Wdfy4-CKO mice showed impaired Ab responses as compared with controls when challenged with Ag. SLE phenotypes were effectively alleviated in Wdfy4-CKO mice, with significantly diminished pristane-elicited production of autoantibodies and glomerulonephritis. Genetic silencing of WDFY4 in B cells increased lipidation of LC3 independent of p62 and Beclin1, which are essential proteins of canonical autophagy. Our in vivo and in vitro data suggest that WDFY4 facilitates noncanonical autophagic activity. Our findings provide a novel functional link underlying the mechanism of SLE in which WDFY4 influences B cell fate via noncanonical autophagy.

Modern dietary pattern is prospectively associated with earlier age at menarche: data from the CHNS 1997-2015.

BACKGROUND: Early age at menarche is associated with risk of several chronic diseases. Prospective study on the association between dietary pattern and timing of menarche is sparse. We examined whether dietary patterns prior to the menarche onset were prospectively associated with menarcheal age in Chinese girls. METHODS: One thousand one hundred eighteen girls aged 6-13 y in the China Health and Nutrition Survey (CHNS) with three-day 24-h recalls and information on potential confounders at baseline were included in the study. Dietary patterns were identified using principal component analysis. Age at menarche was self-reported at each survey. Cox proportional hazard regression models were performed to examine the associations of premenarcheal dietary patterns and menarcheal timing. Hazard ratios (HRs) and 95% confidence intervals (CIs) were calculated. RESULTS: Three major dietary patterns were identified: modern dietary pattern, animal food pattern, and snack food pattern. After adjustment for age at baseline, region, ethnicity, maternal education level, energy intake at baseline, and body mass index Z-score at baseline, girls in the highest quartile of modern dietary pattern score had a 33% higher probability of experiencing menarche at an earlier age than those in the lowest quartile (HR: 1.33, 95% CI: 1.002-1.77, p for trend = 0.03). No significant association was found for the animal food pattern or snack food pattern. CONCLUSIONS: Higher adherence to modern dietary pattern during childhood is associated with an earlier menarcheal age. This association was independent of premenarcheal body size.

Cervical Spinal Involvement in a Chinese Pedigree With Pontine Autosomal Dominant Microangiopathy and Leukoencephalopathy Caused by a 3' Untranslated Region Mutation of COL4A1 Gene.

Background and Purpose- Pontine autosomal dominant microangiopathy and leukoencephalopathy, a recently defined subtype of cerebral small vessel disease, is associated with mutations in COL4A1 (collagen type IV alpha 1 chain) 3' untranslated region. We here describe a pontine autosomal dominant microangiopathy and leukoencephalopathy pedigree with COL4A1 mutation presenting both pontine and cervical spinal cord involvement. Methods- For the diagnostic purpose, brain and spinal magnetic resonance imaging scanning, skin biopsy, and whole-exome sequencing were performed on the patients in the pedigree. Suspected pathogenic variants were further confirmed by cosegregation analysis using Sanger sequencing in the family members. Results- We identified a mutation located at the binding site of miR-29 (microRNA-29) in 3' untranslated region of COL4A1(c.*32G>A). The pontine autosomal dominant microangiopathy and leukoencephalopathy patients in this pedigree carried this variant, whereas other healthy family members but one did not. Magnetic resonance imaging showed lesions in the pons, white matter, and cervical spinal cord. Skin biopsy revealed thickened basal lamina in vessels. Conclusions- For the first time, we reported cervical spinal involvement in pontine autosomal dominant microangiopathy and leukoencephalopathy and expanded the clinical spectrum of this disease.

A De Novo Frameshift Mutation in TNFAIP3 Impairs A20 Deubiquitination Function to Cause Neuropsychiatric Systemic Lupus Erythematosus.

PURPOSE: Genome-wide association study of systemic lupus erythematosus (SLE) revealed tumor necrosis factor alpha-induced protein 3 (TNFAIP3, A20) as a susceptibility gene. Here, we report a de novo mutation in TNFAIP3 in a Chinese patient with neuropsychiatric SLE (NPSLE). METHODS: Whole exome sequencing was performed for the patient and healthy members from the family. Suspected pathogenic variants were further analyzed and co-segregation was confirmed by Sanger sequencing. Real-time PCR and western blot were performed with peripheral blood mononuclear cells (PBMCs) and patient-derived T cells. Transfected HEK293T cells, human umbilical vein endothelial cells, normal human astrocytes, and microglia were used for in vitro studies. RESULTS: A de novo frameshift mutation in TNFAIP3 was found in the NPSLE patient. Western blot analysis showed activated NF-κB and mitogen-activated protein kinase pathways. Real-time PCR revealed elevated expression of pro-inflammatory cytokines. On immunoprecipitation assay, the mutant A20 altered the K63-linked ubiquitin level of TRAF6 via its ubiquitin-editing function. CONCLUSIONS: The mutant A20 may play a role in weakening the tight junction of the blood-brain barrier to cause neurologic symptoms. We report a rare variant of TNFAIP3 in a patient with NPSLE and reveal its autoimmune disease-causing mechanism in both peripheral tissues and the central nervous system.

Whole-Genome Linkage Analysis with Whole-Exome Sequencing Identifies a Novel Frameshift Variant in NEFH in a Chinese Family with Charcot-Marie-Tooth 2: A Novel Variant in NEFH for Charcot-Marie-Tooth 2.

BACKGROUND: Charcot-Marie-Tooth disease (CMT) is the most common neurodegenerative disorder of the peripheral nervous system. More than 50 genes/loci were found associated with the disease. We found a family with autosomal-dominant CMT2. OBJECTIVE: To reveal the pathogenic gene of the family and further investigate the function of the variant. METHODS: DNA underwent whole-genome linkage analysis for all family members and whole-exome sequencing for 2 affected members. Neurofilament light polypeptide and wild-type or mutant neurofilament heavy polypeptide (NEFH) were co-transfected into SW13 (vim-) cells. The nefh-knockdown zebrafish model was produced by using morpholino antisense oligonucleotides. RESULTS: We identified a novel insertion variant (c.3057insG) in NEFH in the family. The variant led to the loss of a stop codon and an extended 41 amino acids in the protein. Immunofluorescence results revealed that mutant NEFH disrupted the neurofilament network and induced aggregation of NEFH protein. Knockdown of nefh in zebrafish caused a slightly or severely curled tail. The motor ability of nefh-knockdown embryos was impaired or even absent, and the embryos showed developmental defects of axons in motor neurons. The abnormal phenotype and axonal developmental defects could be rescued by injection of human wild-type but not human mutant NEFH mRNA. CONCLUSIONS: We identified a novel stop loss variant in NEFH that is likely pathogenic for CMT2, and the results provide further evidence for the role of an aberrant assembly of neurofilament in CMT.

[Consumptions of Meat, Dietary Fat, and Fatty-acids and Prevalence of Overweight/Obesity in Children and Adolescents-a Cross-sectional Survey in Chengdu].

OBJECTIVES: To determine the prevalence of overweight and obesity in children and adolescents aged 7-15 years in Longquanyi District of Chengdu and its association with consumptions of meat, dietary fat and fatty-acids. METHODS: A total of 1 811 children and adolescents aged 7-15 years in Longquanyi District were selected using stratified cluster sampling strategy. Their body mass, height and waist circumference were measured. The prevalence of overweight/obesity was estimated based on body mass index (BMI), body mass index standard deviation score (BMI SDS), and waist-to-height ratio (WHtR). Daily consumptions of meat, dietary fat and fatty-acids were calculated using data collected through a food frequency questionnaire and 3-d 24 h dietary recall. The children with overweight/obesity were compared with those with normal body mass in food/nutrient consumptions using Wilcoxon tests. The BMI SDS, WHtR, and prevalence of overweight and obesity were also compared between those having low, moderate and high food/nutrient consumptions using Chi-square tests or Kruskal-Wallis tests. RESULTS: About 10.34% and 6.59% of participants were found to be overweight and obese, respectively. Boys had higher prevalence of overweight (12.05%) and overweight/obesity (18.97%) than girls (8.55%, 14.80%) ( P<0.05). Girls consumed more meat (including red meat and white meat), saturated fatty-acid (SFA) and monounsaturated fatty-acid (MUFA) than boys ( P<0.05). The consumptions of meat (both red meat and white meat), SFA and MUFA increased with age ( P<0.05). Overweight/obese girls consumed more SFA, MUFA and fat (%EN) than those of normal weight. The BMI SDS and WHtR of girls increased with fat (%EN) consumptions ( P<0.05). The BMI SDS of girls also increased with MUFA consumptions ( P<0.05). CONCLUSIONS: Consumptions of red meat, dietary fat, SFA, and MUFA are associated with overweight/obesity of girls aged 7-15 years in Chengdu. Further studies are needed to understand the gender differences.

[Dietary Fiber and Pubertal Development among Children and Adolescents--a Cross-sectional Study in Chengdu, Sichuan].

OBJECTIVE: To determine the association between intake of dietary fiber and pubertal development among children and adolescents in Chengdu. METHODS: A cross-sectional survey was undertaken in 1 340 children and adolescents aged 9-15 years. Data about dietary intake were collected through 24-h dietary self-recall. Pubertal development was measured by trained investigators using Tanner criteria. Consumptions of total fiber and fiber from different sources were compared among the participants with different stages of pubertal development. RESULTS: Data from 1 328 children and adolescents were analyzed. Boys (n = 667) at a later stage of pubertal development consumed less total fiber and fruit fiber than those at an earlier stage (P < 0.05). Similarly, girls (n = 651) at a later stage of pubertal development consumed less fruit fiber than those at an earlier stage (P < 0.05). CONCLUSION: Dietary fiber intake, especially fruit fiber, is lower in children and adolescents with early commencement of puberty development. Further studies are needed to establish the relationship between dietary fiber and pubertal development.

Caspase-1 inhibitor ameliorates experimental autoimmune myasthenia gravis by innate dendric cell IL-1-IL-17 pathway.

BACKGROUND: IL-1ß has been shown to play a pivotal role in autoimmunity. Cysteinyl aspartate-specific proteinase-1 (caspase-1) inhibitor may be an important drug target for autoimmune diseases. However, the effects of caspase-1 inhibitor on myasthenia gravis (MG) remain undefined. METHODS: To investigate the effects of caspase-1 inhibitor on experimental autoimmune myasthenia gravis (EAMG), an animal model of MG, caspase-1 inhibitor was administered to Lewis rats immunized with region 97-116 of the rat AChR α subunit (R97-116 peptide) in complete Freund's adjuvant. The immunophenotypical characterization by flow cytometry and the levels of autoantibody by ELISA were carried out to evaluate the neuroprotective effect of caspase-1 inhibitor. RESULTS: We found that caspase-1 inhibitor improved EAMG clinical symptom, which was associated with decreased IL-17 production by CD4+ T cells and γδ T cells, lower affinity of anti-R97-116 peptide IgG. Caspase-1 inhibitor decreased expression of CD80, CD86, and MHC class II on DCs, as well as intracellular IL-1ß production from DCs. In addition, caspase-1 inhibitor treatment inhibited R97-116 peptide-specific cell proliferation and decreased follicular helper T cells relating to EAMG development. CONCLUSIONS: Our results suggest that caspase-1 inhibitor ameliorates experimental autoimmune myasthenia gravis by innate DC IL-1-IL-17 pathway and provides new evidence that caspase-1 is an important drug target in the treatment of MG and other autoimmune diseases.