Immune dysregulation is a critical factor in the pathogenesis of inflammatory bowel disease (IBD), and numerous studies have focused on the impact of immune cell metabolic pathways. Immune system cells dynamically adapt to the microenvironment, regulating the body's response to external stimuli through intricate metabolic pathways. This review summarizes the mechanisms by which glycolysis, fatty acid and amino acid metabolisms influence immune metabolism and thereby modulate IBD progression, offering new perspectives for the diagnosis and treatment of IBD.

Autism spectrum disorder (ASD) is a neurodevelopmental condition originating in early life, presenting significant challenges for affected families and society. ASD is characterized by atypical brain development occurring throughout gestation. Maternal diet during pregnancy plays a crucial role in ASD pathogenesis. Specifically, prenatal multivitamin supplementation and adequate vitamin D intake may reduce ASD risk; similar protective effects have been associated with polyunsaturated fatty acid (PUFA) and iron consumption. Although research findings are inconsistent, prenatal nutritional interventions represent a promising strategy for mitigating the future risk of mental health and other disorders in offspring. Therefore, this article reviews key nutrients implicated in ASD risk during pregnancy, to provide a theoretical foundation for developing precise perinatal nutritional intervention plans.

Immune dysregulation is a critical factor in the pathogenesis of inflammatory bowel disease (IBD), and numerous studies have focused on the impact of immune cell metabolic pathways. Immune system cells dynamically adapt to the microenvironment, regulating the body's response to external stimuli through intricate metabolic pathways. This review summarizes the mechanisms by which glycolysis, fatty acid and amino acid metabolisms influence immune metabolism and thereby modulate IBD progression, offering new perspectives for the diagnosis and treatment of IBD.

Autism spectrum disorder (ASD) is a neurodevelopmental condition originating in early life, presenting significant challenges for affected families and society. ASD is characterized by atypical brain development occurring throughout gestation. Maternal diet during pregnancy plays a crucial role in ASD pathogenesis. Specifically, prenatal multivitamin supplementation and adequate vitamin D intake may reduce ASD risk; similar protective effects have been associated with polyunsaturated fatty acid (PUFA) and iron consumption. Although research findings are inconsistent, prenatal nutritional interventions represent a promising strategy for mitigating the future risk of mental health and other disorders in offspring. Therefore, this article reviews key nutrients implicated in ASD risk during pregnancy, to provide a theoretical foundation for developing precise perinatal nutritional intervention plans.

Objective:To explore the efficacy and safety of ustekinumab (UST) in the treatment of refractory Crohn′s disease (CD) in children.Methods:The clinical features, diagnosis and treatment of 6 patients with children refractory CD and CD-like monogenic inflammatory bowel disease (IBD) who were treated with ustekinumab (UST) at the Department of Gastroenterology, Beijing Children′s Hospital affiliated to Capital Medical University from August 2021 to January 2023 were retrospectively analyzed. The relevant literatures were reviewed and summarized by searching the articles on the treatment of refractory CD in children with UST published in Wanfang database, CNKI, PubMed and Medline.Results:All the six children with refractory CD and CD-like monogenic IBD were boys. Among them, 1 case was diagnosed as IL-10RA receptor defect, 1 case was diagnosed as IPEX syndrome, and 1 case diagnosed as X-linked lymphoproliferative syndrome (XLP-2) . Two patients were treated with UST only, three patients were treated with UST combined with thalidomide until presented clinical remission, and one patient was treated with UST in combination with IFX. Six children were treated with UST for over 24 weeks, and all of them presented clinical response. After treatment for more than 24 weeks, 4 cases presented endoscopic response.None of the 6 patients had special adverse drug reactions during follow-up for 24 weeks. A total of 16 literatures were retrieved. Conclusion:UST may induce and maintain remission in children with refractory CD.

Objective:To explore the efficacy and safety of ustekinumab (UST) in the treatment of refractory Crohn′s disease (CD) in children.Methods:The clinical features, diagnosis and treatment of 6 patients with children refractory CD and CD-like monogenic inflammatory bowel disease (IBD) who were treated with ustekinumab (UST) at the Department of Gastroenterology, Beijing Children′s Hospital affiliated to Capital Medical University from August 2021 to January 2023 were retrospectively analyzed. The relevant literatures were reviewed and summarized by searching the articles on the treatment of refractory CD in children with UST published in Wanfang database, CNKI, PubMed and Medline.Results:All the six children with refractory CD and CD-like monogenic IBD were boys. Among them, 1 case was diagnosed as IL-10RA receptor defect, 1 case was diagnosed as IPEX syndrome, and 1 case diagnosed as X-linked lymphoproliferative syndrome (XLP-2) . Two patients were treated with UST only, three patients were treated with UST combined with thalidomide until presented clinical remission, and one patient was treated with UST in combination with IFX. Six children were treated with UST for over 24 weeks, and all of them presented clinical response. After treatment for more than 24 weeks, 4 cases presented endoscopic response.None of the 6 patients had special adverse drug reactions during follow-up for 24 weeks. A total of 16 literatures were retrieved. Conclusion:UST may induce and maintain remission in children with refractory CD.

Objective:To investigate the clinical phenotype and prognosis among different genotypes of progressive familial intrahepatic cholestasis(PFIC) by cases analysis.Methods:The PFIC cases diagnosed at Beijing Children′s Hospital from 2015 to 2022 were collected, and the clinical phenotypic characteristics, treatment and prognosis were compared and analyzed.Results:A total of 628 cases of cholestatic liver disease were diagnosed, and 26 cases of PFIC were found, accounting for 4.1%.The number of PFIC 2 were the most, 14(53.8%)cases; three(11.5%) cases were PFIC 1; five(19.2%)cases were PFIC 3; while two(7.7%) cases were PFIC 4 and PFIC 6, respectively, and there was no case of PFIC 5.Type 1, 2, 4, and 6 had early onset ages(2 days to 21 months), while type 3 had a wide range of onset ages(8 to 145 months). The symptoms included jaundice(96.2%), pruritus(42.3%), and mucosal bleeding(15.4%). All three cases of type 1 had extrahepatic manifestations of diarrhea and malnutrition.Two cases of type 3 were found to have end-stage liver disease.Cases of PFIC 3 had increased serum γ-glutamyltransferase(97.2-439.5 U/L), while those of other types were normal.The bile acids were all increased(10.1-599.6 μmol/L). Abdominal ultrasound mainly showed liver enlargement(80.8%)and enhanced echogenicity of liver parenchyma(73.1%), enlargement of the spleen(61.5%). Ultrasound liver elastography ranged from 6.3 kPa to 23.1 kPa, there were 21(80.8%) cases ≥9 kPa.Among 26 cases, one case was lost to follow-up, and 11 cases were effective by oral medication alone.Fourteen children were still suffering from relapse or progress after drug treatment: four cases received liver transplantation (three cases had a good prognosis and one case died), two cases received biliary drainage, six cases were still taking drugs orally, and two cases died without active intervention in disease progress.Conclusion:Type 2 is the most common type in PFIC.The onset of most cases is in infancy.Jaundice, pruritus and hepatosplenomegaly are common clinical manifestations, and extrahepatic manifestations can be seen in type 1 cases.Type 3 cases can start with end-stage liver disease.Bile acid of all cases are increased.Except for type 3, the serum γ-glutamyltransferase of cases are normal.Oral medication has certain effects on some cases, but more than half progress, and some need biliary diversion or liver transplantation.

Objective:To summarize and analyze the clinical features of food protein-induced enterocolitis syndrome (FPIES).Methods:The medical history and follow-up data of 5 children with FPIES diagnosed in Department of Gastroenterology, Beijing Children′s Hospital of Capital Medical University from July 2018 to September 2019 were collected, and their clinical characteristics were summarized and analyzed.Results:Five children with FPIES were all infants, including 3 females and 2 males.Before the onset of the disease, the cases visited multiple departments and the average number of visits before diagnosis was 3.There were 4 cases of milk protein allergy and 1 case of egg white allergy.The patients had acute vomiting [5 cases (100%)], diarrhea [4 cases (80%)], early shock symptoms [5 cases (100%)], transient fever [2 cases (40%)]. Hematogenous leukocytes were increased in 3 cases (60%), C-reactive protein was increased in 1 case (20%), faecal leukocytes(+ )[2 cases (40%)], occult blood (+ ) [1 case (20%)]. Four cases were tested for food allergen specific IgE, of which 2 cases (40%) were positive for milk protein.After avoiding allergens, 3 patients (60%) needed intravenous rehydration treatment and 2 cases (40%) received oral rehydration treatment.The above 5 cases recovered quickly.Three patients (60%) used antibiotics.Four cases (80%) of the first-degree relatives of FPIES had a clear history of allergy.Families of children with FPIES had low awareness of the disease before the diagnosis was made, and the allergens were strictly avoided according to the doctor′s instructions after the diagnosis was made.Similar allergic reactions did not occur again, and complementary foods were gradua-lly added under the guidance of the doctor.Two patients had multiple food allergies.The body weight and length of 2 children with growth retardation were catching up with each other.Conclusions:FPIES is a serious food allergy related gastrointestinal disease which is easy to be misdiagnosed clinically.The diagnosis requires a combination of the family and personal allergy history, diet records, the characteristic performance of disease onset, the effect of diet avoi-dance and the necessary differential diagnosis.The long-term management and monitoring after diagnosis is also very important.

Objective:To investigate endoscopic and clinical characteristics of intestinal Behcet′s disease (BD) in children.Methods:General information, clinical manifestations, laboratory tests, imaging examinations and endoscopic characteristics of 14 children with intestinal BD treated in the Department of Gastroenterology, Beijing Children′s Hospital Affiliated to Capital Medical University from January 2016 to March 2020 were retrospectively analyzed.Results:Fourteen children with intestinal BD were recruited, involving 5 males and 9 females with the age of 1 month to 12 years and 11 months [(6.68±3.73) years old], and a median disease course of 6 months.All of them had gastrointestinal symptoms, including 11 cases with abdominal pain, 7 cases with diarrhea and 2 cases with vomiting.Complications of children with intestinal BD included gastrointestinal bleeding, intestinal perforation and stenosis.Extragastrointestinal symptoms included recurrent oral aphthosis in all the 14 cases, fever in 11 cases, skin lesions in 9 cases, vascular manifestations in 8 cases, positive pathergy test in 6 cases, joint manifestations in 5 cases, neurological manifestations in 1 case, and malnutrition in 6 cases.Observed by the endoscopy, lesions were mainly distributed in the distal ileum [61.5%(8/13 cases)]and ileocecal part[53.8%(7/13 cases)], with the main manifestation of ulcer.There were 3 cases with single ulcer and 10 cases with multiple ulcers, including 7 cases with deep and large ulcers.Pathological examinations of endoscopic lesions showed that the main features were mucosa chronic nonspecific inflammation, mucosa chronic active inflammation with ulcer and vasculitis.Conclusions:Clinical manifestations of intestinal BD vary a lot and are non-specific.Some children with intestinal BD may develop severe complications.Endoscopic lesions of intestinal BD have certain characteristics, which contribute to the diagnosis.Gastroenterologists need to be fully aware of intestinal BD.

Objective:To evaluate the clinical efficacy and safety of Infliximab (IFX) in pediatric Crohn′s disease (CD).Methods:The efficacy of IFX therapy in 30 patients suffering from active CD who were not completely improved with traditional medicine and enteral nutrition or had intolerance to the medicine in Beijing Children′s Hospital Affiliated to Capital Medical University from December 2014 to December 2019 were retrospectively analyzed.Pediatric Crohn′s Disease Activity Index (PCDAI), blood biochemistry indices, mucosal healing, nutritional status, and adverse reactions were compared and evaluated.Results:Thirty active CD cases, with 18 males and 12 females, were enrolled, and the average age was (8.63±4.76) years old.Three cases who didn′t complete 3 times of IFX injection and 1 case who lost to be followed up were excluded.A total of 26 cases of CD in active period were enrolled in this study on efficacy.The clinical remission and response rate of 26 cases were 61.5% and 84.6%, respectively, at 14-week of IFX therapy.The clinical remission and response rate of 21 cases were 71.4% and 85.7%, respectively, at 30-week.The clinical remission and response rate of 15 cases were 86.7% and 93.3%, respectively, at 54-week.At week 14 th, PCDAI score [(9.56±8.05) scores vs.(29.02±10.86) scores] decreased compared with before treatment ( t=7.339, P<0.05). The levels of erythrocyte sedimentation rate [(15.54±10.26) mm/1 h vs.(33.77±21.30) mm/1 h] and C-reactive protein [(4.79±12.94 ) mg/L vs.(16.33±23.43) mg/L] were obviously decreased, and the hemoglobin [(126.27±16.51) g/L vs.(110.58±16.45) g/L], hematocrit [(37.03±3.95)% vs.(33.52±4.32)%], and albumin levels [(42.30±3.03) g/L vs.(37.13±5.68) g/L] were remarkably increased compared with those before treatment ( t=3.932, 1.993, -3.398, -3.060, -4.009, all P<0.05). Height for age Z score and body mass index Z score were increased after IFX treatment, without statistically significant differences (all P>0.05). Conclusions:IFX therapy had good clinical efficacy in controlling inflammatorys and inducing clinical remission in pediatric CD.