Doença rara de desregulação imunológica com imunodeficiência caracterizada por doença inflamatória intestinal grave e progressiva de início infantil com pancolite, doença perianal (ulceração, fístulas), infecções respiratórias, geniturinárias e cutâneas recorrentes, artrite e alto risco de linfoma de células B.
Introdução
O que você precisa saber de cara
Doença rara de desregulação imunológica com imunodeficiência caracterizada por doença inflamatória intestinal grave e progressiva de início infantil com pancolite, doença perianal (ulceração, fístulas), infecções respiratórias, geniturinárias e cutâneas recorrentes, artrite e alto risco de linfoma de células B.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
3 genes identificados com associação a esta condição. Padrão de herança: Autosomal recessive.
Shared cell surface receptor required for the activation of five class 2 cytokines: IL10, IL22, IL26, IL28, and IFNL1. The IFNLR1/IL10RB dimer is a receptor for the cytokine ligands IFNL2 and IFNL3 and mediates their antiviral activity. The ligand/receptor complex stimulate the activation of the JAK/STAT signaling pathway leading to the expression of IFN-stimulated genes (ISG), which contribute to the antiviral state
Membrane
Inflammatory bowel disease 25, autosomal recessive
A chronic, relapsing inflammation of the gastrointestinal tract with a complex etiology. It is subdivided into Crohn disease and ulcerative colitis phenotypes. Crohn disease may affect any part of the gastrointestinal tract from the mouth to the anus, but most frequently it involves the terminal ileum and colon. Bowel inflammation is transmural and discontinuous; it may contain granulomas or be associated with intestinal or perianal fistulas. In contrast, in ulcerative colitis, the inflammation is continuous and limited to rectal and colonic mucosal layers; fistulas and granulomas are not observed. Both diseases include extraintestinal inflammation of the skin, eyes, or joints.
Cell surface receptor for the cytokine IL10 that participates in IL10-mediated anti-inflammatory functions, limiting excessive tissue disruption caused by inflammation. Upon binding to IL10, induces a conformational change in IL10RB, allowing IL10RB to bind IL10 as well (PubMed:16982608). In turn, the heterotetrameric assembly complex, composed of two subunits of IL10RA and IL10RB, activates the kinases JAK1 and TYK2 that are constitutively associated with IL10RA and IL10RB respectively (PubMed:
Cell membraneCytoplasm
Inflammatory bowel disease 28, autosomal recessive
A chronic, relapsing inflammation of the gastrointestinal tract with a complex etiology. It is subdivided into Crohn disease and ulcerative colitis phenotypes. Crohn disease may affect any part of the gastrointestinal tract from the mouth to the anus, but most frequently it involves the terminal ileum and colon. Bowel inflammation is transmural and discontinuous; it may contain granulomas or be associated with intestinal or perianal fistulas. In contrast, in ulcerative colitis, the inflammation is continuous and limited to rectal and colonic mucosal layers; fistulas and granulomas are not observed. Both diseases include extraintestinal inflammation of the skin, eyes, or joints.
Major immune regulatory cytokine that acts on many cells of the immune system where it has profound anti-inflammatory functions, limiting excessive tissue disruption caused by inflammation. Mechanistically, IL10 binds to its heterotetrameric receptor comprising IL10RA and IL10RB leading to JAK1 and STAT2-mediated phosphorylation of STAT3 (PubMed:16982608). In turn, STAT3 translocates to the nucleus where it drives expression of anti-inflammatory mediators (PubMed:18025162). Targets antigen-prese
Secreted
Variantes genéticas (ClinVar)
174 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
9 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome de imunodesregulação-doença inflamatória do intestino-artrite-infecções recorrentes
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
From Gut to Metabolism: Exploring the Intersection of Inflammatory Bowel Disease and Systemic Metabolic Dysfunction.
A Doença Inflamatória Intestinal (DII) é uma condição sistêmica complexa, frequentemente coexistindo com disfunções metabólicas como obesidade e diabetes. Essa conexão agrava a DII, reduz a eficácia dos tratamentos biológicos e *aumenta o risco de infecções*, perpetuando um ciclo de desregulação imune e lesão metabólica. Para pacientes e médicos, isso ressalta a importância da triagem metabólica e de uma abordagem multidisciplinar para otimizar o manejo da DII, visando melhorar resultados e desenvolver terapias que atuem tanto na inflamação intestinal quanto na disfunção metabólica.
🇧🇷 traduzidoAre we missing early pancreatic dysfunction? Expanding faecal elastase testing beyond pancreatic disease.
A Insuficiência Pancreática Exócrina (IPE) é mais comum do que se pensa em pacientes com sintomas gastrointestinais inespecíficos, como os da Síndrome do Intestino Irritável (SII), podendo afetar 11-21% e coexistir com outras condições como doença celíaca e DII. O exame de elastase fecal-1 (FE-1) é uma ferramenta crucial para diagnosticar a IPE, que pode ser um marcador precoce de disfunção pancreática em alguns casos, cujos sintomas muitas vezes se sobrepõem a outras doenças. Médicos e pacientes devem considerar um rastreio mais amplo, pois a identificação precoce da IPE permite intervenções, como mudanças no estilo de vida, com potenciais benefícios a longo prazo, expandindo sua relevância para além das doenças pancreáticas tradicionais.
🇧🇷 traduzidoMetabotherapy for intestinal disease: using metabolites to prevent and treat disorders of the gut.
Este artigo destaca que pequenas moléculas no intestino, provenientes da dieta, microrganismos e do próprio corpo, são cruciais para a saúde digestiva e estão diretamente envolvidas no desenvolvimento de diversas doenças intestinais, como inflamação e câncer. A *metaboterapia*, que consiste em ajustar esses níveis de metabólitos por via oral, surge como uma estratégia terapêutica promissora e ainda subutilizada para prevenir e tratar um vasto leque de condições gastrointestinais, oferecendo novas abordagens para pacientes e médicos.
🇧🇷 traduzidoFusobacterium nucleatum plays a pathogenic role in a murine model of irritable bowel syndrome by modulating intestinal purine metabolism and promoting mast cell activation.
Um estudo em modelos murinos identificou a bactéria *Fusobacterium nucleatum* como um agente patogênico chave na Síndrome do Intestino Irritável (SII). Essa bactéria induz sintomas de SII ao ativar células imunes (mastócitos) através de uma alteração no metabolismo de purinas no intestino, que leva à produção de ácido úrico. Os resultados sugerem promissoras abordagens terapêuticas, incluindo o uso de antibióticos (como metronidazol) ou a modulação da atividade dos mastócitos, focando em pacientes com SII onde *F. nucleatum* possa estar presente.
🇧🇷 traduzidoIgA Nephropathy in Adults: A Review.
A Nefropatia por IgA (NIgA) é a doença glomerular imunomediada mais comum, afetando os rins e podendo levar à insuficiência renal em até metade dos casos em 10 anos. Geralmente diagnosticada em adultos jovens, é suspeitada por hematúria ou proteinúria e confirmada por biópsia renal em casos com proteinúria >0.5g/dia. O tratamento foca em modificações de estilo de vida, controle rigoroso da pressão arterial (alvo <120/70 mmHg), e terapias que visam reduzir a formação de imunocomplexos de IgA, diminuir a lesão glomerular e gerenciar a perda de néfrons.
🇧🇷 traduzidoPublicações recentes
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Inflammatory ILC2s migrate to distal tissues during infection using stage-specific S1P receptors.
Preparation and mechanism study of Alhagi honey polysaccharide-aluminum Pickering emulsion adjuvant for improving intestinal mucosal immune function.
LC3-associated phagocytosis in macrophage responses to Paracoccidioides spp.
Lactobacillus rhamnosus confers protection against enteropathogenic bacteria by enhancing mucosal immunity and epithelial barrier function.
📚 EuropePMCmostrando 198
Adherence to Recommended Immunisation Schedules for Patients With Inflammatory Bowel Disease (IBD) on Biologics: A Retrospective Study at Our Lady's Hospital, Navan, Ireland.
CureusFrom Gut to Metabolism: Exploring the Intersection of Inflammatory Bowel Disease and Systemic Metabolic Dysfunction.
Journal of clinical gastroenterologyBeyond the binary: understanding inflammatory bowel disease in the context of HIV-induced immunodeficiency.
Expert review of clinical immunologyAre we missing early pancreatic dysfunction? Expanding faecal elastase testing beyond pancreatic disease.
Current opinion in gastroenterologyMetabotherapy for intestinal disease: using metabolites to prevent and treat disorders of the gut.
Nature reviews. Gastroenterology & hepatologyCase Report: Tricho-hepato-enteric syndrome in an infant presented with colorectal ulceration and severe respiratory superinfection.
Frontiers in immunologyEmerging Therapeutic Approaches for Modulating the Intestinal Microbiota.
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Case reports in gastrointestinal medicineScience of fecal microbiota transplant: From history to cutting-edge clinical practice.
World journal of gastrointestinal endoscopyFusobacterium nucleatum plays a pathogenic role in a murine model of irritable bowel syndrome by modulating intestinal purine metabolism and promoting mast cell activation.
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Italian journal of pediatricsRecent Trend of Laboratory Tests in Common Gastrointestinal Tract Disorders.
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Seminars in arthritis and rheumatismMechanisms and Nanomedicine Interventions of Acute Lung Injury Induced by Intestinal Ischemia-Reperfusion: A Mini Review.
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Current issues in molecular biologyPsychological Stress-Induced Local Immune Response to Food Antigens Increases Pain Signaling Across the Gut in Mice.
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BMJ openTTC7A Variants Results in Gastrointestinal Defects and Immunodeficiency Syndrome: Case Series and Literature Review.
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Gut microbesHidradenitis suppurativa.
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Arteriosclerosis, thrombosis, and vascular biologyIntestine-Decipher Engineered Capsules Protect Against Sepsis-induced Intestinal Injury via Broad-spectrum Anti-inflammation and Parthanatos Inhibition.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Revealing the dance of NLRP3: spatiotemporal patterns in inflammasome activation.
Immunometabolism (Cobham, Surrey)Bovine Colostrum in Pediatric Nutrition and Health.
NutrientsProtective effect of Haoqin Qingdan decoction on pulmonary and intestinal injury in mice with influenza viral pneumonia.
Frontiers in pharmacologyClostridium butyricum, a future star in sepsis treatment.
Frontiers in cellular and infection microbiologyThe Intestine in Acute and Long COVID: Pathophysiological Insights and Key Lessons.
The Yale journal of biology and medicineThe microbiota metabolite, phloroglucinol, confers long-term protection against inflammation.
Gut microbesModifiable Psychological Factors are Associated With Clusters of Pain, Fatigue, Fecal Incontinence, and Irritable Bowel Syndrome-Type Symptoms in Inflammatory Bowel Disease: A Latent Profile Analysis.
Journal of Crohn's & colitisDysbiosis of gut microbiota in COVID-19 is associated with intestinal DNA phage dynamics of lysogenic and lytic infection.
Microbiology spectrumRapid systemic spread and minimal immune responses following SIVsab intrarectal transmission in African green monkeys.
JCI insightShared genetic architecture between COVID-19 and irritable bowel syndrome: a large-scale genome-wide cross-trait analysis.
Frontiers in immunologyRole of mucosal IgA antibodies as novel therapies to enhance mucosal barriers.
Seminars in immunopathologyReview of Immunologic Manifestations of COVID-19 Infection and Vaccination.
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Annals of human geneticsHidradenitis Suppurativa from a Multi-Omic Scope.
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Food & functionOmicron XBB.1.5 subvariant causes severe pulmonary disease in K18-hACE-2 mice.
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Inflammatory bowel diseasesComprehensive Catalog of Variants Potentially Associated with Hidradenitis Suppurativa, Including Newly Identified Variants from a Cohort of 100 Patients.
International journal of molecular sciencesHemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome in pediatric Inflammatory Bowel Disease: clinical characteristics and outcomes.
European journal of pediatricsRapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
Allergologie selectWhole Exome Sequencing Identifies Epithelial and Immune Dysfunction-Related Biomarkers in Food Protein-Induced Enterocolitis Syndrome.
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Frontiers in pharmacologyConstrictive Pericarditis and Protein-Losing Enteropathies: Exploring the Heart-Gut Axis.
Journal of clinical medicineEffects of Physical Exercise on the Microbiota in Irritable Bowel Syndrome.
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International journal of molecular sciencesInflammation: Is It a Healer, Confounder, or a Promoter of Cardiometabolic Risks?
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British journal of hospital medicine (London, England : 2005)Gastrointestinal system involvement in patients with primary immunodeficiency: a single center experience.
Scandinavian journal of gastroenterologyTuberculosis and Immune Reconstitution Inflammatory Syndrome in Patients With Inflammatory Bowel Disease and Anti-TNFα Treatment: Insights From a French Multicenter Study and Systematic Literature Review With Emphasis on Paradoxical Anti-TNFα Resumption.
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The Journal of rheumatologyClinical characteristics and the risk factors for the exacerbation of symptoms in patients with inflammatory bowel disease during the COVID-19 pandemic.
Frontiers in medicineDetection of SARS-CoV-2 Viral Genome and Viral Nucleocapsid in Various Organs and Systems.
International journal of molecular sciencesKnowledge, Attitudes, and Practices Regarding Herpes Zoster Vaccination Among Specialists.
Population health managementGuillain-Barré syndrome in ulcerative colitis and SARS-CoV-2 infection: a case report and literature review.
Future science OAThe role of interleukin-36 in health and disease states.
Journal of the European Academy of Dermatology and Venereology : JEADVGastrointestinal Conditions: Acute Infectious Gastroenteritis and Colitis.
FP essentialsCurrent Developments in the Prevention and Improvement of Intestinal Disorders: A Mini-Review.
Endocrine, metabolic & immune disorders drug targetsCoombs-Negative Hemolytic Anemia in an Elderly COVID-19 Patient.
CureusIFIH1 loss of function predisposes to inflammatory and SARS-CoV-2-related infectious diseases.
Scandinavian journal of immunologyDifferential Colonization and Mucus Ultrastructure Visualization in Bovine Ileal and Rectal Organoid-Derived Monolayers Exposed to Enterohemorrhagic Escherichia coli.
International journal of molecular sciencesMucosal vaccination in a murine gnotobiotic model of Giardia lamblia infection.
Infection and immunityPostprandial symptoms in disorders of gut-brain interaction and their potential as a treatment target.
GutCampylobacter jejuni virulence factors: update on emerging issues and trends.
Journal of biomedical scienceA step closer to understanding how a diet high in simple carbohydrates may cause dysbiosis.
The Journal of clinical investigationImpact of COVID-19 on Pediatric Inflammatory Bowel Diseases-From Expectations to Reality.
Journal of personalized medicinePost-COVID-19 syndrome in everyday clinical practice: interdisciplinary expert position statement endorsed by the Polish Society of Civilization Diseases.
Polish archives of internal medicineCytomegalovirus-Induced Hemophagocytic Lymphohistiocytosis in an Immunocompromised Patient with Inflammatory Bowel Disease.
Case reports in hematologyNociplastic pain mechanisms and toll-like receptors as promising targets for its management.
PainSymptoms and Severity of COVID-19 in Patients with Immune-Mediated Inflammatory Diseases: Experience of a University Medical Center.
Autoimmune diseasesAn updated advancement of bifunctional IL-27 in inflammatory autoimmune diseases.
Frontiers in immunologyThe association of three vaccination doses with reduced gastrointestinal symptoms after severe acute respiratory syndrome coronavirus 2 infections in patients with inflammatory bowel disease.
Frontiers in medicineExtracellular sombrero vesicles are hallmarks of eosinophilic cytolytic degranulation in tissue sites of human diseases.
Journal of leukocyte biologyAn improved mouse model of sepsis based on intraperitoneal injections of the enriched culture of cecum slurry.
Life sciencesSignatures of disease outcome severity in the intestinal fungal and bacterial microbiome of COVID-19 patients.
Frontiers in cellular and infection microbiologyRole of autoantibodies in the pathophysiology of irritable bowel syndrome: a review.
Frontiers in physiologyDopamine receptor D2 confers colonization resistance via microbial metabolites.
NatureImpact of COVID-19 on paediatric chronic intestinal failure: A tertiary care children's hospital experience.
Journal of pediatric gastroenterology and nutritionBiomolecular Actions by Intestinal Endotoxemia in Metabolic Syndrome.
International journal of molecular sciencesAn Atypical Case of Yersinia enterocolitica Infection in a Patient Suspected With Ulcerative Colitis Flare-Up.
CureusCeliac Disease-Related Conditions: Who to Test?
Gastroenterology[Indications and results of intestinal transplantation for short bowel syndrome after mesenteric ischemia].
Chirurgie (Heidelberg, Germany)From Viral Infection to Autoimmune Reaction: Exploring the Link between Human Herpesvirus 6 and Autoimmune Diseases.
MicroorganismsLow-FODMAP diet and hidradenitis suppurativa: the role of nutritionists in the management of dermato-endocrine disorders.
Minerva endocrinologyHumoral immune response and safety of SARS-CoV-2 vaccination in very early onset inflammatory bowel disease.
Journal of pediatric gastroenterology and nutritionDifferences in enteric neuronal density in the NSE-Noggin mouse model across institutes.
Scientific reportsBifidobacterium adolescentis - a beneficial microbe.
Beneficial microbesInvestigating the Impact of Varied Dietary Protein Levels on Litopenaeus vannamei: An Exploration of the Intestinal Microbiota and Transcriptome Responses.
Animals : an open access journal from MDPIRoles of ubiquitin-specific proteases in inflammatory diseases.
Frontiers in immunologySHIV-C109p5 NHP induces rapid disease progression in elderly macaques with extensive GI viral replication.
Journal of virology"Deficiency in ELF4, X-Linked": a Monogenic Disease Entity Resembling Behçet's Syndrome and Inflammatory Bowel Disease.
Journal of clinical immunologyX-Linked Lymphoproliferative Syndrome: A Spectrum of Clinical and Immunological Profile and Novel Pathogenic Variants from Chandigarh, India.
International archives of allergy and immunologyAn Elderly Patient Developed Ulcerative Colitis after SARS-CoV-2 mRNA Vaccination: A Case Report and Review of the Literature.
Internal medicine (Tokyo, Japan)Prevalence of urologic sequelae and bladder and bowel dysfunctions in patients with congenital Zika syndrome: A multicenter evaluation of the Zika virus bladder and bowel sequelae assistance network.
Journal of pediatric urologyHIV enteropathy and 'Slim disease': Historical and current perspectives.
International journal of infectious diseases : IJID : official publication of the International Society for Infectious DiseasesNutritional and health effects of bovine colostrum in neonates.
Nutrition reviewsClinical Utility of SARS-CoV-2 Serological Testing and Defining a Correlate of Protection.
VaccinesRisk of severe coronavirus disease 2019 despite vaccination in patients requiring treatment with immune-suppressive drugs: A nationwide cohort study of US Veterans.
Transplant infectious disease : an official journal of the Transplantation SocietyEffect of anti-SARS-CoV-2 BNT162b2 mRNA vaccination on thrombin generation in children with inflammatory bowel disease.
Frontiers in immunologyCase Report: The impact of severe cryptosporidiosis on the gut microbiota of a pediatric patient with CD40L immunodeficiency.
Frontiers in cellular and infection microbiologyDengue virus infection induces selective expansion of Vγ4 and Vγ6TCR γδ T cells in the small intestine and a cytokine storm driving vascular leakage in mice.
PLoS neglected tropical diseasesB cell-mediated CD4 T-cell costimulation via CD86 exacerbates pro-inflammatory cytokine production during autoimmune intestinal inflammation.
Mucosal immunologyMicrobiota Transplant and Gynecological Disorders: The Bridge between Present and Future Treatments.
MicroorganismsSerum Anti-Spike Antibodies Are Not Affected by Immunosuppressants in SARS-CoV-2 Vaccinations Given to Brazilian Patients with Inflammatory Bowel Disease.
Healthcare (Basel, Switzerland)Fungal microbiota sustains lasting immune activation of neutrophils and their progenitors in severe COVID-19.
Nature immunologyBiofilm's Impact on Inflammatory Bowel Diseases.
CureusReview of microbiota gut brain axis and innate immunity in inflammatory and infective diseases.
Frontiers in cellular and infection microbiologyNovel CARMIL2 (RLTPR) Mutation Presenting with Hyper-IgE and Eosinophilia: A Case Report.
Endocrine, metabolic & immune disorders drug targetsModulating microbiome-immune axis in the deployment-related chronic diseases of Veterans: report of an expert meeting.
Gut microbesEnhanced IL-17 Producing and Maintained Cytolytic Effector Functions of Gut Mucosal CD161+CD8+ T Cells in SIV-Infected Rhesus Macaques.
VirusesHumoral Immunity in Immunosuppressed IBD Patients after the Third SARS-CoV-2 Vaccination: A Comparison with Healthy Control Subjects.
VaccinesFlaxseed Supplementation in Chicken Feed Accelerates Salmonella enterica subsp. enterica Serovar Enteritidis Clearance, Modulates Cecum Microbiota, and Influences Ovarian Gene Expression in Laying Hens.
BiomoleculesIncidence of immune-mediated inflammatory diseases following COVID-19: a matched cohort study in UK primary care.
BMC medicineEarly life adverse exposures in irritable bowel syndrome: new insights and opportunities.
Frontiers in pediatricsCircRNA254 functions as the miR-375 sponge to inhibit coelomocyte apoptosis via targeting BAG2 in V. splendidus-challenged Apostichopus japonicus.
Fish & shellfish immunologyEmerging Paradigms in Inflammatory Disease Management: Exploring Bioactive Compounds and the Gut Microbiota.
Brain sciencesThe role of caspase-8 in inflammatory signalling and pyroptotic cell death.
Seminars in immunologyCOVID-19-induced gastrointestinal autonomic dysfunction: A systematic review.
World journal of clinical casesGastrointestinal involvement in post-acute Coronavirus disease (COVID)-19 syndrome.
Current opinion in infectious diseasesHost-microbiota relationship in the pathophysiology of aseptic abscess syndrome: protocol for a multicentre case-control study (ABSCESSBIOT).
BMJ openSystemic Disease and the Liver Part 2: Pregnancy-Related Liver Injury, Sepsis/Critical Illness, Hypoxia, Psoriasis, Scleroderma/Sjogren's Syndrome, Sarcoidosis, Common Variable Immune Deficiency, Cystic Fibrosis, Inflammatory Bowel Disease, and Hematologic Disorders.
Surgical pathology clinicsElevated levels of enteric IgA in an unimmunised mouse model of Hyper IgM syndrome derived from gut-associated secondary lymph organs even in the absence of germinal centres.
Frontiers in cellular and infection microbiologyDietary Implications of the Bidirectional Relationship between the Gut Microflora and Inflammatory Diseases with Special Emphasis on Irritable Bowel Disease: Current and Future Perspective.
NutrientsLow vaccination and infection rate of Omicron in patients with inflammatory bowel disease: a comparative study of three unique cohorts.
Frontiers in public healthAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Síndrome de imunodesregulação-doença inflamatória do intestino-artrite-infecções recorrentes.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Síndrome de imunodesregulação-doença inflamatória do intestino-artrite-infecções recorrentes
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- From Gut to Metabolism: Exploring the Intersection of Inflammatory Bowel Disease and Systemic Metabolic Dysfunction.
- Are we missing early pancreatic dysfunction? Expanding faecal elastase testing beyond pancreatic disease.
- Metabotherapy for intestinal disease: using metabolites to prevent and treat disorders of the gut.
- Fusobacterium nucleatum plays a pathogenic role in a murine model of irritable bowel syndrome by modulating intestinal purine metabolism and promoting mast cell activation.
- IgA Nephropathy in Adults: A Review.
- Modeling Clostridioides difficile toxin pathogenesis and antiserum protection in an immunocompetent intestine-on-chip platform.
- Inflammatory ILC2s migrate to distal tissues during infection using stage-specific S1P receptors.
- Preparation and mechanism study of Alhagi honey polysaccharide-aluminum Pickering emulsion adjuvant for improving intestinal mucosal immune function.
- LC3-associated phagocytosis in macrophage responses to Paracoccidioides spp.
- Lactobacillus rhamnosus confers protection against enteropathogenic bacteria by enhancing mucosal immunity and epithelial barrier function.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:238569(Orphanet)
- MONDO:0016542(MONDO)
- GARD:13016(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q56013846(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
