Pular para o conteúdo
Diarreia sindrômica
ORPHA:84064CID-10 · K52.8CID-11 · DA90.0DOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
AudiçãoInício neonatalHerança AR
Também conhecida comoSD

Uma doença grave do intestino, presente desde o nascimento, que causa diarreia incontrolável no primeiro mês de vida e faz com que a criança tenha dificuldade para crescer e ganhar peso. Ela também está ligada a características diferentes no rosto, problemas no cabelo e, em alguns casos, a problemas no sistema de defesa do corpo (imunidade) e ao fato de o bebê não ter crescido o suficiente ainda na barriga da mãe.

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Introdução

O que você precisa saber de cara

💬
EM LINGUAGEM SIMPLES

Síndrome trico-hepato-enteral é uma condição que afeta o cabelo (trico-), o fígado (hepato-) e os intestinos (enteral), bem como outros tecidos e órgãos do corpo. Essa condição também é conhecida como diarreia sindrômica porque a diarreia crônica, de difícil tratamento, é uma de suas principais características. Nas primeiras semanas de vida, bebês afetados desenvolvem diarreia aquosa que ocorre várias vezes ao dia. Mesmo com suporte nutricional por meio de alimentação intravenosa (nutrição parenteral), muitas dessas crianças apresentam falha no crescimento, o que significa que não ganham peso ou crescem na taxa esperada. A maioria das crianças com síndrome trico-hepato-enteral nasce pequena e permanece mais baixa que seus pares ao longo da vida.

Cabelo anormal é outra característica da síndrome trico-hepato-enteral. O cabelo em pessoas com a condição é descrito como lanoso, frágil, irregular e facilmente arrancado. Sob microscópio, alguns fios de cabelo podem apresentar variação de diâmetro, com áreas mais espessas e mais finas. Essa característica é conhecida como trichorrhexis nodosa.

Outros sinais e sintomas da síndrome trico-hepato-enteral podem incluir doença hepática; anomalias cutâneas; e características faciais distintivas, incluindo testa larga, base do nariz larga e olhos amplamente espaçados. No geral, as características faciais são descritas como “ásperas”. A maioria das pessoas com a condição também apresenta anomalias no sistema imunológico que podem torná‑las mais propensas a desenvolver infecções. Menos frequentemente, a síndrome trico-hepato-enteral está associada a anomalias cardíacas (cardíacas). Deficiência intelectual leve foi relatada em pelo menos metade de todas as crianças com a condição.

A síndrome trico-hepato-enteral costuma ser potencialmente fatal na infância, particularmente em crianças que desenvolvem doença hepática ou infecções graves.

Fonte: MedlinePlus Genetics (NLM/NIH), traduzido
📋
Informacoes curadas por IA — podem conter imprecisoes

Uma doença grave do intestino, presente desde o nascimento, que causa diarreia incontrolável no primeiro mês de vida e faz com que a criança tenha dificuldade para crescer e ganhar peso. Ela também está ligada a características diferentes no rosto, problemas no cabelo e, em alguns casos, a problemas no sistema de defesa do corpo (imunidade) e ao fato de o bebê não ter crescido o suficiente ainda na barriga da mãe.

Publicações científicas
51 artigos
Último publicado: 2026 Jan

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
116
pacientes catalogados
Início
Antenatal
+ infancy, neonatal
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: K52.8
Você se identifica com essa condição?
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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

🫃
Digestivo
15 sintomas
🧬
Pele e cabelo
10 sintomas
😀
Face
9 sintomas
❤️
Coração
7 sintomas
🧠
Neurológico
4 sintomas
🦴
Ossos e articulações
3 sintomas

+ 34 sintomas em outras categorias

Características mais comuns

90%prev.
Diarreia intratável
Muito frequente (99-80%)
90%prev.
Cabelo lanoso
Muito frequente (99-80%)
90%prev.
Imunodeficiência
Muito frequente (99-80%)
90%prev.
Formato facial anormal
Muito frequente (99-80%)
55%prev.
Hipopigmentação do cabelo
Frequente (79-30%)
55%prev.
Atraso global do desenvolvimento
Frequente (79-30%)
94sintomas
Muito frequente (4)
Frequente (21)
Ocasional (10)
Muito raro (17)
Sem dados (42)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 94 características clínicas mais associadas, ordenadas por frequência.

Diarreia intratávelIntractable diarrhea
Muito frequente (99-80%)90%
Cabelo lanosoWoolly hair
Muito frequente (99-80%)90%
ImunodeficiênciaImmunodeficiency
Muito frequente (99-80%)90%
Formato facial anormalAbnormal facial shape
Muito frequente (99-80%)90%
Hipopigmentação do cabeloHypopigmentation of hair
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico51PubMed
Últimos 10 anos63publicações
Pico202212 papers
Linha do tempo
2026Hoje · 2026🧪 2020Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

2 genes identificados com associação a esta condição. Padrão de herança: Autosomal recessive.

SKIC2Superkiller complex protein 2Disease-causing germline mutation(s) inTolerante
VIAS BIOLÓGICAS (2)
mRNA decay by 3' to 5' exoribonucleaseAssociation of TriC/CCT with target proteins during biosynthesis
MECANISMO DE DOENÇA

Trichohepatoenteric syndrome 2

A syndrome characterized by intrauterine growth retardation, severe diarrhea in infancy requiring total parenteral nutrition, facial dysmorphism, immunodeficiency, and hair abnormalities, mostly trichorrhexis nodosa. Hepatic involvement contributes to the poor prognosis of affected patients.

OUTRAS DOENÇAS (2)
trichohepatoenteric syndrome 2trichohepatoenteric syndrome
HGNC:10898UniProt:Q15477
SKIC3Superkiller complex protein 3Disease-causing germline mutation(s) inTolerante
VIAS BIOLÓGICAS (1)
mRNA decay by 3' to 5' exoribonuclease
MECANISMO DE DOENÇA

Trichohepatoenteric syndrome 1

A syndrome characterized by intrauterine growth retardation, severe diarrhea in infancy requiring total parenteral nutrition, facial dysmorphism, immunodeficiency, and hair abnormalities, mostly trichorrhexis nodosa. Hepatic involvement contributes to the poor prognosis of affected patients.

OUTRAS DOENÇAS (2)
trichohepatoenteric syndrome 1trichohepatoenteric syndrome
HGNC:23639UniProt:Q6PGP7

Variantes genéticas (ClinVar)

416 variantes patogênicas registradas no ClinVar.

🧬 SKIC2: NM_006929.5(SKIC2):c.354+1G>T ()
🧬 SKIC2: NC_000006.12:g.31967715del ()
🧬 SKIC2: NM_006929.5(SKIC2):c.1258del (p.Val420fs) ()
🧬 SKIC2: NM_006929.5(SKIC2):c.2732-1G>A ()
🧬 SKIC2: NM_006929.5(SKIC2):c.2370dup (p.Lys791fs) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 208 variantes classificadas pelo ClinVar.

166
42
Patogênica (79.8%)
VUS (20.2%)
VARIANTES MAIS SIGNIFICATIVAS
SKIC2: NM_006929.5(SKIC2):c.2476C>T (p.Gln826Ter) [Likely pathogenic]
SKIC2: NM_006929.5(SKIC2):c.779dup (p.Val261fs) [Pathogenic]
SKIC2: NM_006929.5(SKIC2):c.2341-1G>C [Likely pathogenic]
SKIC2: NC_000006.11:g.(?_31926951)_(31937529_?)del [Pathogenic]
SKIC3: NM_014639.4(SKIC3):c.154G>T (p.Glu52Ter) [Pathogenic]

Vias biológicas (Reactome)

2 vias biológicas associadas aos genes desta condição.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Diarreia sindrômica

🗺️

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

Onde estão os ensaios

Nenhum país com ensaio aberto no momento.

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
47 papers (10 anos)
#1

Case Report: Tricho-hepato-enteric syndrome in an infant presented with colorectal ulceration and severe respiratory superinfection.

Frontiers in immunology2026

Tricho-hepato-enteric syndrome (THES) is a rare genetic disorder characterized by early-onset intractable diarrhea, intrauterine growth retardation, hair abnormalities, and liver disease during early infancy. THES is often associated with combined immunodeficiency caused by defective interferon-γ production in T cells and hypogammaglobulinemia. However, very few cases of a severe clinical course in infancy have been reported. Here, we report the case of a 2-month-old boy who presented with intractable diarrhea, growth retardation, and hair anomaly. Although fasting and central venous nutrition reduced stool frequency, effective weight gain was not achieved. A colonoscopy revealed multiple irregular ulcers without any cytomegalovirus (CMV)-positive cells. Nevertheless, CMV was detected in peripheral blood using a polymerase chain reaction, and the patient was initially treated with ganciclovir. However, this approach was not clinically effective. The second endoscopy revealed new colonic ulcers with mild active inflammation, and treatment with prednisolone was partially effective. The Immunological evaluation revealed no impaired findings, except for low blastogenesis in T cells. However, the patient developed severe progressive respiratory failure caused by superinfection with Pneumocystis jirovecii and CMV and died at 6 months of age. Clinical sequencing analysis identified compound heterozygous frameshift variants c.195dupA (p.A66Sfs*3) and c.3426dupA (p.A1143Sfs*4) in TTC37 (NM_014639.4), confirming the diagnosis of THES. THES can have a fatal clinical course even during infancy. Detailed immunological and genetic analyses, in addition to endoscopic examination, are crucial for the definitive diagnosis and management of patients with very early-onset inflammatory bowel disease and inborn errors of immunity with systemic features.

#2

Reduced immunoglobulin requirement after dupilumab for atopic dermatitis in trichohepatoenteric syndrome.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology2026 Jan
#3

Hidden Aberrant Transcripts in TTC37 Cause Trichohepatoenteric Syndrome.

Clinical genetics2025 Jan

The patient had clinical suspicion of THES. Complex genetic analyzes using WES, WGS were performed without success in the diagnosis. Further molecular analyzes using RNA and protein was necessary to reach the final correct THES diagnosis.

#4

A Case Report and Literature Review on Osteo-Oto-Hepato-Enteric Syndrome in Premature Infants Caused by UNC45A Deficiency.

Molecular genetics &amp; genomic medicine2025 Oct

To report the clinical manifestations, treatment, and genetic diagnosis of a patient with osteo-oto-hepato-enteric (O2HE) syndrome. A retrospective analysis was performed on a Chinese premature infant born at 36 + 5 weeks of gestation. The analysis included maternal pregnancy and delivery history, prenatal ultrasound findings, clinical manifestations, diagnosis and treatment process, and UNC45A gene mutation results for the infant and parents. A literature review was also conducted. The mother had a history of six spontaneous abortions. A late-pregnancy prenatal ultrasound revealed polyhydramnios and diffuse intestinal dilation. The infant developed watery stools and diarrhea shortly after birth and was clinically suspected to have congenital chloride diarrhea. Supportive treatment was administered, and whole-exome sequencing was performed for the family. Two heterozygous mutations were identified in the UNC45A gene, including c.2455C > T (p.Arg819Ter), a novel, previously unreported variant. The infant was ultimately diagnosed with osteo-oto-hepatoenteric syndrome. Genetic analysis of the UNC45A gene is valuable for diagnosing O2HE syndrome. The novel mutation c.2455C > T (p.Arg819Ter) enriches the mutation spectrum of UNC45A, providing further theoretical support for diagnosis and genetic counseling of O2HE.

#5

Trichohepatoenteric syndrome type 1: expanding the clinical spectrum of THES type 1 due to a homozygous variant in the SKIC3 gene.

BMC pediatrics2024 Jul 10

Trichohepatoenteric syndrome (THES), also known as phenotypic diarrhea or syndromic diarrhea, is a rare autosomal recessive genetic disorder caused by mutations in SKIC2 (THES-type 2) or SKIC3 (THES-type 1) and is characterized by early onset diarrhea, woolly brittle hair, facial dysmorphic features and liver disease. We report the case of a 24-month-old girl who presented with chronic diarrhea since the neonatal period along with intrauterine growth restriction (IUGR), developmental delay, dysmorphic features, congenital heart defects, liver disease, and recurrent infections. The diagnosis was made through whole-exome sequencing analysis, which detected a homozygous variant (c.4070del, p.Pro1357Leufs*10) in the SKIC3 gene. The patient required parenteral nutrition and was hospitalized for the first 10 months of life and then discharged on PN after showing improvement. She remained stable on PN after discharge despite a few admissions for central line infections. Recent follow-up at the age of 2 years revealed that she was stable on long-term parenteral nutrition and that she had advanced chronic liver disease.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC35 artigos no totalmostrando 63

2026

Case Report: Tricho-hepato-enteric syndrome in an infant presented with colorectal ulceration and severe respiratory superinfection.

Frontiers in immunology
2026

Reduced immunoglobulin requirement after dupilumab for atopic dermatitis in trichohepatoenteric syndrome.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology
2025

A Case Report and Literature Review on Osteo-Oto-Hepato-Enteric Syndrome in Premature Infants Caused by UNC45A Deficiency.

Molecular genetics &amp; genomic medicine
2024

Trichohepatoenteric Syndrome: A Report of Two Children From Bahrain With a Novel Mutation and a Literature Review.

Cureus
2024

PytheasDB: An open-access graphical database of clinical data on rare pediatric digestive diseases.

Intractable &amp; rare diseases research
2025

Hidden Aberrant Transcripts in TTC37 Cause Trichohepatoenteric Syndrome.

Clinical genetics
2024

Trichohepatoenteric syndrome type 1: expanding the clinical spectrum of THES type 1 due to a homozygous variant in the SKIC3 gene.

BMC pediatrics
2024

Expanding the phenotypic and genotypic characteristics of trichohepatoenteric syndrome: a report of eight patients from five unrelated families.

Molecular biology reports
2024

A Case Report on Tricho-Hepato-Enteric Syndrome: The SKIC3 Gene in Focus.

Cureus
2024

Trichohepatoenteric syndrome and cytomegalovirus infection: Case report and literature summary.

SAGE open medical case reports
2024

Chronic diarrhoea due to trichohepatoenteric syndrome (THES) in an infant.

BMJ case reports
2024

Gut microbiota and polycystic ovary syndrome, focus on genetic associations: a bidirectional Mendelian randomization study.

Frontiers in endocrinology
2023

Dual Diagnosis of Trichohepatoenteric Syndrome and Lipoid Proteinosis in a Turkish Child.

Molecular syndromology
2024

Long term outcomes in children with trichohepatoenteric syndrome.

American journal of medical genetics. Part A
2023

SKI complex: A multifaceted cytoplasmic RNA exosome cofactor in mRNA metabolism with links to disease, developmental processes, and antiviral responses.

Wiley interdisciplinary reviews. RNA
2023

[Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases].

Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
2022

Novel TTC37 mutations in a patient with Trichohepatoenteric syndrome: a case report and literature review.

Translational pediatrics
2022

Hyper IgM in tricho-hepato-enteric syndrome due to TTC37 mutation.

Immunologic research
2022

RNA exosome drives early B cell development via noncoding RNA processing mechanisms.

Science immunology
2022

The mammalian SKIV2L RNA exosome is essential for early B cell development.

Science immunology
2022

A yeast model for trichohepatoenteric syndrome suggests strong loss of Ski2 function in most causative mutations.

microPublication biology
2022

UNC45A deficiency causes microvillus inclusion disease-like phenotype by impairing myosin VB-dependent apical trafficking.

The Journal of clinical investigation
2022

Case Report: A Novel Homozygous Frameshift Mutation of the SKIV2L Gene in a Trichohepatoenteric Syndrome Patient Presenting With Short Stature, Premature Ovarian Failure, and Osteoporosis.

Frontiers in genetics
2022

Congenital enteropathies involving defects in enterocyte structure or differentiation.

Best practice &amp; research. Clinical gastroenterology
2022

The human SKI complex regulates channeling of ribosome-bound RNA to the exosome via an intrinsic gatekeeping mechanism.

Molecular cell
2022

Sensing of RNA stress by mTORC1 drives autoinflammation.

The Journal of clinical investigation
2022

Cytoplasmic RNA quality control failure engages mTORC1-mediated autoinflammatory disease.

The Journal of clinical investigation
2021

A Case of Mild Trichohepatoenteric Syndrome With New Variant Mutation in SKIV2L Gene: Case Report.

Cureus
2021

Case Report: Novel Compound-Heterozygous Variants of SKIV2L Gene that Cause Trichohepatoenteric Syndrome 2.

Frontiers in genetics
2022

Tricho-hepato-enteric syndrome: Retrospective multicenter experience in Saudi Arabia.

Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association
2021

Congenital Diarrhea and Enteropathies in Infants: Approach to Diagnosis.

Indian journal of pediatrics
2021

Expanding the clinical spectrum in trichohepatoenteric syndrome.

American journal of medical genetics. Part A
2020

A New Variant Mutation in SKIV2L Gene in Case of Trichohepatoenteric Syndrome.

Pediatric reports
2020

The spectrum of primary immunodeficiencies at a tertiary care hospital in Pakistan.

The World Allergy Organization journal
2020

Ski3/TTC37 deficiency associated with trichohepatoenteric syndrome causes mitochondrial dysfunction in Drosophila.

FEBS letters
2019

An RNA Metabolism and Surveillance Quartet in the Major Histocompatibility Complex.

Cells
2019

A Novel TTC37 Mutation Causing Clinical Symptoms of Trichohepatoenteric Syndrome Such as Pyoderma Gangrenosum and Immunodeficiency Without Severe Diarrhea.

Journal of investigational allergology &amp; clinical immunology
2019

Tricho-hepatic-enteric syndrome (THES) without intractable diarrhoea.

Gene
2019

Ethanol lock and immunoglobulin load: Improving the clinical course of trichohepatoenteric syndrome.

Annals of allergy, asthma &amp; immunology : official publication of the American College of Allergy, Asthma, &amp; Immunology
2018

Trichohepatoenteric syndrome: A rare mutation in SKIV2L gene in the first Balkan reported case.

SAGE open medical case reports
2018

Combined Immunodeficiency in Patients With Trichohepatoenteric Syndrome.

Frontiers in immunology
2018

Tricho-Hepato-Enteric Syndrome mutation update: Mutations spectrum of TTC37 and SKIV2L, clinical analysis and future prospects.

Human mutation
2018

Genetic and Structural Analysis of a SKIV2L Mutation Causing Tricho-hepato-enteric Syndrome.

Digestive diseases and sciences
2018

A new mutation in the C-terminal end of TTC37 leading to a mild form of syndromic diarrhea/tricho-hepato-enteric syndrome in seven patients from two families.

American journal of medical genetics. Part A
2018

Missense mutation of TTC7A mimicking tricho-hepato-enteric (SD/THE) syndrome in a patient with very-early onset inflammatory bowel disease.

European journal of medical genetics
2017

Tricho-hepato-enteric syndrome with novel SKIV2L gene mutations: A case report.

Medicine
2018

Graft versus host disease in a pediatric multiple organ transplant recipient with trichohepatoenteric syndrome - a unique case report.

International journal of dermatology
2018

Trichohepatoenteric Syndrome Presenting with Severe Infection and Later Onset Diarrhoea.

Journal of clinical immunology
2018

The RNA exosome and RNA exosome-linked disease.

RNA (New York, N.Y.)
2017

[A case of tricho-hepato-enteric syndrome].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2017

Exome sequencing identifies a novel TTC37 mutation in the first reported case of Trichohepatoenteric syndrome (THE-S) in South Africa.

BMC medical genetics
2017

IBD-Like Features in Syndromic Diarrhea/Trichohepatoenteric Syndrome.

Journal of pediatric gastroenterology and nutrition
2016

Targeted next-generation sequencing identification of a novel missense mutation of the SKIV2L gene in a patient with trichohepatoenteric syndrome.

Molecular medicine reports
2017

Emerging roles of the processing of nucleic acids and Toll-like receptors in innate immune responses to nucleic acids.

Journal of leukocyte biology
2016

Novel mutations in SKIV2L and TTC37 genes in Malaysian children with trichohepatoenteric syndrome.

Gene
2016

Identifying Mutations of the Tetratricopeptide Repeat Domain 37 (TTC37) Gene in Infants With Intractable Diarrhea and a Comparison of Asian and Non-Asian Phenotype and Genotype: A Global Case-report Study of a Well-Defined Syndrome With Immunodeficiency.

Medicine
2016

Trichohepatoenteric Syndrome or Syndromic Diarrhea-Report of Three Members in a Family, First Report from Iran.

Case reports in pathology
2016

The role of enterocyte defects in the pathogenesis of congenital diarrheal disorders.

Disease models &amp; mechanisms
2016

The localisation of the apical Par/Cdc42 polarity module is specifically affected in microvillus inclusion disease.

Biology of the cell
2015

Tricho-hepato-enteric syndrome (THE-S): two cases and review of the literature.

European journal of pediatrics
2015

Corrigendum: Novel TTC37 Mutations in a Patient with Immunodeficiency without Diarrhea: Extending the Phenotype of Trichohepatoenteric Syndrome.

Frontiers in pediatrics
2015

Expanding phenotypic and allelic heterogeneity of tricho-hepato-enteric syndrome.

Journal of pediatric gastroenterology and nutrition
2015

Novel TTC37 Mutations in a Patient with Immunodeficiency without Diarrhea: Extending the Phenotype of Trichohepatoenteric Syndrome.

Frontiers in pediatrics

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Diarreia sindrômica.

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Doença com base genética

Um médico geneticista pode ajudar no diagnóstico de Diarreia sindrômica e no aconselhamento genético da família.

Vai consultar um especialista? Confirme o registro dele no conselho.
Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

A diarreia sindrômica é causada por alterações genéticas com padrão de herança autossômica recessiva. Mutações nos genes SKIC2 e SKIC3 foram identificadas como as responsáveis pelo desenvolvimento do quadro.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

7 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. Reduced immunoglobulin requirement after dupilumab for atopic dermatitis in trichohepatoenteric syndrome.
    Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology2026PMID 41531377
  2. ORPHA:84064
    Orphanet
  3. GARD:5258
    GARD (NIH)
  4. Q56014636
    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.

Citar este verbete

Raras. (s.d.). Diarreia sindrômica. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/diarreia-sindromica

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Compêndio · Raras BR

Diarreia sindrômica

ORPHA:84064 · MONDO:0009105
Prevalência
<1 / 1 000 000
Casos
116 casos conhecidos
Herança
Autosomal recessive
CID-10
K52.8 · Outras gastroenterites e colites especificadas, não-infecciosas
CID-11
Início
Antenatal, Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C1857276
EuropePMC
Wikidata
Papers 10a
Evidência
🥉 Relato de caso

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Rara