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Bronquiectasia idiopática
ORPHA:60033CID-10 · J47CID-11 · CA24DOENÇA RARA

A bronquiectasia idiopática (IB) é uma doença pulmonar progressiva caracterizada por dilatação crônica dos brônquios e destruição das paredes brônquicas na ausência de qualquer causa subjacente (como doença pós-infecciosa, aspiração, imunodeficiência, anomalias congênitas e anomalias ciliares).

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Introdução

O que você precisa saber de cara

📋

A bronquiectasia idiopática (IB) é uma doença pulmonar progressiva caracterizada por dilatação crônica dos brônquios e destruição das paredes brônquicas na ausência de qualquer causa subjacente (como doença pós-infecciosa, aspiração, imunodeficiência, anomalias congênitas e anomalias ciliares).

Publicações científicas
105 artigos
Último publicado: 2026 Feb 22

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
29.0
Europe
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: J47
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫁
Pulmão
9 sintomas
📏
Crescimento
1 sintomas
❤️
Coração
1 sintomas
🛡️
Imunológico
1 sintomas
🫃
Digestivo
1 sintomas
😀
Face
1 sintomas

+ 10 sintomas em outras categorias

Características mais comuns

100%prev.
Bronquiectasia
90%prev.
Infecção do trato respiratório
Muito frequente (99-80%)
90%prev.
Fisiologia anormal do sistema respiratório
Muito frequente (99-80%)
90%prev.
Tosse produtiva
Muito frequente (99-80%)
55%prev.
Crepitações
Frequente (79-30%)
55%prev.
Hemoptise
Frequente (79-30%)
24sintomas
Muito frequente (4)
Frequente (8)
Ocasional (6)
Muito raro (1)
Sem dados (5)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

BronquiectasiaBronchiectasis
Muito frequente100%
Infecção do trato respiratórioRespiratory tract infection
Muito frequente (99-80%)90%
Fisiologia anormal do sistema respiratórioAbnormal respiratory system physiology
Muito frequente (99-80%)90%
Tosse produtivaProductive cough
Muito frequente (99-80%)90%
CrepitaçõesCrackles
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico105PubMed
Últimos 10 anos40publicações
Pico20166 papers
Linha do tempo
2026Hoje · 2026🧪 2005Primeiro ensaio clínico📈 2016Ano 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

4 genes identificados com associação a esta condição. Padrão de herança: Not applicable.

SCNN1AEpithelial sodium channel subunit alphaDisease-causing germline mutation(s) inTolerante
FUNÇÃO

This is one of the three pore-forming subunits of the heterotrimeric epithelial sodium channel (ENaC), a critical regulator of sodium balance and fluid homeostasis (PubMed:30251954, PubMed:32729833, PubMed:8023962, PubMed:8278374, PubMed:9792722). ENaC operates in epithelial tissues, where it mediates the electrodiffusion of sodium ions from extracellular fluid through the apical membrane of cells, with water following osmotically (PubMed:24124190, PubMed:28710092, PubMed:8278374). It plays a ke

LOCALIZAÇÃO

Apical cell membraneCell projection, ciliumCytoplasmic granuleCytoplasmCytoplasmic vesicle, secretory vesicle, acrosomeCell projection, cilium, flagellum

VIAS BIOLÓGICAS (2)
Stimuli-sensing channelsSensory perception of salty taste
MECANISMO DE DOENÇA

Pseudohypoaldosteronism 1B1, autosomal recessive

A form of pseudohypoaldosteronism type 1, a rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. The disorder affects multiple organs, and is characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatremia, hyperkalemia, metabolic acidosis, failure to thrive and weight loss.

EXPRESSÃO TECIDUAL(Ubíquo)
Glândula salivar
160.4 TPM
Rim - Medula
137.4 TPM
Esôfago - Mucosa
129.2 TPM
Tireoide
128.6 TPM
Pulmão
77.2 TPM
OUTRAS DOENÇAS (6)
pseudohypoaldosteronism, type IB1, autosomal recessivebronchiectasis with or without elevated sweat chloride 2Liddle syndrome 3Liddle syndrome
HGNC:10599UniProt:P37088
SCNN1GEpithelial sodium channel subunit gammaDisease-causing germline mutation(s) inRestrito
FUNÇÃO

This is one of the three pore-forming subunits of the heterotrimeric epithelial sodium channel (ENaC), a critical regulator of sodium balance and fluid homeostasis (PubMed:30251954, PubMed:32729833, PubMed:7550319, PubMed:7762608, PubMed:9792722). ENaC operates in epithelial tissues, where it mediates the electrodiffusion of sodium ions from extracellular fluid through the apical membrane of cells, with water following osmotically (PubMed:24124190). It plays a key role in maintaining sodium home

LOCALIZAÇÃO

Apical cell membrane

VIAS BIOLÓGICAS (2)
Stimuli-sensing channelsSensory perception of salty taste
MECANISMO DE DOENÇA

Liddle syndrome 2

A form of Liddle syndrome, an autosomal dominant disorder characterized by early onset of hypertension, hypokalemic alkalosis, and suppression of plasma renin activity and aldosterone secretion.

EXPRESSÃO TECIDUAL(Tecido-específico)
Rim - Medula
47.1 TPM
Rim - Córtex
25.7 TPM
Vagina
22.7 TPM
Cérebro - Hemisfério cerebelar
14.9 TPM
Próstata
14.7 TPM
OUTRAS DOENÇAS (6)
pseudohypoaldosteronism, type IB3, autosomal recessiveLiddle syndrome 2bronchiectasis with or without elevated sweat chloride 3Liddle syndrome
HGNC:10602UniProt:P51170
CFTRCystic fibrosis transmembrane conductance regulatorCandidate gene tested inTolerante
FUNÇÃO

Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed:26823428). Mediates the transport of chloride ions across the cell membrane (PubMed:10792060, PubMed:11524016, PubMed:11707463, PubMed:12519745, PubMed:12529365, PubMed:12588899, PubMed:12727866, PubMed:15010471, PubMed:17036051, PubMed:1712898, PubMed:17182731, PubMed:19398555, PubMed:19621064, PubMed:22178883, PubMed:25330774, PubMed:26846474, PubMed:2808770

LOCALIZAÇÃO

Apical cell membraneEarly endosome membraneCell membraneRecycling endosome membraneEndoplasmic reticulum membraneNucleus

VIAS BIOLÓGICAS (1)
ABC-family proteins mediated transport
MECANISMO DE DOENÇA

Cystic fibrosis

A common generalized disorder of the exocrine glands which impairs clearance of secretions in a variety of organs. It is characterized by the triad of chronic bronchopulmonary disease (with recurrent respiratory infections), pancreatic insufficiency (which leads to malabsorption and growth retardation) and elevated sweat electrolytes. It is the most common genetic disease in Caucasians, with a prevalence of about 1 in 2'000 live births. Inheritance is autosomal recessive.

OUTRAS DOENÇAS (8)
cystic fibrosiscongenital bilateral aplasia of vas deferens from CFTR mutationaquagenic palmoplantar keratodermacongenital bilateral absence of vas deferens
HGNC:1884UniProt:P13569
SCNN1BEpithelial sodium channel subunit betaDisease-causing germline mutation(s) inTolerante
FUNÇÃO

This is one of the three pore-forming subunits of the heterotrimeric epithelial sodium channel (ENaC), a critical regulator of sodium balance and fluid homeostasis (PubMed:30251954, PubMed:32729833, PubMed:7762608, PubMed:9792722). ENaC operates in epithelial tissues, where it mediates the electrodiffusion of sodium ions from extracellular fluid through the apical membrane of cells, with water following osmotically (PubMed:24124190). It plays a key role in maintaining sodium homeostasis through

LOCALIZAÇÃO

Apical cell membraneCytoplasmic vesicle membrane

VIAS BIOLÓGICAS (2)
Stimuli-sensing channelsSensory perception of salty taste
MECANISMO DE DOENÇA

Pseudohypoaldosteronism 1B2, autosomal recessive

A form of pseudohypoaldosteronism type 1, a rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. The disorder affects multiple organs, and is characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatremia, hyperkalemia, metabolic acidosis, failure to thrive and weight loss.

EXPRESSÃO TECIDUAL(Ubíquo)
Esôfago - Mucosa
79.6 TPM
Vagina
59.3 TPM
Skin Not Sun Exposed Suprapubic
38.9 TPM
Skin Sun Exposed Lower leg
37.9 TPM
Pulmão
34.9 TPM
OUTRAS DOENÇAS (6)
pseudohypoaldosteronism, type IB2, autosomal recessiveLiddle syndrome 1bronchiectasis with or without elevated sweat chloride 1Liddle syndrome
HGNC:10600UniProt:P51168

Variantes genéticas (ClinVar)

2,336 variantes patogênicas registradas no ClinVar.

🧬 SCNN1B: NM_000336.3(SCNN1B):c.1153-3C>G ()
🧬 SCNN1B: NM_000336.3(SCNN1B):c.1394del (p.Glu465fs) ()
🧬 SCNN1B: GRCh38/hg38 16p13.11-12.1(chr16:15368750-28342902)x4 ()
🧬 SCNN1B: NM_000336.3(SCNN1B):c.101_119del (p.Asn34fs) ()
🧬 SCNN1B: NM_000336.3(SCNN1B):c.1487A>G (p.Asn496Ser) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 1 variantes classificadas pelo ClinVar.

1
Patogênica (100.0%)
VARIANTES MAIS SIGNIFICATIVAS
SCNN1A: NM_001038.6(SCNN1A):c.942del (p.Asn315fs) [Likely pathogenic]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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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-clínico5
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 5 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Bronquiectasia idiopática

🗺️

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

6 ensaios clínicos encontrados.

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

Timeline de publicações
41 papers (10 anos)
#1

Characteristics of patients with idiopathic bronchiectasis in comparison to post-infectious bronchiectasis in South Korea.

Tuberculosis and respiratory diseases2026 Jan 27

Bronchiectasis has a complex, heterogeneous pathogenesis with various aetiologies, with idiopathic bronchiectasis being the majority. This study aimed to investigate the characteristics of patients with idiopathic bronchiectasis, in comparison with post-infectious bronchiectasis. We analyzed idiopathic and post-infectious (including post-TB) bronchiectasis patients from the Korean Multicenter Bronchiectasis Audit and Research Collaboration (KMBARC) registry, a prospective cohort study (1). Among the 866 patients enrolled in the study, 346 (40.0%) patients were determined as idiopathic, 363 (41.9%) patients as post-infectious. The idiopathic group exhibited shorter disease duration of bronchiectasis, higher BMI, lower prevalence of COPD, higher prevalence of rhinosinusitis, predominance of lower lobe distribution in bronchiectasis, lower usage of regular respiratory treatment, better pulmonary function, and statistically lower bronchiectasis severity index (BSI) compared to post-infectious bronchiectasis. Multivariable logistic regression analysis was performed for the variables of gender, age, body mass index, the history of asthma, COPD, rhinosinusitis, rheumatoid arthritis and gastroesophageal reflux disease (GERD), and smoking status. A higher body mass index (odds ratio, 1.09; 95% confidence interval, 1.04-1.15) and the history of rhinosinusitis (3.10; 1.57-6.14) were associated with idiopathic bronchiectasis. Conversely, the history of COPD was associated with post-infectious bronchiectasis (0.57; 0.41-0.80). The characteristics of patients with idiopathic bronchiectasis might be a higher body mass index (BMI) and the history of rhinosinusitis in comparison with post-infectious bronchiectasis, potentially serving as exploratory clues to underlying systemic or non-pulmonary factors. Further research is needed to clarify their clinical significance.

#2

CFTR activity in nasal potential difference of adults with idiopathic bronchiectasis.

Respiratory research2026 Mar 03
#3

Phenotyping Bronchiectasis Frequent Exacerbator: A Single Centre Retrospective Cluster Analysis.

Biomedicines2025 Aug 30

Background: Bronchiectasis is a chronic respiratory condition characterized by permanent bronchial dilation, recurrent infections, and progressive lung damage. A subset of patients, known as frequent exacerbators, experience multiple exacerbations annually, leading to accelerated lung function decline, hospitalizations, and reduced quality of life. The aim of this study is to identify distinct phenotypes and treatable traits in bronchiectasis frequent exacerbators, since it could be crucial for optimizing patient management. Research question: Could clinically distinct phenotypes and treatable traits be identified among frequent exacerbators with bronchiectasis to guide personalized management strategies? Methods: We analysed a cohort of 56 bronchiectasis frequent exacerbator patients using 21 clinically relevant variables, including pulmonary function tests, radiological patterns, and microbiological data. Hierarchical clustering and k-means algorithms were applied to identify subgroups. Key outcomes included cluster-specific characteristics, treatable traits, and their implications for management. Results: Four distinct clusters were identified: 1. Mild, idiopathic bronchiectasis (Cluster 1): Predominantly mild disease (FACED), idiopathic etiology (93.3%), and cylindrical bronchiectasis with moderate obstruction (60%). 2. Rheumatological and NTM-associated bronchiectasis (Cluster 2): Patients with systemic inflammatory diseases (50%) and NTMever (50%) but minimal infections by Pseudomonas aeruginosa. 3. Mild, post-infective bronchiectasis (Cluster 3): Exclusively mild disease, mixed idiopathic and post-infective etiologies, and preserved lung function. 4. Severe, chronic infection phenotype (Cluster 4): Severe disease with high colonization rates of Pseudomonas aeruginosa (71.4%), advanced structural damage (57.1% varicose, 50% cystic bronchiectasis), and frequent exacerbations. Interpretation: This analysis highlights the heterogeneity of bronchiectasis and its frequent exacerbator phenotype. The treatable traits framework underscores the importance of aggressive infection control and management of airway inflammation in severe cases, while milder clusters may benefit from preventive strategies. These findings support the integration of precision medicine in bronchiectasis care, focusing on phenotype-specific interventions to improve outcomes.

#4

Acrophialophora jodhpurensis and Nocardia in idiopathic bronchiectasis: Colonizers or culprits?'.

Indian journal of medical microbiology2025
#5

Multi-drug resistant Pseudomonas aeruginosa isolation is an independent risk factor for recurrent hemoptysis after bronchial artery embolization in patients with idiopathic bronchiectasis: a retrospective cohort study.

Respiratory research2024 Oct 26

Currently, there is a lack of research on multi-drug resistant Pseudomonas aeruginosa (MDR-PA) isolation in bronchiectasis-related hemoptysis. The aim of this study to analyze the risk factors for recurrent hemoptysis following bronchial artery embolization (BAE) and compare the recurrent hemoptysis-free rates between MDR-PA, non-MDR-PA, and non-PA isolation. A retrospective study was performed of patients diagnosed with idiopathic bronchiectasis-related recurrent hemoptysis who underwent BAE at an university-affiliated hospital. Patients were categorized based on PA susceptibility tests into non-PA, non-MDR-PA, and MDR-PA groups. Univariate and multivariate Cox regression were conducted to identify independent risk factors for recurrent hemoptysis. The Kaplan-Meier curves was conducted to compare recurrent hemoptysis-free rates after BAE for non-PA, non-MDR-PA, and MDR-PA. A total of 432 patients were included. 181 (41.90%) patients experienced recurrent hemoptysis during a median follow-up period of 25 months. MDR-PA isolation (adjusted hazard ratio (aHR) 2.120; 95% confidence interval (CI) [1.249, 3.597], p = 0.005) was identified as an independent risk factor for recurrent hemoptysis. Antibiotic treatment (aHR 0.666; 95% CI [0.476, 0.932], p = 0.018) reduced the risk of recurrent hemoptysis. The cumulative recurrent hemoptysis-free rates for non-PA, non-MDR-PA, and MDR-PA were as follows: at 3 months, 88.96%, 88.24%, and 75.86%, respectively; at 1 year, 73.13%, 69.10%, and 51.72%; and at 3 years, 61.91%, 51.69%, and 41.10% (p = 0.034). MDR-PA isolation was an independent risk factor of recurrent hemoptysis post-BAE. Reducing the occurrence of MDR-PA may effectively decrease the recurrence rates of hemoptysis.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC32 artigos no totalmostrando 40

2026

CFTR activity in nasal potential difference of adults with idiopathic bronchiectasis.

Respiratory research
2026

Characteristics of patients with idiopathic bronchiectasis in comparison to post-infectious bronchiectasis in South Korea.

Tuberculosis and respiratory diseases
2025

Phenotyping Bronchiectasis Frequent Exacerbator: A Single Centre Retrospective Cluster Analysis.

Biomedicines
2025

Acrophialophora jodhpurensis and Nocardia in idiopathic bronchiectasis: Colonizers or culprits?'.

Indian journal of medical microbiology
2024

Multi-drug resistant Pseudomonas aeruginosa isolation is an independent risk factor for recurrent hemoptysis after bronchial artery embolization in patients with idiopathic bronchiectasis: a retrospective cohort study.

Respiratory research
2024

Destroyed lung contributes to the recurrence of hemoptysis after bronchial artery embolization in patients with post-tuberculosis bronchiectasis.

Journal of infection and public health
2024

Deciphering Idiopathic Bronchiectasis One Gene at a Time.

American journal of respiratory and critical care medicine
2024

Asymptomatic Dysphagia and Aspiration in Patients with Idiopathic Bronchiectasis.

Lung
2024

Bronchiectasis in renal transplant patients: a cross-sectional study.

European journal of medical research
2023

Idiopathic bronchiectasis. What are we talking about?

Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
2023

Usefulness of High-resolution Computed Tomography for Macrolide Therapy of Idiopathic Bronchiectasis.

The open respiratory medicine journal
2023

Pseudomonas aeruginosa isolation is an important predictor for recurrent hemoptysis after bronchial artery embolization in patients with idiopathic bronchiectasis: a multicenter cohort study.

Respiratory research
2022

Prevalence of chronic rhinosinusitis and its relating factors in patients with bronchiectasis: findings from KMBARC registry.

The Korean journal of internal medicine
2022

Leveraging Open Electronic Health Record Data and Environmental Exposures Data to Derive Insights Into Rare Pulmonary Disease.

Frontiers in artificial intelligence
2021

Heterogeneity of non-cystic-fibrosis bronchiectasis in multiethnic Singapore: A prospective cohort study at a tertiary pulmonology centre.

Annals of the Academy of Medicine, Singapore
2021

Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis.

Seminars in respiratory and critical care medicine
2021

Outcomes of bronchial artery embolization for the management of hemoptysis.

La Tunisie medicale
2021

Bronchial artery embolization: A gratifying life-saving procedure.

The Indian journal of tuberculosis
2020

Performance of Multidimensional Severity Scoring Systems in Patients with Post-Tuberculosis Bronchiectasis.

International journal of chronic obstructive pulmonary disease
2020

Co-infection of SARS-CoV-2 and Bordetella bronchiseptica in a young man with idiopathic non-cystic bronchiectasis and vitamin D3 deficiency.

Respiratory medicine case reports
2020

Characterization of the severity of dyspnea in patients with bronchiectasis: correlation with clinical, functional, and tomographic aspects.

Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
2019

Noncystic Fibrosis Bronchiectasis: Evaluation of an Extensive Diagnostic Protocol in Determining Pediatric Lung Disease Etiology.

Pediatric allergy, immunology, and pulmonology
2020

Impact of mannose-binding lectin 2 gene polymorphisms on disease severity in noncystic fibrosis bronchiectasis in children.

Pediatric pulmonology
2019

New-onset asthma in a bilateral lung transplant patient.

BMJ case reports
2019

[Correlation between chest CT features and clinical characteristics of patients with bronchiectasis].

Zhonghua yi xue za zhi
2018

Achromobacter xylosoxidans in idiopathic cystic bronchiectasis.

BMJ case reports
2018

Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis.

Lung
2018

Survival of pathogenic Mycobacterium abscessus subsp. massiliense in Acanthamoeba castellanii.

Research in microbiology
2017

Etiology of Bronchiectasis in a Cohort of 2047 Patients. An Analysis of the Spanish Historical Bronchiectasis Registry.

Archivos de bronconeumologia
2016

Eradication of Mycobacterium abscessus Pulmonary Infection in a Child With Idiopathic Bronchiectasis.

Global pediatric health
2017

Bronchiectasis in yellow nail syndrome.

Respirology (Carlton, Vic.)
2016

Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants.

Annals of the American Thoracic Society
2016

The Multiple Faces of Non-Cystic Fibrosis Bronchiectasis. A Cluster Analysis Approach.

Annals of the American Thoracic Society
2016

Allergic bronchopulmonary aspergillosis complicating Swyer-James-Macleod's syndrome: case report and review of literature.

European annals of allergy and clinical immunology
2016

Unsupervised learning technique identifies bronchiectasis phenotypes with distinct clinical characteristics.

The international journal of tuberculosis and lung disease : the official journal of the International Union against Tuberculosis and Lung Disease
2016

Effects of functional endoscopic sinus surgery on the treatment of bronchiectasis combined with chronic rhino-sinusitis.

Acta oto-laryngologica
2015

Etiology of Non-Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity.

Annals of the American Thoracic Society
2015

Allergic bronchopulmonary aspergillosis in an adult with Kartagener syndrome.

BMJ case reports
2015

Aetiology and clinical characteristics of patients with bronchiectasis in a Chinese Han population: A prospective study.

Respirology (Carlton, Vic.)
2015

Aetiology of bronchiectasis in Guangzhou, southern China.

Respirology (Carlton, Vic.)

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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.

  1. Characteristics of patients with idiopathic bronchiectasis in comparison to post-infectious bronchiectasis in South Korea.
    Tuberculosis and respiratory diseases· 2026· PMID 41589011mais citado
  2. CFTR activity in nasal potential difference of adults with idiopathic bronchiectasis.
    Respiratory research· 2026· PMID 41776502mais citado
  3. Phenotyping Bronchiectasis Frequent Exacerbator: A Single Centre Retrospective Cluster Analysis.
    Biomedicines· 2025· PMID 41007687mais citado
  4. Acrophialophora jodhpurensis and Nocardia in idiopathic bronchiectasis: Colonizers or culprits?'.
    Indian journal of medical microbiology· 2025· PMID 40975236mais citado
  5. Multi-drug resistant Pseudomonas aeruginosa isolation is an independent risk factor for recurrent hemoptysis after bronchial artery embolization in patients with idiopathic bronchiectasis: a retrospective cohort study.
    Respiratory research· 2024· PMID 39462395mais citado
  6. Alpha-1 Antitrypsin Deficiency Beyond COPD and Emphysema: A Narrative Review.
    Med Sci (Basel)· 2026· PMID 41892821recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:60033(Orphanet)
  2. MONDO:0018956(MONDO)
  3. GARD:16664(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q32778(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

Bronquiectasia idiopática
Compêndio · Raras BR

Bronquiectasia idiopática

ORPHA:60033 · MONDO:0018956
Prevalência
1-5 / 10 000
Herança
Not applicable
CID-10
J47 · Bronquectasia
CID-11
Início
All ages
Prevalência
29.0 (Europe)
MedGen
UMLS
C0006267
EuropePMC
Wikidata
Wikipedia
Papers 10a
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