Em audiologia, perda auditiva periférica é um déficit auditivo de etiologia multifatorial que pode sofrer influência de fatores ambientais ou genéticos, que se manifesta em duas classificações principais, perda condutiva e perda neurosensorial, que tem como origem porções específicas do sistema audiorreceptor. Além disso, atualmente também adota-se a caracterização para a perda auditiva mista, perda auditiva funcional e por deficiência auditiva central.
Introdução
O que você precisa saber de cara
Malformação congênita das vias aéreas pulmonares, tipo 1, é uma anomalia rara caracterizada por cistos únicos ou múltiplos na região pulmonar. Frequentemente, apresenta-se com sintomas respiratórios ou é assintomática, sendo diagnosticada por imagem.
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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
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 — Malformação congênita das vias aéreas pulmonares, tipo 1
Centros de Referência SUS
24 centros habilitados pelo SUS para Malformação congênita das vias aéreas pulmonares, tipo 1
Centros para Malformação congênita das vias aéreas pulmonares, tipo 1
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
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
Type 1 Congenital Pulmonary Airway Malformation: Diagnostic Accuracy of Radiological Features.
There are several different types of congenital lung malformation (CLM). Malignant transformation to adenocarcinoma is almost exclusively associated with type 1 congenital pulmonary airway malformation (CPAM). Histological examination is the only reliable method to define malformation subtype. To determine if the largest cyst diameter on CT imaging is an accurate diagnostic test for the presence or absence of type 1 CPAM on histological analysis. All cases of antenatally identified CLM that underwent surgical resection between 2004 and 2023 were identified. The index test (diameter of largest cyst on CT imaging) was determined for each case. The reference standard test was the diagnosis of type 1 CPAM on the histopathology report. Measures of accuracy of the index test in detecting the presence or absence of type 1 CPAM were calculated. For asymptomatic cases (n = 117) the prevalence of type 1 CPAM was 23.9%. Largest cyst diameter discriminated well between those with and without type 1 CPAM, with an area under the receiver operating characteristic curve of 0.852 (95% CI 0.77-0.91, p < 0.001). At a threshold diameter of ≥ 5 mm the index test performed accurately: Sensitivity 85.7% (95% CI 67.3-96.0), specificity 76.5% (95% CI 65.8-85.2), positive predictive value 55.8% (95% CI 45.3-65.8) and negative predictive value 93.9% (95% CI 86.1-97.5). In this cohort type 1 CPAM has a low prevalence and could be accurately distinguished through radiological features.
Surgical Management of Congenital Lung Malformations in Children-A Single-Center Analysis of 25 Years of Experience.
Congenital lung malformations (CLMs) in pediatric patients encompass various structural abnormalities arising during fetal development, which can range from benign to life-threatening. The most common types include congenital pulmonary airway malformation (CPAM) and bronchopulmonary sequestration (BPS). This study aimed to retrospectively analyze patients treated surgically for CLMs, focusing on indications for surgery, surgical techniques, and outcomes. Data were collected from the medical records of patients who underwent thoracoscopy (n = 140) or thoracotomy (n = 52) between 2000 and 2024. Among these, 50 patients were diagnosed with CLMs, who were taken for further analysis. Study group inclusion criteria were performing a CT/X-ray imaging examination indicating the presence of a defect, surgery, and available pathology results. Exclusion criteria were incomplete data or lack of surgical procedure. Final study group included 37 patients who met inclusion criteria for further analysis. Detailed analysis encompassed demographics, clinical presentation, diagnostic methods, treatment, and follow-up. The cohort included patients diagnosed with CPAM type I (n = 12), CPAM type II (n = 7), pulmonary sequestration (n = 10), and other congenital malformations such as bronchogenic cyst (n = 2), congenital cystic pulmonary disease (n = 2), CPAM type IV-pleuropulmonary blastoma type I (PPB) (n = 1), juvenile emphysema (n = 2), and mediastinal cyst (n = 1). The average age at diagnosis was 37.61 months. The cohort consisted of 17 females and 20 males. The right lung was involved in 41.18% of cases, and the left lung in 58.82%. Symptoms at presentation included pneumonia (n = 9), respiratory failure (n = 8), emphysema (n = 3), and pneumothorax (n = 2). Fifteen patients were asymptomatic, and the diagnosis was incidental. Seven patients had other congenital diseases, such as heart defects. None of the patients other than the child with PPB were offered genetic diagnostics, albeit for DICER1 or KRAS mutations. The study underscores the heterogeneity in age and clinical presentation at the time of CLM diagnosis, highlighting the importance of an individualized and tailored approach to management.
Challenges with Congenital Lung Cysts: When to Consider DICER1 Testing? A Narrative Review.
Congenital pulmonary airway malformations (CPAMs), according to the Stocker classification, comprise five types, two of which are non-cystic in nature (types 0 and 3) whereas the others present as cystic lung lesions (types 1, 2 and 4). While there is consensus that symptomatic lesions should be managed surgically, the asymptomatic cases are more problematic in terms of therapeutic intervention. The dilemma is further complicated by CPAM types 1 and 4 and their preneoplastic potential. In the case of CPAM type 1, there are reports of lipidic adenocarcinoma arising from the mucinous component. Type 4 has been equated to type I or cystic pleuropulmonary blastoma (PPB), a proposition that has been challenged in the past. Pleuropulmonary blastoma is associated with a heterozygous germline or somatic variants in DICER1. It was recognized that the earliest stage of the sarcomatous progression of PPB was a multicystic lesion in the periphery of the lung whose architectural features were identical to CPAM type 4. This narrative review addresses key aspects of the pathogenetic and diagnostic issues of type I PPB/CPAM type 4, as well as the association of DICER1 and PPB, offering valuable insights for pediatricians and clinicians caring for young adults who are impacted by the presence of a germline DICER1 variant.
Perioperative Care for Pediatric Patients Undergoing Lung Surgery: Retrospective Single Center Review.
Procedures involving lung surgery in the pediatric population are relatively uncommon and tend to be centralized in a limited number of institutions. Anesthesia literature is also sparse. To have a clear overview of frequency, underlying pathologies, ICU and hospital stay, anesthetic techniques, one lung ventilation, and perioperative analgesia. We conducted a retrospective review in a single-center tertiary hospital, from January 2014 to 2023. We included children aged 0-16 years who underwent major lung surgery and received anesthesia managed by the pediatric anesthesia team. Patients with congenital diaphragmatic hernia, esophageal atresia, or those undergoing surgery for pectus excavatum were excluded. Our main outcome measures include the type of underlying pathology and surgical procedure, ICU and hospital stay, methods of one-lung ventilation, source of perioperative analgesia, and the incidence of (postoperative) complications. We included 73 patients, 55% male and 45% female. The median age was 2.8 years and the median weight was 12.9 kg. Congenital pulmonary airway malformation was diagnosed in 43%, and 45% underwent a (partial) lobectomy. The proportion of video-assisted thoracoscopic surgery was comparable to that of open thoracotomy. One-lung ventilation (OLV) was used in 81%, primarily facilitated by a bronchial blocker. Epidural catheterization with ropivacaine for perioperative pain management was used in 71%. The proportion of patients receiving intravenous morphine on postoperative Days 1, 2, 3, 4, and 5 was 40%, 34%, 19%, 15%, and 11%, respectively. Insufficient pain control was reported in 14%. 70% were admitted to the ICU for one night. The average length of hospital stay was 8 days. We addressed the anesthetic care of pediatric lung surgery procedures. OLV was required in the majority of the population and a bronchial blocker was the preferred method. Epidural analgesia was the preferred choice to tackle perioperative pain.
Spontaneous pneumothorax as the initial presentation of congenital pulmonary airway malformation in a teenager without prior symptoms.
Congenital Pulmonary Airway Malformation (CPAM) is a rare developmental lung anomaly, usually diagnosed prenatally but often asymptomatic until early childhood. It can present with respiratory distress, recurrent infections, or rarely, spontaneous pneumothorax (PTX). We report a 15-year-old female who presented with spontaneous PTX. After chest tube placement and lung re-expansion, imaging revealed an 8 cm cystic lesion in the left lower lobe, which was surgically resected. Histopathology confirmed CPAM type 1. While PTX in adolescents is typically due to primary spontaneous causes, infection, or trauma, this case highlights the need to consider congenital anomalies like CPAM in the differential diagnosis, especially in patients without neoplastic or vascular risk factors.
Publicações recentes
Ver todas no PubMed📚 EuropePMC253 artigos no totalmostrando 119
Type 1 Congenital Pulmonary Airway Malformation: Diagnostic Accuracy of Radiological Features.
Pediatric pulmonologySurgical Management of Congenital Lung Malformations in Children-A Single-Center Analysis of 25 Years of Experience.
The clinical respiratory journalChallenges with Congenital Lung Cysts: When to Consider DICER1 Testing? A Narrative Review.
Acta medica portuguesaPerioperative Care for Pediatric Patients Undergoing Lung Surgery: Retrospective Single Center Review.
Paediatric anaesthesiaSpontaneous pneumothorax as the initial presentation of congenital pulmonary airway malformation in a teenager without prior symptoms.
Radiology case reportsThe essential role of cytoskeleton and ciliary abnormalities in the development of congenital pulmonary airway malformations.
Pediatric surgery internationalA Case of Type 1 Congenital Pulmonary Airway Malformation (CPAM) in an Eight-Year-Old Girl Without a History of Recurrent Pneumonia or Respiratory Failure, Presenting With a Pulmonary Abscess.
CureusCongenital Lung Malformations: A Comprehensive Overview of Current Knowledge-Narrative Review.
Archivos de bronconeumologiaType III Congenital Pulmonary Airway Malformation: A Case Report.
Iranian journal of pathologyNeonatal Congenital Pulmonary Airway Malformation Causing Respiratory Failure Managed With Early Surgical Resection: A Case Report.
British journal of hospital medicine (London, England : 2005)Atypical mesenchyme in congenital pulmonary airways malformation: a promising new focus.
Respiratory researchMultiple congenital pulmonary malformations in a black-capped Bolivian squirrel monkey (Saimiri boliviensis boliviensis).
Journal of comparative pathologyA diagnostic challenge: The unusual combination of Kommerell's diverticulum and pulmonary sequestration due to type 1 congenital pulmonary airway malformation in a young patient.
Radiology case reportsComputed tomography (CT)-based classification and management pathways for hybrid lesions in children: a comparative analysis of congenital pulmonary airway malformation (CPAM) and bronchopulmonary sequestration (BPS).
Clinical radiologyCritical Care Dilemma: Complex Congenital Pulmonary Airway Malformation With Pulmonary Hypertension in a Neonate.
CureusCongenital pulmonary airway malformation type 2 associated with intralobar pulmonary sequestration in an adult patient: a case report.
Future science OACongenital Pulmonary Airway Malformation with Pulmonary Arteriovenous Malformation in Adulthood: A Case Report.
Surgical case reportsComprehensive morphological, immunohistochemical, and molecular characterization of congenital pulmonary airway malformation (CPAM).
Virchows Archiv : an international journal of pathologyCharacterization of 45 cases of congenital lung malformation and association of inflammation with symptomatology.
Scientific reportsRespiratory assessment and management of newborns and children with congenital lung diseases: a cohort study.
Italian journal of pediatricsCongenital bronchogenic cyst: A case study on early detection and surgical intervention.
International journal of surgery case reportsNeonatal Surgery for Congenital Lung Malformations: Indications, Outcomes and Association With Malignancy.
Journal of pediatric surgeryCongenital Pulmonary Hybrid Lesions: Clinical Presentation, Surgical Management, and Outcomes.
Indian journal of pediatricsClinical features of atypical solid-type congenital pulmonary airway malformation (CPAM) type 1.
Pediatrics and neonatologyCongenital pulmonary airway malformation in a cat.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncCongenital cystic adenomatoid malformation in a toddler: Unusual presentation with pleural effusion.
Radiology case reportsInvasive Mucinous Adenocarcinoma in a Newborn With Antenatally Diagnosed Congenital Pulmonary Airway Malformation: A Case Report.
CureusCongenital Pulmonary Airway Malformation in Preterm Infants: A Case Report and Review of the Literature.
Life (Basel, Switzerland)Clinical and histopathological spectrum of congenital pulmonary airway malformations: A case series.
International journal of surgery case reportsIntegrative analysis of bulk and single-cell RNA sequencing reveals the gene expression profile and the critical signaling pathways of type II CPAM.
Cell & bioscienceThe ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran.
BMC pediatricsDefective mesenchymal Bmpr1a-mediated BMP signaling causes congenital pulmonary cysts.
eLifeTracheal agenesis versus tracheal atresia: anatomical conditions, pathomechanisms and causes with a possible link to a novel MAPK11 variant in one case.
Orphanet journal of rare diseases[Clinicopathological and genetic characteristics of congenital cystic adenomatoid malformation of lung and its associated lung cancer in adults].
Zhonghua bing li xue za zhi = Chinese journal of pathologyExperience of video-assisted thoracic surgery treatment of congenital pulmonary airway malformation in infants less than 3 months of age.
Translational pediatricsRare presentation of pneumothorax in a young woman with underlying congenital pulmonary airway malformation.
BMJ case reportsThe Effect of Steroids on Prenatally Diagnosed Lung Lesions.
Journal of pediatric surgeryNo Pathogenic DICER1 Gene Variants in a Cohort Study of 28 Children With Congenital Pulmonary Airway Malformation.
Journal of pediatric surgeryInfantile type I pleuropulmonary blastoma presenting with dyspnea due to compression by pneumothorax and an occupying tumor: a case report.
Surgical case reportsA complete spectrum of congenital cystic adenomatoid malformation of the lung deceptive clinical presentations and histological surprises; a single institutional study from a tertiary care hospital in North India.
Lung India : official organ of Indian Chest SocietyCongenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020.
Boletin medico del Hospital Infantil de MexicoCongenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyEarly resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature.
BMC pediatricsLung ultrasound description of a newborn with bronchial atresia: A case report.
Ultrasound (Leeds, England)Congenital pleuropulmonary blastoma in a newborn with a variant of uncertain significance in DICER1 evaluated by RNA-sequencing.
Maternal health, neonatology and perinatologyMorphologic Features in Congenital Pulmonary Airway Malformations and Pulmonary Sequestrations Correlate With Mutation Status: A Mechanistic Approach to Classification.
The American journal of surgical pathologyThe long-term outcome following thoraco-amniotic shunting for congenital lung malformations.
Journal of pediatric surgeryType II congenital pulmonary airway malformation with primary ciliary dyskinesia in a 4-year-old child: A case report.
Pediatric pulmonologyModified thoracoscopic wedge resection of limited peripheral lesions in S10 for children with congenital pulmonary airway malformation: Initial single-center experience.
Frontiers in pediatricsThoracoscopic repair of neonatal left diaphragmatic hernia with sac combined with both extralobar pulmonary sequestration and congenital pulmonary airway malformation.
Asian journal of endoscopic surgeryThoracoscopic segmentectomy for congenital pulmonary airway malformation arising in a lung with an accessory fissure: A case report.
Asian journal of endoscopic surgeryExtralobar Pulmonary Sequestration in Adrenal Mimicking Neuroblastoma: A Case Report.
Fetal and pediatric pathologyInnovative Fetal Therapy for a Giant Congenital Pulmonary Airway Malformation with Hydrops.
Fetal diagnosis and therapyMultimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.
Seminars in ultrasound, CT, and MRSarcomatoid change in adenocarcinoma arising in adulthood congenital pulmonary airway malformation.
Advances in respiratory medicineNeonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.
Journal of pediatric surgeryP1-20: Adulthood presentation of congenital pulmonary airway malformation type-1.
Respirology (Carlton, Vic.)Application and effects of an early childhood education machine on analgesia and sedation in children after cardiothoracic surgery.
Journal of cardiothoracic surgeryAn adult with episodic retrosternal chest pain: an unusual presentation of congenital pulmonary airway malformation - case report.
Journal of cardiothoracic surgeryMucinous Adenocarcinoma With Intrapulmonary Metastasis Harboring KRAS and GNAS Mutations Arising in Congenital Pulmonary Airway Malformation.
American journal of clinical pathologyFetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions.
Pediatric radiologyPrediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyLeft interval thoracoscopic pneumonectomy for type II communicating bronchopulmonary foregut malformation in a 17-month-old girl.
International journal of surgery case reportsObstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations.
Journal of neonatal-perinatal medicineDeletion of Yy1 in mouse lung epithelium unveils molecular mechanisms governing pleuropulmonary blastoma pathogenesis.
Disease models & mechanismsPleuropulmonary blastoma: Difficulty in diagnosis and treatment of a case in Vietnam.
Pediatric hematology and oncologySingle-cell transcriptome profiling reveals the mechanism of abnormal proliferation of epithelial cells in congenital cystic adenomatoid malformation.
Experimental cell researchMucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases.
HistopathologyEarly KRAS oncogenic driver mutations in nonmucinous tissue of congenital pulmonary airway malformations as an indicator of potential malignant behavior.
Human pathologyComparison of the Prognostic Factors of Fetuses With Congenital Pulmonary Airway Malformations According to Type.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in MedicineThoracoscopic Resection in the Treatment of Spontaneous Pneumothorax.
Sisli Etfal Hastanesi tip bulteniComputed tomography features can distinguish type 4 congenital pulmonary airway malformation from other cystic congenital pulmonary airway malformations.
European journal of radiologyPleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer 1 mutation.
Virchows Archiv : an international journal of pathologyA case report of an unusual non-mucinous papillary variant of CPAM type 1 with KRAS mutations.
BMC pulmonary medicineNew insights on congenital pulmonary airways malformations revealed by proteomic analyses.
Orphanet journal of rare diseasesObstetric and neonatal outcomes in pregnancies complicated by fetal lung masses: does final histology matter?
The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal ObstetriciansType I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases.
Indian journal of pathology & microbiologyValue of routine ultrasound examination at 35-37 weeks' gestation in diagnosis of fetal abnormalities.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyAn adult patient with congenital pulmonary airway malformation and an esophageal cyst.
Annals of translational medicineCongenital Cystic Adenomatoid Malformation Volume Ratio in Prenatal Assessment of Prognosis of Fetal Pulmonary Sequestrations.
Current medical scienceInvasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report.
Acta chirurgica BelgicaGiant Secondary Overgrowth of Type-1 Pulmonary Cystic Airway Malformation Upon Development of Anaplastic Lymphoma Kinase-Rearranged Adenocarcinoma.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerAlteration of cystic airway mesenchyme in congenital pulmonary airway malformation.
Scientific reportsManifestation of congenital pulmonary airway malformation in a 26-year-old adult, requiring surgery.
Interactive cardiovascular and thoracic surgeryCystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation.
Annals of diagnostic pathologyIn prenatally diagnosed CPAM, does the affected lobe influence the timing of symptom onset?
Pediatric surgery internationalEarly vs late resection of asymptomatic congenital lung malformations.
Journal of pediatric surgeryThe Use of Fetal Bronchoscopy in the Diagnosis and Management of a Suspected Obstructive Lung Mass.
AJP reportsCongenital cystic adenomatoid malformation in adults detected after infection.
Respirology case reportsAn investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.
Journal of pediatric surgery[Pulmonary lobectomy in children: the sooner the better?].
Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia PediatricaCongenital cystic adenomatoid malformation in adults, presenting as a single cyst.
Asian cardiovascular & thoracic annalsCongenital cystic adenomatoid malformation - diagnostic and therapeutic procedure: 8-year experience of one medical centre.
Kardiochirurgia i torakochirurgia polska = Polish journal of cardio-thoracic surgeryTypes II and III congenital pulmonary airway malformation with hydrops treated in utero with percutaneous sclerotherapy.
Prenatal diagnosisAccessory scrotum and congenital perineal lipoma in a child with type 2 congenital pulmonary airway malformation: A report of an unusual.
International journal of health sciencesCase of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate.
Korean journal of pediatricsCongenital Cystic Adenomatoid Malformation Diagnosed During First-Trimester Ultrasound Scan.
The American journal of case reportsCongenital Cystic Lung Lesions: Redefining the Natural Distribution of Subtypes and Assessing the Risk of Malignancy.
The American journal of surgical pathologyIt's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation.
The Thoracic and cardiovascular surgeon reportsDiagnosis and treatment of communicating bronchopulmonary foregut malformation: Report of two cases and review of the literature.
MedicineType II congenital pulmonary airway malformation associated with intralobar pulmonary sequestration: report of a case and review of classification criteria.
PathologicaAssociation between Congenital Lung Malformations and Lung Tumors in Children and Adults: A Systematic Review.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerPulmonary Kirsten Rat Sarcoma Virus Mutation Positive Mucinous Adenocarcinoma Arising in a Congenital Pulmonary Airway Malformation, Mixed Type 1 and 2.
The Annals of thoracic surgeryType I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV.
Neonatology[FETAL THERAPY: INTRAUTERINE THORACO-AMNIOTIC SHUNTING IN MACROCYSTIC TYPE CYSTIC ADENOMATOID MALFORMATION OF THE LUNG: REVIEW OF THE LITERATURE AND CASE REPORT].
Akusherstvo i ginekologiiaThoracoamniotic Shunts in Macrocystic Lung Lesions: Case Series and Review of the Literature.
Fetal diagnosis and therapyOutcome of infants operated on for congenital pulmonary malformations.
Pediatric pulmonologyPulmonary Arteriovenous Fistula: Clinical and Histologic Spectrum of Four Cases.
Journal of pathology and translational medicineRetrospective Evaluation of Children with Congenital Pulmonary Airway Malformation: A Single Center Experience of 20 Years.
Fetal and pediatric pathologyCongenital Cystic Adenomatoid Malformation, Type II: A Rare Cause of Haemoptysis.
The Indian journal of chest diseases & allied sciences[Pediatric lung lesions: a clinicopathological study of 215 cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyCan congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?
Journal of pediatric surgeryCongenital cystic adenomatoid malformation type I.
Autopsy & case reportsExtralobar sequestration of lung associated with congenital diaphragmatic hernia and malrotation of gut.
Lung India : official organ of Indian Chest SocietyCongenital pulmonary airway malformation: A report of two cases.
World journal of clinical cases[Congenital pulmonary airway malformation of lung in fetus: a clinicopathological analysis].
Zhonghua bing li xue za zhi = Chinese journal of pathologyUnusual signal intensity of congenital pulmonary airway malformation on fetal magnetic resonance imaging.
Pediatric radiologyDiffuse capillary malformation in association with fetal pleural effusion: report of five patients.
Pediatric dermatologyExtracellular matrix pleural tent for persistent air leak and air space in a child after upper lobectomy.
The Annals of thoracic surgeryAssociações
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Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
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.
- Type 1 Congenital Pulmonary Airway Malformation: Diagnostic Accuracy of Radiological Features.
- Surgical Management of Congenital Lung Malformations in Children-A Single-Center Analysis of 25 Years of Experience.
- Challenges with Congenital Lung Cysts: When to Consider DICER1 Testing? A Narrative Review.
- Perioperative Care for Pediatric Patients Undergoing Lung Surgery: Retrospective Single Center Review.
- Spontaneous pneumothorax as the initial presentation of congenital pulmonary airway malformation in a teenager without prior symptoms.
- Diagnostic Challenges in a Hybrid Congenital Pulmonary Airway Malformation: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:280832(Orphanet)
- MONDO:0017249(MONDO)
- GARD:21094(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55786936(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
