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[PMID]:29465555
[Au] Autor:Sheth HS; Maldonado F; Lentz RJ
[Ad] Endereço:D. Y. Patil University School of Medicine, Mumbai, India.
[Ti] Título:Two cases of Dieulafoy lesions of the bronchus with novel comorbid associations and endobronchial ablative management.
[So] Source:Medicine (Baltimore);97(8):e9754, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Dieulafoy lesions are aberrantly large submucosal arteries most frequently associated with gastrointestinal hemorrhage. They are rarely identified in the bronchial submucosa and can cause massive hemoptysis. PATIENT CONCERNS: We present three episodes of massive hemoptysis in two patients, the first with comorbid Alagille syndrome including multiple cardiac and pulmonary vascular abnormalities and the second with thyroid cancer metastatic to the mediastinum. DIAGNOSES: All episodes were due to Dieulafoy lesions of the bronchus based on bronchoscopic appearance. INTERVENTIONS: Bronchoscopic ablation using Nd:YAP laser was attempted both patients. OUTCOMES: Nd:YAP laser successfully ablated the Dieulafoy lesion in the first case with long-term relief from recurrent hemoptysis. The first episode in the second patient responded to bronchial artery embolization; laser ablation of a different Dieulafoy lesion responsible for the second episode was unsuccessful but additional bronchial artery embolization has provided relief from further episodes. LESSONS: Bronchoscopic ablation of Dieulafoy lesions of the bronchus can provide durable relief from recurrent symptoms. Clinical and anatomical features should be considered carefully before intervention, which should only be attempted by experienced operators with appropriate ancillary support available.
[Mh] Termos MeSH primário: Técnicas de Ablação/métodos
Broncopatias/cirurgia
Broncoscopia/métodos
Hemoptise/cirurgia
Malformações Vasculares/cirurgia
[Mh] Termos MeSH secundário: Idoso
Síndrome de Alagille/patologia
Brônquios/irrigação sanguínea
Brônquios/cirurgia
Broncopatias/complicações
Comorbidade
Feminino
Hemoptise/etiologia
Seres Humanos
Masculino
Neoplasias do Mediastino/secundário
Meia-Idade
Neoplasias da Glândula Tireoide/patologia
Malformações Vasculares/complicações
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180302
[Lr] Data última revisão:
180302
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180222
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009754


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[PMID]:28743768
[Au] Autor:Savvidou S; Koco E; Tsagourias M; Matamis D
[Ad] Endereço:Department of ICU, 'Papageorgiou' General Hospital, Thessaloniki, Greece.
[Ti] Título:A central venous catheter that revealed a vascular paradox.
[So] Source:Thorax;72(10):957-959, 2017 10.
[Is] ISSN:1468-3296
[Cp] País de publicação:England
[La] Idioma:eng
[Mh] Termos MeSH primário: Veias Braquiocefálicas/anormalidades
Cateterismo Venoso Central
Veias Pulmonares/anormalidades
Malformações Vasculares/diagnóstico
[Mh] Termos MeSH secundário: Remoção de Dispositivo
Diagnóstico Diferencial
Ecocardiografia Transesofagiana
Seres Humanos
Masculino
Meia-Idade
Radiografia Torácica
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; VIDEO-AUDIO MEDIA
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180215
[Lr] Data última revisão:
180215
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170727
[St] Status:MEDLINE
[do] DOI:10.1136/thoraxjnl-2017-210210


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[PMID]:28468163
[Au] Autor:Abukawa H; Kono M; Hamada H; Okamoto A; Satomi T; Chikazu D
[Ad] Endereço:*Oral and Maxillofacial Surgery Service, Tokyo Medical University Hachioji Medical Center †Department of Oral and Maxillofacial Surgery, Tokyo Medical University Hospital, Tokyo ‡Oral and Maxillofacial Surgery Service, Tokyo Medical University Ibaraki Medical Center, Ibaraki §Department of Oral and Maxillofacial Surgery, Tokyo Medical University, Tokyo, Japan.
[Ti] Título:Indications of Potassium Titanyl Phosphate Laser Therapy for Slow-Flow Vascular Malformations in Oral Region.
[So] Source:J Craniofac Surg;28(3):771-774, 2017 May.
[Is] ISSN:1536-3732
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Indications for laser therapy for slow-flow vascular malformations in the oral and maxillofacial regions have not been clearly documented. The authors aimed to estimate the frequency of resolution of slow-flow vascular malformations and to identify risk and prognostic factors associated with resolution in potassium titanyl phosphate (KTP) laser treatment. METHODS: This study was designed as a prospective cohort study. Patients who had diagnosed slow-flow vascular malformations were continuously assigned to receive KTP laser therapy. All patients had intralesional laser photocoagulation performed under local anesthesia. Administered power of the KTP laser was fixed at 2 watts throughout the procedure in all patients. The primary endpoint was to understand the frequency of resolution of slow-flow vascular malformations in KTP laser treatment. Secondary endpoints were: treatment outcomes based on lesion size; treatment outcomes based on location; treatment outcomes based on total energy in joules; types of complications. Treatment outcomes were judged by a clinical assessment as well as reduction in lesion size on magnetic resonance imaging. RESULTS: Data were obtained from 26 patients (9 men, 17 women) with 38 lesions. The average lesion size was 13.5 ±â€Š7.7 mm. Treatment outcomes based on lesion size showed that cure and regression were obtained in lesions less than 30 mm in size. However, lesions larger than 30 mm showed no response. Lesions in the tongue and lips showed higher cure rates than in other areas. Treatment outcomes based on administered total energy in joules showed that 68% of lesions were treated and responded well at less than 400 joules. Complication rate was relatively high in the buccal mucosal lesions. Immediate postoperative complications such as necrosis were more common in high-energy administration than in low-energy administration. CONCLUSION: Our results indicated that KTP laser therapy was effective for slow-flow vascular malformations less than 30 mm in size without significant side effects.
[Mh] Termos MeSH primário: Velocidade do Fluxo Sanguíneo/fisiologia
Terapia a Laser/métodos
Lasers de Estado Sólido/uso terapêutico
Boca/irrigação sanguínea
Fosfatos
Cirurgia Bucal/métodos
Titânio
Malformações Vasculares/cirurgia
[Mh] Termos MeSH secundário: Feminino
Seguimentos
Hemodinâmica
Seres Humanos
Terapia com Luz de Baixa Intensidade/métodos
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Estudos Prospectivos
Resultado do Tratamento
Ultrassonografia Doppler em Cores
Malformações Vasculares/diagnóstico
Malformações Vasculares/fisiopatologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Phosphates); 12690-20-9 (potassium titanylphosphate); D1JT611TNE (Titanium)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180209
[Lr] Data última revisão:
180209
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000003445


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[PMID]:29262449
[Au] Autor:Ge CX; Tai MZ; Chen T; Li KL; Qin ZP
[Ad] Endereço:Special Department of Hemangiomas, Linyi Tumor Hospital, Linyi 276001, Shandong Province, China.
[Ti] Título:[Treatment analyses of 143 patients with maxillofacial and cervical venous malformations involved in isthmus faucium area].
[So] Source:Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi;52(12):909-914, 2017 Dec 07.
[Is] ISSN:1673-0860
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:To analyze the clinical data and summarize therapeutic experiences of cervicofacial venous malformations involving isthmus faucium area. Clinical records from 143 patients with venous malformations involving isthmus faucium area treated at our hospital between January 2012 and January 2016 were reviewed. There were 70 males and 73 females. Age ranged from 1 to 52 years old, with a median age of 14.5 years. There were 19 cases with lesions involving in only 1 subanatomic area above and 124 cases with lesions involving in more than 1 subanatomic areas, including 63 cases with lesions involving in more than 2 areas. There were 50 patients presenting with additional maxillofacial and cervical lesions. Clinical symptoms included snoring ( =98), indistinct phonation ( =49), and tonsil hypertrophy more than degree â…¡ ( =19). Tracheotomy was performed in 3 patients prior to hospitalization, contigency tracheotomy during hospitalization in 10 patients, and oral trachea cannula in other patients. All therapeutic procedures, including single chemical ablation with ethanol injection ( =94), single lesion resection ( =9) and both of them ( =40), were performed under general anesthesia. Treatment remedies included mesh suture, macroglossia reduction and excision of maxillofacial and cervical lesions for patients presenting with extensive malformations extending to maxillofacial and cervical area. Tonsil resection were done in patients having tonsil venous malformations or tonsil hypertrophy more than degree II. Achauer's 4-grade criterion was applied to evaluate the treatment outcomes. SPSS 18.0 software was used to analyze the data. Trachea cannula were not extubated untill 24 to 48 hours after treatment. Emergency tracheotomy was done in 2 cases after extubations because of dyspnea, and successful extubations were obtained in other cases. There were no advents of pulmonary vascular spasm or pulmonary embolism. There was significant difference between before and after operation (snore: χ(2)=105.431, ambiguous pronunciation: χ(2)=59.698, tonsil hypertrophy more than degree â…¡: χ(2)=33.530, all <0.01). The patients were followed-up for 1-4 years, and there were 123 cases at grade â…£ (complete disappear of lesions in 62 cases without recurrence), 17 at grade â…¢ , 3 at grade â…¡, and no case at gradeâ… . Chemical ablation with ethanol injection for venous malformations involving isthmus faucium area is recommended, wheras combined remedies including injection, mesh suture, macroglossia reduction, and excision of cervicofacial lesions are suggested in treatment of extensive lesions extending to maxillofacial and cervical area. Tonsil resection should be done in patients having tonsil venous malformations or tonsil hypertrophy more than degree â…¡, which is safe and highly effective, with good reservation of function, in the treatment of maxillofacial and cervical venous malformations involving isthmus faucium area.
[Mh] Termos MeSH primário: Etanol/administração & dosagem
Orofaringe/irrigação sanguínea
Malformações Vasculares/terapia
Veias/anormalidades
[Mh] Termos MeSH secundário: Adolescente
Adulto
Criança
Pré-Escolar
Terapia Combinada/métodos
Feminino
Seres Humanos
Hipertrofia/cirurgia
Lactente
Injeções
Masculino
Meia-Idade
Pescoço/irrigação sanguínea
Tonsila Palatina/irrigação sanguínea
Tonsila Palatina/patologia
Tonsila Palatina/cirurgia
Recidiva
Traqueotomia
Resultado do Tratamento
Malformações Vasculares/complicações
Malformações Vasculares/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
3K9958V90M (Ethanol)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180207
[Lr] Data última revisão:
180207
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171221
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.1673-0860.2017.12.007


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[PMID]:28468227
[Au] Autor:Ensari N; Gür ÖE; Selçuk ÖT; Renda L; Osma Ü; Eyigör H; Çekiç B
[Ad] Endereço:*Department of ENT †Department of Radiology, Antalya Training and Research Hospital, Antalya, Turkey.
[Ti] Título:Is Presence of Vascular Loop in Magnetic Resonance Imaging Always Related to Tinnitus?
[So] Source:J Craniofac Surg;28(3):e295-e298, 2017 May.
[Is] ISSN:1536-3732
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: One of the most common otological complaints is tinnitus in adults. When there is a complaint of unilateral tinnitus and retrocochlear pathology is suspected, imaging methods are applied. However, the imaging findings obtained may not always be compatible with the severity and localization of the tinnitus. AIM: The aim of the present study was to determine whether or not there was a significant correlation between the findings on magnetic resonance imaging (MRI) and the presence and severity of tinnitus in patients with the complaint of unilateral tinnitus. METHOD: The study included 44 patients with no loss of hearing who presented with the complaint of unilateral tinnitus. The relationship between tinnitus severity and vascular loop presence was investigated. RESULTS: Various types of vascular loop were determined in 14 patients. No statistically significant relationship was determined between the presence of vascular loop and the Tinnitus Handicap Inventory scores. CONCLUSION: The results of this study showed no significant difference in respect of the presence of vascular loop on the MRI findings of symptomatic and healthy ears. The presence of vascular loop on MRI is not always a pathological event and should be considered only as an examination finding that could be an anatomic variation.
[Mh] Termos MeSH primário: Artéria Basilar/anormalidades
Imagem por Ressonância Magnética
Zumbido/etiologia
Malformações Vasculares/diagnóstico por imagem
Artéria Vertebral/anormalidades
[Mh] Termos MeSH secundário: Artéria Basilar/diagnóstico por imagem
Feminino
Seres Humanos
Masculino
Índice de Gravidade de Doença
Zumbido/diagnóstico por imagem
Malformações Vasculares/complicações
Artéria Vertebral/diagnóstico por imagem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180202
[Lr] Data última revisão:
180202
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000003546


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[PMID]:28468226
[Au] Autor:Eom YS; Lim SY
[Ad] Endereço:Department of Plastic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
[Ti] Título:Characteristics of Pediatric Cheek Mass.
[So] Source:J Craniofac Surg;28(3):e292-e295, 2017 May.
[Is] ISSN:1536-3732
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: Patients with cheek mass are often referred to our medical center's department of plastic surgery. Most patients with deep cheek mass have the characteristic of a slowly growing, painless mass. We reviewed the lesion's pathology of this complex anatomic area in the pediatric population, focusing on differential diagnosis and the recent surgical strategies. METHODS: We retrospectively reviewed 56 patients visiting our department from 2009 to 2016. Data analysis included the patients' lesion characteristics, clinical presentation, presumptive diagnosis, results of preoperative investigations, pathological diagnosis, and treatment details. RESULTS: Patient age ranged from 1 day to 19 years (mean 7.81 years). The most common presentation was an asymptomatic, slowly enlarging mass. Preoperative diagnoses were made after clinical examination, and then the imaging study was performed. Surgical intervention was performed in 31 patients. The most common histopathological types were vascular anomalies. The next order of prevalence, from most prevalent to least, was lipomatosis, neurofibroma, and teratoma cases. CONCLUSION: As for our patients, various histopathological types of pediatric cheek mass were presented. Being suspicious of a rare disease and having a prudent diagnostic skill were important for a successful diagnosis. The primary treatment of these lesions is surgical excision with a meticulous surgical approach. In our patients, the prognosis of such treatment was good. This study provides a differential diagnosis of cheek mass in the pediatric population and signifies that the prevalence and types of cheek mass in pediatrics are different from those of the adult population.
[Mh] Termos MeSH primário: Bochecha
Lipomatose/diagnóstico
Neurofibroma/diagnóstico
Teratoma/diagnóstico
Malformações Vasculares/diagnóstico
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Diagnóstico Diferencial
Feminino
Seres Humanos
Lactente
Recém-Nascido
Lipomatose/cirurgia
Masculino
Neurofibroma/cirurgia
Estudos Retrospectivos
Teratoma/cirurgia
Malformações Vasculares/cirurgia
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180202
[Lr] Data última revisão:
180202
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE
[do] DOI:10.1097/SCS.0000000000003547


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[PMID]:28455146
[Au] Autor:Busch K; Chandra R; Buckenham T; Kiat H
[Ad] Endereço:Faculty of Medicine and Health Sciences, Macquarie University, Sydney, Australia. Electronic address: kathryn.busch@macquarievascular.com.au.
[Ti] Título:Detection of Anomalous Cervical Internal Carotid Artery Branches by Colour Duplex Ultrasound.
[So] Source:Eur J Vasc Endovasc Surg;53(6):776-782, 2017 Jun.
[Is] ISSN:1532-2165
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: Conventional anatomical descriptions of the cervical internal carotid artery (ICA) report that no branches arise from this segment. However reports of ICA branches exist. The study aim was to determine the prevalence of anomalous branches of the ICA using colour duplex ultrasound (CDU). METHODS: Four hundred consecutive patients (800 carotid vessels) referred to a tertiary hospital vascular laboratory for investigation of carotid disease were included. A clear differentiation of a low resistive ICA and a high resistive external carotid artery (ECA) waveform was required. CDU was performed on a Philips IU22 ultrasound system with a 9-3 MHz linear array transducer employing a standard carotid imaging protocol. The origin of each ICA branch was identified using B-mode and CDU. Anatomical orientation of ICA branches and distance from bifurcation was recorded and spectral CDU analysis was performed. RESULTS: Twenty ICA branches, tracking cephaladly, were detected in 16 patients (4%), or 2.5% (20/800) vessels. The median age was 73 years; 50% were female and 50% male. All patients demonstrated a single branch [unilateral anomaly (n = 12 patients), bilateral anomaly (n = 4) patients]. Eighty-five percent arose from the posterior wall of the ICA. The median distance from ICA bifurcation was 4 mm (range 0-18 mm). ICA branch diameters ranged from 1.2 mm to 2.4 mm (median 1.4 mm, mean 1.6 mm). Eighty percent of ICA branches had less than 50% stenosis. Spectral analysis revealed high resistive waveforms in all branches. CONCLUSIONS: Branches from the ICA exist and can be characterised with CDU. Prevalence in a tertiary hospital referral cohort is 4%. Given their potential clinical implications, standard carotid imaging protocols should consider routine assessment and reporting of ICA branches.
[Mh] Termos MeSH primário: Artéria Carótida Interna/diagnóstico por imagem
Ultrassonografia Doppler em Cores
Malformações Vasculares/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Artéria Carótida Interna/anormalidades
Feminino
Seres Humanos
Masculino
Meia-Idade
New South Wales/epidemiologia
Valor Preditivo dos Testes
Prevalência
Estudos Prospectivos
Centros de Atenção Terciária
Malformações Vasculares/epidemiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180116
[Lr] Data última revisão:
180116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170430
[St] Status:MEDLINE


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[PMID]:29233366
[Au] Autor:Kurihara N; Saito H; Usami S; Imai K; Konno H; Atari M; Fujishima S; Kamiya A; Ogawa JI; Minamiya Y
[Ad] Endereço:Department of Thoracic Surgery, Akita University Graduate School of Medicine, Akita, Japan. Electronic address: kurihara@gipc.akita-u.ac.jp.
[Ti] Título:Lung Cancer Surgery in Partial Anomalous Pulmonary Venous Connection Patients.
[So] Source:Ann Thorac Surg;105(1):e7-e9, 2018 Jan.
[Is] ISSN:1552-6259
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:We report pulmonary resections for lung cancers in 2 patients with partial anomalous pulmonary venous connection (PAPVC) identified preoperatively. In case 1, right upper lobectomy was performed as the definitive operation for both lung cancer and PAPVC in the same lobe. In case 2, because lung cancer and PAPVC existed in different lobes, cardiac catheterization was performed to evaluate the need for correction of the PAPVC. Then, left lower lobectomy was safely performed without correcting the PAPVC located in the left upper lobe. The treatment plan for patients with PAPVC who require pulmonary resection should be carefully considered.
[Mh] Termos MeSH primário: Neoplasias Pulmonares/cirurgia
Pneumonectomia
Veias Pulmonares/anormalidades
Malformações Vasculares/cirurgia
[Mh] Termos MeSH secundário: Idoso
Seres Humanos
Neoplasias Pulmonares/complicações
Neoplasias Pulmonares/diagnóstico por imagem
Masculino
Meia-Idade
Veias Pulmonares/diagnóstico por imagem
Malformações Vasculares/complicações
Malformações Vasculares/diagnóstico por imagem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171226
[Lr] Data última revisão:
171226
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171214
[St] Status:MEDLINE


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[PMID]:28727248
[Au] Autor:Adams DM; Brandão LR; Peterman CM; Gupta A; Patel M; Fishman S; Trenor CC
[Ad] Endereço:Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
[Ti] Título:Vascular anomaly cases for the pediatric hematologist oncologists-An interdisciplinary review.
[So] Source:Pediatr Blood Cancer;65(1), 2018 Jan.
[Is] ISSN:1545-5017
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Vascular anomalies (VAs) are classified as tumors or malformations depending on their clinical characteristics, pathological diagnosis, and genomic information. Diagnosis can be challenging because of the heterogeneity of clinical presentation; thus, the best diagnosis and care are provided by an interdisciplinary team of specialists. Over the past 10 years, an increasing number of pediatric hematologist/oncologists are caring for patients with VAs secondary to new medical therapy options and clinical trials. This paper focuses on complicated VA issues often seen by the pediatric hematologist/oncologist. The paper reviews clinical pearls on diagnosis, histology, radiology, and treatment options.
[Mh] Termos MeSH primário: Malformações Vasculares
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Ensaios Clínicos como Assunto
Feminino
Hematologia
Seres Humanos
Lactente
Masculino
Oncologia
Malformações Vasculares/diagnóstico
Malformações Vasculares/diagnóstico por imagem
Malformações Vasculares/patologia
Malformações Vasculares/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171201
[Lr] Data última revisão:
171201
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170721
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26716


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[PMID]:28963191
[Au] Autor:Cunha SI; Magnusson PU; Dejana E; Lampugnani MG
[Ad] Endereço:From the Department of Immunology, Genetics, and Pathology, Uppsala University, Sweden (S.I.C., P.U.M., E.D.); FIRC Institute of Molecular Oncology, Milan, Italy (E.D., M.G.L.); and Istituto di Ricerche Farmacologiche Mario Negri, Milan, Italy (M.G.L.).
[Ti] Título:Deregulated TGF-ß/BMP Signaling in Vascular Malformations.
[So] Source:Circ Res;121(8):981-999, 2017 Sep 29.
[Is] ISSN:1524-4571
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Correct organization of the vascular tree requires the balanced activities of several signaling pathways that regulate tubulogenesis and vascular branching, elongation, and pruning. When this balance is lost, the vessels can be malformed and fragile, and they can lose arteriovenous differentiation. In this review, we concentrate on the transforming growth factor (TGF)-ß/bone morphogenetic protein (BMP) pathway, which is one of the most important and complex signaling systems in vascular development. Inactivation of these pathways can lead to altered vascular organization in the embryo. In addition, many vascular malformations are related to deregulation of TGF-ß/BMP signaling. Here, we focus on two of the most studied vascular malformations that are induced by deregulation of TGF-ß/BMP signaling: hereditary hemorrhagic telangiectasia (HHT) and cerebral cavernous malformation (CCM). The first of these is related to loss-of-function mutation of the TGF-ß/BMP receptor complex and the second to increased signaling sensitivity to TGF-ß/BMP. In this review, we discuss the potential therapeutic targets against these vascular malformations identified so far, as well as their basis in general mechanisms of vascular development and stability.
[Mh] Termos MeSH primário: Vasos Sanguíneos/metabolismo
Proteínas Morfogenéticas Ósseas/metabolismo
Neovascularização Fisiológica
Transdução de Sinais
Fator de Crescimento Transformador beta/metabolismo
Malformações Vasculares/metabolismo
[Mh] Termos MeSH secundário: Animais
Vasos Sanguíneos/anormalidades
Vasos Sanguíneos/fisiopatologia
Proteínas Morfogenéticas Ósseas/genética
Modelos Animais de Doenças
Predisposição Genética para Doença
Hemangioma Cavernoso do Sistema Nervoso Central/genética
Hemangioma Cavernoso do Sistema Nervoso Central/metabolismo
Hemangioma Cavernoso do Sistema Nervoso Central/fisiopatologia
Seres Humanos
Camundongos Transgênicos
Mutação
Fenótipo
Fatores de Risco
Telangiectasia Hemorrágica Hereditária/genética
Telangiectasia Hemorrágica Hereditária/metabolismo
Telangiectasia Hemorrágica Hereditária/fisiopatologia
Fator de Crescimento Transformador beta/genética
Malformações Vasculares/genética
Malformações Vasculares/fisiopatologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Bone Morphogenetic Proteins); 0 (Transforming Growth Factor beta)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171010
[Lr] Data última revisão:
171010
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171001
[St] Status:MEDLINE
[do] DOI:10.1161/CIRCRESAHA.117.309930



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