Neurofibromatose Online (Neurofibromatosis)

– Neurofibromatoses (NF) constituem um grupo de doenças genéticas com predisposição ao crescimento de múltiplos tumores: tipo 1 (NF1), tipo 2 (NF2) e schwannomatose (SCH). Estas doenças têm em comum a origem neural dos tumores e os sinais cutâneos. Devido à sua variabilidade fenotípica, curso progressivo, multiplicidade de órgãos acometidos e evolução imprevisível, as NF frequentemente necessitam de especialistas em NF para o acompanhamento.

– A neurofibromatose tipo I (doença de von Recklinghausen) é uma doença autossômica dominante cujo diagnóstico é feito com base em critérios clínicos. É um distúrbio relacionado a mutações no gene neurofibromina 1 (NF1), um gene supressor tumoral. As três principais manifestações: neurofibromas, manchas café com leite e nódulos de Lisch ocorrem em mais de 90% dos pacientes até a puberdade. Além dos neuromas acústicos unilaterais, a neurofibromatose 1, podem também apresentar, sardas axilares e inguinais, gliomas ópticos, hamartomas de íris e lesões ósseas displásicas. Atualmente, não existe cura para a NF1, porém existem medidas paliativas que melhoram as perspectivas de vida útil dos indivíduos afetados. São pacientes que, em geral, apresentam expectativa de vida normal, atividades acadêmicas e profissionais produtivas, além de vida afetiva regular. O aconselhamento genético é importante no intuito de orientar os pais de uma criança afetada, assim como esclarecê-los a respeito do risco de recorrência em outras gestações

– A neurofibromatose tipo II é um distúrbio autossômico dominante raro, causado por gene para neurofibromina 2 (NF2), um inibidor da proliferação celular contato-dependente. Suas principais características são os neuromas acústicos bilaterais, que podem ser acompanhados por outros tumores do sistema nervoso central ou periférico, incluindo neurofibromas, meningiomas, gliomas e schwannomas.

 

Increase breast cancer risk in women with neurofibromatosis type 1: a meta-analysis and systematic review of the literature

Lorena P. Suarez-Kelly, Lianbo Yu et al

Hered Cancer Clin Pract. 2019; 17: 12

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6434896/

 

Jejunal stromal tumor and neurofibromatosis.

Kayser, Daniela Fonseca Lisboa et al.

  1. Coloproctol. (Rio J.), Dec 2019, vol.39, no.4, p.385-388

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S2237-93632019000400385&lng=en&nrm=iso&tlng=en

 

Correction of severe dystrophic scoliosis in neurofibromatosis 1 with posterior vertebral column resection in multiple levels

Barros, Alderico Girão Campos de et al

Coluna/Columna, Sept 2019, vol.18, no.3, p.187-191

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1808-18512019000300187&lng=en&nrm=iso&tlng=en

 

Antiphospholipid syndrome and neurofibromatosis type I: a coincidence or new association?

Mbekeani, Joyce N. et al.

Arq. Bras. Oftalmol., Mar 2019, vol.82, no.2, p.155-157

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27492019000200155&lng=en&nrm=iso&tlng=en

 

Burden of adult neurofibromatosis 1: development and validation of a burden assessment tool

Marie-Laure Armand, Charles Taieb, Aline Bourgeois et al

the French national network on rare skin diseases (FIMARAD)

Orphanet J Rare Dis. 2019; 14: 94.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6500066/

 

Evaluation of QoL in neurofibromatosis patients: a systematic review and meta-analysis study

Akram Sanagoo, Leila Jouybari, Fatemeh Koohi, Fatemeh Sayehmiri

BMC Neurol. 2019; 19: 123.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6560778/

 

Impact of surgery on long-term results of hearing in neurofibromatosis type-2 associated vestibular schwannomas

Isabel Gugel, Florian Grimm, Marina Liebsch, et al

Cancers (Basel) 2019 Sep; 11(9): 1376

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6770212/

 

Robust surgical approach for cutaneous neurofibroma in neurofibromatosis type 1

Bahir H. Chamseddin, La’Nette Hernandez, Dezehree Solorzano, Juan Vega, Lu Q. Le

JCI Insight. 2019 Jun 6; 4(11): e128881

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6629109/

 

Cutaneous neurofibromas in the genomics era: current understanding and open questions

R J Allaway, S J C Gosline, S La Rosa et al

British Journal of Cancer 2018, vol 118: 1539-1548

https://www.nature.com/articles/s41416-018-0073-2

 

Hallazgos clínicos y epidemiológicos en la neurofibromatosis tipo 1 y el complejo esclerosis tuberosa en una serie de pacientes pediátricos.

Cammarata-Scalisi, Francisco et al.

 Bol. Med. Hosp. Infant. Mex., Oct 2018, vol.75, no.5, p.287-294

http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1665-11462018000500287&lng=es&nrm=iso

 

Early genetic diagnosis of neurofibromatosis type 2 from skin plaque pexiform schwannomas in childhood

Elisabeth Castellanos, Adrià Plana, Cristina Carrato et al

for CSUR Phakomatoses Multidisciplinary Clinics HUGTiP-ICO-IGTP

JAMA Dermatol. 2018 Mar; 154(3): 341–346

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5885846/

 

A recurrent laryngeal nerve malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 1.

Alves Júnior, Sérgio Ferreira et al.

Arq. Neuro-Psiquiatr., Sept 2018, vol.76, no.9, p.635-635

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2018000900635&lng=en&nrm=iso

 

Amusia and its electrophysiological correlates in neurofibromatosis type 1.

Cota, Bruno Cézar Lage et al.

Arq. Neuro-Psiquiatr., May 2018, vol.76, no.5, p.287-295

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2018000500287&lng=en&nrm=iso&tlng=en

 

Attention déficit hyperactivity disorderr in neurofibromatosis type 1: evaluation with a continuouus performance test

Rony Cohen, Ayelet Halevy, Sharon Aharon, Avinoam Shuper

J Clin Neurol. 2018 Apr; 14(2): 153–157

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5897196/

 

The characteristics of 76 atypical neurofibromas as precursors to neurofibromatosis 1 associated malignant peripheral nerve sheath tumors

Christine S Higham, Eva Dombi, Aljosja Rogiers, et al

Neuro Oncol. 2018 May; 20(6): 818–825

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5961015/

 

Surgical Treatment of a Massive Neurofibroma of the Head and Neck in a Patient with Neurofibromatosis Type 1: A Case Report

Qin, Haiyan et al.

Int. J. Morphol., Mar 2018, vol.36, no.1, p.194-20

https://scielo.conicyt.cl/scielo.php?script=sci_arttext&pid=S0717-95022018000100194&lng=es&nrm=iso

 

Medullary unidentified bright objects in neurofibromatosis typ 1: a case series

Alessandra D’Amico, Federica Mazio, Lorenzo Ugga et al

BMC Pediatr. 2018; 18: 91.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5832195/

 

Cutaneous neurofibromas in neurofibromatosis type I: a quantiative natural history study

Ashley Cannon, Mei-Jan Chen, Peng Li, et al

Orphanet J Rare Dis. 2018; 13: 31.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5803843/

 

Cutaneous neurofibromas in the genomics era: current understanding and open question

Robert J. Allaway, Sara J. C. Gosline, Salvatore La Rosa et al

Br J Cancer. 2018 Jun 12; 118(12): 1539–1548

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6008439/

 

Worries and needs of adults and parentes of adults with neurofibromatosis type 1

Andre B. Rietman, Hanneke van Helden, Pauline H. Both et a

Am J Med Genet A. 2018 May; 176(5): 1150–1160

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5947156/

 

Increased insulin sensitivity in individuals with neurofibromatosis type 1.

Martins, Aline Stangherlin et al.

Arch. Endocrinol. Metab., Feb 2018, vol.62, no.1, p.41-46

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S2359-39972018000100041&lng=en&nrm=iso

 

Estimulación eléctrica medular en el paciente con neurofibromatosis tipo 1: reporte de un caso

Rojas-Fernández, V. et al.

Rev. Soc. Esp. Dolor, Ago 2017, vol.24, no.4, p.211-214

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1134-80462017000400211&lng=es&nrm=iso

 

Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.Vasconcelos, Roberto Andr

Torres de et al.

Arq. Neuro-Psiquiatr., June 2017, vol.75, no.6, p.366-371

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2017000600366&lng=en&nrm=iso

 

Revising neurofibromatosis type 2 diagnostic criteria to exclude LZTR1-related schwannomatosis

Miriam J. Smith, Naomi L. Bowers, Michael Bulman, et al

Neurology. 2017 Jan 3; 88(1): 87–92

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5200853/

 

Computerized cognitive training for children with neurofibromatosis type 1: a pilot resting-state fMRI study

Yuliya N. Yoncheva, Kristina K. Hardy, Daniel J. Lurie,

Psychiatry Res. 2017 Aug 30; 266: 53–58

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5582983/

 

Clinical presentation and prognostic indicators in 100 adults and children with neurofibromatosis 1 associated non-optic pathway brain gliomas

Susan Byrne, Steve Connor, Karine Lascelles, et al

J Neurooncol. 2017; 133(3): 609–614

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5537330/

 

Receptor of ghrelin is expressed in cutaneous neurofibroma of individuals with neurofibromatosis 1

Rafaela E. Rozza-de-Menezes, Nicolle C. Gaglionone, Raquel M. Andrade-Losso et al

Orphanet J Rare Dis. 2017; 12: 186

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5738781/

 

Orbital/Peri-orbital plexiform neurofibromas in children with neurofibromatosis type 1: multi-disciplinary recommendations for care

Robert A. Avery, James A. Katowitz, Michael J. Fisher, et al

Ophthalmology. 2017 Jan; 124(1): 123–132

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5173440/

 

Evaluation of quality of life in adults with neurofibromatosis 1 (NF1) using the impacto f NF1 on Quality of Life (INF1-QOL) questionnaire

Rosalie E Ferner, Mary Thomas, Gemma Mercer,  et al

Health Qual Life Outcomes. 2017; 15: 34.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5307827/

 

Neurofibromatosis: an update of ophthalmic characteristics and applications of optical coherence tomography

Barmak Abdolrahimzadeh, Domenica Carmen Piraino, Giorgio Albanese et al

Clin Ophthalmol. 2016; 10: 851–860

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4874640/

 

Current status and recommendations for biomarkers and biobanking in neurofibromatosis

  1. Oliver Hanemann, Jaishri O. Blakeley, Fabio P. Nunes et al

For the REiNS International Collaboration

Neurology. 2016 Aug 16; 87(7 Suppl 1): S40–S48

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5578360/

 

Efficacy and biomarker study of bevacizumab for hearing loss resulting from neurofibromatosis type 2-associated vestibular schwannomas

Jaishri O. Blakeley, Xiaobu Ye, Dan G. Duda et al

J Clin Oncol. 2016 May 10; 34(14): 1669–1675.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4872317/

 

Characterization and utilization of an international neurofibromatosis web-based patient-entered registry: an observational study

Mindell Seidlin, Robert Holzman, Pamela Knight, et al

on behalf of the Children’s Tumor Foundation

PLoS One. 2017; 12(6): e0178639

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5482445/

 

Neurofibromatosis: a review of NF1, NF2, and schwannomatosis

Jesse Lee Kresak, Meggen Walsh

J Pediatr Genet. 2016 Jun; 5(2): 98–104

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4918700/

 

Childhood neurofibromatosis type 2 (NF2) and related disorders: from bench to bedside and biologically targeted therapies

  1. Ruggieri, A.D. Praticò, A. Serra, L. Maiolino et al

Acta Otorhinolaryngol Ital. 2016 Oct; 36(5): 345–367

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5225790/

 

Cochlear implant in a patient with neurofibromatosis type 2 undergoing radiotherapy.

Pimentel, Patrícia Santos et al.

Braz. j. otorhinolaryngol., Apr 2016, vol.82, no.2, p.242-243

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1808-86942016000200242&lng=en&nrm=iso

 

Body composition in adults with neurofibromatosis type 1.

Souza, Marcio et al

Rev. Assoc. Med. Bras., Dec 2016, vol.62, no.9, p.831-836

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0104-42302016000900831&lng=en&nrm=iso&tlng=en

 

Disease burden and symptom structure of autismo in neurofibromatosis type 1: a study of the International NF1 Consortium Team (INFACT)

Stephanie M. Morris, Maria T. Acosta, Shruti Garg et al

JAMA Psychiatry. 2016 Dec 1; 73(12): 1276–1284

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5298203/

 

Avaliação da motricidade orofacial em indivíduos com neurofibromatose tipo 1.

Silva, Carla Menezes da, Santos, Cristiane Aparecida dos and Rezende, Nilton Alves de

Rev. CEFAC, Fev 2015, vol.17, no.1, p.100-110

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-18462015000100100&lng=en&nrm=iso

 

Neurofibromatosis tipo 1 (NF1) y su diagnóstico molecular como estrategia del diagnóstico diferencial y a edades tempranas.

Gómez, Martha and Batista, Oriana

Rev. méd. Chile, Oct 2015, vol.143, no.10, p.1320-133

https://scielo.conicyt.cl/scielo.php?script=sci_arttext&pid=S0034-98872015001000011&lng=es&nrm=iso

 

Neurofibromatoses: part 1 – Diagnosis and differential diagnosis.

Rodrigues, Luiz Oswaldo Carneiro et al.

Arq. Neuro-Psiquiatr., Mar 2014, vol.72, no.3, p.241-250

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2014000300241&lng=en&nrm=iso

 

Neurofibromatosis: part 2 – clinical management

Batista, Pollyanna Barros et al.

Arq. Neuro-Psiquiatr., June 2015, vol.73, no.6, p.531-543

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2015000600531&lng=en&nrm=iso

 

Estimulación eléctrica medular en el paciente con neurofibromatosis tipo 1: reporte de un caso

Rojas-Fernández, V. et al.

Rev. Soc. Esp. Dolor, Ago 2017, vol.24, no.4, p.211-214

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1134-80462017000400211&lng=es&nrm=iso

 

Neurofibromatosis: type 1 and 2

S Borofsky and L M Levy

American Journal of Neuroradiology 2013, vol 34 (12)

http://www.ajnr.org/content/34/12/2250

 

Neurofibromatose tipo 1: relato de um caso clínico.

Marques, Ana Catarina and Dinis, Fátima

Rev Port Med Geral Fam, Set 2013, vol.29, no.5, p.322-326

http://www.scielo.mec.pt/scielo.php?script=sci_arttext&pid=S2182-51732013000500007&lng=pt&nrm=iso

 

■  Dr Paulo Fernando Leite

Cardiologia/Prevenção Cardiovascular

Estratificação de Risco Cardiovascular

Consultório: Rua Padre Rolim 815/sala 601

Tel: 33245518

CRMMG: 7026

Email: pfleite1873@gmail.com

Data: Dez 2019

Manuais & Livros Medicina Online

– Textos completos originais

– Acesso gratuito online

– Textos em português (portuguese)

 

Manual de Cuidados Intensivos em Hepatologia

Sociedade Brasileira de Hepatologia e Associação de Medicina Intensiva Brasileira

Editores: Paulo Lisboa Bittencourt, Cláudio Celestino Zollinger, Edmundo Pessoa de Almeida Lopes

Editora Manole, São Paulo, 2 Edição

http://sbhepatologia.org.br/wp-content/uploads/2017/10/Manual-de-Cuidados-Intensivos-em-Hepatologia-1.pdf

 

Medicina de Emergência – Revisão Rápida 2017

Editores: Herlon Saraiva Martins, Rômulo Augusto dos Santos, Rodrig

Antonio Brandão Neto, Frederico Arnaud

Editora Manole, São Paulo, 2 Edição

http://cursos.somiti.org.br/arquivos/upload_download/arquivo_upload_20170323025718.pdf

 

Manual do Gestor Hospitalar

Federação Brasileira de Hospitais – 2019

Organizadores: Andréa Prestes, José Antonio Ferreira Cirino, Rosana Oliveira e Viviã de Sousa

http://fbh.com.br/wp-content/uploads/2019/05/MANUAL-FBH.pdf

 

Aspectos da História da Oftalmologia e do Conselho Brasileiro de Oftalmologia/Política Nacional de Atenção à Oftalmologia

Conselho Brasileiro de Oftalmologia 2018

Newton Kara José, Fernando Cesar Abib/Milton Ruiz Alves

Cultura Médica, c2018, Rio de Janeiro

http://www.cbo.com.br/novo/publicacoes/livro_tema_oficial_2018.pdf

 

Telemedicina Morsch – materiais gratuitos

https://telemedicinamorsch.com.br/materiais-gratuitos

 

Manual de Ginecologia Endócrina

FEBRASGO 2015

https://www.febrasgo.org.br/images/arquivos/manuais/Manuais_Novos/Manual_Ginecologia_Endocrina.pdf

 

Manual de Orientações Básicas para Prescrição Médica

Célia Maria Dias Madruga, Eurípides Sebastião Mendonça de Souza

Segunda Edição Revista e Ampliada, Brasília 2011

Conselho Federal de Medicina e Conselho de Medicina do Estado da Paraíba

Segunda Edição Revista e Ampliada, Brasília 2011

https://portal.cfm.org.br/images/stories/biblioteca/cartilhaprescrimed2012.pdf

 

■  Dr Paulo Fernando Leite

Cardiologia/Prevenção Cardiovascular

Estratificação de Risco Cardiovascular

Consultório: Rua Padre Rolim 815/sala 601

Tel: 33245518

Belo Horizonte – MG/Brasil

CRMMG: 7026

Email: pfleite1873@gmail.com

Data: dez 2019