Blog - INTERNET MÉDICA

Síndrome de Mauriac Online (Mauriac Syndrome)

Por Paulo Leite 22/04/2019

¨A síndrome de Mauriac é uma forma rara de diabetes mellitus tipo 1 (DM1), caracterizada por hepatomegalia (glicogenose hepática), retardo puberal e estatural, diabetes de difícil controle, dislipidemia, elevação de transaminases e redução de IGF-1 (fator de crescimento insulina símile). É um estado raro, mais comum em crianças e adolescentes, com difícil controle glicêmico e maior tendência a complicações. O quadro clínico, em geral, cursa com déficit pôndero-estatural, obesidade, dor em flanco direito associada a náuseas e vômitos secundários à hepatomegalia (1,2). Ainda não se pode quantificar o grau de deficiência do controle glicêmico em crianças com diabetes tipo 1 que determina o aparecimento desse quadro. O tratamento da SM é o mesmo realizado para as formas tradicionais de DM1, porém deve haver um controle insulínico altamente rígido com monitorização constante dos padrões glicêmicos do indivíduo (23). O início do tratamento deve ser cauteloso, através do ajuste da dosagem de insulina necessária ao longo da terapêutica, principalmente quando já houver retinopatia associada.¨

■ Hepatomegalia and type 1 diabetes: a clinical case of Mauriac´s syndrome

Fortunato Lombardo, Stefano Passanisi, Albino Gasbarro et al

Ital J Pediatr. 2019; 45: 3

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322227/

■ Mauriac syndrome: a rare complication of type 1 diabetes mellitus

Pinto MJF, Melo N, Flores L, Cunha F.

Eur J Case Rep Intern Med. 2018 Dec 27;5(12):000969

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346977/

■ Old syndrome-new approach: Mauriac syndrome treated with continuous insulin delivery

Kocova M, Milenkova L.

SAGE Open Med Case Rep. 2018 Jul 4;6

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6041848/

■ Síndrome de Mauriac reporte de caso clínico

M P G Lizarazo

Rev Med 2017, vol 25 (2)

https://revistas.unimilitar.edu.co/index.php/rmed/article/view/3208

■ Glycogenic hepatopathy: a narrative review

Sherigar JM, Castro J, Yin YM, Guss D, Mohanty SR.

World J Hepatol. 2018 Feb 27;10(2):172-185

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5838438/

■ Síndrome de Mauriac Reporte de Caso Clínico

Gallardo Lizarazo, Maria del Pilar

Rev.fac.med, Dic 2017, vol.25, no.2, p.74-77

http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0121-52562017000200074&lng=en&nrm=iso

■ Presentación de un caso de síndrome de Mauriac

Y A Zapata, C A Q Cadavid, H F S Alzate et al

Revista Endocrino 2017

http://revistaendocrino.org/index.php/rcedm/article/view/38/68

■ A rare case of persistent lactic acidosis in the ICU: glycogenic hepatopathy and Mauriac syndrome

Deemer KS, Alvarez GF.

Case Rep Crit Care. 2016;2016:6072909

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5035668/

■ Glycogenic hepatopathy in young adults: a case series

Silva, Marco et al.

Rev. esp. enferm. dig., Oct 2016, vol.108, no.10, p.672-676

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082016001000017&lng=es&nrm=iso

■ Mauriac´s syndrome: an uncommon and old complication of type 1 diabetes mellitus

Vânia Balderrama Brondani, Adriana de Mattos Antonio, Mara Barbosa Gayoso et al

Diabetol Metab Syndr. 2015; 7(Suppl 1): A25

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4653404/

■ Glycogenic hepatopathy: a rare and reversible cause of elevated transaminases in diabetic patients. Case report

García-Suárez, Covadonga et al.

Rev. esp. enferm. dig., Feb 2015, vol.107, no.2, p.111-112

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082015000200008&lng=es&nrm=iso

■ Diagnosis of hepatic glycogenosis in poorly controlled type 1 diabetes mellitus

Stefania Giordano, Antonio Martocchia, Lavinia Toussan et al

World J Diabetes. 2014 Dec 15; 5(6): 882–88

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4265875/

■ Mauriac syndrome in a child with a positive antinuclear antibody screen

John F. Pohl

Gastroenterol Res Pract. 2009; 2009: 765318

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2787018/

■ Síndrome de Pierre Mauriac y diabetes mellitus tipo 1 descontrolada

D Espinoza-Peralta, F J Gutierrez-Llamas, I García-Juárez

Revista de Gastroenterologia de México 2014, vol 79: 202-203

http://www.revistagastroenterologiamexico.org/en-pierre-mauriac-syndrome-uncontrolled-type-articulo-S2255534X14000632

■ Síndrome de Mauriac: Uma Apresentação Rara de Uma Doença Mais Comum.

Constantino, Cláudia et al.

Nascer e Crescer, Dez 2010, vol.19, no.4, p.278-281

http://www.scielo.mec.pt/scielo.php?script=sci_arttext&pid=S0872-07542010000400008&lng=pt&nrm=iso

■ Transplante de pâncreas na síndrome de Mauriac: evolução clínica e laboratorial após um ano de acompanhamento.

Maia, Frederico F.R. and Araújo, Levimar R.

Arq Bras Endocrinol Metab, Jun 2005, vol.49, no.3, p.455-45

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302005000300019&lng=en&nrm=iso

■ Síndrome de Mauriac: forma rara do diabetes mellitus tipo 1.

Maia, Frederico F.R. and Araújo, Levimar R.

Arq Bras Endocrinol Metab, Jun 2002, vol.46, no.3, p.310-315

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302002000300016&lng=en&nrm=iso

■ Dr Paulo Fernando Leite

Cardiologia/Prevenção Cardiovascular

Estratificação de Risco Cardiovascular

Novo endereço:

Consultório: Rua Padre Rolim 815/sala 815

Tel: 33245518 (Consulta Particular/Unimed)

– Centro Médico Unimed BH/Contagem/Pedro I

Belo Horizonte/MG/Brasil

CRMMG: 7026

Email: pfleite1873@gmail.com

Implante de Valva Aórtica Transcateter Online (Transcatheter Aortic Valve Implantation)

Por Paulo Leite 22/04/2019

¨O implante de valva aórtica transcateter (TAVI, do inglês transcatheter aortic valve implantation) constitui tratamento alternativo para pacientes com estenose aórtica de alto risco cirúrgico ou inoperáveis. A indicação de implante por cateter de prótese aórtica (TAVI, do inglês transcatheter aortic valve implantation) já encontra-se estabelecida em diretrizes nacionais e internacionais para o tratamento das valvopatias. Recomenda-se que o TAVI seja o tratamento de escolha para pacientes portadores de estenose aórtica grave e considerados inoperáveis (classe I, nível de evidência B) e que deva ser considerado uma estratégia alternativa à cirurgia de troca valvular naqueles indivíduos com alto risco cirúrgico (classe IIa, nível de evidência B). Estimativas apontam que mais de 100 mil pacientes já tenham sido tratados em todo o mundo, e os resultados atuais em centros com vasta experiência demonstraram elevadas taxas de sucesso do procedimento (acima de 95%), com índices de mortalidade aos 30 dias < 5%. O TAVI deve ser desenvolvido dentro de um programa para o tratamento da estenose aórtica, e não encarado meramente como um procedimento.¨

■ Comparison of transcatheter aortic valve implantation with other approaches to treat aortic valve stenosis: a systematic review and meta-analysis

G Wagner, S Steiner, G Gartlehner et al

Systematic Reviews 2019, vol 8: 44

https://systematicreviewsjournal.biomedcentral.com/articles/10.1186/s13643-019-0954-3

■ Aortic stenosis and transcatheter aortic valve implantation

Kim C, Hong MK.

Korean Circ J. 2019 Apr;49(4):283-297.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428950/

■ Papel da Avaliação Aortográfica Quantitativa da Regurgitação Aórtica por Videodensitometria na Orientação do Implante da Valva Aórtica Transcateter

Miyazaki, Yosuke et al.

Arq. Bras. Cardiol., Aug 2018, vol.111, no.2, p.193-202

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2018001400193&lng=pt&nrm=iso&tlng=pt (portuguese)

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2018001400193&lng=pt&nrm=iso&tlng=en (english)

■ Analysis of electrocardiographic intervals before and after transcatheter aortic valve implantation predict the need for permanent pacing

Ball TN, Vasudevan A, Mi Ko J, Assar MD, McCullough PA, Stoler RC.

Proc (Bayl Univ Med Cent). 2018 Sep 11;31(4):407-413

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6413979/

■ Comprehensive geriatric assessment in patients undergoing transcatheter aortic valve implantation – results from the GGA-TAVI multicentre registry

Andrea Ungar, Giulio Mannarino, Nathalie van der Velde et al

BMC Cardiovasc Disord. 2018; 18: 1

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5755352/

■ Early versus newer generation devices for transcatheter aortic valve implantation in routine clinical practice: a propensity score matched analysis

Thomas Pilgrim, Joe K T Lee, Crochan J O’Sullivan et al

Open Heart. 2018; 5(1): e000695

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5786915/

■ Otimal antiplatelet strategy after transcatheter aortic valve implantation: a meta-analaysis

Yousif Ahmad, Ozan Demir, Christopher Rajkumar et al

Open Heart. 2018; 5(1): e000748.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5786926/

■ Impact of transcatheter aortic valve implantation on the risk of mortality in patients with severe aortic valve diseases: a health insurance-based analysis

Alexander Barth, Seyrani Yücel, Hüseyin Ince, Gabriele Doblhammer

Open Heart. 2018; 5(1): e000756

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5922570/

■ Outcome after transvascular transcatheter aortic valve implantation in 2016

L Gaede, J Blumenstein, C Liebetrau et al

European Heart Journal 2018, vol 39 (8): 667-675

https://academic.oup.com/eurheartj/article/39/8/667/4710062

■ Transcatheter aortic valve replacemnt: optimizing outcomes for healthy recovert

Goel, Kashish; Holmes, David R. Jr

Journal of Cardiopulmonary Rehabilitation and Prevention 2018, vol 38 (1): 1-7

https://journals.lww.com/jcrjournal/Fulltext/2018/01000/Transcatheter_Aortic_Valve_Replacement__OPTIMIZING.1.aspx

■ Cost effectiveness of transcatheter aortic implantation in patients arotic stenosis in Japan

S Kodera, A Kiyosue, J Ando, I Komuro

Journal of Cardiology 2018, vol 71 (3): p223-229

https://www.journal-of-cardiology.com/article/S0914-5087(17)30277-0/fulltext

■ Transcatheter aortic valve replacement: Suitable for all?

K Minakata

Journal of Cardiology 2018, vol 71 (3): p221-p222

https://www.journal-of-cardiology.com/article/S0914-5087(17)30311-8/fulltext

■ Outcomes in transcatheter aortic valve replacement for bicuspid versus tricuspid aortic valve stenosis

S Yoon, S Bleiziffer, O De Backer et al

Journal of the American College of Cardiology 2017, vol 69 (21): 2579-2589

http://www.onlinejacc.org/content/69/21/2579

■ Increased mortality from complications of pulmonary hypertension in patients indergoing transcatheter aortic valve replacement

Raj Parikh, Benson Varghese, Huma N. Khatoon et al

Pulm Circ. 2017 Jun; 7(2): 391–398

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5467925/

■ Retinal embolic events: frequency and impact following transcatheter aortic valve implatation (TAVI) for aortic stenosis

William J Fusi-Rubiano, Yit C Yang, Andrew F Smallwood et al

BMJ Open Ophthalmol. 2017; 1(1): e000033

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5721649/

■ SOURCE 3: 1-year outcomes post-transcatheter aortic valve implantation using the latest generation of the ballon-expandable transcatheter heart valve

O Wendler, G Schymik, H Treede et al

European Heart Journal 2017, vol 38 (36): 2117-2722

https://academic.oup.com/eurheartj/article/38/36/2717/3866739

■ Pre- and post-surgical evaluation of the inflammatory response in patients with aortic stenosis treated with different types of prosthesis

Maria Elena Soto, Jose Luis Salas, Jesus Vargas-Barron et al

BMC Cardiovasc Disord. 2017; 17: 100

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5391573/

■ Transfemoral transcatheter aortic valve implantation in patients with end-stage renal disease and kidney transplant recipients

F Al-Rashid, A Binholz, H A Hildebrandt et al

Scientific Reports 2017, vol 7 , article number: 14397

https://www.nature.com/articles/s41598-017-14486-7

■ Assessment of aortic valve disease – a clinician oriented review

A D Margulescu

World J Cardiol 2017, vol 9 (6): 481-495

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491466/

■ Conservative management of aortic root rupture complicated with cardiac tamponade following aortic valve implantation

Luca Vannini, Rut Andrea, Manel Sabaté

World J Cardiol. 2017 Apr 26; 9(4): 391–395

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411975/

■ Evolução e Prognóstico da Regurgitação Paravalvar Após Implante de Valva Aórtica

Meneguz-Moreno, Rafael Alexandre et al

Arq. Bras. Cardiol., Dec 2017, vol.109, no.6, p.590-598

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2017001500590&lng=pt&nrm=iso&tlng=pt (portuguese)

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2017001500590&lng=pt&nrm=iso&tlng=en (english)

■ Surgical or transcatheter aortic-valve replacement in intermediate-risk patients

Michael J. Reardon, Nicolas M. Van Mieghem, Jeffrey J. Popma et al

for the SURTAVI Investigators*

N Engl J Med 2017; 376:1321-1331

http://www.nejm.org/doi/full/10.1056/NEJMoa1700456

■ Reversibility of cardiac function predicts outcomes after transcatether aortic valve replacement in patients with severe aortic stenosi

K Sato, A Kumar, B M Jones et al

Journal of the American Heart Association 2017, vol 6: e005798

http://jaha.ahajournals.org/content/6/7/e005798

■ Transcatheter aortic valve implantation with or without percutanous coronary artery revascularization strategy: a systematic review and meta-analysis

R A Kotronias, C S Kwok, S George et al

Journal of the American Heart Association 2017, vol 6: e005960

http://jaha.ahajournals.org/content/6/6/e005960

■ Long-term outcomes of patients with mediastinal radiation – associated severe aortic stenosis and subsequente surgical aortic valve replacemnt: a matched cohort study

E Donnellan, A Masri, D R Johnston et al

Journal of the American Heart Association 2017, vol 6: e005396

http://jaha.ahajournals.org/content/6/5/e005396

■ Traditional cardiovascular risk factors and their relation to future surgery for valvular heart disease or ascending aortic disease: a case-referent study

J Ljungberg, B Johansson, K G Engström et al

Journal of the American Heart Association 2017, vol 6: e005133

http://jaha.ahajournals.org/content/6/5/e005133

■ Effect of body mass index on clinical outcome and all-cause mortality in patients undergoing transcatheter aortic valve implantation