Search | SciELO

Need help?

Add field +

Search history

No documents were found for your search

Results: 9

au:KARAM, G.

Filters

Collection + Options

All
Brazil

8
Colombia

1

Journal + Options

All
Anais da Academia Brasileira de Ciências

1
Brazilian Journal of Psychiatry

1
Cerâmica

1
Genetics and Molecular Biology

1
International braz j urol

1
Revista Brasileira de Anestesiologia

1
Revista Brasileira de Ortopedia

1
Revista Colombiana de Reumatología

1
Revista Direito GV

1

Language

All
Portuguese

3

Publication Year + Options

All
2013

2
2018

2
2000

1
2009

1
2010

1
2016

1
2017

1

SciELO Thematic Areas + Options

All
Health Sciences

5
Applied Social Sciences

1
Biological Sciences

1
Engineering

1
Multidisciplinary

1

Show all...

WoS Subject Categories + Options

All
Anesthesiology

1
Biochemistry

1
Biology

1
Ceramics

1
Genetics

1
Heredity

1
Law

1
Materials

1
Molecular

1
Multidisciplinary

1
Nephrology

1
Orthopedics

1
Psychiatry

1
Rheumatology

1
Science

1

Show all...

WoS Citation Index + Options

All
Science Citation Index Expanded

5
Social Sciences Citation Index

1

Citables and non citables

All
Citable

9

Type of Literature + Options

All
Article

8
Review article

1

Order by

Page of 1 Next

Select this page

Print | Send to email | Export |

Facebook
Twitter
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

0 Selected itens

List all
Erase all

Métodos para la medición de la adherencia a medicamentos modificadores de la enfermedad orales en artritis reumatoide y factores asociados con baja adherencia farmacológica
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics
- Dimensions
- PlumX
- Altmetric

Rincón-Rincón, Jenny Rocío

; Jaimes-Fernández, Diego Alejandro

; García-Casallas, Julio César

; Beltrán, Adriana

; Téllez, Angélica

; Fernández-Ávila, Daniel G.

; Mora-Karam, Claudia

Revista Colombiana de Reumatología

Journal Metrics
- Sobre o periódico
- SciELO Analytics

dec 2018, Volume 25 N. 4 Pages 261 - 270

Abstract: Es En | Text: Es En | PDF: Es | PDF: En

RESUMEN Objetivos: Revisar definiciones, factores asociados a adherencia, métodos para medición y determinación de adherencia a medicamentos modificadores de la enfermedad orales en artritis reumatoide. Métodos: Se realizó una búsqueda de la literatura en las bases de datos de Pubmed hasta diciembre de 2017 mediante términos MeSH (((«Arthritis, Rheumatoid» [Mesh] AND «Medication Adherence» [Mesh]) OR «Patient Compliance» [Mesh]) AND «Antirheumatic Agents» [Mesh]) de artículos que estuvieran en idioma espafñol o inglés e incluyeran solo población adulta. Resultados: De un total de 387 artículos encontrados, 43 se incluyeron para la revisión general, con información sobre definiciones de adherencia, cumplimiento, concordancia y persistencia, componentes, clasificación y dimensiones, factores de riesgo relacionados con la no adherencia al tratamiento, descripción de los métodos de medición. Solo 9 artículos midieron adherencia e incluyeron información sobre factores relacionados con adherencia a medicamentos orales en artritis reumatoide. Conclusiones: La adherencia al tratamiento farmacológico en artritis reumatoide es subóptima y se relaciona con menor efectividad en el control de la actividad inflamatoria. Los principales factores relacionados con baja adherencia incluyen problemas de acceso y disponibilidad del medicamento, mayor actividad y duración de la enfermedad, polifarmacia, uso de medicamentos por periodos prolongados, bajo estrato socioeconómico, etnia, reacciones adversas por medicamentos, percepción de inefectividad de la medicación y enfermedades concomitantes. Es necesario incorporar de forma sistemática la medición de adherencia farmacológica dentro de la práctica clínica rutinaria y la identificación de los factores de riesgo más frecuentes asociados a una baja adherencia con el fin de diseñar estrategias encaminadas a mejorar la adherencia de los pacientes y lograr mejores desenlaces clínicos.

ABSTRACT Objectives: To review the definitions, associated factors, as well as the methods for the measurement and determination of adherence to oral disease modifying drugs in rheumatoid arthritis. Methods: A search of the literature was carried out in the PUBMED databases up to December 2017 using MeSH terms: ((("Arthritis, Rheumatoid" [Mesh] AND "Medication Adherence" [Mesh]) OR "Patient Compliance" [Mesh]) AND "Antirheumatic Agents" [Mesh]). Only articles that included an adult population and were in Spanish or English were reviewed. Results: From the 387 articles found, 43 were included for general review (definitions of adherence, compliance, concordance and persistence, components, classification and dimensions of adherence, risk factors related to non-adherence, description of direct and indirect methods for measuring adherence). Only 9 articles measured adherence and included information about risk factors related to non-adherence to oral treatment in rheumatoid arthritis. Conclusions: The adherence to pharmacological treatment in rheumatoid arthritis is sub-optimal and is associated with less effectiveness in the control of inflammatory activity. The main factors related to low adherence include problems of drug access and availability, increased activity and duration of the disease, polypharmacy, use of medications for prolonged periods, socioeconomic stratum, ethnicity, adverse drug reactions, perception of ineffectiveness of the medication, and concomitant diseases. It is necessary to incorporate the systematic measurement of pharmacological adherence within clinical practice. It is also important to identify the most frequent risk factors associated with low adherence, in order to design strategies aimed at improving patient adherence and achieve better clinical outcomes.

https://doi.org/10.1016/j.rcreu.2018.07.002 602 downloads

Immunophenotyping lymphocyte and acute phase proteins in canine X-linked muscular dystrophy
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics
- Dimensions
- PlumX
- Altmetric

ABREU, DILAYLA K. DE

; MONTEIRO, JANAINA M.

; SOUZA, CAROLINA C.

; KARAM, RAFAEL G.

; FERNANDES, RENATA A.

; LESSA, THAIS B.

; FAGLIARI, JOSÉ J.

; MIGLINO, MARIA ANGELICA

; AMBROSIO, CARLOS EDUARDO

Anais da Academia Brasileira de Ciências

Journal Metrics
- Sobre o periódico
- SciELO Analytics

sep 2018, Volume 90 N. 3 Pages 2977 - 2990

Abstract: En | Text: En | PDF: En

Abstract Duchenne Muscular Dystrophy (DMD) is the most common X-linked muscular disease affecting humans. The Golden Retriever Muscular Dystrophy model (GRMD) is considerthe most suitable for several studies. This assay aims to quantify lymphocyte subpopulations CD4, CD5, and CD8, and standardize, the serum electrophoretic profile, to understand their contribution to the pathologic process in normal Golden Retriever dogs (GR group) and dystrophic´s (GRMD group), through the umbilical cord blood, in dogs aged from 2 to 3 months (GR II and GRMD II), and in dogs over 1 year of age (GR III and GRMD III). No significant differences were observed between the CD8+ lymphocyte subpopulations of the groups studied. The CD4+ and CD5+ lymphocyte subpopulations were significantly higher in the GRMD III group compared to the GR III group. Twenty-two different proteins in the gel were identified. The serum concentrations of the proteins belonging to the GR I and GRMD I groups were significantly lower than those of the other groups. We show that expression of acute phase proteins are worst during the aging of the dogs. We hope to expand knowledge to better understand the GRMD model and the translational data.

https://doi.org/10.1590/0001-3765201820170838 1137 downloads

Nondepolarizing muscle relaxant improves direct laryngoscopy view with no effect on face mask ventilation
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics
- Dimensions
- PlumX
- Altmetric

Rizk, Marwan S.

; Zeineldine, Salah M.

; El-Khatib, Mohamad F.

; Yazbeck-Karam, Vanda G.

; Ayoub, Sophie D.

; Bou-Khalil, Pierre K.

; Abi-Nader, Elie

; Ghabach, Marc M.

; Ayoub, Chakib M.

Revista Brasileira de Anestesiologia

Journal Metrics
- Sobre o periódico
- SciELO Analytics

jul 2017, Volume 67 N. 4 Pages 383 - 387

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

Resumo Justificativa: A ventilação difícil ou impossível via máscara facial complicada pela intubação traqueal difícil durante a indução da anestesia ocorre em 0,4% dos casos de anestesia em adultos, possivelmente leva a complicações fatais. Devido a tais catástrofes, recomendou-se que a administração de relaxantes musculares seja feita após a confirmação de ventilação adequada via máscara facial, sem uma validação científica sólida dessa conduta. Métodos: Neste estudo observacional, a facilidade de ventilação e os escores de visibilidade em laringoscopia direta antes e após a administração de cisatracúrio foram avaliados em 90 adultos jovens e saudáveis, sem riscos anestésicos e sem intubação difícil prevista, agendados para cirurgias eletivas gerais. Resultados: Antes do relaxamento muscular, 43 pacientes (48%) eram Cormack Grau I, enquanto os 47 (52%) restantes eram ou Cormack Grau II (28, 31%) ou Cormack Grade III (19, 21%). Após o relaxamento muscular com cisatracúrio, o número de pacientes com Cormack Grau I aumentou significativamente de 43 (48%) para 65 (72%) (p = 0,0013). Apenas um paciente (5%) dos 19 melhorou sua classificação de Cormack do Grau III para o Grau I, enquanto 16 dos 19 (84%) melhoraram suas classificações de Cormack do Grau III para o grau II após o uso de cisatracúrio. A qualidade da ventilação via máscara facial não diferiu com ou sem relaxantes musculares em todos os pacientes. Conclusão: O uso de cisatracúrio em adultos jovens saudáveis submetidos a cirurgias eletivas gerais sem intubação traqueal difícil prevista não teve efeito sobre a qualidade da ventilação via máscara facial, mesmo resultando em melhora quantificável da visibilidade da laringe.

Abstract Background: Difficult or impossible face mask ventilation complicated with difficult tracheal intubation during anesthesia induction occurs in 0.4% of adult anesthesia cases, possibly leading to life-threatening complications. Because of such catastrophes, muscle relaxants have been recommended to be administered after confirming adequate face mask ventilation without a solid scientific validation of this principal. Methods: In this observational study, the ease of ventilation and the scores of direct laryngoscopy views before and after administration of cisatracurium were assessed in ninety young healthy adults, without anesthetic risks and without foreseen difficult intubation and who were scheduled for general elective surgeries. Results: Before muscle relaxation, 43 patients (48%) were Cormack Grade I, while the remaining 47 patients (52%) were either Cormack Grade II (28 patients, 31%) or Cormack Grade II (19 patients, 21%). Following muscle relaxation with cisatracurium, the number of patients with Cormack Grade I significantly increased from 43 patients (48%) to 65 patients (72%) (p = 0.0013). Only 1 patient out of 19 patients (5%) improved his Cormack grade from Grade III to Grade I while 16 out 19 patients (84%) improved their Cormack grade from Grade III to Grade II after the use of cisatracurium. The quality of face mask ventilation did not differ with and without muscle relaxants in all patients. Conclusion: The use of cisatracurium in healthy young adults undergoing general elective surgeries with no anticipated difficult endotracheal intubation had no effect on the quality of face mask ventilation despite resulting in a quantifiable improvement in the laryngeal view.

https://doi.org/10.1016/j.bjane.2016.07.005 2592 downloads

Correlation between anthropometric data and length and thickness of the tendons of the semitendinosus and gracilis muscles used for grafts in reconstruction of the anterior cruciate ligament
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics
- Dimensions
- PlumX
- Altmetric

Pereira, Rafael Noschang

; Karam, Francisco Consoli

; Schwanke, Roberto Luís

; Millman, Rubens

; Foletto, Zilmar Minetto

; Schwanke, Carla Helena Augustin

Revista Brasileira de Ortopedia

Journal Metrics
- Sobre o periódico
- SciELO Analytics

apr 2016, Volume 51 N. 2 Pages 175 - 180

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

OBJETIVO: A estimativa pré-operatória do comprimento e do diâmetro dos tendões semitendinoso (ST) e grácil (G) pode auxiliar e permitir que os cirurgiões tenham a oportunidade de escolher opções de enxerto. O objetivo deste estudo foi pesquisar se existe correlação entre as medidas antropométricas, como altura, peso, índice de massa corpórea (IMC), idade e sexo do paciente, com a espessura e o comprimento dos tendões ST e G. MÉTODOS: Entre junho de 2012 e agosto de 2013, foram coletados os dados de 64 pacientes que se submeteram ao procedimento cirúrgico de reconstrução do ligamento cruzado anterior em que se usaram como enxerto os tendões dos músculos ST e G. Foram analisadas variáveis como idade, sexo, peso, altura e índice de massa corporal (IMC), comprimento e diâmetro dos tendões dos músculos ST e G. RESULTADOS: Houve uma correlação positiva entre altura e diâmetro total do enxerto de quádruplo (r = 0,254 p = 0,043), comprimento total do tendão ST (r = 0,450, p < 0,01), diâmetro do ST duplo (r = 0,270 p = 0,031), ST triplo (r = 0,347 p = 0,005), comprimento do tendão G (r = 0,249 p = 0,047) e diâmetro do G duplo (r = 0,258 p = 0,039). No entanto, idade (r = -0,015 p = 0,908), peso (r = 0,165 p = 0,193) e índice de massa corporal (r = 0,012 p = 0,926) não apresentaram correlação. CONCLUSÃO: Nossos resultados mostram que idade, peso e IMC não se correlacionaram com o diâmetro e comprimento do enxerto, enquanto a altura tinha uma correlação positiva com o comprimento total dos tendões flexores e com o diâmetro do enxerto dos flexores (ST e G).

OBJECTIVE: Preoperative estimation of the length and diameter of the semitendinosus (ST) and gracilis (G) tendons can assist surgeons and allow them to have the opportunity to choose alternative grafts. The aim of this study was to investigate whether anthropometric measurements such as height, weight and body mass index (BMI) or the patient's age and sex have any correlation with the thickness and the length of ST and G tendons. METHODS: Data were gathered from 64 patients who underwent the surgical procedure of anterior cruciate ligament reconstruction using the tendons of the ST and G muscles as grafts, between June 2012 and August 2013. Variables such as age, sex, weight, height, body mass index (BMI) and length and diameter of the tendons of the ST and G muscles were analyzed. RESULTS: There was a positive correlation between the height and total diameter of the quadruple graft (r = 0.254; p = 0.043), total length of the ST tendon (r = 0.450; p < 0.01), diameter of the double ST (r = 0.270; p = 0.031), triple ST (r = 0.347; p = 0.005), length of G tendon (r = 0.249; p = 0.047) and diameter of the double-G (r = 0.258; p = 0.039). However, age (r = -0.015; p = 0.908), weight (r = 0.165; p = 0.193) and body mass index (r = 0.012; p = 0.926) showed no correlation. CONCLUSION: Our results show that age, weight and BMI did not correlate with the diameter and length of the graft, while the height had a positive correlation with the total length of the flexor tendons and the diameter of the graft from the flexors (ST and G).

https://doi.org/10.1016/j.rboe.2016.01.011 1906 downloads

Uroflowmetry in a Large Population of Brazilian Men Submitted to a health check up program and its correlation with ipss and prostate size
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics
- Dimensions
- PlumX
- Altmetric

Zambon, Joao Paulo

; Batezini, Nelson Sivonei da Silva

; Karam Junior, Amir Jamil

; Conceicao, Raquel Dilguerian Oliveira

; Carvalho, Jose Antonio Maluf de

; Almeida, Fernando G.

International braz j urol

Journal Metrics
- Sobre o periódico
- SciELO Analytics

dec 2013, Volume 39 N. 6 Pages 841 - 846

Abstract: En | Text: En | PDF: En

Purpose The aim of this study was to assess the uroflowmetry data in a large population of asymptomatic Brazilian men submitted to a health check up program and their correlation to IPSS and prostate size. Materials and Methods Asymptomatic men underwent a health check-up program between January and December 2012. The inclusion criteria were men between 40 and 70 years, IPSS ≤ 7, without bladder, prostate, urethral surgery, neurological diseases, urinary tract infection, PSA < 4.0 ng/dL and urinary volume higher than 150 mL. Urological assessment consisted of clinical history, IPSS, digital rectal examination (DRE), prostate specific antigen (PSA), urinalysis, ultrasonography and uroflowmetry. Results A total of 1041 asymptomatic men were included in this study. The average age was 49 years and average maximum flow rate was 17.4 mL/s. In spite of IPSS and prostate size increase with aging, they had a weak correlation with Qmax cutoffs (10 mL/s and 15 mL/s). A total of 85 men (8.3%) had more than 60 years, and even in this group, Qmax was higher than 15 mL/s. Out of 1041 men, 117 had IPSS less than 8 and Qmax less than 10 mL/s. Conclusions In asymptomatic men there is a weak correlation between IPSS, prostate size and uroflowmetric data. The establishment of different normal cutoffs seems to be complicated and uroflowmetry data should be interpreted with caution in order to avoid misdiagnosis.

https://doi.org/10.1590/S1677-5538.IBJU.2013.06.10 4030 downloads

Family burden related to mental and physical disorders in the world: results from the WHO World Mental Health (WMH) surveys
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics
- Dimensions
- PlumX
- Altmetric

Viana, Maria Carmen

; Gruber, Michael J.

; Shahly, Victoria

; Alhamzawi, Ali

; Alonso, Jordi

; Andrade, Laura H.

; Angermeyer, Matthias C.

; Benjet, Corina

; Bruffaerts, Ronny

; Caldas-de-Almeida, Jose Miguel

; Girolamo, Giovanni de

; Jonge, Peter de

; Ferry, Finola

; Florescu, Silvia

; Gureje, Oye

; Haro, Josep Maria

; Hinkov, Hristo

; Hu, Chiyi

; Karam, Elie G.

; Lépine, Jean-Pierre

; Levinson, Daphna

; Posada-Villa, Jose

; Sampson, Nancy A.

; Kessler, Ronald C.

Brazilian Journal of Psychiatry

Journal Metrics
- Sobre o periódico
- SciELO Analytics

jun 2013, Volume 35 N. 2 Pages 115 - 125

Abstract: En | Text: En | PDF: En

Objective: To assess prevalence and correlates of family caregiver burdens associated with mental and physical conditions worldwide. Methods: Cross-sectional community surveys asked 43,732 adults residing in 19 countries of the WHO World Mental Health (WMH) Surveys about chronic physical and mental health conditions of first-degree relatives and associated objective (time, financial) and subjective (distress, embarrassment) burdens. Magnitudes and associations of burden are examined by kinship status and family health problem; population-level estimates are provided. Results: Among the 18.9-40.3% of respondents in high, upper-middle, and low/lower-middle income countries with first-degree relatives having serious health problems, 39.0-39.6% reported burden. Among those, 22.9-31.1% devoted time, 10.6-18.8% had financial burden, 23.3-27.1% reported psychological distress, and 6.0-17.2% embarrassment. Mean caregiving hours/week was 12.9-16.5 (83.7-147.9 hours/week/100 people aged 18+). Mean financial burden was 15.1% of median family income in high, 32.2% in upper-middle, and 44.1% in low/lower-middle income countries. A higher burden was reported by women than men, and for care of parents, spouses, and children than siblings. Conclusions: The uncompensated labor of family caregivers is associated with substantial objective and subjective burden worldwide. Given the growing public health importance of the family caregiving system, it is vital to develop effective interventions that support family caregivers.

https://doi.org/10.1590/1516-4446-2012-0919 7614 downloads

Constitucionalismo e democracia: soberania e poder constituinte
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics

Chueiri, Vera Karam de

; Godoy, Miguel G.

Revista Direito GV

Journal Metrics
- Sobre o periódico
- SciELO Analytics

jun 2010, Volume 6 N. 1 Pages 159 - 174

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

Conciliar democracia e constitucionalismo é uma tarefa tão complexa quanto problemática. Eis o paradoxo: A democracia significa o povo decidindo as questões politicamente relevantes da sua comunidade, inclusive os conteúdos da constituição; e o constitucionalismo significa, por sua vez, limites à soberania popular. A constituição se autoimpõe como manifestação da soberania popular e do poder constituinte, vinculando ambos. Assim, a conjugação entre constitucionalismo e democracia remete a outra, que está na sua base, qual seja, entre soberania e poder constituinte. o artigo pretende explorar essas duas relações, a da soberania com o poder constituinte e a do constitucionalismo com a democracia, mostrando as tensões que lhe são constitutivas, sobretudo para afirmar uma concepção de democracia deliberativa defendida por carlos santiago nino e roberto gargarella.

To conciliate democracy and constitutionalism is a complex and problematic task. There is the paradox: Democracy means the people deciding relevant political issues of their community including the contents of their constitution. Constitutionalism, on its side, means to limit popular sovereignty. Constitution imposes itself as the manifestation of popular sovereignty and constituent power linking both. Thus, the conjugation of constitutionalism and democracy leads to another which is in its origin, i.e., sovereignty and constituent power. This article aims at discussing these two relations, i.e., constituent power and sovereignty and constitutionalism and democracy showing their constituting tensions in order to affirm nino's and gargarella's conception of deliberative democracy.

20027 downloads Cited 1 time in SciELO

Properties of pre-cast terrazzo tiles and recommended specifications
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics

Karam, G.

; Tabbara, M.

Cerâmica

Journal Metrics
- Sobre o periódico
- SciELO Analytics

mar 2009, Volume 55 N. 333 Pages 84 - 87

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

Foi feito um programa experimental direcionado para estabelecer propriedades físicas e mecânicas como referências básicas de telhas de terrazzo de peças de mármore pré-moldadas disponíveis comercialmente e para investigar os efeitos de vários parâmetros de processo de fabricação nessas propriedades. A resistência transversal, a densidade, a absorção de água e a resistência ao desgaste foram medidas em função da pressão de moldagem e do tempo de residência do molde de um projeto de mistura padrão. Um teste simples de índice de desgaste de superfície foi desenvolvido e aplicado na comparação de telhas de terrazzo pré-moldadas com pedras de telhas naturais de referência. As recomendações para a melhoria das especificações existentes e o desenvolvimento de medidas para controle de qualidade são apresentadas.

A targeted experimental program was carried out to establish basic reference physical and mechanical properties of commercially available pre-cast marble chip terrazzo tiles and to investigate the effects of varying manufacturing process parameters on those properties. The transverse strength, density, water absorption, and abrasion resistance were measured as a function of casting pressure and residence time in the casting mold for a standard mix design. A simple surface abrasion index test was developed and applied comparing pre-cast terrazzo tiles with reference natural tiling stones. Recommendations for improving existing specifications and developing quality control measures are presented.

40315 downloads

Clinical and biochemical findings in 7 patients with X-linked adrenoleukodystrophy treated with Lorenzo's Oil
Facebook Twitter

Other social networks
Google+
LinkedIn
Reddit
StambleUpon
CiteULike
Mendeley
Other networks

Metrics
- SciELO Analytics

Vargas, Carmen R.

; Barschak, Alethéa G.

; Coelho, Daniella M.

; Furlanetto, Vivian

; Souza, Carolina F.M. de

; Karam, Simone M.

; Jardim, Laura

; Wajner, Moacir

; Giugliani, Roberto

Genetics and Molecular Biology

Journal Metrics
- Sobre o periódico
- SciELO Analytics

dec 2000, Volume 23 N. 4 Pages 697 - 701

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

A adrenoleucodistrofia ligada ao X (X-ALD) é uma desordem hereditária do metabolismo peroxissomal, bioquimicamente caracterizada pelo acúmulo de ácidos graxos de cadeia muito longa (`very long chain fatty acids"- VLCFA) em diferentes tecidos e em fluidos biológicos, sendo os principais ácidos acumulados o hexacosanóico (C26:0) e o tetracosanóico (C24:0). O acúmulo destes ácidos graxos está associado com desmielinização cerebral e insuficiência adrenal. A incidência desta condição é estimada em 1 para 25.000 em homens. Pelo menos seis fenótipos podem ser distinguidos, sendo a adrenoleucodistrofia (ALD) cerebral infantil e a adrenomieloneuropatia (AMN) os mais comuns. O tratamento preconizado consiste na utilização da mistura gliceroltrioleato/gliceroltrierucato (GTO/GTE), conhecida como Óleo de Lorenzo, combinada com dieta pobre em VLCFA. Existem ainda, terapias alternativas como transplante de medula óssea e imunossupressão, além da utilização de lovastatina e fenilacetato de sódio. Neste trabalho fez-se uma avaliação do tratamento com Óleo de Lorenzo associado à dieta restrita em VLCFA de 7 pacientes homens com X-ALD analisando a evolução clínica e bioquímica. Os pacientes apresentaram uma redução média de 50% nos valores de C26:0 e de 42,8% na razão C26:0/C22:0 após o início do tratamento. A maioria dos pacientes permaneceu clinicamente bem e aproximadamente 30% dos pacientes apresentaram uma progressão rápida no curso clínico da doença. Parece não haver uma clara correlação bioquímico-clínica do tratamento. Os resultados nos mostram que novas terapias mais eficazes para X-ALD são necessárias para que se possa obter um melhor prognóstico da doença com progressão mais lenta dos sintomas apresentados ou mesmo reversão dos sintomas já presentes nos pacientes.

X-Linked adrenoleukodystrophy (X-ALD) is a hereditary disorder of the peroxisomal metabolism biochemically characterized by the accumulation of very long chain fatty acids (VLCFA) in tissues and biological fluids. The major accumulated acids are hexacosanoic acid (C26:0) and tetracosanoic acid (C24:0). The disorder is characterized clinically by central and peripheral demyelination and adrenal insufficiency closely related to the accumulation of fatty acids. The incidence of X-ALD is estimated to be 1:25,000 males. At least six phenotypes can be distinguished. The most common phenotypes are childhood cerebral ALD and adrenomyeloneuropathy (AMN). The recommended therapy consists of the use of the glyceroltrioleate/glyceroltrierucate (GTO/GTE) mixture, known as Lorenzo's Oil, combined with a VLCFA-poor diet. There are alternative treatments such as bone marrow transplantation and immunosuppression, as well as the use of lovastatin and sodium phenylacetate. In the present study we report the clinical and biochemical course of 7 male patients with X-ALD treated with Lorenzo's Oil and a VLCFA-restricted diet. Treatment produced 50% reduction in C26:0 and 42.8% reduction in the C26:0/C22:0 ratio. Most patients remained clinically well, although approximately 30% of them presented a rapid clinical deterioration. The results showed a poor biochemical-clinical correlation for treatment, indicating that new therapies for X-ALD are needed in order to obtain a better prognosis for patients.

6733 downloads

Showing

itens per page

Page of 1 Next

Select one or more results

All Alphabetic

Results

Comparative chart Download CSV

Statistics of

Send result

To*

Use ; (semicolon) to insert more emails.

Send to email

This page

Selected records

All references (max. 300)

Your email

Subject

Comments

Export format:

RIS (for EndNote, ProCite, Reference Manager, RefWorks, Citavi, etc.)

BibTeX

Citation

CSV (for Excel, etc)

Export:

This page

Selected itens

All references (max. 2000)

Sem resultados

No documents were found for your search

Glossary and search help

You can enrich your search in a very simple way. Use the search indexes combined with the connectors (AND or OR) and specify more your search.

For example, if you want to search for articles about
cases of dengue in Brasil in 2015, use:
ti:dengue and publication_year:2015 and aff_country:Brasil

See below the complete list of search indexes that can be used:

Index code	Element
ti	article title
au	author
kw	article keywords
subject	subject (title words, abstract and keywords)
ab	abstract
ta	journal short title (e.g. Cad. Saúde Pública)
journal_title	journal full title (e.g. Cadernos de Saúde Pública)
la	publication language code (e.g. pt - Portuguese, es - Spanish)
type	document type
pid	publication identifier
publication_year	publication year of publication
sponsor	sponsor
aff_country	country code of the author's affiliation
aff_institution	author affiliation institution
volume	article volume
issue	article issue
elocation	elocation
doi	DOI number
issn	journal ISSN
in	SciELO colection code (e.g. scl - Brasil, col - Colômbia)
use_license	article usage license code

SciELO - Scientific Electronic Library Online
Rua Dr. Diogo de Faria, 1087 – 9º andar – Vila Clementino 04037-003 São Paulo/SP - Brasil
Email: scielo@scielo.org

All the contents of this site www.scielo.br, except where otherwise noted, is licensed under a Creative Commons Attribution License.
more »