Síndromes autoinflamatórias hereditárias na faixa etária pediátrica

Síndromes autoinflamatórias hereditárias na faixa etária pediátrica

São Paulo, 31 de Janeiro de 2011 Objetivo: Descrever as principais síndromes autoinflamatórias hereditárias na faixa etária pediátrica. Fontes dos Dados: Foi realizada uma revisão da literatura nas bases de dados PubMed e SciELO, utilizando as palavras-chave "síndromes autoinflamatórias” e "criança”, e incluindo referências bibliográficas relevantes. Síntese dos Dados: As principais síndromes autoinflamatórias são causadas por defeitos monogênicos em proteínas da imunidade inata, sendo consideradas imunodeficiências primárias. Elas são caracterizadas clinicamente por sintomas inflamatórios sistêmicos recorrentes ou contínuos e devem ser diferenciadas das doenças infecciosas, autoimunes e outras imunodeficiências primárias. Nesta revisão, foram enfatizadas características epidemiológicas, manifestações clínicas, alterações laboratoriais, prognóstico e terapia das principais síndromes autoinflamatórias: febre familiar do Mediterrâneo; síndrome periódica associada ao receptor de fator de necrose tumoral; criopirinopatias; deficiência de mevalonato-quinase; artrite granulomatosa pediátrica; síndrome de pioderma gangrenoso, artrite piogênica e acne; síndrome de Majeed; e deficiência do antagonista do receptor de interleucina-1. As criopirinopatias discutidas foram: doença inflamatória multissistêmica de início neonatal ou síndrome neurológica, cutânea e articular crônica infantil, síndrome de Muckle-Wells e síndrome autoinflamatória familiar associada ao frio. Conclusões: É importante que o pediatra reconheça as síndromes autoinflamatórias hereditárias mais prevalentes, pois o encaminhamento ao reumatologista pediátrico pode permitir um diagnóstico precoce e uma instituição de tratamento adequado, possibilitando uma melhora da qualidade de vida dos pacientes.   Palavras-chave: Síndrome autoinflamatória; criança; febre familiar do Mediterrâneo; criopirinopatias; TRAPS; NOMID. Síndromes autoinflamatórias hereditárias na faixa etária pediátrica

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En este día....

ON THIS DAY

February 12

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On Feb. 12, 1973, the first release of American prisoners of war from the Vietnam conflict took place.
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On Feb. 12, 1809, Charles Darwin, who originated the theory of evolution by natural selection, was born. Following his death on April 19, 1882, his obituary appeared in The Times.

Go to obituary » | Other birthdays »

On This Date

1554	Lady Jane Grey, who had claimed the throne of England for nine days, was beheaded after being charged with treason.
1733	English colonists led by James Oglethorpe founded Savannah, Ga.
1809	Naturalist Charles Darwin was born in Shrewsbury, England.
1870	Women in the Utah Territory gained the right to vote.
1909	The National Association for the Advancement of Colored People was founded in New York.
1912	Pu Yi, the last emperor of China, abdicated, marking the end of the Qing Dynasty.
1915	The cornerstone for the Lincoln Memorial was laid in Washington, D.C.
1924	George Gershwin's "Rhapsody in Blue" premiered in New York City.
1999	The Senate voted to acquit President Bill Clinton on charges of perjury and obstruction of justice.
2000	Charles M. Schulz, creator of the "Peanuts" comic strip, died at age 77.
2000	Hall of Fame football coach Tom Landry died at age 75.
2001	The NEAR spacecraft touched down on Eros, completing the first landing on an asteroid.
2004	Defying a California law, San Francisco officials began performing weddings for same-sex couples.
2009	A commuter plane crashed into a suburban Buffalo, N.Y., home, killing all 49 aboard and a person in the house.

Current Birthdays

Former Sen. Arlen Specter, D-Pa., turns 81 years old today.

AP Photo/Gene J. Puskar

Actress Christina Ricci turns 31 years old today.

AP Photo/Dan Steinberg

1923	Franco Zeffirelli, Director, turns 88
1926	Joe Garagiola, Sportscaster, turns 85
1934	Bill Russell, Basketball Hall of Famer, turns 77
1936	Joe Don Baker, Actor, turns 75
1939	Ray Manzarek, Rock musician (The Doors), turns 72
1950	Steve Hackett, Rock musician (Genesis), turns 61
1952	Michael McDonald, Singer (The Doobie Brothers), turns 59
1953	Joanna Kerns, Actress ("Growing Pains"), turns 58
1955	Arsenio Hall, Actor, turns 56
1968	Josh Brolin, Actor ("W.," "No Country for Old Men"), turns 43
1968	Chynna Phillips, Singer (Wilson Philips), turns 43
1979	Jesse Spencer, Actor ("House"), turns 32

Historic Birthdays

Charles Darwin2/12/1809 - 4/19/1882English naturalist who documented evolution.Go to obituary »

53	Thomas Campion 2/12/1567 - 3/1/1620 English poet,composer and musical theorist
44	Caspar Bartholin 2/12/1585 - 7/13/1629 Danish physician/theologian
65	Cotton Mather 2/12/1663 - 2/13/1728 American Congregational minister/author
92	Peter Cooper 2/12/1791 - 4/4/1883 American inventor,manufacturer and philanthropist
56	Abraham Lincoln 2/12/1809 - 4/15/1865 16th President of the United States
40	John Graham Chambers 2/12/1843 - 3/4/1883 English sportsman and journalist
89	John L. Lewis 2/12/1880 - 6/11/1969 American labor leader and founder of the C.I.O.
96	Alice Roosevelt Longworth 2/12/1884 - 2/20/1980 American politically influential daughter of Theodore Roosevelt
66	Max Beckmann 2/12/1884 - 12/27/1950 German expressionist painter/printmaker
88	Omar Bradley 2/12/1893 - 4/8/1981 American general, 1st chairman of the Joint Chiefs of Staff
47	Louis Buchalter 2/12/1897 - 3/4/1944 American crime boss
81	Roy Harris 2/12/1898 - 10/1/1979 American composer/teacher
81	Joseph Alioto 2/12/1916 - 1/29/1998 Mayor of San Francisco

CDC updates meningococcal vaccine guidelines

Vaccine-Preventable Diseases

Posted February 1, 2011

CDC updates meningococcal vaccine guidelines

CDC. MMWR. 2011;60:72-75.

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When the CDC releases its 2011 Child and Adolescent Immunization schedule on Feb. 11, it will include updated recommendations for the use of quadrivalent meningococcal conjugate vaccines in adolescents, according to today’s Morbidity and Mortality Weekly Report.

Based on a review of data that included the vaccine-effectiveness and cost-effectiveness of different vaccination strategies for adolescents, the CDC’s Advisory Committee on Immunization Practices now recommends routine vaccination of adolescents aged 11 or 12 years, with a booster dose at 16 years; and a two-dose primary series given 2 months apart for those aged 2 to 54 years with persistent complement component deficiency and functional or anatomic asplenia, and for adolescents with HIV.

Meningococcal conjugate vaccines were licensed in 2005. Since then, further data on bacterial antibody persistence, US trends in meningococcal disease epidemiology, and vaccine-effectiveness indicated that many vaccinated adolescents were only protected for 5 years. This meant a dose given at age 11 or 12 years may only provide immunity until age 16 years, when risk of the disease begins to peak.

Although the disease incidence rate has been on the decline since 2000, the peak in disease among 18-year-olds has persisted, even after routine vaccination. From 2000-2004 to 2005-2009, the number of serogroups C and Y cases, which represent most vaccine-preventable incidences of the disease, were down 74% in 11- to 14-year-olds; for 15- to 18-year-olds, the decrease was 27%. Cases of infected people who were vaccinated against the disease have also been reported.

A case-control study (case patients, n=108; controls, n=158) of a meningococcal conjugate vaccine (Menactra, Sanofi-Pasteur) showed the overall vaccine-effectiveness in those vaccinated 0 to 5 years before the study was 78% (95% CI, 29-93). The vaccine-effectiveness for those vaccinated less than 1 year earlier was 95% (95% CI, 10-100); vaccine-effectiveness for those vaccinated 1 year earlier was 91% (95% CI, 10-101); vaccine-effectiveness for those vaccinated 2 to 5 years earlier was 58% (95% CI, –72 to 89).

Nueva Guía de Infecciones de Transmisión Sexual (ITS)

Nueva Guía de Infecciones de Transmisión Sexual (ITS)

Publicado el 17 diciembre, 2010 por Jesus Redondo

Se acaba de publicar lasnueva guía sobre infecciones de transmisión sexual por parte de los CDC. Esta nueva guía supone una actualización de las publicadas en 2006.  Recogen algunos de los más candentes problemas relacionados con esta patología, como es el fenomeno creciente de la resistencia de gonococo y Treponema Pallidum, además de actualizaciones del proceso diagnóstico de algunos de los procesos más frecuentes.

AHRQ: Methodological Evaluation of Observational Research

AHRQ: Methodological Evaluation of Observational Research

7022011

Development of Quality Criteria To Evaluate Nontherapeutic  Studies of Incidence, Prevalence, or Risk Factors of Chronic Diseases: Pilot Study of New Checklists:

• Methodological Evaluation of Observational REsearch (MORE)—Observational Studies of Incidence or Prevalence of Chronic Diseases
• Methodological Evaluation of Observational Research (MEVORECH)—Observational Studies of Risk Factors of Chronic Diseases

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Abstract

Objective. To develop two checklists for the quality of observational studies of incidence or risk factors of diseases.

Study design and setting. Initial development of the checklists was based on a systematic literature review. The checklists were refined after pilot trials of validity and reliability were conducted by seven experts, who tested the checklists on 10 articles.

Results. The checklist for studies of incidence or prevalence of chronic disease had six criteria for external validity and five for internal validity. The checklist for risk factor studies had 6 criteria for external validity, 13 criteria for internal validity, and 2 aspects of causality. A Microsoft Access database produced automated standardized reports about external and internal validities. Pilot testing demonstrated face and content validities and discrimination of reporting vs. methodological qualities. Interrater agreement was poor. The experts suggested future reliability testing of the checklists in systematic reviews with preplanned protocols, a priori consensus about research-specific quality criteria, and training of the reviewers.

Conclusions. We propose transparent and standardized quality assessment criteria of observational studies using the developed checklists. Future testing of the checklists in systematic reviews is necessary to develop reliable tools that can be used with confidence