RESUMEN
OBJECTIVE: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations. RECOMMENDATIONS: 1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints. 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene. 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment. 4. The therapy of choice is colchicine; this drug has proven its effectiveness in preventing acute inflammatory episodes and progression toward amyloidosis in adults. 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine.
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Amiloidosis Familiar/prevención & control , Colchicina/uso terapéutico , Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/terapia , Guías de Práctica Clínica como Asunto , Pirina/genética , Amiloidosis Familiar/genética , Medicina Basada en la Evidencia , Fiebre Mediterránea Familiar/genética , Humanos , Fenotipo , SíndromeRESUMEN
OBJECTIVE: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. RECOMMENDATIONS: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1ß inhibitors prevents progression of bone lesions.
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Síndromes Periódicos Asociados a Criopirina/diagnóstico , Síndromes Periódicos Asociados a Criopirina/terapia , Proteína con Dominio Pirina 3 de la Familia NLR/genética , Guías de Práctica Clínica como Asunto , Edad de Inicio , Síndromes Periódicos Asociados a Criopirina/genética , Medicina Basada en la Evidencia , Fiebre , Humanos , Inflamación/genética , Inflamación/inmunología , Interleucina-1beta , Mutación , Pronóstico , Índice de Severidad de la Enfermedad , UrticariaRESUMEN
OBJECTIVE: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. RECOMMENDATIONS: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports.
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Fiebre/terapia , Linfadenitis/terapia , Faringitis/terapia , Guías de Práctica Clínica como Asunto , Estomatitis Aftosa/terapia , Adenoidectomía , Fiebre/diagnóstico , Fiebre/cirugía , Humanos , Linfadenitis/diagnóstico , Linfadenitis/cirugía , Faringitis/diagnóstico , Faringitis/cirugía , Estomatitis Aftosa/diagnóstico , Estomatitis Aftosa/cirugía , Síndrome , TonsilectomíaRESUMEN
OBJECTIVE To estimate the prevalence of sexual initiation and contraceptive use at the last sexual intercourse of Brazilian adolescents, according to sociodemographic features. METHODS The data were obtained from the Study of Cardiovascular Risks in Adolescents (ERICA), a national school-based cross-sectional study. We included 74,589 adolescents from 32 geographic strata (27 capitals and five sets of municipalities with more than 100,000 inhabitants of each of the five macro-regions of the Country). Information on sexual initiation and contraceptive use at the last sexual intercourse (male condom and oral contraceptive pill) has been used. We have estimated prevalence and confidence intervals (95%CI) considering sample weights according to sex, age, type of school, residence status, macro-region and capitals. RESULTS We observed that 28.1% (95%CI 27.0-29.2) of the adolescents had already initiated sexual life, with higher prevalence among those aged 17 years (56.4%, 95%CI 53.9-58.9), males (33.5%, 95%CI 31.8-35.2), studying at public schools (29.9%, 95%CI 28.5-31.4), and from the Northern region (33.9%, 95%CI 32.3-35.4), mainly from Macapa, Manaus, and Rio Branco. Among those who had started their sexual life, 82.3% (95%CI 81.1-83.4) reported the use of contraceptive methods at the last intercourse, and the prevalence of use was higher among adolescents aged 17 years (85.3%, 95%CI 82.7-87.6), females (85.2%, 95%CI 83.8-86.5) and those living in the Southern region (85.9%, 95%CI 82.9-88.5). Male condom was used by 68.8% (95%CI 66.9-70.7), with no difference by type of school or macro-regions; the contraceptive pill was used by 13.4% (CI95% 12.2-14.6), and more frequently used among women (24.7%, 95%CI 22.5-27,0) and 17-year-old adolescents (20.8%, 95%CI 18.2-23.6) from urban settings(13.7%, 95%CI 12.5-14.9) and from the Southern region (22.6%, 95%CI 19.0-26.8), and less often in the Northern region. CONCLUSIONS ERICA's data analysis on sexuality and contraception shows heterogeneities in the prevalence of sexual initiation and use of contraceptive methods among Brazilian adolescents, depending on their age, where they live, and the type of school they study at. Younger adolescents and those living in the Northern region seem to be more vulnerable to the consequences of unprotected sexual intercourses.
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Conducta Sexual/estadística & datos numéricos , Adolescente , Conducta del Adolescente/fisiología , Brasil , Condones/estadística & datos numéricos , Anticoncepción , Conducta Anticonceptiva , Estudios Transversales , Femenino , Humanos , Masculino , Características de la Residencia , Encuestas y CuestionariosRESUMEN
OBJECTIVE To determine the prevalence of metabolic syndrome and its components in Brazilian adolescents. METHODS We evaluated 37,504 adolescents who were participants in the Study of Cardiovascular Risks in Adolescents (ERICA), a cross-sectional, school-based, national study. The adolescents, aged from 12 to 17 years, lived in cities with populations greater than 100,000 inhabitants. The sample was stratified and clustered into schools and classes. The criteria set out by the International Diabetes Federation were used to define metabolic syndrome. Prevalences of metabolic syndrome were estimated according to sex, age group, school type and nutritional status. RESULTS Of the 37,504 adolescents who were evaluated: 50.2% were female; 54.3% were aged from 15 to 17 years, and 73.3% were from public schools. The prevalence of metabolic syndrome was 2.6% (95%CI 2.3-2.9), slightly higher in males and in those aged from 15 to 17 years in most macro-regions. The prevalence was the highest in residents from the South macro-region, in the younger female adolescents and in the older male adolescents. The prevalence was higher in public schools (2.8% [95%CI 2.4-3.2]), when compared with private schools (1.9% [95%CI 1.4-2.4]) and higher in obese adolescents when compared with nonobese ones. The most common combinations of components, referring to 3/4 of combinations, were: enlarged waist circumference (WC), low HDL-cholesterol (HDL-c) and high blood pressure; followed by enlarged WC, low HDL-c and high triglycerides; and enlarged WC, low HDL-c, high triglycerides and blood pressure. Low HDL was the second most frequent component, but the highest prevalence of metabolic syndrome (26.8%) was observed in the presence of high triglycerides. CONCLUSIONS ERICA is the first Brazilian nation-wide study to present the prevalence of metabolic syndrome and describe the role of its components. Despite the prevalence of Metabolic Syndrome being low, the high prevalences of some components and participation of others in the syndrome composition shows the importance of early diagnosis of this changes, even if not grouped within the metabolic syndrome.