Association of complementary and integrative therapy use and symptoms among Turkish patients with familial Mediterranean fever / 中西医结合学报

OBJECTIVE@#This study explored the correlations between the use of complementary and integrative therapies (CITs) and symptoms among Turkish patients with familial Mediterranean fever (FMF).@*METHODS@#This is a cross-sectional and descriptive study. The study was conducted with 1119 FMF patients who were registered to the social networking site for Behcet's and the FMF Patients Association (Befemder) in Turkey, between January 2018 and February 2019. Data were collected using an online survey, for which a three-part questionnaire was created using a Google form. Descriptive statistics, chi-square test and logistic regression analysis were used to analyze the data.@*RESULTS@#It was determined that 53.2% of the individuals who participated in the research used various forms of CITs and that 32.8% used vitamin and mineral supplements (calcium, iron, and vitamin B12, C and D), 25.0% used nutritional supplements (fish oil and honey), and 24.6% used oral herbs (ginger, turmeric, green tea and rosemary) and mind-body methods (relaxation, respiration exercise and meditation). It was determined that the percentage of participants that used CITs was higher among women (odds ratio [OR] = 1.825; 95% confidence interval [CI] 1.421-2.344), those with joint pain (OR = 1.385; 95% CI 1.047-1.832), those with difficulty breathing (OR = 1.323; 95% CI 1.031-1.697), those with gastrointestinal symptoms (OR = 1.405; 95% CI 1.089-1.814) and those who had a family member with FMF (OR = 1.437; 95% CI 1.115-1.851).@*CONCLUSION@#More than half of the individuals used at least one type of CIT for symptom control.

Peritonitis fúngica por Rhodotorula mucilaginosa en una paciente en diálisis peritoneal automatizada: revisión de la literatura / Fungal peritonitis due to Rhodotorula mucilaginosa in a patient with automated peritoneal dialysis: literature review

Fungal peritonitis is a major complication of peritoneal dialysis associated with high mortality. Most survivors have a high rate of abandonment of peritoneal dialysis. We report a case of fungal peritonitis due to an unusual agent. An 83 year-old woman, with a history of type 2 diabetes mellitus and multiple episodes of bacterial peritonitis associated to technical flaws in the implementation of automated peritoneal dialysis, was admitted due to abdominal pain and cloudy peritoneal fluid. Rhodotorula mucilaginosa was identified in the peritoneal fluid by MALDI-TOF. She was treated with catheter removal and oral posaconazole for 14 days showing clinical resolution and non-recurrence.

La peritonitis fúngica es una complicación mayor de la diálisis peritoneal, con una alta mortalidad asociada y la mayoría de los sobrevivientes presentan una alta tasa de abandono de diálisis peritoneal como terapia de reemplazo renal. Se presenta un caso de peritonitis fúngica por un agente infrecuente. Mujer de 83 años, diabética con múltiples episodios de peritonitis bacteriana asociada a fallas técnicas en la ejecución de diálisis peritoneal automatizada, ingresa por cuadro clínico de dolor abdominal y líquido peritoneal turbio. Se confirmó la presencia de Rhodotorula mucilaginosa en líquido peritoneal mediante MALDI-TOF. Fue tratada con retiro del catéter y posaconazol oral por 14 días, presentando una evolución favorable.

Diretrizes de conduta e tratamento de síndromes febris periódicas associadas a febre familiar do Mediterrâneo / Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da febre familiar do Mediterrâneo (FFM). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de 5 questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados, e avaliados pelo título e resumo, 10.341 trabalhos e selecionados 46 artigos para sustentar as recomendações. Recomendações: 1. O diagnóstico da FFM é baseado nas manifestações clínicas, caracterizadas por episódios febris recorrentes associados a dor abdominal, torácica ou artrite de grandes articulações; 2. A FFM é uma doença genética que apresenta traço autossômico recessivo ocasionada por mutação no gene MEFV; 3. Exames laboratoriais são inespecíficos e demonstram níveis séricos elevados de proteínas inflamatórias na fase aguda da doença, mas também, com frequência, níveis elevados mesmo entre os ataques. Níveis séricos de SAA podem ser especialmente úteis no monitoramento da eficácia do tratamento; 4. A colchicina é a terapia de escolha e demonstrou eficácia na prevenção dos episódios inflamatórios agudos e progressão para amiloidose em adultos; 5. Com base na informação disponível, o uso de medicamentos biológicos parece ser opção para pacientes com FFM que não respondem ou que são intolerantes à terapia com colchicina.

Abstract 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 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: 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 effectiveness in preventing acute inflammatory episodes and progression towards 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.

Síndromes autoinflamatorios / Autoinflammatory syndromes

Los síndromes autoinflamatorios (SAI) se caracterizan por periodos recurrentes de inflamación no mediada por anticuerpos ni linfocitos T y sin desencadenantes conocidos. Investigaciones acerca de alteraciones en la regulación del inflamasoma, la producción anómala de interleukina-1 beta (IL - 1B) y el rol del factor de necrosis tumoral alfa (TNF-a) que ocurre en algunas de estas enfermedades, han permitido ampliar el conocimiento sobre los mecanismos de activación de la inmunidad innata y el uso de terapias biológicas como alternativas de tratamiento.

Autoinflammatory syndromes (AIS) are characterized by recurrent periods of inflammation, not mediated by antibody and T lymphocytes and without triggers known. Investigations about alterations in regulation of inflammasome, abnormal production of interleukin-1 beta (IL - 1b) and the role of tumor necrosis factor alpha (TNF-a) that occurs in some of these diseases, has improved knowledge about the mechanisms of activation of innate immunity and the use of biological therapies such as treatment options.

Fiebre mediterránea familiar: una afección frecuentemente subdiagnosticada / Febre mediterrânea familiar: uma afecção frequentemente subdiagnosticada

La fiebre mediterránea familiar (FMF) es una enfermedad inflamatoria crónica, hereditaria, de herencia autosómica recesiva, causada por mutaciones en el gene denominado MEFV. Se caracteriza por episodios recurrentes de fiebre e inflamación multisistémica. Se trata de una enfermedad frecuente en descendientes de poblaciones mediterráneas, del norte de África, Israel, Turquía, Armenia y países árabes. Se presenta el caso de un paciente, descendiente de armenios, con semiología y evolución características. Se analiza el diagnóstico, pronóstico, tratamiento y el asesoramiento genético correspondiente.

Familial Mediterranean fever in children: a single centre experience in Jordan

Familial Mediterranean fever is quite prevalent among Arabs. We reviewed the files of 56 patients diagnosed with familial Mediterranean fever and followed up at King Hussein Medical Centre in Jordan over 4 years for their clinical profile, course, genotype, treatment and complications. There were 30 males and 26 females with a mean age at onset of 5.2 years. Abdominal pain [79%] was the commonest manifestation, followed by arthritis [13%] and chest pain [4%]. Family history was positive in 50% of patients. Regarding treatment, 97% of patients responded well to colchicine, and amyloidosis was not documented in any patients after 5 years follow- up. The commonest genotype was M694 [64%], followed by heterozygous M694V- V726A [23%] and E148Q [8%]

Fiebre mediterránea familiar: revisión de literatura / Familial mediterranean fever: review of the literature

La Fiebre mediterránea familiar corresponde a una enfermedad inflamatoria, de herencia autosómica recesiva, caracterizada por episodios febriles y serotisis, descrita mayoritariamente en grupos étnicos originarios de la costa mediterránea. La amiloidosis secundaria es la principal causa de mortalidad. El estudio de la mutación genética causante de esta enfermedad es útil para el diagnóstico, de no estar disponible, una prueba terapéutica con Colchicina es una herramienta valiosa. El uso de ésta droga es el tramiento de elección.

Familial Mediterranean fever, a study of 32 cases

From April 1983 to September 1990, 32 patients with familial mediterranean fever [FMF] were studied. FMF is characterized by short, self-limited, febrile episodes that occur with inflammation of serosal surfaces. Major symptoms include fever and abdominal pain, presenting as acute surgical abdomen. These attacks are associated with considerable morbidity and in some patients lead to unnecessary surgery. Diagnosis of this disease depends on the absence of any objective markers and the recognition of the symptoms in a susceptible individual. Duration of disease on admission was 1.5-19 years. Except in two pairs of siblings, no familial association was noted. This is the first review of the cases of FMF from Iran

Fiebre mediterranea familiar en la ciudad de México / Familial mediterranean fever in Mexico city

Con el fin de difundir la existencia de pacientes con fiebre mediterránea familiar en la ciudad de México, se informan 47 casos en 9 familias, cuyas manifestaciones principales fueron: dolor abdominal en 46, fiebre en 45, dolor pleural en 10 y dolor articular en 8. Todas estas manifestaciones se presentaron en forma recurrente. Ninguno de los pacientes hasta la fecha tiene amiloidosis