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      Evaluación gastrointestinal en los pacientes con fiebre mediterránea familiar con dolor abdominal crónico y tratados con colchicina: serie de casos Translated title: Gastrointestinal evaluation in colchicine-treated familial Mediterranean fever patients with chronic abdominal pain: Cases series

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          Abstract

          El objetivo de este estudio fue evaluar a los pacientes con fiebre mediterránea familiar (familial Mediterranean fever, FMF) y dolor abdominal crónico resistentes al tratamiento con colchicina. Se incluyó a 48 pacientes diagnosticados en nuestro consultorio de reumatología pediátrica que tenían dolor abdominal a pesar del tratamiento con colchicina. A todos los pacientes se los derivó a un gastroenterólogo pediátrico. Se registraron las características del dolor, tales como aparición, duración y frecuencia; se planificó una endoscopía digestiva para obtener un diagnóstico diferencial. Se determinó la presencia de una mutación del gen MEFV en 46 pacientes. La mediana de la duración del tratamiento fue de 2,8 años. Aproximadamente el 60% de los pacientes tenían dolor abdominal todos los días o de dos a tres veces a la semana; en el 73% de los casos, duró menos de tres horas. A 41 pacientes se les realizó una endoscopía digestiva alta. La gastroduodenitis es un hallazgo frecuente en los pacientes con FMF y dolor abdominal persistente a pesar del tratamiento. Los pacientes con los puntajes más altos de severidad de la enfermedad tenían inflamación digestiva grave.

          Translated abstract

          The aim of the study to evaluate familial mediterranean fever (FMF) patients with chronic abdominal pain unresponsive to colchicine treatment. Forty-eight patients who diagnosed in our Pediatric Rheumatology clinics and suffering from abdominal pain despite colchicine treatment were include. All patients were referred to a pediatric gastroenterologist. The pain characteristics such as onset, duration and frequency were recorded; gastrointestinal (GI) endoscopy was planned for differential diagnosis. MEFV mutation was determined in 46 patients. The median duration of treatment was 2.8 years. Approximately 60% of the patients suffered from abdominal pain every day or 2-3 times a week, in 73% of the cases it lasted less than three hours. Forty-one patients underwent upper GI endoscopy. Gastroduodenitis is a common finding in persisting abdominal pain despite therapy of FMF patients. The patients with the highest disease severity scores had severe inflammation within the entire GI system.

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          Colchicine use in children and adolescents with familial Mediterranean fever: literature review and consensus statement.

          The daily application of colchicine is the standard therapy for prophylaxis of attacks and amyloid deposition in familial Mediterranean fever. However, because of many issues (eg, dosage, time of introduction, etc), no standardized treatment recommendations have been established. In this work we review the available literature on colchicine use with respect to its indication, efficacy, mode of application, and safety in children and adolescents with familial Mediterranean fever. On the basis of this analysis, a consensus statement on the application of colchicine in children and adolescents with familial Mediterranean fever was developed by caregivers from Germany, Austria, and Turkey.
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            Rome II versus Rome III classification of functional gastrointestinal disorders in pediatric chronic abdominal pain.

            The updated Rome III criteria for pediatric functional gastrointestinal disorders (FGIDs) include new FGID categories and changes to the Rome II criteria for various FGIDs. To our knowledge, the implications of these revisions for patient classification have not been identified. The purpose of this study was to compare classification results using Rome II versus Rome III criteria for FGIDs associated with chronic abdominal pain. Participants were 368 pediatric patients whose subspecialty evaluations for chronic abdominal pain yielded no evidence of organic disease. The children's gastrointestinal symptoms were assessed with the parent-report version of the Questionnaire on Pediatric Gastrointestinal Symptoms (QPGS). More patients met the criteria for a pediatric pain-related FGID according to the Rome III criteria (86.6%) than the Rome II criteria (68.0%). In comparison with the results from the Rome II criteria, the Rome III criteria classified a greater percentage of children as meeting criteria for Abdominal Migraine (23.1% vs 5.7%) and Functional Abdominal Pain (11.4% vs 2.7%). Irritable Bowel Syndrome was the most common diagnosis according to both Rome II (44.0%) and Rome III (45.1%). Changes to the Rome criteria make the Rome III criteria more inclusive, allowing classification of 86.6% of pediatric patients with medically unexplained chronic abdominal pain.
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              Abdominal and digestive system associations of familial Mediterranean fever.

              Familial Mediterranean fever (FMF) is a hereditary episodic febrile syndrome that is expressed by acute spells of fever, painful manifestations in the abdomen, chest and joints, and slow development of nephropathic amyloidosis. Despite the recent cloning of the FMF gene (MEFV) and the identification of about 40 disease-related mutations, the diagnosis is still clinically dependent, and the pathogenesis and most of the clinical heterogeneity remain to be explained. Because episodic abdominal pain affects 95% of FMF patients, most of them are seen by gastroenterologists and undergo complete or partial abdominal imaging before the diagnosis is made. Focusing on recent advances in FMF, this article reviews both common and infrequent manifestations that a gastroenterologist may encounter during workups of FMF patients. These include episodic abdominal pain, paralytic or mechanical ileus, constipation, diarrhea, ascites, malabsorption, bowel infarction, and bleeding, arising directly from FMF or secondary to FMF common associations such as amyloidosis, vasculitides, inflammatory bowel disease, irritable bowel syndrome, or colchicine side effects. This article will help the gastroenterologist to cope with most clinical situations related to the abdominal and alimentary tract in patients with FMF.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Journal
                aap
                Archivos argentinos de pediatría
                Arch. argent. pediatr.
                Sociedad Argentina de Pediatría (Buenos Aires, , Argentina )
                0325-0075
                1668-3501
                October 2018
                : 116
                : 5
                : e649-e654
                Affiliations
                [01] Balikesir orgnameBalikesir Üniversitesi orgdiv1Facultad de Medicina orgdiv2Departamento de Pediatría Turquía
                [02] Balikesir orgnameBalikesir Üniversitesi orgdiv1Facultad de Medicina orgdiv2Departamento de Pediatría Turquía
                [03] Esmirna orgnameMinisterio de Salud de Esmirna orgdiv1Hospital Universitario y de Investigación de Tepecik orgdiv2Unidad de Inmunología y Reumatología Pediátricas Turquía
                Article
                S0325-00752018000500014
                10.5546/aap.2018.e649
                df468d90-65e5-49f0-a6c8-43cce8fe078d

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 01 October 2017
                : 04 March 2018
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 15, Pages: 0
                Product

                SciELO Argentina

                Categories
                Presentación de casos clínicos (versión electrónica)

                Diagnostic-familial mediterranean fever,Dolor abdominal,Gastroenterología,Diagnóstico,Fiebre mediterránea familiar,Adominal pain,Gastroenterology

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