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  • 1
    ISSN: 1432-2323
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé La fièvre Méditerranéenne Familiale (FMF) est également connue sous le nom de Polysérite Héréditaire, une maladie présente dès la naissance chez les Arméniens, Turcs, les habitants des Balkans et les Juifs d'Afrique du Nord. Le diagnostic définitif de FMF est basé sur les données cliniques et l'histoire familiale puisqu'il n'y existe encore aucun test diagnostique spécifique. Un des principaux caractères cliniques de la maladie est la survenue de tableau de péritonite récidivante, qui ressemble, cliniquement et biologiquement, en tous points à une crise d'appendicite aiguë. Ceci explique pourquoi jusqu'à deux tiers des patients avec une FMF sont opérés en urgence. alors que leur appendice est le plus souvent normal. Comme l'appendicectomie laparoscopique a été démontrée comme une méthode súre, et pour éviter de faire un diagnostic erroné et éviter une chirurgie inutile, nous avons réalisé une appendicectomie laparoscopique chez 13 patients ayant une MFM dont les áges allaient de 8 à 32 ans. Tous les patients souffraient de leur MFM entre 1 et 12 ans (durée moyenne=3.8 ans) et présentaient une moyenne de 3.5 épisodes de tableaux de péritonites par an. Dans tous les cas, il a été possible de conclure l'intervention par laparoscopie sans avoir besoin de convertir en chirurgie ouverte. La durée moyenne de séjour à l'hôpital a eté de 3.07 jours. Il y a eu une seule complication: une infection superficielle de la plaie opératoire (7.6%) et la durée moyenne avant la reprise d'activité normale a été de 8.5 jours. Nous concluons que laparoscopie est utile et sûre chez le patient ayant une MFM. Elle permet d'exclure l'appendicite comme l'origine du tableau de péritonite chez ces patients, et aide ainsi à éviter une chirurgie inutile.
    Abstract: Resumen La Fiebre Mediterránea Familiar (FMF), también conocida como Poliserositis Hereditaria, es una enfermedad hereditaria que se encuentra comunmente en Armenios, Turcos, Arabes, Balcanos y Judíos originarios de las naciones del norte de Africa. El diagnóstico definitivo de la FMF se fundamenta en los hallazgos clínicos y en la historia familiar, puesto que aun no se ha hallado una prueba diagnóstica especifica. Entre sus principales características clínicas están los episodios agudos recurrentes de peritonitis. Durante cada episodio los hallazgos en el examen clínico y en las determinaciones de laboratorio pueden ser muy similares a los de la apendicitis aguda. Por consiguiente, hasta dos tercios de los pacientes con FMF son sometidos a apendicectomía de urgencia con el hallazgo de un apéndice normal en la mayoría de los casos. Puesto que la apendicectomía laparoscópica ha demostrado ser segura y ventajosa, y con miras a prevenir un error diagnóstico y una cirugía de emergencia innecesaria, hemos practicado la apendicectomia laparoscópica en 13 pacientes con FMF con edades entre 8 y 32 años. Estos pacientes sufrían la enfremedad entre 1 y 12 años (promedio 3.8), y presentaban un promedio de 3.5 episodios anuales de peritonitis por FMF. La totalidad de los procedimientos se completaron por laparoscopia y ninguno requirió conversión a cirugía abierta. El promedio de estancia postoperatoria fue de 3.07 días. La única complicación fue una infección superficial de herida en una paciente (7.6%) y el tiempo medio hasta la recuperación de actividad normal fue de 8.5 días. Nuestra conclusión es que la apendicectomía laparoscópica electiva en pacientes con FMF es un procedimiento seguro. Ayuda a excluir la apendicitis como causa de la peritonitis en los pacientes con FMF y puede prevenir una cirugía de emergencia innecesaria.
    Notes: Abstract Familial Mediterranean fever (FMF) also known as bereditary polyserositis, is an inherited disorder commonly found in Armenians, Turks, Arabs, Balkans, and Jews originating from North African countries. The diagnosis of FMF is based on clinical findings and family history, as no specific diagnostic test is yet available. One of its main clinical features is recurrent acute episodes of peritonitis. During such an episode, physical examination and laboratory findings may be similar to those for acute appendicitis. Therefore up to two-thirds of FMF patients undergo emergency appendectomy, with the appendix being normal in most cases. As laparoscopic appendectomy has proved to be safe and advantageous, and to prevent misdiagnosis and unnecessary emergency surgery, we performed elective laparoscopic appendectomy in 13 FMF patients ranging in age from 8 to 32 years. They had been suffering from the disease for 1 to 12 years (mean 3.8) and had had an average of 3.5 yearly episodes of FMF peritonitis. All procedures were concluded by laparoscopy without conversion to open surgery. The average postoperative hospital stay was 3.07 days. The only complication was superficial wound infection in one patient (7.6%), and the mean time to regain full normal activity was 8.5 days. We conclude that elective laparoscopic appendectomy in FMF patients is safe. It helps to exclude appendicitis as a cause for peritonitis in these patients and may prevent unnecessary emergency surgery.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    BJOG 108 (2001), S. 0 
    ISSN: 1471-0528
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Objective To study the prevalence, the nature and the genotype correlation of menstruation associated familial Mediterranean fever attacks.Methods One hundred and forty-one female patients with familial Mediterranean fever were studied. A questionnaire regarding the presence and nature of menstruation associated with familial Mediterranean fever was designed and filled in by the authors during the patients’ visits to the familial Mediterranean fever clinic. The patients who had a positive history for this manifestation were analysed for their familial Mediterranean fever mutations.Results Ten out of 141 familial Mediterranean fever female patients (7%) had menstruation-associated familial Mediterranean fever attacks. These patients varied in their disease age of onset and disease duration. Increase of colchicine dose, daily or during the perimenstrual period or oral contraceptives were beneficial in preventing these familial Mediterranean fever attacks. No correlation was found with specific mutations causing familial Mediterranean fever.Conclusions Menstruation-associated familial Mediterranean fever attacks are relatively uncommon. They are not related to the age of the women, the chronicity of their disease or to the mutations they bear. Various therapeutic approaches have to be tried in order to abolish these attacks. A decrease in oestrogen level during menstruation may have a role in this unique manifestation of familial Mediterranean fever.
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    ISSN: 1365-4632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A 45-year-old woman presented with a 2-week history of weakness with rapidly progressing abdominal distention. Two weeks previously she had an abdominal ultrasound that showed no evidence of ascites or malignant changes.The patient was known to suffer from Turner's syn-drome 45XO/46XX and Hashimoto's thyroiditis for which she was treated with L-thyroxine. She also had numerous nevi, some of which were biopsied recently and interpreted as having no malignant features.On admission, the patient complained of weakness and abdominal discomfort. She was of short stature and was pale. Her secondary sexual characteristics were fully devel-oped. Many pigmented nevi were scattered over her skin, without clinical signs of malignant transformation. Fundo-scopy showed no signs of malignancy in the choroid layer. The abdomen was tense and edematous. Breast examina-tion was normal. An ECG and chest x-rays were also within normal limits. Computerized tomography showed a large amount of fluid with small masses spreading in the peri-toneal cavity. Aspiration of the abdominal fluid revealed ma-lignant cells that stained positively with S-100, melanoma-specific antigens, and with Masson-Fontana (Fig. 1).
    Type of Medium: Electronic Resource
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