Familial Mediterranean Fever in Children Presenting with Attacks of Fever Alone

Padeh, Shai; Livneh, Avi; Pras, Elon; Shinar, Yael; Lidar, Merav; Feld, Olga; Berkun, Yackov

doi:10.3899/jrheum.090687

Cited by 45 publications

(45 citation statements)

References 21 publications

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“…Familial Mediterranean fever (FMF) is a recessively inherited disease associated with a mutated anti-inflammatory protein, known as pyrin/marenostrin [1]. Non-Ashkenazi Jews, Turks, Arabs, and Armenians are more commonly affected.…”

Section: Introductionmentioning

confidence: 99%

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Normal QT dispersion in colchicine-resistant familial Mediterranean fever (FMF)

et al. 2012

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The association between familial Mediterranean fever (FMF) and subclinical cardiac disease remains controversial. The aim of the current study was to evaluate whether FMF patients, who do not respond to colchicine treatment, and thereby endure persistent inflammation, have increased QT dispersion (QTd) values. Twenty-two FMF patients and 22 age- and sex-matched control subjects were included in the study. Repolarization and QT dispersion parameters were computed from 12-lead ECG recording using designated computer software, and results of five beats were subsequently averaged. Both FMF patients and controls had similar comorbidities, similar values of average QT, average corrected QT interval length, average QTd interval, average QT corrected dispersion, QT dispersion ratio, JT dispersion (JTd), and JT corrected dispersion. In conclusion, FMF patients who were unresponsive to colchicine treatment and did not develop amyloidosis had normal QTd and JTd parameters, indicating a non-increased risk for repolarization-associated ventricular arrhythmias.

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Section: Introductionmentioning

confidence: 99%

“…In some cases, Erysipelas-like erythema may also appear. Although today, the molecular basis of the disease is better understood, and genetic testing is available, the diagnosis remained clinical [1,2]. Persistent inflammation in about 30 % of FMF patients may progress to amyloidosis, in some.…”

Section: Introductionmentioning

confidence: 99%

Normal QT dispersion in colchicine-resistant familial Mediterranean fever (FMF)

et al. 2012

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“…86,136,145 The attacks occur suddenly, with high-grade fever (38.5À40 C) and severe malaise. 151 Moderate to severe abdominal pain occurs in 95% of patients, and is secondary to an acute generalized peritonitis. 152 On physical examination patients often have abdominal distension and intense rebound pain, mimicking an acute abdomen, which leads to abdominal surgery (appendectomy or cholecystectomy) in 30À40% of FMF patients.…”

Section: Geneticsmentioning

confidence: 99%

Classic Autoinflammatory Diseases

Jesús

Ferguson

Goldbach‐Mansky

2014

Stiehm's Immune Deficiencies

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“…Candi date criteria sug gested by some as mar kers of a severe disease-including early age of dis ease onset, high colchicine dose needed to prevent attacks, certain features of the attack (such as frequency), carriage of two (versus one only) MEFV mutations, homo zygosity for the Met694Val variant and experiencing typical arthritis episodes-could be tested in this setting. 7,8 Finally, any such consortium might encoun ter two expected difficulties in deter mining new severity scoring systems for paediatric FMF. First, Padeh et al 8,9 have shown that during the first 2 years of life (that is, very early age of onset) the dis ease pheno type is very mild and manifests with attacks of fever alone.…”

Section: News and Viewsmentioning

confidence: 99%