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benign paroxysmal peritonitis [keywords]
- Related factors should be considered in evaluation of mean platelet volume in patients with familial Mediterranean fever. [LETTER]
- Anadolu Kardiyol Derg 2014 Aug 22.
- Familial Mediterranean fever in Syrian children: phenotype-genotype correlation. [JOURNAL ARTICLE]
- Rheumatol Int 2014 Aug 24.
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of serosal membranes. In this study, 103 unrelated Syrian children were included. Mutation screening of the MEditerranean FeVer gene was performed for 12 mutations. Abdominal pain was observed in 91 (88.3 %) of the patients, fever in 82 (79.6 %), arthritis in 27 (26.2 %), pleuritis in 7 (6.7.5 %), rash and erysipelas-like erythema in 5 (4.8 %), myalgia in 5 (4.8 %), headache in 5 (4.8 %) and Henoch-Schonlein purpura in 1 (0.97 %). The most frequent mutation was M694V. In order to determine the association between M694V and clinical features of FMF, we compared the disease features between patients with and without this mutation. The presence of M694V was found to be associated with more severe course of FMF, earlier age of onset and more frequent arthritis in the Syrian children with FMF compared to other FMF patients who do not have this mutation.
- The hereditary autoinflammatory disorders uncovered. [REVIEW]
- Autoimmun Rev 2014 Aug 20.
There is a thriving interest in the field of hereditary autoinflammatory disorders (HAID), a gamut of heterogeneous conditions deriving from an aberrant orchestration of innate immunity, unified by the common feature of seemingly unprovoked inflammation, which might be systemic or occur in localized niches of the organism. Recurrent fever and episodic inflammation in the joints, serosal membranes, skin, gut, and other organs are the common denominator of HAID. Mutations in the inflammasome-related genes have been associated with different HAID, showing the intimate link existing between interleukin-1 (IL-1)-structured inflammasome and their pathogenesis. Differential diagnosis of HAID can be challenging, as there are no universally accepted diagnostic protocols, and near half of patients may remain without any genetic abnormality identified. The use of IL-1-antagonists has been associated with beneficial effects in a large number of HAID associated with excessive IL-1 signalling, such as cryopyrin-associated periodic syndromes, familial Mediterranean fever, and deficiency of IL-1 receptor antagonist. This review will discuss about the key-clues of HAID which might guide for an early recognition and drive decisions for treatment.
- The Effect of Rilonacept versus Placebo on Health-Related Quality of Life in Patients with Poorly Controlled Familial Mediterranean Fever. [Journal Article]
- Biomed Res Int 2014.:854842.
Objective.To examine the effect of rilonacept on the health-related quality of life (HRQoL) in patients with poorly controlled familial Mediterranean fever (FMF). Methods. As part of a randomized, double-blinded trial comparing rilonacept and placebo for the treatment of FMF, patients/parents completed the modified Child Health Questionnaire (CHQ) at baseline, and at the start and end of each of 4 treatment courses, 2 each with rilonacept and placebo.
Results.Fourteen subjects were randomized; mean age was 24.4 ± 11.8 years. At baseline the physical HRQoL score was significantly less (24.2 ± 49.5) but the psychosocial score was similar to the population norm (49.5 ± 10.0). There were significant improvements in most HRQoL concepts after rilonacept but not placebo. Significant differences between rilonacept and placebo were found in the physical (33.7 ± 16.4 versus 23.7 ± 14.5, P = 0.021) but not psychosocial scores (51.4 ± 10.3 versus 49.8 ± 12.4, P = 0.42). The physical HRQoL was significantly impacted by the treatment effect and patient global assessment.
Conclusion.Treatment with rilonacept had a beneficial effect on the physical HRQoL in patients with poorly controlled FMF and was also significantly related to the patient global assessment. This trial is registered with ClinicalTrials.gov Identifier NCT00582907.
- Efficacy of Interleukin-1 Targeting Treatments in Patients with Familial Mediterranean Fever. [JOURNAL ARTICLE]
- Inflammation 2014 Aug 20.
Herein, we reported our experience in colchicine-resistant familial Mediterranean fever (FMF) patients who are treated with anti-interleukin-1 (IL-1) drugs. A retrospective review of medical records of anti-IL-1 recipients was performed. The main clinical characteristics of these patients and the evolution after anti-IL-1 were recorded. There were 20 patients (11 male [M] and 9 female [F]). Despite regular colchicine treatment, median number of attacks per month and per year was 1 (1-4) and 12 (4-50), respectively. Twelve patients were receiving anakinra, and eight patients were treated with canakinumab. The number of monthly and yearly attacks after IL-1 treatment was significantly decreased after the biologic agent (p < 0.05). One patient did not respond to the treatment, and one patient developed serious infection during anti-IL-1. We also observed a significant decrease in proteinuria in the amyloidosis complicated FMF patients. Anti-IL-1 targeting drugs seem safe and effective therapies in colchicine-resistant FMF.
- Prevalence of periodontal disease in patients with Familial Mediterranean Fever: A cohort study from central Turkey. [Journal Article]
- Quintessence Int 2014; 45(9):743-8.
The aim of this study was to compare the periodontal status in patients with Familial Mediterranean Fever (FMF) and in those without this disease.84 subjects clinically diagnosed with FMF and 75 systemically healthy controls, matched by age and gender, were recruited. All FMF patients were on a regular daily colchicine treatment and during attack-free periods. Gingival Index (GI), Plaque Index (PI), probing pocket depth (PD), and clinical attachment level (CAL) were measured in all subjects. To evaluate periodontal disease further, patients were stratifi ed into fi ve groups. Education information and smoking habits were recorded.The FMF patients and healthy controls were comparable for age, gender, and smoking status (P > .05). The FMF patients had signifi cantly higher PI and GI values and lower PD and CAL values than those of the control group (P < .05). However, there was no signifi cant diff erence among all groups in terms of periodontal disease severity (P > .05). In the FMF-severe periodontitis group, higher PI and GI values were seen (P < .05). However, there was no signifi cant diff erence between the FMF-severe periodontitis group and the controls with severe periodontitis regarding the PD and CAL values (P > .05).Patients with FMF using colchicine did not manifest higher attachment loss compared to age- and sex-matched systemically healthy controls.
- Prevalence of periodontal disease in patients with Familial Mediterranean Fever: A cohort study from central Turkey. [JOURNAL ARTICLE]
- Quintessence Int 2014 Jul 17.
Objective: The aim of this study was to compare the periodontal status in patients with Familial Mediterranean Fever (FMF) and in those without this disease. Method and Materials: 84 subjects clinically diagnosed with FMF and 75 systemically healthy controls, matched by age and gender, were recruited. All FMF patients were on a regular daily colchicine treatment and during attack-free periods. Gingival Index (GI), Plaque Index (PI), probing pocket depth (PD), and clinical attachment level (CAL) were measured in all subjects. To evaluate periodontal disease further, patients were stratifi ed into fi ve groups. Education information and smoking habits were recorded. Results: The FMF patients and healthy controls were comparable for age, gender, and smoking status (P > .05). The FMF patients had signifi cantly higher PI and GI values and lower PD and CAL values than those of the control group (P < .05). However, there was no signifi cant diff erence among all groups in terms of periodontal disease severity (P > .05). In the FMF-severe periodontitis group, higher PI and GI values were seen (P < .05). However, there was no signifi cant diff erence between the FMF-severe periodontitis group and the controls with severe periodontitis regarding the PD and CAL values (P > .05). Conclusion: Patients with FMF using colchicine did not manifest higher attachment loss compared to age- and sex-matched systemically healthy controls.
- Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever. [JOURNAL ARTICLE]
- J Dermatol 2014 Aug 11.
Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease, which is characterized by recurrent and paroxysmal fever, peritonitis, arthritis, myalgia, and skin rashes. Although various skin lesions such as "erysipelas-like erythema", urticaria, nonspecific purpura, and subcutaneous nodules have been described, cutaneous vasculitis is rare. We report a Japanese case of sporadic FMF accompanied by cutaneous arteritis at the time of febrile attacks of FMF. Gene analysis revealed M694I mutation in a single allele of the MEFV gene, and oral colchicine successfully controlled both periodic fever and subcutaneous nodules of arteritis. Cutaneous necrotizing vasculitis repeatedly emerging with febrile attacks should be included among the skin manifestations of FMF.
- Immigrant health, our health. [Journal Article]
- Eur J Public Health 2014 Aug.:92-5.
This final chapter reviews the main conclusions reached by the Special Issue articles in the areas of EUNAM (EU and North African Migrants: Health and Health Systems) activities, covering well-being, health status, disease panorama and use of health services of immigrants to the EU. The reviewed chapters show that immigrants are a vulnerable population experiencing, in some aspects, discrimination and hardship similar to the socially weakest national population groups. Immigration has changed the disease spectrum, particularly in infectious diseases and recessive conditions such as sickle cell disease and familial Mediterranean fever. Importantly, health questions of immigrants cannot be separated from those of any human health issues. An imminent new immigrant question for the EU will be the massive internal migration. Although the overall disease spectrum may not be vastly different between EU countries, the internal migrants will be exposed to lifestyle-dependent ill health and diseases probably in a similar way as did migrants from outside Europe. Migrant health research requires dedicated funding, which needs to come from central EU sources because multiple nationalities are involved. This funding should be able to project the course of health from the country of origin to the country of destination and back again, which was one of guidelines in the funding that initiated EUNAM.
- Consanguinity and genetic diseases in North Africa and immigrants to Europe. [Journal Article]
- Eur J Public Health 2014 Aug.:57-63.
Endemic diseases are caused by environmental and genetic factors. While in this special issue several chapters deal with environmental factors, including infections, the present focus is on genetic causes of disease clustering due to inbreeding and recessive disease mechanisms. Consanguinity is implying sharing of genetic heritage because of marriage between close relatives originating from a common ancestor. With limited natural selection, recessive genes may become more frequent in an inbred compared with an outbred population. Consanguinity is common in North Africa (NA), and the estimates range from 40 to 49% of all marriages in Tunisia and 29-33% in Morocco. As a consequence, recessive disorders are common in the NA region, and we give some examples. Thalassaemia and sickle cell disease/anaemia constitute the most common inherited recessive disorders globally and they are common in NA, but with immigration they have spread to Europe and to other parts of the world. Another example is familial Mediterranean fever, which is common in the Eastern Mediterranean area. With immigrantion from that area to Sweden, it has become the most common hereditary autoinflammatory disease in that country, and there is no evidence that any native Swede would have been diagnosed with this disease. The examples discussed in this chapter show that the historic movement of populations and current immigration are influencing the concept of 'endemic' disease.