- Cardiac repolarization abnormalities in children with familial Mediterranean fever
CONCLUSIONS: FMF Patients may have increased risk of arrhythmia and should be monitored on regular basis. Compliance to colchicine therapy and better disease control might play a role in decreasing this risk.
- COVID-19 infection among patients with autoinflammatory diseases: a study on 117 French patients compared with 1545 from the French RMD COVID-19 cohort: COVIMAI - the French cohort study of SARS-CoV-2 infection in patient with systemic autoinflammatory diseases
CONCLUSION: As identified in the whole French RMD COVID-19 cohort, patients with SAID on corticosteroids and with multiple comorbidities are prone to develop more severe COVID-19 forms.
- Cytotoxicity of polymers intended for the extrusion-based additive manufacturing of surgical guides
Extrusion-based printing enables simplified and economic manufacturing of surgical guides for oral implant placement. Therefore, the cytotoxicity of a biocopolyester (BE) and a polypropylene (PP), intended for the fused filament fabrication of surgical guides was evaluated. For comparison, a medically certified resin based on methacrylic esters (ME) was printed by stereolithography (n = 18 each group). Human gingival keratinocytes (HGK) were exposed to eluates of the tested materials and an...
- Clinical and Laboratory Parameters of Autoinflammatory Disorders in Single Tertiary Care Center
Autoinflammatory diseases (AIDs) are disorders with an inborn error of innate immunity, characterized by recurrent episodes of fever and inflammatory attacks. The spectrum of AIDs is expanding, but there are no standardized clinical criteria for the diagnosis of the patients. This study aims at establishing the first autoinflammatory registry of an Iranian population focusing on the clinical and laboratory features that may help clinicians for a better understanding and diagnosis of these...
- Familial mediterranean fever patients may have unmet needs for the treatments of exertional leg pain and enthesitis
CONCLUSION: FMF treatments had favourable effect on ELP and enthesitis in FMF patients. However, the response rates would be inadequate. Therefore, there would be unmet need for treatment of both conditions.
- Correlation genotype-phenotype: MEFV gene mutations and Moroccan patients with rheumatoid arthritis
CONCLUSION: the results of this study suggest that MEFV gene mutations appear to be an aggravating factor severity of RA and consequently, patients with RA might be screened for MEFV gene mutations in countries where FMF is frequent. We report also that our study is the first one in our country Morocco.
- A parallelized, perfused 3D triculture model of leukemia forin vitrodrug testing of chemotherapeutics
Leukemia patients undergo chemotherapy to combat the leukemic cells (LCs) in the bone marrow. During therapy not only the LCs, but also the blood-producing hematopoietic stem and progenitor cells (HSPCs) may be destroyed. Chemotherapeutics targeting only the LCs are urgently needed to overcome this problem and minimize life-threatening side-effects. Predictivein vitrodrug testing systems allowing simultaneous comparison of various experimental settings would enhance the efficiency of drug...
- Design of a Planner-Based Intervention to Facilitate Diet Behaviour Change in Type 2 Diabetes
Diet behaviour is influenced by the interplay of the physical and social environment as well as macro-level and individual factors. In this study, we focus on diet behaviour at an individual level and describe the design of a behaviour change artefact to support diet behaviour change in persons with type 2 diabetes. This artefact was designed using a human-centred design methodology and the Behaviour Change Wheel framework. The designed artefact sought to support diet behaviour change through...
- The Preferential Use of Anakinra in Various Settings of FMF: A Review Applied to an Updated Treatment-Related Perspective of the Disease
Familial Mediterranean fever (FMF), the most frequent monogenic autoinflammatory disease, is manifested with recurrent and chronic inflammation and amyloid A (AA) amyloidosis, driven by overproduction of interleukin 1 (IL-1) through an activated pyrin inflammasome. Consequently, non-responsiveness to colchicine, the cornerstone of FMF treatment, is nowadays addressed by IL-1- blockers. Each of the two IL-1 blockers currently used in FMF, anakinra and canakinumab, has its own merits for FMF care....
- The Fetal Medicine Foundation (FMF) Germany after 20 Years - Quality Assurance of Ultrasound Examinations during First Trimester Screening
- Safety of SARS-CoV-2 vaccination in patients with Behcet's syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine
Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet's syndrome (BS) and familial Mediterranean fever (FMF) is limited. We studied the frequency of side effects and disease flares after COVID-19 vaccination with either Pfizer/BioNTech or Sinovac/CoronaVac in 256 patients with BS, 247 with FMF, and 601 with RD. Telephone interviews were conducted using a questionnaire survey in a cross-sectional design in...
- MEFV gene allele frequency and genotype distribution in 3230 patients' analyses by next generation sequencing methods
Familial Mediterranean Fever (FMF, OMIM ID: 249100) is the most common autoinflammatory, autosomal recessive disease caused by mutations in the MEFV gene. It is widespread in the Mediterranean, primarily among Turkish, Armenian, Arab and Jewish. This study aims to examine genotype distributions of common MEFV variants in the Turkish population using targeted NGS and to evaluate all rare mutations. It included 3230 people applying to Ege University Children's Hospital Molecular Medicine...
- Current treatment options for monogenic periodic fever syndromes - the role of interleukin 1 inhibitors
Monogenic periodic fever syndromes are heterogeneous group of autoinflammatory diseases including distinct syndromes, such as cryopyrin-associated periodic syndrome (CAPS), tumor necrosis factor alpha receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency/hyper IgD syndrome (MKD/HIDS), and familial Mediterranean fever (FMF). Individual diseases differ in pathogenesis, clinical manifestations, and severity. However, cytokines from the interleukin 1 (IL-1) family play a key...
- Conus Medullaris Infarction in a Patient With Familial Mediterranean Fever: A Case Report
CONCLUSION: Although CM infarction is rare in patients with FMF, it should be considered in the differential diagnosis when there is a high index of suspicion. The presence of vertebral body infarction with T2 changes on magnetic resonance imaging will indicate similar pathology in the CM.
- Interventions for reducing inflammation in familial Mediterranean fever
BACKGROUND: Familial Mediterranean fever (FMF), a hereditary auto-inflammatory disease, mainly affects ethnic groups living in the Mediterranean region. Early studies reported colchicine may potentially prevent FMF attacks. For people who are colchicine-resistant or intolerant, drugs such as anakinra, rilonacept, canakinumab, etanercept, infliximab or adalimumab might be beneficial. This is an update of the review last published in 2018.
- Frequency and severity of COVID-19 in patients with various rheumatic diseases treated regularly with colchicine or hydroxychloroquine
This study aimed to investigate whether patients regularly using colchicine or hydroxychloroquine (HCQ) have an advantage of protection from coronavirus disease 2019 (COVID-19) or developing less severe disease. Patients who were taking colchicine or HCQ regularly for a rheumatic disease including Familial Mediterranean Fever, Behçet's syndrome, Systemic Lupus Erythematosus, Rheumatoid Arthritis, and Sjogren's syndrome, as well as their healthy household contacts as the control group, were...
- MEFV Mutations in IBD Patients: A Systematic Review and Meta- analysis
CONCLUSIONS: MEFV mutations are common in IBD and are linked with the presence of extra-intestinal manifestations and pancolitis. Further research to assess the clinical significance and evolutionary significance of MEFV mutations in IBD patients is warranted.
- Genotyping of familial Mediterranean fever gene (MEFV)-Single nucleotide polymorphism-Comparison of Nanopore with conventional Sanger sequencing
CONCLUSIONS: These results demonstrated the great potential of current Nanopore sequencing for application in clinical diagnostics, at least for SNP genotyping by amplicon sequencing. Other more complex applications, especially structural variant identification, require further in-depth clinical validation.
- Evaluation of fetal cardiac morphology and functions in pregnant women with familial Mediterranean fever
CONCLUSION: There is no fetal cardiac morphological change in pregnant women with FMF. However, there may be changes in diastolic function. As the maternal FMF duration increases, systolic functions may also change.
- Fingolimod in Multiple Sclerosis and Familial Mediterranean Fever Coexistence
CONCLUSION: This report demonstrates an interesting clinical observation which may have promise for patients suffering both from MS and FMF. The drug's effect on the course of FMF needs further research.
Research: The Latestdeveloper2019-09-06T20:28:21+00:00