- Optimising Preeclampsia First-Trimester Screening Using Three Parameters
CONCLUSION: Although the performance of adapted FMF algorithm to predict preeclampsia gestational was low, it was found superior to prediction by maternal risk factors alone. Adjustment for additional factors or ethnicity-specific values may help in further improvement of detection rate.
- Assessing fatigue and related factors in adolescents with familial Mediterranean fever (FMF): psychometric properties of the PedsQL Multidimensional Fatigue Scale
CONCLUSIONS: PedsQL-MFS seems to be feasible for assessing fatigue in adolescents with FMF. Sex, recent attacks, sleep, and physical activity should be taken into consideration in the fatigue management of patients with FMF.
- Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients
CONCLUSION: About half the patients categorized with ePFAPA syndrome already had extreme features upon presentation. Patients with ePFAPA compared to nPFAPA presented and were diagnosed at an earlier age.
- Autoinflammatory Diseases/Periodic Fevers
Children with intermittent fevers present to pediatricians and other primary care child health providers for evaluation. Most patients will have self-limited, benign infectious illnesses. However, the possibility of a periodic fever syndrome should be considered if febrile episodes become recurrent over an extended period and are associated with particular signs and symptoms during each attack. This review discusses the current conceptualization of autoinflammatory diseases with specific focus...
- Shear wave elastography evaluation of kidneys in children with familial mediterranean fever
CONCLUSIONS: SWE can be a noninvasive quantitative imaging method that can be used to evaluate kidney involvement by detecting changes in kidney stiffness in children with FMF.
- Successful Therapy with Canakinumab and Mepolizumab for Familial Mediterranean Fever and Eosinophilic Pneumonia
No abstract
- Local Staging of Prostate Cancer at MRI: What the Urologist and Radiation Oncologist Want to Know
This slide presentation provides an overview of prostate cancer risk stratification and management, reviews key anatomic and imaging principles pertaining to local staging of prostate cancer, and highlights inflection points where MRI findings regarding local staging may modify clinical management.
- Monogenic autoinflammatory disease-associated cardiac damage
INTRODUCTION: Autoinflammatory diseases (AIDs) constitute several disorders that are characterized by the presence of recurrent episodes of unprovoked inflammation due to dysregulated innate immune system in the absence of autoantibodies or infections. Most of them have a strong genetic background, with mutations in single genes involved in inflammation referred to monogenic AIDs. In this article, we will review the cardiac manifestations in various monogenic AIDs.
- Identification by Exome Sequencing of Predisposing Variants in Familial Cases of Autoinflammatory Recurrent Fevers
Periodic fever syndromes include autoinflammatory disorders (AID) that involve innate immunity. These disorders are characterized by recurrent fevers and aberrant multi-organ inflammation, without any involvement of T or B cells or the presence of autoantibodies. A complex genetic architecture has been recognized for many AID. However, this complexity has only been partially uncovered for familial Mediterranean fever and other conditions that have a classical monogenic origin and Mendelian...
- Familial Mediterranean Fever-Related Amyloidosis in Turkey: A Need for a Familial Mediterranean Fever Registry
Amyloidosis has been well known since Rudolph Virchow named the condition in the 19th century. Most physicians were aware of the association between amyloidosis to chronic suppurative conditions and multiple myeloma. However, familial Mediterranean fever, although probably as ancient as the Galenic era, was appropriately identified only in the 21st century. The nomenclature was variable throughout history, but the name "FMF" has been universally adopted since the report from Heller and...
- Evaluation of first trimester maternal serum inhibin-A for preeclampsia screening
CONCLUSION: Replacing PlGF by inhibin-A or adding inhibin-A as an additional biomarker in and to the FMF triple screening test for preterm PE does not improve screening performance and will fail to identify pregnancies that are currently identified by the FMF triple test.
- Identification and confirmation via in situ hybridization of Merkel cell polyomavirus in rare cases of posttransplant cutaneous T-cell lymphoma
CONCLUSIONS: Our findings raise the question of whether MCPyV may play a role in rare cases of T-lymphoproliferative disorders, particularly in the skin and in the heavily immunosuppressed posttransplant setting.
- Comparison of posterior segment ocular parameters of Familial mediterranean fever with homozygous M694V mutation and carriers with heterozygous M694V mutation in children
CONCLUSION: FMF is a hereditary autoinflammatory disease that causes multi-organ involvement, and this study showed that posterior segment ocular parameters could be affected not only in FMF patients but also in asymptomatic FMF carriers.
- Fractal dimension analysis of different mandibular regions in familial Mediterranean fever patients: A cross-sectional retrospective study
Familial Mediterranean fever (FMF) is a genetic condition that may cause loss of bone mineral density (BMD) due to chronic inflammation. Previously, fractal dimension (FD) analysis values of mandibular cortical bone were shown to be lower in osteoporosis. Therefore, FD might be considered as an auxiliary tool to refer patients for dual-energy x-ray absorptiometry (DXA), which is the gold standard for BMD measurement. The purpose of this cross-sectional retrospective study was to evaluate...
- Generation of three induced pluripotent stem cell lines (RAUi001-A, RAUi001-B and RAUi001-C) from peripheral blood mononuclear cells of a healthy Armenian individual
The study of pathological processes in cells carrying mutations should be carried out in comparison with a healthy control group. Familial Mediterranean fever (FMF), which is caused by a mutation in the MEFV gene, is predominantly found in people of Armenian nationality with the prevalence of 14-100 per 10000. We have obtained induced pluripotent stem cells (iPSCs) from Armenian healthy patient, which will be included as a control group in the study of this disease. iPSCs rapidly proliferate in...
- Prediction of Perinatal and Neurodevelopmental Outcomes in Newborns with a Birth Weight below the 3rd Percentile: Performance of Two International Curves - Prospective Cohort from a Brazilian City
CONCLUSION: Birth weight below the 3rd percentile according to INT or FMF alone was insufficient for a good diagnostic performance of perinatal and neurodevelopmental outcomes. The analyzes performed could not show that one curve is better than the other in our population. INT may have an advantage in resource contingency scenarios as it discriminates fewer NB below the 3rd percentile without increasing adverse outcomes.
- Exertional leg pain represents a severe disease phenotype in childhood familial Mediterranean fever
CONCLUSIONS: Exertional leg pain in pediatric FMF patients is the component of moderate-to-severe disease course, and this may be considerably associated with the presence of M694V mutation.
- An Update on Familial Mediterranean Fever
(1) Background: Familial Mediterranean Fever (FMF) is the prototypal autoinflammatory disease, characterized by recurrent bursts of neutrophilic inflammation. (2) Methods: In this study we look at the most recent literature on this condition and integrate it with novel information on treatment resistance and compliance. (3) Results: The canonical clinical presentation of FMF is in children with self-limited episodes of fever and polyserositis, associated with severe long-term complications, such...
- Invited Commentary: Pelvic MRI Is Now the Basis of Personalized Fibroid Care
No abstract
- Dysregulation of miRNA-30e-3p targeting IL-1β in an international cohort of systemic autoinflammatory disease patients
Autoinflammation is the standard mechanism seen in systemic autoinflammatory disease (SAID) patients. This study aimed to investigate the effect of a candidate miRNA, miR-30e-3p, which was identified in our previous study, on the autoinflammation phenotype seen in SAID patients and to analyze its expression in a larger group of European SAID patients. We examined the potential anti-inflammatory effect of miR-30e-3p, which we had defined as one of the differentially expressed miRNAs in microarray...
Research: The Latestdeveloper2019-09-06T20:28:21+00:00