scholarly journals Endocan: A Novel Marker for Colchicine Resistance in Familial Mediterranean Fever Patients?

2021 ◽  
Vol 9 ◽  
Author(s):  
Ahmet Omma ◽  
Berkan Armaǧan ◽  
Serdar Can Güven ◽  
Sevinç Can Sandıkçı ◽  
Seda Çolak ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Acute Phase ◽  
Inflammatory Diseases ◽  
Cross Sectional Study ◽  
Control Group ◽  
Healthy Individuals ◽  
Acute Phase Reactants ◽  
Cross Sectional ◽  
Colchicine Resistance ◽  
Mediterranean Fever

Introduction: Familial Mediterranean fever (FMF) patients had 5–10% colchicine resistance. Although FMF attacks are characterized by acute phase elevation, there are no biomarkers that can show colchicine resistance yet. The serum endocan levels may elevate in inflammatory and auto-inflammatory diseases.Objectives: This study aimed to evaluate serum endocan levels in FMF patients according to whether attack and colchicine resistance or not and also compare them with classical acute phase reactants.Methods: In this single-center and cross-sectional study, a total of 111 FMF patients and 60 healthy individuals were enrolled. All patients' basic demographic and clinical data were recorded and blood samples were collected.Results: A total of 46 (41.4%) FMF patients had colchicine resistance. In comparison to the FMF patients according to colchicine response, colchicine resistance patients had a significantly higher median (IQR) endocan levels than colchicine responsive patients [36.98 ng/ml (97.41) vs. 13.57 ng/ml (27.87), p = 0.007], but there were no differences between in terms of median ESR and CRP levels. Inversely, serum endocan levels were similar during an attack and attack-free period in FMF patients, although ESR and CRP levels were significantly different. Interestingly, the highest serum endocan levels were in the control group.Conclusion: In conclusion, serum endocan levels were higher in colchicine resistance than colchicine responsive patients, but attack state had no effect on serum endocan levels in our study. Unlike ESR and CRP, serum endocan may be a novel biomarker for detection of colchicine resistance and distinguish the FMF attacks.

scholarly journals AB0419 THE COEXISTENCE OF FAMILIAL MEDITERRANEAN FEVER (FMF) IN SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) PATIENTS - A CROSS SECTIONAL STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1509.1-1510
Author(s):  
T. Klein ◽  
S. Tiosano ◽  
A. Chohen ◽  
H. Amital
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Familial Mediterranean Fever ◽  
Lupus Erythematosus ◽  
Cross Sectional Study ◽  
Review Of The Literature ◽  
Sectional Study ◽  
Systemic Lupus ◽  
Cross Sectional ◽  
Mediterranean Fever ◽  
Age And Sex

Background:Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammatory lesions affecting many organ systems in the body. Familial Mediterranean fever (FMF) is an autosomal recessive disease of chronic autoimmune inflammation characterized by frequently relapsing self-limiting fever and inflammation that may be localized in peritoneum, pleura, joint or skin.1Previous studies have described the similarity of clinical symptoms of FMF among SLE patients. However, the literature on this topic is inconsistent and based mostly on case reports.2-4Objectives:To examine the proportions of coexistence of FMF among SLE patients compared to the general population. We hypothesized that the proportion of FMF among SLE patients is higher than the general population.Methods:This cross-sectional study used the Clalit Health Services database, the largest Health Maintenance Organization in Israel, serving 4,400,000 members. SLE patients were compared to age- and sex-matched controls. Chi- was used for univariate analysis.Results:The study included4886 SLEpatients and 24430 age- and sex-matched controls. The SLE group had a significantly higher proportion of FMF patients compared to non-SLE controls (0.68% and 0.21% respectively; p < 0.001).Table 1. All study populationTable 1.SLE patients and matched controls basic characteristicsNo SLESLEp.overallN=24430N=4886Age51.2±16.551.2±16.51.000Gender: Female20100 (82.3%)4020 (82.3%)1.000FMF52 (0.21%)33 (0.68%)<0.001Table 2. StratificationTable 2.comparison of FMF patients with and without SLEFMF without SLEFMF with SLEp.overallN=52N=33Age44.6±13.750.5±17.70.106Gender: Female45 (86.5%)26 (78.8%)0.523Conclusion:FMF was found to be more common amongst SLE patients compared to matched controls.The current study results suggest that the occurrence of SLE turn patients with an appropriate genetic and environmental setting to develop also FMF. This cross-sectional study sheds light on the coexistence of these two diseases, autoimmune and autoinflammatory.References:[1]Kucuk A, Gezer IA, Ucar R, Karahan AY. Familial mediterranean fever.Acta Medica (Hradec Kralove). 2014;57(3):97-104.[2]Lidar M, Zandman-Goddard G, Shinar Y, Zaks N, Livneh A, Langevitz P. SLE and FMF: A possible negative association between the two disease entities–report of four cases and review of the literature.Lupus. 2008;17(7):663-669.[3]Erten S, Taskaldiran I, Yakut ZI. Are systemic lupus erythematosus patients carrying MEFV gene less prone to renal involvement? report of three cases and review of the literature.Ren Fail. 2013;35(7):1013-1016.[4]Shinar Y, Kosach E, Langevitz P, et al. Familial mediterranean Fever gene (MEFV) mutations as a modifier of systemic lupus erythematosus.Lupus. 2012;21(9):993-998.Disclosure of Interests: :None declared

scholarly journals ESTILO DE VIDA E SUAS RELAÇÕES COM HIPERTENSÃO ARTERIAL SISTÊMICA E DIABETES MELLITUS

2018 ◽  
Vol 7 (1) ◽  
pp. 361-373
Author(s):  
Elias Ferreira Porto ◽  
Claudia Kumpel ◽  
Anselmo Cordeiro de Souza ◽  
Izabel Maria de Oliveira ◽  
Karoline Mayara de Aquiles Bernardo ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
General Health ◽  
Healthy Lifestyle ◽  
Cross Sectional Study ◽  
Health Perception ◽  
Control Group ◽  
Healthy Individuals ◽  
Cross Sectional ◽  
Significant Difference ◽  
The Individual

Avaliar o estilo de vida e percepção do estado geral de saúde em pacientes com Diabetes Mellitus tipo 2 - DM, Hipertensão Arterial Sistêmica - HAS e indivíduos saudáveis. Métodos: Estudo transversal, com grupo controle. Avaliado estilo de vida com Questionário Fantástico, e percepção geral de saúde via uma pergunta âncora de diabéticos do tipo 2 (n = 37), hipertensos (n = 60), e indivíduos saudáveis (n = 43). Análise estatística descritiva, analise de variância, e razão de chance (Odds Rattio – OR). Resultados: Verificou-se diferença significante (p0,0001) na pontuação do Questionário Fantástico entre os indivíduos saudáveis em relação DM e HAS. O pior desempenho no estilo de vida para os três grupos foi nos domínios de atividade física e alimentação. O risco do indivíduo com HAS afirmar que sua saúde é pior do que indivíduos da mesma faixa etária foi OR 1.8 (1.415 a 2.419) e para os indivíduos diabéticos OR de 2.8 (1.776 a 4.579) em relação a indivíduos saudáveis. Conclusão: Diabéticos e hipertensos têm um pior estilo de vida e percepção geral de saúde do que indivíduos saudáveis. Assim o estilo de vida saudável pode reduzir a prevalência e auxiliar no controle de doenças já estabelecidas.Palavras-chave: Estilo de vida. Hipertensão Arterial. Diabetes Mellitus. ABSTRACT: To evaluate the lifestyle and perception of general health status in patients with type 2 diabetes mellitus - DM, systemic arterial hypertension - SAH and healthy individuals. Methods: Cross-sectional study with control group. (N = 37), hypertensive (n = 60), and healthy subjects (n = 43) were assessed using a Fantastic Questionnaire, and general health perception via an anchor question. Descriptive statistical analysis, analysis of variance, and odds ratio (Odds Rattio - OR). Results: There was a significant difference (p 0.0001) in the score of the Fantastic Questionnaire among healthy individuals in relation to DM and SAH. The worst lifestyle performance for the three groups was in the physical activity and feeding domains. The risk of the individual with SAH to state that their health is worse than individuals of the same age group was OR 1.8 (1.415 to 2.419) and for the diabetic individuals OR of 2.8 (1.776 to 4.579) in relation to healthy individuals. Conclusion: Diabetics and hypertensives have a worse lifestyle and overall health perception than healthy individuals. Thus the healthy lifestyle can reduce the prevalence and help in the control of already established diseases.Keywords: Lifestyle. Hypertension. Diabetes Mellitus.

scholarly journals Analysis of heparanase isoforms and cathepsin B in the plasma of patients with gastrointestinal carcinomas: analytical cross-sectional study

2015 ◽  
Vol 133 (1) ◽  
pp. 28-35 ◽  
Author(s):  
Carina Mucciolo Melo ◽  
Clarice Silvia Taemi Origassa ◽  
Thérèse Rachell Theodoro ◽  
Leandro Luongo Matos ◽  
Thaís Aguilar Miranda ◽  
...  
Keyword(s):  
Cathepsin B ◽  
Cross Sectional Study ◽  
Control Group ◽  
Plasma Samples ◽  
Healthy Individuals ◽  
Sectional Study ◽  
Gastrointestinal Carcinoma ◽  
Control Group Design ◽  
Cross Sectional ◽  
Gastrointestinal Carcinomas

CONTEXT AND OBJECTIVE: Heparanase-1 degrades heparan sulfate and has been correlated with tumor progression. Although the isoform heparanase-2 has no catalytic activity, it seems to be important for modulating heparanase-1 activity. Cathepsin B is a proteinase involved in tumor metastasis. The aim of this study was to analyze heparanase isoform expression and cathepsin B activity in plasma samples from patients with gastrointestinal carcinomas, compared with healthy individuals (control group). DESIGN AND SETTING: This was an analytical cross-sectional study. Peripheral blood samples were collected at a Brazilian public hospital, from 21 patients with histopathological diagnoses of gastrointestinal carcinomas and from 43 healthy individuals. The analyses were performed in two Brazilian medical schools. METHODS: Heparanase isoforms were identified and quantified in plasma samples by means of Western blot. The enzymatic activities of heparanase-1 and cathepsin B were also measured. RESULTS: The results demonstrated that the expression of both heparanase isoforms was significantly greater in plasma samples from gastrointestinal carcinoma patients, compared with the control group. Logistic regression analysis showed that increased heparanase-1 and heparanase-2 expression was exclusively dependent on the tumor. There was a significant increase in heparanase-1 and cathepsin B activity in the patients' plasma. CONCLUSION: Overexpression of heparanase-1 and heparanase-2, along with increased heparanase-1 and cathepsin B activity in plasma, is associated with the diagnosis of gastrointestinal carcinoma. These findings provide support for using non-invasive assays (plasma samples) as an auxiliary method for diagnosing gastrointestinal tumors.

scholarly journals Differentiating children with familial Mediterranean fever from other recurrent fever syndromes: The utility of new Eurofever/PRINTO classification criteria

2021 ◽  
Vol 36 (4) ◽  
pp. 493-498
Author(s):  
Rabia Miray Kışla Ekinci ◽  
Sibel Balcı ◽  
Ahmet Hakan Erol ◽  
Dilek Karagöz ◽  
Derya Ufuk Altıntaş ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Sensitivity And Specificity ◽  
Mefv Gene ◽  
Classification Criteria ◽  
Recurrent Fever ◽  
Control Group ◽  
Mevalonate Kinase Deficiency ◽  
Cross Sectional ◽  
M694v Mutation ◽  
Mediterranean Fever

Objectives: In this study, we aimed to investigate the performance of Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria in pediatric patients with familial Mediterranean fever (FMF). Patients and methods:This retrospective, cross-sectional study included a total of 130 pediatric FMF patients (67 males, 63 females; mean age: 12.4±3.6 years; range, 2.5 to 17.7 years) with at least one M694V mutation in MEFV gene between July 2010 and July 2019. Demographic features and disease characteristics were recorded. The control group was consisted of 41 patients (19 males, 22 females; mean age: 7.8±4.0 years; range, 2.1 to 17.8 years) with other hereditary autoinflammatory diseases (AIDs), including periodic fevers with aphthous stomatitis, pharyngitis, and adenitis syndrome (n=30), mevalonate kinase deficiency (n=9), and tumor necrosis factor receptor-associated periodic syndrome (n=2). Sensitivity and specificity of the Eurofever/PRINTO classification criteria were calculated. Results: The sensitivity and specificity were 97.7% and 56.1% for Yalcinkaya-Ozen criteria, respectively and 93.1% and 90.2% for Tel Hashomer criteria, respectively. The Eurofever/PRINTO classification criteria reached a sensitivity and specificity of 94.6% and 82.9% and 93.1% and 80.5%, respectively, when genetic plus clinical criteria and clinical-only criteria were applied. Conclusion: The Eurofever/PRINTO classification criteria have a comparable sensitivity for avoidance of FMF underdiagnosis in childhood. The Yalcinkaya-Ozen criteria have the highest sensitivity without a significant specificity. The Tel Hashomer criteria and Eurofever/PRINTO classification criteria were superior to Yalcinkaya-Ozen criteria to differentiate FMF from other AIDs, thus leading to less complications relevant to underdiagnosis of other AIDs.

scholarly journals Parental knowledge about familial mediterranean fever: a cross-sectional study

2021 ◽  
Vol 63 (6) ◽  
pp. 1048
Author(s):  
Deniz Gezgin Yıldırım ◽  
Sevcan A. Bakkaloğlu ◽  
A. Şebnem Soysal Acar ◽  
Necla Buyan
Keyword(s):  
Familial Mediterranean Fever ◽  
Parental Knowledge ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional ◽  
Mediterranean Fever

scholarly journals Comparison of optical coherence tomography angiography results of adult patients with Familial Mediterranean fever and healthy individuals

2019 ◽  
Vol 11 ◽  
pp. 251584141989205
Author(s):  
Emine Esra Karaca ◽  
Dilay Ozek ◽  
Ahmet Omma ◽  
Ozlem Evren Kemer
Keyword(s):  
Optical Coherence Tomography ◽  
Familial Mediterranean Fever ◽  
Optical Coherence Tomography Angiography ◽  
Foveal Avascular Zone ◽  
Control Group ◽  
Vascular Density ◽  
Optical Coherence ◽  
Healthy Individuals ◽  
Avascular Zone ◽  
Mediterranean Fever

Purpose: The aim of this study is to assess the macular ultrastructure measuring by optical coherence tomography angiography in adult patients with Familial Mediterranean fever. Methods: Participants were 62 Familial Mediterranean fever patients and 54 healthy individuals in control group with similar age and sex. The superficial and deep vascular plexus structures covering the central fovea in an area of 6 mm × 6 mm were measured using AngioVue images taken with optical coherence tomography angiography. Vasculature structure, foveal avascular zone, acircularity index of foveal avascular zone (the ratio of the perimeter of foveal avascular zone and the perimeter of a circle with the equal area), and superficial and deep retinal plexus densities were measured. Results: The inferior deep vascular density was measured: 49.17% ± 8.59% in Familial Mediterranean fever patients, 55.56% ± 5.92% in the control group. The deep inferior-hemi vascular density was measured: 48.59% ± 10.34% in Familial Mediterranean fever patients, 56.54% ± 8.05% in the control group. Deep inferior and deep inferior-hemi vascular density was significantly reduced in Familial Mediterranean fever patients compared with healthy controls ( p = 0.04 and p = 0.03, respectively). Conclusion: The vascular abnormalities in optical coherence tomography angiography show subclinical signs of microangiopathy in Familial Mediterranean fever patients. This observation, which can be obtained only through optical coherence tomography angiography, may be an ocular hallmark for Familial Mediterranean fever disease.

Familial Mediterranean Fever in Siblings

2012 ◽  
Vol 39 (11) ◽  
pp. 2170-2174
Author(s):  
Z. BIRSIN ÖZÇAKAR ◽  
BEYZA DOGANAY ERDOGAN ◽  
ATILLA H. ELHAN ◽  
FATOŞ YALÇINKAYA
Keyword(s):  
Familial Mediterranean Fever ◽  
Disease Severity ◽  
Acute Phase ◽  
Demographic Data ◽  
Intraclass Correlation ◽  
Age At Onset ◽  
Disease Onset ◽  
Acute Phase Reactant ◽  
Acute Phase Reactants ◽  
Mediterranean Fever

Objective.Genetic and environmental factors have been implicated in disease severity and development of amyloidosis in familial Mediterranean fever (FMF). We investigated similarities in clinical characteristics, disease severity, and treatment response within siblings with FMF.Methods.The study group consisted of 2 or more siblings who were followed in our center with the diagnosis of FMF. Siblings were evaluated for demographic data, clinical and laboratory disease features, genetic analysis of MEFV mutations, and disease severity score. The intraclass correlation coefficient (ICC), which can be interpreted as the expected correlation between 2 siblings, was used to reflect within-family similarity.Results.The study included 67 pediatric patients from 31 different families. When we investigated the similarity of siblings after adjusting for genetic effects, we found very low ICC with p > 0.05 in the majority of clinical features, disease severity, and colchicine dosages. However, age at disease onset, age at onset of therapy, attack-free acute-phase reactant levels, and presence of amyloidosis were found to be similar within siblings (relatively high ICC with p < 0.05).Conclusion.Siblings with FMF had different clinical findings and disease severity. They had similar amyloidogenic potential, proven by both similar presence of amyloid and increased levels of acute-phase reactants between attacks. Our findings strongly support that genetic factors may be more dominant in the development of amyloidosis.

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