151 results match your criteria Avi Livneh


Repolarization dispersion in patients with systemic sclerosis.

Eur J Rheumatol 2020 Dec 1. Epub 2020 Dec 1.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objective: Systemic sclerosis (SSc) is associated with increased cardiac morbidity and mortality. Whether some electrocardiographic markers of arrhythmias predispose to early cardiogenic death in SSc remains controversial. This study evaluated the occurrence of previously reported as well as unstudied markers of repolarization in patients with SSc and assessed their prognostic implications. Read More

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December 2020

Amyloid storm: acute kidney injury and massive proteinuria, rapidly progressing to end-stage kidney disease in AA amyloidosis of familial Mediterranean fever.

Rheumatology (Oxford) 2020 Dec 8. Epub 2020 Dec 8.

Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv.

Objective: Amyloid A nephropathy of FMF usually progresses over many years to end-stage renal disease (ESRD). We aim to describe an acute condition, termed here 'amyloid storm', typically manifesting with a rapid (≤2 weeks) increase in serum creatinine and urine protein, that has never been characterized in FMF amyloidosis.

Methods: This retrospective analysis features amyloid storm by comparing between FMF amyloidosis patients who have experienced an episode of amyloid storm (study group) and matched patients who have not (control group). Read More

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December 2020

Burden of illness in hereditary periodic fevers: a multinational observational patient diary study.

Clin Exp Rheumatol 2020 Sep-Oct;38 Suppl 127(5):26-34. Epub 2020 Sep 30.

Medicine F, Sheba Medical Centre, Tel-Hashomer, Ramat-Gan, Israel.

Objectives: This study aimed to characterise the burden of illness of patients with inadequately controlled hereditary periodic fevers (HPFs), during and outside of flares. It was focused on the burden to the patients and also considered the wider impact on their caregivers and families.

Methods: The target population was patients or caregivers of patients with clinically/genetically confirmed colchicine resistant FMF (crFMF), mevalonate kinase deficiency/hyperimmunoglobinaemia D with periodic fever syndrome (MKD/HIDS) or TRAPS, who were expected to flare at least once in a 6-month period based on patient history. Read More

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December 2020

Evaluation of a Proarrhythmic Repolarization Marker (Total Cosine R to T) in Patients With Uncomplicated Familial Mediterranean Fever.

J Clin Rheumatol 2020 Dec;26(8):334-337

Familial Mediterranean fever (FMF) is a systemic autoinflammatory disorder characterized by recurrent attacks of fever and serous inflammation. The association between FMF and risk of cardiac arrhythmia is continuously questioned; some studies report abnormal cardiac repolarization, while others do not. Considering this controversy, we aim to perform in a large cohort of FMF patients a total cosine R to T (TCRT) analysis, a previously unexplored repolarization marker in this disorder. Read More

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December 2020

Saliva immunoglobulin free light chain analysis for monitoring disease activity and response to treatment in multiple sclerosis.

Mult Scler Relat Disord 2020 Sep 24;44:102339. Epub 2020 Jun 24.

Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.

Background: Immunoglobulin free light chains (FLC) have recently gained considerable interest as new promising intrathecal biomarkers of multiple sclerosis (MS). However, lumbar puncture is invasive and not practical for monitoring disease course. This study aimed to assess the utility of saliva FLC as a biomarker of disease activity and response to treatment in MS METHODS: Western blotting was used to study saliva FLC monomers and dimers. Read More

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September 2020

QT variability index in patients with systemic sclerosis.

Eur J Rheumatol 2019 10 1;6(4):179-183. Epub 2018 Oct 1.

Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objective: Systemic sclerosis (SSc) may affect the heart with microvascular dysfunction and lead to an early cardiac death, but the association between certain repolarization indexes and SSc heart disease remains controversial. Our goal was to evaluate a previously unstudied marker of repolarization dynamics, i.e. Read More

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October 2019

[IS THERE A CORRELATION BETWEEN CARRIAGE OF AN MEFV MUTATION AND GOUT?]

Harefuah 2019 Sep;158(9):576-578

The Rheumatology Unit, Sheba Medical Center, Ramat Gan, Israel.

Introduction: Gout is an inflammatory condition mediated by Interleukin-1-beta (IL-1β). A mutation in the MEFV gene (the gene related to Familial Mediterranian fever) may cause an elevation in IL-1β, and is associated with a variety of inflammatory conditions. Reports in the literature are inconsistent as to whether a mutated MEFV gene is related to the phenotype of gout. Read More

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September 2019

Toll-like receptor 2 is overexpressed in Familial Mediterranean fever patients and is inhibited by colchicine treatment.

Best Pract Res Clin Rheumatol 2018 10 19;32(5):651-661. Epub 2019 Feb 19.

Heller Institute of Medical Research, Tel-Aviv University, Tel-Aviv, Israel; Rheumatology Unit, The Chaim Sheba Medical Center, Tel-Hashomer, Israel; Department of Internal Medicine F, The Chaim Sheba Medical Center, Tel-Hashomer, Israel; The Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel; The Talpiot Medical Leadership Program, Sheba Medical Center, Tel-Hashomer, Israel. Electronic address:

Aim: To study the role of Toll-like receptor (TLR) 2 in Familial Mediterranean fever (FMF) inflammatory process.

Methods: TLR2 expression on monocytes of FMF attack-free patients (n = 20) and the effect of sera of FMF patients with an acute attack (n = 9) on TLR2 expression on monocytes of healthy donors were studied by flow cytometry (FACS). TLR2 expression was also studied in THP-1 cells, and TLR2 downstream signaling was studied by ELISA for the secretion of IL-1β and pro-inflammatory cytokines or by western blotting to measure nuclear factor (NF)-κB. Read More

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October 2018

Classification criteria for autoinflammatory recurrent fevers.

Ann Rheum Dis 2019 08 24;78(8):1025-1032. Epub 2019 Apr 24.

Clinica Pediatrica e Reumatologia, PRINTO, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

Background: Different diagnostic and classification criteria are available for hereditary recurrent fevers (HRF)-familial Mediterranean fever (FMF), tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency (MKD) and cryopyrin-associated periodic syndromes (CAPS)-and for the non-hereditary, periodic fever, aphthosis, pharyngitis and adenitis (PFAPA). We aimed to develop and validate new evidence-based classification criteria for HRF/PFAPA.

Methods: Step 1: selection of clinical, laboratory and genetic candidate variables; step 2: classification of 360 random patients from the Eurofever Registry by a panel of 25 clinicians and 8 geneticists blinded to patients' diagnosis (consensus ≥80%); step 3: statistical analysis for the selection of the best candidate classification criteria; step 4: nominal group technique consensus conference with 33 panellists for the discussion and selection of the final classification criteria; step 5: cross-sectional validation of the novel criteria. Read More

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Diagnostic utility of kappa free light chains in multiple sclerosis.

Expert Rev Mol Diagn 2019 04 27;19(4):277-279. Epub 2019 Feb 27.

a Heller Institute of Medical Research, Sheba Medical Center , Tel-Hashomer , Ramat Gan , Israel.

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Kidney disease and plasma cell dyscrasias: ambiguous cases solved by serum free light chain dimerization analysis.

Clin Exp Nephrol 2019 Jun 8;23(6):763-772. Epub 2019 Feb 8.

Laboratory of FMF and Amyloidosis, Heller Institute of Medical Research, Sheba Medical Center, Tel Hashomer, 52621, Ramat Gan, Israel.

Background: Plasma cell dyscrasias (PCD) comprise a wide spectrum of disorders, which may adversely affect the kidney. However, in some PCD cases associated with kidney disease, the routine laboratory tests may be incapable to determine precisely the form of PCD, i.e. Read More

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Efficacy and safety of long-term treatment with intravenous colchicine for familial Mediterranean fever (FMF) refractory to oral colchicine.

Rheumatol Int 2019 03 2;39(3):517-523. Epub 2019 Jan 2.

Department of Internal Medicine F, The Chaim Sheba Medical Center, 52621, Tel-Hashomer, Israel.

Colchicine is the mainstay of treatment for familial Mediterranean fever (FMF). Intravenous (IV) colchicine, administered over several months, has been shown to be effective for FMF patients unresponsive to oral colchicine. The objective of this study was to evaluate the efficacy and safety of long-term IV colchicine treatment in oral colchicine-resistant FMF. Read More

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Inflammation and cardiovascular disease in familial Mediterranean fever. An analysis of hospital admissions for acute cardiovascular event.

Clin Exp Rheumatol 2018 Nov-Dec;36(6 Suppl 115):80-85. Epub 2018 Sep 27.

Medicine F, Sheba Medical Center, Tel-Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Israel.

Objectives: Patients, suffering from inflammatory disorders, are at an increased risk to develop cardiovascular disease (CVD). Here, we examine whether in familial Mediterranean fever (FMF), a model of inflammatory diseases, inflammation also increases the risk to develop cardiovascular (CV) disease.

Methods: To explore the role of inflammation in the occurrence of CVD in FMF, we identified all FMF patients ≤55 years old with CVD, admitted to our center over a 15-year period. Read More

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Familial Mediterranean fever (FMF) phenotype in patients homozygous to the MEFV M694V mutation.

Eur J Med Genet 2019 Jun 29;62(6):103532. Epub 2018 Aug 29.

Department of Medicine F, The Chaim Sheba Medical Center, Tel-Hashomer, 5265601, Israel; Rheumatology Unit, The Chaim Sheba Medical Center, Tel-Hashomer, 5265601, Israel; Heller Institute of Medical Research, The Chaim Sheba Medical Center, Tel-Hashomer, 5265601, Israel; Sackler Faculty of Medicine, Tel-Aviv University, Israel. Electronic address:

The clinical presentation of familial Mediterranean fever (FMF) is remarkably variable, ranging from a quiescent to a severe and disabling disease. The M694V mutation is one of approximately 300 published genetic variations in the FMF gene. While some studies have reported a more severe phenotype for the homozygous M694V mutation, studies dedicated solely to featuring the phenotype of homozygous M694V genotype are meager. Read More

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Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study.

Ann Rheum Dis 2018 11 12;77(11):1558-1565. Epub 2018 Aug 12.

Clinica Pediatrica e Reumatologia-PRINTO, Istituto Giannina Gaslini, Genoa, Italy.

Autoinflammatory diseases (AIDs) are a relatively new family of disorders, defined about 19 years ago. Some of them are hereditary and some are not. The names given to these diseases do not follow any systematic guidelines, and sometimes the same disorder carries several names. Read More

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November 2018

Analysis of microRNAs in familial Mediterranean fever.

PLoS One 2018 22;13(5):e0197829. Epub 2018 May 22.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objectives: Although Familial Mediterranean fever (FMF) is categorized as autosomal recessive, frequent exceptions to this model exist and therefore we aimed to search epigenetic modifications in this disease.

Methods: Ten M694V homozygous FMF patients (the most severe phenotype) were recruited for this study. Patients with inflammatory flare were excluded. Read More

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November 2018

Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes.

N Engl J Med 2018 05;378(20):1908-1919

From the Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, Rome (F.D.B.), Clinica Pediatrica e Reumatologia, Unità Operativa Semplice Dipartimentale di Malattie Autoinfiammatorie e Immunodeficienze, IRCCS, Istituto G. Gaslini, Genoa (M.G.), the Pediatric Clinic, University of Brescia and Spedali Civili, Brescia (M.C.), and the Amyloidosis Research and Treatment Center, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo, Pavia (L.O.) - all in Italy; the Division of Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona (J.A.), and the Internal Medicine Department, Autoimmune and Systemic Diseases Unit, Hospital Vall d'Hebron (S.B.-R.), Barcelona, and the Pediatric Rheumatology Unit, Hospital Universitario y Politécnico La Fe, Valencia (I.C.P.) - all in Spain; the Rheumatology Unit, Hadassah-Hebrew University Hospital (E.B.-C.), and the Pediatric Rheumatology Unit, Shaare Zedek Medical Center (P.J.H.), Jerusalem, and Heller Institute of Medical Research and Medicine Faculty, Sheba Medical Center, Tel-Hashomer and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv (A.L.) - all in Israel; the Division of Pediatrics, University Medical Center Utrecht, Utrecht (J.F.), and the Radboud Expertise Center for Immunodeficiency and Autoinflammation, Department of Internal Medicine, Radboud University Medical Center, Nijmegen (A. Simon) - both in the Netherlands; the Departments of Pediatrics and Medicine, University of California at San Diego and Rady Children's Hospital San Diego, San Diego (H.M.H.); the Department of Pediatric Rheumatology, Centre de Référence des Maladies Auto-inflammatoires et de l'Amylose Inflammatoire, Centre Hospitalier Universitaire (CHU) de Bicêtre, Assistance Publique-Hopitaux de Paris (APHP), Université de Paris Sud (I.K.-P.), and Paris-Descartes University, Imagine Institute, Unité d'Immuno-Hématologie et Rhumatologie Pédiatrique, Hôpital Necker-Enfants Malades, APHP (P.Q.), Paris; the National Amyloidosis Centre, University College London Division of Medicine, Royal Free Campus (H.J.L.), and University College London, Great Ormond Street Institute of Child Health, and Great Ormond Street Hospital for Children NHS Foundation Trust (P.B.), London; the Department of Pediatrics, Hacettepe University, Ankara (S.O.), and the Department of Pediatric Rheumatology, Cerrahpasa Medical School (O.K.), and Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology (A.G.), Istanbul University, Istanbul - all in Turkey; the Department of Pediatrics, Division of Pediatric Rheumatology, Cleveland Clinic, Cleveland (A.Z.); the Department of Infectious Diseases and General Internal Medicine, CHU Sart-Tilman, University of Liège, Liege (M.M.), and the Department of Infectious Diseases and Immunity, Jessa Hospital, University of Hasselt, Hasselt (J.V.H.) - both in Belgium; the Department of Clinical Immunology, Center for Pediatric Hematology, Oncology, and Immunology, Moscow (A. Shcherbina); Pediatric Rheumatology of Western Switzerland, University of Lausanne, Lausanne, (M.H.), and Novartis, Basel (K.L., A. Speziale, G.J.) - both in Switzerland; the Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan (R.H.); and the Department of Pediatrics, Semmelweis Egyetem, Budapest, Hungary (T.C.).

Background: Familial Mediterranean fever, mevalonate kinase deficiency (also known as the hyperimmunoglobulinemia D syndrome), and the tumor necrosis factor receptor-associated periodic syndrome (TRAPS) are monogenic autoinflammatory diseases characterized by recurrent fever flares.

Methods: We randomly assigned patients with genetically confirmed colchicine-resistant familial Mediterranean fever, mevalonate kinase deficiency, or TRAPS at the time of a flare to receive 150 mg of canakinumab subcutaneously or placebo every 4 weeks. Patients who did not have a resolution of their flare received an add-on injection of 150 mg of canakinumab. Read More

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The use of serum free light chain dimerization patterns assist in the diagnosis of AL amyloidosis.

Br J Haematol 2018 07 16;182(1):86-92. Epub 2018 May 16.

Heller Institute of Medical Research, Sheba Medical Centre, Tel-Hashomer, Israel.

The discrimination between benign and malignant forms of plasma cell dyscrasia (PCD) is often difficult. Free light chain monomer-dimer pattern analysis (FLC-MDPA) may assist in solving this dilemma and distinguish between AL amyloidosis and benign PCD. Serum samples of patients with AL amyloidosis and benign PCD were analysed in a blinded manner. Read More

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Reply.

Arthritis Rheumatol 2018 07 14;70(7):1167-1168. Epub 2018 May 14.

Sheba Medical Center, Tel Hashomer, Israel.

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Search for new biomarkers of pediatric multiple sclerosis: application of immunoglobulin free light chain analysis.

Clin Chem Lab Med 2018 06;56(7):1081-1089

Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Israel.

Background: Identifying new biomarkers is needed to overcome the diagnostic difficulties of pediatric multiple sclerosis (MS). Recently, we developed a new technique including CSF analysis of free light chain (FLC) monomers and dimers, which can improve diagnosis of adult MS. The present study has been designed to evaluate the utility of our technique for MS diagnosis in children. Read More

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Late ventricular potentials in familial Mediterranean fever with and without AA amyloidosis.

Eur J Rheumatol 2017 Sep 1;4(3):184-188. Epub 2017 Sep 1.

The Heller Institute of Medical Research and Medicine F, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel.

Objective: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by episodic and chronic inflammation that may lead to both accelerated coronary atherosclerosis and cardiac AA amyloidosis. We hypothesized that late ventricular potentials (LPs), an established electrocardiographic susceptibility marker of ventricular arrhythmias, will be more common in FMF than in the adjusted normal population due to these two types of inflammation-associated cardiac effects. Therefore, we aimed to evaluate the occurrence of LPs in FMF patients with and without amyloidosis. Read More

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September 2017

Reply to: More erroneous statements about the use of frequentist statistical methods in medical research.

Authors:
Avi Livneh

Clin Exp Rheumatol 2019 Nov-Dec;37 Suppl 122(6). Epub 2017 Oct 26.

Department of Medicine 6, Sheba Medical Center, Tel Hashomer, Israel.

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December 2019

A high and equal prevalence of the Q703K variant in NLRP3 patients with autoinflammatory symptoms and ethnically matched controls.

Clin Exp Rheumatol 2017 Nov-Dec;35 Suppl 108(6):82-85. Epub 2017 Oct 6.

Sheba Medical Centre, affiliated to the Sackler School of Medicine, Tel Aviv University, Israel.

Objectives: Cryopyrin associated periodic syndromes (CAPS) comprise a spectrum of autoinflammatory disorders of varying severity caused by mutations in the NLRP3 gene. The NLRP3-Q703K allele has been reported both as a functional polymorphism and as a low penetrance mutation.

Methods: To describe the clinical phenotype of subjects with the Q703K allele and to report the frequency of this allele among patients with autoinflammatory symptoms and healthy controls. Read More

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February 2018

Familial Mediterranean Fever and Incidence of Cancer: An Analysis of 8,534 Israeli Patients With 258,803 Person-Years.

Arthritis Rheumatol 2018 01 7;70(1):127-133. Epub 2017 Dec 7.

Tel Aviv University, Tel Aviv, Israel.

Objective: Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested as recurrent serosal inflammation. An association between FMF and malignancy has not been evaluated. The aim of this study was to estimate cancer risk in a large cohort of FMF patients from a single institution. Read More

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January 2018

Colchicine intoxication in familial Mediterranean fever patients using clarithromycin for the treatment of Helicobacter pylori: a series of six patients.

Rheumatol Int 2018 Jan 3;38(1):141-147. Epub 2017 Oct 3.

Department of Medicine F, Sheba Medical Center, Tel Hashomer, 5265601, Ramat Gan, Israel.

Familial Mediterranean fever is a hereditary disease, characterized by recurrent episodes of inflammation. Colchicine, the mainstay of therapy, is administered continuously to all diagnosed FMF patients. Drug-drug interaction between colchicine and clarithromycin, resulting in colchicine intoxication, has been noted, mainly in association with gout and pneumonia. Read More

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January 2018

Carriage of Mediterranean Fever (MEFV) Mutations in Patients with Postpericardiotomy Syndrome (PPS).

Isr Med Assoc J 2017 Sep;19(9):562-565

Department of Internal Medicine F, Sheba Medical Center, Tel Hashomer, Israel.

Background: Postpericardiotomy syndrome (PPS) is characterized by pleuro-pericardial inflammation, which occurs in patients undergoing surgical procedures involving the pleura, pericardium, or both. The syndrome is considered to be immune mediated. However, its pathogenesis is not fully understood. Read More

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September 2017

Familial Mediterranean Fever Is Associated With Increased Mortality After Kidney Transplantation-A 19 Years' Single Center Experience.

Transplantation 2017 10;101(10):2621-2626

1 Department of Internal Medicine B, Rabin Medical Center, Beilinson Campus, Petah-Tikva, Israel. 2 Department of Nephrology and Hypertension, Rabin Medical Center, Beilinson Campus, Petah-Tikva, Israel. 3 Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel. 4 Department of Transplantation, Rabin Medical Center, Beilinson Campus, Petah- Tikva, Israel. 5 Department of Medicine and Heller Institute of Medical Research, Sehba Medical Center, Ramat-Gan, Israel.

Background: Current data regarding the outcome of kidney transplantation in patients with familial Mediterranean fever (FMF) who reach end-stage renal disease (ESRD) due to reactive amyloidosis A (AA) are scarce and inconclusive.

Methods: The outcomes of 20 patients with FMF and biopsy-proven AA amyloidosis that were transplanted between 1995 and 2014 were compared with 82 control patients (32 with diabetes mellitus and 50 with nondiabetic kidney disease). Major outcome data included overall patient and graft survivals. Read More

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October 2017

Recommendations for the inclusion of Fabry disease as a rare febrile condition in existing algorithms for fever of unknown origin.

Intern Emerg Med 2017 Oct 19;12(7):1059-1067. Epub 2017 Jul 19.

Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy.

Fever of unknown origin (FUO) is a rather rare clinical syndrome representing a major diagnostic challenge. The occurrence of more than three febrile attacks with fever-free intervals of variable duration during 6 months of observation has recently been proposed as a subcategory of FUO, Recurrent FUO (RFUO). A substantial number of patients with RFUO have auto-inflammatory genetic fevers, but many patients remain undiagnosed. Read More

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October 2017

Reply.

Arthritis Rheumatol 2017 09 21;69(9):1914. Epub 2017 Jul 21.

Sheba Medical Center at Tel-Hashomer and Tel-Aviv University Tel-Aviv, Israel.

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September 2017

[POST COLONOSCOPY AND POLYPECTOMY ENDOCARDITIS - A CASE CHALLENGING THE CURRENT AMERICAN GUIDELINES FOR ANTIMICROBIAL PROPHYLAXIS].

Harefuah 2016 Nov;155(11):650-652

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Introduction: Short text: Click here for article written by Naama Rappoport, Neta Gotlieb, Olga Feld, Avi Livneh HAREFUAH 2016: 155: November: 637-641. Read More

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November 2016