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cryoglobulinemia

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https://www.readbyqxmd.com/read/28543916/hcv-rna-genomic-sequences-and-hcv-e2-glycoprotein-in-sural-nerve-biopsies-from-hcv-infected-patients-with-peripheral-neuropathy
#1
Sabino Russi, Domenico Sansonno, Salvatore Monaco, Sara Mariotto, Sergio Ferrari, Fabio Pavone, Gianfranco Lauletta, Franco Dammacco
AIMS: Peripheral neuropathy (PN), the major neurological complication of chronic HCV infection, is frequently associated with mixed cryoglobulinemia (MC) and small-vessel systemic vasculitis. While humoral and cell-mediated immune mechanisms are suspected to act together in an aberrant immune response that results in peripheral nerve damage, the role of HCV remains largely speculative. The possible demonstration of HCV in peripheral nerve tissue would obviously assume important pathogenic implications...
May 24, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28538208/notch4-and-mhc%C3%A2-class-ii-polymorphisms-are-associated-with%C3%A2-hcv-related-benign-and-malignant-lymphoproliferative-diseases
#2
Laura Gragnani, Elisa Fognani, Valli De Re, Massimo Libra, Adriana Garozzo, Patrizio Caini, Guia Cerretelli, Andrea Giovannelli, Serena Lorini, Monica Monti, Silvia Bagnoli, Irene Piaceri, Anna Linda Zignego
Mixed cryoglobulinemia (MC), is a HCV-related, clinically benign, lymphoproliferative disorder (LPD) that may evolve into a non Hodgkin's lymphoma (NHL). Significant associations were found between two single nucleotide polymorphisms near NOTCH4 (rs2071286) and the HLA class II (rs9461776) genes and HCV-related MC syndrome (MCS). We analyzed NOTCH4 rs2071286 and HLA-II rs9461776 in 3 HCV-related LPD groups (asymptomatic MC, MCS, NHL) with HCV infection without lymphoproliferative disorders.We found a positive relationship between NOTCH4 rs207186 T minor allele frequency (MAF) and patients with HCV-related LPDs at risk of NHL (Chi-square test for trend = 14...
March 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28536920/clinicopathological-features-of-multiple-mononeuropathy-associated-with-systemic-lupus%C3%A2-erythematosus-a-multicenter-study
#3
Elodie Rivière, Fleur Cohen Aubart, Thierry Maisonobe, François Maurier, Christophe Richez, Bruno Gombert, Marie Gousseff, Daniel Adoue, Alexis Mathian, Miguel Hié, Julien Haroche, Zahir Amoura
Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients receiving a diagnosis of MM during SLE. Ten patients were included (8 women and 2 men, median age at MM diagnosis: 40.4 years). SLE was diagnosed before MM in 9/10 patients (median time 8.2 years)...
May 23, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28533861/-cutaneous-leukocytoclastic-vasculitis-about-85-cases
#4
Amina Aounallah, Aicha Arouss, Najet Ghariani, Wafa Saidi, Badreddine Sriha, Mohamed Denguezli, Colandane Belajouza, Rafia Nouira
Clinical manifestation, etiology and outcome of leukocytoclastic vasculitis are little studied. The aim of our study was to examine epidemiological, clinical etiological, and evolutionary characteristics of this entity. We conducted a cross-sectional data collection from medical records of 85 patients with leukocytoclastic vasculitis in the Department of Dermatology at the Farhat Hached University Hospital, Sousse between January 2000 and December 2013. Epidemiological, clinical, paraclinical, etiological data sheets had been completed for each patient...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28520564/normothermic-cardiopulmonary-bypass-in-patient-with-waldenstr%C3%A3-m-s-macroglobulinemia-and-cryoglobulinemia-a-case-report
#5
Shiho Satomi, Asuka Kasai, Eisuke Hamaguchi, Yasuo M Tsutsumi, Katsuya Tanaka
Waldenström's macroglobulinemia (WM) manifests as hyperviscosity syndrome. Cryoglobulinemia, which may increase blood viscosity or induce thrombosis in association with decreased body temperature, can occur in combination with WM. We describe the management of an 82-year-old woman with WM, hyperviscosity syndrome, and cryoglobulinemia who required open aortic valve replacement. Decreased body temperature in this patient was prevented during cardiopulmonary bypass by using a forced air warming system and normothermic cardioplegia with continuous warm blood cardioplegia perfusion...
May 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28514410/-cryoglobulinemic-vasculitis-in-chronic-hepatitis-c-genetic-aspects
#6
M G Artemova, D T Abdurakhmanov
Cryoglobulinemia (CG) is detected in more than 50% of patients with chronic hepatitis C (CHC); however, only 15-25% of them develop cryoglobulinemic vasculitis (CV) that is a systemic vasculitis due to the formation of immune deposits, which affects small (less than medium-sized) vessels and which is frequently fatal for the patient. The causes of CG only in some patients with CHC and the pathogenesis of CV remain unstudied; however, the accumulated data allow one to identify the special contribution of the patient's genetic factors to the development of the disease...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28509128/a-case-of-rapid-amelioration-of-hepatitis-c-virus-associated-cryoglobulinemic-membranoproliferative-glomerulonephritis-treated-by-interferon-free-directly-acting-antivirals-for-hcv-in-the-absence-of-immunosuppressant
#7
Fumiaki Obata, Taichi Murakami, Junko Miyagi, Sayo Ueda, Taizo Inagaki, Masanori Minato, Hiroyuki Ono, Kenji Nishimura, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Fumi Kishi, Seiji Kishi, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Toshio Doi
Mixed cryoglobulinemic syndrome, which is a systemic vasculitis characterized by the immune complex deposition in small- and medium-sized arteries and most often due to chronic hepatitis C virus (HCV) infection, sometimes clinically manifests as refractory glomerulonephritis or nephritic syndrome. Patients with mixed cryoglobulinemic nephropathy who have a rapidly progressive glomerulonephritis should receive immunosuppressive therapy. After disease stabilization, patients should receive concurrent therapy for the underlying HCV infection...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#8
REVIEW
Predrag Ostojic, Ivan R Jeremic
Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28507188/cryoglobulinemia-in-sj%C3%A3-gren-syndrome-a-disease-subset-that-links-higher-systemic-disease-activity-autoimmunity-and-local-b-cell-proliferation-in-mucosa-associated-lymphoid-tissue
#9
Luca Quartuccio, Chiara Baldini, Roberta Priori, Elena Bartoloni, Francesco Carubbi, Alessia Alunno, Saviana Gandolfo, Serena Colafrancesco, Roberto Giacomelli, Roberto Gerli, Guido Valesini, Stefano Bombardieri, Salvatore De Vita
OBJECTIVE: To compare systemic disease activity by validated tools, i.e., the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (ESSDAI) and the Clinical ESSDAI (ClinESSDAI) scores, between primary Sjögren syndrome (pSS) with positive serum cryoglobulins and pSS without serum cryoglobulins. METHODS: There were 825 consecutive patients with pSS who were retrospectively evaluated. RESULTS: The ESSDAI and the ClinESSDAI scores were significantly higher in cryoglobulin-positive patients (p < 0...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28507081/reversion-of-anergy-signatures-in-clonal-cd21low-b-cells-of-mixed-cryoglobulinemia-after-clearance-of-hcv-viremia
#10
Martina Del Padre, Laura Todi, Milica Mitrevski, Ramona Marrapodi, Stefania Colantuono, Massimo Fiorilli, Milvia Casato, Marcella Visentini
Hepatitis C virus (HCV) causes mixed cryoglobulinemia (MC) by driving clonal expansion of IgM(+)CD27(+) B cells. These cells display both the features of anergy induced by continual engagement of the B cell receptor (BCR), such as high expression of phosphorylated extracellular signal regulated kinase (pERK) and reduced lifespan, and of virus-specific exhaustion such as CD21(low) phenotype and defective response to ligation of BCR and Toll-like receptor 9 (TLR9). Usually MC regresses after eradication of HCV with interferon, whose immunomodulatory activity might contribute to this effect...
May 15, 2017: Blood
https://www.readbyqxmd.com/read/28503888/cryoglobulinaemic-glomerulonephritis-artefacts-associated-with-laboratory-diagnosis-and-need-for-renal-biopsy
#11
Ajish Radhamma, Jobert Anjelo, Tony J Elias
Cryoglobulinemia as a cause of renal impairment is uncommon but needs to be considered in viral hepatitis and haematological malignancies. Often detection and estimation of cryoglobulins are confounded by collection and processing errors. This report highlights the need for stringent processing measures if the clinical suspicion is high.
May 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28490478/mixed-cryoglobulinemia-a-diagnostic-and-therapeutic-challenge
#12
Maria Túlio, Liliana Carvalho, Tiago Bana E Costa, Cristina Chagas
Mixed cryoglobulinemia is frequently secondary to hepatitis C virus infection. Diagnosis and therapeutic management are challenging, depending on the spectrum and severity of manifestations, as well as on the presence of comorbidities. We describe a case of a 79-year-old woman with a non-cirrhotic hepatitis C virus infection presenting with weakness, arthralgias, purpuric rash with left leg ulcerative lesions, bilateral peripheral sensorimotor polyneuropathy, renal impairment and cardiac failure. The investigation was compatible with a severe type II mixed cryoglobulinemia with multisystemic involvement, including a low-grade B cell lymphoma and concomitant intestinal tuberculosis...
May 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28476397/cryoglobulinemia-a-rare-cause-of-digital-gangrene-after-elective-foot-surgery
#13
Catherine L Churchill, James R Polowczyk
The development of gangrene after elective digital surgery is a rare complication. We present a case of a patient with normal preoperative vascular examination findings and a history significant for untreated hepatitis C. The patient underwent revisional second toe arthrodesis and plantar plate repair with pin fixation. After surgery, she developed gangrene and ultimately required partial digital amputation. The wound at the amputation site worsened postoperatively, warranting a rheumatologic evaluation that revealed previously undiagnosed cryoglobulinemia...
May 2017: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
https://www.readbyqxmd.com/read/28473046/mixed-essential-cryoglobulinemia
#14
Pratyaksha Sankhyan, Alexei Gonzalez-Estrada
No abstract text is available yet for this article.
May 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28470091/acute-cutaneous-necrosis-a-guide-to-early-diagnosis-and-treatment
#15
Karen Karimi, Ashika Odhav, Ramya Kollipara, Jesse Fike, Carol Stanford, John C Hall
Acute cutaneous necrosis is characterised by a wide range of aetiologies and is associated with significant morbidity and mortality, warranting complex considerations in management. Early recognition is imperative in diagnosis and management of sudden gangrenous changes in the skin. This review discusses major causes of cutaneous necrosis, examines the need for early assessment, and integrates techniques related to diagnosis and management. The literature, available via PubMed, on acute cutaneous necrotic syndromes was reviewed to summarise causes and synthesise appropriate treatment strategies to create a clinician's guide in the early diagnosis and management of acute cutaneous necrosis...
May 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28469862/myopericarditis-in-a-patient-with-hepatitis-c-and-cryoglobulinemic-renal-disease
#16
Mohamoud A Ali, Waqas Z Kayani, Bradley M Linzie, Gopal V Punjabi, James B Wetmore
Although cryoglobulinemia is a well-appreciated complication of hepatitis C (HC), myopericarditis with resulting pericardial effusion is extremely rare, especially in the absence of a liver transplant. In patients with HC, pericardial effusion with impending tamponade can be a florid and potentially life-threatening manifestation of multiorgan cryoglobulinemic disease.
May 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28467688/effectiveness-and-cost-of-hepatitis-c-virus-cryoglobulinemia-vasculitis-treatment-from-interferon-based-to-direct-acting-antivirals-era
#17
Patrice Cacoub, Mathieu Vautier, Anne Claire Desbois, Antoine Lafuma, David Saadoun
BACKGROUND: The net benefits of new hepatitis C virus (HCV) direct acting antiviral drugs (DAA) in patients with cryoglobulinemia vasculitis (CryoVas) are unknown. OBJECTIVE: To analyze the effectiveness and cost of all treatments used for HCV-CryoVas in the DAA versus pre-DAA era. METHODS: A chart review of all HCV-CryoVas patients who received antivirals from 1993 to 2016 in a tertiary center was performed. Treatment effectiveness was analyzed for clinical, immunological and virological responses...
May 3, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28454112/the-challenge-of-treating-hepatitis-c-virus-associated-cryoglobulinemic-vasculitis-in-the-era-of-anti-cd20-monoclonal-antibodies-and-direct-antiviral-agents
#18
REVIEW
Dario Roccatello, Savino Sciascia, Daniela Rossi, Laura Solfietti, Roberta Fenoglio, Elisa Menegatti, Simone Baldovino
Mixed cryoglobulinemia syndrome (MC) is a systemic vasculitis involving kidneys, joints, skin, and peripheral nerves. While many autoimmune, lymphoproliferative, and neoplastic disorders have been associated with this disorder, hepatitis C virus (HCV) is known to be the etiologic agent in the majority of patients. Therefore, clinical research has focused on anti-viral drugs and, more recently, on the new, highly potent Direct-acting Antiviral Agents (DAAs). These drugs assure sustained virologic response (SVR) rates >90%...
April 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28453396/pharmacogenetic-analysis-of-hepatitis-c-virus-related-mixed-cryoglobulinemia
#19
Jessica Cusato, Lucio Boglione, Amedeo De Nicolò, Chiara Simona Cardellino, Chiara Carcieri, Giuseppe Cariti, Giovanni Di Perri, Antonio D'Avolio
AIM: Mixed cryoglobulinemia (MC) is an extra hepatic hepatitis C virus related problem and different studies suggested genetics' role in predicting this complication. We evaluated the influence of SNPs in IL-28B, SLC29A1, SLC28A2, NT5C2, HNF4 and ABCB1 genes in MC prediction. PATIENTS & METHODS: SNPs were evaluated through real-time PCR. RESULTS:  ABCB1 (gene encoding P-glycoprotein) 3435C>T SNP was associated with MC presence (p = 0.034): related to T allele carriers (CC vs CT/TT), we reached a p-value of 0...
April 28, 2017: Pharmacogenomics
https://www.readbyqxmd.com/read/28390781/clinical-disorders-responsible-for-plasma-hyperviscosity-and-skin-complications
#20
REVIEW
Gregorio Caimi, Melania Carlisi, Caterina Urso, Rosalia Lo Presti, Eugenia Hopps
In this brief review, we have examined some clinical disorders which are associated to an altered hemorheological profile and at times accompanied by skin ulcers. This skin condition may be, in fact, observed in patients with primary plasma hyperviscosity such as multiple myeloma, Waldenstrom macroglobulinemia, cryoglobulinemia, cryofibrinogenemia, dysfibrinogenemia and connective tissue diseases. It must be underlined that the altered hemorheological pattern is not the only responsible for this skin complication but, as it worsens the microcirculatory flow, it contributes to determine the occurrence of the skin ulcers...
April 5, 2017: European Journal of Internal Medicine
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