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cryoglobulinemia

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https://www.readbyqxmd.com/read/29671215/a-patient-presenting-with-isolated-hematuria-and-renal-dysfunction-as-rare-manifestation-of-cryoglobulinemic-glomerulonephritis-in-the-course-of-autoimmune-diseases-including-sj%C3%A3-gren-s-syndrome
#1
Masaki Yamanaka, Yoshihide Fujigaki, Hajime Kono, Michito Nagura, Shigeyuki Arai, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis...
April 18, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29644207/through-the-lens-cryoglobulinemia
#2
Shekhar Neema, D Banerjee, S K Pramanik
No abstract text is available yet for this article.
March 2018: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29618689/-osteonecrosis-developing-after-rituximab-containing-chemotherapy-for-waldenstr%C3%A3-m-macroglobulinemia
#3
Fumihiko Nakamura, Risen Hirai, Takashi Higo, Tomiteru Togano, Rieko Sekine
A 71-year-old woman presented with fever, weight loss, and anemia because of recurrent Waldenström macroglobulinemia (WM) with cryoglobulinemia. Treatment with five cycles of doxorubicin, vincristine, cyclophosphamide, and prednisolone (CHOP) therapy was initiated, which resulted in insufficient improvement in anemia. Hence, a combination of rituximab and CHOP therapy was subsequently initiated. The patient complained of lumbago and lower leg pain on day 4 of the chemoimmunotherapy. X-ray findings for the affected sites were unremarkable, and the patient's symptoms gradually and spontaneously subsided...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29607961/a-patient-with-hcv-infection-and-a-sustained-virological-response-to-direct-acting-antiviral-treatment-who-developed-inclusion-body-myositis
#4
Toru Kuwano, Norio Akuta, Fumitaka Suzuki, Shunichiro Fujiyama, Yusuke Kawamura, Hitomi Sezaki, Tetsuya Hosaka, Satoshi Saitoh, Masahiro Kobayashi, Yoshiyuki Suzuki, Mariko Kobayashi, Yasuji Arase, Kenji Ikeda, Hiromitsu Kumada
We report the case of a 75-year-old woman who was found to have hepatitis C virus (HCV) infection in 1987. Before treatment in 2016, she was found to have mixed cryoglobulinemia (MC). Direct-acting antiviral (DAA) treatment produced a sustained virological response 12 (SVR12). She noticed gradual muscle weakness in 2015 and the gradual development of dysarthria and dysphagia in 2017. We performed a muscle biopsy that showed inclusion body myositis (IBM). To the best of our knowledge, this is first case of a patient with HCV infection, MC, and IBM, in which MC and IBM did not improve after an SVR12 was obtained by DAA treatment...
March 30, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29601939/dec1-stra13-is-a-key-negative-regulator-of-activation-induced-proliferation-of-human-b-cells-highly-expressed-in-anergic-cells
#5
Alessandro Camponeschi, Laura Todi, Cristina Cristofoletti, Cristina Lazzeri, Maurizio Carbonari, Milica Mitrevski, Ramona Marrapodi, Martina Del Padre, Massimo Fiorilli, Milvia Casato, Marcella Visentini
The transcription factor DEC1/STRA13 (also known as BHLHE40 and SHARP2) is involved in a number of processes including inhibition of cell proliferation and delay of cell cycle, and is a negative regulator of B cell activation and development in mice. We show here that, unlike in mice, DEC1/STRA13 expression is induced in human naïve and memory resting B cells by activation through the B-cell receptor (BCR) or Toll-like receptor 9 (TLR9). siRNA silencing of DEC1/STRA13 increases the capacity of activated B cells to perform a high number of divisions after TLR9 ligation...
March 27, 2018: Immunology Letters
https://www.readbyqxmd.com/read/29582966/-extra-hepatic-manifestations-associated-with-hepatitis-and-virus-infection
#6
Luca Piscitani, Maria Olimpia Longo, Xhensila Grabocka, Simona Silvestri, Marta Arazzi, Alessandro Pezzutto, Gabriella Micioni, Giorgia Di Fulvio, Lorenzo Ottavio Di Pietro, Alessandra Schiazza, Roberto Di Vito, Mario Bonomini
Hepatitis E virus (HEV) is a significant public health problem that affects almost 20 million individuals annually and cause acute liver injury in 3,5 million. Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestation. The spectrum of these manifestation is still emerging. Acute pancreatitis and neurological, renal, hematologic, and muscoloskeletal manifestations have been described. Renal injury include membranoproliferative glomerulonephritis with or without cryoglobulinemia, membranous glomerulonephritis and tubular necrosis...
March 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29560002/a-rare-case-of-eosinophilic-granulomatosis-with-polyangiitis-associated-with-cryoglobulinemia-presenting-with-a-bullous-skin-eruption-of-the-lower-limbs
#7
D D K Abeyaratne, C Liyanapathirana, C L Fonseka, P W M C S B Wijekoon
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29558353/-cryoglobulins-and-cryoglobulinemic-vasculitis
#8
Dorota Suszek, Maria Majdan
Cryoglobulinemia is defined as the presence of cryoglobulins in the blood. Cryoglobulinemia is often observed in the course of many diseases (infection, hematological disorders, autoimmune disorders) or has an idiopathic character. The classification of cryoglobulinemia is based on the immunological analysis of cryoglobulins and the activity of the rheumatoid factor (RF). The presence of cryoglobulins may induce cryoglobulinemic vasculitis (CV) which manifests with skin changes, arthritis and the dysfunction of internal organs...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29545888/risk-factors-for-new-onset-chronic-kidney-disease-in-patients-who-have-received-a-liver-transplant
#9
Yuehong Li, Binbin Li, Wei Wang, Jiaxuan Lv
The aim of the present study was to analyze the risk factors for new-onset chronic kidney disease (CKD) in patients who have received a liver transplant. A total of 190 patients who underwent liver transplantation between March 2001 and January 2015 were followed up, and analyzed retrospectively. Sex, age, primary disease, preoperative laboratory findings (hemoglobin, albumin, creatinine and glomerular filtration rate), surgical approach, blood loss during the surgery and transfusion volume, postoperative complications, and the average levels of calcineurin inhibitors (CNIs) (from liver transplantation to the onset of CKD) were analyzed...
April 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29545130/-renal-involvement-of-cryoglobulinemia
#10
Alexandre Karras
Cryoglobulins are immunoglobulins that undergo reversible precipitation at low temperatures. They can induce systemic vasculitis, characterized by purpuric cutaneous lesions, arthritis, peripheral neuropathy, hypocomplementemia and glomerular disease. Renal pathology reveals membranoproliferative glomerulonephritis, with particularly intense mesangial cell proliferation and infiltration by macrophages, associated with intracapillary thrombi. This renal disease presents as a nephritic syndrome, with heavy proteinuria, haematuria severe hypertension and rapidly progressive kidney failure that can lead to end-stage renal disease...
March 12, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29526955/improvement-of-proteinuria-due-to-combination-therapy-with-daclatasvir-and-asunaprevir-in-hepatitis-c-virus-associated-renal-disease-without-cryoglobulinemia-a-case-report
#11
Satoshi Takakusagi, Ken Sato, Yuhei Suzuki, Yuichi Yamazaki, Takashi Kosone, Satoru Kakizaki, Motoyasu Kusano, Hitoshi Takagi
We herein report a unique case of hepatitis C virus (HCV)-associated renal disease without cryoglobulinemia that showed proteinuria, hypoproteinemia, ascites, and edema. Due to combination therapy with daclatasvir and asunaprevir, the patient achieved sustained virological response at week 24 of the therapy. Furthermore, the therapy caused marked amelioration of her proteinuria, ascites, edema, and hypoalbuminemia, and finally improved her estimated glomerular filtration rate. There were no adverse events, and the combination therapy was well-tolerated...
March 9, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29523691/structure-function-analyses-of-a-stereotypic-rheumatoid-factor-unravel-the-structural-basis-for-germline-encoded-antibody-autoreactivity
#12
Mitsunori Shiroishi, Yuji Ito, Kenta Shimokawa, Jae Man Lee, Takahiro Kusakabe, Tadashi Ueda
Rheumatoid factors (RFs) are autoantibodies against the fragment-crystallizable (Fc) region of immunoglobulin G (IgG). In individuals with hematological diseases such as cryoglobulinemia and certain B-cell lymphoma forms, the RFs derived from specific heavy- and light-chain germline pairs, so-called "stereotypic RFs," are frequently produced in copious amounts and form immune complexes with IgG in serum. Of note, many structural details of the antigen-recognition mechanisms in RFs are unclear. Here, we report the crystal structure of the RF YES8c derived from the IGHV1-69/IGKV3-20 germline pair, the commonest of the stereotypic RFs, in complex with human IgG1-Fc at 2...
March 9, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29478253/blood-vessel-occlusion-with-erythrocyte-aggregates-causes-burn-injury-progression-microvasculature-dilation-as-a-possible-therapy
#13
Richard A F Clark, Justine Fenner, Arielle Sasson, Steve A McClain, Adam J Singer, Marcia G Tonnesen
Other than radiation, caustic strong alkali insults, and massive over-reactive inflammation (pyoderma gangrenosum), injury progression after trauma is usually secondary to ischemia from decreased blood vessel perfusion. Impeded blood flow contributes to injury progression in a variety of disorders including venous stasis ulcers, arterial ulcers, diabetic ulcers, flap and graft necrosis, sickle cell disease, cryoglobulinemia and other vasculopathies, vasculitis, post-myocardial infarction, and post stroke. The cause of impeded blood flow in these disorders/diseases is well known...
February 25, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29477943/the-use-of-emergency-apheresis-in-the-management-of-plasma-cell-disorders
#14
REVIEW
Sevgi Kalayoglu-Besisik
Hyperviscosity syndrome (HVS) develops most commonly in Waldenström's macroglobulinemia (WM) and multiple myeloma (MM). Plasmapheresis is the immediate therapy and very effective at relieving symptoms by removing paraprotein. The most commonly used replacement fluid is 4%-5% human albumin in physiologic saline. FFP may be used in patients with coagulation abnormalities. Plasmapheresis should be continued until acute symptoms abate. Hyperviscosity impairs the circulation in the retina and causes hemorrhages around the small retinal vessels...
February 20, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29469770/interstitial-granulomatous-dermatitis-in-a-patient-with-chronic-hepatitis-c-and-mixed-cryoglobulinemia
#15
M Rato, F Gil, A F Monteiro, J Aranha, E Tavares
A 55-year-old man presented with a history of asymptomatic, bilateral, figurate dermatosis consisting of erythematous, annular, linear, andarciform, indurated nodules and plaques on the lateral walls of thorax, flanks, and hypogastrium. His medical history was positive for chronic hepatitis C. Skin biopsy was compatible with the diagnosisof interstitial granulomatous dermatitis. Additionalinvestigation revealed positive rheumatoid factor, antinuclear antibodies, and cryoglobulins. Thepatient started treatment with high-potency topical corticosteroid...
January 15, 2018: Dermatology Online Journal
https://www.readbyqxmd.com/read/29445495/rituximab-therapy-for-hepatitis-c-virus-associated-cryoglobulinemic-membranoproliferative-glomerulonephritis
#16
Abhilash Koratala, Xu Zeng
Membranoproliferative glomerulonephritis associated with mixed cryoglobulinemia is the most common form of kidney disease observed in relation to hepatitis C virus (HCV) infection. Rituximab, a monoclonal antibody against CD20, is an effective treatment for severe and/or refractory HCV-related vasculitis and may evade the need for dialysis as in our patient.
February 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29419915/skin-manifestations-in-patients-with-hcv-related-chronic-liver-disease
#17
Rosanna Satta, Giovanni Mario Pes, Bianca Maria Quarta Colosso, Maria Pina Dore
Hepatitis C virus (HCV) is able to trigger both hepatic and extra-hepatic manifestations (1,2). Among the latter, the most investigated is mixed cryoglobulinemia vasculitis (MCV), especially of type II (3, 4), usually detected in 40-60% of patients chronically infected with enhanced the risk to develop lymphomas (5). Autoimmune disorders including thyroid disease, and diabetes mellitus have also been associated with chronic HCV infection (6). Skin manifestations may occur in up to 17% of HCV positive patients (7) and are the result of the immuno-mediated damage induced by the virus itself, and/or the associated liver injury (8)...
February 8, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29386839/intensely-pruritic-papules-and-plaques-in-waldenstrom-s-macroglobulinemia
#18
Solam Lee, Noo Ri Lee, Sung Jay Choe, Beom Jun Kim, Minseob Eom, Eung Ho Choi
Waldenstrom's macroglobulinemia (WM) is lymphoplasmacytoid malignancy that affects B lymphocytes. Cutaneous involvement of WM is rare, but various cutaneous manifestations have been reported. These findings are due to various pathological processes including direct invasion of tumor cells into the skin, deposition of paraproteins, hyperviscosity syndrome, and cryoglobulinemia. A 64-year-old man presented with a 10-day history of pruritic erythematous papules and plaques on his trunk and elbows. The clinical features were suspicious for eczematous dermatitis...
February 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29370761/hepatitis-c-related-cryoglobulinemic-neuropathy-potential-role-of-oxcarbazepine-for-pain-control
#19
Rita Moretti, Paola Caruso, Matteo Dal Ben, Silvia Gazzin, Claudio Tiribelli
BACKGROUND: Peripheral neuropathy is one most common, limiting and invalidating neurological symptom in subjects with hepatitis C virus and mixed cryoglobulinemia. Notably, the medical therapy proposed to eradicate HCV, can frequently exacerbate the painful neuropathy. Therefore, neuropathy therapies are insufficient and inadequate, and comprise immunosuppressive drugs, such as steroid or cyclosporine, intravenous immunoglobulin or plasma exchange. These have shown variable success in case reports, with a presumably temporary effect, but with major side effects...
January 25, 2018: BMC Gastroenterology
https://www.readbyqxmd.com/read/29364334/extrahepatic-manifestations-associated-with-chronic-hepatitis-c-virus-infection
#20
A Flores-Chávez, J A Carrion, X Forns, M Ramos-Casals
Chronic hepatitis C virus (HCV) infection has been associated with both organ-specific and systemic autoimmune diseases, with cryoglobulinemia being the most frequent associated disease. Experimental, virologic, and clinical evidence have demon-strated a close association between HCV infection and some systemic autoimmune diseases, especially Sjögren's syndrome, but also rheumatoid arthritis and lupus. A higher prevalence of hematological processes has also been described in patients with HCV infection, including cytopenias and lymphoproliferative disorders (B-cell lymphoma)...
December 2017: Revista Española de Sanidad Penitenciaria
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