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Thrombotic microangiopathy

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https://www.readbyqxmd.com/read/29778558/successful-kidney-transplant-with-eculizumab-thymoglobulin-and-belatacept-therapy-in-a-highly-sensitised-patient-with-atypical-haemolytic-uraemic-syndrome-due-to-factor-h-mutation
#1
John Fredy Nieto-Ríos, Mónica Zuluaga-Quintero, Diana Carolina Bello-Márquez, Arbey Aristizabal-Alzate, Catalina Ocampo-Kohn, Lina María Serna-Higuita, Lina Arias, Gustavo Zuluaga-Valencia
Atypical haemolytic uremic syndrome is a disease caused by complement regulation abnormalities that generally progresses to chronic end-stage renal disease with a high rate of recurrence in kidney transplantation and a high risk of graft loss. Anti-complement therapy has improved the prognosis of these patients, achieving disease remission in most cases, increasing the likelihood of a successful kidney transplant and increasing patient and graft survival. Drugs with low risk of induction of thrombotic microangiopathies such as belatacept and mycophenolate have also been used with satisfactory results...
May 16, 2018: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29777547/tacrolimus-induced-asymptomatic-thrombotic-microangiopathy-diagnosed-by-laboratory-tests-in-pig-to-rhesus-corneal-xenotransplantation-a-case-report
#2
Jong-Min Kim, Jaeyoung Kim, Se-Hyun Choi, Jun-Seop Shin, Byoung-Hoon Min, Won Young Jeong, Ga-Eul Lee, Min-Sun Kim, Seeun Kwon, Mee Kum Kim, Chung-Gyu Park
Tacrolimus-associated thrombotic microangiopathy (TA-TMA) is a rare complication. TA-TMA is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage due to thrombus. We report asymptomatic TA-TMA diagnosed by laboratory tests in pig-to-rhesus corneal xenotransplantation. Corneal transplantation had been conducted from a wild-type SNU miniature pig to a rhesus macaque. The veterinary records were retrospectively reviewed in this case. The immunosuppressive regimen consisted of rituximab, basiliximab, and IVIg as inductive therapies, and steroids with tacrolimus (0...
May 18, 2018: Xenotransplantation
https://www.readbyqxmd.com/read/29771991/progenitor-renin-lineage-cells-are-not-involved-in-the-regeneration-of-glomerular-endothelial-cells-during-experimental-renal-thrombotic-microangiopathy
#3
Leo Ruhnke, Jan Sradnick, Moath Al-Mekhlafi, Michael Gerlach, Florian Gembardt, Bernd Hohenstein, Vladimir T Todorov, Christian Hugo
Endothelial cells (EC) frequently undergo primary or secondary injury during kidney disease such as thrombotic microangiopathy or glomerulonephritis. Renin Lineage Cells (RLCs) serve as a progenitor cell niche after glomerular damage in the adult kidney. However, it is not clear whether RLCs also contribute to endothelial replenishment in the glomerulus following endothelial injury. Therefore, we investigated the role of RLCs as a potential progenitor niche for glomerular endothelial regeneration. We used an inducible tet-on triple-transgenic reporter strain mRen-rtTAm2/LC1/LacZ to pulse-label the renin-producing RLCs in adult mice...
2018: PloS One
https://www.readbyqxmd.com/read/29766468/gemcitabine-induced-thrombotic-microangiopathy-with-nephrotic-syndrome
#4
Daisuke Katagiri, Fumihiko Hinoshita
We encountered a case of gemcitabine (GEM)-induced secondary thrombotic microangiopathy (TMA) with nephrotic syndrome. Advanced pancreatic cancer with liver metastasis had originally been diagnosed. Renal biopsy showed focal reduplication of the glomerular basement membrane, endothelial cell swelling, and narrowed capillary lumens with fragmented erythrocytes and fibrin deposition, compatible with TMA. Regular monitoring of renal function during GEM treatment and discontinuation of treatment if acute kidney injury (AKI) might occur is crucial, because AKI combined with TMA is life-threatening...
May 15, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29766467/thrombotic-microangiopathy-on-kidney-biopsy-in-a-patient-with-tafro-syndrome
#5
Toshikazu Ozeki, Masashi Tsuji, Junichiro Yamamoto, Chisato Shigematsu, Shoichi Maruyama
TAFRO syndrome represents a characteristic constellation of symptoms comprising Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, and Organomegaly, and is considered to be a clinicopathologic variant of idiopathic multicentric Castleman disease. A 51-year-old woman was admitted to the hospital complaining of abdominal distension. Findings on physical examination were indicative of anasarca. Computed tomography revealed mild splenomegaly, pericardial effusion, pleural effusion, ascites, and paraaortic lymphadenopathy...
May 15, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29758394/impact-of-thrombotic-microangiopathy-on-renal-outcomes-and-survival-after-hematopoietic-stem-cell-transplantation
#6
Merve Postalcioglu, Haesook T Kim, Faruk Obut, Osman Arif Yilmam, Jiqiao Yang, Benjamin C Byun, Sophie Kupiec-Weglinski, Robert Soiffer, Jerome Ritz, Joseph H Antin, Edwin Alyea, John Koreth, Corey Cutler, Philippe Armand, Julie M Paik, David E Leaf, Vincent T Ho, Reza Abdi
Transplantation-associated thrombotic microangiopathy (TA-TMA) is a serious complication of hematopoietic stem cell transplantation (HSCT). We characterized the incidence, risk factors, and long-term outcomes associated with TA-TMA by performing a comprehensive review of all adult patients (n=1,990) undergoing allogeneic HSCT at the Dana Farber Cancer Institute/Brigham and Women's Hospital between 2005 and 2013. Using the City of Hope criteria, we identified 258 (13%) and 508 (26%) patients with "definite" and "probable" TMA, respectively...
May 11, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29753419/severe-renal-failure-and-thrombotic-microangiopathy-induced-by-malignant-hypertension-successfully-treated-with-spironolactone
#7
H M Villafuerte Ledesma, J M Peña Porta, P Iñigo Gil, P Martin Azara, J E Ruiz Lalglesia, A Tomás LaTorre, A Martínez Burillo, P Vernet Perna, R Álvarez Lipe
Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0...
May 9, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29744115/thrombotic-thrombocytopenic-purpura-possibly-triggered-by-graves-disease
#8
Saurabh D Chitnis, Tuoyo O Mene-Afejuku, Amandeep Aujla, Ahmed Shady, Gaby S Gil, Eder Hans Cativo, Andrea Popescu-Martinez
Thrombotic thrombocytopenic purpura (TTP) is a part of a spectrum of thrombotic microangiopathy syndromes which are mainly characterized by platelet aggregation causing microangiopathic hemolytic anemia, thrombocytopenia and microvascular occlusion. In literature, very few cases expressing a direct association between pre-existing Grave's disease and TTP have been described. A 37-year-old African-American woman with past medical history of Grave's disease and polysubstance abuse who presented with complaints of dyspnoea at rest and chest pain was diagnosed to have TTP on further evaluation...
October 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29743397/-transplantation-associated-thrombotic-microangiopathy-confirmed-by-renal-biopsy
#9
Satoshi Miyamoto, Shunsuke Kimura, Yosuke Hosoya, Daisuke Hasegawa, Hisashi Ishida, Atsuro Daida, Toshihiro Matsui, Yuri Yoshimoto, Shinsuke Hirabayashi, Takuya Fujimaru, Tadashi Kumamoto, Shin-Ichiro Mori, Koyu Suzuki, Atsushi Manabe
An eight-year-old girl with myelodysplastic syndrome (refractory cytopenia) received a bone marrow transplant (BMT) from an unrelated donor because of immunosuppressive therapy failure. Following administration of foscarnet for cytomegalovirus reactivation at day40 post-BMT, serum creatinine increased, and proteinuria, hematuria, and hypertension gradually exacerbated and became prolonged. However, neither schistocytosis nor other organ damage was evident. At six months post-BMT, renal biopsy revealed diffuse glomerular damage with glomerular lobulation, a double contour of the glomerular basement membrane, erythrocyte congestion and thrombi in the glomerular endocapillaries, and mesangiolysis, confirming the diagnosis of transplantation-associated thrombotic microangiopathy (TA-TMA)...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29741245/pathogenesis-and-diagnosis-of-disseminated-intravascular-coagulation
#10
REVIEW
M Levi
Several clinical conditions, in particular those associated with a systemic inflammatory response, can cause some degree of activation of coagulation but when the procoagulant stimulus is sufficiently severe and overcomes the natural anticoagulant mechanisms of coagulation, disseminated intravascular coagulation (DIC) may occur. The clinical manifestations of DIC encompass multiorgan dysfunction caused by fibrin-platelet clots in the microcirculation, and bleeding caused by consumption of platelets and coagulation factors...
May 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29739088/thrombotic-microangiopathy-like-hemolysis-in-vitamin-b12-deficiency-related-macrocytic-anemia
#11
Masayoshi Yamanishi, Shigeru Koba, Takaaki Jo, Tohru Kotera, Shinsaku Imashuku
BACKGROUND: Hemolytic features in patients with pernicious anemia have not been emphasized. METHODS: Seven Japanese patients at 60 - 88 years of age with vitamin B12 deficiency-related hemolytic anemia were assessed. RESULTS: Serum vitamin B12 levels in these cases were 46 - 89 pg/mL (normal reference range: 233 - 914 pg/mL). Clinically, the patients presented with thrombotic microangiopathy (TMA)-like hemolytic features (including macrocytic anemia, schistocytes on blood smears, high serum lactate dehydrogenase, hyperbilirubinemia, and low serum haptoglobin)...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29737462/validation-of-plasmic-score-and-follow-up-data-in-a-cohort-of-patients-with-suspected-microangiopathies-from-southern-italy
#12
Giovanni Luca Tiscia, Angelo Ostuni, Nicola Cascavilla, Filomena Cappucci, Potito Scalzulli, Cosima Battista, Antonio Abrescia, Filippo Aucella, Caterina Buquicchio, Maurizio Brigante, Giovanna D'Andrea, Bruno Di Paolo, Giulio Giordano, Barbara Infante, Silvia Piano, Prudenza Ranieri, Livio Tullo, Elvira Grandone
Severe ADAMTS13 deficiency (activity < 10%) is pathognomonic of thrombotic thrombocytopenic purpura. ADAMTS13 testing is time-consuming and unavailable in many hospitals. Recently, a seven-variables score named PLASMIC score, has been developed to stratify acute patients, based on their risk of having a severe ADAMTS13 deficiency. We present the application of this score in a cohort of patients referred to our Center. From 2012 to 2017, 42 patients with suspected thrombotic microangiopathies from 6 Centers were referred to Hemostasis and Thrombosis Center of "Casa Sollievo della Sofferenza" Hospital/Research Institute for ADAMTS13 testing...
May 8, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29732228/de-novo-atypical-haemolytic-uremic-syndrome-after-kidney-transplantation
#13
Arnaud Devresse, Martine de Meyer, Selda Aydin, Karin Dahan, Nada Kanaan
De novo thrombotic microangiopathy (TMA) can occur after kidney transplantation. An abnormality of the alternative pathway of complement must be suspected and searched for, even in presence of a secondary cause. We report the case of a 23-year-old female patient who was transplanted with a kidney from her mother for end-stage renal disease secondary to Hinman syndrome. Early after transplantation, she presented with 2 episodes of severe pyelonephritis, associated with acute kidney dysfunction and biological and histological features of TMA...
2018: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29730854/renal-involvement-in-antiphospholipid-syndrome
#14
REVIEW
Francisco Vileimar Andrade de Azevedo, Diego Germano Maia, Jozelio Freire de Carvalho, Carlos Ewerton Maia Rodrigues
This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries...
May 5, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29725655/iga-dominant-glomerulopathy-and-thrombotic-microangiopathy-after-chemotherapy
#15
Zeljko Dvanajscak, Bethany E Karl, Amber P Sanchez, Vighnesh Walavalkar
No abstract text is available yet for this article.
March 2018: KI Reports
https://www.readbyqxmd.com/read/29725629/acute-kidney-injury-in-pregnancy-the-changing-landscape-for-the-21st-century
#16
REVIEW
Swati Rao, Belinda Jim
Pregnancy-related acute kidney injury (Pr-AKI) remains a large public health problem, with decreasing incidences in developing countries but seemingly increasing incidences in the United States and Canada. These epidemiologic changes are reflective of the advances in medical and obstetric care, as well as changes in underlying maternal risk factors. The risk factors associated with advanced maternal age, such as hypertension, diabetes, chronic kidney disease, and those associated with reproductive technologies such as multiple gestations, are increasing...
March 2018: KI Reports
https://www.readbyqxmd.com/read/29717384/the-complement-alternative-pathway-and-preeclampsia
#17
REVIEW
Layan Alrahmani, Maria Alice V Willrich
PURPOSE OF REVIEW: Significant and intricate immune adaptations are essential for the establishment and maintenance of normal pregnancy. Preeclampsia is a morbid, potentially life-threatening disease for both mother and neonate that occurs uniquely in pregnancy, at least in part, due to maternal immune maladaptation. We aim to review the literature that focuses on case reports, diagnostic approaches, and treatment strategies for disorders of the complement alternative pathway (CAP) as related to preeclampsia...
May 1, 2018: Current Hypertension Reports
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#18
Sanjeev Sethi, S Vincent Rajkumar, Vivette D D'Agati
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage...
April 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29703488/clinical-characteristics-of-disseminated-intravascular-coagulation-in-patients-with-solid-and-hematological-cancers
#19
Marcel Levi
Malignant disease can be complicated by disseminated intravascular coagulation (DIC). DIC is defined as systemic intravascular triggering of coagulation (resulting in intravascular fibrin clot formation) and concurrent depletion of clotting factors and platelets (increasing the risk of hemorrhage). The clinical presentation of DIC in patients with cancer has usually a less fulminant presentation than DIC that may accompany other underlying disorders, such as sepsis and trauma. A more insidious, but also more protracted, diffuse activation of coagulation can proceed without any symptom...
April 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29703465/thrombotic-microangiopathy-in-cancer
#20
REVIEW
Ilene Ceil Weitz
Thrombotic microangiopathy (TMA) is clinical syndrome based on the presence of thrombocytopenia (platelet count <150 K or a reduction of the platelet count by >30% from baseline) accompanied by fragmentation hemolysis (MAHA) and evidence of organ damage. It can be seen in a variety of disorders including thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), shigatoxin related hemolytic uremic syndrome (STEC-HUS). Cancer itself has long been associated with both macro and microvascular thrombosis...
April 2018: Thrombosis Research
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