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https://www.readbyqxmd.com/read/28429383/altered-eigenvector-centrality-is-related-to-local-resting-state-network-functional-connectivity-in-patients-with-longstanding-type-1-diabetes-mellitus
#1
Eelco van Duinkerken, Menno M Schoonheim, Richard G IJzerman, Annette C Moll, Jesus Landeira-Fernandez, Martin Klein, Michaela Diamant, Frank J Snoek, Frederik Barkhof, Alle-Meije Wink
INTRODUCTION: Longstanding type 1 diabetes (T1DM) is associated with microangiopathy and poorer cognition. In the brain, T1DM is related to increased functional resting-state network (RSN) connectivity in patients without, which was decreased in patients with clinically evident microangiopathy. Subcortical structure seems affected in both patient groups. How these localized alterations affect the hierarchy of the functional network in T1DM is unknown. Eigenvector centrality mapping (ECM) and degree centrality are graph theoretical methods that allow determining the relative importance (ECM) and connectedness (degree centrality) of regions within the whole-brain network hierarchy...
April 21, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28428030/complement-inhibition-with-eculizumab-for-thrombotic-microangiopathy-rescues-a-living-donor-kidney-transplant-in-a-patient-with-antiphospholipid-antibody-syndrome
#2
Praveen Ramakrishnan Geethakumari, Patrick Mille, Rakesh Gulati, Srikanth Nagalla
Antiphospholipid antibody syndrome (APS) is an enigmatic heterogeneous disorder despite several revelations in its pathobiology. Renal transplantation in patients with APS has been notoriously difficult due to the high risk of development of thrombotic microangiopathy (TMA), which is often refractory to conventional treatment modalities such as aggressive anticoagulation and plasmapheresis. We describe a case of a 58-year-old male with secondary APS undergoing living unrelated renal transplantation for end-stage renal disease from lupus nephritis...
March 10, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28424147/treatment-of-leukoencephalopathy-with-calcifications-and-cysts-with-bevacizumab
#3
Alex J Fay, Allison A King, Joshua S Shimony, Yanick J Crow, Jan E Brunstrom-Hernandez
BACKGROUND: Leukoencephalopathy with calcifications and cysts is a rare, autosomal recessive cerebral microangiopathy that causes progressive white matter disease, calcifications, and cysts within the brain. It is typically associated with slowly progressive psychomotor regression, seizures, and movement disorders. Although leukoencephalopathy with calcifications and cysts affects only the central nervous system, it demonstrates remarkable neuropathologic and radiologic overlap with Coats plus, a disorder of small vessels of the brain, eyes, gastrointestinal tract, and bone...
March 23, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28417064/adenocarcinoma-of-the-lung-presenting-as-thrombotic-thrombocytopenic-purpura
#4
Ahmed Dirweesh, Waqas Siddiqui, Muhammad Khan, Praneet Iyer, Marc Seelagy
Acquired thrombotic thrombocytopenic purpura (TTP) usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. It occurs in approximately three in one million adults and 1 in 10 million children annually. The incidence is increased in females and blacks. Diagnosing TTP and initiating therapy with plasma exchange is a must to avoid preventable complications. Acquired thromotic microangiopathy has been linked to collagen vascular diseases, use of certain medications, organ transplants, infections, pregnancy and cancer...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#5
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416508/hus-and-atypical-hus
#6
T Sakari Jokiranta
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by shiga-toxin producing E. coli (STEC) infection, atypical HUS (aHUS), usually caused by uncontrolled complement activation, or secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical (i.e. STEC-HUS) follows a gastrointestinal infection with STEC, while aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416121/silent-coronary-heart-disease-in-patients-with-type-2-diabetes-application-of-a-screening-approach-in-a-follow-up-study
#7
Saula Vigili de Kreutzenberg, Anna Solini, Edoardo Vitolo, Alessandra Boi, Simonetta Bacci, Sara Cocozza, Rossella Nappo, Angela Rivellese, Angelo Avogaro, Marco Giorgio Baroni
AIMS: The cost-effectiveness of screening for silent coronary heart disease (CHD) in type 2 diabetes (DM2) is still debated. METHODS: We applied a diagnostic algorithm for silent CHD detection, in a cohort of 102 asymptomatic DM2 subjects (57±7years), attending 5 Italian outpatient clinics, to verify its predictive value. The risk of silent CHD was calculated considering classical risk factors, and presence of microangiopathy/macroangiopathy. Patients were divided in 3 groups, i...
April 4, 2017: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/28415160/a-first-case-of-high-flow-nasal-cannula-oxygen-therapy-in-patients-with-pulmonary-tumor-thrombotic-microangiopathy
#8
Hyonsoo Joo, Do-Hyun Na, Jaeho Seung, Tong Yoon Kim, Gi June Min, Chin Kook Rhee
No abstract text is available yet for this article.
April 18, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28401611/pegylated-bovine-carboxyhaemoglobin-utilisation-in-a-thrombotic-thrombocytopenic-purpura-patient
#9
C Sam, P Desai, D Laber, A Patel, N Visweshwar, M Jaglal
OBJECTIVE: To determine if pegylated bovine carboxyhaemoglobin can be utilised in a thrombotic thrombocytopenic purpura (TTP) patient. BACKGROUND: TTP is a condition characterized by thrombotic microangiopathy and has a high mortality rate when left untreated. Therapeutic plasma exchange is well established as the most effective and evidence-based treatment of TTP. METHODS: The ability to administer plasma exchange therapy is limited in Jehovah's Witnesses who decline blood products due to religious beliefs...
April 12, 2017: Transfusion Medicine
https://www.readbyqxmd.com/read/28393653/anti-pentraxin-3-auto-antibodies-might-be-protective-in-lupus-nephritis-a-large-cohort-study
#10
Mo Yuan, Ying Tan, Yun Pang, Yong-Zhe Li, Yan Song, Feng Yu, Ming-Hui Zhao
OBJECTIVES: Anti-pentraxin 3 (PTX3) auto-antibodies were found to be associated with the absence of renal involvement in systemic lupus erythematosus (SLE). This study is to investigate the prevalence of anti-PTX3 auto-antibodies and their clinical significance based on a large Chinese lupus nephritis cohort. METHODS: One hundred and ninety-six active lupus nephritis patients, 150 SLE patients without clinical renal involvement, and 100 healthy controls were enrolled...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28390986/pericardial-effusion-following-hematopoietic-cell-transplantation-in-children-is-associated-with-increased-risk-of-mortality
#11
Kelly Cox, Rajesh Punn, Elizabeth Schnorr, Benjamin A Pinsky, Sandhya Kharbanda
Hematopoietic cell transplantation (HCT) is curative for many pediatric malignant and non-malignant disorders, but is associated with significant morbidity and mortality, including development of pericardial effusion (PEF). We report the results of a retrospective chart review performed to assess the incidence, risk factors and prognostic effect of PEF in the pediatric HCT patients at Lucile Packard Children's Hospital (LPCH), Stanford University. One hundred and nineteen patients receiving HCT between January of 2010 through December of 2013 were selected through the LPCH Pediatric Stem Cell Transplant Program database...
April 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#12
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28380082/heparin-induced-thrombocytopenia-as-a-cause-of-prolonged-low-platelet-count-in-a-patient-with-thrombotic-thrombocytopenic-purpura-treated-with-plasmapheresis
#13
Agata Winiarska, Norbert Kwella, Tomasz Stompór
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder belonging to thrombotic microangiopathies (TMA) and is caused by functional deficiency of the ADAMTS-13 metalloproteinase. Plasma exchange (PE) remains the treatment of choice in this disease. Here, were describe the case of a patient who apparently recovered from TTP following multiple sessions of PE, but remained thrombocytopenic. Careful analysis revealed the development of heparin-induced thrombocytopenia (HIT) that precluded platelet count (PLT) normalization...
April 5, 2017: Acta Biochimica Polonica
https://www.readbyqxmd.com/read/28370442/degos-disease-not-just-a-scar-lethal-outcome-inspite-of-immunomodulatory-therapy
#14
Calderón-Castrat Ximena, Yuste-Chaves Manuela, Hernández Arantza, Santos-Briz Ángel, Fernández-López Emilia
Degos disease (DD) or papulosis atrophicans maligna, is an extremely infrequent thrombotic microangiopathy of unknown etiology((1)) . Clinically, it is characterized by small erythematous papules with an atrophic center that may simulate a simple scar and distinct histopathological feature that is not always present. Due to the rarity of this entity, diagnosis may go unnoticed with the first onset of cutaneous lesions. Therefore, high clinical suspicion and adequate clinicopathological correlation are required to establish an early diagnosis and consequently treatment...
March 29, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28368376/predictors-of-overall-survival-among-patients-treated-with-sirolimus-tacrolimus-vs-methotrexate-tacrolimus-for-gvhd-prevention
#15
F Khimani, J Kim, L Chen, E Dean, V Rizk, B Betts, T Nishihori, F Locke, A Mishra, L Perez, E Ayala, M Kharfan-Dabaja, M Nieder, H Fernandez, C Anasetti, J Pidala
Sirolimus (SIR)/tacrolimus (TAC) is an alternative to methotrexate (MTX)/TAC. However, rational selection among these GvHD prophylaxis approaches to optimize survival of individual patients is not possible based on current evidence. We compared SIR/TAC (n=293) to MTX/TAC (n=414). The primary objective was to identify unique predictors of overall survival (OS). Secondary objective was to compare acute and chronic GvHD, relapse, non-relapse mortality, thrombotic microangiopathy (TMA), hepatic veno-occlusive disease (VOD/SOS), and acute kidney injury...
April 3, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28357636/probable-catastrophic-antiphospholipid-syndrome-with-intracerebral-hemorrhage-secondary-to-epstein-barr-viral-infection
#16
Mark P Plummer, Adam M H Young, Ronan O'Leary, Maxwell S Damian, Andrea Lavinio
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe variant of antiphospholipid syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein-Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management. METHODS: All data are from a single patient admitted to the Neurosciences Critical Care Unit in Addenbrooke's Hospital, Cambridge, in March 2016...
March 29, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28357565/anti-glomerular-basement-membrane-glomerulonephritis-with-thrombotic-microangiopathy-a-case-report
#17
Xiao-Juan Yu, Sha-Sha Han, Su-Xia Wang, Xiao-Yu Jia, Wei-Yi Guo, Zhao Cui, Feng Yu, Fu-de Zhou, Ming-Hui Zhao
No abstract text is available yet for this article.
March 29, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28356675/thrombotic-microangiopathy-due-to-viperidae-bite-two-case-reports
#18
T Dineshkumar, J Dhanapriya, R Sakthirajan, K Thirumalvalavan, A A Kurien, T Balasubramaniyan, N Gopalakrishnan
Snake bite is mainly an occupational hazard and causes serious health problems in rural India. Acute kidney injury (AKI) occurs in 5-30% cases. Renal pathologic findings include acute tubular necrosis, cortical necrosis, interstitial nephritis, glomerulonephritis, and vasculitis. Thrombotic microangiopathy (TMA) occurrence after a snake bite is reported rarely. Here, we present two patients who developed TMA after viper bite treated with hemodialysis and plasmapheresis. Renal biopsy showed fibrin thrombi in glomeruli and arterioles with cortical necrosis...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356668/targeting-renin-angiotensin-system-in-malignant-hypertension-in-atypical-hemolytic-uremic-syndrome
#19
V Raghunathan, S K Sethi, M A Dragon-Durey, M Dhaliwal, R Raina, P Jha, S B Bansal, V Kher
Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356666/acyclovir-induced-thrombotic-microangiopathy
#20
R Goli, K K Mukku, S B R Devaraju, M S Uppin
Acyclovir is a commonly used antiviral drug. Acute kidney injury (AKI) due to intratubular crystal precipitation and interstitial nephritis is well known. Here we present a case of acyclovir induced AKI in a 61 year old male with herpes zoster, which presented like thrombotic microangiopathy with acute interstitial nephritis. This is the first case report on acyclovir causing thrombotic microaniopathy with partial improvement in renal function after plasmapharesis.
March 2017: Indian Journal of Nephrology
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