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Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
Yu-Min Shen
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange...
2016: Thrombosis Journal
Daniel W Abbott, Kenneth D Friedman, Matthew S Karafin
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic hemolytic anemia that requires emergent treatment with plasma exchange and is one of the most important conditions for which apheresis service professionals are consulted. Careful interpretation of initial laboratory values and the peripheral blood smear is a critical first step to determining the need for plasma exchange because other conditions can show deceptively similar red cell morphology, and ADAMTS13 levels are often not rapidly available...
September 28, 2016: Transfusion and Apheresis Science
Wilson Kc Leung, L Gao, Parco M Siu, Christopher Wk Lai
An explosion in global epidemic of type 2 diabetes mellitus poses major rise in cases with vascular endothelial dysfunction ranging from micro- (retinopathy, nephropathy and neuropathy) to macro-vascular (atherosclerosis and cardiomyopathy) conditions. Functional destruction of endothelium is regarded as an early event that lays the groundwork for the development of renal microangiopathy and subsequent clinical manifestation of nephropathic symptoms. Recent research has shed some light on the molecular mechanisms of type 2 diabetes-associated comorbidity of endothelial dysfunction and nephropathy...
October 17, 2016: Life Sciences
I Mancini, I Ricaño-Ponce, E Pappalardo, A Cairo, M M Gorski, G Casoli, B Ferrari, M Alberti, D Mikovic, M Noris, C Wijmenga, F Peyvandi
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor cleaving protease, ADAMTS13. Similarly to other autoimmune disorders, evidences of a genetic contribution have been reported, including the association of the human leukocyte antigen (HLA) class II complex with disease risk OBJECTIVE: To identify novel genetic risk factors in acquired TTP...
October 20, 2016: Journal of Thrombosis and Haemostasis: JTH
Mei-Rong Du, Gui-Xia Ju, Nian-Sheng Li, Jun-Lin Jiang
Microvascular complications are the leading causes of acquired blindness, end-stage renal failure, and varieties of neuropathy associated with diabetes. Asymmetric dimethylarginine (ADMA), an endogenous nitric oxide synthase inhibitor, is involved in endothelial dysfunction, oxidative stress, and inflammation associated with the progression of diabetic microvascular complications. Elevated ADMA has been detected in experimental animals and patients with diabetic microangiopathy like retinopathy, nephropathy, and neuropathy...
October 2016: Journal of Cardiovascular Pharmacology
Aneta Kruszec, Przemysław Kotyla
Systemic sclerosis (SSc) is coccenctive tissue disease characterized by progressive fibrosis of the skin and internal organs leading to their failure, autoimmune dysfunction and the morphological and functional changes in the blood vessels. Primary cardiac involvement is common for patients with SSc, and when it becomes clinically evident appears as a bad prognostic factor. There is increasing evidence that heart failure occurs as a result of repeated episodes of ischemia leading to focal irreversible myocardial fibrosis...
September 29, 2016: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
Donald M Arnold, Christopher J Patriquin, Ishac Nazy
No abstract text is available yet for this article.
October 17, 2016: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
Tsukasa Saito, Yuichiro Kawamura, Nobuyuki Sato, Naoyuki Hasebe
OBJECTIVE: Atrial fibrillation (AF) is a cardiac arrhythmia that does not infrequently induce ischemic strokes; however, little research has been reported on focal cerebral microangiopathic lesions in patients with AF. Recently cerebral microbleeds (CMBs) have been noticed for their potential implication in cerebral small vessel dis- ease. Therefore, we had 2 goals in the present study: (1) to compare the prevalence of CMBs in patients with AF with that in patients without AF, and (2) to prove that CMBs could be a clinical predictive factor for the development of future cerebral microangiopathy in patients with AF without a history of symptomatic cerebral infarction in a prospective manner...
September 2016: Journal of Hypertension
Pierre Paradis, Sofiane Ouerd, Noureddine Idris-Khodja, Michelle Trindade, Suellen C Coelho, Karin A Jandeleit-Dahm, Ernesto L Schiffrin
OBJECTIVE: The prognosis of type-1 diabetes remains poor and is primarily related to the increased risk of vascular complications. Overproduction of reactive oxygen species by NADPH oxidase (NOX) is believed to play an important role in diabetes-related vascular injury. NOX1 may play a role in the macrovascular disease, whereas NOX4 may have protective actions. Nevertheless, their role in diabetic microangiopathy is less well understood. We hypothesized that deletion of Nox1 would prevent diabetes-induced endothelial dysfunction and vascular remodeling of small arteries whereas Nox4 would exaggerate vascular injury...
September 2016: Journal of Hypertension
Kei Nakajima, Kaname Suwa
BACKGROUND: Numerous etiologies are implicated in the complications of diabetes. A link between diabetes and the predisposition to certain cancers, including colon cancer, has been established during the last decade. However, the relationship between fecal occult blood test (FOBT), a screening method used principally for detection of colon cancer, and diabetes has not been explored. METHODS: We investigated the association of positive FOBT with HbA1c of 5.70-6.49% (prediabetes) and HbA1c of ≥6...
October 15, 2016: Clinical Biochemistry
Omer Akyol, Sumeyya Akyol, Chu-Huang Chen
Endothelial cells (EC) respond to injury by releasing numerous factors, including von Willebrand factor (VWF). High circulating levels of unusually large VWF multimers (UL-VWFM) have strong procoagulant activity and facilitate platelet adhesion and aggregation by interacting with platelets after an acute event superimposed on peripheral arterial disease and coronary artery disease. ADAMTS13-a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13-regulates a key physiological process of coagulation in the circulation by cleaving VWF multimers into small, inactive fragments...
October 13, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
Jeffrey L Turner, Joshua Reardon, Tanios Bekaii-Saab, Spero R Cataland, Matthew J Arango
No abstract text is available yet for this article.
September 20, 2016: Clinical Colorectal Cancer
Fusun Gediz, Bahriye Kadriye Payzin, Sertac Ecemis, Naile Güler, Asu Fergun Yilmaz, Fusun Topcugil, Afig Berdeli
INTRODUCTION: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy, which develops as a result of defective activity of the alternative complement pathway and excessive complement activation due to genetic or acquired factors. No satisfactory responses were obtained by plasmapheresis, corticosteroids and fresh frozen plasma (FFP) transfusion. However, promising results are obtained in recent years by eculuzimab treatment, which inhibits C5 activation. OBJECTIVE: To evaluate the efficacy, safety and effect of eculizumab on quality of life of adult aHUS patients followed in our center...
September 30, 2016: Transfusion and Apheresis Science
Adrien Flahault, Marguerite Vignon, Marion Rabant, Aurélie Hummel, Laure-Hélène Noël, Danielle Canioni, Bertrand Knebelmann, Felipe Suarez, Khalil El Karoui
INTRODUCTION: We report the case of a multicentric Castleman disease (MCD) with initial renal involvement. Although the renal involvement in this case was typical of MCD, it constitutes a rare presentation of the disease, and in our case the renal manifestations led to the haematological diagnosis. CLINICAL FINDINGS/PATIENT CONCERNS: The patient was admitted for fever, diarrhea, anasarca, lymphadenopathies and acute renal failure. Despite intravenous rehydration using saline and albumin, renal function worsened and the patient required dialysis...
October 2016: Medicine (Baltimore)
Anton M Kolomeyer, Ken K Nischal, Ellen Mitchell
PURPOSE: To report a case of bilateral cavernous sinus thrombosis (CST) in a patient with tacrolimus-associated posttransplant thrombotic microangiopathy. METHODS: Case report. RESULTS: An 8-year-old boy with a medical history of orthotopic heart transplant, posttransplant lymphoproliferative disease, and recurrent infections was hospitalized for nausea, vomiting, and diarrhea. His ocular history included accommodative esotropia, hyperopia with astigmatism, Molluscum contagiosum lid lesions, and idiopathic intracranial hypertension...
October 13, 2016: European Journal of Ophthalmology
Ramachandran Rameshkumar, Sriram Krishnamurthy, Rajesh Nachiappa Ganesh, Subramanian Mahadevan, Parameswaran Narayanan, Ponnarmeni Satheesh, Puneet Jain
BACKGROUND: Septic acute kidney injury (AKI) accounts for more than half of all cases of AKI in critically ill children. The renal histology was found to alter the management in more than two-third of cases of adult acute renal failure. Better insight into the pathogenesis of pediatric septic AKI could be based on developing a clearer appreciation of the histopathological changes. No comprehensive study of the histopathological features of septic AKI in critically ill children has yet been performed...
October 13, 2016: Clinical and Experimental Nephrology
Daniel Croom, Heather Tracy
Thrombotic microangiopathy (TMA) syndromes represent a spectrum of illnesses that share common clinical and pathologic features of microangiopathic hemolytic anemia, thrombocytopenia, and organ injury from pathologic small-vessel thrombosis. At least nine primary TMA syndromes have been described and classified based on common probable etiologies, diagnostic criteria, and treatments. The most recognized of the TMA syndromes include thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS)...
2016: Journal of Special Operations Medicine: a Peer Reviewed Journal for SOF Medical Professionals
Priti Agarwal, C L Nawal, Deepak Sharma
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
T A Polyakova, A V Arablinsky, O S Levin
The role of the cerebral microbleeds (CMB) as a potential predictor of hemorrhagic complications of thrombolytic therapy is discussed. CMB are small perivascular hemosiderin deposits in the brain detected by magnetic resonance imaging in gradient echo T2* or SWI sequences. They are associated (along with leukoaraiosis or lacunes) with cerebral microangiopathies. Most often CMB are associated with hypertensive arteriopathy (the main cause of CMB localization) or cerebral amyloid angiopathy (the main cause of cerebral CMB)...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
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