WorldWideScience

Sample records for renal thrombotic microangiopathy

  1. Review of Thrombotic Microangiopathy (TMA, and Post- Renal Transplant TMA

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    Ardalan Mohammad

    2006-01-01

    Full Text Available Thrombotic microangiopathy (TMA is a rare but devastating disorder; it involves small vessels and is characterized by intravascular thrombi of aggregated platelets leading to thrombocytopenia and variable degrees of organ ischemia and anemia, which is due to erythrocyte fragmentation in microcirculation. Childhood cases with predominant renal involvement are referred as the hemolytic uremic syndrome (HUS, and adults with major central neurological involvement are labeled as thrombotic thrombocytopenia purpura (TTP. Endothelial damage due to toxins and/or lack of defense against complement activation have a central role. Recent discovery of the von Willebrand Factor cleaving protease (ADAMTS 13 has offered new insight into the pathogenesis of TMA. TMA is also a well-recognized serious complication of renal transplantation. Clinical features of intravascular hemolysis are not always found. It may occur as de novo or recurrent and the majority of de novo cases are related to cyclosporin therapy. Viral infections, severe renal ischemia and acute vascular rejection are less frequent causes. Recurrence is negligible in diarrhea-associated HUS in childhood, but non-diarrheal HUS recurs in majority of adults following renal transplantation. Renal transplantation is contraindicated in familial/relapsing recurrent forms of HUS.

  2. "Unusual Cause Of Tophi With Renal Thrombotic Microangiopathy".

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    Sahu, Kamal Kant; Law, Arjun Datt; Kumar, Ganesh; Dhir, Varun; Naseem, Shano; Nada, Ritambhra; Varma, Subhash Chander; Malhotra, Pankaj

    2016-06-01

    Chronic neutrophilic leukemia (CNL) is a rare entity amongst myeloproliferative neoplasms (MPNs). The classical presentation of CNL is with splenomegaly, mature neutrophilic leucocytosis and hyperuricemia. We herein report a case who presented with symptoms of acute gouty arthritis. Physical examination showed typical red, tender tophi in the right hand, right foot and both pinnae suggesting an acute episode of gout. During evaluation, moderate splenomegaly, mature neutrophilia, hyperuricemia and sub-nephrotic range range proteinuria were noted. Bone marrow examination and kidney biopsy was done. Final diagnosis of CNL with acute gouty arthritis and chronic renal thrombotic microangiopathy (TMA) was made. Although hyperuricemia is a common finding in MPNs but presentation of our case with symptoms of acute tophi and chronic TMA is atypical.

  3. [Thrombotic microangiopathies

    DEFF Research Database (Denmark)

    Nielsen, O.J.; Friis-Hansen, L.

    2009-01-01

    pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious...

  4. Combined pulmonary hypertension and renal thrombotic microangiopathy in cobalamin C deficiency.

    NARCIS (Netherlands)

    Komhoff, M.; Roofthooft, M.T.; Westra, D.; Teertstra, T.K.; Losito, A.; Kar, N.C.A.J. van de; Berger, R.M.

    2013-01-01

    Pulmonary arterial hypertension (PAH) and renal thrombotic microangiopathy (rTMA) are rare diseases in childhood, frequently leading to death and end-stage renal disease, respectively. Their combined occurrence has been reported anecdotally. We investigated the clinical, biochemical, and genetic

  5. Combined Pulmonary Hypertension and Renal Thrombotic Microangiopathy in Cobalamin C Deficiency

    NARCIS (Netherlands)

    Komhoff, Martin; Roofthooft, Marcus T.; Westra, Dineke; Teertstra, Thea K.; Losito, Attilio; de Kar, Nicole C. A. J. van; Berger, Rolf M. F.

    Pulmonary arterial hypertension (PAH) and renal thrombotic microangiopathy (rTMA) are rare diseases in childhood, frequently leading to death and end-stage renal disease, respectively. Their combined occurrence has been reported anecdotally. We investigated the clinical, biochemical, and genetic

  6. Renal thrombotic microangiopathy and FIP1L1/PDGFRα-associated myeloproliferative variant of hypereosinophilic syndrome.

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    Langlois, Anne Lyse; Shehwaro, Nathalie; Rondet, Claire; Benbrik, Youssef; Maloum, Karim; Gueutin, Victor; Rouvier, Philippe; Izzedine, Hassane

    2013-08-01

    We report a case of renal thrombotic microangiopathy (TMA) in a myeloproliferative variant of hypereosinophilic syndrome (HES) in a 24-year-old man which resolved with imatinib therapy. This is one of a few cases in the literature to date describing TMA in HES, suggesting that the pathogenesis of thrombosis is at least in part related to damage from activated eosinophils.

  7. Acyclovir-induced thrombotic microangiopathy

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    Goli, R.; Mukku, K. K.; Devaraju, S. B. R.; Uppin, M. S.

    2017-01-01

    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. PMID:28356666

  8. Dengue Fever-induced Thrombotic Microangiopathy: An Unusual Cause of Renal Failure.

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    Bhargava, V; Gupta, P; Kauntia, R; Bajpai, G

    2017-01-01

    Dengue fever is a tropical infection, which is mosquito-borne disease, caused by dengue virus and spread by Aedes mosquitoes. The incidence of dengue fever has risen rapidly over the past few years. About half of dengue infections are asymptomatic, and a great majority present with fever and body ache. However, the occurrence of complications is well known, including acute kidney injury (AKI). AKI in dengue is usually attributable to a pre-renal cause. Thrombotic microangiopathy is an extremely rare complication of dengue fever, with only a few published case reports in medical literature. This case intends to highlight the importance of recognizing dengue fever-induced thrombotic microangiopathy by physicians and pathologists, enabling better diagnosis and management of this life-threatening condition.

  9. Thrombotic microangiopathy: An unusual cause of renal failure in rheumatoid arthritis

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    Sakthirajan, R.; Dhanapriya, J.; Dineshkumar, T.; Gopalakrishnan, N.; Murugan, S.; Balasubramaniyan, T.

    2017-01-01

    Rheumatoid arthritis (RA) is one of the commonest rheumatological diseases. Renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. Only a few cases of TMA in patients with RA were reported to date. We describe a 45-year-old female patient with RA who presented with oliguria and edema. Renal biopsy showed TMA with patchy cortical necrosis. She improved with hemodialysis and plasmapheresis. PMID:28182045

  10. Antineoplastic agents and thrombotic microangiopathy.

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    Garcia, Gwenalyn; Atallah, Jean Paul

    2017-03-01

    Thrombotic microangiopathy is an uncommon but reported adverse effect of a variety of antineoplastic drugs, including chemotherapy agents such as mitomycin C and gemcitabine, and newer targeted agents such as the vascular endothelial growth factor inhibitors. We present a review of thrombotic microangiopathy associated with antineoplastic agents and its implications in current cancer therapy.

  11. Thrombotic microangiopathies and antineoplastic agents.

    Science.gov (United States)

    Grangé, Steven; Coppo, Paul

    2017-04-01

    Thrombotic microangiopathy is a well-described complication of cancer treatment. Its incidence has increased these last decades, as a result of a better awareness of this complication in cancer patients in one hand, but also of a larger array of therapeutic compounds including anti-vascular endothelium growth factor (VEGF) drugs. It is therefore mandatory to recognize these conditions since they have a significant impact in thrombotic microangiopathies management and prognosis. Practitioners should be aware of the more classical antineoplastic agents associated with thrombotic microangiopathies, the mechanisms by which they induce them, and the resulting management and prognosis. Since malignancy itself can induce thrombotic microangiopathies, it is also mandatory to know how to distinguish rapidly those caused by antineoplastic agents from those associated with cancer, for an adapted management. Thrombotic microangiopathies associated with chemotherapy remain of dismal prognosis. A better understanding of pathophysiology in these forms of thrombotic microangiopathies, in association with a more empirical approach through the use of new therapeutic agents that can also help in the understanding on new mechanisms a posteriori, should improve their prognosis. The preliminary encouraging results reported with complement blockers in this field could represent a convincing example. Copyright © 2017 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.

  12. Renal thrombotic microangiopathy revisited: When a lesion is not a clinical finding

    Directory of Open Access Journals (Sweden)

    De Serres Sacha

    2010-01-01

    Full Text Available Despite advances in the field of thrombotic microangiopathy (TMA and associated syndromes such as thrombotic thrombocytopenic purpura (TTP and hemolytic uremic syndrome (HUS, they still leave several issues unresolved. For instance, actual diagnostic criteria on which therapeutic decisions rely are relatively narrow and focused on TTP-HUS, with the consequence that non-idiopathic and atypically-presenting TMA are overlooked. In addition, nosologic classifi-cations of TMA disorders have varied substantially over the years, but are still devised from historical rather than mechanistic data. As such, it is perhaps not surprising that even today TMA is erroneously used as an interchangeable term with TTP-HUS, and missed or inappropriately diagnosed on various occasions. Yet, recognizing TMA is of crucial importance given that this lesion often manifests with potentially reversible renal failure. In this editorial, which is presented from a Nephrologist′s perspective, we propose that TMA disorders need to be reclassified to include most types of presentations and confirmed or excluded through more elaborate diagnostic approaches.

  13. THROMBOTIC MICROANGIOPATHY ASSOCIATED WITH OPANA ER INTRAVENOUS ABUSE A Case Report.

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    Jabr, Fadi I; Yu, Ling

    2016-01-01

    Thrombotic microangiopathy is characterized by endothelial changes and microvascular stenosis. Several entities such as pregnancy, infection, connective tissue diseases, and drugs are associated with secondary thrombotic microangiopathy. Recently, new reformulation of Opana ER had been associated with thrombotic microangiopathy when injected intravenously. Here, we report the case of a 37-year-old man who developed renal failure and hemolytic anemia secondary to Opana ER intravenous abuse. Renal biopsy pathology was consistent with thrombotic microangiopathy likely caused by Opana ER intravenous abuse.

  14. Thrombotic microangiopathies and acute kidney injury induced by ...

    African Journals Online (AJOL)

    Thrombotic microangiopathies and acute kidney injury induced by artificial termination ... of women with hemolytic anemia, thrombocytopenia and acute kidney injury ... Key words: Acute renal failure, case studies, induced abortion, pregnancy, ...

  15. Everolimus, cyclosporine, and thrombotic microangiopathy: clinical role and preventive tools in renal transplantation.

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    Nava, F; Cappelli, G; Mori, G; Granito, M; Magnoni, G; Botta, C; Solazzo, A; Fontana, F; Baisi, A; Bonucchi, D

    2014-09-01

    Thrombotic microangiopathy (TMA) is characterized by endothelial cell injury and formation of fibrin thrombi within capillary and arterioles. In renal allograft recipients, TMA mainly presents as hemolytic uremic syndrome. Its occurrence is rare, and diagnosis requires a high degree of suspicion. Drug toxicity, in particular from calcineurin inhibitors (CNIs) and mTOR inhibitors (mTORi), is the most common cause posttransplant and has recently been emphasized in the setting of lung transplantation. The goal of this study was to investigate the role of mTORi as an added risk factor in the development of TMA to propose strategies for modulation of immunosuppressive (IS) therapy. From a database of 496 renal graft recipients, we analyzed 350 renal graft biopsy specimens gathered at our center from 1998 to 2012. In patients undergoing combined therapy with mTORi and CNI, we compared drugs levels in TMA-affected and TMA-free groups, using mTORi and CNI TLC and the summation of [everolimus TLC+(cyclosporine C2/100)] (Σ) as a surrogate marker of combined exposition to 2 drugs. Receiver-operating characteristic analysis of association of EVL TLC+(C2/100) was performed for patients exposed to mTORi. Histologic features of TMA were found in 36 patients (prevalence of 7.3%). The caseload was divided into 2 groups: not drug-related TMA (n=19) and drug-related TMA (n=17). Despite the prevalence of TMA in patients exposed to mTORi being greater (8 of 153; prevalence, 5.3%) compared with therapies without mTORi (9 of 324; prevalence, 2.8%), statistical difference was not reached. Patients treated with mTORi who developed de novo drug-related TMA had higher blood levels of IS drugs compared with those who did not develop TMA. Receiver-operating characteristic analysis found a significant threshold of 12.5 ng/mL (area under the curve, 0.803; P=.006). Results confirm the pivotal role of IS drugs in the onset of de novo TMA. On the basis of literature, we could speculate a sequence

  16. Renal thrombotic microangiopathy caused by interferon beta-1a treatment for multiple sclerosis

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    Mahe J

    2013-08-01

    Full Text Available Julien Mahe,1 Aurélie Meurette,2 Anne Moreau,3 Caroline Vercel,2 Pascale Jolliet1,4 1Clinical Pharmacology Department, Institute of Biology, University Hospital, Nantes, France; 2Clinical Nephrology and Immunology Department, University Hospital, Nantes, France; 3Laboratory of Pathology, University Hospital, Nantes, France; 4EA 4275 Biostatistics, Pharmacoepidemiology and Subjective Measures in Health Sciences, University of Nantes, Nantes, France Abstract: Interferon beta-1a is available as an immunomodulating agent for relapsing forms of multiple sclerosis. Common side effects include flu-like symptoms, asthenia, anorexia, and administration site reaction. Kidney disorders are rarely reported. In this study we describe the case of a woman who has been undergoing treatment with interferon beta-1a for multiple sclerosis for 5 years. She developed a hemolytic-uremic syndrome with intravascular hemolysis in a context of severe hypertension. A kidney biopsy showed a thrombotic microangiopathy. This observation highlights an uncommon side effect of long-term interferon beta-1a therapy. Pathophysiological mechanisms leading to this complication might be explained by the antiangiogenic activity of interferon. Keywords: thrombotic microangiopathy, interferon beta, hemolytic-uremic syndrome, antiangiogenic activity

  17. Renal thrombotic microangiopathy in mice with combined deletion of endocytic recycling regulators EHD3 and EHD4.

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    Manju George

    Full Text Available Eps15 Homology Domain-containing 3 (EHD3, a member of the EHD protein family that regulates endocytic recycling, is the first protein reported to be specifically expressed in the glomerular endothelium in the kidney; therefore we generated Ehd3(-/- mice and assessed renal development and pathology. Ehd3(-/- animals showed no overt defects, and exhibited no proteinuria or glomerular pathology. However, as the expression of EHD4, a related family member, was elevated in the glomerular endothelium of Ehd3(-/- mice and suggested functional compensation, we generated and analyzed Ehd3(-/-; Ehd4(-/- mice. These mice were smaller, possessed smaller and paler kidneys, were proteinuric and died between 3-24 weeks of age. Detailed analyses of Ehd3(-/-; Ehd4(-/- kidneys demonstrated thrombotic microangiopathy (TMA-like glomerular lesions including thickening and duplication of glomerular basement membrane, endothelial swelling and loss of fenestrations. Other changes included segmental podocyte foot process effacement, mesangial interposition, and abnormal podocytic and mesangial marker expression. The glomerular lesions observed were strikingly similar to those seen in human pre-eclampsia and mouse models of reduced VEGF expression. As altered glomerular endothelial VEGFR2 expression and localization and increased apoptosis was observed in the absence of EHD3 and EHD4, we propose that EHD-mediated endocytic traffic of key surface receptors such as VEGFR2 is essential for physiological control of glomerular function. Furthermore, Ehd3(-/-; Ehd4(-/- mice provide a unique model to elucidate mechanisms of glomerular endothelial injury which is observed in a wide variety of human renal and extra-renal diseases.

  18. Clinical and Renal Biopsy Findings Predicting Outcome in Renal Thrombotic Microangiopathy: A Large Cohort Study from a Single Institute in China

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    Xiao-Juan YU

    2014-01-01

    Full Text Available Objective. The current study aimed to investigate the spectrum of etiologies and associated disorders of renal biopsy-proven thrombotic microangiopathy (TMA patients. Methods. The clinical, laboratory, and renal histopathological data of patients with renal TMA from 2000 to 2012 in our institute were collected and reviewed. Results. One hundred and nine TMA patients were enrolled in this study. The mean age was 34.0 ± 11.1 years. Seventy patients (64.2% were male and thirty-nine patients (35.8% were female. There were eight patients (7.3% with hemolytic uremic syndrome (HUS. Sixty-one patients (56.0% were secondary to malignant hypertension. Fourteen patients (12.8% were pregnancy-associated TMA. Other associated disorders included 17 patients with connective tissue disorders, 2 patients with hematopoietic stem cell transplantation, 4 patients with Castleman’s disease, 1 patient with cryoglobulinemia, and 2 patients with glomerulopathy. During followup, 8 patients died due to severe infection, 17 patients had doubling of serum creatinine, and 44 had end-stage renal disease. In multivariate analysis, male, elevated serum creatinine, and decreased hemoglobin were independently associated with poor renal outcomes. Conclusions. Renal TMA changes consisted of different disorders with various etiologies. aHUS, pregnancy-associated TMA, and malignant hypertension accounted for the majority of patients in our cohort.

  19. Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy in first degree relatives; a case report

    OpenAIRE

    Idorn Thomas; Schejbel Lone; Rydahl Casper; Heaf James; Jølvig Karen; Bergstrøm Marie; Garred Peter; Kamper Anne-Lise

    2012-01-01

    Abstract Background Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy are rare diseases with no known coherence. Case Presentation A daughter and her biological mother were diagnosed with pregnancy-induced thrombotic microangiopathy and anti-glomerular basement membrane glomerulonephritis, respectively. Both developed end-stage renal disease. Exploration of a common aetiology included analyses of HLA genotypes, functional and genetic aspects of the complement...

  20. Defining the genetics of thrombotic microangiopathies.

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    Vieira-Martins, Paula; El Sissy, Carine; Bordereau, Pauline; Gruber, Aurelia; Rosain, Jeremie; Fremeaux-Bacchi, Veronique

    2016-04-01

    The spectrum of the thrombotic microangiopathies (TMA) encompasses a heterogeneous group of disorders with hereditary and acquired forms. Endothelial cell injury in the microvasculature is common to all TMAs, whatever the pathophysiological process. In this review we describe genetic mutations characteristic of certain TMAs and review their contributions to disease. Recent identification of novel pathologic mutations has been enabled by exome studies. The monogenic forms of TMA are more frequently caused by recessive alterations in von Willebrand factor cleaving protease ADAMST13, leading to congenital thrombotic thrombocytopenic purpura, or cobalamine C and DGKE genes, leading to an atypical hemolytic-uremic syndrome (aHUS)-like TMA. aHUS, whether idiopathic or linked to a known complement amplifying condition, is a TMA that primarily affects kidney function. It often results from a combination of an underlying genetic susceptibility with environmental factors activating the alternative complement pathway. Pathogenic variants in at least five complement genes coding for complement factor H (CFH) complement factor I (CFI), MCP (CD46), C3 and complement factor B (CFB) have been demonstrated to increase the risk of developing aHUS, but several more genes have been implicated. A new challenge is to separate disease-associated genetic variants from the broader background of variants or polymorphisms present in all human genomes that are rare, potentially functional, but may or may not be pathogenic.

  1. Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy.

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    Gharbi, Chems; Bourry, Edward; Rouvier, Philippe; Hacini, Sabria; Letaief, Ahmed; Baumelou, Alain; Izzedine, Hassane

    2010-10-01

    Although uncommon, thrombotic microangiopathy (TMA) is one of the most serious complications in patients with systemic lupus erythematosus. A 30-year-old black woman admitted to our hospital because of fever, fatigue, 'dark' urine and rapidly progressive renal failure was found to have systemic lupus erythematous and atypical hemolytic uremic syndrome. Kidney biopsy showed WHO class IV lupus nephritis with crescents and TMA. Hemodialysis was initiated for worsening renal failure. The patient was treated with corticosteroids, monthly pulse intravenous Cyclophosphamide, plasmapheresis and Rituximab on a weekly basis for 4 weeks. The patient's blood pressure was aggressively controlled using antihypertensive agents. Despite this extensive therapy, she remained dialysis dependent although hematological parameters returned to normal values.

  2. Thrombotic Microangiopathy Revealing Bone Metastases from an Ethmoid Sinus Carcinoma

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    Mony Chenda Morisse

    2016-08-01

    Full Text Available Cancer-related thrombotic microangiopathy (TMA is a rare entity whose clinical and biological characteristics have been described in various tumors. Here we describe the first case of cancer-related TMA revealing diffuse bone metastases from an ethmoid sinus carcinoma.

  3. Catastrophic APS in the context of other thrombotic microangiopathies.

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    Rodríguez-Pintó, Ignasi; Espinosa, Gerard; Cervera, Ricard

    2015-01-01

    The catastrophic antiphospholipid syndrome (CAPS) is a rare disease that affects 1 % of cases with antiphospholipid syndrome (APS). CAPS can mimic or overlap with different thrombotic diseases; many patients present with a microthrombotic storm or thrombotic microangiopathic hemolytic anemia (TMHA). Thus, the differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP), typical and atypical hemolytic uremic syndrome (HUS), systemic infections, malignancies, pregnancy-related disorders, malignant hypertension, heparin-induced thrombocytopenia, and drug-induced thrombotic microangiopathies. Antiphospholipid antibody (aPL) positivity has been proposed as the clue in this differential diagnosis; however, aPL can also occur in healthy people and in those with infections or malignancies. Thus, the differential diagnosis of an aPL-positive patient presenting with a microthrombotic storm is broad; the workup should include a special attention to signs of infection and disseminated malignant disease, assessing the funduscopic signs of malignant hypertension, testing ADAMTS13 activity and anti-heparin-platelet factor 4 (HPF4) antibodies, and searching previous exposure to certain drugs. This article aims to review the main diseases included in the differential diagnosis of CAPS in the context of other thrombotic microangiopathies.

  4. Pulmonary Tumor Thrombotic Microangiopathy: Clinical, Radiologic, and Histologic Correlation

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    Rohit Godbole

    2015-01-01

    Full Text Available Pulmonary tumor thrombotic microangiopathy (PTTM is a clinicopathologic disease entity in which the tumor cells embolize to the pulmonary vasculature leading to a series of maladaptive reactions including the activation of coagulation and fibrocellular intimal thickening. The resultant stenosis of blood vessels leads to pulmonary hypertension and eventual death from cor pulmonale. In this report, we present a case of PTTM presenting as the initial manifestation of metastatic gastric carcinoma in a young man. Although unusual in its occurrence as the initial manifestation of gastric carcinoma, the case is illustrative in its clinical, radiological and histological presentation.

  5. [Thrombotic microangiopathies: HUS/TTP. Physiopathological aspects].

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    Sánchez Avalos, J C

    2000-01-01

    In thrombotic thrombocytopenic purpura (TTP) and in the hemolytic uremic syndrome (HUS) fibrin-platelet thrombi occlude arterioles and capillaries. The mechanism of endothelial cell injury and the mechanism of thrombosis are the most important physiopathological events in this pathology and are largely unknown. In HUS due to the Shiga toxin, the lesion of the endothelial cells is due to penetration of the toxin into the cell via the Gb3 receptor. Endothelial cell death is a consequence of altered protein synthesis at the ribosomal level. Cytokines released during the inflammatory process, possibly enhance the endothelial damage. Genetic and immunologic predisposing factors for the development of HUS have also been postulated. In idiopathic, secondary and familial HUS/TTP the mechanism of endothelial lesion is unknown, but multiple responsible factors have been advocated such as infections, drugs, pregnancy, autoantibodies, apoptosis inducing molecules, etc. and other genetic, hormonal or immunologic predisposing factors may also be involved. Factor H deficiency has been blamed in familiar cases. The most important cause of microcirculation thrombosis is the thrombogenic capacity of endothelial cell "activation" or injury induced by multiple mechanisms. The predominant source of plasma vW factor multimers is apparent in the altered endothelial cell. The unusually large vWF multimers are more effective at binding to platelet glycoprotein Ib-IX and IIb-IIIa complexes and inducing aggregation, as also occurred with the low weight multimers formed with excessive proteolysis, as described in the acute phase of HUS/TTP. The recent report of congenital deficiency of a vWF protease in familial TTP and its functional inhibition by autoantibodies in acquired cases is characteristic of TTP. This protease inhibition has never been described in HUS and might represent pathogenetic differences between TTP and HUS, and contribute to the differential diagnosis, but further

  6. Ischemic colitis as a manifestation of thrombotic microangiopathy following bone marrow transplantation.

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    Komeno, Yukiko; Ogawa, Seishi; Ishida, Tateru; Takeuchi, Kengo; Tsujino, Shiho; Kurokawa, Mineo; Aoki, Katsunori; Kanda, Yoshinobu; Chiba, Shigeru; Motokura, Toru; Fukayama, Masashi; Hirai, Hisamaru

    2003-12-01

    Thrombotic microangiopathy (TMA) is a microvascular disorder characterized by platelet aggregation and hemolytic anemia. In the setting of bone marrow transplantation (BMT), ischemic colitis due to TMA is difficult to differentiate from acute graft-versus-host disease. We report a 32-year-old man who presented ischemic colitis due to TMA after unrelated BMT for myelodysplastic syndrome. He suffered from treatment-resistant bloody diarrhea, and died of renal failure and Aspergillus pleuritis on day 253 post-BMT. Autopsy revealed endothelial injuries of arterioles and ischemic changes in the intestines and kidneys. Clinical and pathological characteristics of ischemic colitis due to BMT-associated TMA are described.

  7. A COL4A5 mutation with glomerular disease and signs of chronic thrombotic microangiopathy.

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    Wuttke, Matthias; Seidl, Maximilian; Malinoc, Angelica; Prischl, Friedrich C; Kuehn, E Wolfgang; Walz, Gerd; Köttgen, Anna

    2015-12-01

    COL4A5 mutations are a known cause of Alport syndrome, which typically manifests with haematuria, hearing loss and ocular symptoms. Here we report on a 16-year-old male patient with a negative family history who presented with proteinuria, progressive renal failure and haemolysis, but without overt haematuria or hearing loss. A renal biopsy revealed features of atypical IgA nephropathy, while a second biopsy a year later showed features of focal segmental glomerulosclerosis, but was finally diagnosed as chronic thrombotic microangiopathy. Targeted sequencing of candidate genes for steroid-resistant nephrotic syndrome and congenital thrombotic microangiopathy was negative. Despite all therapeutic efforts, including angiotensin-converting enzyme inhibition, immunosuppressive therapy, plasma exchanges and rituximab, the patient progressed to end-stage renal disease. When a male cousin presented with nephrotic syndrome years later, whole-exome sequencing identified a shared disruptive COL4A5 mutation (p.F222C) that showed X-linked segregation. Thus, mutations in COL4A5 give rise to a broader spectrum of clinical presentation than commonly suspected, highlighting the benefits of comprehensive rather than candidate genetic testing in young patients with otherwise unexplained glomerular disease. Our results are in line with an increasing number of atypical presentations of single-gene disorders identified through genome-wide sequencing.

  8. Pulmonary tumor thrombotic microangiopathy in an unknown primary cancer

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    Gayathri P Amonkar

    2014-01-01

    Full Text Available Pulmonary tumor thrombotic microangiopathy (PTTM is a highly fatal complication of cancer leading to acute cor pulmonale and pulmonary hypertension. We present a case of 47-year-old male patient who developed acute breathlessness and died suddenly. The pulmonary vessels at autopsy on histopathologic examination showed the presence of fibrocellular intimal proliferation, fibrin thrombi and few tumor emboli consisting of malignant adenocarcinoma cells. There was associated lymphangiosis carcinomatosis. No primary visceral tumor was found despite extensive search. The patient had died following acute cor pulmonale with sudden pulmonary hypertension due to PTTM. This entity (PTTM must be kept as a differential diagnosis in patients presenting with acute breathlessness especially in cases of cancers.

  9. Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy in first degree relatives; a case report

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    Idorn Thomas

    2012-07-01

    Full Text Available Abstract Background Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy are rare diseases with no known coherence. Case Presentation A daughter and her biological mother were diagnosed with pregnancy-induced thrombotic microangiopathy and anti-glomerular basement membrane glomerulonephritis, respectively. Both developed end-stage renal disease. Exploration of a common aetiology included analyses of HLA genotypes, functional and genetic aspects of the complement system, ADAMTS13 activity and screening for autoantibodies. The daughter was heterozygous carrier of the complement factor I G261D mutation, previously described in patients with membranoproliferative glomerulonephritis and atypical haemolytic uremic syndrome. The mother was non-carrier of this mutation. They shared the disease associated complement factor H silent polymorphism Q672Q (79602A>G. Conclusion An unequivocal functional or molecular association between these two family cases was not found suggesting that the patients probably share another, so far undiagnosed and unknown, predisposing factor. It seems highly unlikely that two infrequent immunologic diseases would occur by unrelated pathophysiological mechanisms within first degree relatives.

  10. Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.

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    Kundra, Ajay; Wang, Jen Chin

    2017-04-01

    Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome. (3) There were no cases reported for polycythemia vera (PV) or lymphoma. (4) Sex distribution was nearly equivalent with the exception in patients with multiple sclerosis where there was female predominance (12 of 16 with reported data). (5) For pooled analysis, the average duration of treatment with IFN before TMA was diagnosed was 40.4 months. (6) Comparative analysis showed that patients with MS required the highest cumulative dose exposure before developing TMA (MS 68.6 months, CML 35.5 months, HCV 30.4 months). (7) Cases of confirmed TTP (where A disintegrin and Metalloprotease with thrombospondin type 1 motif 13: ADAMTS 13 level was measured) showed presence of an inhibitor. (8) In all cases of confirmed TTP, moderate to severe thrombocytopenia was a striking clinical feature at presentation while this was not a consistent finding in all other cases of TMA. (9) Outcome analysis revealed complete remission in 27 (40%), persistent chronic kidney disease (CKD) in 28 (42%) and fatality in 12 patients (18%). (10) Treatment with corticosteroids, plasma exchange and rituximab resulted in durable responses. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. THROMBOTIC MICROANGIOPATHY IN HAEMATOPOIETIC CELL TRANSPLANTATION:AN UPDATE

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    Evi Stavrou

    2010-10-01

    Full Text Available Allogeneic hematopoietic cell transplantation (HCT represents a vital procedure for patients with various hematologic conditions. Despite advances in the field, HCT carries significant morbidity and mortality. A rare but potentially devastating complication is transplantation-associated thrombotic microangiopathy (TA-TMA. In contrast to idiopathic TTP, whose etiology is attributed to deficient activity of ADAMTS13, (a member of the A Disintegrin And Metalloprotease with Thrombospondin 1 repeats family of metalloproteases, patients with TA-TMA have > 5% ADAMTS13 activity. Pathophysiologic mechanisms associated with TA-TMA, include loss of endothelial cell integrity induced by intensive conditioning regimens, immunosuppressive therapy, irradiation, infections and graft-versus-host (GVHD disease. The reported incidence of TA-TMA ranges from 0.5% to 75%, reflecting the difficulty of accurate diagnosis in these patients. Two different groups have proposed consensus definitions for TA-TMA, yet they fail to distinguish the primary syndrome from secondary causes such as infections or medication exposure. Despite treatment, mortality rate in TA-TMA ranges between 60% to 90%. The treatment strategies for TA-TMA remain challenging. Calcineurin inhibitors should be discontinued and replaced with alternative immunosuppressive agents.  Daclizumab, a humanized monoclonal anti-CD25 antibody, has shown promising results in the treatment of TA-TMA. Rituximab or the addition of defibrotide, have been reported to induce remission in this patient population. In general, plasma exchange is not recommended.

  12. Acute kidney injury in pregnancy: the thrombotic microangiopathies.

    Science.gov (United States)

    Ganesan, Chitra; Maynard, Sharon E

    2011-01-01

    Acute kidney injury (AKI) is a rare but serious complication of pregnancy. Although prerenal and ischemic causes of AKI are most common, renal insufficiency can complicate several other pregnancy-specific conditions. In particular, severe preeclampsia/HELLP syndrome, acute fatty liver of pregnancy (AFLP) and thrombotic thrombocytopenic purpura (TTP) are all frequently complicated by AKI, and share several clinical features which pose diagnostic challenges to the clinician. In this article, we discuss the clinical and laboratory features, pathophysiology and treatment of these 3 conditions, with particular attention to renal manifestations. It is imperative to distinguish these conditions to make appropriate therapeutic decisions which can be lifesaving for the mother and fetus. Typically AFLP and HELLP improve after delivery of the fetus, whereas plasma exchange is the first-line treatment for TTP.

  13. A case report: Cavitary infarction caused by pulmonary tumor thrombotic microangiopathy in a patient with pancreatic intraductal papillary mucinous neoplasm

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    Bae, Kyoung Kyg; Kwon, Woon Jung; Choi, Seong Hoon; Lee, Jong Hwa; Cha, Hee Jeong [Ulsan University Hospital, University of Ulsan School of Medicine, Ulsan (Korea, Republic of)

    2015-08-15

    Pulmonary tumor embolism is commonly discovered at autopsy, but is rarely suspected ante-mortem. Microangiopathy is an uncommon and distinct form of simple tumor pulmonary embolism. Here, we present a 52-year-old male with tumor thrombotic microangiopathy and pulmonary infarction, which might have originated from intraductal papillary mucinous tumor of the pancreas. Multiple wedge-shaped consolidations were found initially and aggravated with cavitation. These CT features of pulmonary infarction were pathologically confirmed to result from pulmonary tumor thrombotic microangiopathy.

  14. Thrombotic Microangiopathy with Skin Localization Secondary to Cytarabine-Daunorubicin Association: Report of a Case

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    S. Regragui

    2012-01-01

    Full Text Available The thrombotic microangiopathy is a syndrome characterized by the combination of mechanical hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. In addition to the idiopathic form, several cases are identified as secondary to pregnancy, infections, disease systems, organ transplants, and cancer. Other forms are secondary to drugs including antimitotics. We report the case of a patient followed for acute myelogenous leukemia. She received induction chemotherapy combining daunorubicin and cytarabine, complicated by thrombotic thrombocytopenic purpura.

  15. Pulmonary tumor thrombotic microangiopathy with cor pulmonale due to desmoplastic small round cell tumor.

    Science.gov (United States)

    Sadimin, Evita T; Collier, Adrienne G; Gaffney, Joseph W; Fyfe, Billie

    2012-04-01

    A 12-year-old boy presented acutely after an episode of syncope with perioral cyanosis. He died 19 hours after admission due to cor pulmonale as a complication of metastatic desmoplastic small round cell tumor in the lungs with associated tumor thrombotic microangiopathy.

  16. Thrombotic microangiopathy caused by oral contraceptives in a kidney transplant recipient.

    Science.gov (United States)

    Shirai, Hiroyuki; Yashima, Jun; Tojimbara, Tamotsu; Honda, Kazuho

    2016-07-01

    Thrombotic microangiopathy (TMA) after kidney transplantation has various aetiologies, including acute antibody-mediated rejection, bacterial or viral infection and immunosuppressive drugs, particularly calcineurin inhibitors. We present the case of a 28-year-old woman who developed TMA 30 months after the transplantation of an ABO-incompatible kidney from a living unrelated donor. The patient developed a sudden onset of allograft renal dysfunction and became uremic. She was transferred to our institution from a community hospital with strongly suspected acute allograft rejection. Intensive treatments for both T- and B-cell mediated acute rejection, including steroid pulse therapy, double-filtration plasmapheresis, antithymocyte globulin (1.5 mg/kg × 14 days) and rituximab (100 mg), were initiated during haemodialysis. However, her renal allograft function did not improve. Histopathological analysis 8 days after the treatment indicated TMA, despite the absence of apparent acute T-cell- or acute antibody-mediated rejection. There were no symptoms of infectious diseases, such as intestinal haemorrhagic colitis or viral infection. We concluded that the use of oral contraceptives, which had been initiated 3 weeks before TMA onset for the treatment of irregular vaginal bleeding, was the aetiologic agent.

  17. A Clinicopathologic Study of Thrombotic Microangiopathy in IgA Nephropathy

    Science.gov (United States)

    Hill, Gary S.; Karras, Alexandre; Jacquot, Christian; Moulonguet, Luc; Kourilsky, Olivier; Frémeaux-Bacchi, Véronique; Delahousse, Michel; Van Huyen, Jean-Paul Duong; Loupy, Alexandre; Bruneval, Patrick; Nochy, Dominique

    2012-01-01

    Thrombotic microangiopathy (TMA) occurs in IgA nephropathy, but its clinical significance is not well described. We retrospectively examined a series of 128 patients diagnosed with IgA nephropathy between 2002 and 2008 who had a mean follow-up of 44±27 months. In our series, 53% presented with lesions of TMA, acute or organized, in arteries and/or arterioles. Among patients with TMA, 4% were normotensive, 25% had controlled hypertension, and 71% had uncontrolled hypertension. Of those with uncontrolled hypertension, 26% had malignant hypertension. Histologically, the group with TMA had a significantly greater percentage of sclerotic glomeruli and worse tubulointerstitial fibrosis than those of the group without TMA. However, a significant minority of patients had near-normal histology, with minimal tubular atrophy (20%) and/or <20% interstitial fibrosis (24%). TMA rarely occurred in the absence of significant proteinuria. During follow-up, a doubling of serum creatinine or ESRD occurred in all patients with laboratory evidence of TMA, in 42% of those with morphologic evidence but no laboratory evidence of TMA, and in 11% of those without TMA. In summary, lesions of TMA are frequent in IgA nephropathy and may occur in normotensive patients with near-normal renal histology. Although the pathophysiologic mechanisms involved remain undetermined, the current study rules out severe hypertension or advanced renal disease as sole causes. PMID:22052055

  18. Two cases of kidney transplantation-associated thrombotic microangiopathy successfully treated with eculizumab.

    Science.gov (United States)

    Ikeda, Takashi; Okumi, Masayoshi; Unagami, Kohei; Kanzawa, Taichi; Sawada, Anri; Kawanishi, Kunio; Omoto, Kazuya; Ishida, Hideki; Tanabe, Kazunari

    2016-07-01

    Transplantation-associated thrombotic microangiopathy (TA-TMA) is relatively rare and requires immediate intervention to avoid irreversible organ damage or death; however, consensus regarding the treatment approach is lacking. Atypical haemolytic uraemic syndrome (aHUS) is a rare disease caused by dysregulation of the alternative complement pathway resulting in TMA. aHUS is histologically similar to TA-TMA; approximately 60% of TA-TMA patients have complement dysregulation. Eculizumab, a humanized anti-C5 monoclonal antibody, inhibits terminal membrane-attack complex formation and TMA progression. Eculizumab has been successfully used to treat aHUS post-transplant. We present two cases of kidney TA-TMA due to unknown causes, suspected antibody-mediated rejection, or calcineurin inhibitor (CNI)-related toxicity that developed on day 1 or 2 post-kidney transplantation. Low platelet count and haemoglobin level with red cell fragments were detected. Despite steroid pulse, plasma exchange (PE), and intravenous immunoglobulin therapy, TA-TMA did not improve; therefore, eculizumab was administered despite no genetic testing. Laboratory data, including renal function, improved immediately. TA-TMA treatment primarily involves PE initiation or CNI discontinuation; eculizumab can be used to safely treat TA-TMA and then be ceased in the short term. Therefore, eculizumab administration might be beneficial for kidney TA-TMA as early as the diagnosis of refractory to PE.

  19. A Case of Fibrillary Glomerulonephritis Associated with Thrombotic Microangiopathy and Anti-Glomerular Basement Membrane Antibody

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    Akishi Momose

    2015-02-01

    Full Text Available We present the first report of a case of fibrillary glomerulonephritis (FGN associated with thrombotic microangiopathy (TMA and anti-glomerular basement membrane antibody (anti-GBM antibody. A 54-year-old man was admitted to our hospital for high fever and anuria. On the first hospital day, we initiated hemodialysis for renal dysfunction. Laboratory data revealed normocytic-normochromic anemia with schistocytes in the peripheral smear, thrombocytopenia, increased serum lactate dehydrogenase, decreased serum haptoglobin, and negative results for both direct and indirect Coombs tests. Based on these results, we diagnosed TMA. Assays conducted several days later indicated a disintegrin-like and metalloprotease with a thrombospondin motif 13 (ADAMTS13 activity of 31.6%, and ADAMTS13 inhibitors were negative. We started plasma exchange using fresh frozen plasma and steroid pulse therapy. Anti-GBM antibody was found to be positive. Renal biopsy showed FGN. Blood pressure rose on the 46th hospital day, and mild convulsions developed. Based on magnetic resonance imaging of the head, the patient was diagnosed with reversible posterior leukoencephalopathy syndrome. Hypertension persisted despite administration of multiple antihypertensive agents, and the patient experienced a sudden generalized seizure. Computed tomography of the head showed multiple cerebral hemorrhages. However, his blood pressure subsequently decreased and the platelet count increased. TMA remitted following 36 plasma exchange sessions, but renal function was not restored, and maintenance hemodialysis was continued. The patient was discharged on the 119th day of hospitalization. In conclusion, it was shown that TMA, FGN and anti-GBM antibody were closely related.

  20. Catastrophic antiphospholipid antibody syndrome in a child with thrombotic microangiopathy.

    Science.gov (United States)

    Prasad, N; Bhadauria, D; Agarwal, N; Gupta, A; Gupta, P; Jain, M; Lal, H

    2012-07-01

    Thrombotic microangiopathic hemolytic anemia (TMHA) is not uncommon in clinical nephrology practice while antiphospholipid syndrome (APS) is uncommon. Although less than 1% of patients with APS develop catastrophic APS (CAPS), its potential lethal outcome because of thrombosis in multiple organs and subsequent multiorgan failure emphasizes its importance in nephrology practice. Here is a case of catastrophic APS in a 7-year-old girl, who presented to us with TMHA associated with antiphospholipid antibodies and subsequently died because of CAPS.

  1. Microangiopatia trombótica en adultos Thrombotic microangiopathy in adults

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    Gonzalo J. Barrientos

    2006-08-01

    Full Text Available Las microangiopatías trombóticas (MAT incluyen la púrpura trombótica trombocitopénica (PTT, el síndrome urémico hemolítico (SUH y las microangiopatías del embarazo. Se describen ocho pacientes adultos con cuadros de microangiopatía trombótica, que fueron atendidos en el Hospital Italiano de Buenos Aires entre 2003 y 2004. El promedio de edad fue de 40 años, con igual proporción de hombres y mujeres. En cuatro de los ocho pacientes descriptos el diagnóstico se realizó al ingreso. Cuatro pacientes evolucionaron con características de SUH, tres como PTT y uno como MAT del embarazo. Todos los pacientes presentaron trombocitopenia y anemia hemolítica microangiopática. La insuficiencia renal fue la tercera característica más frecuente. Aquellos con diagnóstico de PTT presentaron los cuadros de mayor gravedad. Todos los pacientes fueron tratados con plasmaféresis. El tratamiento inmunosupresor también fue utilizado. Cuatro pacientes se recuperaron completamente, 2 fallecieron, 1 permanece con insuficiencia renal crónica con requerimiento de hemodiálisis y 1 fue colectomizado. Las MAT son desórdenes oclusivos de la microcirculación, con repercusión sistémica y/o renal. Existe superposición de los cuadros de PTT/SUH, y éstos pueden ser idiopáticos o secundarios. La importancia de un diagnóstico y tratamiento precoz radica en su elevada morbimortalidad. Entre los diagnósticos diferenciales figuran la sepsis, las enfermedades oncológicas, las vasculitis sistémicas, la eclampsia y otros. La infusión del plasma y principalmente la plasmaféresis son los tratamientos fundamentales. Aún se requiere mejorar el manejo y la evolución de estos síndromes, dada la elevada morbimortalidad que conllevan.Thrombotic microangiopathic hemolytic anemias include thrombotic thrombocytopenic purpura (TTP, hemolytic uremic syndrome (HUS and pregnancy associated thrombotic microangiopathy (TMA. Eight adult patients (four males and four

  2. Thrombotic Microangiopathy Care Pathway: A Consensus Statement for the Mayo Clinic Complement Alternative Pathway-Thrombotic Microangiopathy (CAP-TMA) Disease-Oriented Group.

    Science.gov (United States)

    Go, Ronald S; Winters, Jeffrey L; Leung, Nelson; Murray, David L; Willrich, Maria A; Abraham, Roshini S; Amer, Hatem; Hogan, William J; Marshall, Ariela L; Sethi, Sanjeev; Tran, Cheryl L; Chen, Dong; Pruthi, Rajiv K; Ashrani, Aneel A; Fervenza, Fernando C; Cramer, Carl H; Rodriguez, Vilmarie; Wolanskyj, Alexandra P; Thomé, Stephan D; Hook, C Christopher

    2016-09-01

    Thrombotic microangiopathies (TMAs) comprise a heterogeneous set of conditions linked by a common histopathologic finding of endothelial damage resulting in microvascular thromboses and potentially serious complications. The typical clinical presentation is microangiopathic hemolytic anemia accompanied by thrombocytopenia with varying degrees of organ ischemia. The differential diagnoses are generally broad, while the workup is frequently complex and can be confusing. This statement represents the joint recommendations from a multidisciplinary team of Mayo Clinic physicians specializing in the management of TMA. It comprises a series of evidence- and consensus-based clinical pathways developed to allow a uniform approach to the spectrum of care including when to suspect TMA, what differential diagnoses to consider, which diagnostic tests to order, and how to provide initial empiric therapy, as well as some guidance on subsequent management. Copyright © 2016 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  3. Blood and marrow transplant clinical trials network toxicity committee consensus summary: thrombotic microangiopathy after hematopoietic stem cell transplantation.

    Science.gov (United States)

    Ho, Vincent T; Cutler, Corey; Carter, Shelly; Martin, Paul; Adams, Roberta; Horowitz, Mary; Ferrara, James; Soiffer, Robert; Giralt, Sergio

    2005-08-01

    The syndrome of microangiopathic hemolysis associated with renal failure, neurologic impairment, or both is a recognized complication of hematopoietic stem cell transplantation. This entity is often called hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP), yet it is clear that the pathophysiology of transplant-associated HUS/TTP is different from that of classic HUS or TTP. Furthermore, the incidence of this syndrome varies from 0.5% to 76% in different transplant series, primarily because of the lack of a uniform definition. The toxicity committee of the Blood and Marrow Transplant Clinical Trials Network has reviewed the current literature on transplant-related HUS/TTP and recommends that it be henceforth renamed posttransplantation thrombotic microangiopathy (TMA). An operational definition for TMA based on the presence of microangiopathic hemolysis and renal and/or neurologic dysfunction is proposed. The primary intervention after diagnosis of TMA should be withdrawal of calcineurin inhibitors. Plasma exchange, although frequently used in this condition, has not been proven to be effective. In the absence of definitive trials, plasma exchange cannot be considered a standard of care for TMA. It is hoped that these positions will improve the identification and reporting of this devastating complication after hematopoietic stem cell transplantation and facilitate future clinical studies for its prevention and treatment.

  4. Does early initiation of therapeutic plasma exchange improve outcome in pediatric stem cell transplant-associated thrombotic microangiopathy?

    Science.gov (United States)

    Jodele, Sonata; Laskin, Benjamin L; Goebel, Jens; Khoury, Jane C; Pinkard, Susan L; Carey, Patricia M; Davies, Stella M

    2013-03-01

    The use of therapeutic plasma exchange (TPE) in hematopoietic stem cell transplant-associated thrombotic microangiopathy (TA-TMA) is controversial because the exact mechanism of injury in TA-TMA is not yet understood. The study objective was to retrospectively review the outcome of children receiving TPE for TA-TMA at our institution. We hypothesized that patients initiating TPE earlier in their disease course would receive a greater benefit than those starting later, regardless of the therapeutic mechanism. We identified 10 consecutive pediatric patients with TA-TMA treated with TPE. Nine of these patients showed normalization of the laboratory variables associated with microangiopathy during their TPE course, but only five patients recovered renal function and survived TA-TMA. The five survivors started TPE a median of 17 days (range, 4-25 days) after TA-TMA diagnosis while the five patients who died started TPE a median of 32 days (range, 17-73 days) after TA-TMA was diagnosed. Three of the five survivors had multiorgan failure at TA-TMA diagnosis and completely recovered with early institution of TPE. These three survivors were able to discontinue renal replacement therapy, and all achieved a normal posttreatment creatinine. The five patients with later institution of TPE progressed to end-stage renal disease and all died. There were no serious TPE-related complications in either group. This is the first report evaluating TPE response in regard to procedure initiation time after TA-TMA diagnosis. Our data suggests that early initiation of TPE might be beneficial even in patients with multiorgan failure due to TA-TMA. © 2012 American Association of Blood Banks.

  5. Thrombotic Microangiopathy in the Setting of HIV Infection: A Case Report and Review of the Differential Diagnosis and Therapy.

    Science.gov (United States)

    Saab, Karim R; Elhadad, Sonia; Copertino, Dennis; Laurence, Jeffrey

    2016-08-01

    Before the modern era of HIV/AIDS therapeutics, which enabled a cascade of early recognition of infection, prompt initiation of effective antiretroviral therapies, and close follow-up, severe forms of microvascular clotting disorders known as thrombotic microangiopathies (TMAs) were frequent in the setting of advanced HIV disease. Their incidence was as high as 7% in the period 1984-1999, but fell dramatically, to therapy: with recognition of autoantibodies against the von Willebrand factor cleaving protease ADAMTS13 as the etiology of most cases of one major form of TMA, thrombotic thrombocytopenic purpura, it permitted appropriate use of life-saving interventions based on plasma exchange and immune suppression. A more recent factor in TMA therapeutics was the 2011 approval by the US FDA and European EMA of eculizumab, a humanized monoclonal antibody against complement component C5, for the treatment of atypical hemolytic uremic syndrome, another major form of TMA. Despite these milestones, life- and organ-threatening TMAs still occur in untreated HIV disease and, to a much lesser extent, in those patients with suppressed viral loads. Confusion in terms of the differential diagnosis of these TMAs also impedes use of directed treatments. This report utilizes a case study of a young woman with advanced AIDS who presented with a severe TMA, characterized by coma and renal failure, to highlight the diagnostic and therapeutic challenges raised by complex hematologic conditions occurring in the setting of HIV.

  6. A case of progressive hypertension preceding gemcitabine-associated thrombotic microangiopathy complicated by acute kidney injury and stroke.

    LENUS (Irish Health Repository)

    Phelan, Paul J

    2009-01-01

    Gemcitabine-associated thrombotic microangiopathy is being increasingly recognized as a serious complication of treatment. We report a normotensive patient who developed progressive hypertension after commencing gemcitabine therapy. She also developed subtle changes in her platelet count and serum creatinine months before her emergent presentation. Clinicians should be aware of new onset or worsening hypertension and \\'mild\\' biochemical changes in gemcitabine-treated patients.

  7. De novo post-transplant thrombotic microangiopathy localized only to the graft in autosomal dominant polycystic kidney disease with thrombophilia

    Science.gov (United States)

    Rolla, Davide; Fontana, Iris; Ravetti, Jean Louis; Marsano, Luigina; Bellino, Diego; Panaro, Laura; Ansaldo, Francesca; Mathiasen, Lisa; Storace, Giulia; Trezzi, Matteo

    2015-01-01

    Introduction: Thrombotic microangiopathy (TMA) is a serious complication of renal transplantation and is mostly related to the prothrombotic effect of calcineurin inhibitors (CNIs). A subset of TMA (29%-38%) is localized only to the graft. Case 1: A young woman suffering from autosomal dominant polycystic kidney disease (ADPKD) underwent kidney transplant. After 2 months, she showed slow renal deterioration (serum creatinine from 1.9 to 3.1 mg/dl), without hematological signs of hemolytic-uremic syndrome (HUS); only LDH enzyme transient increase was detected. Renal biopsy showed TMA: temporary withdraw of tacrolimus and plasmapheresis was performed. The renal function recovered (serum creatinine 1.9 mg/dl). From screening for thrombophilia, we found a mutation of the Leiden factor V gene. Case 2: A man affected by ADPKD underwent kidney transplantation, with delay graft function; first biopsy showed acute tubular necrosis, but a second biopsy revealed TMA, while no altered hematological parameters of HUS was detected. We observed only a slight increase of lactate dehydrogenase (LDH) levels. The tacrolimus was halved and plasmapheresis was performed: LDH levels normalized within 10 days and renal function improved (serum creatinine from 9 to 2.9 mg/dl). We found a mutation of the prothrombin gene. Only a renal biopsy clarifies the diagnosis of TMA, but it is necessary to pay attention to light increasing level of LDH. Conclusion: Prothrombotic effect of CNIs and mTOR inhibitor, mutation of genes encoding factor H or I, anticardiolipin antibodies, vascular rejection, cytomegalovirus infection are proposed to trigger TMA; we detected mutations of factor II and Leiden factor V, as facilitating conditions for TMA in patients affected by ADPKD. PMID:26693501

  8. Thrombotic microangiopathies and acute kidney injury induced by artificial termination of pregnancy.

    Science.gov (United States)

    Wu, H; Zou, H B; Xu, Y; Zhang, L

    2014-01-01

    Thrombotic microangiopathy (TMA) is a rare, but potentially lethal condition requiring rapid recognition, diagnosis and initiation of therapy. Here, we present two cases of women with hemolytic anemia, thrombocytopenia and acute kidney injury shortly after surgical termination of pregnancy. Histological examination of their kidneys revealed endothelial cell swelling and luminal stenosis or fibrin-containing thrombi in the glomeruli and arterioles, which support the diagnosis of TMA. The patients were treated with hemodialysis, plasma infusion and corticosteroids with or without immunosuppressive agents. Three weeks after treatment, one patient was cured and symptoms of the other patient markedly improved. Reporting of more cases of TMA associated with surgical termination of pregnancy will provide further insights into this rare disease, possibly aiding in identifying risk factors and improving time to clinical diagnosis, treatment and prognosis.

  9. Immune Complex Mediated Glomerulonephritis with Acute Thrombotic Microangiopathy following Newly Detected Hepatitis B Virus Infection in a Kidney Transplant Recipient

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    Tracey Salter

    2016-01-01

    Full Text Available Hepatitis B virus (HBV presents a risk to patients and staff in renal units. To minimise viral transmission, there are international and UK guidelines recommending HBV immunisation for patients commencing renal replacement therapy (RRT and HBV surveillance in kidney transplant recipients. We report the case of a 56-year-old male who was immunised against HBV before starting haemodialysis. He received a deceased donor kidney transplant three years later, at which time there was no evidence of HBV infection. After a further six years he developed an acute kidney injury; allograft biopsy revealed an acute thrombotic microangiopathy (TMA with glomerulitis, peritubular capillaritis, and C4d staining. Due to a “full house” immunoprofile, tests including virological screening were undertaken, which revealed acute HBV infection. Entecavir treatment resulted in an improvement in viral load and kidney function. HBV genotyping demonstrated a vaccine escape mutant, suggesting “past resolved” infection that reactivated with immunosuppression, though posttransplant acquisition cannot be excluded. This is the first reported case of acute HBV infection associated with immune complex mediated glomerulonephritis and TMA. Furthermore, it highlights the importance of HBV surveillance in kidney transplant recipients, which although addressed by UK guidelines is not currently practiced in all UK units.

  10. Immune Complex Mediated Glomerulonephritis with Acute Thrombotic Microangiopathy following Newly Detected Hepatitis B Virus Infection in a Kidney Transplant Recipient

    Science.gov (United States)

    Burton, Hannah; Douthwaite, Sam; Newsholme, William; Horsfield, Catherine

    2016-01-01

    Hepatitis B virus (HBV) presents a risk to patients and staff in renal units. To minimise viral transmission, there are international and UK guidelines recommending HBV immunisation for patients commencing renal replacement therapy (RRT) and HBV surveillance in kidney transplant recipients. We report the case of a 56-year-old male who was immunised against HBV before starting haemodialysis. He received a deceased donor kidney transplant three years later, at which time there was no evidence of HBV infection. After a further six years he developed an acute kidney injury; allograft biopsy revealed an acute thrombotic microangiopathy (TMA) with glomerulitis, peritubular capillaritis, and C4d staining. Due to a “full house” immunoprofile, tests including virological screening were undertaken, which revealed acute HBV infection. Entecavir treatment resulted in an improvement in viral load and kidney function. HBV genotyping demonstrated a vaccine escape mutant, suggesting “past resolved” infection that reactivated with immunosuppression, though posttransplant acquisition cannot be excluded. This is the first reported case of acute HBV infection associated with immune complex mediated glomerulonephritis and TMA. Furthermore, it highlights the importance of HBV surveillance in kidney transplant recipients, which although addressed by UK guidelines is not currently practiced in all UK units.

  11. Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT.

    Science.gov (United States)

    Laskin, B L; Goebel, J; Davies, S M; Khoury, J C; Bleesing, J J; Mehta, P A; Filipovich, A H; Paff, Z N; Lawrence, J M; Yin, H J; Pinkard, S L; Jodele, S

    2011-05-01

    Patients undergoing auto-SCT for neuroblastoma present a unique population to study transplant-associated thrombotic microangiopathy (TA-TMA), due to standardized chemotherapy and later exposure to radiation and cis-retinoic acid (cis-RA). We retrospectively analyzed 20 patients after auto-SCT to evaluate early clinical indicators of TA-TMA. A total of 6 patients developing TA-TMA (30% prevalence) were compared with 14 controls. Four of six patients were diagnosed with TA-TMA by 25 days after auto-SCT. Compared with controls, TA-TMA patients had higher average systolic and diastolic blood pressure levels during high-dose chemotherapy and developed hypertension by day 13 after auto-SCT. Proteinuria was a significant marker for TA-TMA, whereas blood and platelet transfusion requirements were not. Serum creatinine did not differ between groups post transplant. However, patients with TA-TMA had a 60% decrease in renal function from baseline by nuclear glomerular filtration rate, compared with a 25% decrease in those without TA-TMA (P=0.001). There was no TA-TMA-related mortality. Significant complications included end-stage renal disease (n=1) and polyserositis (n=3). Patients with TA-TMA were unable to complete cis-RA therapy after auto-SCT. We suggest that careful attention to blood pressure and urinalysis will assist in the early diagnosis of TA-TMA, whereas serum creatinine seems to be an insensitive marker for this condition.

  12. Tacrolimus-induced thrombotic microangiopathy in orthotopic liver transplant patients: case series of four patients.

    Science.gov (United States)

    Nwaba, A; MacQuillan, G; Adams, L A; Garas, G; Delriviere, L; Augustson, B; DeBoer, B; Moody, H; Jeffrey, G P

    2013-03-01

    Thrombotic microangiopathy (TMA) is a potentially fatal complication in solid organ and bone marrow transplant patients, with reported incidence of 0.5-3% and mortality of about 75%. To emphasise the importance of early diagnosis and prompt commencement of therapy results in improved clinical outcomes. A retrospective study of all patients who underwent orthotopic liver transplantation (OLTX) at the Western Australian Liver Transplantation Service from May 1994 to December 2010 was conducted to identify patients who developed tacrolimus-induced TMA. We identified four patients with tacrolimus-induced TMA post-OLTX, derived from a cohort of 104 patients treated with tacrolimus in our institution. The mean age at diagnosis was 40 years, and the mean time of onset was 63 ± 7.5 weeks after OLTX. The indications for OLTX in the four patients were fulminant hepatic failure in three (Wilson disease, paracetamol overdose and post-partum thrombotic thrombocytopenic purpura) and hepatitis C virus-related cirrhosis. All patients had tacrolimus post-OLTX. At diagnosis, tacrolimus was discontinued in all patients, and three of the four patients underwent plasma exchange and all patients improved clinically. Mean duration of follow up was 15 ± 7.5 months. There was no mortality 6 months post-TMA. Early diagnosis with immediate discontinuation or conversion of calcineurin inhibitors and plasma exchange should be offered to OLTX patients with TMA as it results in good outcomes. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

  13. A Case of the nephrotic syndrome in bone marrow transplantation recipient, histologically showing overlapped glomerular lesions of thrombotic microangiopathy and membranous nephropathy.

    Science.gov (United States)

    Masuzawa, Naoko; Nishimura, Ayako; Kitani, Takashi; Tamagaki, Keiichi; Sugitani, Mio; Nagoshi, Hisao; Kuroda, Junya; Konishi, Eiichi

    2017-09-20

    Nephrotic syndrome (NS) rarely occurs in post-hematopoietic stem cell transplantation (HSCT) recipients but represents the renal manifestation of graft-versus-host disease (GVHD). Membranous nephropathy (MN) accounts for almost two thirds of post-HSCT NS and is caused by immune complex deposition. Renal thrombotic microangiopathy (TMA) without fulfillment of clinical criteria for TMA has been underreported because of reduced opportunity for histological examination. However, renal TMA has recently been reported in association with GVHD and humoral immunological reactions. Although both MN and TMA after HSCT are associated with GVHD and immunological abnormalities, these diseases are exceptionally coexistent in renal biopsy specimens. We herein describe a case of post-HSCT NS, histologically showing overlapped lesions of TMA and MN. Renal biopsy specimen after presentation of NS revealed early stage MN and TMA with evidence of chronicity. TMA was thought to have preceded MN, and renal biopsy at the phase of pre-nephrotic proteinuria might reveal earlier histological changes of isolated renal TMA. Detection of subclinical renal TMA earlier by spontaneous renal biopsy can help prevent progression of renal injury or overlapping of other renal pathologies. We also demonstrated Th2 predominant intraglomerular infiltration of lymphocytes by immunohistochemistry. © 2017 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

  14. Intestinal thrombotic microangiopathy following reduced-intensity umbilical cord blood transplantation.

    Science.gov (United States)

    Narimatsu, H; Kami, M; Hara, S; Matsumura, T; Miyakoshi, S; Kusumi, E; Kakugawa, Y; Kishi, Y; Murashige, N; Yuji, K; Masuoka, K; Yoneyama, A; Wake, A; Morinaga, S; Kanda, Y; Taniguchi, S

    2005-09-01

    Thrombotic microangiopathy (TMA) is a significant complication after hematopoietic stem-cell transplantation (HSCT); however, there is little information on it following reduced-intensity cord blood transplantation (RI-CBT). We reviewed the medical records of 123 adult patients who received RI-CBT at Toranomon Hospital between January 2002 and August 2004. TMA was diagnosed in seven patients based on intestinal biopsy (n = 6) or autopsy results (n = 1). While these patients showed some clinical symptoms such as diarrhea and/or abdominal pain, mental status alterations or neurological disorders were not observed in any of them. Laboratory results were mostly normal at the onset of TMA; >2% fragmented erythrocytes (n = 1), 200 IU/dl lactic dehydrogenase (LD) (n = 4). On endoscopic examination, TMA lesions, consisting of ulcers, erosions, and diffuse exfoliation, were distributed spottily from terminal ileum to rectum. Intestinal graft-versus-host disease (GVHD) and cytomegalovirus (CMV) colitis were confirmed in five and four patients, respectively. With therapeutic measures including supportive care (n = 4), fresh frozen plasma (n = 1), and a reduction of immunosuppressive agents (n = 1), TMA improved in four patients. The present study demonstrates that intestinal TMA is a significant complication after RI-CBT. Since conventional diagnostic criteria can overlook TMA, its diagnosis requires careful examination of the gastrointestinal tract using endoscopy with biopsy.

  15. Pulmonary Tumor Thrombotic Microangiopathy Induced by Ureteral Carcinoma: A Necropsy Case Report

    Directory of Open Access Journals (Sweden)

    Satoshi Marumo

    2014-08-01

    Full Text Available Background: Pulmonary tumor thrombotic microangiopathy (PTTM is a fatal cancer-related pulmonary complication with rapidly progressing dyspnea and pulmonary hypertension that occasionally induces sudden death. We report the first case of PTTM induced by ureter carcinoma. Case Presentation: The patient was an 80-year-old Japanese female with chief complaints of dry cough and dyspnea. An echocardiogram revealed severe pulmonary hypertension. A chest radiograph showed ground glass opacity of bilateral lower lung field predominance, and an abdominal computed tomography scan revealed a left ureter mass suggestive of ureter carcinoma. The patient died of respiratory failure on the eighth day of hospitalization. Postmortem examination indicated that the primary lesion was a left ureter cancer with tumor microemboli extending to both lungs through the right side of the heart. The final diagnosis of this case was PTTM induced by ureter carcinoma. Conclusion: The pathogenesis and pathophysiology of PTTM remains obscure with no effective management available. In cases of rapidly progressing respiratory failure with pulmonary hypertension, it is necessary to consider PTTM in the differential diagnosis.

  16. Type I interferon causes thrombotic microangiopathy by a dose-dependent toxic effect on the microvasculature

    Science.gov (United States)

    Kavanagh, David; Jury, Alexa; Williams, Jac; Scolding, Neil; Bellamy, Chris; Gunther, Claudia; Ritchie, Diane; Gale, Daniel P.; Kanwar, Yashpal S.; Challis, Rachel; Buist, Holly; Overell, James; Weller, Belinda; Flossmann, Oliver; Blunden, Mark; Meyer, Eric P.; Krucker, Thomas; Evans, Stephen J. W.; Campbell, Iain L.; Jackson, Andrew P.; Chandran, Siddharthan

    2016-01-01

    Many drugs have been reported to cause thrombotic microangiopathy (TMA), yet evidence supporting a direct association is often weak. In particular, TMA has been reported in association with recombinant type I interferon (IFN) therapies, with recent concern regarding the use of IFN in multiple sclerosis patients. However, a causal association has yet to be demonstrated. Here, we adopt a combined clinical and experimental approach to provide evidence of such an association between type I IFN and TMA. We show that the clinical phenotype of cases referred to a national center is uniformly consistent with a direct dose-dependent drug-induced TMA. We then show that dose-dependent microvascular disease is seen in a transgenic mouse model of IFN toxicity. This includes specific microvascular pathological changes seen in patient biopsies and is dependent on transcriptional activation of the IFN response through the type I interferon α/β receptor (IFNAR). Together our clinical and experimental findings provide evidence of a causal link between type I IFN and TMA. As such, recombinant type I IFN therapies should be stopped at the earliest stage in patients who develop this complication, with implications for risk mitigation. PMID:27663672

  17. A Case of Recurrent Breast Cancer Identified by Pulmonary Tumor Thrombotic Microangiopathy

    Directory of Open Access Journals (Sweden)

    Tomomi Abe

    2017-07-01

    Full Text Available Pulmonary tumor thrombotic microangiopathy (PTTM is a rare, cancer-related, pulmonary complication that causes hypoxia and pulmonary hypertension. We report on a 42-year-old woman who was diagnosed with recurrent breast cancer that was detected due to the presence of PTTM. Eleven months after surgery for heterochronous bilateral cancer of the left breast, she developed progressive dyspnea but computerized tomography showed no pulmonary thromboembolism, and a transthoracic echocardiography revealed mild pulmonary hypertension. She was diagnosed with PTTM by cytology from pulmonary artery catheterization and perfusion lung scintigraphy. Also, the patients complained of back pain after admission, bone scintigraphy showed multiple bone metastases. Despite the early diagnosis of PTTM, her platelet count decreased, her performance status rapidly deteriorated, and her dyspnea worsened. Thus, we could not treat her with chemotherapy. She died due to respiratory failure 19 days after admission. To the best of our knowledge, this is the first report of recurrent breast cancer identified by the manifestation of PTTM. Although PTTM is a rare phenomenon, it should be considered in the differential diagnosis of acute dyspnea or pulmonary hypertension in patients with breast cancer. Furthermore, upon diagnosis, the patient should be referred to a cardiologist as soon as possible.

  18. The thrombotic microangiopathy Registry of North America: A United States multi-institutional TMA network.

    Science.gov (United States)

    Metjian, Ara; Tanhehco, Yvette C; Aqui, Nicole; Bhoj, Vijay G; Jamensky, Lita; Marques, Marisa B; Onwuemene, Oluwatoyosi; Pham, Huy P; Arepally, Gowthami M

    2016-10-01

    The thrombotic microangiopathy (TMA) Registry Network of North America (TRNA) is a collaborative network organized for the purpose of developing a multi-institutional registry and network to conduct clinical studies in a rare patient population. The TRNA was founded in 2013 by four academic medical centers (Columbia University Medical Center, Duke University Medical Center, University of Alabama at Birmingham, and University of Pennsylvania) to develop a national and demographically diverse dataset of patients with TMA. A clinical database was developed by network members using REDCap (Research Electronic Data Capture), a web-based database developed for clinical research. To facilitate rapid Institutional Review Board (IRB) approval at multiple sites, the TRNA utilized IRBshare, a streamlined IRB process to allow patient recruitment and enrollment into the TMA registry. This article reviews the process used to establish the TRNA network and discusses the significance of the first multi-institutional clinical apheresis network developed in the United States. J. Clin. Apheresis 31:448-453, 2016. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

  19. Using rheopheresis for stem cell transplantation-associated thrombotic microangiopathy (TA-TMA).

    Science.gov (United States)

    González-Vicent, Marta; Herrero, Blanca; Guillén, María; Sevilla, Julián; Díaz, Miguel Ángel

    2013-10-01

    Stem cell Transplantation-Associated Thrombotic Microangiopathy (TA-TMA) is an awful complication with high morbidity and mortality. The reported incidence varies from 0.5% to 75% due to the difficulty of diagnosis in these patients. They do not respond to plasma exchange and despite new treatments, such as defibrotide and rituximab, mortality rate ranges between 60% and 90%. Rheopheresis is a specific application of membrane differential filtration for extracorporeal hemorheotherapy that has been used in the diabetic foot syndrome, venous leg ulcer, pulmonary hypertension, sudden hearing loss, macula degeneration and peripheral arterial occlusive disease. The main therapeutic basis of Rheopheresis is the reduction of blood and plasma viscosity that results in improvements of microcirculation and blood flow. The physiopathologic mechanism associated with TA-TMA is the loss of endothelial cell integrity with hypercoagulability secondary to infections, immunosuppressive therapy and graft-versus-host disease. Because of this, we believe that treatment with Rheoapheresis may improve microcirculation and resolve TA-TMA. We report two patients diagnosed of severe TA-MA successfully treated with Rheopheresis using a selective fibrinogen adsorption system (Rheosorb) with a LIFE-18 apheresis unit (Miltenyi Biotec), an integrated plasma therapy instrument. Copyright © 2013 Elsevier Ltd. All rights reserved.

  20. Eculizumab Therapy in Children with Severe Hematopoietic Stem Cell Transplantation–Associated Thrombotic Microangiopathy

    Science.gov (United States)

    Jodele, Sonata; Fukuda, Tsuyoshi; Vinks, Alexander; Mizuno, Kana; Laskin, Benjamin L.; Goebel, Jens; Dixon, Bradley P.; Teusink, Ashley; Pluthero, Fred G.; Lu, Lily; Licht, Christoph; Davies, Stella M.

    2017-01-01

    We recently observed that dysregulation of the complement system may be involved in the pathogenesis of hematopoietic stem cell transplantation–associated thrombotic microangiopathy (HSCT-TMA). These findings suggest that the complement inhibitor eculizumab could be a therapeutic option for this severe HSCT complication with high mortality. However, the efficacy of eculizumab in children with HSCT-TMA and its dosing requirements are not known. We treated 6 children with severe HSCT-TMA using eculizumab and adjusted the dose to achieve a therapeutic level >99 μg/mL. HSCT-TMA resolved over time in 4 of 6 children after achieving therapeutic eculizumab levels and complete complement blockade, as measured by low total hemolytic complement activity (CH50). To achieve therapeutic drug levels and a clinical response, children with HSCT-TMA required higher doses or more frequent eculizumab infusions than currently recommended for children with atypical hemolytic uremic syndrome. Two critically ill patients failed to reach therapeutic eculizumab levels, even after dose escalation, and subsequently died. Our data indicate that eculizumab may be a therapeutic option for HSCT-TMA, but HSCT patients appear to require higher medication dosing than recommended for other conditions. We also observed that a CH50 level ≤ 4 complement activity enzyme units correlated with therapeutic eculizumab levels and clinical response, and therefore CH50 may be useful to guide eculizumab dosing in HSCT patients as drug level monitoring is not readily available. PMID:24370861

  1. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience.

    Science.gov (United States)

    Coppo, Paul; Schwarzinger, Michael; Buffet, Marc; Wynckel, Alain; Clabault, Karine; Presne, Claire; Poullin, Pascale; Malot, Sandrine; Vanhille, Philippe; Azoulay, Elie; Galicier, Lionel; Lemiale, Virginie; Mira, Jean-Paul; Ridel, Christophe; Rondeau, Eric; Pourrat, Jacques; Girault, Stéphane; Bordessoule, Dominique; Saheb, Samir; Ramakers, Michel; Hamidou, Mohamed; Vernant, Jean-Paul; Guidet, Bertrand; Wolf, Martine; Veyradier, Agnès

    2010-04-23

    Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA). In this context, the early identification of a severe, antibody-mediated, ADAMTS13 deficiency may allow to start targeted therapies such as B-lymphocytes-depleting monoclonal antibodies. To date, assays exploring ADAMTS13 activity require skill and are limited to only some specialized reference laboratories, given the very low incidence of the disease. To identify clinical features which may allow to predict rapidly an acquired ADAMTS13 deficiency, we performed a cross-sectional analysis of our national registry from 2000 to 2007. The clinical presentation of 160 patients with TMA and acquired ADAMTS13 deficiency was compared with that of 54 patients with detectable ADAMTS13 activity. ADAMTS13 deficiency was associated with more relapses during treatment and with a good renal prognosis. Patients with acquired ADAMTS13 deficiency had platelet count < 30 x 10(9)/L (adjusted odds ratio [OR] 9.1, 95% confidence interval [CI] 3.4-24.2, P<.001), serum creatinine level < or =200 micromol/L (OR 23.4, 95% CI 8.8-62.5, P<.001), and detectable antinuclear antibodies (OR 2.8, 95% CI 1.0-8.0, P<.05). When at least 1 criteria was met, patients with a severe acquired ADAMTS13 deficiency were identified with positive predictive value of 85%, negative predictive value of 93.3%, sensitivity of 98.8%, and specificity of 48.1%. Our criteria should be useful to identify rapidly newly diagnosed patients with an acquired ADAMTS13 deficiency to better tailor treatment for different pathophysiological groups.

  2. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience.

    Directory of Open Access Journals (Sweden)

    Paul Coppo

    Full Text Available Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA. In this context, the early identification of a severe, antibody-mediated, ADAMTS13 deficiency may allow to start targeted therapies such as B-lymphocytes-depleting monoclonal antibodies. To date, assays exploring ADAMTS13 activity require skill and are limited to only some specialized reference laboratories, given the very low incidence of the disease. To identify clinical features which may allow to predict rapidly an acquired ADAMTS13 deficiency, we performed a cross-sectional analysis of our national registry from 2000 to 2007. The clinical presentation of 160 patients with TMA and acquired ADAMTS13 deficiency was compared with that of 54 patients with detectable ADAMTS13 activity. ADAMTS13 deficiency was associated with more relapses during treatment and with a good renal prognosis. Patients with acquired ADAMTS13 deficiency had platelet count < 30 x 10(9/L (adjusted odds ratio [OR] 9.1, 95% confidence interval [CI] 3.4-24.2, P<.001, serum creatinine level < or =200 micromol/L (OR 23.4, 95% CI 8.8-62.5, P<.001, and detectable antinuclear antibodies (OR 2.8, 95% CI 1.0-8.0, P<.05. When at least 1 criteria was met, patients with a severe acquired ADAMTS13 deficiency were identified with positive predictive value of 85%, negative predictive value of 93.3%, sensitivity of 98.8%, and specificity of 48.1%. Our criteria should be useful to identify rapidly newly diagnosed patients with an acquired ADAMTS13 deficiency to better tailor treatment for different pathophysiological groups.

  3. Long-term outcomes of thrombotic microangiopathy treated with plasma exchange: A systematic review.

    Science.gov (United States)

    Thejeel, Bashiar; Garg, Amit X; Clark, William F; Liu, Aiden R; Iansavichus, Arthur V; Hildebrand, Ainslie M

    2016-06-01

    With the adoption of plasma exchange as standard treatment for thrombotic microangiopathy (TMA), more patients are surviving and long-term outcomes have greater relevance. We conducted a systematic review to synthesize and evaluate the quality of evidence on long-term outcomes of TMA among adults treated with plasma exchange and to identify factors that may be associated with a worse long-term prognosis. We searched databases from 1980 to 2013 for eligible articles published in any language. We included studies that reported outcomes in at least ten adults with a history of TMA treated with plasma exchange and at least 6 months of follow-up. We abstracted data in duplicate and assessed the methodological quality of each study using an assessment tool developed based on recommended validity criteria. We screened 6672 articles, reviewed 213, and included 34 studies totaling 1182 patients (study median [range], 24 [10-118]). The mean (or median) follow-up ranged from 6 months to 13 years. The cumulative incidence of relapse and mortality was highly variable and ranged from 3 to 84 and 0 to 61%, respectively. The incidence of other outcomes across 10 studies also varied (outcomes included hypertension, kidney disease, preeclampsia, stroke, seizure, severe cognitive impairment, and depression); in three other studies, long-term neurocognitive function and health-related quality of life were significantly lower than in the general population. Patients who survive an episode of TMA may be susceptible to long-term vascular complications, but the magnitude of this risk and how to mitigate it remains unclear. Am. J. Hematol. 91:623-630, 2016. © 2016 Wiley Periodicals, Inc.

  4. Thrombotic Microangiopathy in Inverted Formin 2-Mediated Renal Disease

    DEFF Research Database (Denmark)

    Challis, Rachel C; Ring, Troels; Xu, Yaobo

    2017-01-01

    and her mother also had Charcot-Marie-Tooth disease. Using whole-exome sequencing, we identified a mutation in the inverted formin 2 gene (INF2) in the mutational hotspot for FSGS. Subsequent analysis of the Newcastle aHUS cohort identified another family with a functionally-significant mutation in INF2...

  5. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Naóe; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia.

  6. Thrombotic microangiopathy associated with bortezomib treatment in a patient with relapsed multiple myeloma

    Directory of Open Access Journals (Sweden)

    Urpu Salmenniemi

    2012-06-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP and hemolytic-uremic syndrome (HUS describe microvascular occlusive disorders characterized by thrombocytopenia due to increased platelet aggregation and fragmentation hemolysis. We report here what to our knowledge is the second case of TTP/HUS associated with bortezomib treatment.

  7. Thrombotic microangiopathy-like disorder after living-donor liver transplantation: A single-center experience in Japan

    Institute of Scientific and Technical Information of China (English)

    Tomohide Hori; Akira Mori; Hajime Segawa; Kimiko Yurugi; Yasutsugu Takada; Hiroto Egawa; Atsushi Yoshizawa; Takuma Kato; Kanako Saito; Linan Wang; Mie Torii; Toshimi Kaido; Feng Chen; Ann-Marie T Baine; Lindsay B Gardner; Shinji Uemoto; Fumitaka Oike; Yasuhiro Ogura; Kohei Ogawa; Yukihide Yonekawa; Koichiro Hata; Yoshiya Kawaguchi; Mikiko Ueda

    2011-01-01

    AIM: To investigate thrombotic microangiopathy (TMA) in liver transplantion, because TMA is an infrequent but life-threatening complication in the transplantation field.METHODS: A total of 206 patients who underwent living-donor liver transplantation (LDLT) were evaluated, and the TMA-like disorder (TMALD) occurred in seven recipients.RESULTS: These TMALD recipients showed poor outcomes in comparison with other 199 recipients.Although two TMALD recipients successfully recovered, the other five recipients finally died despite intensive treatments including repeated plasma exchange (PE) and re-transplantation.Histopathological analysis of liver biopsies after LDLT revealed obvious differences according to the outcomes.Qualitative analysis of antibodies against a disintegrin-like domain and metalloproteinase with thrombospondin type 1 motifs (ADAMTS-13) were negative in all patients.The fragmentation of red cells, the microhemorrhagic macules and the platelet counts were early markers for the suspicion of TMALD after LDLT.Although the absolute values of von Willebrand factor (vWF) and ADAMTS-13 did not necessarily reflect TMALD, the vWF/ADAMTS-13 ratio had a clear diagnostic value in all cases.The establishment of adequate treatments for TMALD, such as PE for ADAMTS-13 replenishment or treatments against inhibitory antibodies, must be decided according to each case.CONCLUSION: The optimal induction of adequate therapies based on early recognition of TMALD by the reliable markers may confer a large advantage for TMALD after LDLT.

  8. Successful early intervention for hyperacute transplant-associated thrombotic microangiopathy following pediatric hematopoietic stem cell transplantation.

    Science.gov (United States)

    Jodele, Sonata; Bleesing, Jack J; Mehta, Parinda A; Filipovich, Alexandra H; Laskin, Benjamin L; Goebel, Jens; Pinkard, Susan L; Davies, Stella M

    2012-03-01

    TA-TMA is a serious complication of hematopoietic stem cell transplantation, presenting as microangiopathic hemolytic anemia with severe renal injury and mortality as high as 60%. Diagnosis and treatment of TA-TMA is very challenging after HSCT because anemia, thrombocytopenia, hypertension, and renal impairment are multifactorial, leading to delayed recognition and management of this complication. We report a successful outcome following early intervention for hyperacute TA-TMA after allogeneic HSCT. © 2010 John Wiley & Sons A/S.

  9. Pernicious Anemia Associated Cobalamin Deficiency and Thrombotic Microangiopathy: Case Report and Review of the Literature

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    Farhanah Yousaf

    2017-01-01

    Full Text Available A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam. Presence of hemolytic anemia, schistocytosis, thrombocytopenia, and elevated lactate dehydrogenase (LDH was suggestive of thrombotic thrombocytopenic purpura (TTP. However, presence of leukopenia, macrocytes, and an inadequate reticulocyte response to the degree of anemia served as initial clues to an alternative diagnosis. Two and one units of packed red blood cells were transfused on day 1 and day 3, respectively. In addition, one unit of platelets was transfused on day 2. Daily therapeutic plasma exchange (TPE was initiated and continued until ADAMTS-13 result ruled out TTP. A low cobalamin (vitamin B12 level was evident at initial laboratory work-up and subsequent testing revealed positive intrinsic factor-blocking antibodies supporting a diagnosis of pernicious anemia with severe cobalamin deficiency. Hematological improvement was observed following vitamin B12 supplementation. The patient was discharged and markedly improved on day 9 with outpatient follow-up for cobalamin supplementation.

  10. Thrombotic microangiopathy after allogeneic stem cell transplantation in the era of reduced-intensity conditioning: The incidence is not reduced.

    Science.gov (United States)

    Shimoni, Avichai; Yeshurun, Moshe; Hardan, Izhar; Avigdor, Abraham; Ben-Bassat, Isaac; Nagler, Arnon

    2004-07-01

    Thrombotic microangiopathy (TMA) is one of the most severe complications of stem cell transplantation (SCT). Endothelial cell injury caused by the toxic effects of high-dose chemoradiotherapy is likely the primary event in pathogenesis. The incidence, clinical settings, and risk factors for TMA in the era of nonmyeloablative conditioning have not been well defined. The data on 147 consecutive SCTs in a single center were collected, and patients with TMA were identified. Patient characteristics, response to therapy, and outcome were recorded, and risk factors were determined. TMA occurred in 22 of 147 transplantations, with a projected incidence of 20% +/- 4%. TMA occurred in 3 clinical settings: classic multifactorial TMA, TMA associated with severe hepatic graft-versus-host disease (GVHD), and TMA associated with second SCT, with a projected incidence of 8% +/- 3%, 73% +/- 14%, and 70% +/- 16% of patients at risk, respectively. TMA occurred after 23% +/- 6% of nonmyeloablative and 16% +/- 5% of myeloablative conditioning regimens (not significant). Univariate analysis determined SCT from unrelated donors, SCT during advanced or active disease, second SCT within 6 months of a prior SCT, and acute GVHD as risk factors for TMA. The last 2 factors remained significant in a multivariate model. Thirty-two percent of patients responded to therapy. The peri-TMA mortality rate was 68% +/- 10%. Six patients had diffuse alveolar hemorrhage complicating TMA. SCT-associated TMA is a relatively common complication with unsatisfactory therapy and grim prognosis. Fludarabine-based nonmyeloablative conditioning does not confer a lesser risk for TMA. This observation may relate to the selective use of these regimens in elderly and heavily pretreated patients or to the lack of reduction of GVHD with these regimens, and fludarabine itself may be involved in causing endothelial damage. Further exploration of novel preventive and therapeutic measurements is required in high

  11. Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome: Case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Beatriz De-Leon-Bojorge; Samuel Zaltzman-Girsevich; Arturo Ortega-Salgado; Adelina Prieto-Patron; Ruth Córdoba-Córdoba; Marco Yamazaki-Nakashimada

    2006-01-01

    Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population. We describe a 17-year-old patient with SLE, who developed nephritis that was well controlled with medications. Initial treatment consisted of azathioprine, aspirin and prednisone with stable control of her symptoms. Two years later she developed a right quadrant abdominal pain,and an abdominal ultrasound revealed microlithiasic cholecystitis. Open cholecystectomy was performed and the histopathological findings revealed vasculitis with thrombotic microangiopathy in the gallbladder. This case presentation illustrates that calculous or acalculous cholecystitis should be considered as a manifestation of active SLE and APS.

  12. Clinical feature analysis of 24 children with thrombotic microangiopathy%小儿血栓性微血管病24例临床特征分析

    Institute of Scientific and Technical Information of China (English)

    王晓英; 郭红; 罗新辉

    2015-01-01

    Objective To explore the clinical feature and therapeutic strategy for children with thrombotic microangiopathy (TMA). Methods The clinical manifestation, auxiliary examination and treatment of 24 cases of children with TMA was analyzed retrospectively. Hemolysis urine toxin syndrome (HUS)occurred in 22 cases, and thrombotic thrombocytopenic purpura (TTP) occurred 2 cases. Results Sixteen cases was onset from May to July,and 8 cases was onset from September to November. Typical HUS (D+HUS) was 8 cases, and atypical HUS (D-HUS) was 14 cases. In 22 HUS children, 18 cases were given hemodialysis or peritoneal dialysis treatment. The illness were significantly improved, and the platelet count and renal function fully recovered normal. But 1 case appeared neurological symptoms such as headache, facial paralysis on one side, gastrointestinal bleeding, and fever, after getting better. Eventually the patient died of disseminated intravascular coagulation. Two cases of TTP were given plasma exchange , anti-infection, large dose of methylprednisolone and anti- platelet aggregation treatment . After treatment, the level of hemoglobin and blood platelets returned normal and consciousness was recuperated. Conclusions HUS and TTP are similar in pathogenesis and clinical manifestation,and it is necessary to be differentiated. Early diagnosis and proper treatment is the key to save the life of children with TMA. As soon as the diagnosis is clear, hemodialysis or peritoneal dialysis treatment should be given to HUS, and plasma exchange to TTP,to quickly control the condition and improve the clinical symptoms.%目的:探讨小儿血栓性微血管病(TMA)的临床特征、治疗方法及预后。方法对24例TMA患儿的临床表现、辅助检查及治疗方法进行回顾性分析。24例TMA患儿中,溶血尿毒综合征(HUS)22例,血栓性血小板减少性紫癜(TTP)2例。结果16例发病在5~7月,8例发病在9~11月。典型性HUS 8

  13. Eculizumab treatment in a patient with hematopoietic stem cell transplantation-associated thrombotic microangiopathy and steroid-refractory acute graft versus host disease

    Directory of Open Access Journals (Sweden)

    Cristina Fernández

    2015-12-01

    Full Text Available A 30-year-old man with acquired aplastic anemia underwent an HLA-identical bone marrow transplant. He developed a grade III acute graft versus host disease (GVHD refractory to various lines of treatment. On post-transplant day 196, he was diagnosed with stem cell transplantation-associated thrombotic microangiopathy (HSCT-TMA and he received treatment with eculizumab 900 mg iv weekly for 4 doses followed by a single dose of 1200 mg 2 weeks later. After the first dose of eculizumab, the patient ceased to require transfusions and a progressive improvement in analytical parameters for microangiopathy was observed until their complete normalization. Coinciding with the improved of HSCT-TMA, the patient presented a clear response to his acute GVHD with disappearance of the diarrhea and bilirubin normalization. He was discharged eight weeks after the start of treatment. Unfortunately, one month later, the patient was readmitted for a GVHD relapse and he died two weeks later by an acute respiratory distress syndrome. In our case, the rapid clinical and analytical response to early treatment with eculizumab supports the implication of the complement in HSCT-TMA and suggests that the drug has a beneficial effect when used as coadjuvant therapy in acute GVHD.

  14. Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative.

    Science.gov (United States)

    Bendapudi, Pavan K; Li, Ang; Hamdan, Ayad; Uhl, Lynne; Kaufman, Richard; Stowell, Christopher; Dzik, Walter; Makar, Robert S

    2015-12-01

    The Harvard TMA Research Collaborative is a multi-institutional registry-based effort to study thrombotic microangiopathies (TMA). Laboratory and clinical parameters were recorded for 254 cases of suspected autoimmune thrombotic thrombocytopenic purpura (TTP). Patients with severe ADAMTS13 deficiency (activity ≤10%, N = 68) were more likely to be young, female and without a history of cancer treatment or transplantation. While all patients with severe deficiency were diagnosed with autoimmune TTP, those without severe deficiency frequently had disseminated intravascular coagulation, drug-associated TMA and transplant-related TMA. Patients with severe ADAMTS13 deficiency had superior overall survival at 360 d compared to those without severe deficiency (93·0% vs. 47·5%, P 10% varied significantly across the institutions in our consortium (13·2-63·8%, P 10% between the three hospitals (P = 0·98). Our data show that patients with severe ADAMTS13 deficiency represent a clinically distinct cohort that responds well to TPE. In contrast, TMA without severe ADAMTS13 deficiency is associated with increased mortality that may not be influenced by TPE. © 2015 John Wiley & Sons Ltd.

  15. Microangiopatias trombóticas: púrpura trombocitopênica trombótica e síndrome hemolítico-urêmica Thrombotic microangiopathies: thrombotic thrombocytopenic purpura / hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Maria Goretti Polito

    2010-09-01

    complemento. Uma série de mutações e polimorfismo em genes que codificam proteínas reguladoras do complemento sozinhas ou em combinação podem levar a SHU atípica. Aproximadamente 60% dos casos de SHU atípica têm mutações do tipo "perda da função" em genes que codificam as proteínas reguladoras do complemento, as quais protegem as células hospedeiras da ativação do complemento: fator H do complemento (FHC, fator I (FIC e proteína cofator de membrana (PCM ou CD46, ou mutações do tipo "ganho da função" em genes que codificam o FHC ou C3. Além disso, aproximadamente 10% dos pacientes com SHU atípica têm deficiência na função do FHC devido a anticorpos anti-FHC. Mesmo que as MATs sejam condições altamente heterogêneas, um terço dos pacientes tem deficiência severa da ADA-MTS13. Transfusões de plaquetas são contraindicadas nesses pacientes. Infusão de plasma ou plasma exchange (PE é o único tratamento eficiente.Thrombotic microangiopathies (TMAs are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic- uremic syndrome (HUS and thrombotic thrombocytopenic purpura (TTP. Other disorders occasionally present with similar manifestations. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different disorders have been described: HUS and TTP. Injury to the endothelial cell is the central and likely inciting factor in the sequence of events leading to TMA. Loss of physiological thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, abnormal von Willebrand factor release and fragmentation, and increased vascular shear stress may then sustain and amplify the microangiopathic process. Intrinsic abnormalities of the complement system and of the von Willebrand factor pathway may account for a genetic predisposition to the

  16. Acute Renal Failure, Microangiopathic Haemolytic Anemia, and Secondary Oxalosis in a Young Female Patient

    OpenAIRE

    Stepien, Karolina M; Peter Prinsloo; Tony Hitch; McCulloch, Thomas A.; Rebecca Sims

    2011-01-01

    A 29-year old female presented with a one-week history of vomiting, diarrhoea, abdominal pain, and headache. On admission, she had acute renal failure requiring dialysis. Tests revealed a hemolytic anemia with thrombocytopenia. An initial diagnosis of thrombotic thrombocytopenic microangiopathy was made and plasma exchange was instigated. However, renal biopsy did not show thrombotic microangiopathy but instead revealed acute kidney injury with mild tubulointerstitial nephritis and numerous o...

  17. Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: Causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre.

    Science.gov (United States)

    Grall, Maximilien; Azoulay, Elie; Galicier, Lionel; Provôt, François; Wynckel, Alain; Poullin, Pascale; Grange, Steven; Halimi, Jean-Michel; Lautrette, Alexandre; Delmas, Yahsou; Presne, Claire; Hamidou, Mohamed; Girault, Stéphane; Pène, Frédéric; Perez, Pierre; Kanouni, Tarik; Seguin, Amélie; Mousson, Christiane; Chauveau, Dominique; Ojeda-Uribe, Mario; Barbay, Virginie; Veyradier, Agnès; Coppo, Paul; Benhamou, Ygal

    2017-01-30

    Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia. At admission, misdiagnosed patients were more frequently females (P = .034) with a history of autoimmune disorder (P = .017) and had organ involvement in 67% of cases; they had more frequently antinuclear antibodies (P = .035), a low/undetectable schistocyte count (P = .001), a less profound anemia (P = .008), and a positive direct antiglobulin test (DAT) (P = .008). In multivariate analysis, female gender (P = .022), hemoglobin level (P = .028), a positive DAT (P = .004), and a low schistocytes count on diagnosis (P < .001) were retained as risk factors of misdiagnosis. Platelet count recovery was significantly longer in the misdiagnosed group (P = .041) without consequence on mortality, exacerbation and relapse. However, patients in the misdiagnosed group had a less severe disease than those in the accurately diagnosed group, as evidenced by less organ involvement at TTP diagnosis (P = .006). TTP is frequently misdiagnosed with autoimmune cytopenias. A low schistocyte count and a positive DAT should not systematically rule out TTP, especially when associated with organ failure.

  18. Role of RAS inhibitor in the treatment of lupus nephritis with thrombotic microangiopathy%RAS抑制剂在狼疮肾炎伴血栓性微血管病中的应用

    Institute of Scientific and Technical Information of China (English)

    李超; 李航; 文煜冰; 王海云; 马杰; 王颖; 刘炳岩; 高瑞通; 陶建瓴

    2014-01-01

    Objective To investigate whether RAS inhibitors can improve renal function in the treatment of lupus nephritis (LN) with thrombotic microangiopathy (TMA).Methods A total of 15 LN patients with TMA proven by renal pathology,from January 2000 to December 2013 in PUMCH,were included.The serum creatinine (Scr) and blood pressure (BP) before and after using RAS inhibitors were analyzed.Results (1)Male/female ratio was 1∶ 14.All of the patients had renal dysfunction,and median peak value of Scr was 396 μmol/L (160 ~ 643 μmol/L).5 cases (33.3%) required acute dialysis during hospitalization.Hypertension occurred in 15 patients,while 6 cases (40.0%) were diagnosed malignant hypertension.(2) Anemia and thrombocytopenia occurred in 15 and 14 cases,respectively.Three cases (20.0%) were diagnosed MAHA definitely and 5 cases (33.3%) were diagnosed MAHA probably.(3) Renal biopsy showed class Ⅱ in 1 case,Ⅲ in 4 cases,Ⅳ-(G) in 2 cases,Ⅳ(S) in 5 cases and Ⅳ+Ⅴ in 3 cases.Active lesions were predominant in both glomeruli and renal vasculopathy.(4) All the patients received steroid and immunosuppressive therapy,of whom 9 cases were given steroid pulse therapy.Thirteen cases received cyclophosphamide,and the rest 2 cases received cyclophosphamide and mycophenolate.After steroid pulse therapy,there were only 5 patients (55.6%) who got decreased Scr.In 13 patients (86.7%),hypertension was ameliorated and Scr decreased within one week after implementing RAS inhibitors,which fell medianly 15.8% and 17.0%,respectively.(5) Eleven of the 15 patients were followed from 8 to 135 months (median 32 months),and the other 4 patients were lost.Five cases who was on dialysis during hospitalization became independent of renal replacement therapy,while the other cases also got improved renal function.Conclusions Patients of LN with TMA who develop AKI and refractory hypertension should be treated with RAS inhibitors.Improved renal survival and successful

  19. A single-center prospective study on the safety of plasma exchange procedures using a double-viral-inactivated and prion-reduced solvent/detergent fresh-frozen plasma as the replacement fluid in the treatment of thrombotic microangiopathy.

    Science.gov (United States)

    Vendramin, Chiara; McGuckin, Siobhan; Alwan, Ferras; Westwood, John-Paul; Thomas, Mari; Scully, Marie

    2017-01-01

    Patients presenting with acute episodes of thrombotic microangiopathies (TMAs) require urgent access to plasma exchange (PEX). OctaplasLG, a solvent/detergent fresh-frozen plasma product that has undergone viral inactivation and prion reduction step, has been used in our institution since 2013, replacing Octaplas. We prospectively reviewed 981 PEX procedures where OctaplasLG was the replacement fluid in 90 patients admitted acutely with a TMA presentation within our institution from January 1, 2013, to December 31, 2015. We recorded citrate toxicities, plasma reactions, viral transfer, complications related to central venous catheter, and venous thrombotic events (VTEs). Citrate toxicities were 5.4%, plasma reactions were 2%, and all were classified as Grade 1 or 2. VTE had an incidence of 12.2%, although 50% of the episodes occurred in early remission when patients were not receiving PEX. No line insertions complications were recorded. Line-associated infections were 2.2%. Hepatitis B and C serology and human immunodeficiency virus (HIV) were checked on admission. There were four patients who may have had passive transient transfer of hepatitis B antibodies from pooled plasma. No hepatitis C or HIV viral transfer was documented after treatment and no seroconversion was detected after treatment. Our data have demonstrated that the incidence of complications during PEX is low and using OctaplasLG is comparable to the low incidence of reactions. No cases of anaphylaxis, transfusion-related acute lung injury, or fatal plasma reactions were seen. There was no evidence of viral transmission or seroconversion after treatment. © 2016 AABB.

  20. Chikungunya Fever Presenting as Life Threatening Thrombotic Thrombocytopenic Purpura.

    Science.gov (United States)

    Kumar, Vimal; Jain, Rujul; Kumar, Arvind; Nischal, Neeraj; Jorwal, Pankaj; Soneja, Manish; Arava, Sudheer; Wig, Naveet

    2017-07-01

    It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic thrombocytopenic purpura leading to multiple areas of skin necrosis, peripheral digital gangrene, haemolytic anemia, renal failure and severe thrombocytopenia with bleeding. This complication was most likely due to inhibitor autoantibody formation against ADAMTS13 triggered by chikungunya virus leading to thrombotic thrombocytopenic purpura. Patient was treated with plasmapheresis and other supportive careto which she responded. Her symptoms subsided, and she is symptom free and leading normal life in her follow up visits. © Journal of the Association of Physicians of India 2011.

  1. [Thrombotic occlusion of the terminal aorta associated to a renal fusion and pelvic location ("pancake" kidney). First case report].

    Science.gov (United States)

    Martins, Carlos; Moura, Carlos; Almeida, Paulo; Dinis da Gama, A

    2006-01-01

    Coexistence of thrombotic occlusion of the terminal aorta with a "pancake" kidney is reported in 63-year old man, diagnosed by CT-scans and selective angiography, to assess the renal circulation and for surgical planning. Surgical exposure was transperitoneal and revascularization was accomplished through a bifurcation Dacron graft from the supraceliac aorta to both femorals, associated to the renal revascularization, by means of PTFE graft from the prosthesis to the renal artery. The success of procedure was later demonstrated by angio-RM, showing both grafts working in excellent condition. According to an extensive bibliographic review, this seems to be the first case reporting the association of a thrombotic occlusion of the terminal aorta to a "pancake" kidney, thus justifying its presentation and divulgation.

  2. Renal thrombotic microangiopathy in patients with cblC defect : review of an under-recognized entity

    NARCIS (Netherlands)

    Beck, Bodo B; van Spronsen, FrancJan; Diepstra, Arjan; Berger, Rolf M F; Kömhoff, Martin

    2016-01-01

    Methylmalonic aciduria and homocystinuria, cobalamin C (cblC) type, is the most common genetic type of functional cobalamin (vitamin B12) deficiency. This metabolic disease is characterized by marked heterogeneity of neurocognitive disease (microcephaly, seizures, developmental delay, ataxia, hypoto

  3. Renal thrombotic microangiopathy in patients with cblC defect : review of an under-recognized entity

    NARCIS (Netherlands)

    Beck, Bodo B; van Spronsen, FrancJan; Diepstra, Arjan; Berger, Rolf M F; Kömhoff, Martin

    2017-01-01

    Methylmalonic aciduria and homocystinuria, cobalamin C (cblC) type, is the most common genetic type of functional cobalamin (vitamin B-12) deficiency. This metabolic disease is characterized by marked heterogeneity of neurocognitive disease (microcephaly, seizures, developmental delay, ataxia, hypot

  4. Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature.

    Science.gov (United States)

    Naniwa, Taio; Banno, Shogo; Sugiura, Yoshiki; Yokota, Kaori; Oosawa, Tomoyo; Maeda, Shinji; Hayami, Yoshihito; Takahashi, Nobuyuki; Ueda, Ryuzo; Matsumoto, Yoshifuji

    2007-01-01

    We describe three cases of acute renal failure with diffuse alveolar hemorrhage, which is designated pulmonary-renal syndrome (PRS), in systemic sclerosis (SSc) and review the literature to better define this rare but severe complication of SSc. The clinical course of three SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage are reported, and the literature published between 1967 and 2005 is reviewed following a PubMed search. Including our cases, a total of 19 SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage have been reported. Pulmonary-renal syndrome developing in SSc patients can be categorized clinicopathologically into three entities: PRS with thrombotic microangiopathy, PRS with small vessel vasculitides accompanied with SSc, and d-penicillamine-induced Goodpasture-like syndrome. Patients with scleroderma PRS with thrombotic microangiopathy, to which group our all patients belong, often developed diffuse alveolar hemorrhage after receiving high-dose corticosteroid therapy. Pulmonary-renal syndrome is a fatal complication of SSc and results from different pathogenic processes. Prompt differential diagnosis between the subsets is critical, because therapeutic strategy may differ in the use of high-dose corticosteroid and plasma exchange between the subsets of PRS. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy.

  5. Risk factors and clinical profile of thrombotic thrombocytopenic purpura in systemic lupus erythematosus patients. Is this a distinctive clinical entity in the thrombotic microangiopathy spectrum?: a case control study.

    Science.gov (United States)

    Merayo-Chalico, Javier; Demichelis-Gómez, Roberta; Rajme-López, Sandra; Aparicio-Vera, Luis; Barrera-Vargas, Ana; Alcocer-Varela, Jorge; Gómez-Martín, Diana

    2014-11-01

    The association of thrombotic thrombocytopenic purpura (TTP) with systemic lupus erythematosus (SLE) is rare. It is associated with high morbidity and mortality. Information about risk factors and clinical outcomes is scant. A retrospective case-control study was performed in a referral center in Mexico City between 1994 and 2013. Patients were diagnosed with TTP if they fulfilled the following criteria: microangiopathic haemolytic anaemia, thrombocytopenia, high LDH levels, normal fibrinogen and negative Coombs' test. Patients with SLE were diagnosed with ≥ 4 ACR criteria. We included three study groups: group A included patients with SLE-associated TTP (TTP/SLE; cases n = 22, TTP events n = 24); patients with non-autoimmune TTP (NA-TTP; cases n = 19, TTP events n = 22) were included in group B and patients with SLE without TTP (n = 48) in group C. After multivariate analysis, lymphopenia lupus patients hospitalised for other causes Lymphopenia is an independent risk factor for TTP/SLE. It is likely that patients with TTP/SLE present with less evident clinical features, so the level of suspicion must be higher to avoid delay in treatment. Copyright © 2014 Elsevier Ltd. All rights reserved.

  6. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy.

    Science.gov (United States)

    Chaturvedi, Swasti; Brandao, Leonardo; Geary, Denis; Licht, Christoph

    2011-06-01

    Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.

  7. Renal Failure in Pregnancy.

    Science.gov (United States)

    Balofsky, Ari; Fedarau, Maksim

    2016-01-01

    Renal failure during pregnancy affects both mother and fetus, and may be related to preexisting disease or develop secondary to diseases of pregnancy. Causes include hypovolemia, sepsis, shock, preeclampsia, thrombotic microangiopathies, and renal obstruction. Treatment focuses on supportive measures, while pharmacologic treatment is viewed as second-line therapy, and is more useful in mitigating harmful effects than treating the underlying cause. When supportive measures and pharmacotherapy prove inadequate, dialysis may be required, with the goal being to prolong pregnancy until delivery is feasible. Outcomes and recommendations depend primarily on the underlying cause.

  8. Renal involvement in antiphospholipid syndrome.

    Science.gov (United States)

    Pons-Estel, Guillermo J; Cervera, Ricard

    2014-02-01

    Renal involvement can be a serious problem for patients with antiphospholipid syndrome (APS). However, this complication has been poorly recognized and studied. It can be present in patients who have either primary or systemic lupus erythematosus-associated APS. Clinical and laboratory features of renal involvement in APS include hypertension, hematuria, acute renal failure, and progressive chronic renal insufficiency with mild levels of proteinuria that can progress to nephrotic-range proteinuria. The main lesions are renal artery stenosis, venous renal thrombosis, and glomerular lesions (APS nephropathy) that may be acute (thrombotic microangiopathy) and/or chronic (arteriosclerosis, arterial fibrous intimal hyperplasia, tubular thyroidization, arteriolar occlusions, and focal cortical atrophy). APS can also cause end-stage renal disease and allograft vascular thrombosis. This article reviews the range of renal abnormalities associated with APS, and their diagnosis and treatment options.

  9. Acute Renal Failure, Microangiopathic Haemolytic Anemia, and Secondary Oxalosis in a Young Female Patient

    Directory of Open Access Journals (Sweden)

    Karolina M. Stepien

    2011-01-01

    Full Text Available A 29-year old female presented with a one-week history of vomiting, diarrhoea, abdominal pain, and headache. On admission, she had acute renal failure requiring dialysis. Tests revealed a hemolytic anemia with thrombocytopenia. An initial diagnosis of thrombotic thrombocytopenic microangiopathy was made and plasma exchange was instigated. However, renal biopsy did not show thrombotic microangiopathy but instead revealed acute kidney injury with mild tubulointerstitial nephritis and numerous oxalate crystals, predominantly in the distal tubules. The patient had been taking large doses (>1100 mg daily of vitamin C for many months. She also gave a history of sclerotherapy using injections of an ethylene glycol derivative for superficial leg veins. The patient completed five sessions of plasma exchange and was able to discontinue dialysis. She eventually achieved full renal recovery. She has now discontinued sclerotherapy and vitamin supplementation.

  10. Acute Renal Failure, Microangiopathic Haemolytic Anemia, and Secondary Oxalosis in a Young Female Patient

    Science.gov (United States)

    Stepien, Karolina M.; Prinsloo, Peter; Hitch, Tony; McCulloch, Thomas A.; Sims, Rebecca

    2011-01-01

    A 29-year old female presented with a one-week history of vomiting, diarrhoea, abdominal pain, and headache. On admission, she had acute renal failure requiring dialysis. Tests revealed a hemolytic anemia with thrombocytopenia. An initial diagnosis of thrombotic thrombocytopenic microangiopathy was made and plasma exchange was instigated. However, renal biopsy did not show thrombotic microangiopathy but instead revealed acute kidney injury with mild tubulointerstitial nephritis and numerous oxalate crystals, predominantly in the distal tubules. The patient had been taking large doses (>1100 mg daily) of vitamin C for many months. She also gave a history of sclerotherapy using injections of an ethylene glycol derivative for superficial leg veins. The patient completed five sessions of plasma exchange and was able to discontinue dialysis. She eventually achieved full renal recovery. She has now discontinued sclerotherapy and vitamin supplementation. PMID:21785726

  11. Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations.

    Science.gov (United States)

    Rangel, M L; Alghamdi, I; Contreras, G; Harrington, T; Thomas, D B; Barisoni, L; Andrews, D; Wolf, M; Asif, A; Nayer, A

    2013-07-01

    Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.

  12. Cerebral microangiopathies; Zerebrale Mikroangiopathien

    Energy Technology Data Exchange (ETDEWEB)

    Linn, Jennifer [Klinikum der Universitaet Muenchen (Germany). Abt. fuer Neuroradiologie

    2011-03-15

    Cerebral microangiopathies are a very heterogenous group of diseases characterized by pathological changes of the small cerebral vessels. They account for 20 - 30 % of all ischemic strokes. Degenerative microangiopathy and sporadic cerebral amyloid angiography represent the typical acquired cerebral microangiopathies, which are found in over 90 % of cases. Besides, a wide variety of rare, hereditary microangiopathy exists, as e.g. CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy), Fabrys disease and MELAS syndrome (Mitochondrial myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes). (orig.)

  13. Acute renal failure and severe rhabdomyolysis in a patient with resistant thrombotic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Al Qahtani S

    2011-10-01

    Full Text Available Saad Al Qahtani Intensive Care Department, Critical Care Response Team, King Abdulaziz Medical City (KAMC, National Guard Health Affairs; King Saud Bin Abdulaziz University for Health Sciences, College of Medicine, Riyadh, Kingdom of Saudi Arabia Abstract: Thrombotic thrombocytopenic purpura (TTP is a rare, life-threatening disorder. This paper describes the case of a 39-year-old Sudanese male who presented to the emergency room with fever, jaundice, decreased level of consciousness, and worsening kidney function for 7 days, a high lactate dehydrogenase level (1947, severe thrombocytopenia (platelets 8, and numerous schistocytes in the peripheral blood smear. The patient was admitted with a diagnosis of TTP for plasma exchange. Fourteen days later, his creatinine kinase (CK level rose to >50,000 IU; rhabdomyolysis was suggested. Continuous venovenous hemodialysis (CVVHD was started. The patient's CK level remained high, despite CVVHD, until the 6th day, after which this parameter gradually started to decrease. This report highlights a resistant case of TTP that presented with concomitant severe rhabdomyolysis, which demanded aggressive, continuous intervention. Keywords: TTP, CVVHD, continuous venovenous hemodialysis

  14. Renal venous thrombosis in an infant with predisposing thrombotic factors: color Doppler ultrasound and MR evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Argyropoulou, Maria I.; Papadopoulou, Frederica; Nikolopoulos, Pangiotis [Department of Radiology, Medical School, University of Ioannina, 45110, Ioannina (Greece); Giapros, Vassilios I.; Drougia, Aikaterini A.; Andronikou, Styliani [Neonatology Clinic, Medical School, University of Ioannina, 45110, Ioannina (Greece); Vartholomatos, Georgios A. [Department of Haematology, Medical School, University of Ioannina, 45110, Ioannina (Greece)

    2003-08-01

    We report a case of a neonate with hereditary thrombophilia presenting with renal venous thrombosis (RVT). Early color Doppler findings of RVT were lacking venous flow, and the arterial diastolic flow was reversed. This very high-resistance arterial flow is for the first time described in neonatal RVT. Magnetic resonance imaging showed low signal intensity of the renal pyramids on T1- and T2-weighted images, suggesting acute hemorrhage. After intravenous contrast injection, persistent cortical enhancement was observed along with lack of medullary enhancement. Despite the progressive reestablishment of some venous drainage, the kidney showed atrophy and loss of function. (orig.)

  15. Scleroderma Renal Crisis: A Pathology Perspective

    Directory of Open Access Journals (Sweden)

    Ibrahim Batal

    2010-01-01

    Full Text Available Scleroderma renal crisis (SRC is an infrequent but serious complication of systemic sclerosis (SSc. It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.

  16. Rare presentations of cytomegalovirus infection in renal allograft recipients.

    Science.gov (United States)

    Ardalan, Mohammadreza

    2012-01-01

    Cytomegalovirus is the most common viral infection after kidney transplantation. Clinical presentations of cytomegalovirus infection range from asymptomatic infection to organ-specific involvement. Most symptomatic infections manifest as fever and cytopenia. The gastrointestinal tract is the most common site of tissue-invasive infection, often presenting as diarrhea or gastrointestinal bleeding. Gastrointestinal obstruction, perforation, thrombosis of large gastrointestinal veins, splenic artery thrombosis, and pancreatitis are rare gastrointestinal presentations of cytomegalovirus infection. Renal-allograft ureteral stricture and skin involvement are other rare presentations of cytomegalovirus infection. hemophagocytic syndrome, thrombotic microangiopathy, adrenal insufficiency, and renal allograft artery stenosis are other rare symptoms of cytomegalovirus infection.

  17. Thrombotic microangiopathic syndromes associated with drugs, HIV infection, hematopoietic stem cell transplantation and cancer.

    Science.gov (United States)

    George, James N; Terrell, Deirdra R; Vesely, Sara K; Kremer Hovinga, Johanna A; Lämmle, Bernhard

    2012-03-01

    Thrombotic microangiopathy (TMA) has multiple etiologies. In the four disorders described in this review, the primary organ involved is the kidney. Drug-associated TMA can be an acute, immune-mediated disorder or the result of gradual, dose-dependent toxicity. TMA may occur in patients with advanced HIV infection, possibly mediated by angio-invasive infections. TMA following allogeneic hematopoietic stem cell transplantation may also be caused by drug toxicity; the pathogenesis may involve inhibition of vascular endothelial cell growth factor in renal podocytes. Malignancies of many types with systemic microvascular involvement may cause TMA. Recognition that these syndromes may mimic TTP is important to provide appropriate management and to avoid the inappropriate use of plasma exchange treatment.

  18. Eculizumab: safety and efficacy after 17 months of treatment in a renal transplant patient with recurrent atypical hemolytic-uremic syndrome: case report.

    Science.gov (United States)

    Châtelet, V; Lobbedez, T; Frémeaux-Bacchi, V; Ficheux, M; Ryckelynck, J Ph; Hurault de Ligny, B

    2010-12-01

    In a recent study, eculizumab, a humanized monoclonal antibody which targets complement factor C5, appeared to resolve hemolysis and thrombocytopenia leading to recovery of renal function in a transplant patient during an episode of an atypical hemolytic uremic syndrome. We report the efficacy of eculizumab in a patient who presented with a recurrence of atypical hemolytic syndrome at 3 years after renal transplantation. After 17 months of eculizumab treatment, and without concomitant plasma therapy, renal function was maintained, the need for blood transfusions reduced, and acute thrombotic microangiopathy and hemolysis controlled. These data suggested that eculizumab should be considered to be a permanent treatment for this patient.

  19. Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.

    Directory of Open Access Journals (Sweden)

    Masanori Matsumoto

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a type of thrombotic microangiopathy (TMA. Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai ADAMTS13-deficient TTP (ADAMTS13 activity <5% were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01, while older patients were more likely to die during the TTP hospitalization (p<0.05. Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort and gender composition (60% to 100% female in the other cohort. We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.

  20. An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome).

    Science.gov (United States)

    Esmaili, Haydarali; Mostafidi, Elmira; Mehramuz, Bahareh; Ardalan, Mohammadreza; Mohajel-Shoja, Mohammadali

    2016-01-01

    Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation.

  1. Thrombotic microangiopathies and acute kidney injury induced by ...

    African Journals Online (AJOL)

    2013-07-29

    Jul 29, 2013 ... Three weeks after treatment, one patient was cured and symptoms of the other patient ... rare disease, possibly aiding in identifying risk factors and improving time ... treated with hemodialysis, infusion of plasma and high‑dose.

  2. De Novo Fibrillary Glomerulonephritis (FGN in a Renal Transplant with Chronic Hepatitis C

    Directory of Open Access Journals (Sweden)

    Edward J. Filippone

    2013-01-01

    Full Text Available Chronic hepatitis C viremia (HepC has been associated with numerous renal manifestations both in native kidneys and in the setting of renal transplantation. Glomerulonephritis (GN of the renal allograft in the setting of HepC most commonly manifests as type 1 membranoproliferative GN (MPGN, either representing recurrence of the original disease or arising de novo. Other GNs were reported after transplantation in the patient with HepC including membranous nephropathy and thrombotic microangiopathy, as well as an enhanced susceptibility to transplant glomerulopathy. We describe the first case of de novo fibrillary GN in a renal transplant patient with HepC where the primary renal disease was biopsy proven type 1 MPGN. We discuss this relationship in detail.

  3. Thrombotic thrombocytopenic purpura following salvage chemotherapy with paclitaxel, ifosfamide and cisplatin in a patient with a refractory germ cell tumor: A case report and review of the literature

    Science.gov (United States)

    ULAS, ARIFE; SILAY, KAMILE; AKINCI, SEMA; AKINCI, MUHAMMED BULENT; SENDUR, MEHMET ALI; DEDE, DIDEM SENER; POLAT, YUNUS HALIL; YALCIN, BULENT

    2015-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy that is characterized by microvascular thrombosis, thrombocytopenia, hemolysis and end organ damage. An extensive variety of drugs, including certain chemotherapeutic agents, have been associated with TTP. However, paclitaxel, cisplatin and ifosfamide regimen (TIP)-induced TTP has not previously been described. The present study reports the case of a 43-year-old patient with a refractory testicular germ cell tumor who developed acute TTP during TIP chemotherapy. Following the third cycle of TIP chemotherapy, the patient developed fever, anemia, thrombocytopenia and confusion. A diagnosis of TTP was established. Plasmapheresis was initiated as daily treatment in the first week, then continued every other day for 4 weeks. TIP chemotherapy was discontinued. The patient's clinical and neurological symptoms improved markedly after a week. Renal function and hemolysis improved, and the patient was discharged in a stable condition. The patient did not develop any complications and has been in remission for 5 months. The Naranjo adverse drug reaction probability scale indicated a likely association between TTP and the TIP chemotherapy regimen in this patient. This case is also investigated with regard to the associated literature to increase the awareness of TTP following chemotherapy. PMID:26622823

  4. Regenerative Therapies for Diabetic Microangiopathy

    Directory of Open Access Journals (Sweden)

    Roberto Bassi

    2012-01-01

    Full Text Available Hyperglycaemia occurring in diabetes is responsible for accelerated arterial remodeling and atherosclerosis, affecting the macro- and the microcirculatory system. Vessel injury is mainly related to deregulation of glucose homeostasis and insulin/insulin-precursors production, generation of advanced glycation end-products, reduction in nitric oxide synthesis, and oxidative and reductive stress. It occurs both at extracellular level with increased calcium and matrix proteins deposition and at intracellular level, with abnormalities of intracellular pathways and increased cell death. Peripheral arterial disease, coronary heart disease, and ischemic stroke are the main causes of morbidity/mortality in diabetic patients representing a major clinical and economic issue. Pharmacological therapies, administration of growth factors, and stem cellular strategies are the most effective approaches and will be discussed in depth in this comprehensive review covering the regenerative therapies of diabetic microangiopathy.

  5. Thrombotic Thrombocytopenic Purpura Associated with Pneumococcal Sepsis

    Directory of Open Access Journals (Sweden)

    Jeffrey R Schriber

    1993-01-01

    Full Text Available The first documented case of thrombotic thrombocytopenic purpura (TTP associated with pneumococcal septicemia is reported. This association has been previously demonstrated with hemolytic uremic syndrome. The patient presented with recurrent seizures, oliguric renal failure, fever, thrombocytopenia and microangiopathic hemolytic anemia; coagulation studies were normal. Blood and sputum cultures were positive for Streptococcus pneumoniae. The patient responded to therapy with plasmapheresis and antiplatelet agents as well as antibiotics. Coincident infection should be searched for in all cases of TTP.

  6. Renal disease in human immunodeficiency virus - Not just HIV-associated nephropathy.

    Science.gov (United States)

    Vali, P S; Ismal, K; Gowrishankar, S; Sahay, M

    2012-03-01

    The aim of the study was to determine the various histopathological lesions in human immunodeficiency virus (HIV) patients with renal dysfunction and to establish clinicopathological correlation. Over a period of two years from January 2008 to March 2010, 27 HIV positive patients with renal dysfunction were subjected to renal biopsy. Of the 27 patients, 23 were males and four were females (85.2% males, 14.8% females). Mean age was 38.2 ± 10.36 (range 20 - 60) years. The probable mode of acquisition of HIV infection was sexual in 22 patients (81.5%). Thirteen patients (48%) had nephrotic proteinuria. The CD4 count ranged from 77 to 633/microliter. The kidneys were of normal size in 19 (70.4%) and bulky in eight (29.6%) patients. Thirteen patients required renal replacement therapy. Eleven patients had acute tubule-interstitial lesions (40.7%) while 15 (55.5%) had glomerular lesions. The various glomerular lesions were, focal segmental glomerulosclerosis in five, amyloidosis in three, diffuse proliferative GN in two, and membranoproliferative glomerulonephritis (GN), membranous GN, minimal change disease, diabetic nephropathy, crescentic GN, and thrombotic microangiopathy were seen in one each. None of the clinical or laboratory variables, except hypertension, was found to predict glomerular versus non-glomerular lesions on biopsy. In conclusion we show that a variety of glomerular and tubulointerstitial lesions can be seen on renal histology. Hence, renal biopsy is indicated in renal dysfunction associated with HIV for making proper diagnosis and therapy.

  7. Moxifloxacin (Avelox Induced Thrombotic Thrombocytopenic Purpura

    Directory of Open Access Journals (Sweden)

    Sikander P. Surana

    2012-01-01

    Full Text Available We report a case of a 66-year-old African-American female who presented with complaints of progressively worsening weakness, shortness of breath on minimal exertion, lethargy for the last few days, and short episodes of aphasia lasting 20–30 seconds. Prior to presentation, she was treated with two courses of moxifloxacin for sinusitis. Laboratory examination was remarkable for anemia and thrombocytopenia with elevated lactate dehydrogenase and no evidence of renal failure. Peripheral smear showed numerous schistocytes and she was diagnosed with thrombotic thrombocytopenic purpura. Moxifloxacin was identified as the offending agent. The patient was treated with prednisone and plasmapheresis. To the best of our knowledge, this is the first reported case of thrombotic thrombocytopenic purpura associated with the use of moxifloxacin. Although rare, physicians should be aware of this serious complication associated with its use.

  8. [Thrombotic complications in the nephrotic syndrome].

    Science.gov (United States)

    Keusch, G

    1989-08-01

    Thromboembolic episodes are one of the most serious complications in patients with nephrotic syndrome, with an overall incidence of 25%. The most frequent site of thrombosis is the renal vein, with a reported incidence varying from 2-42%. Arterial thromboses are much less common than venous thromboses, with an overall incidence of 3%. Clinical course of renal vein thrombosis may be acute or chronic. Renal venography is the method of choice in its diagnosis. Duplex scanning, computed tomography and magnetic resonance imaging may be as accurate as venography. Once the diagnosis of renal vein thrombosis is established, anticoagulation therapy should be started. Thrombectomy or thrombolytic therapy seem to have little to offer over oral anticoagulation. The increased incidence of thrombotic complications in nephrotic syndrome may be due to a hypercoagulable state distinguished by an increase in coagulation factors (V, VIII, X and fibrinogen); a decrease in the levels of coagulation inhibitors (antithrombin III, protein S); an increase in alpha 2-antiplasmin activity; and exaggerated platelet adhesiveness and aggregation. This pre-thrombotic state may be aggravated by additional rheological factors (immobilization, diuretic therapy etc.). Serum albumin has been found to be an appropriate parameter to assess the risk of thrombosis development in these patients. A serum albumin level below 20 g/l carries a high risk of thromboembolic complications. Prophylactic anticoagulation therapy is therefore indicated in patients with serum albumin below 20 g/l.

  9. Utility of Iron Staining in Identifying the Cause of Renal Allograft Dysfunction in Patients with Sickle Cell Disease

    Directory of Open Access Journals (Sweden)

    Yingchun Wang

    2015-01-01

    Full Text Available Sickle cell nephropathy (SCN is associated with iron/heme deposition in proximal renal tubules and related acute tubular injury (ATI. Here we report the utility of iron staining in differentiating causes of renal allograft dysfunction in patients with a history of sickle cell disease. Case 1: the patient developed acute allograft dysfunction two years after renal transplant. Her renal biopsy showed ATI, supported by patchy loss of brush border and positive staining of kidney injury molecule-1 in proximal tubular epithelial cells, where diffuse increase in iron staining (2+ was present. This indicated that ATI likely resulted from iron/heme toxicity to proximal tubules. Electron microscope confirmed aggregated sickle RBCs in glomeruli, indicating a recurrent SCN. Case 2: four years after renal transplant, the patient developed acute allograft dysfunction and became positive for serum donor-specific antibody. His renal biopsy revealed thrombotic microangiopathy (TMA and diffuse positive C4d stain in peritubular capillaries. Iron staining was negative in the renal tubules, implying that TMA was likely associated with acute antibody-mediated rejection (AAMR, type 2 rather than recurrent SCN. These case reports imply that iron staining is an inexpensive but effective method in distinguishing SCN-associated renal injury in allograft kidney from other etiologies.

  10. Clinicopathologic features of hepatic diabetic microangiopathy

    Institute of Scientific and Technical Information of China (English)

    王征

    2012-01-01

    Objective To study the clinicopathological features of diabetic microangiopathy in liver and diabetic hepatosclerosis(DHS) of elderly male with type 2 diabetes mellitus(T2DM).Methods One hundred and twenty autopsy cases with T2DM (diabetic group) and contemporary 48 cases,non-diabetic

  11. Basiliximab induced non-cardiogenic pulmonary edema in two pediatric renal transplant recipients.

    LENUS (Irish Health Repository)

    Dolan, Niamh

    2009-11-01

    We report two cases of non-cardiogenic pulmonary edema as a complication of basiliximab induction therapy in young pediatric renal transplant patients identified following a retrospective review of all pediatric renal transplant cases performed in the National Paediatric Transplant Centre, Childrens University Hospital, Temple Street, Dublin, Ireland. Twenty-eight renal transplantations, of which five were living-related (LRD) and 23 were from deceased donors (DD), were performed in 28 children between 2003 and 2006. In six cases, transplantations were pre-emptive. Immunosuppression was induced pre-operatively using a combination of basiliximab, tacrolimus and methylprednisolone in all patients. Basiliximab induction was initiated 2 h prior to surgery in all cases and, in 26 patients, basiliximab was re-administered on post-operative day 4. Two patients, one LRD and one DD, aged 6 and 11 years, respectively, developed acute non-cardiogenic pulmonary edema within 36 h of surgery. Renal dysplasia was identified as the primary etiological factor for renal failure in both cases. Both children required assisted ventilation for between 4 and 6 days. While both grafts had primary function, the DD transplant patient subsequently developed acute tubular necrosis and was eventually lost within 3 weeks due to thrombotic microangiopathy and severe acute antibody-mediated rejection despite adequate immunosuppression. Non-cardiogenic pulmonary edema is a potentially devastating post-operative complication of basiliximab induction therapy in young pediatric patients following renal transplantation. Early recognition and appropriate supportive therapy is vital for patient and, where possible, graft survival.

  12. Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange.

    Science.gov (United States)

    Narayanan, Parameswaran; Jayaraman, Aparna; Rustagi, Rashi S; Mahadevan, S; Parameswaran, Sreejith

    2012-07-01

    A nine-year-old girl presented with headache, purpura and mild left hemiparesis. Laboratory evaluation revealed thrombotic microangiopathy with ADAMTS13 deficiency, with auto-antibodies to ADAMTS13. She was treated with plasma exchange and steroids, following which she improved transiently, relapsing within 2 months. The relapse was refractory to conventional therapy and rituximab was tried. She had good response to rituximab and has been in remission for the past 12 months. Rituximab may be a promising option for children with acquired TTP refractory to plasma exchange and steroids.

  13. An Unusual Presentation of Pseudothrombotic Microangiopathy in a Patient with Autoimmune Atrophic Gastritis

    Directory of Open Access Journals (Sweden)

    Alexandre Malek

    2016-01-01

    Full Text Available Introduction. We hereby describe the case of a young female patient who presented with pseudothrombotic microangiopathy, as well as pancytopenia accompanied by autoimmune atrophic gastritis. Case Presentation. A 36-year-old Caucasian woman presented to the emergency department with fatigue and dyspnea on minimal exertion. Physical examination was unremarkable except for pallor and noninjected conjunctiva. Laboratory tests revealed high LDH and low hemoglobin, white blood cells, platelets, and haptoglobin. The peripheral blood smear showed schistocytes suggestive of pseudothrombotic microangiopathy. Low cobalamin level and hyperhomocysteinemia were also detected. Autoimmune atrophic gastritis was confirmed by gastric biopsy and positive anti-intrinsic factor antibodies. Vitamin B12 supplements were given which led to rapid recovery and normalization of blood parameters. Conclusion. This case highlights the importance and serves as a reminder to clinicians to rule out cobalamin deficiency and autoimmune atrophic gastritis in patients presenting with a picture suggestive of thrombotic thrombocytopenic purpura and pancytopenia, which was completely reversible after appropriate replacement therapy without recurring to unnecessary and invasive procedures such as plasma exchange.

  14. Thrombotic Thrombocytopenic Purpura: Etiopathogenesis, Diagnostics and Basic Principles of Treatment

    Directory of Open Access Journals (Sweden)

    Todorović Željko

    2017-03-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a clinical syndrome that manifests with thrombocytopenia, microangiopathic haemolytic anaemia and symptoms and signs of kidney and brain damage, but it rarely involves other organs. The main pathophysiological cause of TTP is diminished metalloproteinase ADAMTS13 activity; the main function of ADAMTS13 is to degrade large multimers of the von Willebrand factor. Diminished activity of ADAMTS13 is caused either by a genetic mutation in the gene that codes ADAMTS13 (congenital TTP or by antibodies that block ADAMTS13 enzyme activity or accelerate the degradation of ADAMTS13 (acquired TTP. Clinically, TTP presents most frequently with signs and symptoms of brain and kidney damage with concomitant haemorrhagic syndrome. TTP is suspected when a patient presents with a low platelet count, microangiopathic haemolytic anaemia (negative Coombs tests, low haptoglobine concentration, increased serum concentration of indirect bilirubin and lactate dehydrogenase, increased number of schysocytes in peripheral blood and the typical clinical presentation. A definitive diagnose can be made only by measuring the ADAMTS13 activity. The differential diagnosis in such cases includes both typical and atypical haemolytic uremic syndrome, disseminated intravascular coagulation, HELLP syndrome in pregnant women and other thrombotic microangiopathies. The first line therapy for TTP is plasma exchange. In patients with acquired TTP, in addition to plasma exchange, immunosuppressive medications are used (corticosteroids and rituximab. In patients with hereditary TTP, the administration of fresh frozen plasma is sometimes required.

  15. Renal transplantation in HIV-infected patients: 2010 update.

    Science.gov (United States)

    Trullas, Joan C; Cofan, Federico; Tuset, Montse; Ricart, María J; Brunet, Mercedes; Cervera, Carlos; Manzardo, Christian; López-Dieguez, María; Oppenheimer, Federico; Moreno, Asuncion; Campistol, Josep M; Miro, Jose M

    2011-04-01

    The prognosis of human immunodeficiency virus (HIV) infection has improved in recent years with the introduction of antiretroviral treatment. While the frequency of AIDS-defining events has decreased as a cause of death, mortality from non-AIDS-related events including end-stage renal diseases has increased. The etiology of chronic kidney disease is multifactorial: immune-mediated glomerulonephritis, HIV-associated nephropathy, thrombotic microangiopathies, and so on. HIV infection is no longer a contraindication to transplantation and is becoming standard therapy in most developed countries. The HIV criteria used to select patients for renal transplantation are similar in Europe and North America. Current criteria state that prior opportunistic infections are not a strict exclusion criterion, but patients must have a CD4+ count above 200 cells/mm(3) and a HIV-1 RNA viral load suppressible with treatment. In recent years, more than 200 renal transplants have been performed in HIV-infected patients worldwide, and mid-term patient and graft survival rates have been similar to that of HIV-negative patients. The main issues in post-transplant period are pharmacokinetic interactions between antiretrovirals and immunosuppressants, a high rate of acute rejection, the management of hepatitis C virus coinfection, and the high cardiovascular risk after transplantation. More studies are needed to determine the most appropriate antiretroviral and immunosuppressive regimens and the long-term outcome of HIV infection and kidney graft.

  16. Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation

    Science.gov (United States)

    Bouatou, Yassine; Bacchi, Véronique Frémeaux; Villard, Jean; Moll, Solange; Martin, Pierre-Yves; Hadaya, Karine

    2015-01-01

    Abstract Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 months, 2 episodes of thrombotic microangiopathy (TMA), after a miscarriage and a preeclampsia, were misinterpreted as triggered by tacrolimus. After each episode however serum creatinine returned to baseline. Five years after transplantation, she had a self-limited rhinosinusitis followed 3 weeks later by an oliguric renal failure. Her complement profile was normal. Graft biopsy showed C3 glomerulonephritis with no “humps” on electron microscopy. No significant renal function improvement followed methylprednisolone pulsing. A second biopsy showed severe acute TMA lesions with C3 glomerular deposits. Despite weekly eculizumab for 1 month, dialysis was resumed. A new workup identified the “at-risk” complement factor H haplotype. Thus, aHUS recurrence should be ruled out in aHUS patients considered at low recurrence risk when a TMA is found in graft biopsy. Prompt eculizumab therapy should be considered to avoid graft loss as aHUS recurrence can first present as a C3 glomerulonephritis. PMID:27500215

  17. Renal histology in the elderly: indications and outcomes.

    LENUS (Irish Health Repository)

    2012-02-01

    Background: Renal disease is being increasingly diagnosed in the elderly. However, reports on biopsy-confirmed renal disease in this population are limited. The aim of this study was to give an overview of the most important indications, diagnoses and outcomes of renal biopsies in the elderly in our center. Methods: This was a retrospective review of all elderly renal biopsies over 5 years. Patients were eligible for inclusion if they were aged =65 years and had had a native kidney biopsy performed. The data recorded included age, sex, indications for biopsy, histological diagnoses and outcomes. Results: During this time, 1,372 native renal biopsies were performed. Of these, 236 (17%) were in patients aged =65 years; 150 male (64%) and 86 female (36%). The most common indications for biopsy were acute renal failure and nephrotic syndrome. Common diagnoses included pauci-immune crescentic glomerulonephritis, tubulointerstitial nephritis, membranous nephropathy, IgA nephropathy and chronic thrombotic microangiopathy. Long-term follow-up of 3 years was available for 102 patients; median serum creatinine at the time of biopsy was 427 micromol\\/L (interquartile range 204-702) and at 3 years post biopsy had fallen to 192 micromol\\/L (interquartile range 152-408). Conclusions: In our center, 17% of native kidney biopsies are performed in elderly patients aged =65 years. In our experience, this procedure was safe and had a 97% diagnostic rate. The available follow-up data of patients suggest that renal histology is not only of benefit in diagnosis but also of potential value in terms of prognosis and treatment.

  18. Renal histology in the elderly: indications and outcomes.

    LENUS (Irish Health Repository)

    Brown, Catherine M

    2011-06-28

    Background: Renal disease is being increasingly diagnosed in the elderly. However, reports on biopsy-confirmed renal disease in this population are limited. The aim of this study was to give an overview of the most important indications, diagnoses and outcomes of renal biopsies in the elderly in our center. Methods: This was a retrospective review of all elderly renal biopsies over 5 years. Patients were eligible for inclusion if they were aged =65 years and had had a native kidney biopsy performed. The data recorded included age, sex, indications for biopsy, histological diagnoses and outcomes. Results: During this time, 1,372 native renal biopsies were performed. Of these, 236 (17%) were in patients aged =65 years; 150 male (64%) and 86 female (36%). The most common indications for biopsy were acute renal failure and nephrotic syndrome. Common diagnoses included pauci-immune crescentic glomerulonephritis, tubulointerstitial nephritis, membranous nephropathy, IgA nephropathy and chronic thrombotic microangiopathy. Long-term follow-up of 3 years was available for 102 patients; median serum creatinine at the time of biopsy was 427 µmol\\/L (interquartile range 204-702) and at 3 years post biopsy had fallen to 192 µmol\\/L (interquartile range 152-408). Conclusions: In our center, 17% of native kidney biopsies are performed in elderly patients aged =65 years. In our experience, this procedure was safe and had a 97% diagnostic rate. The available follow-up data of patients suggest that renal histology is not only of benefit in diagnosis but also of potential value in terms of prognosis and treatment.

  19. LUPUS NEPHRITIS COMPLICATED WITH MALIGNANT HYPERTENSION: FROM RENAL VASCULAR PATHOLOGY TO CLINICAL RELEVANCE

    Institute of Scientific and Technical Information of China (English)

    Jian-ling Tao; Hang Li; Yu Tang; Yu-bing Wen; Xue-wang Li

    2008-01-01

    Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension.Methods We retrospectively studied 19 patients with lupus nephritis complicated with malignant hypertension who underwent renal biopsy between January 2002 and December 2006.Results Of 19 patients, 3 were men and 16 were women, with a mean age of 24. 4±7. 7 years old. All had positive antinuclear antibodies and low serum complement was found in 13 patients. All were anemic and 12 of them were thrombocytopenic. Impaired renal function was found in 17 patients with an average serum creatinine of 184. 5 ± 88.9 μmol/L. Severe intrarenai arteriolar lesion was found in all patients. Six patients had lupus vasculopathy, 11 patients had renal thrombotic microangiopathy lesion, 2 had severe arteriosclerosis. All patients received steroids and immunosuppressive drugs, 15 received angiotensin-converting enzyme inhibitor (ACEI)/angiotensin receptor blocker ( ARB ) with resultant well-controlled blood pressure. Thrombocytopenia and hemolytic anemia resolved remarkably.The renal function improved or recovered in 14 of 17 patients, and 3 developed end-stage renal disease on maintenance dialysis.Conclusions Severe intrarenal vascular lesion complicated with renal nephritis parallels clinical manifestation of malignant hypertension. Renal pathology is the key of treatment strategy emphasizing on the significance of renal vascular involvement and type. On the basis of immunosuppressive drugs and steroids to control systemic lupus activity, timely initiation of ACEI/ARB could be of benefit to blood pressure control and long term renal survival.

  20. Thrombotic thrombocytopenic purpura concomitant with autoimmune thyroiditis

    Directory of Open Access Journals (Sweden)

    Ali Bay

    2011-12-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is characterized by disseminated thrombotic occlusions located in the microcirculation, microangiopathic hemolytic anemia, thrombocytopenia, fever, and renal and neurologic abnormalities. A 14 year old girl admitted to our hospital complaining bruising on her body and prolonged menstrual bleeding. On her physical examination there were very common bruising on four extremities. On the laboratory studies, Hemoglobin was 9 g/dL; Hematocrit, 24%; white blood count 11600/mm3 and thrombocyte count, 9.000/mm3. According to these findings our first diagnose was idiopatic thrombocytopenic purpura so intravenous immunoglobulin was given to patient for two days. Bone marrow aspiration was performed because of persisting thrombocytopenia despite two days IVIG therapy. Increased number of megakaryocytes was seen in bone marrow. Some accompanying symptoms like headache, numbness in per oral region and extremities, difficulty in speaking, and fluctuation in consciousness for short time occurred. The patient was reevaluated; because thrombocytopenia persisted and some neurological symptoms was observed. Due to these findings we thought that TTP was the diagnosed and plasma exchange was started. Increase was seen in platelet count in the second days of treatment. TTP should be considered in children presenting with atypical thrombocytopenia.

  1. Gemcitabine-associated thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Zupancic, Melanie; Shah, Prabodh C; Shah-Khan, Farheen

    2007-07-01

    Gemcitabine-associated thrombotic thrombocytopenic purpura (TTP) is a rare complication of gemcitabine treatment with a incidence ranging from 0.015% to 1.4%. Clinically, this disease manifests as haemolytic anaemia, thrombocytopenia, and renal insufficiency; hypertension and neurological and pulmonary symptoms are also known complications. The risk of TTP increases as the cumulative dose of gemcitabine approaches 20,000 mg/m(2). The pathophysiology of this disease entity is unknown although several theories, involving both immune and non-immune mechanisms, have been proposed. The most effective treatment is discontinuation of gemcitabine, the provision of antihypertensive medications as needed, and consideration of plasmapheresis or use of immunoadsorption column in severe cases.

  2. Resección de un carcinoma renal izquierdo con extensión trombótica hacia la aurícula derecha Resection of a left renal carcinoma with thrombotic extension to right atrium

    Directory of Open Access Journals (Sweden)

    Alejandro Villar Inclán

    2010-09-01

    Full Text Available Se presenta el caso de un hombre de 59 años con diagnóstico de hipernefroma izquierdo y trombosis de la vena cava inferior con extensión hacia la aurícula derecha. Un equipo multidisciplinario realizó una nefrectomía radical izquierda, exposición de la vena cava inferior con técnica de piggyback, atriotomía derecha y cavotomía inferior para extracción del trombo, previo pinzamiento de la vena renal derecha y el hilio hepático. El tiempo quirúrgico total fue de 7 h 30 min. El procedimiento se realizó con anestesia general y un período mínimo de derivación cardiopulmonar. Se utilizó monitorización transoperatoria estandarizada de los parámetros vitales y además ecocardiografía transesofágica para la detección de la remoción completa del trombo y su extracción, así como posibles émbolos desprendidos. La evolución transoperatoria y posoperatoria fue satisfactoria. Se realiza una revisión de la literatura médica internacional sobre el tema y se ofrecen conclusiones.This is the male case aged 59 diagnosed with left hypernephroma and thrombosis of the inferior vena cava with extension to right atrium. A multidisciplinary staff performed a left radical nephrectomy, exposition of inferior vena cava using the piggyback technique, right atriotomy and inferior cavotomy for extension of thrombus and previous clamping of right renal vein and hepatic hilus. The total surgical time was of 7 hours and 30 minutes. The procedure was performed under general anesthesia and a minimal period of cardiopulmonary shunt. Standardized transoperative monitoring of vital parameters was used and also transesophageal echocardiography to detect the total removal of thrombus and its extraction, as well as the potential detached emboli. The transoperative and postoperative course was satisfactory. A review of the international medical literature on this subject was made offering conclusions.

  3. Living donor renal transplantation in patients with antiphospholipid syndrome

    Science.gov (United States)

    Choi, Ji Yoon; Jung, Joo Hee; Shin, Sung; Kim, Young Hoon; Han, Duck Jong

    2016-01-01

    Abstract Introduction: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. Clinical Findings: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin. However, he frequently experienced recurrent DVT despite of anticoagulation therapy. Before the surgery, APS was confirmed based on positive results lupus anticoagulant in serological tests. A 40-year-old man with polycystic kidney disease and a history recurrent DVT tested positive for lupus anticoagulant and anticardiolipin antibodies. Lastly, a 42-year-old woman with ESRD was diagnosed with APS 7 years ago. She also developed DVT and tested positive for lupus anticoagulant and anti-B2-glycoprotein 1. The anticoagulation protocol was as follows in all cases: Warfarin was stopped 5 days before living donor renal transplantation and intravenous heparin therapy was started. During surgery, bolus heparin injections (3000 U) were administered to prevent arterial or venous thrombosis. Heparin was substituted with warfarin on postoperative day 4. The third patient (42/F) developed clinical rejection indicated by increased serum creatinine levels and donor-specific antibodies (DSA) and received steroid pulse therapy, plasmapheresis, and rituximab. This treatment restored graft function to within the normal range. The latest graft function in all patients was maintained at normal levels in the outpatient clinic. Conclusions: Living donor renal transplantation may be successful in patients with APS following perioperative anticoagulation therapy. However, because of the high risk of

  4. A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

    Directory of Open Access Journals (Sweden)

    Risa Yamada

    2011-01-01

    Full Text Available Thrombotic thrombocytopenia purpura (TTP caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA. Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE. In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

  5. Opana ER abuse and thrombotic thrombocytopenic purpura (TTP)-like illness: a rising risk factor in illicit drug users.

    Science.gov (United States)

    Kapila, Aaysha; Chhabra, Lovely; Chaubey, Vinod K; Summers, Jeffery

    2014-03-03

    We report the case of a 22 year-old-woman who presented with upper extremity cellulitis secondary to an infiltration of illicit intravenous drug use. She confessed to the intravenous use of Opana ER (an extended release oral formulation of oxymorphone) which is an opioid drug approved only for oral use. She was found to have clinical evidence of profound thrombotic microangiopathy which resulted due to the intravenous use of Opana ER. She showed full clinical improvement after withholding drug and supportive clinical care. Recent report of Opana ER intravenous abuse was published from Tennessee county and has now been increasingly recognised as one of the causes of thrombocytopenia which mimicks clinically as thrombotic thrombocytopenic purpura. Physicians should be aware of this association as the lack of familiarity to this can pose serious management dilemmas for our patients (especially the polysubstance abusers).

  6. Homocysteine levels in patients with primary and secondary Raynaud's phenomenon. Its association with microangiopathy severity.

    Science.gov (United States)

    Vayá, Amparo; Sánchez, Fernando; Todolí, Jose; Calvo, Javier; Alis, Rafael; Collado, Susana; Ricart, Jose M

    2014-01-01

    The association between hyperhomocysteinemia (HHcy) and Raynaud's phenomenon (RP) remains a matter of debate. In 18 primary RP, 23 secondary RP and 41 controls, we investigated homocysteine (Hcy) levels along with biochemical and inflammatory parameters. The Hcy levels in both primary and secondary RP were elevated when compared with controls (p 0.05), or in the prevalence of cardiovascular risk factors (p > 0.05). When patients were classified according to presence or absence of digital ulcers, as a sign of microangiopathy severity, the former showed higher Hcy levels than the latter (p = 0.035). Our results indicate that both primary and secondary RP patients show a mild increase in Hcy levels, which is not related to age, vitamin deficiencies or impaired renal function, but is related to microangiopathy severity. Therefore the association of HHcy and RP suggest that Hcy may contribute to endothelial dysregulation, which characterizes this disease. Specific studies should be designed to elucidate the pathogenesis of HHcy in these patients.

  7. Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis.

    Science.gov (United States)

    Povey, Hannah; Vundru, Rahul; Junglee, Naushad; Jibani, Mahdi

    2014-11-01

    Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) which encompasses hemolytic anemia, thrombocytopenia, and organ impairment. Around 10% of cases are atypical HUS (aHUS), a rare disease with poor outcomes caused by uncontrolled activation of the alternative complement pathway. This case describes a young woman with clinical manifestations compatible with TMA during childhood and adolescence who was formally diagnosed with aHUS at the age of 21. She was managed with intensive plasma exchange and hemodialysis, which failed to improve her severe acute kidney injury and other hematological manifestations of aHUS. This was further compounded by several episodes of flash pulmonary edema and the posterior reversible encephalopathy syndrome (PRES). Treatment with the monoclonal anti-C5 inhibitor, eculizumab, improved all hematological parameters with almost full renal recovery following 3.5 months of dialysis. So far, long-term use of eculizumab (> 11 months) continues to be effective and without complication. Our case illustrates the difficulty but importance of early consideration of aHUS in patients presenting with TMA. More importantly, we highlight that near-normal renal recovery may be attained with eculizumab in adults even after a long dependence on dialysis - an observation that has not been reported in the literature so far.

  8. TMA secondary to SLE: rituximab improves overall but not renal survival.

    Science.gov (United States)

    Sun, Fangfang; Wang, Xiaodong; Wu, Wanlong; Wang, Kaiwen; Chen, Zhiwei; Li, Ting; Ye, Shuang

    2017-08-30

    Thrombotic microangiopathy (TMA) includes a series of life-threatening disorders. Systemic lupus erythematosus (SLE) is one of the most common acquired causes. To identify predictors of prognosis in patients with TMA secondary to SLE, we conducted a single-center historical study. From January 2013 to June 2016, of 2182 SLE hospitalized patients in the Ren Ji Hospital, a total of 21 consecutive patients with TMA secondary to SLE were identified. The 90-day short-term mortality was 33.3%. The kidney involvement (66.7%) was associated with poor prognosis, while the administration of rituximab (n = 13) was an independent protective factor according to logistic regression analysis. Compared to conventional treatment, i.e., plasma exchange, high-dose glucocorticoids, and intravenous immunoglobulin, the overall survival is significantly higher among patients receiving rituximab add-on (92.2 vs 33.3%, p = 0.0173); however, five out of seven patients with renal involvement in the rituximab group were eventually hemodialysis dependent. Our data indicated that add-on rituximab in the background of conventional therapy may improve the overall but not the renal survival in SLE-TMA patients.

  9. Refractory thrombotic thrombocytopenic purpura in a 16-year-old girl: successful treatment with bortezomib

    NARCIS (Netherlands)

    Balen, T. van; Schreuder, M.F.; Jong, H. de; Kar, N.C.A.J. van de

    2014-01-01

    We present a case of a 16-year-old girl with autoimmune thrombotic thrombocytopenic purpura (TTP), refractory to plasma exchange and high-dose prednisone. Despite the additional treatment with rituximab, she developed renal and neurological complications with ongoing hemolysis and thrombocytopenia.

  10. Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis.

    Science.gov (United States)

    Dimopoulou, Despoina; Dimosiari, Athina; Mandala, Eudokia; Dimitroulas, Theodoros; Garyfallos, Alaxandros

    2017-01-01

    Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far. The first is a 27-year-old woman with a history of polyarticular rheumatoid factor negative juvenile idiopathic arthritis, who presented with thrombocytopenia, anemia, schistocytes on blood smear, headache, and active arthritis. Originally she was treated successfully with plasma exchange, intravenous prednisone, and vincristine, and a few months after the TTP episode, she was commenced on rituximab, resulting in remission of primary disease and no relapse of TTP. The second case refers to a 29-year-old pregnant woman complaining of dizziness and fatigue with microangiopathic hemolytic anemia. She was treated with plasma exchanges, intravenous prednisolone, and INN human normal immunoglobulin with full remission of the TTP episode. Six and half years later, she was diagnosed with multiple sclerosis and was commenced on interferon beta-1 alpha, with no recurrent episode of TTP. These cases broaden the spectrum of autoimmune disorders manifested or complicated clinically by TTP. Furthermore, biological agents such as rituximab appear to be an effective treatment option for refractory cases of TTP related to systemic rheumatic disease, indicating an alternative therapeutic solution in persistent cases of this disorder.

  11. Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Despoina Dimopoulou

    2017-07-01

    Full Text Available Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far. The first is a 27-year-old woman with a history of polyarticular rheumatoid factor negative juvenile idiopathic arthritis, who presented with thrombocytopenia, anemia, schistocytes on blood smear, headache, and active arthritis. Originally she was treated successfully with plasma exchange, intravenous prednisone, and vincristine, and a few months after the TTP episode, she was commenced on rituximab, resulting in remission of primary disease and no relapse of TTP. The second case refers to a 29-year-old pregnant woman complaining of dizziness and fatigue with microangiopathic hemolytic anemia. She was treated with plasma exchanges, intravenous prednisolone, and INN human normal immunoglobulin with full remission of the TTP episode. Six and half years later, she was diagnosed with multiple sclerosis and was commenced on interferon beta-1 alpha, with no recurrent episode of TTP. These cases broaden the spectrum of autoimmune disorders manifested or complicated clinically by TTP. Furthermore, biological agents such as rituximab appear to be an effective treatment option for refractory cases of TTP related to systemic rheumatic disease, indicating an alternative therapeutic solution in persistent cases of this disorder.

  12. Unmasking of complements using proteinase-K in formalin fixed paraffin embedded renal biopsies

    Directory of Open Access Journals (Sweden)

    R Nada

    2016-01-01

    Full Text Available Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN, membranoproliferative type-1 (MPGN-1, immunoglobulin A nephropathy (IgAN, and anti-glomerular basement disease (anti-GBM. Immunofluorescence panel included fluorescein isothiocyanate (FITC conjugated IgG, IgA, IgM, complements (C3 and C1q, light chains (kappa, lambda and fibrinogen antibodies. After standardization of the technique, 75 renal biopsies and 43 autopsies cases were stained. Out of 43 autopsy cases, immune-complex mediated glomerulonephritis (GN was confirmed in 18 cases (Lupus nephritis-11, IgAN-6, MGN-1, complement-mediated dense deposit disease (DDD-1 and monoclonal diseases in 4 cases (amyloidosis-3, cast nephropathy-1. Immune-mediated injury was excluded in 17 cases (focal segmental glomerulosclerosis -3, crescentic GN-6 [pauci-immune-3, anti-GBM-3], thrombotic microangiopathy-5, atherosclerosis-3. Renal biopsies (n-75 where inadequate or no frozen sample was available; this technique classified 52 mesangiocapillary pattern as MPGN type-1-46, DDD-2 and (C3GN-4. Others were diagnosed as IgAN-3, lupus nephritis-2, MGN-4, diffuse proliferative glomerulonephritis (DPGN-1, Non-IC crescentic GN-1, monoclonal diseases-3. In nine cases, DIF on FFPE tissue could not help in making diagnosis. Proteinase-K enzymatic digestion of FFPE renal biopsies can unmask complements (both C3 and C1q in immune-complexes mediated and complement-mediated diseases. This method showed good results on autopsy tissues archived for as long as 15 years.

  13. Unmasking of complements using proteinase-K in formalin fixed paraffin embedded renal biopsies.

    Science.gov (United States)

    Nada, R; Kumar, A; Kumar, V G; Gupta, K L; Joshi, K

    2016-01-01

    Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM). Immunofluorescence panel included fluorescein isothiocyanate (FITC) conjugated IgG, IgA, IgM, complements (C3 and C1q), light chains (kappa, lambda) and fibrinogen antibodies. After standardization of the technique, 75 renal biopsies and 43 autopsies cases were stained. Out of 43 autopsy cases, immune-complex mediated glomerulonephritis (GN) was confirmed in 18 cases (Lupus nephritis-11, IgAN-6, MGN-1), complement-mediated dense deposit disease (DDD-1) and monoclonal diseases in 4 cases (amyloidosis-3, cast nephropathy-1). Immune-mediated injury was excluded in 17 cases (focal segmental glomerulosclerosis -3, crescentic GN-6 [pauci-immune-3, anti-GBM-3], thrombotic microangiopathy-5, atherosclerosis-3). Renal biopsies (n-75) where inadequate or no frozen sample was available; this technique classified 52 mesangiocapillary pattern as MPGN type-1-46, DDD-2 and (C3GN-4). Others were diagnosed as IgAN-3, lupus nephritis-2, MGN-4, diffuse proliferative glomerulonephritis (DPGN)-1, Non-IC crescentic GN-1, monoclonal diseases-3. In nine cases, DIF on FFPE tissue could not help in making diagnosis. Proteinase-K enzymatic digestion of FFPE renal biopsies can unmask complements (both C3 and C1q) in immune-complexes mediated and complement-mediated diseases. This method showed good results on autopsy tissues archived for as long as 15 years.

  14. An insidious presentation of thrombotic thrombocytopenic purpura:A case report and brief literature review

    Institute of Scientific and Technical Information of China (English)

    Shafeek Kiblawi; Elie Harmouche; Ralph Bou Chebl; Gilbert Abou Dagher

    2014-01-01

    Thrombotic thrombocytopenic purpura(TTP) is a rare thrombotic microangiopathy with an estimated incidence of11 cases/million population per year.Early treatment is essential and is curative in this disease where lack of treatment results in90% mortality.We describe an atypical case of a patient withTTP who presented to theEmergencyDepartment for headache, and was found to have thrombocytopenia but only mild anemia that was explained by another disease process.Case:A44-year-old female presented to theEmergencyDepartment for worsening headache and weakness over the last week.She had no fever and no focal neurological deficits but was pale and complained of severe headache.A blood test showed her to be anemic and thrombocytopenic.She explained that she had been having prolonged heavy menses over the last year.She was treated with blood and platelet transfusions, and seen by theGynecology service who treated her for uterine fibroids after which she was discharged.She returned1 week later with the same complaint, and was found to have a stable hemoglobin level but recurrence of thrombocytopenia.ATTP diagnosis was entertained and the workup confirmed it.The patient was treated with plasmapheresis and discharged home with no sequalae.Conclusion:Emergency physicians should keepTTP in mind when approaching cases of thrombocytopenia with mild anemia, even if an alternative diagnosis exists.

  15. Thrombotic thrombocytopenic purpura (TTP or Moschowitz syndrome: a true hematologic emergency

    Directory of Open Access Journals (Sweden)

    Deborah Melis

    2012-01-01

    Full Text Available Introduction: Thrombotic thrombocytopenic purpura (TTP is a thrombotic microangiopathy caused by congenital or inherited disorders involving the processing of the ultra-large forms of von Willebrand factor. As a result, platelet-rich microthrombi form in the small arterial vessels of various organs, particularly those of the brain, heart, and kidneys. The idiopathic autoimmune form of TTP is the most common. There are various subgroups of acquired TTP associated with HIV infection, sepsis, pregnancy, autoimmune disease, various disseminated malignancies, and drugs. If not promptly treated, TTP is associated with high mortality, making it a true medical emergency. Materials and methods: The article is based on a review of the literature published between January and October of 2009. Its aim is to clarify the diagnosis, treatment, and follow-up of TTP. Results: Diagnostic criteria include the presence of microangiopathic hemolytic anemia associated with thrombocytopenia in the absence of other obvious causes. Assays of ADAMTS13 activity and titration of acquired antibodies against this enzyme are indicated in the follow-up of disease and as prognostic indicators. Treatment centers around daily plasma exchange associated with immunosuppressant drug therapy, particularly steroids and more recently the monoclonal anti-CD20 antibody rituximab. Discussion: Despite improved treatment, TTP is still associated with significant mortality (10—20%, particularly when plasma exchange is initiated late. Relapse also occurs in a substantial proportion of patients (10—40% although the frequency of this outcome may be reduced by rituximab therapy.

  16. Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura

    African Journals Online (AJOL)

    Abe Olugbenga

    Thrombotic thrombocytopenic purpura (TTP) is a rare clinical emergency that is ... high index of clinical suspicion and can be easily missed. .... patients with severe ADAMTS13 deficiency .... surgical procedure that involves a high risk bleeding ...

  17. Decreasing incidence of severe diabetic microangiopathy in type 1 diabetes

    DEFF Research Database (Denmark)

    Hovind, Peter; Tarnow, Lise; Rossing, Kasper

    2003-01-01

    ); group B, 1970-1974 (n = 130); group C, 1975-1979 (n = 113); and group D, 1979-1984 (n = 244). Group A, B, and C are prevalence cohorts identified in 1984; group D is an inception cohort. RESULTS: In patients followed for >/=20 years, the cumulative incidence (95% CI) of diabetic nephropathy after 20...... incidence of diabetic microangiopathy in type 1 diabetes over the past 35 years. Improved glycemic control, lower blood pressure (in part due to early aggressive antihypertensive treatment), and reduced prevalence of smoking rates were associated with the improved prognosis.......OBJECTIVE: Conflicting evidence of a decline in incidence of microvascular complications in type 1 diabetes during the last decades has been reported. To assess recent trends in the cumulative incidence of diabetic microangiopathy in type 1 diabetes, we analyzed data from long-term prospective...

  18. Nonbacterial thrombotic endocarditis presenting as intracerebral hemorrhage.

    Science.gov (United States)

    Wigger, Olivier; Windecker, Stephan; Bloechlinger, Stefan

    2016-12-01

    Nonbacterial thrombotic endocarditis is a rare cause of valvular heart disease, most commonly associated with advanced malignancy. The morbidity of this kind of endocarditis lies in its tendency to embolize, while the valve function is usually preserved. The central nervous system is the most common site of embolization, leading to ischemic stroke. We report a case of nonbacterial thrombotic endocarditis complicated by intracerebral hemorrhage as the first manifestation of adenocarcinoma of the lung. The endocarditis led to severe aortic regurgitation. In view of the advanced stage of lung cancer, the patient refused further therapy. He passed away 3 weeks after first diagnosis of the adenocarcinoma.

  19. Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    高维强; 苏健; 白霞; 王兆钺; 阮长耿

    2004-01-01

    Background Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. In this study we investigated the von Willebrand factor-cleaving protease (vWF-cp) activity deficiency in patients with TTP.Methods The plasma or serum vWF-cp activity was measured using a sensitive enzyme-linked immunosorbent assay (ELISA) by detecting the residual collagen binding activity (R-CBA) of von Willebrand factor (vWF) before and after digestion by vWF-cp. Multimers of vWF in plasma of patients with TTP were also analyzed by SDS-agarose electrophoresis. Moreover, the serum vWF-cp activities were compared between the patients with TTP and those with tumors.Results The coefficient of variation for intra-batch and inter-batch of the assay were 3.60% and 8.35%. The plasma and serum vWF-cp activity in healthy individuals were (78.79±9.17)% (n=30) and (79.47±10.78)% (n=53), respectively, while the plasma vWF-cp activity in 5 patients with TTP was markedly decreased [(21.83±19.98)%, P<0.001]. The unusually large vWF multimers were observed in two plasma samples of the patients with TTP. Although the vWF-cp activities in patients with benign and malignant tumors were also decreased (P<0.03 and P<0.001, respectively), they were relatively high in comparison with that of TTP patients (P<0.001).Conclusion Measurement of the vWF-cp activity using R-CBA is a simple and rapid method for diagnosing TTP. The vWF-cp activity in patients with TTP was markedly lower than those of patients with tumors.

  20. ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura.

    Science.gov (United States)

    Tsai, Han-Mou; Raoufi, Mojgan; Zhou, Wenhua; Guinto, Enriqueta; Grafos, Nickolas; Ranzurmal, Safi; Greenfield, Robert S; Rand, Jacob H

    2006-05-01

    Functional assays are commonly used to measure the antibodies of ADAMTS13 found in patients of thrombotic thrombocytopenic purpura (TTP). In this study we used an enzyme-linked immunoassay to analyze the ADAMTS13-binding IgG levels in six groups of individuals: normal, random hospitalized patients, acute TTP, TTP after receiving plasma therapy, TTP in remission, and other types of thrombotic microangiopathy (TMA). The results showed that ADAMTS13-binding IgG levels were elevated in 100% of the acute TTP group, 75% of the TTP group after receiving plasma therapy, and 40% of the remission group. Overall, the ADAMTS13-binding IgG levels correlated with the inhibitory activity levels againstADAMTS13 (r = -0.69, P < 0.0001). The assay also detected elevated IgG binding levels in 5% - 15% of the normal, random, and other TMA control groups. Addition of purified ADAMTS13 protein to the plasma samples suppressed the IgG binding in each of the acute TTP patients, but in none of the non-TTP groups. Serial measurement in a patient that had two exacerbations of TTP within the first three weeks revealed that the ADAMTS13 activity levels remained <0.1 U/ml during this period, and the ADAMTS13-binding IgG remained elevated, suggesting that ADAMTS13 analysis may provide valuable insight to the disease status during the course of therapy. Analysis of ADAMTS13-binding IgG is helpful for the diagnosis and management of TTP.

  1. The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Doruk Erkan

    2016-03-01

    Full Text Available Antiphospholipid syndrome (APS is characterized by thrombosis (arterial, venous, small vessel and/or pregnancy morbidity occurring in patients with persistently positive antiphospholipid antibodies (aPL. Catastrophic APS is the most severe form of the disease, characterized by multiple organ thromboses occurring in a short period and commonly associated with thrombotic microangiopathy (TMA. Similar to patients with complement regulatory gene mutations developing TMA, increased complement activation on endothelial cells plays a role in hypercoagulability in aPL-positive patients. In mouse models of APS, activation of the complement is required and interaction of complement (C 5a with its receptor C5aR leads to aPL-induced inflammation, placental insufficiency, and thrombosis. Anti-C5 antibody and C5aR antagonist peptides prevent aPL-mediated pregnancy loss and thrombosis in these experimental models. Clinical studies of anti-C5 monoclonal antibody in aPL-positive patients are limited to a small number of case reports. Ongoing and future clinical studies of complement inhibitors will help determine the role of complement inhibition in the management of aPL-positive patients.

  2. The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome.

    Science.gov (United States)

    Erkan, Doruk; Salmon, Jane E

    2016-03-05

    Antiphospholipid syndrome (APS) is characterized by thrombosis (arterial, venous, small vessel) and/or pregnancy morbidity occurring in patients with persistently positive antiphospholipid antibodies (aPL). Catastrophic APS is the most severe form of the disease, characterized by multiple organ thromboses occurring in a short period and commonly associated with thrombotic microangiopathy (TMA). Similar to patients with complement regulatory gene mutations developing TMA, increased complement activation on endothelial cells plays a role in hypercoagulability in aPL-positive patients. In mouse models of APS, activation of the complement is required and interaction of complement (C) 5a with its receptor C5aR leads to aPL-induced inflammation, placental insufficiency, and thrombosis. Anti-C5 antibody and C5aR antagonist peptides prevent aPL-mediated pregnancy loss and thrombosis in these experimental models. Clinical studies of anti-C5 monoclonal antibody in aPL-positive patients are limited to a small number of case reports. Ongoing and future clinical studies of complement inhibitors will help determine the role of complement inhibition in the management of aPL-positive patients.

  3. [Obliterating diabetic microangiopathy of the diabetic foot--reality or false conclusion?].

    Science.gov (United States)

    Chantelau, E

    1993-08-01

    Occlusive diabetic microangiopathy like diabetic retinopathy and diabetic nephropathy has not been confirmed unequivocally in the diabetic foot. Non-occlusive disturbances, such as impaired vasomotion due to diabetic neuropathy, have been documented in the diabetic foot. Their clinical relevance, however, remains to be established. Recent reports have shown that the histopathological picture of diabetic gangrene is associated with septic vasculitis rather than diabetic microangiopathy. The assumption that diabetic gangrene in the presence of palpable foot pulses is due to occlusive microangiopathy and thus can be treated only by major amputation, appears to be unfounded.

  4. Renal involvement in antiphospholipid syndrome.

    Science.gov (United States)

    Sciascia, Savino; Cuadrado, Maria José; Khamashta, Munther; Roccatello, Dario

    2014-05-01

    Antiphospholipid syndrome (APS) is an autoimmune disease defined by the presence of arterial or venous thrombotic events and/or pregnancy morbidity in patients who test positive for antiphospholipid antibodies (aPLs). APS can be isolated (known as primary APS) or associated with other autoimmune diseases, such as systemic lupus erythematosus (SLE; known as secondary APS). The kidney is a major target organ in APS and renal thrombosis can occur at any level within the vasculature of the kidney (renal arteries, intrarenal arteries, glomerular capillaries and renal veins); events reflect the site and size of the involved vessels. Histological findings vary widely, including ischaemic glomeruli and thrombotic lesions without glomerular or arterial immune deposits on immunofluorescence. Renal prognosis is affected by the presence of aPLs in patients with lupus nephritis and can be poor. In patients with SLE and aPLs, biopsy should be performed because inflammatory and thrombotic lesions require different therapeutic approaches. Renal involvement in patients with definite APS is treated by anticoagulation with long-term warfarin. The range of renal manifestations associated with APS is broadening and, therefore, aPLs have increasing relevance in end-stage renal disease, transplantation and pregnancy.

  5. Thrombotic Events Associated to Aspirin Therapy

    Directory of Open Access Journals (Sweden)

    Christian Doutremepuich

    2012-01-01

    Full Text Available Acetyl salicylic acid (ASA is widely used in clinical practice. Previous studies done in rats showed unexpected thrombotic potencies of this drug used at ultra-low doses. This review is the first report in which the effects of a wide range of ASA concentration on a microvessel model of laser-induced thrombus formation and Induced Hemorrhagic Time in animals were largely studied.

  6. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura:report of three cases

    Institute of Scientific and Technical Information of China (English)

    张文; 尤欣; 董怡

    2004-01-01

    @@ Systemic lupus erythematosus (SLE) is a multisystemic disease characterized by an autoimmune reaction.Thrombotic thrombocytopenic purpura (TTP) is a rare but severe syndrome with the manifestations of fever,thrombocytopenia, microangiopathic hemolysis,neurological symptoms, and renal involvement. The initial prognosis was reported to be dismal, 90% of patients dying within 3 months of onset. However, with the possibility of combined treatments, the survival rate has considerably improved. Data suggest a possible role of immune mechanisms in the development of TTP. 1,2We here report three cases of SLE complicated with TTP,and review the diagnosis, treatment, and outcome of SLE with TTP.

  7. Post-operative thrombotic thrombocytopenic purpura: a review.

    Science.gov (United States)

    Naqvi, T A; Baumann, M A; Chang, J C

    2004-02-01

    Post-operative thrombotic thrombocytopenic purpura (TTP) is a recently recognised life-threatening clinical syndrome with considerable similarity to classic TTP in presentation and response to early treatment with plasma exchange. To date, 29 cases of TTP associated with surgery have been reported. The majority of cases have complicated vascular surgeries, with a few cases seen following gastrointestinal or orthopaedic procedures. Characteristically, patients develop microangiopathic haemolytic anaemia and consumptive thrombocytopenia 5 to 9 days following surgery with variable presence of fever, impaired renal function and altered mental status. The pathogenesis of post-operative TTP is speculative but may involve the release of large amounts of high-molecular-weight von Willebrand factor (vWF) multimers due to endothelial damage resulting from surgery in the setting of marginal levels of vWF-cleaving enzyme. The myriad of common post-surgical complications that may present with clinical manifestations similar to TTP may result in confusion with the potential for delay in the initiation of life-saving plasma-exchange therapy. It is important that physicians be alert to the phenomenon of post-operative TTP so that prompt recognition and treatment will prevent serious morbidity or mortality.

  8. FISIOPATOLOGÍA DE LA INSUFICIENCIA RENAL AGUDA POR CISPLATINO

    Directory of Open Access Journals (Sweden)

    Rodriguez Macías EL

    2013-09-01

    Full Text Available Cisplatin is one of the major antineoplastic used for treatment of tumors such as testicular, ovarian, cervical, lung, bladder and head, among others. It has been described various types of toxicities induced by cisplatin, but the renal is the main one. This toxicity consist sof an acute reduction in renal plasma flow and a decline in glomerular filtration and installation of a tubular necrosis, with predominant involvement of the distal tubules and accumulation of cellular debris (lumen obstruction. Other mechanisms involved in renal injury are microangiopathy and pro-inflammatory mediators. We conclude that the nephrotoxicity caused by cisplatin can induce renal failure in which tubular structural damage predominates.

  9. [Thrombotic thrombocytopenic purpura disclosing cancer: apropos of 2 cases].

    Science.gov (United States)

    Girard, P; Tardy, B; Page, Y; Mosnier, J F; Tardy-Poncet, B; Bertrand, J C

    1995-01-21

    Thrombotic thrombocytopenic purpura (TTP) causes severe haemolytic anaemia, thrombopenia, fever and neurological and renal involvement. Currently five large aetiologic groups have been identified: viral or bacterial infection, drugs, conjunctive tissue diseases, pregnancy and solid tumours. We observed two cases resulting from an adenocarcinoma. In the first case, a 71-year-old man with chronic silicosis, the presenting signs were asthenia, fever, epistaxis with diffus purpura and spontaneous haematomas of the lower limbs. Diagnosis of TTP was based on routine laboratory tests and the patient responded well to fresh frozen plasma. On the 5th day of treatment, haemoglobin level dropped sharply and melana occurred. Upper digestive tract endoscopy revealed a tumoural formation of the antrum-fundic junction and histology examination of the biopsy confirmed the diagnosis of adenocarcinoma. Ten months after gastrectomy the patient was in excellent health with no relapse of the TTP. In the second case, the presenting signs included spontaneous haematomas, rectorrhagia and low grade fever. Microscopic haematuria and renal failure were observed in addition to the biological syndrome of TTP. The patient responded poorly to fresh frozen plasma and packed cell transfusions. Plasma exchange was equally unsuccessful. The disease continued a fulminant course and the diagnosis of adenocarcinoma located in a pulmonary lymph nodes was made at autopsy. These rare cases of TTP caused by cancer emphasize the importance of a thorough aetiological research. Plasma exchange has been shown to be effective but mortality at 1 year approximately 85% in cancer related cases. Early diagnosis and specific anti-cancer therapy might improve prognosis. We report our personal experience with 16 other similar cases.

  10. Thrombotic complications in children with cancer.

    LENUS (Irish Health Repository)

    Smith, O P

    2012-02-01

    The last decade has seen advances in treatment of life-threatening disease in children--especially cancer where the overall cure rate is now in the region of 80%. Similar to adults, children with cancer are at a substantial risk of developing thromboembolism (TE). One of the costs of achieving this has been more children developing thrombotic disease, the majority of which are related to indwelling vascular catheters and as a resultTE is being diagnosed with increasing frequency in these younger patients. In the Canadian Paediatric Thrombophilia Registry, 20% of the patients with TE had cancer. This figure is in contrast to only 2.3 cases of malignancy\\/1000 children and an estimated incidence of thrombosis of 0.7\\/100,000 in the general paediatric population. However, the true prevalence of TE in children with cancer is unknown as rates can vary from 1% to as high as 44% [5] and this reflects the heterogeneity of such studies in terms of; (i) type of cancer, (ii) was the TE, symptomatic or asymptomatic and (iii) were the studies prospective or retrospective. Happening alongside these advances have been an explosion in our knowledge of the understanding at the molecular level of blood coagulation in particular how the natural anticoagulant and fibrinolytic pathways work and how they differ in children and adults. Stemming from these discoveries new anticoagulant therapeutics have become available to the paediatrian and over the next decade their true place in the treatment of childhood thrombotic disease will be established.

  11. Thrombotic risk in the antiphospholipid syndrome.

    Science.gov (United States)

    Lim, Wendy

    2014-10-01

    The antiphospholipid syndrome (APS) is characterized by venous or arterial thrombosis and/or pregnancy morbidity in patients with persistent presence of antiphospholipid antibodies (aPL). Patients who are diagnosed with APS are identified to have a high risk of recurrent thrombosis, which can occur despite anticoagulant therapy. The optimal type, intensity, and duration of anticoagulant therapy for the treatment of APS remain controversial issues, particularly for arterial thrombosis and recurrent thrombosis. Patients with persistently positive testing for lupus anticoagulant and elevated levels of anticardiolipin antibodies and anti-β2 glycoprotein I antibodies-known as triple positivity-appear to be at increased risk for thrombosis compared with patients who test positive for a single aPL. Recognizing that patients with APS may potentially have different thrombotic risk profiles may assist clinicians in assessing the risks, benefits, and optimal duration of anticoagulation. Future studies that delineate thrombotic risk in APS and evaluate current and novel anticoagulants as well as nonanticoagulant therapies are required.

  12. Thrombotic stroke and myocardial infarction with hormonal contraception

    DEFF Research Database (Denmark)

    Lidegaard, Øjvind; Løkkegaard, Ellen; Jensen, Aksel Karl Georg;

    2012-01-01

    Although several studies have assessed the risk of venous thromboembolism with newer hormonal contraception, few have examined thrombotic stroke and myocardial infarction, and results have been conflicting.......Although several studies have assessed the risk of venous thromboembolism with newer hormonal contraception, few have examined thrombotic stroke and myocardial infarction, and results have been conflicting....

  13. The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura

    KAUST Repository

    Lancellotti, S.

    2015-08-13

    Congenital thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy, inherited with autosomal recessive mode as a dysfunction or severe deficiency of ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin 1 repeats Nr. 13), caused by mutations in the ADAMTS-13 gene. About 100 mutations of the ADAMTS-13 gene were identified so far, although only a few characterised by in vitro expression studies. A new Asp to Gly homozygous mutation at position 173 of ADAMTS-13 sequence was identified in a family of Romanian origin, with some members affected by clinical signs of TTP. In two male sons, this mutation caused a severe (< 3 %) deficiency of ADAMTS-13 activity and antigen level, associated with periodic thrombocytopenia, haemolytic anaemia and mild mental confusion. Both parents, who are cousins, showed the same mutation in heterozygous form. Expression studies of the mutant ADAMTS-13, performed in HEK293 cells, showed a severe decrease of the enzyme’s activity and secretion, although the protease was detected inside the cells. Molecular dynamics found that in the D173G mutant the interface area between the metalloprotease domain and the disintegrin-like domain significantly decreases during the simulations, while the proline-rich 20 residues linker region (LR, 285–304) between them undergoes extensive conformational changes. Inter-domain contacts are also significantly less conserved in the mutant compared to the wild-type. Both a decrease of the inter-domain contacts along with a substantial conformational rearrangement of LR interfere with the proper maturation and folding of the mutant ADAMTS-13, thus impairing its secretion.

  14. Neutrophil Protease Cleavage of Von Willebrand Factor in Glomeruli - An Anti-thrombotic Mechanism in the Kidney.

    Science.gov (United States)

    Tati, Ramesh; Kristoffersson, Ann-Charlotte; Manea Hedström, Minola; Mörgelin, Matthias; Wieslander, Jörgen; van Kooten, Cees; Karpman, Diana

    2017-02-01

    Adequate cleavage of von Willebrand factor (VWF) prevents formation of thrombi. ADAMTS13 is the main VWF-cleaving protease and its deficiency results in development of thrombotic microangiopathy. Besides ADAMTS13 other proteases may also possess VWF-cleaving activity, but their physiological importance in preventing thrombus formation is unknown. This study investigated if, and which, proteases could cleave VWF in the glomerulus. The content of the glomerular basement membrane (GBM) was studied as a reflection of processes occurring in the subendothelial glomerular space. VWF was incubated with human GBMs and VWF cleavage was assessed by multimer structure analysis, immunoblotting and mass spectrometry. VWF was cleaved into the smallest multimers by the GBM, which contained ADAMTS13 as well as neutrophil proteases, elastase, proteinase 3 (PR3), cathepsin-G and matrix-metalloproteinase 9. The most potent components of the GBM capable of VWF cleavage were in the serine protease or metalloprotease category, but not ADAMTS13. Neutralization of neutrophil serine proteases inhibited GBM-mediated VWF-cleaving activity, demonstrating a marked contribution of elastase and/or PR3. VWF-platelet strings formed on the surface of primary glomerular endothelial cells, in a perfusion system, were cleaved by both elastase and the GBM, a process blocked by elastase inhibitor. Ultramorphological studies of the human kidney demonstrated neutrophils releasing elastase into the GBM. Neutrophil proteases may contribute to VWF cleavage within the subendothelium, adjacent to the GBM, and thus regulate thrombus size. This anti-thrombotic mechanism would protect the normal kidney during inflammation and could also explain why most patients with ADAMTS13 deficiency do not develop severe kidney failure.

  15. Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.

    Science.gov (United States)

    Soucemarianadin, Myriam; Benhamou, Ygal; Delmas, Yahsou; Pichereau, Claire; Maury, Eric; Pène, Frédéric; Halimi, Jean-Michel; Presne, Claire; Thouret, Jean-Marc; Veyradier, Agnès; Coppo, Paul

    2016-08-01

    Daily therapeutic plasma exchange (TPE) and rituximab improved thrombotic thrombocytopenic purpura (TTP) prognosis. In the more severe cases, salvage therapies including twice-daily TPE and/or cyclophosphamide may be proposed and require evaluation. TTP was defined as a thrombotic microangiopathy (TMA) with severe (TMA registry, we considered those with a severe disease (i.e., unresponsive to daily TPE and rituximab) who received twice-daily TPE. Nineteen of 289 (6.6%) patients with TTP were treated by twice-daily TPE between 2008 and 2014. Twice-daily TPE was associated with rituximab in 16 cases. The median duration of twice-daily TPE treatment was 3 d (2-22 d). In 6 patients (31.6%), additional treatments (mainly pulses of cyclophosphamide) were performed because of a persistently refractory disease (4 cases) or an exacerbation (2 cases), despite twice-daily TPE. Only one patient (5.3%) died. The other 18 achieved a durable complete remission 25.5 d (13-68 d) after the first TPE. The median follow-up was 14.4 months (7 d-45 months). Twice-daily TPE may be an efficient strategy in the more severe TTP patients with a short-term life-threatening disease that could overcome their poor prognosis. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Clinical symptoms and risk factors in cerebral microangiopathy patients.

    Directory of Open Access Journals (Sweden)

    Sandra Okroglic

    Full Text Available OBJECTIVE: Although the clinical manifestation and risk factors of cerebral microangiopathy (CM remain unclear, the number of diagnoses is increasing. Hence, patterns of association among lesion topography and severity, clinical symptoms and demographic and disease risk factors were investigated retrospectively in a cohort of CM patients. METHODS: Patients treated at the Department of Neurology, University of Bonn for CM (n = 223; 98m, 125f; aged 77.32±9.09 from 2005 to 2010 were retrospectively enrolled. Clinical symptoms, blood chemistry, potential risk factors, demographic data and ratings of vascular pathology in the brain based on the Wahlund scale were analyzed using Pearson's chi square test and one-way ANOVA. RESULTS: Progressive cognitive decline (38.1%, gait apraxia (27.8%, stroke-related symptoms and seizures (24.2%, TIA-symptoms (22% and vertigo (17% were frequent symptoms within the study population. Frontal lobe WMLs/lacunar infarcts led to more frequent presentation of progressive cognitive decline, seizures, gait apraxia, stroke-related symptoms, TIA, vertigo and incontinence. Parietooccipital WMLs/lacunar infarcts were related to higher frequencies of TIA, seizures and incontinence. Basal ganglia WMLs/lacunar infarcts were seen in patients with more complaints of gait apraxia, vertigo and incontinence. Age (p = .012, arterial hypertension (p<.000, obesity (p<.000 and cerebral macroangiopathy (p = .018 were positively related to cerebral lesion load. For increased glucose level, homocysteine, CRP and D-Dimers there was no association. CONCLUSION: This underlines the association of CM with neurological symptoms upon admission in a topographical manner. Seizures and vertigo are symptoms of CM which may have been missed in previous studies. In addition to confirming known risk factors such as aging and arterial hypertension, obesity appears to increase the risk as well. Since the incidence of CM is increasing, future

  17. A rare combination of thrombotic thrombocytopenic purpura and antiphospholipid syndrome.

    Science.gov (United States)

    Viner, Maya; Murakhovskaya, Irina

    2016-11-24

    Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system. In the literature, there have only been nine cases of thrombotic thrombocytopenic purpura associated with primary antiphospholipid syndrome. Seven of the nine cases suffered from one or multiple strokes, a common feature in antiphospholipid syndrome, but an uncommon finding in thrombotic thrombocytopenic purpura. We introduce the possibility of an association between thrombotic thrombocytopenic purpura and the presence of antiphospholipid antibodies. Systematic testing of ADAMTS13 activity and anti-ADAMTS13 antibodies in patients who present with neurological symptoms and thrombocytopenia, in the presence of antiphospholipid antibodies, may help with the diagnosis of the rare thrombotic thrombocytopenic purpura-antiphospholipid syndrome combination.

  18. Treatment of 36 cases of thrombotic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    Suvajdžić-Vuković Nada D.

    2004-01-01

    Full Text Available Thirty-six patients (pts. with thrombotic thrombocytopenic purpura (TTP were treated between May 1990 and May 2003. There were 31 women and 5 men; the average age was 37 years. Twenty-five cases were idiopathic and 11 secondary (3 infection - related, 5 occurred during pregnancy and 3 were drug - associated. The mean lag period between the first symptoms and the diagnosis was 8.5 days (in 14 pts. £ 5; in 22 > 5. On diagnosis neurological symptoms were present in 31, bleeding in 33, fever in 21 and renal impairment in 27 patients. The mean hemoglobin was 67.5 g/L, the mean platelet count was 10´109/L, and the mean reticulocytosis was 17%. The mean serum LDH was 1457 IU. Treatment included plasma exchange (PE in 24 pts. and only plasma infusions in 12 pts. There were 24 complete responders (20 on PE and 12 deaths (4 on PE; PE significantly improved survival (p<0.01. There were 5 treatment-related complications due to the infection and bleeding, 17 exacerbations and 4 relapses. The mean time delay before the onset of symptoms and the treatment initiation lasted for 9 days suggesting the poor disease recognition; the mean time delay from diagnosis to PE institution was 6 days, indicating postponed PE. The mean treatment duration in all patients was 18 days; the mean number of PE cycles needed for the platelet count stabilization was 9. Good prognostic indicators of survival were: the longer prodromal period (>5 days, the secondary form of TTP and the absence of coma at presentation. The use of PE significantly improved survival. TTP is a severe disorder requiring early recognition and diagnosis in general medical care facilities, which should lead to the timely treatment with PE.

  19. Thrombosis in end-stage renal disease.

    Science.gov (United States)

    Casserly, Liam F; Dember, Laura M

    2003-01-01

    Although renal failure has classically been associated with a bleeding tendency, thrombotic events are common among patients with end-stage renal disease (ESRD). A variety of thrombosis-favoring hematologic alterations have been demonstrated in these patients. In addition, "nontraditional" risk factors for thrombosis, such as hyperhomocysteinemia, endothelial dysfunction, inflammation, and malnutrition, are present in a significant proportion of chronic dialysis patients. Hemodialysis (HD) vascular access thrombosis, ischemic heart disease, and renal allograft thrombosis are well-recognized complications in these patients. Deep venous thrombosis and pulmonary embolism are viewed as rare in chronic dialysis patients, but recent studies suggest that this perception should be reconsidered. Several ESRD treatment factors such as recombinant erythropoietin (EPO) administration, dialyzer bioincompatibility, and calcineurin inhibitor administration may have prothrombotic effects. In this article we review the pathogenesis and clinical manifestations of thrombosis in ESRD and evaluate the evidence that chronic renal failure or its management predisposes to thrombotic events.

  20. Refractory thrombotic thrombocytopenic purpura following acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Ebisa Bekele; Bethel Shiferaw; Alexandra Sokolova; Arpan Shah; Phillip Saunders; Alida Podrumar; Javed Iqbal

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13) activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6%after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  1. Neonatal renal vein thrombosis.

    Science.gov (United States)

    Brandão, Leonardo R; Simpson, Ewurabena A; Lau, Keith K

    2011-12-01

    Neonatal renal vein thrombosis (RVT) continues to pose significant challenges for pediatric hematologists and nephrologists. The precise mechanism for the onset and propagation of renal thrombosis within the neonatal population is unclear, but there is suggestion that acquired and/or inherited thrombophilia traits may increase the risk for renal thromboembolic disease during the newborn period. This review summarizes the most recent studies of neonatal RVT, examining its most common features, the prevalence of acquired and inherited prothrombotic risk factors among these patients, and evaluates their short and long term renal and thrombotic outcomes as they may relate to these risk factors. Although there is some consensus regarding the management of neonatal RVT, the most recent antithrombotic therapy guidelines for the management of childhood thrombosis do not provide a risk-based algorithm for the acute management of RVT among newborns with hereditary prothrombotic disorders. Whereas neonatal RVT is not a condition associated with a high mortality rate, it is associated with significant morbidity due to renal impairment. Recent evidence to evaluate the effects of heparin-based anticoagulation and thrombolytic therapy on the long term renal function of these patients has yielded conflicting results. Long term cohort studies and randomized trials may be helpful to clarify the impact of acute versus prolonged antithrombotic therapy for reducing the morbidity that is associated with neonatal RVT.

  2. MEAN PLATELET VOLUME AND RISK OF THROMBOTIC STROKE

    Directory of Open Access Journals (Sweden)

    Prasantha Kumar Thankappan

    2017-07-01

    Full Text Available BACKGROUND Stroke is a major cause of long term morbidity and mortality. Several factors are known to increase the liability to stroke. Platelets play a crucial role in the pathogenesis of atherosclerotic complications, contributing to thrombus formation. Platelet size (mean platelet volume, MPV is a marker and possible determinant of platelet function, large platelets being potentially more reactive. Hence an attempt has-been made to study the association if any between mean platelet volume and thrombotic stroke. The aim of this study was to determine whether an association exists between Mean Platelet Volume (MPV and thrombotic stroke. MATERIALS AND METHODS The study is a case control study and data was collected at Government Medical College Hospital, Kottayam, Kerala a tertiary care referral centre. The study was carried out among fifty patients diagnosed with thrombotic stroke and presenting to the hospital within forty eight hours of onset of symptoms. Fifty age group and sex matched controls were also recruited. Mean platelet volume was obtained using a SYSMEX automated analyser. RESULTS This study has shown a statistically significant relation between mean platelet volume and risk of thrombotic stroke but no statistically significant correlation between clinical severity of stroke and mean platelet volume. CONCLUSION This study has shown an elevation of MPV in acute phase of thrombotic stroke. Platelet mass was found to be more or less a constant. This study did not find a statistically significant correlation between clinical severity of stroke and mean platelet volume.

  3. Wandering spleen: 'presentation in adolescent with high thrombotic risk'.

    Science.gov (United States)

    Tchidjou, Hyppolite K; Castelluzzo, Maria A; Messia, Virginia; Luciani, Matteo; Monti, Lidia; Grimaldi, Chiara; Bernardi, Stefania; D'Argenio, Patrizia

    2014-07-01

    The term 'wandering spleen' refers to an abnormal hypermobility of the spleen, which may be congenital or acquired. The absence or abnormal laxity of splenic ligaments combined with an abnormally long and mobile vascular pedicle predispose to complications such as torsion of the splenic pedicle, infarction and splenic vein thrombosis. The clinical presentation of such disease is highly variable. In this case, we describe an asymptomatic case of wandering spleen in high thrombotic risk patients with cavernoma of splenic vein and infarction of the spleen. Physical examination was normal except the enlarged and no tender consistency spleen palpable at left iliac fossa. Ultrasonography revealed enlarged spleniform mass below its normal position suggesting vascular impairment and subsequently has been confirmed by colour Doppler ultrasound and computed tomography. The family history was positive for ischemic thrombotic vascular diseases and the screening for thrombotic risk has revealed hyperhomocysteinemia, thrombophilic homozygous gene mutations for factor V (H1299R) and MTHFR (C677T). For high thrombotic risk, prophylaxis postsplenectomy was suggested according to the international recommendations with subcutaneous low molecular weight heparin, associated with a preventive treatment with acetyl salicylic acid and folic acid along with B-vitamin. This case report may be helpful for clinicians involved in the care of splenectomized patients, because it has shown the importance of an appropriate pre and postoperative antithrombotic management to reduce as soon as possible the risk of thrombotic events in such patients after splenectomy.

  4. Microangiopatia livedóide associada à síndrome do anticorpo antifosfolípide (SAF Livedoid microangiopathy associated to antiphospholipid antibody syndrome (APS

    Directory of Open Access Journals (Sweden)

    Carla Munhoz Sanches

    2005-12-01

    argues against vasculitis, favouring a thrombotic process. Livedoid microangiopathy attacks mainly young and middle-aged women; can be idiopathic, or associated with coagulation alterations including the factor V Leiden mutation, protein C deficiency, increased plasmatic homocysteine, fibrinolysis abnormalities, platelet activation and antiphospholipid antibody syndrome (APS. We describe a case of a patient with livedoid microangiopathy associated with the presence of APS with multiple ulcers in the lower limbs who showed a clinical improvement only after total anticoagulation with warfarin and association with danazol. Livedoid vasculitis can represent an initial clinical manifestation of a group of diseases which cause occlusive vasculopathy; so, every patient should be investigated for the presence of antiphospholipid antibody or of another cause of thrombophilia.

  5. Renal infarct: a rare disease due to a rare etiology

    Science.gov (United States)

    Akshintala, Divya; Bansal, Saurabh K.; Emani, Vamsi Krishna; Yadav, Manajyoti

    2015-01-01

    Renal infarction is caused by profound hypoperfusion secondary to embolic/thrombotic occlusion of the renal artery or vasospasm of the renal artery. We present a case of a 54-year-old patient who presented with nausea, vomiting, and vague abdominal pain. He had frequent episodes of migraine headaches and he treated himself with as needed rizatriptan. CT scan of the abdomen showed renal cortical infarction. After extensive investigations, etiology of his renal infarct was deemed to be due to rizatriptan. PMID:26091657

  6. Renal infarct: a rare disease due to a rare etiology

    Directory of Open Access Journals (Sweden)

    Divya Akshintala

    2015-06-01

    Full Text Available Renal infarction is caused by profound hypoperfusion secondary to embolic/thrombotic occlusion of the renal artery or vasospasm of the renal artery. We present a case of a 54-year-old patient who presented with nausea, vomiting, and vague abdominal pain. He had frequent episodes of migraine headaches and he treated himself with as needed rizatriptan. CT scan of the abdomen showed renal cortical infarction. After extensive investigations, etiology of his renal infarct was deemed to be due to rizatriptan.

  7. Serum adiponectin predicts all-cause mortality and end stage renal disease in patients with type I diabetes and diabetic nephropathy

    DEFF Research Database (Denmark)

    Jorsal, A.; Tarnow, L.; Frystyk, J.

    2008-01-01

    Adiponectin levels are increased in patients with type I diabetes especially in the presence of microangiopathy. Here we determined the predictive value of serum adiponectin levels and 8 adiponectin gene polymorphisms for mortality, cardiovascular events and end-stage renal disease in type I diab...

  8. Active von Willebrand factor in thrombotic thrombocytopenic purpura and malaria

    NARCIS (Netherlands)

    Groot, E.

    2009-01-01

    Thrombotic thrombocytopenic purpura (TTP) and malaria are two diseases of distinct origin. TTP is a rare disorder caused by a deficiency of the von Willebrand factor (VWF) cleaving protease ADAMTS13. Malaria is a poverty-related disease caused by protozoan parasites from the genus Plasmodium. TTP an

  9. Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's syndrome.

    Science.gov (United States)

    Watanabe, Ryu; Shirai, Tsuyoshi; Tajima, Yumi; Ohguchi, Hiroto; Onishi, Yasushi; Fujii, Hiroshi; Takasawa, Naruhiko; Ishii, Tomonori; Harigae, Hideo

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP), scleroderma renal crisis (SRC), and hemolysis, elevated liver enzyme levels, and a low platelet count (HELLP) syndrome display common symptoms that include microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Therefore, it is important to distinguish between them because their treatments vary: however, the differential diagnosis is sometimes difficult. We report a 32-year-old woman who was referred to our department for further examination of microangiopathic hemolytic anemia, thrombocytopenia, and a slightly elevated serum creatinine level with anti-centromere antibody-positive Raynaud's syndrome in the early puerperal period. TTP, SRC, and HELLP syndrome were considered in the differential diagnosis, but the measurement of a disintegrin-like metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS 13) activity and its inhibitor level led to the diagnosis of TTP. She was successfully treated by plasma exchange and high-dose prednisolone and angiotensin-converting enzyme inhibitor. If microangiopathic hemolytic anemia and thrombocytopenia are observed in perinatal women or patients with signs of systemic sclerosis, the measurement of ADAMTS13 activity and its inhibitor level are essential for diagnosis and therapeutic choice.

  10. Rapid relief of signs/symptoms in chronic venous microangiopathy with pycnogenol: a prospective, controlled study.

    Science.gov (United States)

    Cesarone, M R; Belcaro, G; Rohdewald, P; Pellegrini, L; Ledda, A; Vinciguerra, G; Ricci, A; Gizzi, G; Ippolito, E; Fano, F; Dugall, M; Acerbi, G; Cacchio, M; Di Renzo, A; Hosoi, M; Stuard, S; Corsi, M

    2006-01-01

    The aim of this study was to investigate the clinical efficacy of oral Pycnogenol (Horphag Research Ltd, UK) in patients with severe chronic venous insufficiency. Patients with severe venous hypertension (chronic venous insufficiency, ankle swelling) and history of venous ulcerations were treated with Pycnogenol. Patients received oral Pycnogenol (50 mg capsules, 3 times daily for a total of 150 mg daily) for 8 weeks. A group of 21 patients was included in the treatment group and 18 equivalent patients were observed as controls (no treatment during the observation period). All 21 patients (age 53 years; range, 42-60 years; M:F=11:10) in the treatment group completed the 8-week study. Also the 18 controls completed the follow-up period. There were no drop-outs. The average ambulatory venous pressure was 59.3 (SD 7.2; range 50-68) with a refilling time shorter than 10 seconds (average 7.6; SD 3). There were no differences in ambulatory venous pressure or refilling time between the treatment and control patients. The duration of the disease-from the first signs/symptoms-was on average 5.7 years (SD 2.1). At 4 and 8 weeks, in all Pycnogenol-treated subjects, microcirculatory and clinical evaluations indicated a progressive decrease in skin flux, indicating an improvement in the level of microangiopathy; a significant decrease in capillary filtration; a significant improvement in the symptomatic score; and a reduction in edema. There were no visible effects in controls. In conclusion, this study confirms the fast clinical efficacy of Pycnogenol in patients with chronic venous insufficiency and venous microangiopathy. The study indicates the significant clinical role of Pycnogenol in the management, treatment and control of this common clinical problem. The treatment may be also useful to prevent ulcerations by controlling the level of venous microangiopathy.

  11. Increased vascular resistance in vasodilated skin an indicator of diabetic microangiopathy?

    DEFF Research Database (Denmark)

    Faris, I B; Lassen, N A

    1982-01-01

    The method of isotope clearance has been used to investigate the vascular resistance in skin in severely atherosclerotic patients with and without diabetes. Dilatation of the skin vessels was achieved by the injection of histamine with a radioactive tracer (99mTc). The perfusion pressure...... of the skin was varied by applying graded external counter pressure over the injected area. The blood flow at any given perfusion pressure was lower (and thus the resistance higher) in the diabetic subjects (P = 0.01). These results could be explained if there was microangiopathy affecting the skin vessels....

  12. Decreased distensibility of resistance vessels of the skin in type 1 (insulin-dependent) diabetic patients with microangiopathy

    DEFF Research Database (Denmark)

    Kastrup, J; Nørgaard, T; Parving, H H

    1987-01-01

    The distensibility of the resistance vessels of the skin at the dorsum of the foot was determined in 11 long-term type 1 (insulin-dependent) diabetic patients with nephropathy and retinopathy, nine short-term type 1 diabetic patients without clinical microangiopathy and in nine healthy non-diabetic...... during head-up tilt was only 24% in diabetic subjects with and 48% in diabetic patients without clinical microangiopathy, compared with 79% in normal non-diabetic subjects (P less than 0.0005 and P less than 0.05, respectively). An inverse correlation between microvascular distensibility and degree...

  13. MDCT of acute thrombotic and nonthrombotic pulmonary emboli.

    Science.gov (United States)

    Bhalla, Sanjeev; Lopez-Costa, Ignacio

    2007-10-01

    Acute pulmonary embolism (PE) remains a common clinical challenge. MDCT pulmonary angiography has become the first line imaging study in the diagnosis of PE because of its speed, accuracy, low-interobserver variability, and ability to provide alternative diagnoses. This review article highlights the role of MDCT in the evaluation of acute thrombotic PE in the era of PIOPED 2. MDCT findings of acute PE and some potential pitfalls are covered as well as some of the controversies in imaging young and pregnant patients. MDCT findings of acute non-thrombotic PE are also covered. This latter group may be occult on the angiographic portion of the study but may declare themselves through secondary findings. Their findings and potential mimics are included so that the interpreting radiologist can make the most of a CT to rule out PE.

  14. MDCT of acute thrombotic and nonthrombotic pulmonary emboli

    Energy Technology Data Exchange (ETDEWEB)

    Bhalla, Sanjeev [Division of Cardiothoracic Imaging, Mallinckrodt Institute of Radiology, 510 South Kingshighway Blvd., St. Louis, MO 63110 (United States)], E-mail: bhallas@mir.wustl.edu; Lopez-Costa, Ignacio [Division of Cardiothoracic Imaging, Mallinckrodt Institute of Radiology, 510 South Kingshighway Blvd., St. Louis, MO 63110 (United States)], E-mail: ignacio.lc@mac.com

    2007-10-15

    Acute pulmonary embolism (PE) remains a common clinical challenge. MDCT pulmonary angiography has become the first line imaging study in the diagnosis of PE because of its speed, accuracy, low-interobserver variability, and ability to provide alternative diagnoses. This review article highlights the role of MDCT in the evaluation of acute thrombotic PE in the era of PIOPED 2. MDCT findings of acute PE and some potential pitfalls are covered as well as some of the controversies in imaging young and pregnant patients. MDCT findings of acute non-thrombotic PE are also covered. This latter group may be occult on the angiographic portion of the study but may declare themselves through secondary findings. Their findings and potential mimics are included so that the interpreting radiologist can make the most of a CT to rule out PE.

  15. Delayed onset of fatal basilar thrombotic embolus after whiplash injury

    DEFF Research Database (Denmark)

    Viktrup, L; Knudsen, G M; Hansen, S H

    1995-01-01

    BACKGROUND: Whiplash injuries are generally seen after rear-end rather than frontal car collisions. Previous reports have documented death up to 8 days after serious whiplash injury. We report a case of lethal basilar thrombotic embolus that occurred 2 months after the patient's injury...... in a collision. CASE DESCRIPTION: After whiplash trauma in a car accident, a 50-year-old taxi driver suffered from headache and episodic visual disturbances. Two months after the accident he suddenly lost consciousness and was admitted to the hospital. A CT scan performed at that time was indicative of basilar...... thrombosis. The patient died 3 days later. The autopsy revealed a thrombosis in the right vertebral artery and a thrombotic embolus in the basilar artery. Microscopically, a lesion of the right vertebral artery was found at the level of the atlantoaxial joint. CONCLUSIONS: We conclude that the whiplash...

  16. Anti-thrombotic effects of selective estrogen receptor modulator tamoxifen.

    Science.gov (United States)

    Nayak, Manasa K; Singh, Sunil K; Roy, Arnab; Prakash, Vivek; Kumar, Anand; Dash, Debabrata

    2011-10-01

    Tamoxifen is a known anti-cancer drug and established estrogen receptor modulator. Few clinical studies have earlier implicated the drug in thrombotic complications attributable to lower anti-thrombin and protein S levels in plasma. However, action of tamoxifen on platelet signalling machinery has not been elucidated in detail. In the present report we show that tamoxifen is endowed with significant inhibitory property against human platelet aggregation. From a series of in vivo and in vitro studies tamoxifen was found to inhibit almost all platelet functions, prolong tail bleeding time in mouse and profoundly prevent thrombus formation at injured arterial wall in mice, as well as on collagen matrix perfused with platelet-rich plasma under arterial shear against the vehicle dimethylsulfoxide (DMSO). These findings strongly suggest that tamoxifen significantly downregulates platelet responses and holds potential as a promising anti-platelet/anti-thrombotic agent.

  17. Thrombotic thrombocytopenic purpura in the first trimester of pregnancy

    Directory of Open Access Journals (Sweden)

    Pooja Sikka

    2013-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP occurs more commonly in women and so can be associated with pregnancy. The time during pregnancy with greatest risk for development of TTP is near term and during the post partum period. TTP occurring in early trimester is uncommon and is also associated with great maternal and fetal mortality. We report a successful outcome of pregnancy in a woman with TTP in early first trimester who was treated with therapeutic plasma exchange.

  18. Refractor y thrombotic thrombocytopenic pur pura following acute pancreatitis

    OpenAIRE

    Ebisa Bekele; Bethel Shiferaw; Alexandra Sokolova; Arpan Shah; Phillip Saunders; Alida Podrumar; Javed Iqbal

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female ...

  19. Haemorrhagic and thrombotic diatheses in mouse models with thrombocytosis.

    Science.gov (United States)

    Strassel, Catherine; Kubovcakova, Lucia; Mangin, Pierre H; Ravanat, Catherine; Freund, Monique; Skoda, Radek C; Denis, Cécile V; Dupuis, Arnaud; Herbrecht, Raoul; Gachet, Christian; Lanza, François

    2015-02-01

    We studied haemostasis in two mouse models with thrombocytosis caused by different pathogenic mechanisms. In one strain (Yall;Mpl-/-) thrombocytosis is driven by a misbalance between thrombopoietin and its receptor, whereas in the other strain, thrombocytosis is caused by expressing a human JAK2-V617F transgene (FF1) that depends on activation by Cre-recombinase (VavCre;FF1, MxCre;FF1). Thrombotic responses were increased following some, but not all types of challenges. In a vaso-occlusive thrombotic model following collagen-adrenaline injection we found increased mortality in both strains. Arterial thrombosis, examined after ferric chloride-induced carotid injury, was accelerated but with little impact on maximal thrombus size. In a vena cava stasis model, clots were of similar size as in wild-type controls, but exhibited a different composition with a higher platelet to fibrin ratio. Both thrombocytosis strains displayed increased haemorrhagic tendency in a tail bleeding assay. Yall;Mpl and VavCre;FF1 displayed a lower proportion of the more reactive high-molecular-weight forms of von Willebrand factor in their plasma, mimicking essential thrombocythaemia with very high platelet counts. Bleeding could not be explained by clear defects in platelet activation, which were normal or only weakly decreased. In conclusion, these models of thrombocytosis recapitulate several features of the haemorrhagic and thrombotic diatheses in ET and PV demonstrating potentials but also some limitations to study these major complications.

  20. [Dental treatment and anti-thrombotic therapy. Part II: the era of new anti-thrombotic drugs].

    Science.gov (United States)

    Chackartchi, T; Sachar Helft, S; Findler, M

    2014-01-01

    Surgical intra-oral treatment for patients under antithrombotic therapy presents a challenge for the dental team. Within the last few years evidence based systematic reviews established new clinical guidelines for wide groups of patients which need to use antithrombotic treatment. The expected increase in use of antithrombotic treatment forced the pharmaceutical industry to provide new treatments. The former anticoagulant and anti-platelets aggregation groups of drugs were limited to small variety of medication. The search for the new treatments with ideal properties led to newly invented groups of drugs. In this article we will describe the new advancements in anti-thrombotic treatments. The article will summarize the limited knowledge of surgical management of patients under the new anti-thrombotic medications and the recommended approach for oral surgical procedures.

  1. Aldose reductase C-106T gene polymorphism in type 2 diabetics with microangiopathy in Iranian individuals

    Directory of Open Access Journals (Sweden)

    Majid Reza Sheikh Rezaee

    2015-01-01

    Full Text Available Background: Aldose reductase (AR is the rate-limiting enzyme in the glucose metabolism, which has been implicated in the pathogenesis of diabetic microvascular complications (MVCs. Frequent C-106T polymorphism in the promoter of the AR gene may change the expression of the gene. Aims: The aim of the following study is to study the association between AR C106T genotypes and diabetic MVCs in Iranian population. Materials and Methods: We included 206 type 2 diabetic patients categorized into two groups according to the presence or absence of diabetic microangiopathy. The cases of interest were diabetic neuropathy, retinopathy and nephropathy identified during clinical and or laboratory examination. In addition, 114 age- and sex-matched individuals were selected to serve as a control group. AR genotyping was done using an amplification gel electrophoresis. Results: The frequency of CC genotype was specifically higher in subjects with diabetic retinopathy as compared to those without it (53.2% vs. 38.1%, P = 0.030. Patients with diabetic microangiopathy in general; however, did not differ significantly between AR genotype groups. Conclusion: The C-106T polymorphism in the AR gene is likely a risk factor for development of only retinal complication of diabetes microvascular in Iranian individuals.

  2. Angiotensinogen gene promoter haplotype and microangiopathy-related cerebral damage: results of the Austrian Stroke Prevention Study

    NARCIS (Netherlands)

    H. Schmidt (Helena); F. Fazekas (Franz); G.M. Kostner; R. Schmidt (Reinhold); C.M. van Duijn (Cock)

    2001-01-01

    textabstractBACKGROUND AND PURPOSE: Microangiopathy-related cerebral damage (MARCD) is a common finding in the elderly. It may lead to cognitive impairment and gait disturbances. Arterial hypertension and age are the most important risk factors. We assessed the association between MARCD and sequence

  3. Predicting post-traumatic stress and health anxiety following a venous thrombotic embolism.

    Science.gov (United States)

    Bennett, Paul; Patterson, Katie; Noble, Simon

    2016-05-01

    This research identified psychosocial factors associated with post-traumatic stress and health anxiety following a venous thrombotic embolism. In all, 158 participants, largely registered with a venous thrombotic embolism information website (Lifeblood: The Thrombosis Charity), completed an online survey. Post-traumatic symptom scores were linked to health threat, and not moderated by perceived control over risk for further venous thrombotic embolism. Health anxiety was associated with continuing symptoms and a negative emotional response to the venous thrombotic embolism. There is a need to intervene to reduce both short- and long-term distress in this population, ideally using a stepped-care model.

  4. Isotopic test of capillary permeability to albumin in diabetic patients: effects of hypertension, microangiopathy, and duration of diabetes

    Energy Technology Data Exchange (ETDEWEB)

    Valensi, P.; Attali, J.R.; Behar, A.; Sebaoun, J.

    1987-09-01

    Capillary permeability to albumin (CPA) was studied by performing an isotopic noninvasive test with venous compression on 87 nonselected diabetics with no edema, no cardiac failure, and no peripheral vascular disease. Excessive albumin retention (AR greater than or equal to 8%) ten minutes after removal of the compression was found in 27 patients (31%). The radioactivity disappearance curve was then analyzed using the Fast Fourier Transform (FFT). An abnormal isotopic CPA test was thus found in at least 45 out of the 87 patients. The prevalence of an abnormal test was not different in type 1 and type 2 diabetics. We studied the independent effects of hypertension, presence of specific clinical signs of microangiopathy (retinopathy and/or significant proteinuria), and duration of diabetes. Among diabetics free of specific clinical signs of microangiopathy, the prevalence of an AR greater than or equal to 8% was significantly higher in those with hypertension (11/19) than in those with normal blood pressure (2/28) and in nondiabetic hypertensive patients (0/16). Among normotensive diabetics, the prevalence of an abnormal test was higher, but not significantly, in patients with specific clinical signs of microangiopathy (8/11) than in those free of them (7/18). Seven normotensive diabetics without specific clinical signs of microangiopathy had an abnormal test; five of them had had diabetes for more than five years. The prevalence of diabetes of more than five years duration was significantly higher in patients with an abnormal test (35/45) than in normotensive diabetics free of specific clinical signs of microangiopathy with a normal test (4/11).

  5. Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    João Tadeu Damian Souto Filho

    2011-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD is extremely rare and has only been described in nine patients. We describe the case of a 42-year-old female with MCTD who developed thrombocytopenia, microangiopathic hemolytic anemia, fever, and neurological symptoms. The patient had a good clinical evolution with infusion of high volume of fresh frozen plasma, steroid therapy, and support in an intensive care unit. Although the occurrence of TTP is rare in MCTD patients, it is important to recognize TTP as a cause of thrombocytopenia and hemolytic anemia in any patient with autoimmune diseases. Prompt institution of treatment remains the cornerstone of treatment of TTP even if plasma exchange is not available like what frequently happens in developing countries.

  6. Acute pancreatitis-induced thrombotic thrombocytopenic purpura with recurrent acute pancreatitis.

    Science.gov (United States)

    Fujino, Yasuhisa; Inoue, Yoshihiro; Onodera, Makoto; Kikuchi, Satoshi; Sato, Masayuki; Kojika, Masahiro; Sato, Hisaho; Suzuki, Keijiro; Matsumoto, Masanori

    2016-04-01

    Recent successive reports on acute pancreatitis-induced thrombotic thrombocytopenic purpura (TTP) have revealed that TTP-related microvascular damage is an aggravating factor of acute pancreatitis. Here, we report the case of a 26-year-old man diagnosed with acute pancreatitis due to high alcohol consumption. The patient was unconscious as he had taken an overdose of medication, and presented with fever and renal failure due to acute pancreatitis on admission. Although the pancreatitis subsequently improved, the symptoms were still observed; on the next day, he exhibited hemoglobinuria, anemia, and thrombocytopenia. Moreover, general blood examinations indicated the presence of schistocytes and reduced activity of ADAMTS13 (a disintegrin-like metalloproteinase with thrombospondin type 1 motif 13) to 47 %. Thus, the patient was diagnosed with TTP, and plasma exchange was performed. After the development of TTP, the acute pancreatitis recurred, but a severe pathogenesis was prevented by plasma exchange. Thus, ADAMTS13 activity may be useful for predicting a severe pathogenesis of acute pancreatitis. In ADAMTS13-deficient cases, plasma exchange may be an effective technique for preventing aggravation of acute pancreatitis.

  7. Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice.

    Science.gov (United States)

    Morioka, Yoko; Casari, Caterina; Wohner, Nikolett; Cho, Sungyun; Kurata, Sachiko; Kitano, Ayumi; Christophe, Olivier D; Lenting, Peter J; Li, Renhao; Denis, Cécile V; Prévost, Nicolas

    2014-05-22

    Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that presents with thrombocytopenia, disseminated thrombosis, hemolytic anemia, and organ dysfunction. The etiology of TTP has revealed that patients share a deficiency in plasma protease a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), the enzyme responsible for cleaving ultra-large von Willebrand factor (VWF) multimers into nonthrombogenic fragments. Therefore, existing TTP mouse models were developed by targeted disruption of the ADAMTS13 gene. ADAMTS13(-/-) mice are mostly asymptomatic in the absence of a trigger, as redundant proteases appear to take on VWF processing. As an alternative approach to creating one such model, we devised a strategy based on the expression of a cleavage-resistant VWF mutant in mice. The creation of a disulfide bond within the A2 domain of VWF was found to render VWF multimers resistant to proteolysis by plasma proteases under flow. Furthermore, mice expressing the murine VWF/p.S1494C-p.A1534C mutant present with symptoms characteristics of acute TTP such as thrombocytopenia, red cell shredding, accumulation of VWF-rich thrombi in the microvasculature, and advanced TTP symptoms such as renal dysfunction and splenomegaly. Because this model appears to faithfully emulate the pathophysiology of TTP, it should prove most useful in the study of microangiopathic diseases and their treatment. © 2014 by The American Society of Hematology.

  8. Enhancement of study on hemostatic and thrombotic disorders

    Institute of Scientific and Technical Information of China (English)

    阮长耿

    2004-01-01

    @@ Hemostasis and thrombosis is a discipline combining various aspects of basic biology and clinical medicine. Hemostatic abnormalities and thrombosis are involved in the pathophysiology of many diseases in clinical medicine. The fundamental and clinical research of the bleeding disorders and thrombotic diseases has been developed very quickly in recent years. More than 100 genes have been found to be related with hemostasis and thrombosis. The genetic diagnosis of bleeding disorders, including hemophilia A, hemophilia B, von Willebrand disease and Glanzmann's thrombasthenia, has been greatly improved in China.1

  9. Study of Autophagy and Microangiopathy in Sural Nerves of Patients with Chronic Idiopathic Axonal Polyneuropathy

    Science.gov (United States)

    Samuelsson, Kristin; Osman, Ayman A. M.; Angeria, Maria; Risling, Mårten; Mohseni, Simin; Press, Rayomand

    2016-01-01

    Twenty-five percent of polyneuropathies are idiopathic. Microangiopathy has been suggested to be a possible pathogenic cause of chronic idiopathic axonal polyneuropathy (CIAP). Dysfunction of the autophagy pathway has been implicated as a marker of neurodegeneration in the central nervous system, but the autophagy process is not explored in the peripheral nervous system. In the current study, we examined the presence of microangiopathy and autophagy-related structures in sural nerve biopsies of 10 patients with CIAP, 11 controls with inflammatory neuropathy and 10 controls without sensory polyneuropathy. We did not find any significant difference in endoneurial microangiopathic markers in patients with CIAP compared to normal controls, though we did find a correlation between basal lamina area thickness and age. Unexpectedly, we found a significantly larger basal lamina area thickness in patients with vasculitic neuropathy. Furthermore, we found a significantly higher density of endoneurial autophagy-related structures, particularly in patients with CIAP but also in patients with inflammatory neuropathy, compared to normal controls. It is unclear if the alteration in the autophagy pathway is a consequence or a cause of the neuropathy. Our results do not support the hypothesis that CIAP is primarily caused by a microangiopathic process in endoneurial blood vessels in peripheral nerves. The significantly higher density of autophagy structures in sural nerves obtained from patients with CIAP and inflammatory neuropathy vs. controls indicates the involvement of this pathway in neuropathy, particularly in CIAP, since the increase in density of autophagy-related structures was more pronounced in patients with CIAP than those with inflammatory neuropathy. To our knowledge this is the first report investigating signs of autophagy process in peripheral nerves in patients with CIAP and inflammatory neuropathy. PMID:27662650

  10. Diminished Alveolar Microvascular Reserves in Type 2 Diabetes Reflect Systemic Microangiopathy

    Science.gov (United States)

    Chance, William W.; Rhee, Chanhaeng; Yilmaz, Cuneyt; Dane, D. Merrill; Pruneda, M. Lourdes; Raskin, Philip; Hsia, Connie C.W.

    2008-01-01

    OBJECTIVE—Alveolar microvascular function is moderately impaired in type 1 diabetes, as manifested by restriction of lung volume and diffusing capacity (DLCO). We examined whether similar impairment develops in type 2 diabetes and defined the physiologic sources of impairment as well as the relationships to glycemia and systemic microangiopathy. RESEARCH DESIGN AND METHODS—A cross-sectional study was conducted at a university-affiliated diabetes treatment center and outpatient diabetes clinic, involving 69 nonsmoking type 2 diabetic patients without overt cardiopulmonary disease. Lung volume, pulmonary blood flow (Q̇), DLCO, membrane diffusing capacity (measured from nitric oxide uptake [DLNO]), and pulmonary capillary blood volume (VC) were determined at rest and exercise for comparison with those in 45 healthy nonsmokers as well as with normal reference values. RESULTS—In type 2 diabetic patients, peak levels of oxygen uptake, Q̇ and DLCO, DLNO, and VC at exercise were 10–25% lower compared with those in control subjects. In nonobese patients (BMI DLNO, and VC were fully explained by the lower lung volume and peak Q̇, but these factors did not fully explain the impairment in obese patients (BMI >30 kg/m2). The slope of the increase in VC with respect to Q̇ was reduced ∼20% in patients regardless of BMI, consistent with impaired alveolar-capillary recruitment. Functional impairment was directly related to A1C level, retinopathy, neuropathy, and microalbuminuria in a sex-specific manner. CONCLUSIONS—Alveolar microvascular reserves are reduced in type 2 diabetes, reflecting restriction of lung volume, alveolar perfusion, and capillary recruitment. This reduction correlates with glycemic control and extrapulmonary microangiopathy and is aggravated by obesity. PMID:18492945

  11. A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis

    Directory of Open Access Journals (Sweden)

    Arimoto M

    2012-03-01

    Full Text Available Miyoko Arimoto1, Yutaka Komiyama2, Fumiko Okamae1, Akemi Ichibe1, Setsuko Teranishi1, Hirohiko Tokunaga1, Keiko Nakaya3, Michie Fujiwara3, Manabu Yamaoka4, Shuji Onishi4, Rie Miyamoto5, Naoto Nakamichi5, Shosaku Nomura51Blood Transfusion Unit, Kansai Medical University Takii Hospital, 2Department of Clinical Sciences and Laboratory Medicine, Kansai Medical University, 3Clinical Medical Technology Unit, Kansai Medical University Takii Hospital, 4Blood Transfusion Unit, Kansai Medical University Hirakata Hospital, 5First Department of Internal Medicine, Kansai Medical University, Moriguchi, JapanAbstract: Thrombotic thrombocytopenic purpura (TTP is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.Keywords: thrombotic thrombocytopenic purpura, ADAMTS13, acute pancreatitis, plasma exchange

  12. In vitro Anti-Thrombotic Activity of Extracts from Blacklip Abalone (Haliotis rubra Processing Waste

    Directory of Open Access Journals (Sweden)

    Hafiz Ansar Rasul Suleria

    2016-12-01

    Full Text Available Waste generated from the processing of marine organisms for food represents an underutilized resource that has the potential to provide bioactive molecules with pharmaceutical applications. Some of these molecules have known anti-thrombotic and anti-coagulant activities and are being investigated as alternatives to common anti-thrombotic drugs, like heparin and warfarin that have serious side effects. In the current study, extracts prepared from blacklip abalone (Haliotis rubra processing waste, using food grade enzymes papain and bromelain, were found to contain sulphated polysaccharide with anti-thrombotic activity. Extracts were found to be enriched with sulphated polysaccharides and assessed for anti-thrombotic activity in vitro through heparin cofactor-II (HCII-mediated inhibition of thrombin. More than 60% thrombin inhibition was observed in response to 100 μg/mL sulphated polysaccharides. Anti-thrombotic potential was further assessed as anti-coagulant activity in plasma and blood, using prothrombin time (PT, activated partial thromboplastin time (aPTT, and thromboelastography (TEG. All abalone extracts had significant activity compared with saline control. Anion exchange chromatography was used to separate extracts into fractions with enhanced anti-thrombotic activity, improving HCII-mediated thrombin inhibition, PT and aPTT almost 2-fold. Overall this study identifies an alternative source of anti-thrombotic molecules that can be easily processed offering alternatives to current anti-thrombotic agents like heparin.

  13. [Bilateral renal infarction after discontinuation of anticoagulant therapy].

    Science.gov (United States)

    Lavoignet, Charles-Éric; Le Borgne, Pierrick; Ugé, Sarah; Veneziano, Rinaldo; Brunhuber, Claudia; Kam, Claire; Bilbault, Pascal

    2016-07-01

    Acute renal infarction is an uncommon and often under diagnosed condition mostly because of misleading symptoms. Accurate data regarding clinical presentation, laboratory tests, diagnostic and treatment are lacking. Detection is often delayed or missed because of non-specific clinical presentation. The mechanisms of acute renal infarction are various, mainly embolic or thrombotic. Abdominal CT scan remains the most valuable exam to confirm the diagnosis. Therapeutic guidelines for the treatment of renal embolism have not been well established. The standard treatment strategy includes anticoagulation with or without thrombolysis. Despite the uncertainty regarding management, the renal outcome remains favorable. Some patients do develop some degree of renal insufficiency during the acute episode. We report here the case of a 73-year-old woman with bilateral acute renal infarction after discontinuation of anticoagulant therapy.

  14. Synchronous Ovarian and Endometrial Endometrioid Adenocarcinoma Presenting with Nonbacterial Thrombotic Endocarditis and Pulmonary Thromboembolism: Adenocarcinoma with Thrombotic Events

    Directory of Open Access Journals (Sweden)

    N. K. Erturk

    2015-01-01

    Full Text Available Introduction. Nonbacterial thrombotic endocarditis (NBTE is a rare manifestation of hypercoagulability in patients with malignant neoplasms. Case Report. A fifty-six-year-old woman presented to the emergency service; the clinical workup revealed deep vein thrombosis in right leg and bilateral massive PTE. As the abdominal sections on the spiral CT revealed a giant pelvic mass of ovarian origin, she was referred to our hospital’s gynecologic oncology department. She was scheduled for surgery under enoxaparin. She described numbness on one side of her face. Cranial imaging findings revealed acute ischemic cerebral lesions and transesophageal echocardiogram showed vegetation on the aortic cusp. Under anticoagulation treatment, she underwent hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy. After tumor resection, her neurological symptoms dissolved with aggressive anticoagulant treatment. Pathology result was synchronous endometrial and ovarian adenocarcinoma. Discussion. NBTE is a rare condition often associated with advanced malignancies. Peripheral embolism and venous thrombosis are complications that have been associated with NBTE due to hypercoagulable state. These disorders could be resistant to routine anticoagulant treatment. In case of a thrombotic complication due to ovarian malignancy, surgical resection of the primary tumor may increase the effect of anticoagulant treatment.

  15. Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies

    NARCIS (Netherlands)

    Licht, C.; Greenbaum, L.A.; Muus, P.; Babu, S.; Bedrosian, C.L.; Cohen, D.J.; Delmas, Y.; Douglas, K.; Furman, R.R.; Gaber, O.A.; Goodship, T.; Herthelius, M.; Hourmant, M.; Legendre, C.M.; Remuzzi, G.; Sheerin, N.; Trivelli, A.; Loirat, C.

    2015-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare, possibly life-threatening disease characterized by platelet activation, hemolysis and thrombotic microangiopathy (TMA) leading to renal and other end-organ damage. We originally conducted two phase 2 studies (26 weeks and 1 year) evaluating eculiz

  16. Decrease of thrombomodulin contributes to the procoagulant state of endothelium in hemolytic uremic syndrome.

    NARCIS (Netherlands)

    Fernandez, G.C.; Loo, D.M.W.M. te; Velden, T.J.A.M. van der; Heuvel, L.P.W.J. van den; Palermo, M.S.; Monnens, L.L.

    2003-01-01

    The typical form of hemolytic uremic syndrome (D+HUS) is a thrombotic microangiopathy that causes acute renal failure in children. The etiology of this disease is a toxin called Shiga-like toxin (Stx), present in certain strains of gram-negative bacteria. Vascular endothelial cell (EC) injury appear

  17. Trauma renal Renal trauma

    Directory of Open Access Journals (Sweden)

    Gerson Alves Pereira Júnior

    1999-02-01

    Full Text Available Apresentamos uma revisão sobre trauma renal, com ênfase na avaliação radiológica, particularmente com o uso da tomografia computadorizada, que tem se tornado o exame de eleição, ao invés da urografia excretora e arteriografia. O sucesso no tratamento conservador dos pacientes com trauma renal depende de um acurado estadiamento da extensão da lesão, classificado de acordo com a Organ Injury Scaling do Colégio Americano de Cirurgiões. O tratamento conservador não-operatório é seguro e consiste de observação contínua, repouso no leito, hidratação endovenosa adequada e antibioti- coterapia profilática, evitando-se uma exploração cirúrgica desnecessária e possível perda renal. As indicações para exploração cirúrgica imediata são abdome agudo, rápida queda do hematócrito ou lesões associadas determinadas na avaliação radiológica. Quando indicada, a exploração renal após controle vascular prévio é segura, permitindo cuidadosa inspeção do rim e sua reconstrução com sucesso, reduzindo a probabilidade de nefrectomia.We present a revision of the renal trauma with emphasis in the radiographic evaluation, particularly CT scan that it has largely replaced the excretory urogram and arteriogram in the diagnostic worh-up and management of the patient with renal trauma. The successful management of renal injuries depends upon the accurate assessment of their extent in agreement with Organ Injury Scaling classification. The conservative therapy managed by careful continuous observation, bed rest, appropriate fluid ressuscitation and prophylactic antibiotic coverage after radiographic staging for severely injured kidneys can yield favorable results and save patients from unnecessary exploration and possible renal loss. The indications for immediate exploratory laparotomy were acute abdomen, rapidly dropping hematocrit or associated injuries as determinated from radiologic evaluation. When indicated, renal exploration

  18. Recurrent Thrombotic Vasculopathy in a Former Cocaine User

    Directory of Open Access Journals (Sweden)

    Preeti Jadhav

    2015-01-01

    Full Text Available We report a case of a 35-year-old female who presented to the emergency room (ER complaining of a pruritic rash involving multiple areas of the body. She had a significant history of cocaine use in the past. She had first developed a similar rash in 2013 when she was diagnosed with cocaine-induced vasculitis. Her urine toxicology had been positive for cocaine in the past until July 2013. She was incarcerated and attended a drug rehabilitation program after which she quit cocaine use, which was consistent with negative urine toxicology on subsequent admissions. Further workup did not reveal any other, autoimmune or infectious, etiology of this clinical presentation. The patient underwent biopsy of the skin lesion that was consistent with thrombotic vasculopathy likely secondary to levamisole.

  19. Thrombotic manifestations in SAPHO syndrome. Review of the literature.

    Science.gov (United States)

    Carranco-Medina, Tatiana Elizabeth; Hidalgo-Calleja, Cristina; Calero-Paniagua, Ismael; Sánchez-González, María Dolores; Quesada-Moreno, Alba; Usategui-Martín, Ricardo; Pérez-Garrido, Laura; Gómez-Castro, Susana; Montilla-Morales, Carlos Alberto; Martínez-González, Olga; Del Pino-Montes, Javier

    2015-01-01

    SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted.

  20. Renal arteriography

    Science.gov (United States)

    ... Read More Acute arterial occlusion - kidney Acute kidney failure Aneurysm Atheroembolic renal disease Blood clots Renal cell carcinoma Renal venogram X-ray Review Date 1/5/2016 Updated by: Jason Levy, ...

  1. Venous and Arterial Thrombotic Events in Systemic Lupus Erythematosus.

    Science.gov (United States)

    Hinojosa-Azaola, Andrea; Romero-Diaz, Juanita; Vargas-Ruiz, Angel Gabriel; Nuñez-Alvarez, Carlos A; Cicero-Casarrubias, Alba; Ocampo-Torres, Mario C; Sanchez-Guerrero, Jorge

    2016-03-01

    The incidence of thrombosis in patients with systemic lupus erythematosus (SLE) is 25 to 50-fold higher than in the general population; we aimed to define the characteristics of venous thrombotic events (VTE) and arterial thrombotic events (ATE) to identify the patients at highest risk. The study included 219 patients with recent-onset SLE. At baseline, standardized medical history and laboratory tests were done. Followup visits occurred quarterly, and information about damage accrual, comorbidities, and cardiovascular risk factors was updated annually. Main outcome was development of TE after SLE diagnosis. Thirty-five patients (16%) developed TE (27 VTE, 8 ATE) during 5.21 years of followup; incidence rate 31/1000 patient-years. Most events (57%) developed within the first year of diagnosis, and 69% were not associated with lupus anticoagulant (LAC), determined with 1 method. VTE developed earlier than ATE (2.0 vs 57.5 mos, p = 0.02). In the multivariate analysis, variables preceding VTE included cutaneous vasculitis, nephrotic syndrome, dose of prednisone, and LAC in combination with anti-RNP/Sm antibodies (p < 0.03). Patients with ATE were older (median age 44 vs 29 yrs, p = 0.04), smokers, and had hypertension, diabetes mellitus, dyslipidemia, at least 2 traditional risk factors, nephrotic syndrome, chronic damage, and a higher cumulative dose of prednisone (p < 0.05). LAC in combination with anti-RNP/Sm antibodies was associated with VTE and improved the accuracy for predicting it. Our study suggests that in SLE, VTE and ATE have different risk factors. Understanding these differences is helpful for identifying patients at highest risk. The use of LAC plus anti-RNP/Sm for predicting VTE deserves further study.

  2. 肾脏病患者血小板减少症的诊治策略%Strategies of diagnosis and treatment for thrombocytopenia in patients with renal diseases

    Institute of Scientific and Technical Information of China (English)

    庄永泽

    2013-01-01

    Apart from accelerating hemostasis,promoting clotting,and maintaining the integrity of capillaries,thrombocytes possess functions of regulating the immune system including gathering leukocytes, capturing and containing pathogens,and killing infected cellular targets directly,etc.Thrombocytopenia may occur in both acute kidney injury (AKI)and chronic kidney disease (CKD)patients,but with differences in etiology and pathophysiology.Thrombocytopenia in AKI is mainly caused by infections,medicine,hemolytic-uremic syndrome (HUS),thrombotic thrombocytopenie purpura (TTP),and syndrome of hemolysis,elevated liver enzymes,and low platelets (HELLP).However,thrombocytopenia in CKD is mostly caused by autoimmune diseases, liver diseases, thrombotic microangiopathy (TMA ), idiopathic thrombocytopenic purpura (ITP),hyper-coagulation consumption,heparin-induced thrombocytopenia (HIT),drug allergies, uremia toxin,blood diseases,and disseminated intravascular coagulation (DIC ),etc.Therapy strategies include etiological treatment,pathogenesis treatment,plasmapheresis,platelet transfusions,promoting platelet formation,and symptomatic treatment.Additional attention should be paid to thrombocytopenia in patients with renal diseases,as it could be an essential clue to the cause of the renal disease,reflecting endothelial dysfunction, immune-mediated damage, thromboembolism, and/or adverse drug reactions. Progressive decrease of platelets may be an important indicator of poor prognosis in patients with renal diseases.%血小板除了止血、促进凝血、维持毛细血管完整性外,还具有调节免疫、募集白细胞、捕获和封存致病原、直接杀死受感染的细胞等功能。急性肾损伤和慢性肾脏病患者均可出现血小板减少症,但两者在病因及病理生理方面存在差异。前者病因主要是感染、药物、溶血尿毒综合征(HUS)、血栓性血小板减少性紫癜(TTP)、溶血、肝酶升高、血小板减少

  3. Anti-VEGF Cancer Therapy in Nephrology Practice

    Directory of Open Access Journals (Sweden)

    Hassan Izzedine

    2014-01-01

    Full Text Available Expanded clinical experience with the antivascular endothelial growth factor (VEGF agents has come with increasing recognition of their renal adverse effects. Although renal histology is rarely sought in antiangiogenic-treated cancer patients, kidney damage related to anti-VEGF is now established. Its manifestations include hypertension, proteinuria, and mainly glomerular thrombotic microangiopathy. Then, in nephrology practice, should we continue to perform kidney biopsy, and what should be done with the anti-VEGF agents in case of renal toxicity?

  4. Prevention of diabetic microangiopathy by prophylactic transplant of mobilized peripheral blood mononuclear cells

    Institute of Scientific and Technical Information of China (English)

    Bin ZHOU; Xiao-cang CAO; Zhi-hong FANG; Cui-lin ZHENG; Zhi-bo HAN; He REN; Man-chiu POON; Zhong-chao HAN

    2007-01-01

    Aim: To investigate whether the prophylactic local delivery of mobilized periph-eral blood mononuclear cells (M-PBMNC) could prevent peripheral microangio-pathy in diabetic nude mice. Methods: Diabetic nude mice were induced with intraperitoneal injections of streptozotocin. With the time course of diabetes, we detected the capillary and arteriole density of mice adductor muscles by immuno-histopathy. In situ apoptosis was detected by using TdT-mediated dUTP nick end labeling (TUNEL) methods. M-PBMNC were labeled and locally delivered to the adductor muscles. Mononuclear cells were also isolated and cultured in vitro for the detection and counting of endothelial progenitor cells(EPC). Results: Rarefication of capillaries and arterioles, enhanced apoptosis in adductor muscles,and reduced circulating EPC in diabetic nude mice. Prophylactic local delivery of M-PBMNC halted the progression of microvascular rarefaction in hind-limb skel-etal muscles by inhibiting apoptosis. We detected the survival, migration and incorporation of transplanted M-PBMNC into the murine vasculature in vivo. In addition, more EPC were available from M-PBMNC than non-mobilized cells.Conclusion: These results suggested that the prophylactic local delivery of M-PBMNC may represent a novel approach for the treatment of microvascular complications in diabetics.

  5. Hemorheological profile in primary and secondary Raynaud's phenomenon. Influence of microangiopathy.

    Science.gov (United States)

    Vayá, Amparo; Alis, Rafael; Romagnoli, Marco; Todolí, Jose; Calvo, Javier; Ricart, Jose M

    2014-01-01

    Raynaud's phenomenon (RP) is an episodic peripheral circulatory disorder characterized by local artery spams in subjects exposed to cold or emotional stress. It is not well-established whether RP patients show an altered rheological profile, mostly due to patient classification and clinical severity. We aimed to compare the hemorheological profile in patients with primary and secondary RP with a healthy control group. Eighteen primary RP, 22 secondary RP and 22 healthy controls, were included in the study. RP patients were also divided according to the presence of digital ulcers (7 with, 33 without). Biochemical and hemorheological variables were analyzed, including glucose, triglycerides, total-cholesterol, immunoglobulins, fibrinogen, plasma viscosity, erythrocyte aggregation, erythrocyte deformability and blood viscosity. Age was higher in secondary RP as compared with primary (p = 0.049), while glucose, triglycerides IgA, IgG and plasma viscosity were higher in secondary RP than in healthy subjects (p < 0.05). RP patients with digital ulcers presented higher IgA (p = 0.012), lower erythrocyte aggregation time (p = 0.008) and a trend for higher fibrinogen levels and plasma viscosity (p = 0.064, p = 0.069, respectively). The results of the present study indicate that secondary RP patients show a mild impairment of the rheological profile that aggravates with microangiopathy severity.

  6. Thrombotic and infectious complications of central venous catheters in patients with hematological malignancies.

    NARCIS (Netherlands)

    Boersma, R.S.; Jie, K.S.; Verbon, A.; Pampus, EC van; Schouten, H.C.

    2008-01-01

    Central venous catheters (CVCs) have considerably improved the management of patients with hematological malignancies, by facilitating chemotherapy, supportive therapy and blood sampling. Complications of insertion of CVCs include mechanical (arterial puncture, pneumothorax), thrombotic and infectio

  7. Identification of gene variants in NOS3, ET-1 and RAS that confer risk and protection against microangiopathy in type 2 diabetic obese subjects.

    Science.gov (United States)

    Manea, Simona-Adriana; Robciuc, Alexandra; Guja, Cristian; Heltianu, Constantina

    2011-04-15

    The study aim was to investigate NOS3 VNTR, NOS3 G894T, EDN1 C8002T, ACE I/D, AGT M235T and AGTR1 A1166C in nonobese and obese T2DM patients, and their interaction with the incidence of microangiopathy. T2DM subjects (n=250; 166 nonobese, and 84 obese) were genotyped for the gene variants by PCR/RFLP. The interaction of these polymorphisms with obesity and their contribution to microangiopathy were analyzed by multivariate regression analysis. A higher frequency of NOS3 4a allele was found in obese (P=0.027) vs. nonobese subjects. ACE D (P=0.009) and AGT 235T (P=0.026) alleles were associated with the reduced risk of diabetic nephropathy in nonobese and obese patients, respectively. In obese subjects, NOS3 4a (P=0.011) had a converse effect to NOS3 894T (P=0.043), and EDN1 8002T (P=0.035) on the prevalence of combined microangiopathy (neuropathy/retinopathy/nephropathy) vs. microangiopathy-negative subjects. The study indicates association of RAS variants with obesity and nephropathy, and an opposite effect of NOS3 VNTR and NOS3 G894T on the occurrence of combined microangiopathy. Copyright © 2011 Elsevier Inc. All rights reserved.

  8. Analysis of 1224 renal biopsy data%1224例肾活检病理资料分析

    Institute of Scientific and Technical Information of China (English)

    罗晶晶

    2013-01-01

    Objectives Explore our hospital for the past five years the epidemiologieal characteristics of 1224 cases of renal biopsy information and pathological type distribution characteristics.Methods Between January 2008 and December 2012 in the People's Hospital of the Xinjiang Uygur Autonomous Region(Urumqi,China),1224 attempts of percutaneous renal biopsy were performed in renal patients.In this report,the pathologic entities were analyzed retrospectively.Results The age at renal biopsy rang of 4 to 82 years in 1224 patients with renal diseases with a male 661 cases,female 563 cases,primary glomerular diseases (PGD) accounted for 81.70% of the total renal patients biopsied,secondary glomerular nephritis (SGN) 12.17 %,Tubulointerstitial diseases 5.07 % and the associated nephropathy metabolic accounted for 0.82%,renal transplant recipients 0.16%,and unclassified renal diseases 0.08 %.mesangial proliferative lesion (MsPL) was the most frequent patholigic type (30.30 %) of PGD,followed by IgAN (28.00%),membranous nephropathy(MN) (17.50 %),focal segmental glomerulosclerosis (FSGS,10.50 %).Proliferative sclerosing glomerulonerphritis (3.9%),membranoproliferative glomerulonephritis (3.10%),crescentic glomerulonephritis (2.60%),minimal change glomerulopathy(2.50%),endocapillary proliferative glomemlonephritis(1.6%),The most frequent type of SGN was lupusnephritis (LN) (31.54 %),Hypertensive renal damage(27.52%),nephritis of anaphylactoid purpura (13.42%),amyloidosis (11.4%),diabetic renal (7.38%),hepatitis B virus associated nephritis (2.68%),Thrombotic microangiopathy (2.01%).Conclusions In primary glomerular diseases,mesangial proliferative lesion (MsPL) is still the most frequent pathological type.followed by IgAN,and IgANshowed an increasing trend in the incidence.In addition,secondary renal disease gradually increased,In SGN,LN is the most frequent pathological type.Discussion by renal biopsy and clinical pathology,not only

  9. Reduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus.

    Science.gov (United States)

    Martin-Rodriguez, S; Reverter, J C; Tàssies, D; Espinosa, G; Heras, M; Pino, M; Escolar, G; Diaz-Ricart, M

    2015-10-01

    Severe deficiency of ADAMTS13 activity leads to von Willebrand factor (VWF) ultralarge multimers with high affinity for platelets, causing thrombotic thrombocytopenic purpura. Other pathological conditions with moderate ADAMTS13 activity exhibit a thrombotic risk. We examined the ADAMTS13 activity in systemic lupus erythematosus (SLE) and its value as a thrombotic biomarker. ADAMTS13 activity, VWF antigen and multimeric structure, and vascular cell adhesion molecule 1 (VCAM-1) were measured in plasma samples from 50 SLE patients and 50 healthy donors. Disease activity (systemic lupus erythematosus disease activity index; SLEDAI) and organ damage (systemic lupus international collaborating clinics) scores, thrombotic events, antiphospholipid syndrome (APS) and antiphospholipid antibodies (aPLs) were registered. SLE patients showed decreased ADAMTS13 activity and high VWF levels compared with controls (66 ± 27% vs. 101 ± 8%, P 60%, 60-40% and <40%), comparative analysis showed significant association between ADAMTS13 activity and SLEDAI (P < 0.05), presence of aPLs (P < 0.001), APS (P < 0.01) and thrombotic events (P < 0.01). Reduced ADAMTS13 activity together with increased VWF levels were especially notable in patients with active disease and with aPLs. ADAMTS13 activity, in combination with other laboratory parameters, could constitute a potential prognostic biomarker of thrombotic risk in SLE. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  10. Defining the Thrombotic Risk in Patients with Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Fabrizio Vianello

    2011-01-01

    Full Text Available Polycythemia vera (PV and essential thrombocythemia (ET are two Philadelphia-negative myeloproliferative neoplasms (MPN associated with an acquired mutation in the JAK2 tyrosine kinase gene. There is a rare incidence of progression to myelofibrosis and myeloid metaplasia in both disorders, which may or may not precede transformation to acute myeloid leukemia, but thrombosis is the main cause of morbidity and mortality. The pathophysiology of thrombosis in patients with MPN is complex. Traditionally, abnormalities of platelet number and function have been claimed as the main players, but increased dynamic interactions between platelets, leukocytes, and the endothelium do probably represent a fundamental interplay in generating a thrombophilic state. In addition, endothelial dysfunction, a well-known risk factor for vascular disease, may play a role in the thrombotic risk of patients with PV and ET. The identification of plasma markers translating the hemostatic imbalance in patients with PV and ET would be extremely helpful in order to define the subgroup of patients with a significant clinical risk of thrombosis.

  11. Diabetic microangiopathy in capillaroscopic examination of juveniles with diabetes type 1

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    Grażyna Kaminska-Winciorek

    2012-01-01

    Full Text Available Introduction:The aim of this work was a quantitative and qualitative assessment of a selected part of the microcirculation in children with diabetes type 1 using videocapillaroscopy technique.Material/Methods:The authors tested a group consisting of 145 children (70 boys, 75 girls diagnosed and treated for diabetes type 1 in the Diabetic Clinic of GCZD in Katowice for at least one year. The study included history, clinical examination (including dermatological examination and videocapillaroscopy. Capillaroscopy, a non-invasive, painless and easily repeatable test, was performed using videocapillaroscopy with digital storage of the obtained images. All nailfolds were examined in all children using videocapillaroscopy, and the obtained images were assessed quantitatively and qualitatively for changes in capillary loops in the tested children according to the defined diagnostic procedure.Results:The analysis of capillaroscopic images described selected quantitative and qualitative characteristics. The conducted analysis showed an increase in the number of capillaries and their elongation, the presence of megacapillaries and Raynaud loops, which were accompanied by an intensive red background, indicating possible neoangiogenesis. The increase in the number of capillaries, disturbances in distribution of capillaries and the presence of abnormal capillaries were correlated with the longer duration of diabetes. Raynaud loops were more frequently found in the cases of increased mean values of HbA1c. Higher values of HbA1c influenced the capillaroscopic images, mainly the number of vessels, including Raynaud loops.Conclusions:Videocapillaroscopy technique could be a useful tool to detect the early changes of microangiopathy in children with diabetes type 1.

  12. The impact of serum lipids on risk for microangiopathy in patients with type 2 diabetes mellitus

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    Toth Peter P

    2012-09-01

    Full Text Available Abstract Background Few large-scale, real-world studies have assessed the relative associations of lipid fractions with diabetic microvascular events. The main objective of this study was to evaluate the association of the lipid profile components, high density lipoprotein cholesterol (HDL-C, low density lipoprotein cholesterol (LDL-C, triglycerides (TG, and non-high density lipoprotein cholesterol (non-HDL-C with microvascular complications (MVCs in type 2 diabetes mellitus (T2DM patients. Methods This observational cohort study queried the HealthCore Integrated Research Database (HIRDSM for newly-diagnosed (Index Date 18-64-year-old patients with diabetes mellitus between 01/01/2005-06/30/2010. Inclusion required ≥12 months pre-index continuous health plan eligibility and ≥1 pre-index lipid profile result. Patients with polycystic ovary syndrome and prior MVCs were excluded. Incident complications were defined as the earliest occurrence of diabetic retinopathy, peripheral neuropathy, and/or nephropathy post-index. Cox proportional models and Kaplan-Meier (KM curves were used to evaluate associations among variables. Results Of the patients (N = 72,267, 50.05 % achieved HDL-C, 64.28 % LDL-C, 59.82 % TG, and 56.79 % non-HDL-C American Diabetes Association goals at baseline. During follow-up (mean, 21.74 months, there were 5.21 microvascular events per 1,000 patient-months. A 1-mg/dL increase in HDL-C was associated with 1 % decrease in any MVC risk (P P P P P P  Conclusion This study demonstrates significant independent associations among lipid fractions and risk for microangiopathy. These findings suggest that attaining established ADA goals for HDL-C, TG, and non-HDL-C may reduce risk for microvascular events among patients with diabetes.

  13. Renal Osteodystrophy

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    Aynur Metin Terzibaşoğlu

    2004-12-01

    Full Text Available Chronic renal insufficiency is a functional definition which is characterized by irreversible and progressive decreasing in renal functions. This impairment is in collaboration with glomeruler filtration rate and serum creatinine levels. Besides this, different grades of bone metabolism disorders develop in chronic renal insufficiency. Pathologic changes in bone tissue due to loss of renal paranchyme is interrelated with calcium, phosphorus vitamine-D and parathyroid hormone. Clinically we can see high turnover bone disease, low turnover bone disease, osteomalacia, osteosclerosis and osteoporosis in renal osteodystropy. In this article we aimed to review pathology of bone metabolism disorders due to chronic renal insufficiency, clinic aspects and treatment approaches briefly.

  14. Pulmonary thromboembolism associated with renal insufficiency due to renal artery thrombosis

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    Vučičević-Trobok Jadranka

    2002-01-01

    Full Text Available Pulmonary thromboembolism is a clinical and pathophysiological condition caused by occlusion of pulmonary arteries by thrombotic embolus. Deep venous thrombosis needn't necessarily be evident. Acute renal insufficiency is a syndrome manifested by rapid decrease or even complete urinary retention which may be due to obturation of renal arteries by a thrombus. The condition is clinically manifested by hematuria, oliguria, anuria and death due to uremia. Case Report The patient had a history of disease and a chest X-ray finding both irrelevant for establishing the working diagnosis. Blood gas analysis presented prominent hypoxemia with hyperventilation and ECG finding exhibited right heart overload, pointing to pulmonary thromboembolism. On the third day of treatment with heparin, the patient developed hematuria, oliguria and excessive elevation of nitrogen in blood as a sign of acute renal insufficiency, leading to fatal outcome. Autopsy revealed deep venous thrombosis of the left femoral vein, with massive pulmonary thromboembolism and thrombosis of both femoral arteries, inducing acute renal failure. Discussion Although the patient had a typical radiologic presentation of pulmonary thromboembolism, treatment with heparin was initiated on the basis of his blood gas analysis and ECG findings. Deep venous thrombosis was not clinically evident and acute renal failure could not be explained during patient's lifetime. Conclusion This is a rare case of acute renal failure due to a rare occurrence of bilateral acute thrombosis of renal arteries.

  15. Impaired autoregulation of blood flow in subcutaneous tissue of long-term type 1 (insulin-dependent) diabetic patients with microangiopathy

    DEFF Research Database (Denmark)

    Kastrup, J; Nørgaard, T; Parving, H H

    1985-01-01

    Autoregulation of blood flow in subcutaneous tissue was studied at the level of the lateral malleolus in eight long-term Type 1 (insulin-dependent) diabetic patients with clinical microangiopathy, eight short-term Type 1 diabetic patients without clinical microangiopathy and seven healthy control...... by activation of the leg muscle vein pump (heel raising). Mean arterial blood pressure was thus varied between 60 and 160 mmHg. In normal and short-term diabetic subjects blood flow remained within 10% of control values during the changes in arterial blood pressure. In six of the eight Type 1 diabetic patients...

  16. A mouse model to study thrombotic complications of thalassemia.

    Science.gov (United States)

    Kalish, Yosef; Malyutin, Zeev; Shai, Ela; Dana, Mutaz; Avraham, Limor; Jahshan, Nivin; Rachmilewitz, Eliezer; Fibach, Eitan; Varon, David

    2015-03-01

    Patients with β-thalassemia major and mainly intermedia have an increased risk for developing venous and arterial thrombosis which may be related to circulating pathological red blood cells (RBC) and continuous platelet activation. In the present study we used a modified thalassemic mice model in conjunction with a "real-time" carotid thrombus formation procedure to investigate thrombotic complications of thalassemia. Heterozygous Th3/+ mice, which lack one copy of their β-major and β-minor globin genes, exhibit anomalies in RBC size and shape, chronic anemia and splenomegaly which recapitulate the phenotype of human β-thalassemia intermedia. Flow cytometry measurements showed higher reactive oxygen species generation, indicating oxidative stress, in platelets and RBC of the thalassemic mice compared with wild type mice concomitant with an increase in reduced glutathione content which may represent a compensatory response to oxidative stress, and exposed phosphatidylserine which indicates platelet activation. To elucidate the effect of thalassemia on the development of arterial thrombosis, we studied photochemical-induced real-time thrombus formation in the carotid artery of these mice. The results indicated a significantly shorter "time to occlusion" in the thalassemic mice compared to wild type mice, which was prolonged following in vivo aspirin treatment. We suggest that this mouse model may contribute to our understanding of platelet activation and the hypercoagulable state in thalassemia and lay foundations to screening of anti-platelet drugs as well as anti-oxidants as possible therapeutics for prevention of thrombosis in thalassemia patients. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. An improbable and unusual case of thrombotic thrombocytopenia purpura

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    Jaymon Patel

    2016-09-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a life-threatening medical emergency which may be difficult to recognize given the wide spectrum in which it presents. A delay in treatment may be catastrophic as untreated cases of TTP have a mortality rate exceeding 90%. Given the high fatality rate of untreated TTP and its range of presenting symptoms, we present our unusual case of TTP in a post-splenectomy patient with early treatment and positive outcome. This case describes a 54-year-old female who presented with hematuria and gingival bleeding, followed by the development of a bilateral lower extremity petechial rash. Her past medical history was significant for multiple episodes of TTP, the last of which resulted in a splenectomy and a 20-year history of remission thereafter. On exam, she was alert, well appearing, and neurologically intact. Her only significant finding was a bilateral lower extremity petechial rash. Laboratory studies revealed mild anemia and thrombocytopenia, an elevated lactate dehydrogenase, and a decreased haptoglobin. Peripheral smear showed poikilocytosis, helmet cells, and schistocytes. Corticosteroid therapy was promptly initiated, her platelets were monitored closely, and she underwent urgent therapeutic plasma exchange. Due to the risk of significant morbidity and mortality that may result from delayed treatment of TTP as well as the significant variations of presentation, TTP requires a consistently high index of suspicion. Our patient suffered multiple relapses of TTP within a 30-year span, underwent splenectomy in early adulthood, and presented with atypical symptoms during her most recent relapse illustrating how persistent TTP can be as well as how unusually it may present. Providers should be aware of the vast spectrum of presentation and remember that TTP may recur following splenectomy despite prolonged remission.

  18. Refractor y thrombotic thrombocytopenic pur pura following acute pancreatitis

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    Ebisa Bekele

    2016-09-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13 activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6% after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  19. Case report of atypical hemolytic uremic syndrome with retinal arterial and venous occlusion treated with eculizumab

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    Greenwood GT

    2015-10-01

    Full Text Available Gregory T Greenwood Nephrology Associates, PLLC, Winston-Salem, NC, USA Abstract: Atypical hemolytic uremic syndrome (aHUS is a rare disease caused by chronic, uncontrolled activation of the alternative complement pathway, leading to thrombotic microangiopathy. Renal impairment and progression to end-stage renal disease are common in untreated patients with aHUS, and extrarenal manifestations are being increasingly characterized in the literature. Ocular involvement remains rare in aHUS. This report describes a patient with aHUS with bilateral central retinal artery and vein occlusion, vitreous hemorrhage, and blindness in addition to renal impairment. The patient’s hematologic and renal parameters and ocular manifestation improved following initiation of eculizumab therapy. Keywords: acute kidney injury, complement, dialysis, plasma exchange, thrombotic microangiopathy

  20. Cerebro-retinal microangiopathy with calcifications and cysts due to recessive mutations in the CTC1 gene.

    Science.gov (United States)

    Bisserbe, A; Tertian, G; Buffet, C; Turhan, A; Lambotte, O; Nasser, G; Alvin, P; Tardieu, M; Riant, F; Bergametti, F; Tournier-Lasserve, E; Denier, C

    2015-05-01

    Cerebro-retinal microangiopathy with calcifications and cysts (CRMCC) or Coats plus syndrome is a pleiotropic disorder affecting the eyes, brain, bone and gastrointestinal tract. Its primary pathogenesis involves small vessel obliterative microangiopathy. Recently, autosomal recessively inherited mutations in CTC1 have been reported in CRMCC patients. We herein report an adolescent referred to our hospital following new seizures in a context of an undefined multisystem disorder. Cerebral imaging disclosed asymmetrical leukopathy, intracranial calcifications and cysts. In addition, he presented other typical CRMCC features i.e. a history of intrauterine growth retardation, skeletal demineralization and osteopenia, bilateral exudative vitreo-retinopathy reminiscent of Coats disease, recurrent gastrointestinal hemorrhages secondary to watermelon stomach and variceal bleeding of the esophagus due to idiopathic portal hypertension and telangiectatic and angiodysplasic changes in the small intestine and colon, and anemia due to recurrent bleeding and bone marrow abnormalities. The patient was diagnosed with Coats plus syndrome. CTC1 gene screening confirmed the diagnosis with the identification of heterozygous deleterious mutations. CRMCC due to CTC1 mutations has a broad clinical expressivity. Our case report illustrates the main possible associated phenotypes and their complications, demonstrating the need for a careful etiological search in order to initiate appropriate therapeutic and preventive measures.

  1. Thrombophilia in 67 Patients With Thrombotic Events After Starting Testosterone Therapy.

    Science.gov (United States)

    Glueck, Charles J; Prince, Marloe; Patel, Niravkumar; Patel, Jaykumar; Shah, Parth; Mehta, Nishi; Wang, Ping

    2016-09-01

    We compared thrombophilia in 67 cases (59 men and 8 women) with thrombotic events after starting testosterone therapy (TT) versus 111 patient controls having unprovoked venous thrombotic events without TT. In the 67 patients, thrombosis (47 deep venous thrombosis-pulmonary embolism, 16 osteonecrosis, and 4 ocular thrombosis) occurred 6 months (median) after starting TT. Cases differed from controls for factor V Leiden heterozygosity (16 of the 67 [24%] vs 13 [12%] of the 111, P = .038) and for lupus anticoagulant (9 [14%] of the 64 vs 4 [4%] of the 106, P = .019). After a first thrombotic event and continuing TT, 11 cases had a second thrombotic event, despite adequate anticoagulation, 6 of whom, still anticoagulated, had a third thrombosis. Screening for thrombophilia before starting TT should identify men and women at high risk for thrombotic events with an adverse risk-benefit ratio for TT. When TT is given to patients with familial and acquired thrombophilia, thrombosis may occur and recur in thrombophilic men despite anticoagulation.

  2. About risk factors in the development of thrombotic complications in patients with chronic myeloprolipherative diseases

    Directory of Open Access Journals (Sweden)

    Nikolayenko-Kamyshova T.P.

    2013-03-01

    Full Text Available Steady increasing number of patients with the diseases com¬plicated by thromboses and embolisms requires intensified study. Practically in all oncologic and oncogematologic patients readiness for the development of thrombotic complications is formed even before changes in hemo¬stasiogramm. In the hematologic clinic the problem of thrombotic compli¬cations especially acute in patients with chronic myeloprolipherative diseases (CMD: the manifestation of disease at the age of 50-70 years, its benign prolonged course with factors, which burden vascularly - thrombocytic changes – hypertonic disease, ischemic heart disease, metabolic syndrome - they aggravate the forecast of disease. The purpose of the work was to estimate influence of the risk factors of thrombotic complications in patients with CMD. Possible risk factors in the development of thrombotic complication in 110 patients with CMD were investigated. In 38 of them vascular complications, occurred which manifested in the age group of 50 years and over. During the expressed plethora and or progression of myelopro¬lipherative syndrome in combination with arterial hypertension, coronary heart disease, atherothrombosis of lower extremities vessels as well as changed laboratory data (significant increase of Hb, RBC,WBC,PLT; high level of cholesterol, LDH, uric acid in connection with high level of throm¬botic aggregation greatly enhance the risk of thrombotic complications. Taking into account complex of these changes and administration of the corresponding complex therapy it is possible to warn life threatening states.

  3. Renal perfusion scintiscan

    Science.gov (United States)

    Renal perfusion scintigraphy; Radionuclide renal perfusion scan; Perfusion scintiscan - renal; Scintiscan - renal perfusion ... supply the kidneys. This is a condition called renal artery stenosis. Significant renal artery stenosis may be ...

  4. Takotsubo cardiomyopathy and thrombotic thrombocytopenic purpura preceding a lupus diagnosis: a case report.

    Science.gov (United States)

    Georgiades, F; Demosthenous, S; Braimi, M; Tsitskari, T; Psarelis, S

    2015-11-01

    Takotsubo cardiomyopathy, a rare stress-related cardiomyopathy, has been observed in a few cases secondary to systemic lupus erythematosus (SLE). Herein, we report an unusual case where a postmenopausal woman presented initially with Takotsubo syndrome, later developed thrombotic thrombocytopenic purpura and cerebrovascular events, initially without clinical or laboratory features of SLE. During the course of her illness, she was found to satisfy four of the Systemic Lupus International Collaborating Clinics classification criteria for a SLE diagnosis. This unique presentation of our patient, initially with Takotsubo cardiomyopathy, the development of thrombotic thrombocytopenic purpura and cerebrovascular events preceding the diagnosis of SLE illustrates the importance of clinical observation and follow-up.

  5. Microvascular abnormalities in capillaroscopy correlate with higher serum IL-18 and sE-selectin levels in patients with type 1 diabetes complicated by microangiopathy

    Directory of Open Access Journals (Sweden)

    Maria Górska

    2011-04-01

    Full Text Available Microvascular abnormalities are one of the most important causes of persistent diabetic complications. The aim of this study was to compare microvascular changes examined by nailfold capillaroscopy with serum concentrations of soluble E-selectin (sE-selectin and IL-18 in type 1 diabetic patients with and without microangiopathy. Serum levels of sE-selectin and IL-18 were determined by an enzyme-linked immunosorbent assay in 106 patients with type 1 diabetes and in 40 healthy controls. All diabetic patients were evaluated by extensive clinical, laboratory and capillaroscopic studies. Morphological changes were observed by nailfold capillaroscopy in 86 out of 106 (81% diabetic patients. Severe capillaroscopic changes were seen in 32 out of 54 (59% patients with microangiopathy, but in only seven out of 52 (13% patients without microangiopathy. Higher serum levels of sE-selectin (p < 0.001 and IL-18 (p < 0.05 were demonstrated in diabetic patients compared to controls. Significant differences of sE-selectin (p < 0.001 and IL-18 (p < 0.01 serum concentrations were observed between diabetic patients with microangiopathy and controls. Moreover, comparison between patients with and without microangiopathic complications showed a significantly higher capillaroscopic score and sE-selectin serum concentration in the group with microangiopathy (p < 0.001. Furthermore, diabetic patients with severe microvascular changes in capillaroscopy showed significantly higher IL-18 (p < 0.001 and sE-selectin (p < 0.05 serum levels than subgroups without changes or with mild abnormalities. Our findings suggest that abnormalities in nailfold capillaroscopy may reflect the extent of microvascular involvement and are associated with higher sE-selectin and IL-18 serum levels, as well as with microangiopathic complications in diabetic patients. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 1, pp. 104–110

  6. RENAL CRYOABLATION

    Directory of Open Access Journals (Sweden)

    A. V. Govorov

    2012-01-01

    Full Text Available Renal cryoablation is an alternative minimally-invasive method of treatment for localized renal cell carcinoma. The main advantages of this methodology include visualization of the tumor and the forming of "ice ball" in real time, fewer complications compared with other methods of treatment of renal cell carcinoma, as well as the possibility of conducting cryotherapy in patients with concomitant pathology. Compared with other ablative technologies cryoablation has a low rate of repeat sessions and good intermediate oncological results. The studies of long-term oncological and functional results of renal cryoablation are presently under way.

  7. Renal angiomyolipoma

    DEFF Research Database (Denmark)

    Holm-Nielsen, P; Sørensen, Flemming Brandt

    1988-01-01

    lesion. Three cases of renal angiomyolipoma, 2 of which underwent perfusion-fixation, were studied by electron microscopy to clarify the cellular composition of this lesion. In the smooth muscle cells abundant accumulation of glycogen was found, whereas the lipocytes disclosed normal ultrastructural......-specific vesicular structures. These findings suggest a secondary vascular damage, i.e. the thickened vessels may not be a primary, integral part of renal angiomyolipoma. Evidence of a common precursor cell of renal angiomyolipoma was not disclosed. It is concluded that renal angiomyolipoma is a hamartoma composed...

  8. O-(beta-hydroxyethyl)-rutosides systemic and local treatment in chronic venous disease and microangiopathy: an independent prospective comparative study.

    Science.gov (United States)

    Belcaro, Gianni; Rosaria Cesarone, Maria; Ledda, Andrea; Cacchio, Marisa; Ruffini, Irma; Ricci, Andrea; Ippolito, Edmondo; Di Renzo, Andrea; Dugall, Mark; Corsi, Marcello; Marino Santarelli, Anna Rita; Grossi, Maria Giovanna

    2008-01-01

    O-(beta-hydroxyethyl)-rutosides (HR) is used to treat chronic venous disease and signs and symptoms of chronic venous insufficiency (CVI), varicose veins, and deep venous disease. This independent prospective controlled trial (a registry study) evaluates how the efficacy of HR at the local level (perimalleolar region) can be increased by the administration of a topical HR gel. The study is based on evaluation of microcirculatory variables in patients with severe CVI (ambulatory venous pressure, > 56 mm Hg) and venous microangiopathy. Patients are treated using 1 of the following 3 regimens: oral treatment with 1 g sachets of HR (2 g/d total) plus topical HR 2% gel applied 3 times daily at the internal perimalleolar region; oral treatment only (same dosage), or light elastic compression stockings. Laser Doppler skin flux at rest, skin flux at the perimalleolar region, and transcutaneous PO2 and PCO2 are measured at baseline and at the end of the treatment period. A comparable group of healthy individuals without treatment is observed for 8 weeks. In the treatment groups, flux is increased, PO2 is decreased, and PCO2 is increased compared with normal skin. At 4 and 8 weeks, the improvement in skin flux (which is decreased by all measurements), the increase in PO2, and the decrease in PCO2 (indicating microcirculatory improvement) are statistically significantly greater in the combined oral plus topical treatment group (P < .05). No adverse effects, tolerability problems, or compliance issues are noted. These results indicate an important role of HR in the treatment and control of CVI and venous microangiopathy.

  9. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders.

    NARCIS (Netherlands)

    Besbas, N.; Karpman, D.; Landau, D.; Loirat, C.; Proesmans, W.; Remuzzi, G.; Rizzoni, G.; Taylor, C.M.; Kar, N.C.A.J. van de; Zimmerhackl, L.B.

    2006-01-01

    The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increase

  10. Low level of residual thrombotic obstruction following 6 months of anticoagulant treatment for acute pulmonary embolism

    NARCIS (Netherlands)

    Den Exter, P.L.; Van Es, J.; Kroft, L.J.M.; Erkens, P.G.M.; Douma, Renee; Jonkers, G.; Ten Cate, H.; Beenen, L.; Kamphuisen, P.W.; Huisman, M.V.

    2013-01-01

    Background: In patients with acute pulmonary embolism (PE), systematic assessment of residual thrombotic obstruction after long-term anticoagulation has been understudied. This information may be of clinical importance for diagnostic baseline imaging, in case of clinically suspected recurrent PE or

  11. Doppler sonography of extracranial and intracranial vessels in patients with thrombotic stroke

    Directory of Open Access Journals (Sweden)

    Farhad Iranmanesh

    2006-12-01

    Full Text Available INTRODUCTION: The results of intracranial and extracranial vessel color Doppler sonography, which is now an inseparable part of patient evaluation, vary in different studies. The objective of this study was to evaluate the pattern of vascular involvement in thrombotic stroke and its relationship with risk factors of stroke. METHODS: One-hundred patients (45 males and 55 females with thrombotic stroke underwent transcranial sonography and color Doppler sonography of extracranial vessels. The pattern of vascular involvement was analyzed. The risk factors of stroke were also evaluated. RESULTS: Forty-seven percent of the studied individuals had some variations in their color Doppler sonography of extracranial vessels, 27% had changes in the intracranial vessels and 26% showed changes in both. The most frequently involved vessels among the intracranial and extracranial vessels were the middle cerebral artery and the internal carotid artery, respectively. The pattern of vascular involvement was unrelated to hypertension, cigarette smoking, diabetes mellitus, dyslipidemia or history of ischemic heart disease. Extracranial involvement in patients with positive history of MI was more prevalent than in those without such history. CONCLUSIONS: Extracranial vessel involvement in thrombotic stroke was found to be more prevalent than intracranial vessel involvement in the city of Rafsanjan; however, intracranial vessel involvement was more prevalent than in western countries. KEY WORDS: Thrombotic stroke, color Doppler sonography, intracranial vessels, extracranial, cerebrovascular risk factors

  12. Treatment of plasmapheresis refractory thrombotic thrombocytopenic purpura with double-filtration membrane plasmapheresis.

    Science.gov (United States)

    Karakus, Volkan; Deveci, Burak; Kurtoğlu, Erdal

    2013-06-01

    Thrombotic thrombocytic purpura (TTP) is a life-threatening disorder. Without plasma exchange treatment (PET) the mortality rate is quite high. Double-filtration plasmapheresis is an alternative opportunity for TTP patients refractory to PET. Here we report our experience in a refractory TTP patient who was successfully treated by means of double-filtration plasmapheresis therapy.

  13. Low level of residual thrombotic obstruction following 6 months of anticoagulant treatment for acute pulmonary embolism

    NARCIS (Netherlands)

    Den Exter, P.L.; Van Es, J.; Kroft, L.J.M.; Erkens, P.G.M.; Douma, Renee; Jonkers, G.; Ten Cate, H.; Beenen, L.; Kamphuisen, P.W.; Huisman, M.V.

    Background: In patients with acute pulmonary embolism (PE), systematic assessment of residual thrombotic obstruction after long-term anticoagulation has been understudied. This information may be of clinical importance for diagnostic baseline imaging, in case of clinically suspected recurrent PE or

  14. Risk factors for post-thrombotic syndrome in patients with a first deep venous thrombos

    NARCIS (Netherlands)

    Tick, L.W.; Kramer, M.H.H.; Rosendaal, F.R.; Faber, W.R.; Doggen, C.J.M.

    2008-01-01

    Background: Post-thrombotic syndrome (PTS) is a chronic complication of deep venous thrombosis (DVT). Objectives: To determine the risk of PTS after DVT and to assess risk factors for PTS. Methods: Patients were recruited from the Multiple Environmental and Genetic Assessment (MEGA) study of risk fa

  15. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders.

    NARCIS (Netherlands)

    Besbas, N.; Karpman, D.; Landau, D.; Loirat, C.; Proesmans, W.; Remuzzi, G.; Rizzoni, G.; Taylor, C.M.; Kar, N.C.A.J. van de; Zimmerhackl, L.B.

    2006-01-01

    The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The

  16. [Embolic stroke by thrombotic non bacterial endocarditis in an Antiphospholipid Syndrome patient].

    Science.gov (United States)

    Graña, D; Ponce, C; Goñi, M; Danza, A

    2016-01-01

    The antiphospholipid syndrome (APS) is an acquired thrombophilia, considered a systemic autoimmune disorder. We report a patient with APS who presented multiple cerebral infarcts (stroke) as a complication of a thrombotic non bacterial endocarditis. We review the literature focused on the physiological mechanism that produce this disease and its complications. Clinical features and their prognostic value and the different therapeutic options were also studied.

  17. Renal cancer

    NARCIS (Netherlands)

    Corgna, Enrichetta; Betti, Maura; Gatta, Gemma; Roila, Fausto; De Mulder, Pieter H. M.

    2007-01-01

    In Europe, renal cancer (that is neoplasia of the kidney, renal pelvis or ureter (ICD-9 189 and ICD-10 C64-C66)) ranks as the seventh most common malignancy in men amongst whom there are 29,600 new cases each year (3.5% of all cancers). Tobacco, obesity and a diet poor in vegetables are all acknowle

  18. Renal fallure

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    920705 Endothelin and acute renal failure:study on their relationship and possiblemechanisms. LIN Shanyan(林善锬), et al.Renal Res Lab, Huashan Hosp, Shanghai MedUniv, Shanghai, 200040. Natl Med J China 1992;72(4): 201-205. In order to investigate the role of endothelin

  19. Renal cancer.

    NARCIS (Netherlands)

    Corgna, E.; Betti, M.; Gatta, G.; Roila, F.; Mulder, P.H.M. de

    2007-01-01

    In Europe, renal cancer (that is neoplasia of the kidney, renal pelvis or ureter (ICD-9 189 and ICD-10 C64-C66)) ranks as the seventh most common malignancy in men amongst whom there are 29,600 new cases each year (3.5% of all cancers). Tobacco, obesity and a diet poor in vegetables are all

  20. Renal cancer

    NARCIS (Netherlands)

    Corgna, Enrichetta; Betti, Maura; Gatta, Gemma; Roila, Fausto; De Mulder, Pieter H. M.

    2007-01-01

    In Europe, renal cancer (that is neoplasia of the kidney, renal pelvis or ureter (ICD-9 189 and ICD-10 C64-C66)) ranks as the seventh most common malignancy in men amongst whom there are 29,600 new cases each year (3.5% of all cancers). Tobacco, obesity and a diet poor in vegetables are all

  1. Was the thrombotic risk of rofecoxib predictable from the French Pharmacovigilance Database before 30 September 2004?

    Science.gov (United States)

    Sommet, A; Grolleau, S; Bagheri, H; Lapeyre-Mestre, M; Montastruc, J L

    2008-08-01

    Rofecoxib was withdrawn from the market on 30 September 2004 following the results of a randomized controlled trial. Following this sudden decision, several controversies occurred in the literature to determine whether this adverse drug reaction (ADR) could have been detected earlier. The aim of this study was to investigate whether this kind of signal could have been seen using the French Pharmacovigilance Database before this date of rofecoxib withdrawal. Using cases registered in the French Pharmacovigilance Database from May 2000 to December 2006, we applied the case-noncase method to "serious" thrombotic ADRs reported with oral formulations of rofecoxib or celecoxib in patients older than 15 years. Cases were all notifications of thrombotic ADRs [World Health Organization Adverse Reaction Terminology (WHO-ART) codes 1300] occurred under coxib (rofecoxib, celecoxib) and noncases all other reports registered in the database (whatever the drug). We calculated a cumulative odds ratio (OR) from 20 May 2000 to 31 December 2006, with a special interest for the period before the 30 September 2004. Among the 50,087 "serious" ADRs registered in the database during this period, 1,127 were thrombotic ones. Rofecoxib exposure was significantly associated with high values of odds ratio (OR) [4.2 (95% CI 1.97-8.61)] for thrombotic ADRs as early as the end of 2001. The values of ADR reporting ORs remained high (3.0-3.5) until 2006. For celecoxib, a significant trend occurred only from September 2004. Despite the compulsory limits of the case/noncase methodology, this study found an association between rofecoxib exposure and the occurrence of "serious" thrombotic ADRs as early as the end of the first year of rofecoxib marketing in France. The association between celecoxib and the occurrence of such ADRs appears less clear. Our work also shows the potential use of careful analysis of pharmacovigilance databases (investigating, for example, cumulative values of risk) in the

  2. Renal teratogens.

    Science.gov (United States)

    Morgan, Thomas M; Jones, Deborah P; Cooper, William O

    2014-09-01

    In utero exposure to certain drugs early in pregnancy may adversely affect nephrogenesis. Exposure to drugs later in pregnancy may affect the renin-angiotensin system, which could have an impact on fetal or neonatal renal function. Reduction in nephron number and renal function could have adverse consequences for the child several years later. Data are limited on the information needed to guide decisions for patients and providers regarding the use of certain drugs in pregnancy. The study of drug nephroteratogenicity has not been systematized, a large, standardized, global approach is needed to evaluate the renal risks of in utero drug exposures.

  3. Sarcoidose renal

    Directory of Open Access Journals (Sweden)

    AQUINO MARIA ENEDINA CLAUDINO DE

    2001-01-01

    Full Text Available Em uma mulher de 62 anos, branca, em avaliação pré-operatória de facectomia, foram detectadas alterações urinárias, tendo sido firmados os diagnósticos de calculose renal esquerda e exclusão renal homolateral. No pré-operatório da nefrectomia foram evidenciados processo pulmonar intersticial bilateral e adenopatia torácica, cuja investigação foi adiada para após a cirurgia. No rim retirado foram detectados granulomas epitelióides não necrotizantes, o mesmo ocorrendo posteriormente em biópsia transbrônquica. A paciente foi tratada com metilprednisolona, com discreta melhora pulmonar, o que não ocorreu com a função renal. O diagnóstico final foi de sarcoidose com envolvimento pulmonar, ganglionar torácico e renal.

  4. Renal failure

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930150 Epidermal growth factor and its recep-tor in the renal tissue of patients with acute re-nal failure and normal persons.LIU Zhihong(刘志红),et al.Jinling Hosp,Nanjing,210002.Natl Med J China 1992;72(10):593-595.Epidermal growth factor(EGF)and its receptor(EGF-R)were identified by immunohis-tochemical method(4 layer PAP)in the renaltissue specimens obtained from 11 normal kid-neys and 17 cases of acute renal failure(ARF).The quantitative EGF and EGF-R in the tissuewere expressed as positive tubules per mm~2.The amount of EGF and EGF-R in renal tissue

  5. Isolated renal vein thrombosis associated with MTHFR-1298 and PAI-1 4G gene mutations.

    Science.gov (United States)

    Cinemre, Hakan; Bilir, Cemil; Akdemir, Nermin

    2010-12-01

    Isolated renal vein thrombosis is very rare without the presence of nephrotic syndrome. It is more common in the newborns and infants. Whereas major risk factors in adults are the procoagulant states such as protein C or S deficiency, factor V Leiden mutation, primary or secondary antiphospholipid syndrome, severe hypothyroidism, and trauma. Here, we report a case of isolated renal vein thrombosis associated with MTHFR-1298 and PAI-1 4G gene mutations. It should be noted that the presence of MTHFR-1298 and PAI-1 4G gene mutations together might be one of the examples of genetic mutation combinations that increase the likelihood of a thrombotic event.

  6. Renal failure

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    2005234 Association between serum fetuin-A and clinical outcome in end-stage renal disease patients. WANG Kai(王开), Dept Renal Dis, Renji Hosp Shanghai, 2nd Med Univ, Shanghai 200001. Chin J Nephrol, 2005;21(2):72-75. Objective: To investigate the change of serum fetuin-A level before and after dialysis, and the association of serum fetuin-A level with clinical parameters

  7. Renal failure

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    950351 Serum erythropoietin levels in chronic renalinsufficiency.ZHAI Depei(翟德佩),et al.DeptNephrol.General Hosp,Tianjin Med Univ,Tianjin,300000.Tianjin Med J 1995;23(1):19-21.Patients with chronic renal insufficiency(CRI) areoften associated with anemia.The deficiency of EPOproduction in the kidney is thought to be a key factorin the pathogenesis of renal anemia.Serum erythropoi-

  8. Renal failure

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008463 Protective effect of recombination rat augmenter of liver regeneration on kidney in acute renal failure rats. TANG Xiaopeng(唐晓鹏), et al. Dept Nephrol, 2nd Affili Hosp Chongqing Med Univ, Chongqing 400010.Chin J Nephrol 2008;24(6):417-421. Objective To investigate the protective effects of recombination rat augmenter of liver regeneration (rrALR) on tubular cell injury and renal dysfunction

  9. Renal Hemangiopericytoma

    Directory of Open Access Journals (Sweden)

    İbrahim Halil Bozkurt

    2015-03-01

    Full Text Available Hemangiopericytoma is an uncommon perivascular tumor originating from pericytes in the pelvis, head and tneck, and the meninges; extremely rarely in the urinary system. We report a case of incidentally detected renal mass in which radiologic evaluation was suggestive of renal cell carcinoma. First, we performed partial nephrectomy, and then, radical nephrectomy because of positive surgical margins and the pathological examination of the surgical specimen that revealed a hemangiopericytoma. No additional treatment was administered.

  10. Thrombotic thrombocytopenic purpura: MRI demonstration of persistent small cerebral infarcts after clinical recovery

    Energy Technology Data Exchange (ETDEWEB)

    Gruber, O.; Cramon, D.Y. von [Day-Care Clinic for Cognitive Neurology, University of Leipzig (Germany); Max Planck Institute of Cognitive Neuroscience, Leipzig (Germany); Wittig, I. [Day-Care Clinic for Cognitive Neurology, University of Leipzig (Germany); Wiggins, C.J. [Max Planck Institute of Cognitive Neuroscience, Leipzig (Germany)

    2000-08-01

    Abnormalities in the brain of patients with thrombotic thrombocytopenic purpura (TTP) are infrequent on MRI, often reversible and mainly limited to symptomatic stages of the disease. We report a case in which high-resolution MRI demonstrated multiple persistent small cortical infarcts after clinical remission. High-resolution MRI investigations may detect clinically latent but permanent brain damage, and complement clinical judgement in guiding therapeutic decisions. (orig.)

  11. Is chronic HIV infection associated with venous thrombotic disease? A systematic review.

    Science.gov (United States)

    Klein, S K; Slim, E J; de Kruif, M D; Keller, T T; ten Cate, H; van Gorp, E C M; Brandjes, D P M

    2005-04-01

    Infection with the human immunodeficiency virus (HIV) is still a major health problem world-wide. HIV infection has changed into a chronic infection with the chance of developing long-term complications. Vascular complications are frequently reported in the current literature. HIV and treatment by highly active antiretroviral therapy (HAART) are associated with many cardiovascular risk factors. An increased risk of arterial cardiovascular complications was found in a number of studies. However, data about the risk of venous thrombotic disease (VTE), including potentially fatal conditions as pulmonary embolism, were limited. In a systematic review of the literature, ten relevant epidemiological studies were identified that investigated the risk of venous thrombotic disease in HIV-infected patients. The incidence was increased two- to tenfold in comparison with a healthy population of the same age. However, these studies were mainly retrospective cohort studies that were prone to selection bias, confounding factors were not always mentioned and in all but three control populations were missing. An increased risk of venous thrombotic disease in HIV-infected patients could be explained by the presence of a hypercoagulable state, characterised by an increase in procoagulant factors, such as endothelial TF expression and thrombogenic properties of microparticles, and a decrease in anticoagulant factors, including AT III, HC II and the protein C pathway. Furthermore, the risk of VTE was associated with an increased risk of infections and autoimmune haemolytic anaemia, and was weakly associated with HAART. All together, quite some evidence pointed towards a relationship between HIV infection and venous thrombotic disease, but the association still needs to be established in properly designed epidemiological studies.

  12. Successful recanalization of acute superior mesenteric artery thrombotic occlusion with primary aspiration thrombectomy

    Institute of Scientific and Technical Information of China (English)

    Hye; Jin; Yang; Young; Kwon; Cho; Yun; Ju; Jo; Yoon; Young; Jung; Seung; A; Choi; Suk; Hoon; Lee

    2010-01-01

    Prompt revascularization of the superior mesenteric artery(SMA) thrombotic occlusion can prevent intestinal infarction and decrease necrosis of the bowel segment.Herein,we describe two cases who underwent successful endovascular recanalization for acute SMA thrombosis using a primary aspiration thrombectomy because of possible consequent laparotomy for survey of bowel viability.The two patients had dramatic pain reliefimmediately after the procedure and remained symptomfree during the follow-up period.

  13. A patient with Crohn's disease who presented with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome.

    Science.gov (United States)

    Unverdi, Selman; Ceri, Mevlut; Öztürk, Mehmet Akif; Akbal, Erdem; Ensari, Arzu; Yılmaz, Rahmi; Kocak, Erdem; Inal, Salih; Koklu, Seyfettin; Duranay, Murat

    2011-01-01

    Thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS) is characterized with fever, purpura, anemia due to microangiopathic hemolysis, thrombocytopenia, kidney damage, and neurologic symptoms. The development of TTP/HUS during the course of inflammatory bowel diseases was rarely reported. However, coexistence of TTP/HUS and Crohn's disease in the same patient has not been reported previously. We herein present a case of TTP/HUS who presented with Crohn's disease. He responded to cyclosporine treatment.

  14. The thrombotic potential of circulating tumor microemboli: computational modeling of circulating tumor cell-induced coagulation

    OpenAIRE

    Phillips, Kevin G.; Lee, Angela M.; Tormoen, Garth W.; Rigg, Rachel A.; Kolatkar, Anand; Luttgen, Madelyn; Bethel, Kelly; Bazhenova, Lyudmila; Kuhn, Peter; Newton, Paul; McCarty, Owen J.T.

    2014-01-01

    Thrombotic events can herald the diagnosis of cancer, preceding any cancer-related clinical symptoms. Patients with cancer are at a 4- to 7-fold increased risk of suffering from venous thromboembolism (VTE), with ∼7,000 patients with lung cancer presenting from VTEs. However, the physical biology underlying cancer-associated VTE remains poorly understood. Several lines of evidence suggest that the shedding of tissue factor (TF)-positive circulating tumor cells (CTCs) and microparticles from p...

  15. Potential influences of complement factor H in autoimmune inflammatory and thrombotic disorders

    OpenAIRE

    Ferluga, J.; Kouser, L; Murugaiah, V; Sim, RB; Kishore, U

    2017-01-01

    Complement system homeostasis is important for host self-protection and anti-microbial immune surveillance, and recent research indicates roles in tissue development and remodelling. Complement also appears to have several points of interaction with the blood coagulation system. Deficiency and altered function due to gene mutations and polymorphisms in complement effectors and regulators, including Factor H, has been associated with familial and sporadic autoimmune inflammatory - thrombotic d...

  16. The modified pulse-spray method using Urokinase in subacute and chronic thrombotic arterial occlusion

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Youn Kil; Hahn, Seong Tae; Baek, Jee Hee; Kim, Choon Yul; Shinn, Kyung Sub [The Catholic Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-01-01

    To evaluate the effectiveness and safety of the modified pulse-spray method using Urokinase(UK) in subacute and chronic thrombotic arterial occlusion. Modified pulse-spray methods using UK were performed in seven patients with subacute (1 week-1month) to chronic (1month-5years) occlusive symptoms such as limb pain, claudication and impotence. Angiographic examination revealed thrombotic occlusion of the aorta, common iliac arteries, brachial arterio-venous hemodialysis graft and femoro-popliteal bypass graft. The patients underwent thrombolysis using modified pulse-spray and additional constant infusion of UK. In the presence of underlying stenosis or organized clots, balloon angioplasty or stent placement was performed. Complete lysis was obtained in five of seven patients. For initial lysis, the mean dose of UK was 420,000 units, and the mean modified pulse-spray time was 50 minutes. Mean total dose of UK and mean total time for complete lysis were 800,000 units and 161 minutes, respectively. Thrombolysis of the femoro-popliteal bypass graft failed due to severe occlusion of the distal anastomosis. Partial lysis was achieved in one patient with aorto-illac occlusion, but further thrombolysis was stopped due to bleeding at the puncture site. The modified pulse-spray method using UK is effective in treating subacute and chronic arterial thrombotic occlusion. It augments the speed, safety and efficacy of thrombolysis. When underlying stenosis or organized clots remain after thrombolysis, ballon angioplasty or stent placement would be helpful.

  17. 霉酚酸酯治疗狼疮性肾炎伴血管病变患者血管内皮细胞损伤标记物的变化%The Change levels of endothelial biomarkers in mycophenolate mofetil treatment for patients with lupus nephritis presented with renal vasculopathy

    Institute of Scientific and Technical Information of China (English)

    王梅; 王静; 王晓玲; 王文革; 孔玉科; 赵俊丽; 曾嵘; 王俭勤

    2011-01-01

    Objectives To explore the change levels of circulatory endothelial damage biomarkers, circulating endothelial cells ( CECs), E - selectin, Thrombomodulin ( TM ) and vascular cell adhesion molecule-1(VCAM -1 ), in MMF treatment for patients with lupus nephritis (LN) presented with renal vasculopathy. Methods A prospective study was performed on eight patients with class Ⅳ LN presenting with renal noninflammatory necrotizing vasculopathy (NNV) or thrombotic microangiopathy (TMA). All patients were treated with mycophenolate mofetil (MMF) as induction therapy; four patients required initial renal replacement treatment on admission.The correlation between the change level of the biomarkers and the efficacy of MMF was investigated. CECs were isolated from peripheral blood with anti - CD - 146 - coated immunomagnetic Dynabeads, and the levels of TM, VCAM -1 and E-selectin were measured by ELISA. Results Therapeutic responses were achieved in all patients.There were no changes of the level of those biomarkers in patients required initial renal replacement treatment. A strong positive correlation was found between the level of CECs and proteinuria, urinary red blood cell count, serum creatinine( p<0.05), and a negative correlation was observed between the level of CECs and serum albumin concentration( p<0.05). Conclusions The decrease of CECs was associated with the amelioration of endothelial damage during MMF treatment for patients presented with lupus vasculopathy, thereby, CECs, provided dynamic detection, were considered as a promising marker to evaluate MMF therapeutic potential.%目的 观察霉酚酸酯(MMF)治疗Ⅳ型狼疮性肾炎(LN)伴血管病变过程中,循环内皮细胞(CECs)、E-选择素(E-selectin)、血栓调节蛋白(TM)和血管内皮细胞黏附分子-1(VCAM-1)的变化,以期寻找无创的、反映MMF疗效的特异性分子标记物指导其治疗.方法 8例经肾活检诊断为Ⅳ型LN伴肾间质非炎症坏死性血管病变(NNV)或血

  18. Acute renal infarction associated with homozygous methylenetetrahydrofolate reductase mutation C677T and IgA beta-2-glycoprotein antibodies.

    Science.gov (United States)

    Vlachostergios, Panagiotis J; Dufresne, François

    2015-07-01

    Arterial thrombosis of the kidney(s) is a rare clinical entity usually presenting as a result of cardioembolic disease, though rare inherited hypercoagulable states have also been implicated. Within this context, both hyperhomocysteinemia triggered by a mutated methylenetetrahydrofolate reductase (MTHFR) gene product and the presence of antiphospholipid antibodies have been separately associated with arterial thrombotic events, including renal artery embolism. We present a case of combined homozygous MTHFR C677T mutation and IgA beta-2-glycoprotein antibody positivity resulting in acute renal infarction and previous silent myocardial infarction. An acute and otherwise unexplained thrombotic event of unusual location always warrants further investigation, which should include testing for hereditary thrombophilic disorders.

  19. Interferons beta have vasoconstrictive and procoagulant effects: a woman who developed livedo reticularis and Raynaud phenomenon in association with interferon beta treatment for multiple sclerosis.

    Science.gov (United States)

    Rot, Uroš; Ledinek, Alenka Horvat

    2013-12-01

    A 31-year-old woman with MS developed livedo reticularis and secondary Raynaud phenomenon 2.5 years after introduction of interferon beta-1b. The symptoms disappeared after withdrawal of the drug. Livedo reticularis and Raynaud phenomenon as well as pulmonary arterial hypertension, venous sinus thrombosis, pulmonary embolism and renal thrombotic microangiopathy have all been described in association with interferon beta therapy. These complications strongly suggest that type I interferons have vasoconstrictive and procoagulant effects with potentially serious systemic complications.

  20. Acute kidney injury during pregnancy.

    Science.gov (United States)

    Van Hook, James W

    2014-12-01

    Acute kidney injury complicates the care of a relatively small number of pregnant and postpartum women. Several pregnancy-related disorders such as preeclampsia and thrombotic microangiopathies may produce acute kidney injury. Prerenal azotemia is another common cause of acute kidney injury in pregnancy. This manuscript will review pregnancy-associated acute kidney injury from a renal functional perspective. Pathophysiology of acute kidney injury will be reviewed. Specific conditions causing acute kidney injury and treatments will be compared.

  1. Oral anticoagulant therapy during and after coronary angioplasty the intensity and duration of anticoagulation are essential to reduce thrombotic complications.

    Science.gov (United States)

    ten Berg, J M; Hutten, B A; Kelder, J C; Verheugt, F W; Plokker, H W

    2001-04-24

    In the randomized Balloon Angioplasty and Anticoagulation Study (BAAS), the addition of oral anticoagulants to aspirin significantly reduced early and late events after coronary angioplasty. However, bleeding episodes were increased. The present report studied the intensity and the duration of anticoagulation as predictors of thrombotic and bleeding events. A total of 530 patients, 34% of whom received a stent, were treated with aspirin plus coumarins. Half of the patients were randomized to angiographic follow-up. The target international normalized ratio (INR) was 2.1 to 4.8 during angioplasty and 6-month follow-up. Thrombotic events were death, myocardial infarction, target lesion revascularization, and thrombotic stroke. Bleeding complications were hemorrhagic stroke, major extracranial bleeding, and false aneurysm. "Optimal" anticoagulation was defined as an INR in the target range for at least 70% of the follow-up time. There were 17 early thrombotic events (3.2%), 7 early bleeding episodes (1.3%), and 10 false aneurysms (1.9%). The incidence rate for both early thrombotic and bleeding events was lowest in patients in the target range. A total of 61 late thrombotic events occurred (11.6%). Optimal anticoagulation was an independent predictor of late thrombotic events (relative risk, 0.33; 95% CI, 0.19 to 0.57) and was associated with a 0.21 mm (95% CI, 0.17 to 0.42) larger vessel lumen at 6 months. Late bleeding episodes (1.4%) were lowest in patients in the target range. Coumarins started before coronary angioplasty with a target INR of 2.1 to 4.8 led to the lowest procedural event rate, without an increase in bleeding episodes. During follow-up, optimal anticoagulation was associated with a decrease in the incidence of late events by 67% and a significant improvement in 6-month angiographic outcome.

  2. Renal Cysts

    Science.gov (United States)

    ... as “simple” cysts, meaning they have a thin wall and contain water-like fluid. Renal cysts are fairly common in ... simple kidney cysts, meaning they have a thin wall and only water-like fluid inside. They are fairly common in ...

  3. Renal failure

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    970363 Effect on serum PTH and 1, 25(OH)2 D3levels of rapid correction of metabolic acidosis in CRFpatients with secondary hyperparathyroidism. YUANQunsheng(袁群生), et al. Renal Div, PUMC Hosp,Beijing, 100730. Chin J Nephrol 1996; 12(6): 328-331.

  4. Drug-induced renal injury

    African Journals Online (AJOL)

    Drugs can cause acute renal failure by causing pre-renal, intrinsic or post-renal toxicity. Pre-renal ... incidence of drug dose adjustment in renal impairment in the SAMJ. ... Fever, haemolytic anaemia, thrombocytopenia, renal impairment and.

  5. Renal failure (chronic)

    OpenAIRE

    Clase, Catherine

    2011-01-01

    Chronic renal failure is characterised by a gradual and sustained decline in renal clearance or glomerular filtration rate (GFR). Continued progression of renal failure will lead to renal function too low to sustain healthy life. In developed countries, such people will be offered renal replacement therapy in the form of dialysis or renal transplantation. Requirement for dialysis or transplantation is termed end-stage renal disease (ESRD).Diabetes, glomerulonephritis, hypertension, pyelone...

  6. Renal Impairment and Prognosis of Patients with Atrial Fibrillation Undergoing Coronary Intervention - The AFCAS Trial.

    Directory of Open Access Journals (Sweden)

    Heli M Lahtela

    Full Text Available Renal impairment is a well-known risk factor for cardiovascular complications, but the effect of different stages of renal impairment on thrombotic/thromboembolic and bleeding complications in patients with atrial fibrillation (AF undergoing percutaneous coronary intervention (PCI remains largely unknown. We sought to evaluate the incidence and clinical impact of four stages of renal impairment in patients with AF undergoing PCI.We assessed renal function by estimated glomerular filtration rate (eGFR and outcomes in 781 AF patients undergoing PCI by using the data from a prospective European multicenter registry. End-points included all-cause mortality, major adverse cardiac and cerebrovascular events (MACCE and bleeding events at 12 months.A total of 195 (25% patients had normal renal function (eGFR ≥90 mL/min, 290 (37% mild renal impairment (eGFR 60-89, 263 (34% moderate renal impairment (eGFR 30-59 and 33 (4% severe renal impairment (eGFR <30. Degree of renal impairment remained an independent predictor of mortality and MACCE in an adjusted a Cox regression model. Even patients with mild renal impairment had a higher risk of all-cause mortality (HR 2.25, 95%CI 1.02-4.98, p=0.04 and borderline risk for MACCE (HR 1.56, 95%CI 0.98- 2.50, p=0.06 compared to those with normal renal function.Renal impairment is common in patients with AF undergoing PCI and even mild renal impairment has an adverse prognostic effect in these patients requiring multiple antithrombotic medications.

  7. Pseudo-thrombotic thrombocytopenic purpura: A rare presentation of pernicious anemia

    Directory of Open Access Journals (Sweden)

    Ashvin K Tadakamalla

    2011-01-01

    Full Text Available Context: Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of pernicious anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura. Case Report : A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B 12 was found to be low. Treatment with intra-muscular vitamin B 12 led to symptomatic and hematologic improvement. Pernicious anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis. Conclusion : Clinicians must be aware of unusual clinical presentation of vitamin B 12 deficiency with schistocytes as the management is simple and effective.

  8. Low levels of vitamin D are common in primary antiphospholipid syndrome with thrombotic disease

    Directory of Open Access Journals (Sweden)

    P.L. Meroni

    2012-12-01

    Full Text Available The aim of this study was to assess vitamin D (vit.D levels in patients with primary antiphospholipid syndrome (PAPS, the association between hypovitaminosis D and clinical manifestations, and the effect of vit.D supplementation on serum levels. Vit.D serum levels of 115 PAPS patients, classified according to the 2006 revised criteria at the Rheumatology Department, Brescia, and of 128 voluntary healthy donors (NHD were tested in collaboration with DiaSorin (Saluggia, Italy using the LIAISON® chemiluminescent immunoassay. Clinical data were derived from clinical charts. Vit.D deficiency was more prevalent in PAPS than NHD (17% vs 5%. During the summer, vit.D levels were lower in PAPS than NHD (median 28 vs 40.1 ng/mL, P<0.01. PAPS were subdivided according to clinical characteristics (thrombotic vs obstetric. Both groups had lower vit.D levels compared to NHD. Thrombotic PAPS had significantly lower levels than obstetric PAPS (median 20.8 vs 33.3, P<0.01. Sixteen patients (14% received oral 25-OH vit.D supplementation (average 400 UI/die, but 63% of them did not reach serum levels above 30 ng/mL. PAPS showed significantly lower levels of vit.D than NHD. Hypovitaminosis D was seen to cluster in patients with thrombosis which may suggest that the lack of vit.D could be one of the many factors involved in the thrombotic process. Low-dose supplementation did not seem to be effective in a small group of patients.

  9. Brainstem involvement as a cause of central sleep apnea: pattern of microstructural cerebral damage in patients with cerebral microangiopathy.

    Directory of Open Access Journals (Sweden)

    Thomas Duning

    Full Text Available BACKGROUND: The exact underlying pathomechanism of central sleep apnea with Cheyne-Stokes respiration (CSA-CSR is still unclear. Recent studies have demonstrated an association between cerebral white matter changes and CSA. A dysfunction of central respiratory control centers in the brainstem was suggested by some authors. Novel MR-imaging analysis tools now allow far more subtle assessment of microstructural cerebral changes. The aim of this study was to investigate whether and what severity of subtle structural cerebral changes could lead to CSA-CSR, and whether there is a specific pattern of neurodegenerative changes that cause CSR. Therefore, we examined patients with Fabry disease (FD, an inherited, lysosomal storage disease. White matter lesions are early and frequent findings in FD. Thus, FD can serve as a "model disease" of cerebral microangiopathy to study in more detail the impact of cerebral lesions on central sleep apnea. PATIENTS AND METHODS: Genetically proven FD patients (n = 23 and age-matched healthy controls (n = 44 underwent a cardio-respiratory polysomnography and brain MRI at 3.0 Tesla. We applied different MR-imaging techniques, ranging from semiquantitative measurement of white matter lesion (WML volumes and automated calculation of brain tissue volumes to VBM of gray matter and voxel-based diffusion tensor imaging (DTI analysis. RESULTS: In 5 of 23 Fabry patients (22% CSA-CSR was detected. Voxel-based DTI analysis revealed widespread structural changes in FD patients when compared to the healthy controls. When calculated as a separate group, DTI changes of CSA-CSR patients were most prominent in the brainstem. Voxel-based regression analysis revealed a significant association between CSR severity and microstructural DTI changes within the brainstem. CONCLUSION: Subtle microstructural changes in the brainstem might be a neuroanatomical correlate of CSA-CSR in patients at risk of WML. DTI is more sensitive and specific than

  10. Mutations in CTC1, encoding the CTS telomere maintenance complex component 1, cause cerebroretinal microangiopathy with calcifications and cysts.

    Science.gov (United States)

    Polvi, Anne; Linnankivi, Tarja; Kivelä, Tero; Herva, Riitta; Keating, James P; Mäkitie, Outi; Pareyson, Davide; Vainionpää, Leena; Lahtinen, Jenni; Hovatta, Iiris; Pihko, Helena; Lehesjoki, Anna-Elina

    2012-03-09

    Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) is a rare multisystem disorder characterized by extensive intracranial calcifications and cysts, leukoencephalopathy, and retinal vascular abnormalities. Additional features include poor growth, skeletal and hematological abnormalities, and recurrent gastrointestinal bleedings. Autosomal-recessive inheritance has been postulated. The pathogenesis of CRMCC is unknown, but its phenotype has key similarities with Revesz syndrome, which is caused by mutations in TINF2, a gene encoding a member of the telomere protecting shelterin complex. After a whole-exome sequencing approach in four unrelated individuals with CRMCC, we observed four recessively inherited compound heterozygous mutations in CTC1, which encodes the CTS telomere maintenance complex component 1. Sanger sequencing revealed seven more compound heterozygous mutations in eight more unrelated affected individuals. Two individuals who displayed late-onset cerebral findings, a normal fundus appearance, and no systemic findings did not have CTC1 mutations, implying that systemic findings are an important indication for CTC1 sequencing. Of the 11 mutations identified, four were missense, one was nonsense, two resulted in in-frame amino acid deletions, and four were short frameshift-creating deletions. All but two affected individuals were compound heterozygous for a missense mutation and a frameshift or nonsense mutation. No individuals with two frameshift or nonsense mutations were identified, which implies that severe disturbance of CTC1 function from both alleles might not be compatible with survival. Our preliminary functional experiments did not show evidence of severely affected telomere integrity in the affected individuals. Therefore, determining the underlying pathomechanisms associated with deficient CTC1 function will require further studies.

  11. Cardiovascular thrombotic events in arthritis trials of the cyclooxygenase-2 inhibitor celecoxib.

    Science.gov (United States)

    White, William B; Faich, Gerald; Borer, Jeffrey S; Makuch, Robert W

    2003-08-15

    To determine whether the cyclooxygenase-2 (COX-2) inhibitor celecoxib affects cardiovascular thrombotic risk, we analyzed the incidence of cardiovascular events for celecoxib, placebo, and nonsteroidal anti-inflammatory drugs (NSAIDs) in the entire controlled, arthritis clinical trial database for celecoxib. The primary analysis used the Antiplatelet Trialists' Collaboration end points, which include: (1) cardiovascular, hemorrhagic, and unknown deaths, (2) nonfatal myocardial infarction, and (3) nonfatal stroke. Other secondary thrombotic events were also examined. Separate analyses were performed for all patients and for those not taking aspirin. Data from all controlled, completed arthritis trials of > or =4 weeks duration, including 13 new drug application studies and 2 large post-marketing trials (CLASS and SUCCESS) were included for analyses. Patients were randomized to celecoxib at doses from 100 to 400 mg twice daily (18,942 patients; 5,668.2 patient-years of exposure), diclofenac 50 to 75 mg twice daily, ibuprofen 800 mg thrice daily, naproxen 500 mg twice daily (combined NSAID exposure of 11,143 patients; 3,612.2 patient-years), or placebo (1,794 subjects; 199.9 subject-years). Data from a long-term uncontrolled trial with 5,209 patients (6,950 patients-years) treated with celecoxib were included in a supplemental analysis. The entire 15-trial database was searched for possible serious thrombotic events as well as to identify all deaths. For these patients, detailed clinical data were obtained and reviewed by 2 of the investigators (WBW and JSB), who were independently and blinded to exposure, to classify the event as primary, secondary, or neither. All analyses were done using the intent-to-treat population, and time-to-event analyses were performed using per-patient data. To examine heterogeneity of results among studies, tests of interaction were performed using the Cox model. Incidences of the primary and secondary events were not significantly

  12. Valsartan Decreases Platelet Activity and Arterial Thrombotic Events in Elderly Patients with Hypertension

    Institute of Scientific and Technical Information of China (English)

    Fang Wu; Hong-Yan Wang; Fan Cai; Ling-Jie Wang; Feng-Ru Zhang; Xiao-Nan Chen; Qian Yang

    2015-01-01

    Background:Angiotensin type 1 receptor (AT1R) antagonists are extensively used for blood pressure control in elderly patients with hypertension.This study aimed to investigate the inhibitory effects of AT1R antagonist valsartan on platelet aggregation and the occurrence of cardio-cerebral thrombotic events in elderly patients with hypertension.Methods:Two-hundred and ten patients with hypertension and aged > 60 years were randomized to valsartan (n =140) or amlodipine (n =70) on admission.The primary endpoint was platelet aggregation rate (PAR) induced by arachidonic acid at discharge,and the secondary endpoint was the rate of thrombotic events including brain infarction and myocardial infarction during follow-up.Human aortic endothelial cells (HAECs) were stimulated by angiotensin Ⅱ (Ang Ⅱ,100 nmol/L) with or without pretreatment of valsartan (100 nmol/L),and relative expression of cyclooxygenase-2 (COX-2) and thromboxane B2 (TXB2) and both p38 mitogen-activated protein kinase (p38MAPK) and nuclear factor-kB (NF-kB) activities were assessed.Statistical analyses were performed by GraphPad Prism 5.0 software (GraphPad Software,Inc.,California,USA).Results:PAR was lower after treatment with valsartan (1 1.49 ± 0.69% vs.18.71 ± 2.47%,P < 0.001),associated with more reduced plasmalevels of COX-2 (76.94 ± 7.07 U/L vs.116.4 ± 15.89 U/L,P < 0.001) and TXB2 (1667 ± 56.50 pg/ml vs.2207 ± 180.20 pg/ml) (all P < 0.001).Plasma COX-2 and TXB2 levels correlated significantly with PAR in overall patients (r =0.109,P < 0.001).During follow-up (median,18 months),there was a significantly lower thrombotic event rate in patients treated with valsartan (14.3% vs.32.8%,P =0.002).Relative expression of COX-2 and secretion of TXB2 with concordant phosphorylation ofp38MAPK and NF-kB were increased in HAECs when stimulated by Ang Ⅱ (100 nmol/L) but were significantly decreased by valsartan pretreatment (100 nmol/L).Conclusions:AT1R antagonist valsartan

  13. [Thrombotic vasculopathy probably associated with cocaine contaminated with levamisole: report of 2 cases].

    Science.gov (United States)

    Martínez-Velasco, María Abril; Flores-Suárez, Luis Felipe; Toussaint-Caire, Sonia; Rodríguez-Carreón, Angélica; Díaz-Lozano, Marisol; Sánchez-Armendáriz, Karen

    2015-01-01

    The vasculities are complex diseases. Their cutaneous manifestations are very important and often mirror several pathologies. Cocaine use has been related to both, vasculitis and thrombotic vasculopathy and pseudovasculitis. A new syndrome has been described in association with its adulteration with levamisole. It can be very serious, leading patients to death. This is relevant as levamisole-adultered cocaine seems to be increasingly offered to consumers. Our goal is to report the first two cases in Mexico, which faces an important raise in cocaine use, emphasizing that a high suspicion based on certain characteristics allows for early recognition and adequate treatment.

  14. Excellent long-term results with iliac stenting in local anesthesia for post-thrombotic syndrome

    DEFF Research Database (Denmark)

    Klitfod, Lotte; Just, Sven; Foegh, Pia

    2015-01-01

    BACKGROUND: Only 20% of iliac veins will recanalize on anticoagulation (AC) treatment alone and may, therefore, develop venous obstruction after iliofemoral deep venous thrombosis (DVT). A considerable number of these patients will suffer from post-thrombotic syndrome (PTS) leading to impaired qu...... stent was 89% (17/19) and 16 patients (84 %) had almost or total symptom relief at follow-up. CONCLUSION: Endovascular stenting of iliac obstruction in local anesthesia is minimally invasive and shows excellent long-term outcomes for patients suffering from PTS....

  15. Platelets miRNA as a Prediction Marker of Thrombotic Episodes

    Directory of Open Access Journals (Sweden)

    Michal Bijak

    2016-01-01

    Full Text Available The blood platelets are crucial for the coagulation physiology to maintain haemostatic balance and are involved in various pathologies such as atherosclerosis and thrombosis. The studies of recent years have shown that anucleated platelets are able to succeed protein synthesis. Additionally, mRNA translation in blood platelets is regulated by miRNA molecules. Recent works postulate the possibility of using miRNAs as biomarkers of atherosclerosis and ischemic episodes. This review article describes clinical studies that presented blood platelets miRNAs expression profile changes in different thrombotic states, which suggest use of these molecules as predictive biomarkers.

  16. Elevated D-dimers in attacks of hereditary angioedema are not associated with increased thrombotic risk

    OpenAIRE

    Reshef, A; Zanichelli, A.; LONGHURST, H; Relan, A; Hack, C E

    2015-01-01

    Background Recommended management of attacks of hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (C1-INH-HAE) includes therapy with exogenous C1INH. Thrombotic/thromboembolic events (TEE) have been reported with plasma-derived C1INH, but so far none with recombinant human C1INH (rhC1INH). This phase III, randomized, placebo (saline)-controlled study evaluated the safety of rhC1INH 50 IU/kg for the treatment of acute attacks in 74 patients with C1-INH-HAE. Methods M...

  17. Acute Simultaneous Thrombotic Occlusion of Multiple Coronary Arteries in Acute Myocardial Infarction: A Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Mahdi Daei

    2017-05-01

    Full Text Available Introduction Simultaneous multiple coronary artery thrombosis is a rare finding in ST segment elevation myocardial infarction (STEMI, and has a high mortality rate. Case Presentation We report a case of myocardial infarction with cardiogenic shock, left bundle branch block, and multiple ST segment elevation on the electrocardiogram and thrombotic occlusion of the left circumflex, optus marginal, and left anterior descending arteries on emergency coronary angiography. Thrombus aspiration was performed at left circumflex, optus marginal, and left anterior descending arteries. Conclusions In patients with STEMI, multiple coronary thrombosis is unusual and associated with high patient mortality.

  18. Renale Osteopathie

    OpenAIRE

    Horn S

    2001-01-01

    Die renale Osteopathie umfaßt Erkrankungen des Knochens, die bei Patienten mit chronischen Nierenerkrankungen auftreten, wie den sekundären bzw. tertiären Hyperparathyreoidismus, die adynamische Knochenerkrankung und die Osteopathie nach Nierentransplantation. Durch die Identifikation des Kalzium-Sensing-Rezeptors bzw. des Vitamin D-Rezeptors hat sich unser Verständnis der Zusammenhänge in den letzten Jahren erheblich verbessert. Neue Medikamente versprechen effizientere Prophylaxe- und Thera...

  19. Renale Knochenerkrankungen

    Directory of Open Access Journals (Sweden)

    Mayer G

    2008-01-01

    Full Text Available Störungen des Mineral- und Knochenstoffwechsels sind bei fast allen Patienten mit chronischen Nierenerkrankungen anzutreffen. Pathogenetisch spielt eine Neigung zur Phosphatretention bei einer Reduktion der glomerulären Filtrationsrate die zentrale Rolle. Neben typischen, aber sehr variablen Veränderungen der Knochenstruktur (renale Osteopathie besteht auch eine sehr enge Assoziation zwischen diesen Störungen und dem massiv erhöhten kardiovaskulären Risiko der Patienten.

  20. Obesity and renal hemodynamics

    NARCIS (Netherlands)

    Bosma, R. J.; Krikken, J. A.; van der Heide, J. J. Homan; de Jong, P. E.; Navis, G. J.

    2006-01-01

    Obesity is a risk factor for renal damage in native kidney disease and in renal transplant recipients. Obesity is associated with several renal risk factors such as hypertension and diabetes that may convey renal risk, but obesity is also associated with an unfavorable renal hemodynamic profile

  1. Obesity and renal hemodynamics

    NARCIS (Netherlands)

    Bosma, R. J.; Krikken, J. A.; van der Heide, J. J. Homan; de Jong, P. E.; Navis, G. J.

    2006-01-01

    Obesity is a risk factor for renal damage in native kidney disease and in renal transplant recipients. Obesity is associated with several renal risk factors such as hypertension and diabetes that may convey renal risk, but obesity is also associated with an unfavorable renal hemodynamic profile inde

  2. [Evaluation of the antithrombotic strategy in low thrombotic risk patients who underwent aortic valve replacement wth bioprosthesis].

    Science.gov (United States)

    Aceves-Velázquez, Eduardo; Vieyra-Herrera, Gerardo; Rodríguez-Chávez, Laura; Herrera-Alarcón, Valentín

    2017-07-15

    According to current guidelines, in patients without additional risk factors who underwent aortic valve replacement with a bioprosthesis, anticoagulation in the first 3 months after surgery is still a matter of debate. According to current evidence, aspirin in low doses is a reasonable alternative to vitamin K antagonists (VKA). We compared the incidence of thrombotic and hemorrhagic complications in patients with low thrombotic risk who underwent aortic valve replacement with a bioprosthesis in the National Institute of Cardiology of Ignacio Chávez of Mexico. The hypothesis: aspirin as monotherapy has a beneficial effect compared to VKA. The studied patients were the low thrombotic risk patients who underwent aortic valve replacement with a bioprostesis in the National Institute of Cardiology of Ignacio Chávez of Mexico from 2011 to 2015. The groups studied were: aspirin only, VKA only and the combination of VKA plus aspirin. The patients were retrospectively followed during 12 months and the thrombotic and hemorrhagic complications were documented. Two hundred thirty-one patients were analized. Only one patient presented a hemorrhagic complication in the VKA only group. No thrombotic complications were observed. In the present study no thrombotic complications were observed in patients who did not receive anticoagulation in the first 3 months after a aortic valve replacement with a bioprosthesis after a follow up period of 12 months. This suggests that the use of aspirin only is safe during this period. Copyright © 2017 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

  3. Risk of cardiovascular thrombotic events after surgical castration versus gonadotropin-releasing hormone agonists in Chinese men with prostate cancer

    Directory of Open Access Journals (Sweden)

    Jeremy YC Teoh

    2015-06-01

    Full Text Available We investigated the cardiovascular thrombotic risk after surgical castration (SC versus gonadotropin-releasing hormone agonists (GnRHa in Chinese men with prostate cancer. All Chinese prostate cancer patients who were treated with SC or GnRHa from year 2000 to 2009 were reviewed and compared. The primary outcome was any new-onset of cardiovascular thrombotic events after SC or GnRHa, which was defined as any event of acute myocardial infarction or ischemic stroke. The risk of new-onset cardiovascular thrombotic event was compared between the SC group and the GnRHa group using Kaplan-Meier method. Multivariate Cox regression analysis was performed to adjust for other potential confounding factors. A total of 684 Chinese patients was included in our study, including 387 patients in the SC group and 297 patients in the GnRHa group. The mean age in the SC group (75.3 ± 7.5 years was significantly higher than the GnRHa group (71.8 ± 8.3 years (P < 0.001. There was increased risk of new cardiovascular thrombotic events in the SC group when compared to the GnRHa group upon Kaplan-Meier analysis (P = 0.014. Upon multivariate Cox regression analysis, age (hazard ratio [HR] 1.072, 95% confidence interval [CI] 1.04-1.11, P< 0.001, hyperlipidemia (HR 2.455, 95% CI 1.53-3.93, P< 0.001, and SC (HR 1.648, 95% CI 1.05-2.59, P= 0.031 were significant risk factors of cardiovascular thrombotic events. In conclusion, SC was associated with increased risk of cardiovascular thrombotic events when compared to GnRHa. This is an important aspect to consider while deciding on the method of androgen deprivation therapy, especially in elderly men with known history of hyperlipidemia.

  4. Transluminal Attenuation Gradient for Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    Science.gov (United States)

    Grande Gutierrez, Noelia; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2014-11-01

    Kawasaki Disease (KD) can result in coronary aneurysms in up to 25% of patients if not treated early putting patients at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines for administering anti-coagulation therapy currently rely on anatomy alone. Previous studies including patient specific modeling and computer simulations in KD patients have suggested that hemodynamic data can predict regions susceptible to thrombus formation. In particular, high Particle Residence Time gradient (PRTg) regions have shown to correlate with regions of thrombus formation. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length. TAG has been used for characterizing coronary artery stenoses, however this approach has not yet been used in aneurysmal vessels. The aim of this study is to analyze the correlation between TAG and PRTg in KD patients with aneurysms and evaluate the use of TAG as an index to quantify thrombotic risk. Patient specific anatomic models for fluids simulations were constructed from CT angiographic image data from 3 KD aneurysm patients and one normal control. TAG values for the aneurysm patients were markedly lower than for the non-aneurysmal patient (mean -18.38 vs. -2). In addition, TAG values were compared to PRTg obtained for each patient. Thrombotic risk stratification for KD aneurysms may be improved by incorporating TAG and should be evaluated in future prospective studies.

  5. CFD-based Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    Science.gov (United States)

    Sengupta, Dibyendu; Kung, Ethan; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2012-11-01

    Coronary aneurysms occur in 25% of untreated Kawasaki Disease (KD) patients and put patients at increased risk for myocardial infarction and sudden death. Clinical guidelines recommend using aneurysm diameter >8 mm as the arbitrary criterion for treating with anti-coagulation therapy. This study uses patient-specific modeling to non-invasively determine hemodynamic parameters and quantify thrombotic risk. Anatomic models were constructed from CT angiographic image data from 5 KD aneurysm patients and one normal control. CFD simulations were performed to obtain hemodynamic data including WSS and particle residence times (PRT). Thrombosis was clinically observed in 4/9 aneurysmal coronaries. Thrombosed vessels required twice as many cardiac cycles (mean 8.2 vs. 4.2) for particles to exit, and had lower mean WSS (1.3 compared to 2.8 dynes/cm2) compared to vessels with non-thrombosed aneurysms of similar max diameter. 1 KD patient in the cohort with acute thrombosis had diameter < 8 mm. Regions of low WSS and high PRT predicted by simulations correlated with regions of subsequent thrombus formation. Thrombotic risk stratification for KD aneurysms may be improved by incorporating both hemodynamic and geometric quantities. Current clinical guidelines to assess patient risk based only on aneurysm diameter may be misleading. Further prospective study is warranted to evaluate the utility of patient-specific modeling in risk stratifying KD patients with coronary aneurysms. NIH R21.

  6. Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

    Directory of Open Access Journals (Sweden)

    Cesar A. Perez

    2011-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE. Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

  7. [Testing system design and analysis for the execution units of anti-thrombotic device].

    Science.gov (United States)

    Li, Zhelong; Cui, Haipo; Shang, Kun; Liao, Yuehua; Zhou, Xun

    2015-02-01

    In an anti-thrombotic pressure circulatory device, relays and solenoid valves serve as core execution units. Thus the therapeutic efficacy and patient safety of the device will directly depend on their performance. A new type of testing system for relays and solenoid valves used in the anti-thrombotic device has been developed, which can test action response time and fatigue performance of relay and solenoid valve. PC, data acquisition card and test platform are used in this testing system based on human-computer interaction testing modules. The testing objectives are realized by using the virtual instrument technology, the high-speed data acquisition technology and reasonable software design. The two sets of the system made by relay and solenoid valve are tested. The results proved the universality and reliability of the testing system so that these relays and solenoid valves could be accurately used in the antithrombotic pressure circulatory equipment. The newly-developed testing system has a bright future in the aspects of promotion and application prospect.

  8. Anti-thrombotic effect of rutin isolated from Dendropanax morbifera Leveille.

    Science.gov (United States)

    Choi, Jun-Hui; Kim, Dae-Won; Park, Se-Eun; Lee, Hyo-Jeong; Kim, Ki-Man; Kim, Kyung-Je; Kim, Myung-Kon; Kim, Sung-Jun; Kim, Seung

    2015-08-01

    Dendropanax morbifera H. Lev. is well known in Korean traditional medicine for improvement of blood circulation. In this study, rutin, a bioflavonoid having anti-thrombotic and anticoagulant activities was isolated from a traditional medicinal plant, D. morbifera H. Lev. The chemical characteristics of rutin was studied to be quercetin 3-O-α-l-rhamnopyranosyl-(1-6)-β-d-glucopyranoside using high performance liquid chromatography mass spectrometry (HPLC-MS), proton nuclear magnetic resonance ((1)H NMR) and carbon-13 nuclear magnetic resonance ((13)C NMR). Turbidity and fibrin clotting studies revealed that rutin reduces fibrin clot in concentration dependent manner. Rutin was found to prolong activated partial thromboplastin time (aPTT), prothrombin time (PT) and closure time (CT). Furthermore, it decreased the activity of pro-coagulant protein, thrombin. In vivo study showed that rutin exerted a significant protective effect against collagen and epinephrine (or thrombin) induced acute thromboembolism in mice. These results suggest that rutin has a potent to be an anti-thrombotic agent for cardiovascular diseases.

  9. A perspective on the measurement of ADAMTS13 in thrombotic thrombocytopaenic purpura.

    Science.gov (United States)

    Pimanda, John E; Chesterman, Colin N; Hogg, Philip J

    2003-04-01

    The recent discovery of the von Willebrand Factor (vWF) cleaving protease (ADAMTS13) and the association of its deficiency with thrombotic thrombocytopaenic purpura (TTP) has generated both enormous interest and considerable confusion. Ultra large von Willebrand Factor (UL vWF) multimers are present in the plasmas of patients with chronic relapsing TTP in remission but disappear during an attack. This observation led to the recognition that UL vWF multimers precipitate the thrombotic occlusion of arterioles, a feature that characterizes TTP. Multiple mutations in ADAMTS13 are associated with congenital TTP and neutralizing autoantibodies have been demonstrated in the acquired TTP syndrome. Although a number of functional assays for this enzyme have been described, the more rigorously evaluated assays are difficult to perform outside a research laboratory. There is also an enduring uncertainty about the specificity of ADAMTS13 deficiency for the diagnosis of acquired TTP and a perception that the result does not alter patient management. The cloning of the ADAMTS13 gene has also raised the prospect of recombinant enzyme therapy for the treatment of TTP, and this has heightened the need for a simple assay. In this review, we evaluate the value of measuring this enzyme in the management of TTP.

  10. Complement Interactions with Blood Cells, Endothelial Cells and Microvesicles in Thrombotic and Inflammatory Conditions.

    Science.gov (United States)

    Karpman, Diana; Ståhl, Anne-lie; Arvidsson, Ida; Johansson, Karl; Loos, Sebastian; Tati, Ramesh; Békássy, Zivile; Kristoffersson, Ann-Charlotte; Mossberg, Maria; Kahn, Robin

    2015-01-01

    The complement system is activated in the vasculature during thrombotic and inflammatory conditions. Activation may be associated with chronic inflammation on the endothelial surface leading to complement deposition. Complement mutations allow uninhibited complement activation to occur on platelets, neutrophils, monocytes, and aggregates thereof, as well as on red blood cells and endothelial cells. Furthermore, complement activation on the cells leads to the shedding of cell derived-microvesicles that may express complement and tissue factor thus promoting inflammation and thrombosis. Complement deposition on red blood cells triggers hemolysis and the release of red blood cell-derived microvesicles that are prothrombotic. Microvesicles are small membrane vesicles ranging from 0.1 to 1 μm, shed by cells during activation, injury and/or apoptosis that express components of the parent cell. Microvesicles are released during inflammatory and vascular conditions. The repertoire of inflammatory markers on endothelial cell-derived microvesicles shed during inflammation is large and includes complement. These circulating microvesicles may reflect the ongoing inflammatory process but may also contribute to its propagation. This overview will describe complement activation on blood and endothelial cells and the release of microvesicles from these cells during hemolytic uremic syndrome, thrombotic thrombocytopenic purpura and vasculitis, clinical conditions associated with enhanced thrombosis and inflammation.

  11. TNF-alpha and annexin A2: inflammation in thrombotic primary antiphospholipid syndrome.

    Science.gov (United States)

    Bećarević, Mirjana

    2016-12-01

    Antiphospholipid syndrome (APS) is characterized by thromboses and/or pregnancy losses. Laboratory criterion for the diagnosis of APS is the presence of antiphospholipid antibodies (anticardiolipin, anti-beta2-glycoprotein I (aβ2gpI) and lupus anticoagulant). On the one hand, the latest classification criteria for the diagnosis of APS emphasized that thrombotic manifestations of the syndrome should be without any signs of an inflammatory process, while on the other hand, some recent reports have suggested that APS is a "pro-inflammatory state." This article is focused on the importance of TNF-alpha and annexin A2 (anxA2) for patients with vascular (thrombotic) manifestations of the primary APS. The classic antithrombotic and antiplatelet therapy does not protect APS patients from the development of recurrent thrombosis. Therefore, an urgent need for the introduction of new therapeutic approaches in the treatment of APS patients is obvious. This review provides a rationale for the necessity for the use of immunomodulatory medications that could interfere with β2gpI binding to its receptor(s), such as anxA2, and/or inhibit TNF-alpha activity.

  12. In vitro reproduction of device deformation leading to thrombotic complications and failure of flow diversion.

    Science.gov (United States)

    Estrade, Laurent; Makoyeva, Alina; Darsaut, Tim E; Ghostine, Jimmy; Kouznetsov, Evgueni; Salazkin, Igor; Roy, Daniel; Weill, Alain; Raymond, Jean

    2013-12-01

    Flow diverters (FDs) are increasingly used for complex intracranial aneurysms. As these self-expanding devices are deployed across an aneurysm neck, they can undergo deformations. The potential clinical consequences of FD deformations remain unclear. We describe an immediate thrombotic complication attributed to a stereotypical stenotic deformation of an FD extremity that can occur when landing zones are of insufficient length. This case is supplemented with in vitro studies showing the relationship between i) the length of the landing zones and ii) discrepancies between the diameter of the device and recipient vessel, and the severity of FD stenosis. In vitro, a shorter landing zone was associated with a progressive stenotic deformation of the terminal ends of all FDs studied. This deformation was more pronounced when the diameter of the device was oversized compared to the size of the recipient tube. In our clinical case, the presence of this deformation led to an immediate thrombotic complication, requiring deployment of a second stent to correct the observed stenosis. In addition, treatment failure ultimately led to a fatal rupture, a failure that can be explained by residual flows through a more porous transition zone, another characteristic FD deformation which occurs when they are oversized as compared to the parent vessel, but free to expand at the level of the aneurysm. Proper selection of device diameter and length of the landing zone is important, and may decrease the incidence of deformation-related complications.

  13. [Risk assessment of thrombotic events after the use of activated factor VII].

    Science.gov (United States)

    Martínez López, M C; Alcaraz Romero, A J; Martínez López, A B; Fernández-Llamazares, C M; Ramos Navarro, C

    2013-09-01

    The objective of this study was to analyze the incidence of thrombotic complications related to recombinant human factor viia (rFVIIa) therapy for severe postoperative bleeding in cardiac surgery. A retrospective matched case-control study was conducted over two years, including 72 children admitted to intensive care unit and treated with rFVIIa because of a severe bleeding during or after cardiac surgery. A control group of 63 patients was chosen, who were statistically comparable in sex, weight, diagnosis, surgical risk according RASCH-1 score, and surgical characteristics, was chosen. There were no significant differences between cases and controls either in the rate of thrombosis (20% vs 28%, P=.540), or in the mortality rate (16% vs 9.5%, P=.208). In our study, the rFVIIa therapy was shown to be useful in controlling severe operative bleeding in pediatric cardiac surgery, but does not seem to increase the risk of thrombotic complications or mortality rate in the postoperative period. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  14. Bilateral Renal Mass-Renal Disorder: Tuberculosis

    Directory of Open Access Journals (Sweden)

    Ozlem Tiryaki

    2013-01-01

    Full Text Available A 30-year-old woman has presented complaining of weakness and fatigue to her primary care physician. The renal sonography is a routine step in the evaluation of new onset renal failure. When the renal masses have been discovered by sonography in this setting, the functional imaging may be critical. We reported a case about bilateral renal masses in a young female patient with tuberculosis and renal insufficiency. Magnetic resonance (MR has revealed the bilateral renal masses in patient, and this patient has been referred to our hospital for further management. The patient’s past medical and surgical history was unremarkable.

  15. Anti-jugular vein thrombotic effect of Morinda citrifolia L. [noni] in male SD rats

    Directory of Open Access Journals (Sweden)

    Mian-Ying Wang

    2011-09-01

    Full Text Available Background: Venous thromboembolism (VTE is a common and serious medical condition, which is estimably responsible for more than 300,000 hospital admissions annually in the USA. Pulmonary embolism (PE is a major complication of VTE, which contributes to 12% death of hospitalized patients. Heparin is the most common anti-coagulant, but severe allergic reactions, bleeding, and thrombocytopenia limit its use. Thus, seeking a botanical, nontoxic antithrombotic alternative is an interesting area. Morinda citrifolia L. [noni] is a medicinal plant used in folk remedies by Polynesians for over 2,000 years. It has been reported to have a broad range of therapeutic and preventive effects. The bioactivities of NJ have been continuously discovered with antioxidative, anti-inflammatory, analgesic, and immune modifying activities. Our novel hypothesis is whether NJ has an anti-venous thrombotic effect in rodents. To examine our hypothesis, this study was designed to examine the anti-thrombotic effect of NJ on the jugular vein thrombosis model induced by ferric chloride in SD rats.Material and Methods: NJ and placebo used in this study were donated by Morinda Holding Inc. NJ was formulated with grape juice and blue berry juice. Placebo was prepared by using the same procedure of NJ preparation, but without NJ in it. Thirty-six male SD rats were divided into six groups. Anti-venous thrombotic activities of 5% NJ, 10% NJ, heparin, and 10% NJ plus heparin were examined and compared with the positive and blank controls. Thrombosis was induced by application of a filter paper soaked in 50% ferric chloride on the right jugular vein. AFunctional Foods in Health and Disease 2011; 9:297-3092-cm fragment of the occluded vein (thrombus was removed and weighed after 1-hour maturation. Blood samples were collected for platelet count, aPTT, and PT tests.Results: The weight of a 2-cm fragment of normal jugular vein was 9.9 ± 2.1 mg, while the weight of the occluded vein in

  16. Distal renal tubular acidosis

    Science.gov (United States)

    Renal tubular acidosis - distal; Renal tubular acidosis type I; Type I RTA; RTA - distal; Classical RTA ... excreting it into the urine. Distal renal tubular acidosis (Type I RTA) is caused by a defect ...

  17. Proximal renal tubular acidosis

    Science.gov (United States)

    Renal tubular acidosis - proximal; Type II RTA; RTA - proximal; Renal tubular acidosis type II ... by alkaline substances, mainly bicarbonate. Proximal renal tubular acidosis (Type II RTA) occurs when bicarbonate is not ...

  18. Renal tuberculosis

    Directory of Open Access Journals (Sweden)

    Džamić Zoran

    2016-01-01

    Full Text Available Tuberculosis is still a significant health problem in the world, mostly in developing countries. The special significance lies in immunocompromised patients, particularly those suffering from the HIV. Urogenital tuberculosis is one of the most common forms of extrapulmonary tuberculosis, while the most commonly involved organ is the kidney. Renal tuberculosis occurs by hematogenous dissemination of mycobacterium tuberculosis from a primary tuberculosis foci in the body. Tuberculosis is characterized by the formation of pathognomonic lesions in the tissues - granulomata. These granulomata may heal spontaneously or remain stable for years. In certain circumstances in the body associated with immunosuppression, the disease may be activated. Central caseous necrosis occurs within tuberculoma, leading to formation of cavities that destroy renal parenchyma. The process may gain access to the collecting system, forming the caverns. In this way, infection can be spread distally to renal pelvis, ureter and bladder. Scaring of tissue by tuberculosis process may lead to development of strictures of the urinary tract. The clinical manifestations are presented by nonspecific symptoms and signs, so tuberculosis can often be overlooked. Sterile pyuria is characteristic for urinary tuberculosis. Dysuric complaints, flank pain or hematuria may be presented in patients. Constitutional symptoms of fever, weight loss and night sweats are presented in some severe cases. Diagnosis is made by isolation of mycobacterium tuberculosis in urine samples, by cultures carried out on standard solid media optimized for mycobacterial growth. Different imaging studies are used in diagnostics - IVU, CT and NMR are the most important. Medical therapy is the main modality of tuberculosis treatment. The first line anti-tuberculosis drugs include isoniazid, rifampicin, pyrazinamide and ethambutol. Surgical treatment is required in some cases, to remove severely damaged kidney, if

  19. Renale Osteopathie

    Directory of Open Access Journals (Sweden)

    Horn S

    2001-01-01

    Full Text Available Die renale Osteopathie umfaßt Erkrankungen des Knochens, die bei Patienten mit chronischen Nierenerkrankungen auftreten, wie den sekundären bzw. tertiären Hyperparathyreoidismus, die adynamische Knochenerkrankung und die Osteopathie nach Nierentransplantation. Durch die Identifikation des Kalzium-Sensing-Rezeptors bzw. des Vitamin D-Rezeptors hat sich unser Verständnis der Zusammenhänge in den letzten Jahren erheblich verbessert. Neue Medikamente versprechen effizientere Prophylaxe- und Therapiemöglichkeiten. Wir beeinflussen dadurch nicht nur die Morbidität und Lebensqualität, sondern auch die Mortalität unserer Patienten.

  20. Renal disease in pregnancy.

    Science.gov (United States)

    Thorsen, Martha S; Poole, Judith H

    2002-03-01

    Anatomic and physiologic adaptations within the renal system during pregnancy are significant. Alterations are seen in renal blood flow and glomerular filtration, resulting in changes in normal renal laboratory values. When these normal renal adaptations are coupled with pregnancy-induced complications or preexisting renal dysfunction, the woman may demonstrate a reduction of renal function leading to an increased risk of perinatal morbidity and mortality. This article will review normal pregnancy adaptations of the renal system and discuss common pregnancy-related renal complications.

  1. Renal cortical infarction following treatment with sumatriptan in a kidney allograft recipient.

    Science.gov (United States)

    Sharma, Shree G; Post, Jarrod B; Herlitz, Leal C; Markowitz, Glen

    2013-02-01

    Renal cortical infarction is a rare cause of acute kidney injury that results from inadequate blood flow to the kidney, most commonly as a consequence of thrombotic or embolic occlusion of the renal artery or profound hypoperfusion. We report the case of a 78-year-old female kidney transplant recipient who developed a migraine headache, took sumatriptan, and soon after developed pain over the allograft and oligoanuric acute kidney injury. Kidney allograft biopsy showed renal cortical infarction. The mechanism of action of sumatriptan involves vasoconstriction, which counters the vasodilatation that is central to the pathogenesis of migraines. This case raises important questions regarding the safety of triptans with calcineurin inhibitors (which also act to vasoconstrict), particularly in elderly patients.

  2. Scleroderma renal crisis in a case of mixed connective tissue disease

    Directory of Open Access Journals (Sweden)

    Mukul Vij

    2014-01-01

    Full Text Available Mixed connective tissue disease (MCTD is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma and polymyositis. Scleroderma renal crisis (SRC is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

  3. Renal calculus

    CERN Document Server

    Pyrah, Leslie N

    1979-01-01

    Stone in the urinary tract has fascinated the medical profession from the earliest times and has played an important part in the development of surgery. The earliest major planned operations were for the removal of vesical calculus; renal and ureteric calculi provided the first stimulus for the radiological investigation of the viscera, and the biochemical investigation of the causes of calculus formation has been the training ground for surgeons interested in metabolic disorders. It is therefore no surprise that stone has been the subject of a number of monographs by eminent urologists, but the rapid development of knowledge has made it possible for each one of these authors to produce something new. There is still a technical challenge to the surgeon in the removal of renal calculi, and on this topic we are always glad to have the advice of a master craftsman; but inevitably much of the interest centres on the elucidation of the causes of stone formation and its prevention. Professor Pyrah has had a long an...

  4. Rituximab in the treatment of patients with systemic Iupus erythematosus-associated thrombotic thrombocytopenic purpura with normaI ADAMTS-13 activity:three cases and Iiterature review%利妥昔单抗治疗ADAMTS-13活性正常系统性红斑狼疮相关血栓性血小板减少性紫癜三例并文献复习

    Institute of Scientific and Technical Information of China (English)

    李嗣钊; 卢昕; 周丽静; 陈亚宁; 尚健; 姚合斌

    2016-01-01

    biopsy which revealed typeⅡ lupus nephritis (LN) and typeⅣ andⅤ LN with renal thrombotic microangiopathy respectively. All 3 patients received methylprednisolone pulse and intravenous immunoglobulin or in combination with mycophenolate mofetil therapy before SLE-associated TTP was diagnosed, however they got worse. As the diagnosis was considered, 2 patients received plasma exchange (PE) combined with glucocorticoids and/or mycophenolate mofetil (MMF), but there were still no improvement. Then 3 patients were rescued by RTX therapy for 2 to 4 episode, after that they achieved complete remission. With mean follow-up of 14 months they showed a sustained remission. 1 patient died from severe pneumonia at 6 months of follow-up. From literature review, a total of 6 cases including our cas suffered from SLE-associated TTP with normal ADAMTS-13 activity, some of them were treated by glucocorticoids combined with PE and/or immunosuppressive failure, then switched to RTX therapy, some of them received RTX in combination with glucocorticoids and PE as an initial treatment, they all got complete remission.ConcIusion SLE-associated TTP should be considered when SLE complicated with fever, anemia accompanied with thrombocytopenia and high LDH level. Increased peripheral broken red cells count and renal biopsy are necessary for the diagnosis of this disease. Patients with SLE-associated TTP with normal ADAMTS-13 activity usually perform high disease activity, severe nervous system and kidney damage. RTX combined with glucocorticoids and/or PE and immunosuppressive may significantly improve effectiveness of treatment and the prognosis of these patients.%目的:探讨利妥昔单抗(RTX)治疗血管性血友病因子裂解蛋白酶(ADAMTS-13)活性正常的系统性红斑狼疮(SLE)相关血栓性血小板减少性紫癜(TTP)的疗效。方法分析中国人民解放军海军总医院内分泌风湿免疫科先后应用 RTX 治疗3例ADAMTS-13

  5. Adhesion of thrombotic components to the surface of a clinically used oxygenator is not affected by Trillium coating

    NARCIS (Netherlands)

    van den Goor, J.M.; van Oeveren, W.; Rutten, P.M.; Tijssen, J.G.; Eijsman, L.

    2006-01-01

    The Trillium (R) coating is designed to minimize adsorption of protein and the attachment of cells and other particles. The present study was undertaken to investigate the effect of surface coating on the adhesion of thrombotic components (activated platelets, white blood cells and fibrin) to the su

  6. Opportunities for improvement in anti-thrombotic therapy and other strategies for the management of acute coronary syndromes

    DEFF Research Database (Denmark)

    Bueno, Héctor; Sinnaeve, Peter; Annemans, Lieven

    2016-01-01

    .4%); 1705 (16.1%) had triple anti-platelet therapy, and 849 (8%) single anti-platelet therapy. STEMI patients more often received pre-hospital anti-thrombotics, and prasugrel, GP IIb/IIIa inhibitors and UFH in-hospital (all p

  7. Adhesion of thrombotic components to the surface of a clinically used oxygenator is not affected by Trillium coating

    NARCIS (Netherlands)

    van den Goor, J.M.; van Oeveren, W.; Rutten, P.M.; Tijssen, J.G.; Eijsman, L.

    2006-01-01

    The Trillium (R) coating is designed to minimize adsorption of protein and the attachment of cells and other particles. The present study was undertaken to investigate the effect of surface coating on the adhesion of thrombotic components (activated platelets, white blood cells and fibrin) to the

  8. CD8+DR+ T-Cells and C3 Complement Serum Concentration as Potential Biomarkers in Thrombotic Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Elizabeth Sarmiento

    2014-01-01

    Full Text Available Purpose. To assess complement factors and T lymphocyte activation subset abnormalities in patients with thrombotic antiphospholipid syndrome (APS as potential biomarkers for development of clinical complications. Methods. We assessed C3, C4, factor B concentrations (nephelometry, complement haemolytic functional activity (CH100, radial immune diffusion, and the activation status of CD4+ and CD8+ T-cells (three-colour flow cytometry in patients with thrombotic APS. Antiphospholipid (aPL positive patients without APS-related clinical criteria, systemic lupus erythematosus (SLE patients, and healthy individuals were evaluated as controls. A clinical followup was performed to assess the potential relationship between the immunological parameters and development of APS-related complications. Results. Lower concentrations of C3 and higher levels of CD8+DR+ cells were risk factors for development of APS-related complications during followup, including rethrombosis and neuropsychiatric symptoms. Patients with diagnosed thrombotic APS had significantly lower levels of C3, C4, and CH100 as well as higher percentages of activated CD4+DR+ and of CD8+DR+ T-cells than healthy controls but similar to that observed in autoimmune disease controls. Conclusion. Lower C3 and C4 complement levels and higher percentages of CD8+DR+ T-cells were observed in thrombotic APS patients. The potential role of these abnormalities as biomarkers of clinical outcome warrants further evaluation in a multicenter study.

  9. Renal actinomycosis with concomitant renal vein thrombosis.

    Science.gov (United States)

    Chang, Dong-Suk; Jang, Won Ik; Jung, Ji Yoon; Chung, Sarah; Choi, Dae Eun; Na, Ki-Ryang; Lee, Kang Wook; Shin, Yong-Tai

    2012-02-01

    Renal actinomycosis is a rare infection caused by fungi of the genus Actinomyces. A 74-year-old male was admitted to our hospital because of gross hematuria with urinary symptoms and intermittent chills. Computed tomography of the abdomen showed thrombosis in the left renal vein and diffuse, heterogeneous enlargement of the left kidney. After nephrectomy, sulfur granules with chronic suppurative inflammation were seen microscopically, and the histopathological diagnosis was renal actinomycosis. Our case is the first report of renal actinomycosis with renal vein thrombosis.

  10. Thrombocytosis: Diagnostic Evaluation, Thrombotic Risk Stratification, and Risk-Based Management Strategies

    Directory of Open Access Journals (Sweden)

    Jonathan S. Bleeker

    2011-01-01

    Full Text Available Thrombocytosis is a commonly encountered clinical scenario, with a large proportion of cases discovered incidentally. The differential diagnosis for thrombocytosis is broad and the diagnostic process can be challenging. Thrombocytosis can be spurious, attributed to a reactive process or due to clonal disorder. This distinction is important as it carries implications for evaluation, prognosis, and treatment. Clonal thrombocytosis associated with the myeloproliferative neoplasms, especially essential thrombocythemia and polycythemia vera, carries a unique prognostic profile, with a markedly increased risk of thrombosis. This risk is the driving factor behind treatment strategies in these disorders. Clinical trials utilizing targeted therapies in thrombocytosis are ongoing with new therapeutic targets waiting to be explored. This paper will outline the mechanisms underlying thrombocytosis, the diagnostic evaluation of thrombocytosis, complications of thrombocytosis with a special focus on thrombotic risk as well as treatment options for clonal processes leading to thrombocytosis, including essential thrombocythemia and polycythemia vera.

  11. Thrombotic thrombocytopenic purpura secondary to ABO group incompatible blood transfusion in a patient after cardiac surgery

    Directory of Open Access Journals (Sweden)

    Yalcin Solak

    2013-01-01

    Full Text Available The triggers of secondary thrombotic thrombopcytopenic purpura (TTP include drug toxicity, radiation and high-dose chemotherapy, angioinvasive infections, surgery and acute graft versus host disease. TTP secondary to surgery have been reported in a number of cases. Most of the cases have been occurred after open heart surgery. Extensive endothelial damage is held responsible as the initiating mechanism in postoperative TTP cases. However, there is no report of secondary TTP describing development owing to ABO incompatible blood transfusion. Here, we describe a patient who developed TTP after transfusion of ABO incompatible blood during hospitalization for bypass surgery. We also propose a hypothesis which may account for the possible underlying mechanism.

  12. Venous and arterial thrombotic risks with thalidomide: evidence and practical guidance.

    Science.gov (United States)

    Palumbo, Antonio; Palladino, Carmela

    2012-10-01

    Oral immunomodulatory drugs (IMiDs), namely thalidomide, lenalidomide and pomalidomide, interfere with several pathways important for disease progression. Today they play a crucial role in the treatment of multiple myeloma patients, and have considerably improved myeloma outcomes. These agents, and thalidomide in particular, are associated with higher rates of thromboembolic events, both venous and arterial. Individual risk factors for thromboembolic events include advanced age, previous history of thromboembolism, an indwelling central venous catheter, comorbid conditions (e.g. infections, diabetes, cardiac disease, obesity), current or recent immobilization, recent surgery and inherited thrombophilic abnormalities. Cancer therapy and cancer itself also increase the risk of thromboembolic events. The aim of this review is to help clinicians to define the risk of thrombotic events in patients treated with thalidomide and thus to provide practical recommendations to manage thromboprophylaxis in these patients.

  13. Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Niaz Faraz

    2010-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued re-mission after eight months of follow-up. Rituximab appears to be an effective treatment in re-fractory cases of TTP associated with SLE.

  14. Thrombocytosis: Diagnostic Evaluation, Thrombotic Risk Stratification, and Risk-Based Management Strategies

    Science.gov (United States)

    Bleeker, Jonathan S.; Hogan, William J.

    2011-01-01

    Thrombocytosis is a commonly encountered clinical scenario, with a large proportion of cases discovered incidentally. The differential diagnosis for thrombocytosis is broad and the diagnostic process can be challenging. Thrombocytosis can be spurious, attributed to a reactive process or due to clonal disorder. This distinction is important as it carries implications for evaluation, prognosis, and treatment. Clonal thrombocytosis associated with the myeloproliferative neoplasms, especially essential thrombocythemia and polycythemia vera, carries a unique prognostic profile, with a markedly increased risk of thrombosis. This risk is the driving factor behind treatment strategies in these disorders. Clinical trials utilizing targeted therapies in thrombocytosis are ongoing with new therapeutic targets waiting to be explored. This paper will outline the mechanisms underlying thrombocytosis, the diagnostic evaluation of thrombocytosis, complications of thrombocytosis with a special focus on thrombotic risk as well as treatment options for clonal processes leading to thrombocytosis, including essential thrombocythemia and polycythemia vera. PMID:22084665

  15. TRANSPLANTE RENAL

    Directory of Open Access Journals (Sweden)

    Soraia Geraldo Rozza Lopes

    2014-01-01

    Full Text Available El objetivo del estudio fue comprender el significado de espera del trasplante renal para las mujeres en hemodiálisis. Se trata de un estudio cualitativo-interpretativo, realizado con 12 mujeres en hemodiálisis en Florianópolis. Los datos fueron recolectados a través de entrevistas en profundidad en el domicilio. Fue utilizado el software Etnografh 6.0 para la pre-codificación y posterior al análisis interpretativo emergieron dos categorías: “las sombras del momento actual”, que mostró que las dificultades iniciales de la enfermedad están presentes, pero las mujeres pueden hacer frente mejor a la enfermedad y el tratamiento. La segunda categoría, “la luz del trasplante renal”, muestra la esperanza impulsada por la entrada en la lista de espera para un trasplante.

  16. Renal failure

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930564 Dwell times affect the local host de-fence mechanism of peritoneal dialysis patients.WANG Tao(汪涛),et al.Renal Instit,SunYatsen Med Univ,Guangzhou,510080.Chin JNephrol 1993;9(2):75—77.The effect of different intraperitoneal awelltimes on the local host defence in 6 peritonealdialysis patients was studied.A significant de-crease in the number of peritoneal cells,IgG con-centration and the phagoeytosis and bactericidalactivity of macrophages was determined when thedwell time decreased from 12 to 4 hs or form 4 to0.5hs,but the peroxidase activity in macrophagesincreased significantly.All variables,except theperoxidase activity in macrophages,showed nosignificant difference between patients of high or

  17. Traumatismo renal

    OpenAIRE

    Rocha, Sofia Rosa Moura Gomes da

    2009-01-01

    Introdução: A realização deste trabalho visa a elaboração de uma revisão sistematizada subordinada à temática da traumatologia renal. Objectivos: Os principais objectivos deste trabalho são: apurar a etiologia, definir a classificação, analisar o diagnóstico e expôr o tratamento e as complicações. Desenvolvimento: Os traumatismos são a principal causa de morte antes dos 40 anos. O rim é o órgão do aparelho génito-urinário mais frequentemente atingido. Os traumatismos renais são mais fre...

  18. In vitro thrombotic tendency of reactive thrombocytosis in critically ill patients: a prospective case-control study.

    Science.gov (United States)

    Duff, O C; Ho, K M; Maybury, S M

    2012-05-01

    It is uncertain whether reactive thrombocytosis is associated with an increased risk of thrombosis. This prospective case-control study assessed the in vitro thrombotic tendency of patients with reactive thrombocytosis. Forty-eight patients with reactive thrombocytosis, defined by platelet count >500x10(9)/l and 55 similar, randomly selected critically ill patients who did not have reactive thrombocytosis were considered. In vitro thrombotic tendency in both groups of patients was assessed using maximal amplitude (normal range 54 to 72 mm) and alpha angle (normal range 47 to 74°) on the thromboelastograph. The associations between reactive thrombocytosis and C-reactive protein, the coagulation profile and Sequential Organ Failure Assessment score were also evaluated. Patients with reactive thrombocytosis had an associated increased in vitro thrombotic tendency (maximal amplitude 77 vs 69 mm, mean difference 8 mm, 95% confidence interval 4.9 to 10.9, P=0.001), a higher fibrinogen concentration (7.2 vs 5.8 g/l, P=0.003), and a higher incidence of infection requiring antibiotics (50 vs 27%, P=0.025) compared to patients without thrombocytosis. Platelet count had a relatively linear relationship with the maximal amplitude and the alpha angle of the thromboelastograph tracing (Pearson correlation coefficient: 0.53, P=0.001). In the multivariate analysis, only reactive thrombocytosis (odds ratio 5.9, 95% confidence interval 1.3-27.8, P=0.025) and activated partial thromboplastin time (odds ratio 0.93 per second increment, 95% confidence interval 0.87 to 0.99, P=0.016) were significantly associated with a strong in vitro thrombotic tendency. In summary, reactive thrombocytosis was associated with infection requiring antibiotics and evidence of increased in vitro thrombotic tendency in critically ill patients.

  19. Association between thrombotic risk factors and extent of fibrosis in patients with non-alcoholic fatty liver diseases

    Institute of Scientific and Technical Information of China (English)

    N Assy; I Bekirov; Y Mejritsky; L Solomon; S Szvalb; O Hussein

    2005-01-01

    AIM: To evaluate the prevalence of genetic and acquired prothrombotic risk factors and their association with the extent of fibrosis and fatty infiltration in patients with non-alcoholic fatty liver disease (NAFLD).METHODS: Forty-four patients with chronic hepatitis (28 men and 16 women, with mean age of 45±11 and 49±12 years, respectively) constituted the patient population of this study. The groups were divided as follows: 15 patients with fatty liver (FL); 15 with non-alcoholic steatohepatitis (NASH); 14 with chronic viral hepatitis (CH) diagnosed by histology and liver technetium scan or ultrasound; and 10 healthy individuals. Thrombophilic, coagulation factors and genetic mutations were diagnosed by standard hemostatic and molecular coagulation assays.RESULTS: Activated protein C (APC) resistance and protein S were the most prevalent thrombotic risk factors (6% and 10% in NAFLD vs 21% and 14% in CH; P<0.01 and P<0.05, respectively). One thrombotic risk factor was identified in 41% of patients (23% mild fibrosis, 18% severe fibrosis) and two thrombotic risk factors in 6% of patients with NAFLD and severe fibrosis. While no differences in APC ratio, lupus anticoagulant, fibrinogen, factor V Leiden,prothrombin, and MTHFR mutation were found. Protein S levels were significantly lower in NASH patients than in patients with FL alone (92±19 vs106±2, P<0.01). Protein C levels were markedly higher in patients with NAFLD and mild or severe fibrosis as compared to the patients with CH, respectively (128±40 vs96±14, P<0.001 or 129±36 vs 88±13, P<0.01).CONCLUSION: Up to 46% of patients with NAFLD may have thrombotic risk factors, and the presence of thrombotic risk factors is correlated with the extent of hepatic fibrosis,suggesting a crucial role of the coagulation system in the pathogenesis of hepatic fibrosis.

  20. Depression Following Thrombotic Cardiovascular Events in Elderly Medicare Beneficiaries: Risk of Morbidity and Mortality

    Directory of Open Access Journals (Sweden)

    Christopher M. Blanchette

    2009-01-01

    Full Text Available Purpose. Depression and antidepressant use may independently increase the risk of acute myocardial infarction and mortality in adults. However, no studies have looked at the effect of depression on a broader thrombotic event outcome, assessed antidepressant use, or evaluated elderly adults. Methods. A cohort of 7,051 community-dwelling elderly beneficiaries who experienced a thrombotic cardiovascular event (TCE were pooled from the 1997 to 2002 Medicare Current Beneficiary Survey and followed for 12 months. Baseline characteristics, antidepressant utilization, and death were ascertained from the survey, while indexed TCE, recurrent TCE, and depression (within 6 months of indexed TCE were taken from ICD-9 codes on Medicare claims. Time to death and first recurrent TCE were assessed using descriptive and multivariate statistics. Results. Of the elders with a depression claim, 71.6% had a recurrent TCE and 4.7% died within 12 months of their indexed TCE, compared to 67.6% and 3.9% of those elders without a depression claim. Of the antidepressant users, 72.6% experienced a recurrent TCE and 3.9% died, compared to 73.7% and 4.6% in the subset of selective serotonin reuptake inhibitor (SSRI users. Depression was associated with a shorter time to death (P=.008 in the unadjusted analysis. However, all adjusted comparisons revealed no effect by depression, antidepressant use, or SSRI use. Conclusions. Depression was not associated with time to death or recurrent TCEs in this study. Antidepressant use, including measures of any antidepressant use and SSRI use, was not associated with shorter time to death or recurrent TCE.

  1. Hereditary Thrombophilia and thrombotic events in pregnancy: single-center experience.

    Science.gov (United States)

    Coriu, L; Ungureanu, R; Talmaci, R; Uscatescu, V; Cirstoiu, M; Coriu, D; Copaciu, E

    2014-01-01

    Pregnancy is a normal physiological state that predisposes to thrombosis, determined by hormonal changes in the body. These changes occur in the blood flow (venous stasis), changes in the vascular wall (hypotonia, endothelial lesion) and changes in the coagulation factors (increased levels of factor VII, factor VIII, factor X, von Willebrand factor) and decreased activity levels of natural anticoagulants (protein C, protein S). In this study, we tried to determine a possible association between thrombosis and inherited thrombophilia in pregnant women. This is a retrospective study of 151 pregnant women with a history of complicated pregnancy: maternal thrombosis and placental vascular pathology (intrauterine growth restriction, preeclampsia, recurrent pregnancy loss), who were admitted in our hospital during the period January 2010 to July 2014. We performed genetic analyses to detect the factor V Leiden mutation, the G20210A mutation in the prothrombin gene, the C677T mutation and the A1298C mutation in methylenetetrahydrofolate reductase (MTHFR) gene. The risk of thrombosis in patients with factor V Leiden is 2.66 times higher than the patients negative for this mutation (OR 2.66 95% CI 0.96-7.37 P=0.059). We did not find any statistical association with mutations in the MTHFR gene. Pregnant women with a family history of thrombosis present a 2.18-fold higher risk of thrombosis (OR 2.18 CI 0.9-5.26 P=0.085). Of 151 pregnant women, thrombotic events occurred in 24 patients: deep vein thrombosis, pulmonary embolism, cerebral venous sinus thrombosis and ischemic stroke. The occurrence of thrombotic events was identified in the last trimester of pregnancy, but especially postpartum. Thrombosis in pregnancy is a redoubtable complication requiring an excellent cooperation between the obstetrician and anesthesiologist.

  2. Activation of Blood Coagulation in Two Prototypic Autoimmune Skin Diseases: A Possible Link with Thrombotic Risk.

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    Massimo Cugno

    Full Text Available Coagulation activation has been demonstrated in two prototypic autoimmune skin diseases, chronic autoimmune urticaria and bullous pemphigoid, but only the latter is associated with increased thrombotic risk. Two markers of coagulation activation (prothrombin fragment F1+2 and fibrin fragment D-dimer were measured by immunoenzymatic methods in plasma samples from 30 patients with active chronic autoimmune urticaria, positive for autologous serum skin test, 30 patients with active bullous pemphigoid and 30 healthy subjects. In skin biopsies, tissue factor expression was evaluated by both immunohistochemistry and in situ hybridization. F1+2 and D-dimer levels were higher in active chronic autoimmune urticaria (276.5±89.8 pmol/L and 5.56±4.40 nmol/L, respectively than in controls (145.2±38.0 pmol/L and 1.06±0.25 nmol/L; P=0.029 and P=0.011 and were much higher in active bullous pemphigoid (691.7±318.7 pmol/L and 15.24±9.09 nmol/L, respectively (P<0.0001. Tissue factor positivity was evident in skin biopsies of both disorders with higher intensity in bullous pemphigoid. F1+2 and D-dimer, during remission, were markedly reduced in both disorders. These findings support the involvement of coagulation activation in the pathophysiology of both diseases. The strong systemic activation of coagulation in bullous pemphigoid may contribute to increase the thrombotic risk and provides the rationale for clinical trials on anticoagulant treatments in this disease.

  3. Immune recognition of ADAMTS13 in acquired TTP

    NARCIS (Netherlands)

    Verbij, F.C.

    2017-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a severe thrombotic micro-angiopathy presenting with hemolytic anemia and thrombocytopenia that results from an acquired or congenital functional deficiency of the von Willebrand factor cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with

  4. The US color Doppler in acute renal failure.

    Science.gov (United States)

    Nori, G; Granata, A; Leonardi, G; Sicurezza, E; Spata, C

    2004-12-01

    Imaging techniques, especially ultrasonography and Doppler, can give an effective assistance in the differential diagnosis of acute renal failure (ARF). An resistance Index (RI) value >0.75 is reported as optimal in attempting differential diagnosis between acute tubular necrosis (ANT) and prerenal ARF. In hepatorenal syndrome (HRS) RIs is very increased. In some renal vasculitis, as nodose panarteritis (PN), hemolytic-uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), parenchymal perfusion is reduced and RI increased. In lupus nephritis the RI values are correlated with creatinine level and normal RI are considered as a good prognostic tool. In acute primitive or secondary glomerulonephritis (GN), RI value is normal, with diffuse parenchymal hypervascularization. In acute crescentic and proliferative GN and tubulo-interstitial disease, color Doppler (CD) and power Doppler (PD) reveal a decreased renal parenchymal perfusion, which correlates with increased RI values. In acute thrombosis of renal artery, US color Doppler (DUS) reveals either an absence of Doppler signal or a tardus-parvus pulse distal to the vascular obstruction. In this situation it is possible to visualize hyperthropic perforating vessels that redirect their flow from the capsular plexus to the renal parenchyma. In acute thrombosis of the renal vein Doppler analysis of parenchymal vessels reveals remarkable RI values, sometimes with reversed diastolic flow. In postrenal ARF an adjunct to the differentiation between obstruction and non obstructive dilatation can be found through RIs. Diagnostic criteria of obstruction as reported by literature are: RI>0.70 in the obstructed kidney and, mostly, a difference in RI between the 2 kidneys >0.06-0.1.

  5. Successful Living-Related Renal Allograft in a Recipient With Factor V Leiden Deficiency: A Case Report.

    Science.gov (United States)

    Florou, Evangelia; Koukoulaki, Maria; Theodoros, Theodoridis; Kalatzis, Vasileios; Vougas, Vasileios; Stamataki, Elissavet; Kokkinou, Vasiliki Christopoulou; Kostakis, Alkiviadis; Drakopoulos, Spiros

    2017-02-01

    Thrombophilia due to activated protein C resistance (Leiden mutation) is the most common inherited thrombophilic disorder with 5% incidence in whites. Renal transplant of these patients entails a risk of vascular thrombosis soon after the transplant; and acute rejection episodes and graft loss within the first year. We present a case of a successful living-related renal transplant in man with a recent history of repeat episodes of vascular access thrombosis attributed to inherited thrombophilia (heterozygosity for factor V mutation Q506 and homozygosity for mutation T677 for methylene-tetrahydrofolate reductase). Transplant recipient was administered anticoagulation therapy with low molecular weight heparin pre- and postoperatively. No thrombotic or hemorrhagic events occurred posttransplant. A high suspicion of thrombophilic disorders in patients with end-stage renal disease with vascular access thrombotic events should be screened further to prevent failure of a subsequent renal transplant. Inherited thrombophilic disorders may not exclude living-related kidney transplant provided that anticoagulation therapy is admin-istered perioperatively.

  6. Shiga toxin-associated hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: distinct mechanisms of pathogenesis.

    Science.gov (United States)

    Tarr, Phillip I

    2009-02-01

    The hemolytic uremic syndrome (HUS) of childhood is, in its most common form, a non-immune microangiopathic hemolytic anemia. HUS typically follows an enteric infection with a Shiga toxin-producing Escherichia coli, usually belonging to serotype O157:H7. The antecedent infection is almost always manifested as non-bloody diarrhea. In about 80% of cases, the diarrhea becomes bloody between one and five days after the onset of diarrhea. The courses of acute gastrointestinal infections, and of HUS, in adults and children are similar. There are no convincing data that this easily recognizable form of microangiopathy is related to any inborn or acquired deficiency of ADAMTS13, and plasma therapies are not justified on either theoretical or empirical grounds. Similarly, there are no realistic animal data to support use of plasma exchange or infusion in children or adults with, or at risk for, HUS secondary to gastrointestinal infection with E. coli O157:H7. Best clinical practices involve rapid and accurate clinical and microbiological identification of infected patients, volume expansion, and support of the intestinal and extraintestinal complications that can ensue during acute enteric infection and associated HUS. Clinical clues include a sequence of events where the stool becomes bloody after a several-day interval of bloody diarrhea, considerable abdominal pain, five or more stools in the 24 h before presentation, pain on defection, and absence of fever at the time of presentation. Diagnosis should rely primarily on sorbitol MacConkey agar culture. Shiga toxin testing should not be used as the only screen to identify infected patients.

  7. CHARACTERISTICS OF LIPID METABOLISM IN PATIENTS WITH TYPE 1 DIABETES, DEPENDING ON THE AVAILABILITY OF DIABETIC MICROANGIOPATHY AND DIET

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    Yu. G. Samoilova

    2014-01-01

    was performed using frequency histograms. To select a criterion to assess the significance of paired differences check compliance form normal distribution using the criterion χ2, and also controlled the general equality of variances using the F-test Fisher. Given that these conditions are satisfied only part of the empirical distributions of features tested the hypothesis that the general average in all cases was performed using Mann–Whitney U-test for independent variables. Null hypothesis was rejected when p < 0.05.In the study of lipid metabolism in patients with type 1 diabetes, no increase in the level of cholesterol, triglycerides, HDL, LDL and VLDL – cholesterol in patients with a combination of diabetic nephropathy and retinopathy compared with patients with type 1 diabetes without diabetic microangiopathy. Investigation of the nature of power in patients with type 1 diabetes, allowed us to estimate its impact on lipid metabolism.

  8. The atherogenic dyslipidemia ratio [log(TG/HDL-C] is associated with residual vascular risk, beta-cell function loss and microangiopathy in type 2 diabetes females

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    Hermans Michel P

    2012-10-01

    Full Text Available Abstract Background Atherogenic dyslipidemia (AD, defined as low HDL-C plus elevated triglycerides (TG, comorbid to T2DM, increases cardiometabolic risk for CAD even when LDL-C is at target. In T2DM males, AD was shown to correlate with β-cell function loss, yet it is not established whether this applies across gender. Aim To establish the prevalence and severity of AD in T2DM females, and to determine how it relates to cardiometabolic phenotype, glucose homeostasis, micro- and macrovascular complications, and 10-year absolute CV risk (UKPDS Risk Engine. Methods 340 T2DM females were ranked according to quintiles (Q of the continuous variable log(TG/HDL-C, with AD prevalence defined as HDL-C -1 plus TG ≥150 mg.dL-1, and β-cell function assessed with HOMA. Results AD prevalence was 35%; mean HDL-C and TG were 52 (15 and 160 (105 mg.dL-1. AD was significantly related to central fat, metabolic syndrome, sedentarity and skeletal sarcopenia, as well as to hsCRP, fibrinogen, uric acid, cystatin-C, Big ET-1, and 10-year UKPDS CV risk. AD correlated stepwise with lower β-cell function and hyperbolic product, and with accelerated loss of residual insulin secretion, higher HbA1c and prevalent microangiopathy. Conclusions log(TG/HDL-C is a simple means to grade AD and residual macrovascular risk in T2DM females. This ratio associates with major non-LDL cardiometabolic variables and ranks predicted CAD risk. In addition, log(TG/HDL-C identifies worsening glucose homeostasis, poorer glycemic control, and prevalent microangiopathy.

  9. Thicker carotid intima-media thickness and increased plasma VEGF levels suffered by post-acute thrombotic stroke patients

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    Yueniwati Y

    2016-12-01

    Full Text Available Yuyun Yueniwati,1 Ni Komang Darmiastini,1 Eko Arisetijono2 1Radiology Department, Faculty of Medicine, Brawijaya University, Malang, Indonesia; 2Neurology Department, Faculty of Medicine, Brawijaya University, Malang, Indonesia Background and objectives: Atherosclerosis causes reduction of the oxygen supply to structures in the far arterial wall, provoking the release of factors that drive angiogenesis of vasa vasorum, including VEGF. Other studies have revealed the inflammatory response in atherosclerosis and the role of platelet factor 4 (PF4 as an anti-angiogenic chemokine through the inhibition of VEGF. This cross-sectional study aims at measuring the effect of atherosclerosis assessed through carotid intima-media thickness (CIMT against plasma VEGF levels in patients with post-acute thrombotic stroke. Materials and methods: CIMT was assessed sonographically using GE Logiq S6 with 13 MHz frequency linear probe. VEGF-A plasma levels were measured using enzyme-linked immunosorbent assay (ELISA method. Differences among variables were compared statistically. The data were analyzed using Pearson correlation. Results: A total of 25 patients with post-acute thrombotic stroke were identified in days 7 to 90. CIMT thickening was indicated in 88% of patients (1.202 ± 0.312 mm, while an increase in plasma VEGF was identified in all patients (178.28 ± 93.96 ng/mL. There was no significant correlation between CIMT and plasma VEGF levels in patients with post-acute thrombotic stroke (p=0.741. A significant correlation was recognized between CIMT and total cholesterol (p=0.029 and low-density lipoprotein (p=0.018. Conclusion: There were no significant correlations between CIMT and plasma VEGF levels in patients with post-acute thrombotic stroke. However, plasma VEGF increased in patients with thrombotic stroke. CIMT measurement is a promising noninvasive modality to assess the vascular condition of patients with stroke and diabetes, while plasma VEGF

  10. Nonbacterial thrombotic endocarditis with embolic cerebral vascular accidents in a patient with advanced, recurrent clear cell carcinoma of the ovary: A case report.

    Science.gov (United States)

    Liang, Lusha W; Perez, Alexander R; Cangemi, Nicholas A; Young, Robert J; Makker, Vicky

    2016-04-01

    •Nonbacterial thrombotic endocarditis can occur in ovarian clear cell carcinoma.•We report on NBTE-associated embolic cerebrovascular infarcts in advanced OCCC.•Further NBTE-associated embolic events can be prevented with anticoagulant therapy.

  11. Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern

    Science.gov (United States)

    Peralta Roselló, Carmen; Baltar Martín, José María; Castillo Eraso, Lorena; de Álvaro Moreno, Fernando; Martínez Vea, Alberto; Visus-Fernández de Manzanos, María Teresa

    2016-01-01

    In patients with atypical hemolytic uremic syndrome (aHUS), complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every 14 ± 2 days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol. PMID:28025630

  12. Curcuma oil ameliorates insulin resistance & associated thrombotic complications in hamster & rat

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    Vishal Singh

    2015-01-01

    Full Text Available Background & objectives: Curcuma oil (C. oil isolated from turmeric (Curcuma longa L. has been shown to have neuro-protective, anti-cancer, antioxidant and anti-hyperlipidaemic effects in experimental animal models. However, its effect in insulin resistant animals remains unclear. The present study was carried out to investigate the disease modifying potential and underlying mechanisms of the C. oil in animal models of diet induced insulin resistance and associated thrombotic complications. Methods: Male Golden Syrian hamsters on high fructose diet (HFr for 12 wk were treated orally with vehicle, fenofibrate (30 mg/kg or C. oil (300 mg/kg in the last four weeks. Wistar rats fed HFr for 12 wk were treated orally with C. oil (300 mg/kg in the last two weeks. To examine the protective effect of C. oil, blood glucose, serum insulin, platelet aggregation, thrombosis and inflammatory markers were assessed in these animals. Results: Animals fed with HFr diet for 12 wk demonstrated hyperlipidaemia, hyperglycaemia, hyperinsulinaemia, alteration in insulin sensitivity indices, increased lipid peroxidation, inflammation, endothelial dysfunction, platelet free radical generation, tyrosine phosphorylation, aggregation, adhesion and intravascular thrombosis. Curcuma oil treatment for the last four weeks in hamsters ameliorated HFr-induced hyperlipidaemia, hyperglycaemia, insulin resistance, oxidative stress, inflammation, endothelial dysfunction, platelet activation, and thrombosis. In HFr fed hamsters, the effect of C. oil at 300 mg/kg [ ] was comparable with the standard drug fenofibrate. Curcuma oil treatment in the last two weeks in rats ameliorated HFr-induced hyperglycaemia and hyperinsulinaemia by modulating hepatic expression of sterol regulatory element binding protein 1c (SREBP-1c, peroxisome proliferator-activated receptor-gamma co-activator 1 (PGC-1α and PGC-1β genes known to be involved in lipid and glucose metabolism. Interpretation

  13. Curcuma oil ameliorates insulin resistance & associated thrombotic complications in hamster & rat.

    Science.gov (United States)

    Singh, Vishal; Jain, Manish; Misra, Ankita; Khanna, Vivek; Prakash, Prem; Malasoni, Richa; Dwivedi, Anil Kumar; Dikshit, Madhu; Barthwal, Manoj Kumar

    2015-06-01

    Curcuma oil (C. oil) isolated from turmeric (Curcuma longa L.) has been shown to have neuro-protective, anti-cancer, antioxidant and anti-hyperlipidaemic effects in experimental animal models. However, its effect in insulin resistant animals remains unclear. The present study was carried out to investigate the disease modifying potential and underlying mechanisms of the C. oil in animal models of diet induced insulin resistance and associated thrombotic complications. Male Golden Syrian hamsters on high fructose diet (HFr) for 12 wk were treated orally with vehicle, fenofibrate (30 mg/kg) or C. oil (300 mg/kg) in the last four weeks. Wistar rats fed HFr for 12 wk were treated orally with C. oil (300 mg/kg) in the last two weeks. To examine the protective effect of C. oil, blood glucose, serum insulin, platelet aggregation, thrombosis and inflammatory markers were assessed in these animals. Animals fed with HFr diet for 12 wk demonstrated hyperlipidaemia, hyperglycaemia, hyperinsulinaemia, alteration in insulin sensitivity indices, increased lipid peroxidation, inflammation, endothelial dysfunction, platelet free radical generation, tyrosine phosphorylation, aggregation, adhesion and intravascular thrombosis. Curcuma oil treatment for the last four weeks in hamsters ameliorated HFr-induced hyperlipidaemia, hyperglycaemia, insulin resistance, oxidative stress, inflammation, endothelial dysfunction, platelet activation, and thrombosis. In HFr fed hamsters, the effect of C. oil at 300 mg/kg [ ] was comparable with the standard drug fenofibrate. Curcuma oil treatment in the last two weeks in rats ameliorated HFr-induced hyperglycaemia and hyperinsulinaemia by modulating hepatic expression of sterol regulatory element binding protein 1c (SREBP-1c), peroxisome proliferator-activated receptor-gamma co-activator 1 (PGC-1)α and PGC-1β genes known to be involved in lipid and glucose metabolism. High fructose feeding to rats and hamsters led to the development of insulin

  14. Retinal artery and vein thrombotic occlusion during pregnancy: markers for familial thrombophilia and adverse pregnancy outcomes

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    Kurtz WS

    2016-05-01

    pregnancy may be a marker for familial or acquired thrombophilia, which confers increased thrombotic risk to the mother and pregnancy, associated with spontaneous abortion or eclampsia. OVO during pregnancy, particularly when coupled with antecedent adverse pregnancy outcomes, should prompt urgent thrombophilia evaluation and institution of thromboprophylaxis to prevent adverse maternal and fetal–placental thrombotic events. Keywords: thrombophilia, ocular thrombosis, retinal vascular occlusion, CRVO, BRAO, pregnancy, miscarriage, fetal loss, ocular vascular occlusion, pre-eclampsia, eclampsia

  15. Potential influences of complement factor H in autoimmune inflammatory and thrombotic disorders.

    Science.gov (United States)

    Ferluga, Janez; Kouser, Lubna; Murugaiah, Valarmathy; Sim, Robert B; Kishore, Uday

    2017-04-01

    Complement system homeostasis is important for host self-protection and anti-microbial immune surveillance, and recent research indicates roles in tissue development and remodelling. Complement also appears to have several points of interaction with the blood coagulation system. Deficiency and altered function due to gene mutations and polymorphisms in complement effectors and regulators, including Factor H, have been associated with familial and sporadic autoimmune inflammatory - thrombotic disorders, in which autoantibodies play a part. These include systemic lupus erythematosus, rheumatoid arthritis, atypical haemolytic uremic syndrome, anti-phospholipid syndrome and age-related macular degeneration. Such diseases are generally complex - multigenic and heterogeneous in their symptoms and predisposition/susceptibility. They usually need to be triggered by vascular trauma, drugs or infection and non-complement genetic factors also play a part. Underlying events seem to include decline in peripheral regulatory T cells, dendritic cell, and B cell tolerance, associated with alterations in lymphoid organ microenvironment. Factor H is an abundant protein, synthesised in many cell types, and its reported binding to many different ligands, even if not of high affinity, may influence a large number of molecular interactions, together with the accepted role of Factor H within the complement system. Factor H is involved in mesenchymal stem cell mediated tolerance and also contributes to self-tolerance by augmenting iC3b production and opsonisation of apoptotic cells for their silent dendritic cell engulfment via complement receptor CR3, which mediates anti-inflammatory-tolerogenic effects in the apoptotic cell context. There may be co-operation with other phagocytic receptors, such as complement C1q receptors, and the Tim glycoprotein family, which specifically bind phosphatidylserine expressed on the apoptotic cell surface. Factor H is able to discriminate between self and

  16. Elevated D-dimers in attacks of hereditary angioedema are not associated with increased thrombotic risk

    Science.gov (United States)

    Reshef, A; Zanichelli, A; Longhurst, H; Relan, A; Hack, C E

    2015-01-01

    Background Recommended management of attacks of hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (C1-INH-HAE) includes therapy with exogenous C1INH. Thrombotic/thromboembolic events (TEE) have been reported with plasma-derived C1INH, but so far none with recombinant human C1INH (rhC1INH). This phase III, randomized, placebo (saline)-controlled study evaluated the safety of rhC1INH 50 IU/kg for the treatment of acute attacks in 74 patients with C1-INH-HAE. Methods Monitoring for TEE and assessment of risk of deep vein thrombosis (DVT) by the Wells prediction rule were performed, and levels of fibrin degradation products (plasma D-dimers) were assessed before study drug administration (baseline), 2 h, and 7 days posttreatment. Results Plasma D-dimer levels were elevated in 80% of the patients (median [25th–75th percentiles]: 2149 [480–5105] μg/l; normal ≤250 μg/l) and were higher in patients with submucosal (abdominal, oropharyngeal–laryngeal) attacks (3095 [890–10000] μg/l; n = 29) compared with subcutaneous (peripheral, facial) attacks (960 [450–4060] μg/l; n = 35). Median plasma D-dimer levels were comparable across treatment groups at baseline (1874 [475–4568] μg/l rhC1INH; 2259 [586–7533] μg/l saline) and 2 h postinfusion (2389 [760–4974] μg/l rhC1INH; 2550 [310–8410] μg/l saline); median plasma D-dimer levels were decreased by Day 7 in both groups (425 [232–3240] μg/l rhC1INH; 418 [246–2318] μg/l saline). No increased risk of DVT was identified, nor any TEE reported in rhC1INH treated or controls. Conclusion Elevated plasma D-dimer levels were associated with acute C1-INH-HAE attacks, particularly with submucosal involvement. However, rhC1INH therapy was not associated with thrombotic events. PMID:25640891

  17. [Characteristic of fatal thrombotic complications based on the materials from the Thanatology Department of Forensic Medicine Bureau of the Rostov region, for 2004-2007].

    Science.gov (United States)

    Berezovskiĭ, D P; Kolkutin, V V; Kovtunov, V V; Kornienko, I V

    2009-01-01

    The authors report statistical data on the spectrum and frequency of fatal thrombotic complications based on the analysis of materials available from the regional bureau of forensic medical examination collected in the period from 2004 to 2007. All lethal cases are categorized in terms of the patients' age, gender, time and clinical characteristics of the injury, time of surgical intervention. Results of the analysis indicate the necessity of examination of fatal cases of thrombotic complications following a standardized protocol.

  18. Eventos trombóticos em dois irmãos com trombofilia Thrombotic events in two siblings with thrombophilia

    Directory of Open Access Journals (Sweden)

    Ricardo Pereira Silva

    2006-12-01

    Full Text Available Apresentaremos à seguir o caso clínico de dois irmãos com diferentes apresentações de fenômenos trombóticos, nos quais foi constatado a mutação da protrombina.We describe the clinical case of two siblings with different presentations of thrombotic phenomena, in which prothrombin mutation was observed.

  19. Kidney (Renal) Failure

    Science.gov (United States)

    ... How is kidney failure treated? What is kidney (renal) failure? The kidneys are designed to maintain proper fluid ... marrow and strengthen the bones. The term kidney (renal) failure describes a situation in which the kidneys have ...

  20. Renal arteries (image)

    Science.gov (United States)

    A renal angiogram is a test used to examine the blood vessels of the kidneys. The test is performed ... main vessel of the pelvis, up to the renal artery that leads into the kidney. Contrast medium ...

  1. The risk of venous thrombosis in individuals with a history of superficial vein thrombosis and acquired venous thrombotic risk factors.

    Science.gov (United States)

    Roach, Rachel E J; Lijfering, Willem M; van Hylckama Vlieg, Astrid; Helmerhorst, Frans M; Rosendaal, Frits R; Cannegieter, Suzanne C

    2013-12-19

    Superficial vein thrombosis (SVT) increases the risk of venous thrombosis fourfold to sixfold. As most individuals with SVT do not develop venous thrombosis, additional risk factors may explain the risk of developing a venous thrombosis. In the Multiple Environmental and Genetic Assessment of risk factors for venous thrombosis study, we assessed the risk of venous thrombosis in individuals with previous SVT and a mild thrombotic risk factor (smoking or overweight/obesity), a strong risk factor (surgery, hospitalization, plaster cast immobilization, or malignancy), or a reproductive factor in women (oral contraception, postmenopausal hormone therapy, or pregnancy/puerperium). Individuals with previous SVT alone had a 5.5-fold (95% confidence interval [CI], 4.4-6.8) increased risk of venous thrombosis. This was 9.3 (95% CI, 7.2-12.1) combined with a mild thrombotic risk factor, 31.4 (95% CI, 14.6-67.5) with a strong risk factor, and 34.9 (95% CI, 19.1-63.8) in women with a reproductive risk factor. The highest separate risk estimates were found for SVT with surgery (42.5; 95% CI, 10.2-177.6), hospitalization (49.8; 95% CI, 11.9-209.2), or oral contraception (43.0; 95% CI, 15.5-119.3 in women). In conclusion, the risk of venous thrombosis is markedly increased in individuals with previous SVT who have an acquired thrombotic risk factor.

  2. Left atrial strain: a new predictor of thrombotic risk and successful electrical cardioversion

    Directory of Open Access Journals (Sweden)

    Cátia Costa

    2016-06-01

    Full Text Available Background: Left atrial deformation (LAD parameters are new markers of atrial structural remodelling that seem to be affected in atrial fibrillation (AF and atrial flutter (AFL. This study aimed to determine whether LAD can identify patients with a higher risk of thrombosis and unsuccessful electrical cardioversion (ECV. Methods: Retrospective study including 56 patients with AF or AFL undergoing ECV, with previous transthoracic (TTE and transoesophageal echocardiography (TEE studies. Echocardiographic parameters analysed were as follows: left ventricle function, left atrium (LA dimensions, LAD parameters (positive and negative strain peaks, left atrial appendage (LAA filling and emptying velocities and the presence of thrombi. Strain values were analysed according to thrombotic risk and success of ECV. Results: Lower mean values of peak-positive strain (PPS in patients with prothrombotic velocities (<25 cm/s and a higher incidence of thrombi in LAA were observed compared with those with normal velocities. Multivariate analysis revealed PPS normalised by LA maximum volume indexed by body surface area (BSA to be associated with prothrombotic risk (odds ratio 0.000 (95% CI: 0.000–0.243, P 0.017, regardless of CHADs2VASC score. Peak-negative strain normalised by LA volumes indexed by BSA were associated with unsuccessful ECV. Conclusions: Atrial deformation parameters identify AF and AFL patients with a high risk of thrombosis and unsuccessful ECV. Therefore, these new parameters should be included in anticoagulation management and rhythm vs rate control strategies.

  3. Gender Differences in Associations Between Intraprocedural Thrombotic Events During Percutaneous Coronary Intervention and Adverse Outcomes

    DEFF Research Database (Denmark)

    Schoos, Mikkel; Mehran, Roxana; Dangas, George D

    2016-01-01

    Women are frequently reported to have increased morbidity after presentation with acute coronary syndromes and myocardial infarction; however, whether a greater thrombotic tendency contributes to gender differences in clinical outcomes of urgent percutaneous coronary intervention is unknown...... quantitative coronary angiography (QCA) analysis, from the ACUITY and HORIZONS-AMI trials. We compared major adverse cardiac events (MACE) at in-hospital, 30-day, and 1-year follow-up and major bleeding at 30 days according to gender and the presence or absence of IPTE. IPTE was identified in 507 patients (7.......7%), with 119 of 1,744 (6.8%) occurring in women and 388 of 4,847 (8.0%) in men (p = 0.12). IPTE, but not gender, was independently associated with MACE at in-hospital and 30-day follow-up. At 1-year follow-up, the adjusted hazard of MACE was higher in women and in patients with IPTE; however, the risk of MACE...

  4. Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.

    Science.gov (United States)

    Caramazza, Domenica; Quintini, Gerlando; Abbene, Ignazio; Malato, Alessandra; Saccullo, Giorgia; Lo Coco, Lucio; Di Trapani, Rosa; Palazzolo, Roberto; Barone, Rita; Mazzola, Giuseppina; Rizzo, Sergio; Ragonese, Paolo; Aridon, Paolo; Abbadessa, Vincenzo; Siragusa, Sergio

    2010-12-01

    Idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare disease responsive to treatment with plasma exchange (PE) but with a high percentage of relapse or refractory patients. A severe deficiency of ADAMTS-13 (<5% of normal activity), congenital or caused by an autoantibody, may be specific for TTP and it has been proposed that severe ADAMTS-13 deficiency now defines TTP. B cells play a key role in both the development and the perpetuation of autoimmunity, suggesting that B-cell depletion could be a valuable treatment approach for patients with idiopathic TTP-HUS. This review of the literature focuses on the role of rituximab, a chimeric monoclonal antibody directed against CD20 antigen expressed by B lymphocytes, in patients with relapsing or refractory TTP-HUS with or without ADAMTS-13 deficiency, suggesting that rituximab may produce clinical remission in a significant proportion of patients. Rituximab therapy reduces plasma requirement and avoids complications related to salvage-immunosuppressive therapy. In conclusion, rituximab provides an effective, well-tolerated, and safe treatment option for patients with idiopathic TTP-HUS, thus giving an alternative approach to the current treatment based on PE.

  5. Histophilus somni-induced thrombotic meningoencephalitis in cattle from northern Paraná, Brazil

    Directory of Open Access Journals (Sweden)

    Selwyn A. Headley

    2015-04-01

    Full Text Available Thrombotic meningoencephalitis (TME is a fatal neurological disease of cattle, predominantly from North America, that is caused by Histophilus somni with sporadic descriptions from other countries. This manuscript describes the occurrence of spontaneous TME in cattle from northern Paraná, Brazil. Most cattle had acute neurological manifestations characteristic of brain dysfunction. Hematological and cerebrospinal fluid analyses were not suggestive of bacterial infections of the brain. Histopathology revealed meningoencephalitis with vasculitis and thrombosis of small vessels that contained discrete neutrophilic and/or lymphocytic infiltrates admixed with fibrin at the brainstem, cerebral cortex, and trigeminal nerve ganglion of all animals. All tissues from the central nervous system used during this study were previously characterized as negative for rabies virus by the direct immunofluorescence assay. PCR and RT-PCR assays investigated the participation of infectious agents associated with bovine neurological disease by targeting specific genes of H. somni, Listeria monocytogenes, bovine herpesvirus -1 and -5, bovine viral diarrhea virus, and ovine herpesvirus-2. PCR and subsequent sequencing resulted in partial fragments of the 16S rRNA gene of H. somni from brain sections of all animals with histopathological diagnosis of TME; all other PCR/RT-PCR assays were negative. These findings confirmed the participation of H. somni in the neuropathological disease observed in these animals, extend the geographical distribution of this disease, and support previous findings of H. somni from Brazil.

  6. Emergency Plasmapheresis in a case of ThromboticThrombocytopenic Purpura (TTP

    Directory of Open Access Journals (Sweden)

    Mariaserena Pioli Di Marco

    2013-12-01

    Full Text Available An 84 year-old female was admitted to our Department of Vascular Internal Medicine after a sudden onset of weakness on her right side and aphasia along with signs of myocardial ischemia from Electrocardiogram (EKG. Clinical and blood exams led to a suspicion of Moschcowitz syndrome, which was reinforced by the presence of numerous schistocytes on a peripheral blood smear.Due to a rapid deterioration of vital signs as well as alertness, the patient underwent an emergency transfusion and plasmapheresis treatment as recommended by American Society of Apheresis (ASFA guidelines: one plasma volume was replaced with fresh frozen plasma (FFP every 24 hours, for the first eight days, in order to reach at least a level of 150,000 platelets/mm3 over three consecutive days accompanied by a decrease in LDH until to 670 UI/l.After this therapy, the clinical picture significantly improved with a complete recovery of consciousness and the disappearance of neurological defects.Examinations to determine the etiology made us hypothesize a secondary status of thrombotic thrombocytopenic purpura due to an autoimmune disorder compatible with Sjogren’s syndrome. The patient was discharged and prescribed prednisone.Currently the patient is in good clinical condition and continues the therapy with prednisone (5 mg/die.

  7. Thrombotic risk assessment in antiphospholipid syndrome: the role of new antibody specificities and thrombin generation assay.

    Science.gov (United States)

    Sciascia, Savino; Baldovino, Simone; Schreiber, Karen; Solfietti, Laura; Radin, Massimo; Cuadrado, Maria J; Menegatti, Elisa; Erkan, Doruk; Roccatello, Dario

    2016-01-01

    Antiphospholipid syndrome (APS) is an autoimmune condition characterized by the presence of antiphospholipid antibodies (aPL) in subjects presenting with thrombosis and/or pregnancy loss. The currently used classification criteria were updated in the international consensus held in Sidney in 2005. Vascular events seem to result of local procoagulative alterations upon triggers influence (the so called "second-hit theory"), while placental thrombosis and complement activation seem to lead to pregnancy morbidity. The laboratory tests suggested by the current classification criteria include lupus anticoagulant, a functional coagulation assay, and anticardiolipin and anti-β2-glycoprotein-I antibodies, generally detected by solid phase enzyme-linked immunosorbent assay. The real challenge for treating physicians is understanding what is the actual weight of aPL in provoking clinical manifestations in each case. As thrombosis has a multi-factorial cause, each patient needs a risk-stratified approach. In this review we discuss the role of thrombotic risk assessment in primary and secondary prevention of venous and arterial thromboembolic disease in patients with APS, focusing on new antibody specificities, available risk scoring models and new coagulation assays.

  8. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

    Science.gov (United States)

    Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

    2016-01-01

    Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all). Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03). Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

  9. Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

    Directory of Open Access Journals (Sweden)

    Suella Martino

    Full Text Available Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP. Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04. Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all. Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03. Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

  10. [Renal leiomyoma. Case report].

    Science.gov (United States)

    Joual, A; Guessous, H; Rabii, R; Benjelloun, M; Benlemlih, A; Skali, K; el Mrini, M; Benjelloun, S

    1999-01-01

    The authors report a case of renal leiomyoma observed in a 56-year-old man. This cyst presented in the from of loin pain. Computed tomography revealed a homogeneous renal tumor. Treatment consisted of radical nephrectomy. Histological examination of the specimen showed benign renal leiomyoma.

  11. Renal inflammatory myofibroblastic tumor

    DEFF Research Database (Denmark)

    Heerwagen, S T; Jensen, C; Bagi, P

    2007-01-01

    Renal inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor of controversial etiology with a potential for local recurrence after incomplete surgical resection. The radiological findings in renal IMT are not well described. We report two cases in adults with a renal mass treated...

  12. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  13. Unusual presentation of aortic dissection: post-coital acute paraplegia with renal failure.

    Science.gov (United States)

    Galabada, Dinith P; Nazar, Abdul L M

    2014-09-01

    We report the case of a 45-year-old chronic smoker who presented with acute paraplegia occurring during coitus and subsequently developed acute renal failure (ARF) requiring dialysis. He had absent peripheral pulses in the lower limbs with evidence of acute ischemia. Doppler study showed dissecting aneurysm of thoracic aorta, thrombotic occlusion of the distal aorta from L1 level up to bifurcation and occlusion of the right renal artery by a thrombus that was confirmed by magnetic resonance imaging of the spine. He was not subjected to any vascular intervention as his lower limbs were not salvageable due to delay in the diagnosis. Post-coital aortic dissection and aortic dissection presenting with acute paraplegia and ARF are very rare. This is probably the first case report with post-coital acute aortic dissection presenting with paraplegia and ARF. This case emphasizes the importance of a careful examination of peripheral pulses in patients presenting with ARF and paraplegia.

  14. Unusual presentation of aortic dissection: Post-coital acute paraplegia with renal failure

    Directory of Open Access Journals (Sweden)

    Dinith P Galabada

    2014-01-01

    Full Text Available We report the case of a 45-year-old chronic smoker who presented with acute paraplegia occurring during coitus and subsequently developed acute renal failure (ARF requiring dialysis. He had absent peripheral pulses in the lower limbs with evidence of acute ischemia. Doppler study showed dissecting aneurysm of thoracic aorta, thrombotic occlusion of the distal aorta from L1 level up to bifurcation and occlusion of the right renal artery by a thrombus that was confirmed by magnetic resonance imaging of the spine. He was not subjected to any vascular intervention as his lower limbs were not salvageable due to delay in the diagnosis. Post-coital aortic dissection and aortic dissection presenting with acute paraplegia and ARF are very rare. This is probably the first case report with post-coital acute aortic dissection presenting with paraplegia and ARF. This case emphasizes the importance of a careful examination of peripheral pulses in patients presenting with ARF and paraplegia.

  15. A retrospective clinical analysis of 16 patients with scleroderma renal crisis%系统性硬化病合并肾危象16例临床分析

    Institute of Scientific and Technical Information of China (English)

    周佳鑫; 徐东; 侯勇; 王迁; 李梦涛; 曾小峰

    2015-01-01

    were limited cutaneous systemic sclerosis (lcSSc).Among SRC patients,16/16 were negative of anticentromere antibodies(ACAs).All these 16 patients had hypertension and renal insufficiency,including 8 dialysis dependent after the onset of SRC and 7 with thrombotic microangiopathy.There were 3 patients receiving renal biopsy.The pathological findings were mainly summarized as intimal thickening and stenosis of renal arterioles.Among 13 patients with long-term followed-up,11 patients received angiotensin converting enzyme inhibitors(ACEI),5 patients died,2 patients were dialysis dependent.Only 1 patient stopped dialysis after the combination treatment of ACEI and endothelin receptor antagonist.Another 5 patients didn't need dialysis.Conclusion SRC usually occurred at the early course of SSc.dcSSc was more frequent than lcSSc.ACAs were rarely found in SRC patients.The immediate and sufficient use of ACEIs was still the cornerstone of SRC treatment.Future studies are needed to evaluate the efficacy of endothelin receptor antagonist in the treatment of SRC.

  16. Postpartum renal vein thrombosis.

    Science.gov (United States)

    Rubens, D; Sterns, R H; Segal, A J

    1985-01-01

    Renal vein thrombosis in adults is usually a complication of the nephrotic syndrome. Rarely, it has been reported in nonnephrotic women postpartum. The thrombosis may be a complication of the hypercoagulable state associated with both the nephrotic syndrome and pregnancy. Two postpartum patients with renal vein thrombosis and no prior history of renal disease are reported here. Neither patient had heavy proteinuria. In both cases, pyelonephritis was suspected clinically and the diagnosis of renal vein thrombosis was first suggested and confirmed by radiologic examination. Renal vein thrombosis should be considered in women presenting postpartum with flank pain.

  17. Renal infarction resulting from traumatic renal artery dissection.

    Science.gov (United States)

    Kang, Kyung Pyo; Lee, Sik; Kim, Won; Jin, Gong Yong; Na, Ki Ryang; Yun, Il Yong; Park, Sung Kwang

    2008-06-01

    Renal artery dissection may be caused by iatrogenic injury, trauma, underlying arterial diseases such as fibromuscular disease, atherosclerotic disease, or connective tissue disease. Radiological imaging may be helpful in detecting renal artery pathology, such as renal artery dissection. For patients with acute, isolated renal artery dissection, surgical treatment, endovascular management, or medical treatment have been considered effective measures to preserve renal function. We report a case of renal infarction that came about as a consequence of renal artery dissection.

  18. Distinct Renal Pathology and a Chemotactic Phenotype after Enterohemorrhagic Escherichia coli Shiga Toxins in Non-Human Primate Models of Hemolytic Uremic Syndrome

    Science.gov (United States)

    Stearns-Kurosawa, Deborah J.; Oh, Sun-Young; Cherla, Rama P.; Lee, Moo-Seung; Tesh, Vernon L.; Papin, James; Henderson, Joel; Kurosawa, Shinichiro

    2014-01-01

    Enterohemorrhagic Escherichia coli cause approximately 1.5 million infections globally with 176,000 cases occurring in the United States annually from ingesting contaminated food, most frequently E. coli O157:H7 in ground beef or fresh produce. In severe cases, the painful prodromal hemorrhagic colitis is complicated by potentially lethal hemolytic uremic syndrome (HUS), particularly in children. Bacterial Shiga-like toxins (Stx1, Stx2) are primarily responsible for HUS and the kidney and neurologic damage that ensue. Small animal models are hampered by the inability to reproduce HUS with thrombotic microangiopathy, hemolytic anemia, and acute kidney injury. Earlier, we showed that nonhuman primates (Papio) recapitulated clinical HUS after Stx challenge and that novel therapeutic intervention rescued the animals. Here, we present detailed light and electron microscopic pathology examination of the kidneys from these Stx studies. Stx1 challenge resulted in more severe glomerular endothelial injury, whereas the glomerular injury after Stx2 also included prominent mesangiolysis and an eosinophilic inflammatory infiltration. Both toxins induced glomerular platelet-rich thrombi, interstitial hemorrhage, and tubular injury. Analysis of kidney and other organs for inflammation biomarkers showed a striking chemotactic profile, with extremely high mRNA levels for IL-8, monocyte chemoattractant protein 1, and macrophage inflammatory protein 1α and elevated urine chemokines at 48 hours after challenge. These observations give unique insight into the pathologic consequences of each toxin in a near human setting and present potential pathways for therapeutic intervention. PMID:23402998

  19. Eculizumab prevents recurrent antiphospholipid antibody syndrome and enables successful renal transplantation.

    Science.gov (United States)

    Lonze, B E; Zachary, A A; Magro, C M; Desai, N M; Orandi, B J; Dagher, N N; Singer, A L; Carter-Monroe, N; Nazarian, S M; Segev, D L; Streiff, M B; Montgomery, R A

    2014-02-01

    Renal transplantation in patients with antiphospholipid antibodies has historically proven challenging due to increased risk for thrombosis and allograft failure. This is especially true for patients with antiphospholipid antibody syndrome (APS) and its rare subtype, the catastrophic antiphospholipid antibody syndrome (CAPS). Since a critical mechanism of thrombosis in APS/CAPS is one mediated by complement activation, we hypothesized that preemptive treatment with the terminal complement inhibitor, eculizumab, would reduce the extent of vascular injury and thrombosis, enabling renal transplantation for patients in whom it would otherwise be contraindicated. Three patients with APS, two with a history of CAPS, were treated with continuous systemic anticoagulation together with eculizumab prior to and following live donor renal transplantation. Two patients were also sensitized to human leukocyte antigens (HLA) and required plasmapheresis for reduction of donor-specific antibodies. After follow-up ranging from 4 months to 4 years, all patients have functioning renal allografts. No systemic thrombotic events or early graft losses were observed. While the appropriate duration of treatment remains to be determined, this case series suggests that complement inhibitors such as eculizumab may prove to be effective in preventing the recurrence of APS after renal transplantation.

  20. One-year follow-up of neuropsychology, MRI, rCBF and glucose metabolism (rMRGlu) in cerebral microangiopathy

    Energy Technology Data Exchange (ETDEWEB)

    Sabri, O.; Hellwig, D.; Schreckenberger, M.; Kaiser, H.-J.; Wagenknecht, G.; Setani, K.; Reinartz, P.; Zimny, M.; Buell, U. [Department of Nuclear Medicine, Technische Univ. Aachen (Germany); Schneider, R. [Department of Neurology, Technische Univ. Aachen (Germany); Mull, M. [Department of Neuroradiology, Technische Univ. Aachen (Germany); Ringelstein, E.-B. [Department of Neurology, Muenster Univ. (Germany)

    2000-07-01

    Background: MRI shows lacunar infarctions (LI), deep white matter lesions (DWML) and atrophy in cerebral microangiopathy, which is said to lead to vascular dementia. In a first trial series on 57 patients with confirmed pure cerebral microangiopathy (without concomitant macroangiopathy), neuropsychological impairment and (where present) brain atrophy correlated with decreased rCBF and rMRGlu. LI and DWML did not correlate with either neuropsychological impairment or decreased rCBF/rMRGlu. This study was done one year later to detect changes in any of the study parameters. Methods: 26 patients were re-examined for rCBF, rMRGlu, LI, DWML, atrophy and neuropsychological performance (7 cognitive, 3 mnestic, 4 attentiveness tests). Using a special head holder for exact repositioning, rCBF (SPECT) and rMRGlu (PET) were measured and imaged slice by slice. White matter/cortex were quantified using MRI-defined ROIs. Results: After one year the patients did not show significant decreases in rCBF or rMRGlu either in cortex or in white matter (p>0.05), nor did any patient show LI, DWML or atrophy changes on MRI. There were no significant neuropsychological decreases (p>0.05). (orig.) [German] Ziel: In der MRT zeigen sich bei zerebraler Mikroangiopathie (ZMA) lakunaere Infarkte (LI), Deep White Matter Lesions (DWML) und Atrophie (Atr). Die sogenannte vaskulaere Demenz wurde dabei hauptsaechlich auf die Laesionen der weissen Substanz zurueckgefuehrt. In einer ersten Untersuchungsreihe waren bei 57 Patienten mit gesicherter ZMA nur neuropsychologische Defizite (Nps) und, falls vorhanden, Atr als Grundlage fuer erniedrigte rCBF/rMRGlu-Werte zu eruieren, jedoch nicht LI/DWML. Es sollte geklaert werden, ob sich im Verlauf der Erkrankung nach einem Jahr Veraenderungen dieser Parameter ergeben. Methode: 26 Patienten wurden nach einem Jahr erneut neuropsychologisch untersucht (7 kognitive, 3 mnestrische, 4 Aufmerksamkeitstests). Mittels eines speziellen Kopfhalterungssystems wurden in

  1. A high-throughput sequencing test for diagnosing inherited bleeding, thrombotic, and platelet disorders

    Science.gov (United States)

    Simeoni, Ilenia; Stephens, Jonathan C.; Hu, Fengyuan; Deevi, Sri V. V.; Megy, Karyn; Bariana, Tadbir K.; Lentaigne, Claire; Schulman, Sol; Sivapalaratnam, Suthesh; Vries, Minka J. A.; Westbury, Sarah K.; Greene, Daniel; Papadia, Sofia; Alessi, Marie-Christine; Attwood, Antony P.; Ballmaier, Matthias; Baynam, Gareth; Bermejo, Emilse; Bertoli, Marta; Bray, Paul F.; Bury, Loredana; Cattaneo, Marco; Collins, Peter; Daugherty, Louise C.; Favier, Rémi; French, Deborah L.; Furie, Bruce; Gattens, Michael; Germeshausen, Manuela; Ghevaert, Cedric; Goodeve, Anne C.; Guerrero, Jose A.; Hampshire, Daniel J.; Hart, Daniel P.; Heemskerk, Johan W. M.; Henskens, Yvonne M. C.; Hill, Marian; Hogg, Nancy; Jolley, Jennifer D.; Kahr, Walter H.; Kelly, Anne M.; Kerr, Ron; Kostadima, Myrto; Kunishima, Shinji; Lambert, Michele P.; Liesner, Ri; López, José A.; Mapeta, Rutendo P.; Mathias, Mary; Millar, Carolyn M.; Nathwani, Amit; Neerman-Arbez, Marguerite; Nurden, Alan T.; Nurden, Paquita; Othman, Maha; Peerlinck, Kathelijne; Perry, David J.; Poudel, Pawan; Reitsma, Pieter; Rondina, Matthew T.; Smethurst, Peter A.; Stevenson, William; Szkotak, Artur; Tuna, Salih; van Geet, Christel; Whitehorn, Deborah; Wilcox, David A.; Zhang, Bin; Revel-Vilk, Shoshana; Gresele, Paolo; Bellissimo, Daniel B.; Penkett, Christopher J.; Laffan, Michael A.; Mumford, Andrew D.; Rendon, Augusto; Freson, Kathleen; Ouwehand, Willem H.; Turro, Ernest

    2016-01-01

    Inherited bleeding, thrombotic, and platelet disorders (BPDs) are diseases that affect ∼300 individuals per million births. With the exception of hemophilia and von Willebrand disease patients, a molecular analysis for patients with a BPD is often unavailable. Many specialized tests are usually required to reach a putative diagnosis and they are typically performed in a step-wise manner to control costs. This approach causes delays and a conclusive molecular diagnosis is often never reached, which can compromise treatment and impede rapid identification of affected relatives. To address this unmet diagnostic need, we designed a high-throughput sequencing platform targeting 63 genes relevant for BPDs. The platform can call single nucleotide variants, short insertions/deletions, and large copy number variants (though not inversions) which are subjected to automated filtering for diagnostic prioritization, resulting in an average of 5.34 candidate variants per individual. We sequenced 159 and 137 samples, respectively, from cases with and without previously known causal variants. Among the latter group, 61 cases had clinical and laboratory phenotypes indicative of a particular molecular etiology, whereas the remainder had an a priori highly uncertain etiology. All previously detected variants were recapitulated and, when the etiology was suspected but unknown or uncertain, a molecular diagnosis was reached in 56 of 61 and only 8 of 76 cases, respectively. The latter category highlights the need for further research into novel causes of BPDs. The ThromboGenomics platform thus provides an affordable DNA-based test to diagnose patients suspected of having a known inherited BPD. PMID:27084890

  2. Experimental reversal of acute coronary thrombotic occlusion and myocardial injury in animals utilizing streptokinase.

    Science.gov (United States)

    Lee, G; Giddens, J; Krieg, P; Dajee, A; Suzuki, M; Kozina, J A; Ikeda, R M; DeMaria, A N; Mason, D T

    1981-12-01

    Fresh autologous thrombus, 1.0 to 1.5 ml, was injected into the left anterior descending and/or left diagonal coronary arteries of 19 open-chest dogs to produce evolving acute myocardial infarction (AMI). Thrombotic obstruction was documented by coronary angiography. Multilead epicardial ECGs showed ST segment elevations of affected left ventricular (LV) areas within 2 minutes after thrombus injection, and LV segmental wall cyanosis with hypocontraction was observed within 10 minutes in the myocardial areas supplied by the thrombosed artery. Ten animals then received an initial dose of streptokinase (STK), 250,000 U (intravenous), followed by STK, 1000 to 3000 U/min (intracoronary), while nine control dogs untreated with STK received normal saline infusion. All but one STK-treated animal (all nine animals receiving intracoronary STK) had reestablishment of blood flow in the previously occluded vessels within 1 1/2 hours, disappearance of ventricular cyanosis, return of normal LV contractile function, and normalization of elevated ST segments within 1 hour after intracoronary STK therapy. In contrast, in the non-STK-treated control group, all animals had continued coronary obstruction, progressive ST elevations, and worsening LV cyanosis and hypocontraction until death or for more than 3 hours post thrombus; three control animals died of ventricular fibrillation (VF) within 1 hour of thrombus occlusion, three more died of VF within 2 hours post thrombus, and only three survived beyond 2 hours post thrombus. Postmortem examination of non-STK-treated animals revealed extensive residual coronary thrombus. All intracoronary STK-treated animals evidenced absence of residual coronary thrombus at postmortem examination. These data provide clinically relevant evidence that early intracoronary STK effects thrombolysis in AMI by reopening coronary vessels occluded by fresh thrombus, thereby protecting myocardium from further ischemia and necrosis, preserving LV function, and

  3. Portal vein thrombosis relevance on liver cirrhosis: Italian Venous Thrombotic Events Registry.

    Science.gov (United States)

    Violi, Francesco; Corazza, Roberto Gino; Caldwell, Stephen Hugh; Perticone, Francesco; Gatta, Angelo; Angelico, Mario; Farcomeni, Alessio; Masotti, Michela; Napoleone, Laura; Vestri, Annarita; Raparelli, Valeria; Basili, Stefania

    2016-12-01

    Portal vein thrombosis may occur in cirrhosis; nevertheless, its prevalence, and predictors are still elusive. To investigate this issue, the Italian Society of Internal Medicine undertook the "Portal vein thrombosis Relevance On Liver cirrhosis: Italian Venous thrombotic Events Registry" (PRO-LIVER). This prospective multicenter study includes consecutive cirrhotic patients undergoing Doppler ultrasound examination of the portal area to evaluate the prevalence and incidence of portal vein thrombosis over a 2-year scheduled follow-up. Seven hundred and fifty-three (68 % men; 64 ± 12 years) patients were included in the present analysis. Fifty percent of the cases were cirrhotic outpatients. Viral (44 %) etiology was predominant. Around half of the patients had a mild-severity disease according to the Child-Pugh score; hepatocellular carcinoma was present in 20 %. The prevalence of ultrasound-detected portal vein thrombosis was 17 % (n = 126); it was asymptomatic in 43 % of the cases. Notably, more than half of the portal vein thrombosis patients (n = 81) were not treated with anticoagulant therapy. Logistic step-forward multivariate analysis demonstrated that previous portal vein thrombosis (p portal vein thrombosis. Portal vein thrombosis is a frequent complication of cirrhosis, particularly in patients with moderate-severe liver failure. The apparent undertreatment of patients with portal vein thrombosis is a matter of concern and debate, which should be addressed by planning interventional trials especially with newer oral anticoagulants. Clinicaltrials.gov identifier NCT01470547.

  4. Altered fibrin clot structure/function in patients with antiphospholipid syndrome: association with thrombotic manifestation.

    Science.gov (United States)

    Celińska-Lowenhoff, M; Iwaniec, T; Padjas, A; Musiał, J; Undas, A

    2014-08-01

    We tested the hypothesis that plasma fibrin clot structure/function is unfavourably altered in patients with antiphospholipid syndrome (APS). Ex vivo plasma clot permeability, turbidity and susceptibility to lysis were determined in 126 consecutive patients with APS enrolled five months or more since thrombotic event vs 105 controls. Patients with both primary and secondary APS were characterised by 11% lower clot permeability (p<0.001), 4.8% shorter lag phase (p<0.001), 10% longer clot lysis time (p<0.001), and 4.7% higher maximum level of D-dimer released from clots (p=0.02) as compared to the controls. Scanning electron microscopy images confirmed denser fibrin networks composed of thinner fibres in APS. Clots from patients with "triple-antibody positivity" were formed after shorter lag phase (p=0.019) and were lysed at a slower rate (p=0.004) than in the remainder. Clots from APS patients who experienced stroke and/or myocardial infarction were 8% less permeable (p=0.01) and susceptible to lysis (10.4% longer clot lysis time [p=0.006] and 4.5% slower release of D-dimer from clots [p=0.01]) compared with those following venous thromboembolism alone. Multivariate analysis adjusted for potential confounders showed that in APS patients, lupus anticoagulant and "triple-positivity" were the independent predictors of clot permeability, while "triple-positivity" predicted lysis time. We conclude that APS is associated with prothrombotic plasma fibrin clot phenotype, with more pronounced abnormalities in arterial thrombosis. Molecular background for this novel prothrombotic mechanism in APS remains to be established.

  5. Refractory anemia leading to renal hemosiderosis and renal failure

    OpenAIRE

    Sujatha Siddappa; K M Mythri; Kowsalya, R.; Ashish Parekh

    2011-01-01

    Renal hemosiderosis is a rare cause of renal failure and, as a result, may not be diagnosed unless a detailed history, careful interpretation of blood parameters and renal biopsy with special staining is done. Here, we present a rare case of renal hemosiderosis presenting with renal failure.

  6. Refractory anemia leading to renal hemosiderosis and renal failure

    Directory of Open Access Journals (Sweden)

    Sujatha Siddappa

    2011-01-01

    Full Text Available Renal hemosiderosis is a rare cause of renal failure and, as a result, may not be diagnosed unless a detailed history, careful interpretation of blood parameters and renal biopsy with special staining is done. Here, we present a rare case of renal hemosiderosis presenting with renal failure.

  7. Renal replacement therapy for acute renal failure.

    Science.gov (United States)

    Macedo, E; Bouchard, J; Mehta, R L

    2009-09-01

    Renal replacement therapy became a common clinical tool to treat patients with severe acute kidney injury (AKI) since the 1960s. During this time dialytic options have expanded considerably; biocompatible membranes, bicarbonate dialysate and dialysis machines with volumetric ultrafiltration control have improved the treatment for acute kidney injury. Along with advances in methods of intermittent hemodialysis, continuous renal replacement therapies have gained widespread acceptance in the treatment of dialysis-requiring AKI. However, many of the fundamental aspects of the renal replacement treatment such as indication, timing of dialytic intervention, and choice of dialysis modality are still controversial and may influence AKI patient's outcomes. This review outlines current concepts in the use of dialysis techniques for AKI and suggests an approach for selecting the optimal method of renal replacement therapy.

  8. Renal function after renal artery stenting

    Institute of Scientific and Technical Information of China (English)

    George S. Hanzel; Mark Downes; Peter A. McCullough

    2005-01-01

    @@ Atherosclerotic renal artery stenosis (ARAS), a common clinical finding, is increasing in prevalence as the population ages. ARAS is seen in ~ 7% of persons over 65 years of age1 and in ~ 20% of patients at the time of coronary angiography.2 It is an important cause of chronic kidney disease and may result in 11-14% of cases of end stage renal disease.3

  9. Diabetic nephropathy and endothelial dysfunction: Current and future therapies, and emerging of vascular imaging for preclinical renal-kinetic study.

    Science.gov (United States)

    Leung, Wilson Kc; Gao, L; Siu, Parco M; Lai, Christopher Wk

    2016-12-01

    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. Stemming from currently proposed endothelium-centered therapeutic strategies for diabetic nephropathy, this review highlighted some most exploited pathways that involve the intricate coordination of vasodilators, vasoconstrictors and vaso-modulatory molecules in the pathogenesis of diabetic nephropathy. We also emphasized the emerging roles of oxidative and epigenetic modifications of microvasculature as our prospective therapeutics for diabetic renal diseases. Finally, this review in particular addressed the potential use of multispectral optoacoustic tomography in real-time, minimally-invasive vascular imaging of small experimental animals for preclinical renal-kinetic drug trials.

  10. Predictors for the development of post-thrombotic syndrome in patients with primary lower limb deep venous thrombosis: A case-control study.

    Science.gov (United States)

    Siddiqui, Nadeem A; Sophie, Ziad; Zafar, Farhan; Soares, Delvene; Naz, Iram

    2017-02-01

    Introduction Post-thrombotic syndrome is a common and debilitating sequelae of lower limb deep venous thrombosis. Very little awareness is present about the risk factors and about the diagnosis, prevention, and treatment of this condition. Objective The objective of this study is to identify the predictors of post-thrombotic syndrome after lower limb deep venous thrombosis. Materials and methods A case-control study was conducted on all adult patients who were admitted with lower limb deep venous thrombosis at our institution from January 2005 to June 2012. These patients were scheduled for a research clinic visit, which included informed consent, data collection, and physical examination. Patients found to have post-thrombotic syndrome served as cases and those without post-thrombotic syndrome served as controls. Villalta scoring system was used to diagnose the post-thrombotic syndrome and then to assess the severity of the condition in both the groups. Cox regression risk factor analysis was performed to identify the predictors of post-thrombotic syndrome. Results Out of the 125 patients examined, 49 were found to have post-thrombotic syndrome. Risk factors found to be significant were body mass index of more than 35 kg/m(2) ( n = 13, p = 0.003), history of immobilization ( n = 19, p = 0.003), one or more hypercoagable disorders ( n = 32, p = 0.02), iliofemoral deep venous thrombosis ( n = 18, p = 0.001), complete obstruction on ultrasound ( n = 26, p = 0.016), unstable range of international normalized ratio ( n = 23, p = 0.041) and non-compliance for the use of compressions stockings ( n = 14, p = 0.001). On multivariate analysis, one or more hypercoagable disorder, iliofemoral deep venous thrombosis, and non-compliance to the use of compression stockings were found to be independent risk factors for the development of post-thrombotic syndrome. Conclusion One or more hypercoagable disorders, iliofemoral

  11. Imaging of renal osteodystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Jevtic, V. E-mail: vladimir.jevtic@mf.uni-lj.si

    2003-05-01

    Chronic renal insufficiency, hemodialysis, peritoneal dialysis, renal transplantation and administration of different medications provoke complex biochemical disturbances of the calcium-phosphate metabolism with wide spectrum of bone and soft tissue abnormalities termed renal osteodystrophy. Clinically most important manifestation of renal bone disease includes secondary hyperparathyroidism, osteomalacia/rickets, osteoporosis, adynamic bone disease and soft tissue calcification. As a complication of long-term hemodialysis and renal transplantation amyloid deposition, destructive spondyloarthropathy, osteonecrosis, and musculoskeletal infections may occur. Due to more sophisticated diagnostic methods and more efficient treatment classical radiographic features of secondary hyperparathyroidism and osteomalacia/rickets are now less frequently seen. Radiological investigations play an important role in early diagnosis and follow-up of the renal bone disease. Although numerous new imaging modalities have been introduced in clinical practice (scintigraphy, CT, MRI, quantitative imaging), plain film radiography, especially fine quality hand radiograph, still represents most widely used examination.

  12. 系统性红斑狼疮合并抗磷脂抗体相关性肾病的肾组织病理学特点%Renal histopathologic characteristics of antiphoslipid antibody-associated nephropathy in patients with Systemic Lupus Erythematosus

    Institute of Scientific and Technical Information of China (English)

    高瑞通; 俞文清; 文煜冰; 李雪梅; 郑法雷

    2011-01-01

    /or fibrocelluar occlusions of arteries and arterioles (7.7%), 3 cases of focal cortical atrophy(l. 9% )and 8 cases of tubular thyroidization(5. 2% ) were showed in all 46/155 (29. 7% ) cases of chronic APLN and APLN - like lesion. Acute APLN and APLN - like lesions ( thrombotic microangiopathy ) were detected in 14 cases (9.0% ). APLN and APLN-like renal histopathologic lesions of were found in 23/37(62.1%) cases in APL positive group and 32/118(27.1%) cases in APL negative group. The incidence of total renal histopathologic lesions (62.1% vs 27.1% ) and its chronic and acute lesions in APL positive group were all significantly higher than in APL negative group by logistic regression ( P < 0. 05). Conclusions High incidence of renal histopathologic lesions of APLN was found in SLE patients. The lesions were high correlated with APL.

  13. Ten years of cerebral venous thrombosis: male gender and myeloproliferative neoplasm is associated with thrombotic recurrence in unprovoked events.

    Science.gov (United States)

    Lim, H Y; Ng, C; Donnan, G; Nandurkar, H; Ho, Prahlad

    2016-10-01

    Cerebral venous thrombosis (CVT) is a rare venous thrombotic event. We review our local experience in the management of CVT in comparison to other venous thromboembolism (VTE) with specific focus on risk factors for thrombotic recurrence. Retrospective evaluation of consecutive CVT presentations from January 2005 to June 2015, at two major tertiary hospitals in Northeast Melbourne, Australia. This population was compared to a separate audit of 1003 consecutive patients with DVT and PE. Fifty-two patients (30 female, 22 male) with a median age of 40 (18-83) years, presented with 53 episodes of CVT. Twenty-nine episodes (55 %) were associated with an underlying risk factor, with hormonal risk factors in females being most common. The median duration of anticoagulation was 6 months with 11 receiving life-long anticoagulation. Eighty-one percent had residual thrombosis on repeat imaging, which was not associated with recurrence at the same or distant site. Nine (17 %) had CVT-related haemorrhagic transformation with two resultant CVT-related deaths (RR 22.5; p = 0.04). All three VTE recurrences occured in males with unprovoked events (RR 18.2; p = 0.05) who were subsequently diagnosed with myeloproliferative neoplasm (MPN). Compared to the non-cancer VTE population, non-cancer CVT patients were younger, had similar rate of provoked events and VTE recurrence, although with significantly higher rate of MPN diagnosis (RR 9.30 (2.29-37.76); p = 0.002) CVT is a rare thrombotic disorder. All recurrences in this audit occurred in male patients with unprovoked events and subsequent diagnosis of MPN, suggesting further evaluation for MPN may be warranted in patients with unprovoked CVT.

  14. Primary Coronary Embolism as an Unusual Manifestation of Nonbacterial Thrombotic Endocarditis in a Patient with Gastric Cancer

    Directory of Open Access Journals (Sweden)

    Giovanni Ferlan

    2010-01-01

    Full Text Available Nonbacterial thrombotic endocarditis (NBTE is a rare clinical condition characterized by the presence of sterile vegetations on valvular leaflets Gross and Friedberg (1936. The most frequent cause of NBTE is antiphospholipid syndrome Hughson and et al. (1993; malignancy, through an intrinsic condition of hypercoagulability, is the second most common cause Thomas (2001. Systemic thromboembolic complications are frequently associated with this condition, but coronary embolism is not common. We report the case of a patient with NBTE secondary to gastric adenocarcinoma with clinical symptoms of coronary and systemic emboli.

  15. Incidental renal neoplasms

    DEFF Research Database (Denmark)

    Rabjerg, Maj; Mikkelsen, Minne Nedergaard; Walter, Steen;

    2014-01-01

    On the basis of associations between tumor size, pathological stage, histological subtype and tumor grade in incidentally detected renal cell carcinoma vs symptomatic renal cell carcinoma, we discussed the need for a screening program of renal cell carcinoma in Denmark. We analyzed a consecutive...... series of 204 patients with renal tumors in 2011 and 2012. The tumors were classified according to detection mode: symptomatic and incidental and compared to pathological parameters. Eighty-nine patients (44%) were symptomatic, 113 (55%) were incidental. Information was not available in two patients...

  16. Insuficiencia renal aguda.

    OpenAIRE

    Carlos Hernán Mejía

    2009-01-01

    Acute renal failure (ARF) is a clinic syndrome characterized by decline in renal function occurring over a short time period. Is a relatively common complication in hospitalized critically ill patients and is associated with high morbidity and mortality. ARF has often a multi-factorial etiology syndrome usually approached diagnostically as pre-renal, post-renal, or intrinsic ARF. Most intrinsic ARF is caused by ischemia or nephrotoxins and is classically associated with acute tubular necrosis...

  17. Isolated Renal Hydatidosis Presenting as Renal Mass: A Diagnostic Dilemma

    Directory of Open Access Journals (Sweden)

    Datteswar Hota

    2015-07-01

    Full Text Available Hydatid disease is a parasitic infestation by larval form of Echinococcus granulosus. Isolated renal involvement is extremely rare. There are no specific signs and symptoms of renal hydatidosis. However it may present as palpable mass, flank pain, hematuria, malaise, fever, and hydatiduria or as a complication of it such as infection, abscess, hemorrhage, necrosis and pelviureteric junction obstruction, renal failure etc. Except hydatiduria, none are pathognomonic for renal hydatidosis. There is no literature on renal hydatidosis presenting as renal mass we report 2 cases of isolated renal hydatidosis, which mimicked a renal mass on imaging study.

  18. Distal renal tubular acidosis in recurrent renal stone formers

    DEFF Research Database (Denmark)

    Osther, P J; Hansen, A B; Røhl, H F

    1989-01-01

    (1.1%) had complete distal renal tubular acidosis and 14 (15.5%) incomplete distal renal tubular acidosis. Our results confirm that distal renal tubular acidification defects are associated with a more severe form of stone disease and make distal renal tubular acidosis one of the most frequent...... metabolic disturbances in renal stone formers. Distal renal tubular acidosis (dRTA) was relatively more common in female stone formers and most often found in patients with bilateral stone disease (36%). Since prophylactic treatment in renal stone formers with renal acidification defects is available...

  19. Renal pelvis or ureter cancer

    Science.gov (United States)

    Transitional cell cancer of the renal pelvis or ureter; Kidney cancer - renal pelvis; Ureter cancer ... Cancer can grow in the urine collection system, but it is uncommon. Renal pelvis and ureter cancers ...

  20. Comparison of cardiovascular thrombotic events in patients with osteoarthritis treated with rofecoxib versus nonselective nonsteroidal anti-inflammatory drugs (ibuprofen, diclofenac, and nabumetone).

    Science.gov (United States)

    Reicin, Alise S; Shapiro, Deborah; Sperling, Rhoda S; Barr, Eliav; Yu, Qinfen

    2002-01-15

    Aspirin, nonselective nonsteroidal anti-inflammatory drugs (NSAIDs), and specific cyclooxygenase-2 (COX-2) inhibitors each have distinctive effects on COX-1-mediated thromboxane biosynthesis, the major determinant of platelet aggregation. It is unclear whether these effects are associated with differences in thrombogenic risks. To compare the risk for thrombotic cardiovascular events among patients receiving rofecoxib, nonselective NSAIDs, and placebo, cardiovascular safety was assessed in 5,435 participants in 8 phase IIB/III osteoarthritis trials. The median treatment exposure was 31/2 months. The primary end point assessed was the risk of any arterial or venous thrombotic cardiovascular adverse event (AE). A second analysis assessed differences in the Anti-Platelet Trialists' Collaboration (APTC) events, a cluster end point that consists of the combined incidence of (1) cardiovascular, hemorrhagic, and unknown death; (2) myocardial infarction; and (3) cerebrovascular accident. Similar rates of thrombotic cardiovascular AEs were reported with rofecoxib, placebo, and comparator nonselective NSAIDs (ibuprofen, diclofenac, or nabumetone). In trials that compared rofecoxib with NSAIDs, the incidence of thrombotic cardiovascular AEs was 1.93/100 patient-years in the rofecoxib treatment group compared with 2.27/100 patient-years in the combined nonselective NSAID group. In trials that compared rofecoxib with placebo, the incidence of thrombotic cardiovascular AEs was 2.71/100 patient-years in the rofecoxib group compared with 2.57/100 patient-years in the placebo group. Consistent with the risks of cardiovascular AEs, similar rates of APTC events were reported with rofecoxib, placebo, and comparator nonselective NSAIDs. Thus, in the rofecoxib osteoarthritis development program, there was no difference between rofecoxib, comparator nonselective NSAIDs, and placebo in the risks of cardiovascular thrombotic events.

  1. [Hemorrhagic bilateral renal angiomyolipoma].

    Science.gov (United States)

    Benjelloun, Mohamed; Rabii, Redouane; Mezzour, Mohamed Hicham; Joual, Abdenbi; Bennani, Saâd; el Mrini, Mohamed

    2003-09-01

    Renal angiomyolipoma is a rare benign tumour, often associated with congenital diseases especially de Bourneville's tuberous sclerosis. Bilateral angiomyolipoma is exceptional. The authors report a case of bilateral renal angiomyolipoma in a 33-year-old patient presenting with haemorrhagic shock. In the light of this case and a review of the literature, the authors discuss the diagnostic and therapeutic aspects of this disease.

  2. FARMACOFISIOLOGÍA RENAL

    Directory of Open Access Journals (Sweden)

    Musso CG

    2014-03-01

    Full Text Available Renal physiology plays a key role in the pharmacokinetics of many drugs. Knowledge of the particularities of each nephron function (filtration, secretion, reabsorption and excretion and each of renal tubular transport mechanisms (simple diffusion, facilitated diffusion, facilitated transport, active transport, endocytosis and pinocytosis is fundamental to achieve better management of drug prescriptions.

  3. Oral contraceptives, pregnancy and the risk of cerebral thromboembolism: the influence of diabetes, hypertension, migraine and previous thrombotic disease

    DEFF Research Database (Denmark)

    Lidegaard, O

    1995-01-01

    OBJECTIVE: To assess the risk of developing cerebral thromboembolism among pregnant women and among fertile women with hypertension, migraine, diabetes, and previous thrombotic disease, and to investigate the interaction of these risk factors with the use of oral contraceptives. DESIGN: A retrosp......OBJECTIVE: To assess the risk of developing cerebral thromboembolism among pregnant women and among fertile women with hypertension, migraine, diabetes, and previous thrombotic disease, and to investigate the interaction of these risk factors with the use of oral contraceptives. DESIGN......-matched, randomly selected controls. RESPONSE: Of the 692 case and 1584 control questionnaires sent out, 590 (85.1%) and 1396 (88.1%), respectively, were returned. Of the 590 cases, nine had had cerebral thrombosis before 1980, 15 refused to participate, 44 had a revised diagnosis (primarily multiple sclerosis......) and 25 had an unreliable diagnosis, leaving 497 with a reliable cerebral thromboembolic diagnosis. Among the 1396 controls, 26 either refused to participate, were mentally handicapped, lived abroad or returned an uncompleted questionnaire, leaving 1370 controls included in the study. RESULTS: After...

  4. Recurrent Thrombotic Events after Discontinuation of Vitamin K Antagonist Treatment for Splanchnic Vein Thrombosis: A Multicenter Retrospective Cohort Study

    Directory of Open Access Journals (Sweden)

    Nicoletta Riva

    2015-01-01

    Full Text Available It is generally recommended that patients with splanchnic vein thrombosis (SVT should receive a minimum of 3 months of anticoagulant treatment. However, little information is available on the long-term risk of recurrent thrombotic events. The aim of this study was to evaluate the risk of venous and arterial thrombosis after discontinuation of vitamin K antagonist (VKA in SVT patients. Retrospective information from a cohort of SVT patients treated with VKA and followed by 37 Italian Anticoagulation Clinics, up to June 2013, was collected. Only patients who discontinued VKA and did not receive any other anticoagulant drug were enrolled in this study. Thrombotic events during follow-up were centrally adjudicated. Ninety patients were included: 33 unprovoked SVT, 27 SVT secondary to transient risk factors, and 30 with permanent risk factors. During a median follow-up of 1.6 years, 6 venous and 1 arterial thrombosis were documented, for an incidence of 3.3/100 patient-years (pt-y. The recurrence rate was highest in the first year after VKA discontinuation (8.2/100’pt-y and in patients with permanent risk factors (10.2/100’pt-y. Liver cirrhosis significantly increased the risk of recurrence. In conclusion, the rate of recurrent vascular complications after SVT is not negligible, at least in some patient subgroups.

  5. Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Ohanian M

    2011-05-01

    Full Text Available Maro Ohanian, Christian Cable, Kathleen HalkaDepartment of Hematology and Oncology, Scott and White Healthcare, The Texas A&M Health Science Center College of Medicine, Temple, TX, USAAbstract: This case report describes how eculizumab reversed neurologic impairment and improved renal damage in severe atypical hemolytic uremic syndrome. A 50-year-old female, after presenting with diarrhea and abdominal pain, developed pancolitis, acute renal failure, and thrombocytopenia. The patient underwent total abdominal colectomy. Pathology confirmed ischemic colitis with scattered mesenteric microthrombi. Due to mental and respiratory decline, she remained intubated. Continuous venovenous hemodialysis was initiated. Renal failure, neurologic changes, hemolysis, thrombotic microangiopathy, and low complement levels all suggested atypical hemolytic uremic syndrome. Eculizumab 900 mg was administered intravenously on hospital day 6 and continued weekly for four doses followed by maintenance therapy. She recovered neurologically and renally after the third dose, and hematologically by the sixth dose. Her recovery has been sustained on long-term eculizumab treatment. In severe atypical hemolytic uremic syndrome, eculizumab safely reverses neurologic impairment and eliminates the need for dialysis. The optimal duration of treatment with eculizumab remains to be determined.Keywords: eculizumab, thrombotic microangiopathy, atypical hemolytic uremic syndrome

  6. Primary renal hydatidosis

    Directory of Open Access Journals (Sweden)

    Johnsy Merla Joel

    2016-01-01

    Full Text Available Echinococcosis or hydatidosis caused by the tapeworm, Echinococcus granulosus, has the highest prevalence in endemic regions and sheep farming areas. The most common organ involved is the liver (50–75% followed by the lungs (15–20% and other organs (10–20%. Primary involvement of the kidney without the involvement of the liver and lungs, i.e., isolated renal hydatid disease is extremely rare even in endemic areas. The incidence of renal echinococcosis is 2–4%. Renal hydatid cysts usually remain asymptomatic for many years and are multiloculated. A 63-year-old male presented with left loin pain. Computed tomography scan abdomen revealed a presumptive diagnosis of renal hydatid disease. The nephrectomy specimen received in histopathology confirmed the diagnosis. We describe a rare case of primary renal hydatidosis.

  7. Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

    Directory of Open Access Journals (Sweden)

    Rohit Tewari

    2016-01-01

    Full Text Available Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.

  8. Renal replacement therapy after cardiac surgery; renal function recovers

    DEFF Research Database (Denmark)

    Steinthorsdottir, Kristin Julia; Kandler, Kristian; Agerlin Windeløv, Nis

    2013-01-01

    To assess renal outcome in patients discharged from hospital following cardiac surgery-associated acute kidney injury (CSA-AKI) with need for renal replacement therapy.......To assess renal outcome in patients discharged from hospital following cardiac surgery-associated acute kidney injury (CSA-AKI) with need for renal replacement therapy....

  9. Diabetic microangiopathy: impact of impaired cerebral vasoreactivity and delayed angiogenesis after permanent middle cerebral artery occlusion on stroke damage and cerebral repair in mice.

    Science.gov (United States)

    Poittevin, Marine; Bonnin, Philippe; Pimpie, Cynthia; Rivière, Léa; Sebrié, Catherine; Dohan, Anthony; Pocard, Marc; Charriaut-Marlangue, Christiane; Kubis, Nathalie

    2015-03-01

    Diabetes increases the risk of stroke by three, increases related mortality, and delays recovery. We aimed to characterize functional and structural alterations in cerebral microvasculature before and after experimental cerebral ischemia in a mouse model of type 1 diabetes. We hypothesized that preexisting brain microvascular disease in patients with diabetes might partly explain increased stroke severity and impact on outcome. Diabetes was induced in 4-week-old C57Bl/6J mice by intraperitoneal injections of streptozotocin (60 mg/kg). After 8 weeks of diabetes, the vasoreactivity of the neurovascular network to CO2 was abolished and was not reversed by nitric oxide (NO) donor administration; endothelial NO synthase (eNOS) and neuronal NO synthase (nNOS) mRNA, phospho-eNOS protein, nNOS, and phospho-nNOS protein were significantly decreased; angiogenic and vessel maturation factors (vascular endothelial growth factor a [VEGFa], angiopoietin 1 (Ang1), Ang2, transforming growth factor-β [TGF-β], and platelet-derived growth factor-β [PDGF-β]) and blood-brain barrier (BBB) occludin and zona occludens 1 (ZO-1) expression were significantly decreased; and microvessel density was increased without changes in ultrastructural imaging. After permanent focal cerebral ischemia induction, infarct volume and neurological deficit were significantly increased at D1 and D7, and neuronal death (TUNEL+ / NeuN+ cells) and BBB permeability (extravasation of Evans blue) at D1. At D7, CD31+ / Ki67+ double-immunolabeled cells and VEGFa and Ang2 expression were significantly increased, indicating delayed angiogenesis. We show that cerebral microangiopathy thus partly explains stroke severity in diabetes.

  10. HUS and the case for complement.

    Science.gov (United States)

    Conway, Edward M

    2015-10-29

    Hemolytic-uremic syndrome (HUS) is a thrombotic microangiopathy that is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Excess complement activation underlies atypical HUS and is evident in Shiga toxin-induced HUS (STEC-HUS). This Spotlight focuses on new knowledge of the role of Escherichia coli-derived toxins and polyphosphate in modulating complement and coagulation, and how they affect disease progression and response to treatment. Such new insights may impact on current and future choices of therapies for STEC-HUS.

  11. Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy.

    Science.gov (United States)

    Park, Yara A; Hay, Shauna N; King, Karen E; Matevosyan, Karen; Poisson, Jessica; Powers, Amy; Sarode, Ravindra; Shaz, Beth; Brecher, Mark E

    2009-01-01

    Thrombocytopenia with or without microangiopathy following quinine is often referred to as quinine "hypersensitivity." When schistocytes are present it is frequently termed "quinine-associated TTP/HUS." A severe deficiency of the vWF-cleaving protease, ADAMTS13, is associated with idiopathic TTP. A previous study of patients with "quinine-associated TTP/HUS" found that ADAMTS13 activities were not abnormal in 12/12 patients. A retrospective review of TTP patients with quinine-associated thrombotic microangiopathy (TMA) for whom ADAMTS13 was measured before plasma exchange was performed. Six patients were identified. All were females (age range: 43 to 73, mean = 61.7 years) and had taken quinine for leg cramps. Four of the six experienced renal failure requiring dialysis. Five of the patients had D-Dimers levels measured, all were elevated. In four patients the levels were > or = 18 times the upper limit of normal. ADAMTS13 was normal in four patients and mildly decreased in two patients. We conclude that while thrombocytopenia and schistocytosis can be seen in quinine-associated TTP/HUS, the pathophysiology seems to be distinct from that seen in most cases of idiopathic TTP (i.e., severely decreased ADAMTS13 with an inhibitor). We recommend that a TMA in association with quinine be consistently referred to as quinine-associated thrombotic microangiopathy (quinine-TMA) to better distinguish this entity from idiopathic TTP. The use of plasma exchange in quinine-TMA is called into question.

  12. Renal neuroendocrine tumors

    Directory of Open Access Journals (Sweden)

    Brian R Lane

    2009-01-01

    Full Text Available Objectives: Neuroendocrine tumors (NETs are uncommon tumors that exhibit a wide range of neuroendocrine differentiation and biological behavior. Primary NETs of the kidney, including carcinoid tumor, small cell carcinoma (SCC, and large cell neuroendocrine carcinoma (LCNEC are exceedingly rare. Materials and Methods: The clinicopathologic features of renal NETs diagnosed at a single institution were reviewed along with all reported cases in the worldwide literature. Results: Eighty renal NETs have been described, including nine from our institution. Differentiation between renal NETs and the more common renal neoplasms (renal cell carcinoma, transitional cell carcinoma can be difficult since clinical, radiographic, and histopathologic features overlap. Immunohistochemical staining for neuroendocrine markers, such as synaptophysin and chromogranin, can be particularly helpful in this regard. Renal carcinoids are typically slow-growing, may secrete hormones, and pursue a variable clinical course. In contrast, SCC and LCNEC often present with locally advanced or metastatic disease and carry a poor prognosis. Nephrectomy can be curative for clinically localized NETs, but multimodality treatment is indicated for advanced disease. Conclusions: A spectrum of NETs can rarely occur in the kidney. Renal carcinoids have a variable clinical course; SCC and LCNEC are associated with poor clinical outcomes. Diagnosis of NETs, especially LCNEC, requires awareness of their rare occurrence and prudent use of immunohistochemical neuroendocrine markers.

  13. Pregnancy and renal transplantation.

    Science.gov (United States)

    Başaran, O; Emiroğlu, R; Seçme, S; Moray, G; Haberal, M

    2004-01-01

    Ovarian dysfunction, anovulatory vaginal bleeding, amenorrhea, high prolactin levels, and loss of libido are the causes of infertility in women with chronic renal failure. After renal transplantation, endocrine function generally improves after recovery of renal function. In this study we retrospectively evaluated the prepregnancy and postdelivery renal function, outcome of gestation, as well as maternal and fetal complications for eight pregnancies in eight renal transplant recipients between November 1975 and March 2003 of 1095 among 1425. Eight planned pregnancies occurred at a mean of 3.6 years posttransplant. Spontaneous abortion occured in the first trimester in one case. One intrauterine growth retardation was observed with a full-term pregnancy; one intrauterine growth retardation and preterm delivery; one preeclampsia with preterm delivery and urinary tract infection; and one preeclampsia with preterm delivery and oligohydramnios. The mean gestation period was 35.5 +/- 3.0 weeks (31.2 to 38.0). Pregnancy had no negative impact on renal function during a 2-year follow-up. No significant proteinuria or acute rejection episodes were observed. Among the seven deliveries, no congenital anomaly was documented and no postpartum problems for the child and the mother were observed. Our study suggests that successful pregnancy is possible in renal transplant recipients. In cases with good graft function and absence of severe proteinuria or hypertension, pregnancy does not affect graft function or patient survival; however, fetal problems are encountered such as intrauterine growth retardation, low birth weight, and preeclampsia.

  14. Renal autotransplantation: current perspectives.

    Science.gov (United States)

    Stewart, B H; Banowsky, L H; Hewitt, C B; Straffon, R A

    1976-01-01

    Autotransplantation, with or without an extracorporeal renal operation, has been done 39 times in 37 patients. Indications for the procedure included severe ureteral injury in 4 patients, failed supravesical diversion in 2, renal carcinoma in a solitary kidney in 1, renovascular hypertension in 1 and donor arterial reconstruction before renal transplantation in 29. Success was obtained in all but 2 procedures, both of which involved previously operated kidneys with severe inflammation and adhesions involving the renal pelvis and pedicle. Based on our experience and a review of currently available literature we believe that renal autotransplantation and extracorporeal reconstruction can provide the best solution for patients with severe renovascular and ureteral disease not correctable by conventional operative techniques. The technique can be of particular value in removing centrally located tumors in solitary kidneys and in preparing donor kidneys with abnormal arteries for renal transplantation. The role of autotransplantation in the management of advanced renal trauma and calculus disease is less clear. A long-term comparison of patients treated by extracorporeal nephrolithotomy versus conventional lithotomy techniques will be necessary before a conclusion is reached in these disease categories.

  15. Perioperative acute renal failure.

    LENUS (Irish Health Repository)

    Mahon, Padraig

    2012-02-03

    PURPOSE OF REVIEW: Recent biochemical evidence increasingly implicates inflammatory mechanisms as precipitants of acute renal failure. In this review, we detail some of these pathways together with potential new therapeutic targets. RECENT FINDINGS: Neutrophil gelatinase-associated lipocalin appears to be a sensitive, specific and reliable biomarker of renal injury, which may be predictive of renal outcome in the perioperative setting. For estimation of glomerular filtration rate, cystatin C is superior to creatinine. No drug is definitively effective at preventing postoperative renal failure. Clinical trials of fenoldopam and atrial natriuretic peptide are, at best, equivocal. As with pharmacological preconditioning of the heart, volatile anaesthetic agents appear to offer a protective effect to the subsequently ischaemic kidney. SUMMARY: Although a greatly improved understanding of the pathophysiology of acute renal failure has offered even more therapeutic targets, the maintenance of intravascular euvolaemia and perfusion pressure is most effective at preventing new postoperative acute renal failure. In the future, strategies targeting renal regeneration after injury will use bone marrow-derived stem cells and growth factors such as insulin-like growth factor-1.

  16. Midterm renal functions following acute renal infarction

    Directory of Open Access Journals (Sweden)

    Sakir Ongun

    2015-10-01

    Full Text Available The aim of this study was to explore clinical features of renal infarction (RI that may have a role in diagnosis and treatment in our patient cohort and provide data on midterm renal functions. Medical records of patients with diagnosis of acute RI, established by contrast enhanced computed tomography (CT and at least 1 year follow-up data, who were hospitalized in our clinic between 1998 and 2012 were retrospectively reviewed; including descriptive data, clinical signs and symptoms, etiologic factors, laboratory findings, and prescribed treatments. Patients with solitary infarct were treated with acetylsalicylic acid (ASA only, whereas patients with atrial fibrillation (AF or multiple or global infarct were treated with anticoagulants. Estimated Glomerular Filtration Rate (eGFR referring to renal functions was determined by the Modification of Diet in Renal Disease (MDRD formula. Twenty-seven renal units of 23 patients with acute RI were identified. The mean age was 59.7 ± 15.7 years. Fourteen patients (60.8% with RI had atrial fibrillation (AF as an etiologic factor of which four had concomitant mesenteric ischemia at diagnosis. At presentation, 20 patients (86.9% had elevated serum lactate dehydrogenase (LDH, 18 patients (78.2% had leukocytosis, and 16 patients (69.5% had microscopic hematuria. Two patients with concomitant mesenteric ischemia and AF passed away during follow up. Mean eGFR was 70.8 ± 23.2 mL/min/1.73 m2 at admission and increased to 82.3 ± 23.4 mL/min/1.73 m2 at 1 year follow up. RI should be considered in patients with persistent flank or abdominal pain, particularly if they are at high risk of thromboembolism. Antiplatelet and/or anticoagulant drugs are both effective treatment options according to the amplitude of the infarct for preserving kidney functions.

  17. Midterm renal functions following acute renal infarction.

    Science.gov (United States)

    Ongun, Sakir; Bozkurt, Ozan; Demir, Omer; Cimen, Sertac; Aslan, Guven

    2015-10-01

    The aim of this study was to explore clinical features of renal infarction (RI) that may have a role in diagnosis and treatment in our patient cohort and provide data on midterm renal functions. Medical records of patients with diagnosis of acute RI, established by contrast enhanced computed tomography (CT) and at least 1 year follow-up data, who were hospitalized in our clinic between 1998 and 2012 were retrospectively reviewed; including descriptive data, clinical signs and symptoms, etiologic factors, laboratory findings, and prescribed treatments. Patients with solitary infarct were treated with acetylsalicylic acid (ASA) only, whereas patients with atrial fibrillation (AF) or multiple or global infarct were treated with anticoagulants. Estimated Glomerular Filtration Rate (eGFR) referring to renal functions was determined by the Modification of Diet in Renal Disease (MDRD) formula. Twenty-seven renal units of 23 patients with acute RI were identified. The mean age was 59.7 ± 15.7 years. Fourteen patients (60.8%) with RI had atrial fibrillation (AF) as an etiologic factor of which four had concomitant mesenteric ischemia at diagnosis. At presentation, 20 patients (86.9%) had elevated serum lactate dehydrogenase (LDH), 18 patients (78.2%) had leukocytosis, and 16 patients (69.5%) had microscopic hematuria. Two patients with concomitant mesenteric ischemia and AF passed away during follow up. Mean eGFR was 70.8 ± 23.2 mL/min/1.73 m(2) at admission and increased to 82.3 ± 23.4 mL/min/1.73 m(2) at 1 year follow up. RI should be considered in patients with persistent flank or abdominal pain, particularly if they are at high risk of thromboembolism. Antiplatelet and/or anticoagulant drugs are both effective treatment options according to the amplitude of the infarct for preserving kidney functions.

  18. Lactulose and renal failure.

    Science.gov (United States)

    Vogt, B; Frey, F J

    1997-01-01

    The introduction of lactulose as a new therapeutic agent for treatment of hepatic encephalopathy was a major breakthrough in this field. It was hypothesized that lactulose might prevent postoperative renal impairment after biliary surgery in patients with obstructive jaundice. The presumable mechanism purported was the diminished endotoxinemia by lactulose. Unfortunately, such a reno-protective effect has not been shown conclusively until now in clinical studies. In chronic renal failure lactulose is known to promote fecal excretion of water, sodium, potassium, amonium, urea, creatinine and protons. Thus, lactulose could be useful for the treatment of chronic renal failure. However, compliance to the therapy represents a major problem.

  19. Renal tubule cell repair following acute renal injury.

    Science.gov (United States)

    Humes, H D; Lake, E W; Liu, S

    1995-01-01

    Experimental data suggests the recovery of renal function after ischemic or nephrotoxic acute renal failure is due to a replicative repair process dependent upon predominantly paracrine release of growth factors. These growth factors promote renal proximal tubule cell proliferation and a differentiation phase dependent on the interaction between tubule cells and basement membrane. These insights identify the molecular basis of renal repair and ischemic and nephrotoxic acute renal failure, and may lead to potential therapeutic modalities that accelerate renal repair and lessen the morbidity and mortality associated with these renal disease processes. In this regard, there is a prominent vasoconstrictor response of the renal vasculature during the postischemic period of developing acute renal failure. The intravenous administration of pharmacologic doses of atrial natriuretic factor (ANF) in the postischemic period have proven efficacious by altering renal vascular resistance, so that renal blood flow and glomerular filtration rate improve. ANF also appears to protect renal tubular epithelial integrity and holds significant promise as a therapeutic agent in acute renal failure. Of equal or greater promise are the therapeutic interventions targeting the proliferative reparative zone during the postischemic period. The exogenous administration of epidermal growth factor or insulin-like growth factor-1 in the postischemic period have effectively decreased the degree of renal insufficiency as measured by the peak serum creatinine and has hastened renal recovery as measured by the duration of time required to return the baseline serum creatinine values. A similarly efficacious role for hepatocyte growth factor has also been recently demonstrated.

  20. Post-thrombotic syndrome patient education based on the health belief model: self-reported intention to comply with recommendations.

    Science.gov (United States)

    Carolyn, Crumley

    2011-01-01

    This study was designed to evaluate patient responses to a patient education handout on post-thrombotic syndrome prevention based on the Health Belief Model. This quasi-experimental pilot study involved a patient survey to be completed after reviewing a patient education handout. A convenience sample of 25 patients with deep vein thrombosis confirmed by venous Doppler assessment with a lower extremity deep vein thrombosis admitted to a Midwestern community hospital was identified. Seven patients were excluded and 5 declined participation; 13 completed the survey patients. Subjects were older than 18 years and able to read and understand English. Patients with hospice or palliative care service or life expectancy less than 6 months were excluded. Subjects were provided with the post-thrombotic syndrome (PTS), patient education handout, and the PTS patient education survey. The PTS patient education handout consisted of a 1-page informational sheet based on PTS and Health Belief Model literature. The 24-item PTS Patient Education Survey required approximately 15 minutes to complete; items included demographic information, questions regarding previous deep vein thrombosis and Likert scale opinion statements regarding PTS based on Health Belief Model components. Patients meeting inclusion criteria were approached by the investigator, invited to participate in the study, and offered the option of having the investigator collect the survey or return in an addressed, stamped envelope. Respondents tended to agree that PTS was a serious condition and that it would negatively affect their life, primarily in relation to comfort and the ability to engage in leisure activities. Ten participants (76.9%) acknowledged that they were susceptible to PTS, and that elastic graduated compression stockings were effective. The most commonly cited barrier to wearing the stockings was difficulty with application. Five patients (38.5%) agreed that they had the ability to prevent PTS and 9

  1. Schistosome tegumental ecto-apyrase (SmATPDase1 degrades exogenous pro-inflammatory and pro-thrombotic nucleotides

    Directory of Open Access Journals (Sweden)

    Akram A. Da’dara

    2014-03-01

    Full Text Available Schistosomes are parasitic worms that can survive in the hostile environment of the human bloodstream where they appear refractory to both immune elimination and thrombus formation. We hypothesize that parasite migration in the bloodstream can stress the vascular endothelium causing this tissue to release chemicals alerting responsive host cells to the stress. Such chemicals are called damage associated molecular patterns (DAMPs and among the most potent is the proinflammatory mediator, adenosine triphosphate (ATP. Furthermore, the ATP derivative ADP is a pro-thrombotic molecule that acts as a strong activator of platelets. Schistosomes are reported to possess at their host interactive tegumental surface a series of enzymes that could, like their homologs in mammals, degrade extracellular ATP and ADP. These are alkaline phosphatase (SmAP, phosphodiesterase (SmNPP-5 and ATP diphosphohydrolase (SmATPDase1. In this work we employ RNAi to knock down expression of the genes encoding these enzymes in the intravascular life stages of the parasite. We then compare the abilities of these parasites to degrade exogenously added ATP and ADP. We find that only SmATPDase1-suppressed parasites are significantly impaired in their ability to degrade these nucleotides. Suppression of SmAP or SmNPP-5 does not appreciably affect the worms’ ability to catabolize ATP or ADP. These findings are confirmed by the functional characterization of the enzymatically active, full-length recombinant SmATPDase1 expressed in CHO-S cells. The enzyme is a true apyrase; SmATPDase1 degrades ATP and ADP in a cation dependent manner. Optimal activity is seen at alkaline pH. The Km of SmATPDase1 for ATP is 0.4 ± 0.02 mM and for ADP, 0.252 ± 0.02 mM. The results confirm the role of tegumental SmATPDase1 in the degradation of the exogenous pro-inflammatory and pro-thrombotic nucleotides ATP and ADP by live intravascular stages of the parasite. By degrading host inflammatory signals

  2. The clinical expression of hereditary protein C and protein S deficiency: : a relation to clinical thrombotic risk-factors and to levels of protein C and protein S

    NARCIS (Netherlands)

    Henkens, C. M. A.; van der Meer, J.; Hillege, J. L.; Bom, V. J. J.; Halie, M. R.; van der Schaaf, W.

    We investigated 103 first-degree relatives of 13 unrelated protein C or protein S deficient patients to assess the role of additional thrombotic risk factors and of protein C and protein S levels in the clinical expression of hereditary protein C and protein S deficiency. Fifty-seven relatives were

  3. The venous thrombotic risk of oral contraceptives, effects of oestrogen dose and progestogen type: results of the MEGA case-control study

    NARCIS (Netherlands)

    Hylckama Vlieg, van A.; Helmerhorst, F.M.; Vandenbroucke, J.P.; Doggen, C.J.M.; Rosendaal, F.R.

    2009-01-01

    Objective To assess the thrombotic risk associated with oral contraceptive use with a focus on dose of oestrogen and type of progestogen of oral contraceptives available in the Netherlands. Design Population based case-control study. Setting Six participating anticoagulation clinics in the Nethe

  4. Renal scintigraphy in veterinary medicine.

    Science.gov (United States)

    Tyson, Reid; Daniel, Gregory B

    2014-01-01

    Renal scintigraphy is performed commonly in dogs and cats and has been used in a variety of other species. In a 2012 survey of the members of the Society of Veterinary Nuclear Medicine, 95% of the respondents indicated they perform renal scintigraphy in their practice. Renal scintigraphy is primarily used to assess renal function and to evaluate postrenal obstruction. This article reviews how renal scintigraphy is used in veterinary medicine and describes the methods of analysis. Species variation is also discussed.

  5. Primary renal synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Girish D. Bakhshi

    2012-03-01

    Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

  6. Renal protection in diabetes

    DEFF Research Database (Denmark)

    Parving, H H; Tarnow, L; Rossing, P

    1996-01-01

    BACKGROUND: The combination of diabetes and hypertension increases the chances of progressive renal disorder and, ultimately, renal failure. Roughly 40% of all diabetics, whether insulin-dependent or not, develop diabetic nephropathy. Diabetic nephropathy is the single most important cause of end......-stage renal disease in the Western world and accounts for more than a quarter of all end-stage renal diseases. Diabetic nephropathy is a major cause of increased morbidity and mortality in diabetic patients. Increased arterial blood pressure is an early and common phenomenon in incipient and overt diabetic...... nephropathy. The relationship between arterial blood pressure and diabetic nephropathy is a complex one, with diabetic nephropathy increasing blood pressure and blood pressure accelerating the course of nephropathy. OVERVIEW: Calcium antagonists antagonize preglomerular vasoconstriction. Additional putative...

  7. Renal primitive neuroectodermal tumors.

    Science.gov (United States)

    Bartholow, Tanner; Parwani, Anil

    2012-06-01

    Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

  8. Renal vein thrombosis

    Science.gov (United States)

    ... Saunders; 2012:chap 34. Read More Acute kidney failure Arteriogram Blood clots Dehydration Nephrotic syndrome Pulmonary embolus Renal Tumor Review Date 5/19/2015 Updated by: Charles Silberberg, ...

  9. Eligibility for renal denervation

    DEFF Research Database (Denmark)

    Persu, Alexandre; Jin, Yu; Baelen, Marie;

    2014-01-01

    -resistant hypertension (ENCOReD). The analysis included 731 patients. Age averaged 61.6 years, office blood pressure at screening was 177/96 mm Hg, and the number of blood pressure-lowering drugs taken was 4.1. Specialists referred 75.6% of patients. The proportion of patients eligible for renal denervation according......Based on the SYMPLICITY studies and CE (Conformité Européenne) certification, renal denervation is currently applied as a novel treatment of resistant hypertension in Europe. However, information on the proportion of patients with resistant hypertension qualifying for renal denervation after...... undetected secondary causes of hypertension (11.1%). In conclusion, after careful screening and treatment adjustment at hypertension expert centers, only ≈40% of patients referred for renal denervation, mostly by specialists, were eligible for the procedure. The most frequent cause of ineligibility...

  10. Complete renal recovery from severe acute renal failure after thrombolysis of bilateral renal vein thrombosis.

    Science.gov (United States)

    Ramadoss, Suresh; Jones, Robert G; Foggensteiner, Lukas; Willis, Andrew P; Duddy, Martin J

    2012-10-01

    A previously healthy young man presented with acute renal failure due to extensive spontaneous deep vein thrombosis, including the inferior vena cava (IVC) and both renal veins. The patient was treated with selectively delivered thrombolytic therapy over a 7-day-period, which resulted in renal vein patency and complete recovery of renal function. A stent was placed over a segment stenosis of the IVC. No thrombophilic factors were identified. Bilateral renal vein thrombosis in young fit individuals is an unusual cause of acute renal failure. Thrombolytic therapy, even with delay, can completely restore renal function.

  11. OBSTETRIC RENAL FAILURE

    Directory of Open Access Journals (Sweden)

    Rajeshwari

    2015-11-01

    Full Text Available Renal failure in obstetrics is rare but important complication, associated with significant mortality and long term morbidity.1,2 It includes acute renal failure due to obstetrical complications or due to deterioration of existing renal disease. AIMS AND OBJECTIVES: To evaluate the etiology and outcome of renal failure in obstetric patients. METHODS: We prospectively analyzed 30 pregnant and puerperal women with acute renal failure or pre-existing renal disease developing renal failure during pregnancy between November 2007 to sep-2009. Patients who presented/developed ARF during the hospital stay were included in this study. RESULTS: Among 30 patients, mean age was 23 years and 33 years age group. 12 cases (40% patients were primigravidae and 9(30% patients were multigravidae and 9 cases (30% presented in post-partum period. Eighteen cases (60% with ARF were seen in third trimester, followed by in postpartum period 9 cases (30%. Most common contributing factors to ARF were Pre-eclampsia, eclampsia and HELLP syndrome 60%, sepsis 56.6%, post abortal ARF 10%. DIC 40%. Haemorrhage as the aetiology for ARF was present 46%, APH in 20% and PPH in 26.6%. The type of ARF was renal in (63% and prerenal (36%; Oliguric seen in 10 patients (33% and high mortality (30%. Among the 20 pregnant patients with ARF, The average period of gestation was 33±2 weeks (30 -36 weeks, 5 cases (25% presented with intrauterine fetal demise and 18 cases (66% had preterm vaginal delivery and 2 cases (10% had induced abortion. And the average birth weight was 2±0.5 kg (1.5 kg. Eight cases (26% required dialysis. 80% of patients recovered completely of renal functions. 63% patients recovered without renal replacement therapy whereas 17% required dialysis. the maternal mortality was 20%, the main reason for mortality was septic shock and multi organ dysfunction (66%. CONCLUSION: ARF related pregnancy was seen commonly in the primigravidae and in the third trimester, the most

  12. Renal papillary necrosis

    Directory of Open Access Journals (Sweden)

    Stephen A. Geller

    2013-12-01

    Full Text Available In 1877, Dr. Nikolaus Friedreich (1825-1882; student of Virchow who became Professor of Pathology at Heidelberg and who also described Friedreich’s ataxia first described renal papillary necrosis (RPN in patients with prostatic hypertrophy and secondary hydronephrosis. Thereafter in 1937, Froboese and Günther emphasized the association of this entity with diabetes mellitus. These authors also observed renal papillary necrosis in cases of urinary tract obstruction even in the absence of diabetes mellitus.

  13. [Hyperuricemia and renal risk].

    Science.gov (United States)

    Viazzi, Francesca; Bonino, Barbara; Ratto, Elena; Desideri, Giovambattista; Pontremoli, Roberto

    2015-01-01

    Recent studies have revealed an association between elevated levels of uric acid and conditions correlated to chronic kidney diseases such as hypertension, cardiovascular and cerebral disease, insulin resistance. Several pathogenetic mechanisms at cellular and tissue levels could justify a direct correlation between serum uric acid levels and renal damage. Growing evidence indicating a correlation between urate lowering therapy and renal morbidity could encourage the use of urate lowering therapy in primary or secondary prevention in chronic kidney disease.

  14. Mutation of the Kunitz-type proteinase inhibitor domain in the amyloid β-protein precursor abolishes its anti-thrombotic properties in vivo.

    Science.gov (United States)

    Xu, Feng; Davis, Judianne; Hoos, Michael; Van Nostrand, William E

    2017-07-01

    Kunitz proteinase inhibitor (KPI) domain-containing forms of the amyloid β-protein precursor (AβPP) inhibit cerebral thrombosis. KPI domain-lacking forms of AβPP are abundant in brain. Regions of AβPP other than the KPI domain may also be involved with regulating cerebral thrombosis. To determine the contribution of the KPI domain to the overall function of AβPP in regulating cerebral thrombosis we generated a reactive center mutant that was devoid of anti-thrombotic activity and studied its anti-thrombotic function in vitro and in vivo. To determine the extent of KPI function of AβPP in regulating cerebral thrombosis we generated a recombinant reactive center KPI(R13I) mutant devoid of anti-thrombotic activity. The anti-proteolytic and anti-coagulant properties of wild-type and R13I mutant KPI were investigated in vitro. Cerebral thrombosis of wild-type, AβPP knock out and AβPP/KPI(R13I) mutant mice was evaluated in experimental models of carotid artery thrombosis and intracerebral hemorrhage. Recombinant mutant KPI(R13I) domain was ineffective in the inhibition of pro-thrombotic proteinases and did not inhibit the clotting of plasma in vitro. AβPP/KPI(R13I) mutant mice were similarly deficient as AβPP knock out mice in regulating cerebral thrombosis in experimental models of carotid artery thrombosis and intracerebral hemorrhage. We demonstrate that the anti-thrombotic function of AβPP primarily resides in the KPI activity of the protein. Copyright © 2017 Elsevier Ltd. All rights reserved.

  15. Laparoscopic Renal Cryoablation

    Science.gov (United States)

    Schiffman, Marc; Moshfegh, Amiel; Talenfeld, Adam; Del Pizzo, Joseph J.

    2014-01-01

    In light of evidence linking radical nephrectomy and consequent suboptimal renal function to adverse cardiovascular events and increased mortality, research into nephron-sparing techniques for renal masses widely expanded in the past two decades. The American Urological Association (AUA) guidelines now explicitly list partial nephrectomy as the standard of care for the management of T1a renal tumors. Because of the increasing utilization of cross-sectional imaging, up to 70% of newly detected renal masses are stage T1a, making them more amenable to minimally invasive nephron-sparing therapies including laparoscopic and robotic partial nephrectomy and ablative therapies. Cryosurgery has emerged as a leading option for renal ablation, and compared with surgical techniques it offers benefits in preserving renal function with fewer complications, shorter hospitalization times, and allows for quicker convalescence. A mature dataset exists at this time, with intermediate and long-term follow-up data available. Cryosurgical recommendations as a first-line therapy are made at this time in limited populations, including elderly patients, patients with multiple comorbidities, and those with a solitary kidney. As more data emerge on oncologic efficacy, and technical experience and the technology continue to improve, the application of this modality will likely be extended in future treatment guidelines. PMID:24596441

  16. Targeting factor VIII expression to platelets for hemophilia A gene therapy does not induce an apparent thrombotic risk in mice.

    Science.gov (United States)

    Baumgartner, C K; Mattson, J G; Weiler, H; Shi, Q; Montgomery, R R

    2017-01-01

    Essentials Platelet-Factor (F) VIII gene therapy is a promising treatment in hemophilia A. This study aims to evaluate if platelet-FVIII expression would increase the risk for thrombosis. Targeting FVIII expression to platelets does not induce or elevate thrombosis risk. Platelets expressing FVIII are neither hyper-activated nor hyper-responsive. Background Targeting factor (F) VIII expression to platelets is a promising gene therapy approach for hemophilia A, and is successful even in the presence of inhibitors. It is well known that platelets play important roles not only in hemostasis, but also in thrombosis and inflammation. Objective To evaluate whether platelet-FVIII expression might increase thrombotic risk and thereby compromise the safety of this approach. Methods In this study, platelet-FVIII-expressing transgenic mice were examined either in steady-state conditions or under prothrombotic conditions induced by inflammation or the FV Leiden mutation. Native whole blood thrombin generation assay, rotational thromboelastometry analysis and ferric chloride-induced vessel injury were used to evaluate the hemostatic properties. Various parameters associated with thrombosis risk, including D-dimer, thrombin-antithrombin complexes, fibrinogen, tissue fibrin deposition, platelet activation status and activatability, and platelet-leukocyte aggregates, were assessed. Results We generated a new line of transgenic mice that expressed 30-fold higher levels of platelet-expressed FVIII than are therapeutically required to restore hemostasis in hemophilic mice. Under both steady-state conditions and prothrombotic conditions induced by lipopolysaccharide-mediated inflammation or the FV Leiden mutation, supratherapeutic levels of platelet-expressed FVIII did not appear to be thrombogenic. Furthermore, FVIII-expressing platelets were neither hyperactivated nor hyperactivatable upon agonist activation. Conclusion We conclude that, in mice, more than 30-fold higher levels of

  17. An NO donor approach to neuroprotective and procognitive estrogen therapy overcomes loss of NO synthase function and potentially thrombotic risk.

    Directory of Open Access Journals (Sweden)

    Lawren VandeVrede

    Full Text Available Selective estrogen receptor modulators (SERMs are effective therapeutics that preserve favorable actions of estrogens on bone and act as antiestrogens in breast tissue, decreasing the risk of vertebral fractures and breast cancer, but their potential in neuroprotective and procognitive therapy is limited by: 1 an increased lifetime risk of thrombotic events; and 2 an attenuated response to estrogens with age, sometimes linked to endothelial nitric oxide synthase (eNOS dysfunction. Herein, three 3(rd generation SERMs with similar high affinity for estrogen receptors (ERα, ERβ were studied: desmethylarzoxifene (DMA, FDMA, and a novel NO-donating SERM (NO-DMA. Neuroprotection was studied in primary rat neurons exposed to oxygen glucose deprivation; reversal of cholinergic cognitive deficit was studied in mice in a behavioral model of memory; long term potentiation (LTP, underlying cognition, was measured in hippocampal slices from older 3×Tg Alzheimer's transgenic mice; vasodilation was measured in rat aortic strips; and anticoagulant activity was compared. Pharmacologic blockade of GPR30 and NOS; denudation of endothelium; measurement of NO; and genetic knockout of eNOS were used to probe mechanism. Comparison of the three chemical probes indicates key roles for GPR30 and eNOS in mediating therapeutic activity. Procognitive, vasodilator and anticoagulant activities of DMA were found to be eNOS dependent, while neuroprotection and restoration of LTP were both shown to be dependent upon GPR30, a G-protein coupled receptor mediating estrogenic function. Finally, the observation that an NO-SERM shows enhanced vasodilation and anticoagulant activity, while retaining the positive attributes of SERMs even in the presence of NOS dysfunction, indicates a potential therapeutic approach without the increased risk of thrombotic events.

  18. Effective anti-thrombotic therapy without stenting: intravascular optical coherence tomography-based management in plaque erosion (the EROSION study).

    Science.gov (United States)

    Jia, Haibo; Dai, Jiannan; Hou, Jingbo; Xing, Lei; Ma, Lijia; Liu, Huimin; Xu, Maoen; Yao, Yuan; Hu, Sining; Yamamoto, Erika; Lee, Hang; Zhang, Shaosong; Yu, Bo; Jang, Ik-Kyung

    2017-03-14

    Plaque erosion, compared with plaque rupture, has distinctly different underlying pathology and therefore may merit tailored therapy. In this study, we aimed to assess whether patients with acute coronary syndrome (ACS) caused by plaque erosion might be stabilized by anti-thrombotic therapy without stent implantation. This was a single-centre, uncontrolled, prospective, proof-of concept study. Patients with ACS including ST-segment elevation myocardial infarction were prospectively enrolled. If needed, aspiration thrombectomy was performed. Patients diagnosed with plaque erosion by optical coherence tomography (OCT) and residual diameter stenosis 50% reduction of thrombus volume at 1 month compared with baseline. The secondary endpoint was a composite of cardiac death, recurrent ischaemia requiring revascularization, stroke, and major bleeding. Among 405 ACS patients with analysable OCT images, plaque erosion was identified in 103 (25.4%) patients. Sixty patients enrolled and 55 patients completed the 1-month follow-up. Forty-seven patients (47/60, 78.3%; 95% confidence interval: 65.8-87.9%) met the primary endpoint, and 22 patients had no visible thrombus at 1 month. Thrombus volume decreased from 3.7 (1.3, 10.9) mm3 to 0.2 (0.0, 2.0) mm3. Minimal flow area increased from 1.7 (1.4, 2.4) mm2 to 2.1 (1.5, 3.8) mm2. One patient died of gastrointestinal bleeding, and another patient required repeat percutaneous coronary intervention. The rest of the patients remained asymptomatic. For patients with ACS caused by plaque erosion, conservative treatment with anti-thrombotic therapy without stenting may be an option.

  19. Statins but not aspirin reduce thrombotic risk assessed by thrombin generation in diabetic patients without cardiovascular events: the RATIONAL trial.

    Directory of Open Access Journals (Sweden)

    Alejandro Macchia

    Full Text Available BACKGROUND: The systematic use of aspirin and statins in patients with diabetes and no previous cardiovascular events is controversial. We sought to assess the effects of aspirin and statins on the thrombotic risk assessed by thrombin generation (TG among patients with type II diabetes mellitus and no previous cardiovascular events. METHODOLOGY/PRINCIPAL FINDINGS: Prospective, randomized, open, blinded to events evaluation, controlled, 2×2 factorial clinical trial including 30 patients randomly allocated to aspirin 100 mg/d, atorvastatin 40 mg/d, both or none. Outcome measurements included changes in TG levels after treatment (8 to 10 weeks, assessed by a calibrated automated thrombogram. At baseline all groups had similar clinical and biochemical profiles, including TG levels. There was no interaction between aspirin and atorvastatin. Atorvastatin significantly reduced TG measured as peak TG with saline (85.09±55.34 nmol vs 153.26±75.55 nmol for atorvastatin and control groups, respectively; p = 0.018. On the other hand, aspirin had no effect on TG (121.51±81.83 nmol vs 116.85±67.66 nmol, for aspirin and control groups, respectively; p = 0.716. The effects of treatments on measurements of TG using other agonists were consistent. CONCLUSIONS/SIGNIFICANCE: While waiting for data from ongoing large clinical randomized trials to definitively outline the role of aspirin in primary prevention, our study shows that among diabetic patients without previous vascular events, statins but not aspirin reduce thrombotic risk assessed by TG. TRIAL REGISTRATION: ClinicalTrials.gov NCT00793754.

  20. Targeting Strategies for Renal Cell Carcinoma: From Renal Cancer Cells to Renal Cancer Stem Cells

    OpenAIRE

    Zhi-xiang Yuan; Jingxin Mo; Guixian Zhao; Gang Shu; Hua-lin Fu; Wei Zhao

    2016-01-01

    Renal cell carcinoma (RCC) is a common form of urologic tumor that originates from the highly heterogeneous epithelium of renal tubules. Over the last decade, targeting therapies to renal cancer cells have transformed clinical care for RCC. Recently, it was proposed that renal cancer stem cells (CSCs) isolated from renal carcinomas were responsible for driving tumor growth and resistance to conventional chemotherapy and radiotherapy, according to the theory of CSCs; this has provided the rati...

  1. RENAL MALIGNANT NEOPLASMS: RENAL CELL CARCINOMA

    Directory of Open Access Journals (Sweden)

    Elisangela Giachini

    2017-06-01

    Full Text Available The aim of this study is to evaluate the incidence and prevalence of malignant kidney tumors, to contribute to identifying factors which the diagnosis of renal cell carcinomas. Through this study, we understand that kidney disease over the years had higher incidence rates, especially in adults in the sixth decade of life. The renal cell carcinoma (RCC is the third most common malignancy of the genitourinary tract, affecting 2% to 3% of the population. There are numerous ways of diagnosis; however, the most important are ultrasonography, magnetic resonance imaging and computed tomography. In general most of the patients affected by the CCR, have a good prognosis when diagnosed early and subjected to an effective treatment. This study conducted a literature review about the CCR, through this it was possible to understand the development needs of the imaging methods used for precise diagnosis and classification of RCC through the TNM system.

  2. Postpartum atypical haemolytic uraemic syndrome in a pre-eclamptic patient

    Directory of Open Access Journals (Sweden)

    Efterpi Tingi

    2016-03-01

    Full Text Available Postpartum atypical haemolytic uraemic syndrome (aHUS is a rare manifestation, defined as non-immune haemolytic anaemia, thrombocytopenia, and renal thrombotic microangiopathy (TMA typically following a normal vaginal delivery. Most cases of aHUS are caused by uncontrolled chronic activation of alternative complement pathway, resulting in microvascular thrombosis, organ ischaemia and damage. aHUS has a poor prognosis, hence up to 65 percent of patients require haemodialysis or have kidney damage or die. We report a case of postpartum atypical haemolytic uraemic syndrome in a 30 year old multiparous woman, who presented with orthopnoea, chest and abdominal pain, seven days following emergency caesarean section due to severe preeclampsia. Clinical examination and several investigations revealed anaemia, thrombocytopenia and renal failure, findings suggestive of thrombotic microangiopathy, therefore she was admitted in ITU where total plasma exchanged was initiated. The patient improved 10 days post ITU admission and discharged home. This case highlights that the TMAs may be indistinguishable clinically from each other, making a formal diagnosis difficult. Atypical HUS should be suspected during the postpartum period in any woman who presents with haeomolytic anaemia, thrombocytopenia and severe renal impairment. Aggressive treatment with plasma exchange may limit vascular injury and improve prognosis. [Int J Reprod Contracept Obstet Gynecol 2016; 5(3.000: 894-898

  3. Malignant renal tumors in children

    Directory of Open Access Journals (Sweden)

    Justin Scott Lee

    2015-05-01

    Full Text Available Renal malignancies are common in children. While the majority of malignant renal masses are secondary to Wilms tumor, it can be challenging to distinguish from more aggressive renal masses. For suspicious renal lesions, it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment. This review article will discuss the common differential diagnosis that can be encountered when evaluating a suspicious renal mass in the pediatric population. This includes clear cell sarcoma of the kidney, malignant rhabdoid tumor, renal medullary carcinoma and lymphoma. 

  4. Percutaneous renal tumour biopsy.

    Science.gov (United States)

    Delahunt, Brett; Samaratunga, Hemamali; Martignoni, Guido; Srigley, John R; Evans, Andrew J; Brunelli, Matteo

    2014-09-01

    The use of percutaneous renal tumour biopsy (RTB) as a diagnostic tool for the histological characterization of renal masses has increased dramatically within the last 30 years. This increased utilization has paralleled advances in imaging techniques and an evolving knowledge of the clinical value of nephron sparing surgery. Improved biopsy techniques using image guidance, coupled with the use of smaller gauge needles has led to a decrease in complication rates. Reports from series containing a large number of cases have shown the non-diagnostic rate of RTB to range from 4% to 21%. Re-biopsy has been shown to reduce this rate, while the use of molecular markers further improves diagnostic sensitivity. In parallel with refinements of the biopsy procedure, there has been a rapid expansion in our understanding of the complexity of renal cell neoplasia. The 2013 Vancouver Classification is the current classification for renal tumours, and contains five additional entities recognized as novel forms of renal malignancy. The diagnosis of tumour morphotype on RTB is usually achievable on routine histology; however, immunohistochemical studies may be of assistance in difficult cases. The morphology of the main tumour subtypes, based upon the Vancouver Classification, is described and differentiating features are discussed.

  5. Can renal infarction occur after renal cyst aspiration? Case report.

    Science.gov (United States)

    Emre, Habib; Soyoral, Yasemin Usul; Tanik, Serhat; Gecit, Ilhan; Begenik, Huseyin; Pirincci, Necip; Erkoc, Reha

    2011-01-01

    Renal infarction (RI) is a rarely seen disorder, and the diagnosis is often missed. The two major causes of RI are thromboemboli originhating from a thrombus in the heart or aorta, and in-situ thrombosis of a renal artery. We report a case of RI that developed due to renal artery and vein thrombosis, as confirmed by pathological evaluation of the nephrectomy material, three weeks after renal cyst aspiration.

  6. Imaging chronic renal disease and renal transplant in children

    Energy Technology Data Exchange (ETDEWEB)

    Carmichael, Jim; Easty, Marina [Great Ormond Street Hospital, Radiology Department, London (United Kingdom)

    2010-06-15

    At Great Ormond Street Hospital we have the highest number of paediatric renal transplant patients in Europe, taking cases from across the United Kingdom and abroad. Our caseload includes many children with rare complicating medical problems and chronic renal failure related morbidity. This review aims to provide an overview of our experience of imaging children with chronic renal failure and transplants. (orig.)

  7. Renal sympathetic denervation: MDCT evaluation of the renal arteries.

    LENUS (Irish Health Repository)

    Hutchinson, Barry D

    2013-08-01

    Percutaneous transluminal renal sympathetic denervation is a new treatment of refractory systemic hypertension. The purpose of this study was to assess the clinical utility of MDCT to evaluate the anatomic configuration of the renal arteries in the context of renal sympathetic denervation.

  8. Screening renal stone formers for distal renal tubular acidosis

    DEFF Research Database (Denmark)

    Osther, P J; Hansen, A B; Røhl, H F

    1989-01-01

    A group of 110 consecutive renal stone formers were screened for distal renal tubular acidosis (RTA) using morning fasting urinary pH (mfUpH) levels followed by a short ammonium chloride loading test in patients with levels above 6.0. In 14 patients (12.7%) a renal acidification defect was noted...

  9. Thrombotic gene polymorphisms and postoperative outcome after coronary artery bypass graft surgery

    Directory of Open Access Journals (Sweden)

    Zaim Cagin

    2011-09-01

    Full Text Available Abstract Background Emerging perioperative genomics may influence the direction of risk assessment and surgical strategies in cardiac surgery. The aim of this study was to investigate whether single nucleotide polymorphisms (SNP affect the clinical presentation and predispose to increased risk for postoperative adverse events in patients undergoing coronary artery bypass grafting surgery (CABG. Methods A total of 220 patients undergoing first-time CABG between January 2005 and May 2008 were screened for factor V gene G1691A (FVL, prothrombin/factor II G20210A (PT G20210A, angiotensin I-converting enzyme insertion/deletion (ACE-ins/del polymorphisms by PCR and Real Time PCR. End points were defined as death, myocardial infarction, stroke, postoperative bleeding, respiratory and renal insufficiency and event-free survival. Patients were compared to assess for any independent association between genotypes for thrombosis and postoperative phenotypes. Results Among 220 patients, the prevalence of the heterozygous FVL mutation was 10.9% (n = 24, and 3.6% (n = 8 were heterozygous carriers of the PT G20210A mutation. Genotype distribution of ACE-ins/del was 16.6%, 51.9%, and 31.5% in genotypes I/I, I/D, and D/D, respectively. FVL and PT G20210A mutations were associated with higher prevalence of totally occluded coronary arteries (p p = 0.002. ACE D/D genotype was associated with hypertension (p = 0.004, peripheral vascular disease (p = 0.006, and previous myocardial infarction (p = 0.007. Conclusions FVL and PT G20210A genotypes had a higher prevalence of totally occluded vessels potentially as a result of atherothrombotic events. However, none of the genotypes investigated were independently associated with mortality.

  10. Report of 2 cases with thrombotic thrombocytopenic purpura%血栓性血小板减少性紫癜2例报道

    Institute of Scientific and Technical Information of China (English)

    杨学敏; 董晨明; 王爱华

    2012-01-01

    目的 总结兰州大学第二医院2例血栓性血小板减少性紫癜(TTP)的所有实验检测资料.方法 回顾性分析与TTP诊断特征密切相关的实验室检测:血红蛋白(Hb)、血小板(PLT)、网织红细胞(RET)、裂片红细胞(FRC)、尿蛋白(PRO)、尿潜血(BLD)、乳酸脱氢酶(LDH)、总胆红素(TBil)、直接胆红素(DBil)、间接胆红素(IBil)、尿素氮(BUN),肌酐(Cr)、D二聚体(D-D)及骨髓检测,并归纳分析.结果 血浆、PLT、静丙输注无法改善PLT的严重减少;输注红细胞(RBC),Hb进行性下降;尿液PRO及BLD定性持续阳性;溶血检测中的RET、TBil、IBil及LDH持续增高;凝血检测中D-D持续不降;骨髓分析提示2例患者分别是增生性贫血与溶血性贫血并考虑Evans综合症.结论 对于TTP浅显认识导致临床诊治延误.回顾性总结TTP实验室检测及临床特征,发现有经典“五联征”,包括血小板减少症、微血管病性溶血性贫血、神经症状、肾脏损害及发热.%Objective To summarize the examination data of 2 cases with thrombotic thrombocytopenic purpura (TTP)in Lanzhou University Second Hospital. Methods The closely related diagnostic features of TTP were reviewed retrospectively:hemoglobin (Hb), platelet (PLT), reticulocyte (RET), fragments of red blood cell (FRC), urinary protein (PRO ), urinary occult blood (BLD ), lactate dehydrogenase (LDH), total bilirubin (Tbil), direct bilirubin (Dbil),indirect bilirubin (Ibil),urea nitrogen (BUN),creatinine (Cr),D-dimer (D-D)and bone marrow analysis. Results Intravenous infusing plasma, PLT and immunoglobulin could not improve the severe reducing of PLT. Intravenously infusing red blood cells (RBC), Hb declined progressively. PRO and BLD were persistently positive. RET,Tbil,Ibil and LDH of hemolysis test continued to rise. D-D of coagulation test continued not to reduce. Bone marrow analysis showed that 2 patients were proliferative anemia and hemolytic anemia with Evans

  11. Renal Tumor Biopsy Technique

    Institute of Scientific and Technical Information of China (English)

    Lei Zhang; Xue-Song Li; Li-Qun Zhou

    2016-01-01

    Objective:To review hot issues and future direction of renal tumor biopsy (RTB) technique.Data Sources:The literature concerning or including RTB technique in English was collected from PubMed published from 1990 to 2015.Study Selection:We included all the relevant articles on RTB technique in English,with no limitation of study design.Results:Computed tomography and ultrasound were usually used for guiding RTB with respective advantages.Core biopsy is more preferred over fine needle aspiration because of superior accuracy.A minimum of two good-quality cores for a single renal tumor is generally accepted.The use of coaxial guide is recommended.For biopsy location,sampling different regions including central and peripheral biopsies are recommended.Conclusion:In spite of some limitations,RTB technique is relatively mature to help optimize the treatment of renal tumors.

  12. Dyslipoproteinemia in renal transplantation.

    Directory of Open Access Journals (Sweden)

    Gunjotikar R

    1994-01-01

    Full Text Available Twenty-seven live related donor renal allograft recipients were evaluated for dyslipoproteinemia. Twenty-two patients received dual immunosuppression with prednisolone and azathioprine. Five patients received cyclosporin as well. Total cholesterol (Tch, triglycerides (TG, HDL cholesterol (HDLch, LDL cholesterol (LDLch and VLDL cholesterol (VLDLch levels were estimated. Fifteen (56% patients showed significant lipoprotein abnormalities. Renal allograft recipients showed significantly lower levels of Tch (p < 0.05 and LDLch (p < 0.05 and higher levels of TG (p < 0.005 and HDLch (p < 0.05. Diet and beta blockers did not influence lipoprotein levels. A significant negative correlation was noted between post-transplant duration and Tch, TG and VLDLch levels. Increased TG levels were associated with increase in weight and higher daily prednisolone dosage at the time of evaluation. The study confirms the existence of dyslipoproteinemia in renal allograft recipients.

  13. Renal (Kidney) Manifestations in TSC

    Science.gov (United States)

    ... International TSC Research Conference Text Size Get Involved RENAL (KIDNEY) MANIFESTATIONS IN TSC Download a PDF of ... sclerosis complex (TSC) will develop some form of renal (kidney) disease during their lifetime. There are three ...

  14. [Renal transplantation and urinary lithiasis].

    Science.gov (United States)

    Lechevallier, E; Saussine, C; Traxer, O

    2008-12-01

    Renal lithiasis in renal donors is rare. A renal stone in a donor, or in a renal transplant, is not a contraindication for harvesting nor transplantation. If possible, the stone must be removed at the time of the transplantation. The risk of lithiasis is increased in the renal transplant recipient, with a frequency of 2-6%. Metabolic abnormalities for lithiasis are frequent and can be induced by the immunosuppressive treatment, anticalcineurins. Lithiasis can have a poor prognosis in the renal recipient with a risk for infection or renal dysfunction. Small (renal transplant can be followed-up. Stones of 0.5-1.5cm need an extracorporeal lithotripsy with a previous safety JJ stent. Stones greater than 1.5cm can be treated by ureteroscopy or percutaneous surgery.

  15. Renal denervation and hypertension.

    Science.gov (United States)

    Schlaich, Markus P; Krum, Henry; Sobotka, Paul A; Esler, Murray D

    2011-06-01

    Essential hypertension remains one of the biggest challenges in medicine with an enormous impact on both individual and society levels. With the exception of relatively rare monogenetic forms of hypertension, there is now general agreement that the condition is multifactorial in nature and hence requires therapeutic approaches targeting several aspects of the underlying pathophysiology. Accordingly, all major guidelines promote a combination of lifestyle interventions and combination pharmacotherapy to reach target blood pressure (BP) levels in order to reduce overall cardiovascular risk in affected patients. Although this approach works for many, it fails in a considerable number of patients for various reasons including drug-intolerance, noncompliance, physician inertia, and others, leaving them at unacceptably high cardiovascular risk. The quest for additional therapeutic approaches to safely and effectively manage hypertension continues and expands to the reappraisal of older concepts such as renal denervation. Based on the robust preclinical and clinical data surrounding the role of renal sympathetic nerves in various aspects of BP control very recent efforts have led to the development of a novel catheter-based approach using radiofrequency (RF) energy to selectively target and disrupt the renal nerves. The available evidence from the limited number of uncontrolled hypertensive patients in whom renal denervation has been performed are auspicious and indicate that the procedure has a favorable safety profile and is associated with a substantial and presumably sustained BP reduction. Although promising, a myriad of questions are far from being conclusively answered and require our concerted research efforts to explore the full potential and possible risks of this approach. Here we briefly review the science surrounding renal denervation, summarize the current data on safety and efficacy of renal nerve ablation, and discuss some of the open questions that need

  16. Renal Artery Stent Outcomes

    Science.gov (United States)

    Murphy, Timothy P.; Cooper, Christopher J.; Matsumoto, Alan H.; Cutlip, Donald E.; Pencina, Karol M.; Jamerson, Kenneth; Tuttle, Katherine R.; Shapiro, Joseph I.; D’Agostino, Ralph; Massaro, Joseph; Henrich, William; Dworkin, Lance D.

    2016-01-01

    BACKGROUND Multiple randomized clinical trials comparing renal artery stent placement plus medical therapy with medical therapy alone have not shown any benefit of stent placement. However, debate continues whether patients with extreme pressure gradients, stenosis severity, or baseline blood pressure benefit from stent revascularization. OBJECTIVES The study sought to test the hypothesis that pressure gradients, stenosis severity, and/or baseline blood pressure affects outcomes after renal artery stent placement. METHODS Using data from 947 patients with a history of hypertension or chronic kidney disease from the largest randomized trial of renal artery stent placement, the CORAL (Cardiovascular Outcomes in Renal Atherosclerotic Lesions) study, we performed exploratory analyses to determine if subsets of patients experienced better outcomes after stent placement than the overall cohort. We examined baseline stenosis severity, systolic blood pressure, and translesion pressure gradient (peak systolic and mean) and performed interaction tests and Cox proportional hazards analyses for the occurrence of the primary endpoint through all follow-up, to examine the effect of these variables on outcomes by treatment group. RESULTS There were no statistically significant differences in outcomes based on the examined variables nor were there any consistent nonsignificant trends. CONCLUSIONS Based on data from the CORAL randomized trial, there is no evidence of a significant treatment effect of the renal artery stent procedure compared with medical therapy alone based on stenosis severity, level of systolic blood pressure elevation, or according to the magnitude of the transstenotic pressure gradient. (Benefits of Medical Therapy Plus Stenting for Renal Atherosclerotic Lesions [CORAL]; NCT00081731) PMID:26653621

  17. An unusual case of hematuria in a young female: renal artery embolism, mitral stenosis, and sinus rhythm

    Directory of Open Access Journals (Sweden)

    Ashok Kumar

    2016-01-01

    Full Text Available Renal artery embolism (RAE is an uncommon entity that is most often secondary to a cardiac source. Most reported cases have been in patients with underlying atrial fibrillation (AF, and occurrence of RAE, especially in patients with valvular heart disease, and sinus rhythm is very rare. We describe an unusual case of a young female who presented with sudden onset right flank pain, vomiting, anorexia, and hematuria, and was found to have thrombotic occlusion of the distal right renal artery. Although she denied any previous cardiac history, detailed cardiovascular examination revealed the presence of severe rheumatic mitral stenosis without any evidence of AF or left atrial clot. She was initially managed conservatively using low molecular weight heparin followed by oral anticoagulation with resolution of symptoms. A successful balloon mitral valvotomy was performed six weeks later. The patient is asymptomatic at her last follow-up of six months with preserved renal function. In symptomatic patients, clinicians need to consider the possibility of RAE even in patients of valvular heart disease with underlying sinus rhythm. Appropriate management of the underlying cardiac condition is imperative since embolism may be recurrent leading to compromise of renal function, if left untreated.

  18. Renal lithiasis and nutrition.

    Science.gov (United States)

    Grases, Felix; Costa-Bauza, Antonia; Prieto, Rafel M

    2006-09-06

    Renal lithiasis is a multifactorial disease. An important number of etiologic factors can be adequately modified through diet, since it must be considered that the urine composition is directly related to diet. In fact, the change of inappropriate habitual diet patterns should be the main measure to prevent kidney stones. In this paper, the relation between different dietary factors (liquid intake, pH, calcium, phosphate, oxalate, citrate, phytate, urate and vitamins) and each type of renal stone (calcium oxalate monohydrate papillary, calcium oxalate monohydrate unattached, calcium oxalate dihydrate, calcium oxalate dihydrate/hydroxyapatite, hydroxyapatite, struvite infectious, brushite, uric acid, calcium oxalate/uric acid and cystine) is discussed.

  19. Pediatric Renal Neoplasms.

    Science.gov (United States)

    Ranganathan, Sarangarajan

    2009-03-01

    Renal tumors in childhood consist of a diverse group of tumors ranging from the most common Wilms' tumor, to the uncommon and often fatal rhabdoid tumor. Diagnosis is based on morphologic features and aided by ancillary techniques such as immunohistochemistry and cytogenetics. Molecular techniques have helped identify a group of pediatric renal cell carcinomas that have specific translocations, called translocation-associated carcinomas. Differential diagnosis of the various tumors is discussed. Pathogenesis and nephroblastomatosis, the precursor lesions of Wilms tumor, also are discussed briefly, as are the handling of these tumor specimens and prognostic factors. Copyright © 2009 Elsevier Inc. All rights reserved.

  20. Gravidez e transplante renal

    OpenAIRE

    Andrade, Joana Rita Ferreira

    2014-01-01

    Enquadramento: A gravidez é rara em mulheres com Doença Renal Crónica, sobretudo em estadio avançado, em virtude de várias condicionantes como a disfunção ovárica, hemorragias vaginais anovulatórias e amenorreia. Contudo, após transplante renal é possível alimentar o sonho de constituir família, mas é preciso considerar os riscos aumentados para o enxerto e a maior susceptibilidade para complicações da gravidez. Objectivo: Avaliar os riscos e identificar as variáveis que influenciam o suce...

  1. Renal lithiasis and nutrition

    Directory of Open Access Journals (Sweden)

    Prieto Rafel M

    2006-09-01

    Full Text Available Abstract Renal lithiasis is a multifactorial disease. An important number of etiologic factors can be adequately modified trough diet, since it must be considered that the urine composition is directly related to diet. In fact, the change of inappropriate habitual diet patterns should be the main measure to prevent kidney stones. In this paper, the relation between different dietary factors (liquid intake, pH, calcium, phosphate, oxalate, citrate, phytate, urate and vitamins and each type of renal stone (calcium oxalate monohydrate papillary, calcium oxalate monohydrate unattached, calcium oxalate dihydrate, calcium oxalate dihydrate/hydroxyapatite, hydroxyapatite, struvite infectious, brushite, uric acid, calcium oxalate/uric acid and cystine is discussed.

  2. Branchio-oto-renal syndrome.

    Science.gov (United States)

    Jalil, Jawad; Basheer, Faisal; Shafique, Mobeen

    2014-05-01

    The association of branchial arch anomalies (branchial cysts, branchial fistulas), hearing loss and renal anomalies constitutes the branchio-oto-renal (BOR) syndrome also known as Melnick Fraser syndrome. We present a case of this rare disorder in a girl child who presented with profound deafness, preauricular pits, branchial sinuses and renal hypoplasia.

  3. Drug-induced renal disease.

    Science.gov (United States)

    Curtis, J R

    1979-11-01

    The clinical manifestations of drug-induced renal disease may include all the manifestations attributed to natural or spontaneous renal diseases such as acute renal failure, chronic renal failure, acute nephritic syndrome, renal colic, haematuria, selective tubular defects, obstructive nephropathy, etc. It is therefore vital in any patient with renal disease whatever the clinical manifestations might be, to obtain a meticulous drug and toxin inventory. Withdrawal of the offending drug may result in amelioration or cure of the renal disorder although in the case of severe renal failure it may be necessary to utilise haemodialysis or peritoneal dialysis to tide the patient over the period of acute renal failure. Analgesic nephropathy is an important cause of terminal chronic renal failure and it is therefore vital to make the diagnosis as early as possible. The pathogenesis of some drug-induced renal disorders appears to be immunologically mediated. There are many other pathogenetic mechanisms involved in drug-induced renal disorders and some drugs may under appropriate circumstances be responsible for a variety of different nephrotoxic effects. For example, the sulphonamides have been incriminated in examples of crystalluria, acute interstitial nephritis, acute tubular necrosis, generalised hypersensitivity reactions, polyarteritis nodosa and drug-induced lupus erythematosus.

  4. Management of chronic renal failure.

    NARCIS (Netherlands)

    de Zeeuw, D.; Apperloo, AJ; de Jong, P.

    1992-01-01

    There is growing evidence that treatment of patients with renal function impairment will undergo a major shift within the next few years. Along with more or less successful attempts to alleviate the signs and symptoms of reduced renal function, new insights into renal pathophysiology as well as new

  5. Successful Revascularization of an LCx CTO Lesion by Retrograde Approach From an Acute Thrombotic SVG Without Protection Device in an ACS Patient.

    Science.gov (United States)

    Lin, Mei Mei; Wang, Ji Hung

    2016-05-25

    We describe a patient who underwent coronary artery bypass grafting (CABG) surgery with the presentation of acute coronary syndrome (ACS). The diagnostic coronary angiogram showed acute thrombotic and occluded saphenous vein graft (SVG) and proximal right coronary artery (RCA) drug eluting stent (DES) instent restenosis (ISR) with chronic total occlusion (CTO). Our strategy was to recanalize the native left circumflex coronary artery (LCx) CTO instead of SVG or RCA instent CTO. After heparinization for 5 days, the LCx antegrade approach and the retrograde approach from left anterior descending coronary artery (LAD) septal branches were first attempted but failed, and the LCx CTO was successfully revascularized retrogradely via the acute thrombotic SVG without an embolic protection device (EPD).

  6. Woman with Sickle Cell Disease with Current Sigmoid Sinus Thrombosis and History of Inadequate Warfarin Use during a Past Thrombotic Event

    Directory of Open Access Journals (Sweden)

    Asuman Çelikbilek

    2009-05-01

    Full Text Available We report a 20-year-old woman with sickle cell disease (SCD who presented with a severe pulsating headache, nausea, and vomiting. Her history was significant for a past thrombotic event during which she had not used anticoagulation therapy as prescribed. Her mental status was mildly confused. On funduscopic examination, papilledema and retinal hemorrhages were found. Results of a computed tomogram were normal. A lumbar puncture demonstrated increased intracranial pressure (60 cm H2O. Magnetic resonance venography demonstrated a right sigmoid sinus thrombosis. Although SCD has been reported as a cause of thrombotic dural venous sinus events, this case increases the knowledge about neurological complications of SCD. The patient was treated with low molecular weight heparin, blood transfusions, acetazolamide, and methylprednisolone, and her symptoms and signs resolved.

  7. Staphylococcus aureus septicemia presenting as disseminated intravascular coagulation - thrombotic thrombocytopenic purpura overlap and thrombus in inferior vena cava, right atrium and right ventricle: a case report

    Directory of Open Access Journals (Sweden)

    Khwaja Saifullah Zafar

    2015-01-01

    Full Text Available Staphylococcal sepsis following furunculosis and complicated by suspected deep vein thrombosis and septic inferior vena caval, right atrium, right ventricle emboli accompanied by disseminated intravascular coagulation (DIC - thrombotic thrombocytopenic overlap in a 65 years old lady is presented. She was managed successfully with antibiotics and anticoagulation. The case is reported for its rarity and brings to light the vivid manifestations of septicemia specially staphylococcal. [Int J Res Med Sci 2015; 3(1.000: 368-372

  8. Myocardial contrast defect associated with thrombotic coronary occlusion: Pre-autopsy diagnosis of a cardiac death with post-mortem CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Heon; Cha, Jang Gyu [Dept. of Radiology, Soonchunhyang University Hospital, Bucheon (Korea, Republic of); Park, Hye Jin; Lee, Soo Kyoung; Yang, Kyung Moo [Dept. of Forensic Medicine, National Forensic Service, Wonju (Korea, Republic of)

    2015-10-15

    We report the case of a female who died of suspected acute myocardial infarction. Post-mortem CT angiography (PMCTA) was performed with intravascular contrast infusion before the standard autopsy, and it successfully demonstrated the complete thrombotic occlusion of a coronary artery and also a corresponding perfusion defect on myocardium. We herein describe the PMCTA findings of a cardiac death with special emphasis on the potential benefits of this novel CT technique in forensic practice.

  9. Residual vein thrombosis and onset of post-thrombotic syndrome: influence of the 4G/5G polymorphism of plasminogen activator inhibitor-1 gene.

    Science.gov (United States)

    Incalcaterra, Egle; Meli, Francesco; Muratori, Ida; Corrado, Egle; Amato, Corrado; Canino, Baldassare; Ferrara, Filippo

    2014-03-01

    Plasminogen activator inhibitor-1 (PAI-1) is the most important inhibitor of plasminogen activator. The functional 4G/5G polymorphism of the gene coding for PAI-1 may affect PAI-1 plasmatic activity, influencing the imbalance between coagulation and fibrinolysis cascades. In this prospective cohort analytic study, we investigated the role of this single nucleotide polymorphism in the persistence of thrombotic lesion and the occurrence of post-thrombotic syndrome. In a group of 168 patients with post-surgical deep vein thrombosis of the legs, we analyzed the 4G/5G polymorphism in the promoter of PAI-1 gene and plasmatic PAI-1 activity. Enrolled patients were divided in two groups: patients with 4G/5G polymorphism and increased PAI-1 activity (n=85) and patients without 4G/5G polymorphism and normal PAI-1 activity (n=83). All patients were treated according to current protocols and re-examined after 3, 12 and 36 months in order to evaluate the persistence of thrombotic lesion and the occurrence of post-thrombotic syndrome. We found a significantly increased PAI activity in carrier of the 4G allele, who experienced much more frequently a persistence of thrombosis after 3, 12 and 36 months and/or the development of post-thrombosis syndrome, in spite of the anticoagulant treatment. These data not only confirm the role played by PAI-1 activity and by the 4G/5G SNP of the PAI-1 gene, but also suggest that current therapeutic protocols, recommending the administration of low weight molecular heparin and oral anticoagulant for the treatment of deep vein thrombosis, could be non sufficient for patients genetically predisposed to a less efficient clot lysis. Copyright © 2013. Published by Elsevier Ltd.

  10. Acute Thrombotic Occlusion of the Popliteal Artery following Knee Dislocation: A Case Report of Management, Local Unit Practice, and a Review of the Literature

    Science.gov (United States)

    Hindi, Fadi; Ettles, Callum; Pemberton, Mark

    2017-01-01

    Arterial complications following traumatic knee injury are relatively rare but mandate timely recognition and treatment to avoid significant comorbidity and medicolegal ramifications. In this report we describe a case of acute thrombotic occlusion of the popliteal artery occurring after knee dislocation, successfully repaired by intimal fixation and a limited venous patch reconstruction. We present a review of local practice in screening vascular injuries following knee dislocation, aligned with a review of the literature and considerations for practice. PMID:28246569

  11. Pathology of diabetic myocardiac microangiopathy

    Institute of Scientific and Technical Information of China (English)

    Yiqun Yao; Yanmei Chen; Guang Liu; Zhiyong Pei

    2009-01-01

    Objective To investigate the cause of high cardiovascular lethality in patients with diabetics mellitus. Methods Sections from autopsied coronary arteries and myocardium of dead patients with non-insulin-dependent diabetics mellitus and 12 dead control subjects were used for histomorphometric studies. Results The coronary atherosclerotic lesion in diabetics patients was not different in severity from those in controls. Nor was there difference in number of myofibers or diameters of myocardic fibers and capillaries.But the capillary density and the ratio of capillary number to myocardic fiber number in diabetics group were significeantly reduced compared with control group(P<0.0 l),and the capillary basement membrane in the former was significantly thicker than in the latter(P<0.01).Conclision The decrease in number of capillaries and the thickening of basement membrane enhance myocardiac vulnerability to further ischemia and hypoxia,which may undelie high lethality of myocardiopathy in diabetic patients.

  12. [Hypertension and renal disease

    DEFF Research Database (Denmark)

    Kamper, A.L.; Pedersen, E.B.; Strandgaard, S.

    2009-01-01

    hypertension. Mild degrees of chronic kidney disease (CKD) can be detected in around 10% of the population, and detection is important as CKD is an important risk factor for atherosclerotic cardiovascular disease. Conversely, heart failure may cause an impairment of renal function. In chronic progressive...

  13. Insuficiencia renal aguda.

    Directory of Open Access Journals (Sweden)

    Carlos Hernán Mejía

    2009-10-01

    Full Text Available La insuficiencia renal aguda se diagnostica aproximadamente en 5% de los pacientes hospitalizados. Sus principales causas se relacionan con la alteración del flujo sanguíneo renal, sea por depleción de volumen, baja perfusión renal o por distribución intrarrenal inadecuada y obstrucción del árbol urinario. El diagnóstico parte de la historia clínica y un buen examen físico que corrobore el estado de volemia del paciente y se complementa con el uso adecuado de los índices urinarios (excreción de sodio y osmolaridad, el uroanálisis y la ecografía renal. Su tratamiento consiste en una adecuada recuperación del volumen, manejo de los diuréticos, soporte nutricional, conservación del equilibrio hidroelectrolítico y brindar terapia de diálisis si hay toxicidad urémica, hipercaliemia severa (>6.5 mEq/l, acidosis metabólica o sobrecarga severa de volumen.

  14. Management of Renal Cysts

    Science.gov (United States)

    Nalbant, Ismail; Can Sener, Nevzat; Firat, Hacer; Yeşil, Süleyman; Zengin, Kürşad; Yalcınkaya, Fatih; Imamoglu, Abdurrahim

    2015-01-01

    Background and Objectives: Renal cysts have a high prevalence in the general population, and their estimated incidence increases with age. Renal cyst aspiration (usually with sclerotherapy) or open/laparoscopic decortication is a generally effective and safe method in the treatment of symptomatic simple renal cysts. The success rates of laparoscopic decortication and percutaneous aspiration-sclerotherapy were compared to assist in the decision making for the procedure. Methods: A total of 184 patients with symptomatic simple renal cysts were treated with either laparoscopic decortication in 149 cases or percutaneous aspiration-sclerotherapy in 35 cases. The follow-up period was approximately 35 months, and the symptomatic and radiologic success rates of the 2 techniques were compared retrospectively. Results: Laparoscopic decortication was found to have high success rates, a low recurrence rate, and minimal morbidity. Percutaneous aspiration-sclerotherapy is an outpatient procedure with a minimally higher recurrence rate. Conclusion: When a symptomatic cyst is encountered and treatment of the cyst is indicated, laparoscopic decortication is a more efficient method that offers better results than percutaneous aspiration-sclerotherapy. PMID:25848184

  15. Rupture of Renal Transplant

    Directory of Open Access Journals (Sweden)

    Shona Baker

    2015-01-01

    Full Text Available Background. Rupture of renal allograft is a rare and serious complication of transplantation that is usually attributed to acute rejection, acute tubular necrosis, or renal vein thrombosis. Case Presentation. LD, a 26-year-old male with established renal failure, underwent deceased donor transplantation using kidney from a 50-year-old donor with acute kidney injury (Cr 430 mmol/L. LD had a stormy posttransplant recovery and required exploration immediately for significant bleeding. On day three after transplant, he developed pain/graft swelling and another significant haemorrhage with cardiovascular compromise which did not respond to aggressive resuscitation. At reexploration, the renal allograft was found to have a longitudinal rupture and was removed. Histology showed features of type IIa Banff 97 acute vascular rejection, moderate arteriosclerosis, and acute tubular necrosis. Conclusion. Possible ways of avoiding allograft rupture include use of well-matched, good quality kidneys; reducing or managing risk factors that would predispose to delayed graft function; ensuring a technically satisfactory transplant procedure with short cold and warm ischemia times; and avoiding large donor-recipient age gradients.

  16. Primary renal graft thrombosis

    NARCIS (Netherlands)

    Bakir, N; Sluiter, WJ; Ploeg, RJ; van Son, WJ; Tegzess, Adam

    1996-01-01

    Background. Renal allograft thrombosis is a serious complication of kidney transplantation that ultimately leads to graft loss. Its association with acute and hyperacute rejection is well documented; however, in a large proportion of patients the precise cause remains obscure. The exact incidence an

  17. Comparative incidence of a first thrombotic event in purely obstetric antiphospholipid syndrome with pregnancy loss: the NOH-APS observational study.

    Science.gov (United States)

    Gris, Jean-Christophe; Bouvier, Sylvie; Molinari, Nicolas; Galanaud, Jean-Philippe; Cochery-Nouvellon, Eva; Mercier, Erik; Fabbro-Peray, Pascale; Balducchi, Jean-Pierre; Marès, Pierre; Quéré, Isabelle; Dauzat, Michel

    2012-03-15

    The incidence of thrombosis in the purely obstetric form of antiphospholipid syndrome is uncertain. We performed a 10-year observational study of 1592 nonthrombotic women who had experienced 3 consecutive spontaneous abortions before the 10th week of gestation or 1 fetal death at or beyond the 10th week of gestation. We compared the frequencies of thrombotic events among women positive for antiphospholipid Abs (n = 517), women carrying the F5 6025 or F2 rs1799963 polymorphism (n = 279), and women with negative thrombophilia screening results (n = 796). The annual rates of deep vein thrombosis (1.46%; range, 1.15%-1.82%), pulmonary embolism (0.43%; range, 0.26%-0.66%), superficial vein thrombosis (0.44%; range, 0.28%-0.68%), and cerebrovascular events (0.32%; range, 0.18%-0.53%) were significantly higher in aPLAbs women than in the other groups despite low-dose aspirin primary prophylaxis. Women carrying 1 of the 2 polymorphisms did not experience more thrombotic events than women who screened negative for thrombophilia. Lupus anticoagulant was a risk factor for unprovoked proximal and distal deep and superficial vein thrombosis and women in the upper quartile of lupus anticoagulant activity had the highest risk. Despite data suggesting that aPLAbs may induce pregnancy loss through nonthrombotic mechanisms, women with purely obstetric antiphospholipid syndrome are at risk for thrombotic complications.

  18. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

  19. ``Aggressive`` renal angiomyolipoma

    Energy Technology Data Exchange (ETDEWEB)

    Cittadini, G. Jr. [Univ. of Genoa (Italy). Dept. of Radiology; Pozzi Mucelli, F. [Univ. of Trieste (Italy). Dept. of Radiology; Danza, F.M. [Catholic Sacro Cuore Univ., Rome (Italy). Dept. of Radiology; Derchi, L.E. [Univ. of Genoa (Italy). Dept. of Radiology; Pozzi Mucelli, R.S. [Univ. of Trieste (Italy). Dept. of Radiology

    1996-11-01

    We describe the US and CT examinations of 4 patients with renal angiomyolipoma with an `aggressive` appearance, and review the literature. The imaging findings in 4 patients with benign renal angiomyolipomas associated with thrombosis of the renal vein and/or inferior vena cava are presented. CT demonstrated fat densities within both tumor and thrombus. In one patient, small lymph nodes with low density internal areas were detected in the para-aortic region. When considering our patients together with those reported in the literature, we found that most angiomyolipomas with venous invasion were large and centrally located within the kidney. Venous thrombosis was observed in 9 lesions of the right kidney, and in only 4 of the left one. One patient only had symptoms due to the thrombus; 10 had problems due to the tumor; and 3 were asymptomatic. Only 4 patients with pararenal enlarged lymph nodes have been reported on in the imaging literature. Fat-containing nodes were detected by CT in one case only; the others had enlarged nodes of soft-tissue density. In one patient the diagnosis of hamartomatous lymph node invasion was established by angiography. In patients with renal angiomyolipoma, demonstration of both fatty thrombus and the fatty infiltration of lymph nodes of the renal hilum cannot be regarded as an indication of malignancy, but only of local aggessive behavior. Conservative treatment seems possible. Detection of enlarged lymph nodes of soft tissue density may cause difficult diagnostic problems, with the diagnosis addressed only by the presence of associated lesions. (orig./MG).

  20. Ex vivo diffusion tensor imaging and neuropathological correlation in a murine model of hypoxia-ischemia-induced thrombotic stroke.

    Science.gov (United States)

    Shereen, Ahmed; Nemkul, Niza; Yang, Dianer; Adhami, Faisal; Dunn, R Scott; Hazen, Missy L; Nakafuku, Masato; Ning, Gang; Lindquist, Diana M; Kuan, Chia-Yi

    2011-04-01

    Diffusion tensor imaging (DTI) is a powerful method to visualize white matter, but its use in patients with acute stroke remains limited because of the lack of corresponding histologic information. In this study, we addressed this issue using a hypoxia-ischemia (HI)-induced thrombotic model of stroke in adult mice. At 6, 15, and 24  hours after injury, animals were divided into three groups for (1) in vivo T2- and diffusion-weighted magnetic resonance imaging, followed by histochemistry, (2) ex vivo DTI and electron microscopy, and (3) additional biochemical or immunochemical assays. The temporal changes of diffusion anisotropy and histopathology were compared in the fimbria, internal capsule, and external capsule. We found that HI caused a rapid reduction of axial and radial diffusivities in all three axonal bundles. A large decrease in fractional anisotropy, but not in axial diffusivity per se, was associated with structural breakdown of axons. Furthermore, the decrease in radial diffusivity correlated with swelling of myelin sheaths and compression of the axoplasma. The gray matter of the hippocampus also exhibited a high level of diffusion anisotropy, and its reduction signified dendritic degeneration. Taken together, these results suggest that cross-evaluation of multiple DTI parameters may provide a fuller picture of axonal and dendritic injury in acute ischemic stroke.

  1. Preparation and characterization of a novel aspirin derivative with anti-thrombotic and gastric mucosal protection properties.

    Directory of Open Access Journals (Sweden)

    Xi-E Zhen

    Full Text Available The use of acetylsalicylic acid (ASP is limited by its adverse effects, especially the effect on the gastric mucosa. To address this problem, we synthesized a derivative form of ASP, prepared by modification of ASP with nano-hydroxyapatite (a kind of inorganic particle containing Ca(2+. The derivative was named Ca-ASP. Structural study showed that Ca-ASP was a kind of carboxylate containing intramolecular hydrogen bonds. Rats given a high dose of Ca-ASP (5 mmol per kg body weight showed similar anti-thrombotic activity as those given the same dose of ASP, but had much lower gastric mucosal damage than ASP (UI: 2 versus UI: 12.5. These rats also showed reduced expression of COX-2, but their COX-1 expression was similar to that of control rats, but significantly higher than that of ASP-administered rats. Furthermore, the level of prostaglandin E2 (PGE2 was up-regulated in Ca-ASP-administered rats compared to ASP-administered rats. Taken together, the results showed that Ca-ASP possessed similar antithrombotic activity as ASP but without the side effect associated with ASP, and the underlying mechanism may center on inhibiting COX-2 without inhibiting COX-1, and thus favouring the production of PGE2, the prostaglandin that plays a vital role in the suppression of platelet aggregation and thrombosis, as well as in the repair of gastric damage.

  2. Chronic renal disease in pregnancy.

    Science.gov (United States)

    Ramin, Susan M; Vidaeff, Alex C; Yeomans, Edward R; Gilstrap, Larry C

    2006-12-01

    The purpose of this review was to examine the impact of varying degrees of renal insufficiency on pregnancy outcome in women with chronic renal disease. Our search of the literature did not reveal any randomized clinical trials or meta-analyses. The available information is derived from opinion, reviews, retrospective series, and limited observational series. It appears that chronic renal disease in pregnancy is uncommon, occurring in 0.03-0.12% of all pregnancies from two U.S. population-based and registry studies. Maternal complications associated with chronic renal disease include preeclampsia, worsening renal function, preterm delivery, anemia, chronic hypertension, and cesarean delivery. The live birth rate in women with chronic renal disease ranges between 64% and 98% depending on the severity of renal insufficiency and presence of hypertension. Significant proteinuria may be an indicator of underlying renal insufficiency. Management of pregnant women with underlying renal disease should ideally entail a multidisciplinary approach at a tertiary center and include a maternal-fetal medicine specialist and a nephrologist. Such women should receive counseling regarding the pregnancy outcomes in association with maternal chronic renal disease and the effect of pregnancy on renal function, especially within the ensuing 5 years postpartum. These women will require frequent visits and monitoring of renal function during pregnancy. Women whose renal disease is further complicated by hypertension should be counseled regarding the increased risk of adverse outcome and need for blood pressure control. Some antihypertensives, especially angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers, should be avoided during pregnancy, if possible, because of the potential for both teratogenic (hypocalvaria) and fetal effects (renal failure, oliguria, and demise).

  3. CUTANEOUS MANIFESTATIONS OF CHRONIC RENAL FAILURE AND RENAL TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    R. Suganya Gnanadeepam

    2017-07-01

    Full Text Available BACKGROUND The kidney and the skin are the two large networks of the body with abundant blood supply associated with various cutaneous manifestations. This study aims to detect the various cutaneous manifestations and its incidence in patients with chronic renal failure and renal transplantation. MATERIALS AND METHODS This study was done for a period of 1 year from January 2016 to December 2016 at Nephrology OPD ward and Medicine wards, Government KAPV Medical College Hospital, Trichy. During this period, 100 patients who had the presence of skin manifestations were selected and studied (80 renal failure patients and 20 renal transplantation patients. RESULTS Most of the specific cutaneous manifestations of chronic renal failure and renal transplantation were noted in this study. Pruritus and xerosis were the most common manifestations noted in chronic renal failure while infections was commonly noted in renal transplantation patients. CONCLUSION Pruritus and xerosis were the most common among the specific cutaneous manifestations in chronic renal failure followed by nail abnormalities and pigmentary changes. Cutaneous manifestations of renal transplantation were mostly due to infections of which fungal infection is the most common followed by viral infection.

  4. Transarterial embolization for serious renal hemorrhage following renal biopsy.

    Science.gov (United States)

    Zeng, Dan; Liu, Guihua; Sun, Xiangzhou; Zhuang, Wenquan; Zhang, Yuanyuan; Guo, Wenbo; Yang, Jianyong; Chen, Wei

    2013-01-01

    The goal of this study is to evaluate the feasibility and efficacy of percutaneous transarterial embolization for the treatment of serious renal hemorrhage after renal biopsy. Nine patients with renal hemorrhage had frank pain and gross hematuria as main symptoms after renal biopsy. Intrarenal arterial injuries and perinephric hematoma were confirmed by angiography in all cases. The arterial injuries led to two types of renal hemorrhage, Type I: severe renal injure or intrarenal renal artery rupture (n=5), with contrast medium spilling out of the artery and spreading into renal pelvis or kidney capsule in angiography; Type II, pseudo aneurysm or potential risk of intrarenal artery injure (n=4), where contrast medium that spilled out of intraartery was retained in the parenchyma as little spots less than 5 mm in diameter in angiography. Transcatheter superselective intrarenal artery embolization was performed with coils or microcoils (Type I intrarenal artery injure) and polyvinyl alcohol particles (Type II injure). The intrarenal arterial injuries were occluded successfully in all patients. Light or mild back or abdominal pain in the side of the embolized kidney was found in three patients following embolization procedures and disappeared 3 days later. Serum creatinine and perinephric hematoma were stable, and gross hematuresis stopped immediately (n=4) or 3-5 days (n=3) after embolization. In conclusions, transcatheter superselective intrarenal artery embolization as a minimally invasive therapy is safe and effective for treatment of serious renal hemorrhage following percutaneous renal biopsy.

  5. Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl

    Directory of Open Access Journals (Sweden)

    Tahar Gargah

    2012-01-01

    Full Text Available The hemolytic uremic syndrome (HUS, characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure, represents one of the major causes of acute renal failure in infancy and childhood. The typical form occurring after an episode of diarrhea caused by Escherichia coli is the most frequent in children. Other microorganisms also may be responsible for HUS, such as Streptococcus pneumoniae, which causes more severe forms of the disease. We report an 8-month-old girl who presented with pneumonia and subsequently developed HUS. Renal biopsy showed characteristic lesion of thrombotic microangiopathy and extensive cortical necrosis. She was managed with peritoneal dialysis but did not improve and developed severe sepsis due to staphylococcal peritonitis, resulting in the death of the patient. Streptococcus pneumoniae-induced HUS is uncommon, but results in severe disease in the young. There is a high risk of these patients developing end-stage kidney disease in the long term.

  6. Problemas renales de la cirrosis Renal problems of cirrhosis

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    Alvaro García

    1992-02-01

    Full Text Available Presentamos una revisión actualizada y condensada acerca de los problemas renales más relevantes que ocurren en la cirrosis tales como las alteraciones en el manejo del sodio y del agua, el tratamiento de la ascitis y el edema y el enfoque de la falla renal que ocurre en esta enfermedad, es decir síndrome hepato-renal y necrosis tubular aguda.

    We present a condensed and updated review on the most common renal problems occurring in cirrhosis such as the handling of sodium and water, the treatment of ascites and edema and the approach to the renal failure that frequently takes place in this disease, namely hepato-renal syndrome and acute tubular necrosis.

  7. MR imaging findings of renal infarction induced by renal artery

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jun Woo; Kim, Suck; Kim, Yong Woo; Hu, Jin Sam; Choi, Sang Yoel; Moon, Tae Yong; Lee, Suck Hong; Kim, Byung Su; Lee, Chang Hun [Pusan National Univ., Pusan (Korea, Repulic of). Coll. of Medicine

    1998-02-01

    To assess the usefulness of diffusion-weighted imaging (DWI) in evaluating serial parenchymal changes in renal infarction induced by renal artery ligation, by comparing this with the conventional spin echo technique and correlating the results with the histopathological findings. In 22 rabbits, renal infarction was induced by ligation of the renal artery. Spin-echo T1-weighted imaging (T1WI), turbo spin-echo (TSE) T2-weighted imaging (T2WI), and DWI were performed, using a 1.5-T superconductive unit, at 30 minutes, 1 hour, 2,3,6, 12 and 24 hours, and 2, 3, 7 and 20 days after left renal artery ligation. Changes in signal intensity on T1WI, T2WI, and DWI were correlated with histopathologic findings. Diffusion-weighted imaging is useful for the detection of hyperacute renal infarction, and the apparent diffusion coefficient may provide additional information concerning its evolution. (author). 21 refs., 9 figs.

  8. Renal subcapsular haematoma: an unusual complication of renal artery stenting

    Institute of Scientific and Technical Information of China (English)

    XIA Dan; CHEN Shan-wen; ZHANG Hong-kun; WANG Shuo

    2011-01-01

    After successful renal artery angioplasty and stent placement, a patient in a fully anticoagulated state developed hypotension and flank pain. Computed tomography (CT) of the abdomen revealed a large renal subcapsular haematoma which was successfully managed conservatively without embolotherapy and surgical intervention. To prevent hemorrhage after renal artery stenting, it is necessary to underscore the importance of reducing the contrast volume and pressure of angiography, controlling systemic blood pressure, and monitoring guide wire position at all times.

  9. Dopamins renale virkninger

    DEFF Research Database (Denmark)

    Olsen, Niels Vidiendal

    1990-01-01

    Dopamine is an endogenic catecholamine which, in addition to being the direct precursor of noradrenaline, has also an effect on peripheral dopaminergic receptors. These are localized mainly in the heart, splanchnic nerves and the kidneys. Dopamine is produced in the kidneys and the renal metaboli...... dialysis unnecessary in a number of patients on account of increased diuresis and natriuresis. The effect of GFR and the significance for the prognosis are not known....

  10. Renal lithiasis and nutrition

    OpenAIRE

    Prieto Rafel M; Costa-Bauza Antonia; Grases Felix

    2006-01-01

    Abstract Renal lithiasis is a multifactorial disease. An important number of etiologic factors can be adequately modified trough diet, since it must be considered that the urine composition is directly related to diet. In fact, the change of inappropriate habitual diet patterns should be the main measure to prevent kidney stones. In this paper, the relation between different dietary factors (liquid intake, pH, calcium, phosphate, oxalate, citrate, phytate, urate and vitamins) and each type of...

  11. Nutrition and renal disease.

    Directory of Open Access Journals (Sweden)

    Iris de Castaño

    2009-11-01

    Full Text Available Kidney plays an important roll in body homeostasis through excretory, metabolic and endocrine functions. Kidneys filter fluids and solutes and reabsorbed water , electrolytes an minerals. Urine volume and solute excretion are adjusted to keep composition of the extracellular space, serum osmolarity and intravascular volume in constant balance. Kidneys also regulate acid base equilibrium, hormone metabolism and excretion and amino acid concentration. Vitamin D hydroxylation takes place in the kidney, this is the active form of this vitamin, which inhibits PTH. In addition they produce erythropoietin which control hemoglobin concentration in erythrocytes. When renal insufficiency develops, and glormerular filtration rate is between 50 to 75% of normal, this functions are decreased .When renal function is less than 10%, this functions ceased. In children small changes in water, solute, acid base, calcium and phosphorus can alter normal growth and development. If kidneys can not maintain internal equilibrium, specific nutrients should be used. Compensation should be done according to age, type or renal disease and level of glomerular filtration rate.

  12. A second hit for TMA

    National Research Council Canada - National Science Library

    Miyata, Toshiyuki; Fan, Xinping

    2012-01-01

    In this issue of Blood, Fuchs and colleagues provide evidence that circulating DNA and histones, presumably released from neutrophils, would be the second hit for development of thrombotic microangiopathies (TMAs...

  13. TMA: beware of complements.

    Science.gov (United States)

    Ricklin, Daniel; Cines, Douglas B

    2013-09-19

    In this issue of Blood, Jodele and colleagues report that defective complement regulation contributes to the development of thrombotic microangiopathy (TMA) after hematopoietic stem cell transplantation (HSCT) with important implications for diagnosis and management of this severe clinical complication.

  14. Renal artery aneurysm mimicking renal calculus with hydronephrosis.

    Science.gov (United States)

    Chen, Shanwen; Meng, Hongzhou; Cao, Min; Shen, Baihua

    2013-06-01

    A 51-year-old woman was found to have a left renal calculus with hydronephrosis. She underwent unsuccessful extracorporeal shock wave lithotripsy, leading to the recommendation that percutaneous lithotomy was necessary to remove the renal calculus. In view of the unusual shape of the calculus and absence of abnormalities in urine sediment, preoperative computed tomography and renal angiography were performed, which instead showed a calcified left renal artery aneurysm. Subsequent efforts to perform an aneurysmectomy also failed, eventually necessitating left nephrectomy. This case illustrates the pitfalls in the diagnosis of a renal artery aneurysm, which is a relatively common condition that may have unusual presentations. Hence, it is suggested that the possibility of a renal artery aneurysm be considered in the differential diagnosis when one detects a renal calculus with an unusual appearance. In addition, we propose that 3-dimensional reconstruction computed tomography be performed before considering surgical options for such renal calculi to rule out the possibility of a renal artery aneurysm.

  15. Renal scintigraphy in infants with antenatally diagnosed renal pelvis dilatation

    Directory of Open Access Journals (Sweden)

    Ajdinović Boris

    2008-01-01

    Full Text Available Background/Aim. Ureteropelvic junction obstruction and vesicoureteral reflux are the most frequent entities identified on the basis of antenatal hydronephrosis. The aim of this study was to determine the incidence and pattern of abnormal renal scintigraphy findings in postnatal investigation of children with antenatal hydronephrosis. Methods. Twenty four infants (19 boys and five girls presented with antenatal hydronephrosis and mild to moderate hydronephrosis on ultrasound in newborn period were referred for renal scintigraphy. Ten patients with vesicoureteral reflux documented on micturating cystoureterography underwent 99mTc-DMSA renal scintigraphy and 14 patients were subjected to 99mTc-DTPA scintigraphy. Results. Anteroposterior pelvic diameter on ultrasound ranged from 11 to 24 mm. Renal DMSA scans identified congenital scars in two boys with bilateral reflux of grade V and unilateral reflux of grade III. Relative kidney uptake (RKU less than 40% was found in three, and poor kidney function (RKU less than 10% in two patients. Significant obstruction was shown on DTPA diuretic renal scintigraphy in 6/14 patients. Some slowing in dranaige (T1/2 greater than 10 minutes with no reduction in differential renal function was identified in three patients. Differential renal function less than 10% was obtained in one case. Conclusion. A high percent of abnormal renal scintigraphy findings was obtained. Renal scintigraphy was useful in determination of underlying cause of antenatally detected hydronephrosis.

  16. Efficacy of ultrasonography-guided renal biopsy for the evaluation of renal dysfunction following renal transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Jae; Choi, Chul Soon; Min, Seon Jeong; Lee, Gyung Kyu; Lee, Eil Seong; Kang, Ik Won; Bae, Sang Hoon [Hallym University College of Medicine, Chuncheon (Korea, Republic of)

    2003-12-15

    To evaluate the usefulness and complications of renal biopsy under ultrasonography-guidance in renal dysfunction after renal transplantation. Ultrasonography-guided renal biopsy was done in 47 patients with the transplanted kidney. The subjects consisted of 30 males and 17 females, age ranged from 16 to 66 years (average age=38 years). Biopsies were done once in 27 patients, twice in 17 patients, three times in 3 patients, a total of 70 biopsies. The success rate of renal biopsy for the accurate pathologic diagnosis and the incidence and types of complications following biopsy were evaluated. The success rate of renal biopsy for the accurate pathologic diagnosis was 96%(67/70). Pathologic diagnosis included 27 cases of acute rejection (39%), 8 cases of acute tubular necrosis (11%), 4 cases of acute rejection and acute tubular necrosis (6%), 4 cases of cyclosporin toxicity (6%), 4 cases of primary disease recurrence (6%), 4 cases of infection (6%) and others. Complications after renal biopsy included 15 cases of microscopic hematuria (21%), 1 case of gross hematuria with spontaneous cessation and 1 case of life threatening hemorrhage. Ultrasonography-guided renal biopsy is a safe and effective diagnostic method for the evaluation of renal dysfunction following renal transplantation.

  17. Trasplante renal Kidney transplant

    Directory of Open Access Journals (Sweden)

    P. Martín

    2006-08-01

    Full Text Available El trasplante renal es la terapia de elección para la mayoría de las causas de insuficiencia renal crónica terminal porque mejora la calidad de vida y la supervivencia frente a la diálisis. El trasplante renal de donante vivo es una excelente alternativa para el paciente joven en situación de prediálisis porque ofrece mejores resultados. El tratamiento inmunosupresor debe ser individualizado buscando la sinergia inmunosupresora y el mejor perfil de seguridad, y debe adaptarse a las diferentes etapas del trasplante renal. En el seguimiento del trasplante renal hay que tener muy en cuenta los factores de riesgo cardiovascular y los tumores puesto que la muerte del paciente con injerto funcionante es la segunda causa de pérdida del injerto tras el primer año del trasplante. La función alterada del injerto es un factor de mortalidad cardiovascular independiente que requerirá seguimiento y control de todas sus complicaciones para retrasar la entrada en diálisis.The kidney transplant is the therapy of choice for the majority of the causes of chronic terminal kidney insufficiency, because it improves the quality of life and survival in comparison with dialysis. A kidney transplant from a live donor is an excellent alternative for the young patient in a state of pre-dialysis because it offers the best results. Immunosuppressive treatment must be individualised, seeking immunosuppressive synergy and the best safety profile, and must be adapted to the different stages of the kidney transplant. In the follow-up to the kidney transplant, cardiovascular risk factors and tumours must be especially taken into account, given that the death of the patient with a working graft is the second cause of loss of the graft following the first year of the transplant. The altered function of the graft is a factor of independent cardiovascular mortality that will require follow-up and the control of all its complications to postpone the entrance in dialysis.

  18. The effects of Endothelial Protein C Receptor Gene Polymorphisms on sEPCR levels in Venous Thrombotic Patients

    Directory of Open Access Journals (Sweden)

    Afife Karabıyık

    2012-03-01

    Full Text Available OBJECTIVE: The aim of this study was to investigate the detection of the variations in the EPCR gene which has a possible role for thrombosis and the effects of this variations on the plasma sEPCR levels in Turkish population. METHODS: This study included 111 thrombotic patients and 73 healthy controls. Following DNA extraction, PCR, SSCP and DNA sequencing analysis of four exons of the EPCR gene were performed. Plasma sEPCR levels were measured with Enyzme linked immunosorbent assay (ELISA. RESULTS: Three polymorphisms were detected in exon 1 through exon 4. The C3998T (SNP no: rs2069952 is located within intron 2 and the C4678G (A1 haplotype (SNP no: rs9574 is located within 3’ untranslated region (3’UTR. There was no significant differences between C3998T polymorphism in control and patients. There was no significant difference in sEPCR levels in subjects carrying A1 allele. A4600G substitution (A3 haplotype (SNP no: rs867186 was found in exon 4 and it brought 2.04 fold risk for thrombosis. A3 allele carriers had high sEPCR levels, compared to no A3 allele carriers. The mean sEPCR level in patients having A3 allele homozygous is 289 ng /ul and A1 allele homozygous is 113,42 ng/ul. CONCLUSION: A1 haplotype might have a protective effect on thrombosis and A3 haplotype may be associated both with increased plasma sEPCR levels and with an increased risk of venous thrombosis in Turkish population. sEPCR levels were significantly higher in subjects carrying A3 allele (4600A>G in both patients and controls. There was no significant difference in sEPCR levels in subjects carrying A1 allele (4678C>G.

  19. Assessment of Novel Anti-thrombotic Fusion Proteins for Inhibition of Stenosis in a Porcine Model of Arteriovenous Graft.

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    Christi M Terry

    Full Text Available Hemodialysis arteriovenous synthetic grafts (AVG provide high volumetric blood flow rates shortly after surgical placement. However, stenosis often develops at the vein-graft anastomosis contributing to thrombosis and early graft failure. Two novel fusion proteins, ANV-6L15 and TAP-ANV, inhibit the tissue factor/factor VIIa coagulation complex and the factor Xa/factor Va complex, respectively. Each inhibitor domain is fused to an annexin V domain that targets the inhibitor activity to sites of vascular injury to locally inhibit thrombosis. This study's objective was to determine if these antithrombotic proteins are safe and effective in inhibiting AVG stenosis.A bolus of either TAP-ANV or ANV-6L15 fusion protein was administered intravenously immediately prior to surgical placement of a synthetic graft between the external jugular vein and common carotid artery in a porcine model. At surgery, the vein and artery were irrigated with the anti-thrombotic fusion protein. Control animals received intravenous heparin. At 4 weeks, MRI was performed to evaluate graft patency, the pigs were then euthanized and grafts and attached vessels were explanted for histomorphometric assessment of neointimal hyperplasia at the vein-graft anastomosis. Blood was collected at surgery, immediately after surgery and at euthanasia for serum metabolic panels and coagulation chemistries.No acute thrombosis occurred in the control group or in either experimental group. No abnormal serum chemistries, activated clotting times or PT, PTT values were observed after treatment in experimental or control animals. However, at the vein-graft anastomosis, there was no difference between the control and experimental groups in cross-sectional lumen areas, as measured on MRI, and no difference in hyperplasia areas as determined by histomorphometry. These results suggest that local irrigation of TAP-ANV or ANV-6L15 intra-operatively was as effective in inhibiting acute graft thrombosis

  20. Haemostatic aspects of renal transplantation.

    Science.gov (United States)

    Sørensen, P J; Schmidt, E B; Knudsen, F; Nielsen, A H; Kristensen, S D; Dyerberg, J; Kornerup, H J

    1988-01-01

    Platelet function and protein C activity and antigen level was studied in 31 renal transplant recipients and 10 healthy controls. The patients were divided into three groups: (I) cyclosporin treated, (II) azathioprine treated, and (III) azathioprine treated patients with chronic rejection. The platelet function in the renal transplant patients was normal and there was no difference between groups I and II. The specific activity of protein C was decreased in patients after renal transplantation and decreasing protein C activity and progressive renal failure was found to be positively correlated in the azathioprine treated groups.

  1. Renal replacement therapy in ICU

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    C Deepa

    2012-01-01

    Full Text Available Diagnosing and managing critically ill patients with renal dysfunction is a part of the daily routine of an intensivist. Acute kidney insufficiency substantially contributes to the morbidity and mortality of critically ill patients. Renal replacement therapy (RRT not only does play a significant role in the treatment of patients with renal failure, acute as well as chronic, but also has spread its domains to the treatment of many other disease conditions such as myaesthenia gravis, septic shock and acute on chronic liver failure. This article briefly outlines the role of renal replacement therapy in ICU.

  2. Renal myxoma: a case report

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    Carlos Henrique C Souza

    2015-04-01

    Full Text Available Myxomas are rare tumors that can appear in many anatomical locations. There are only 14 cases of renal involvement documented in the literature. This article reports a case of renal myxoma in an elderly woman with recurrent cystitis. After five years of follow-up, the computed tomography (CT revealed a large solid tumor mass in the left kidney. Tumor resection was performed preserving the affected kidney with histopathological diagnosis of renal myxoma. The objective of this study is to report a rare case of renal myxoma, emphasizing the importance of the differential diagnosis from other benign and malignant mesenchymal tumors.

  3. Sporotrichosis in Renal Transplant Patients

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    Paulo Gewehr

    2013-01-01

    Full Text Available The current report describes two renal transplant recipients who presented with sporotrichosis. In addition, the authors review the general aspects of sporotrichosis in renal transplant recipients reported in the literature. Sporotrichosis is a rare fungal infection in transplant patients and has been reported primarily in renal transplant recipients not treated with antifungal prophylaxis. Extracutaneous forms of sporotrichosis without skin manifestations and no previous history of traumatic injuries have been described in such patients and are difficult to diagnose. Renal transplant recipients with sporotrichosis described in the present report were successfully treated with antifungal therapy including amphotericin B deoxycholate, lipid amphotericin B formulations, fluconazole and itraconazole.

  4. CT features of renal infarction

    Energy Technology Data Exchange (ETDEWEB)

    Suzer, Okan; Shirkhoda, Ali; Jafri, S. Zafar; Madrazo, Beatrice L.; Bis, Kostaki G.; Mastromatteo, James F

    2002-10-01

    Purpose: To demonstrate the different patterns of renal infarction to avoid pitfalls. To present 'flip-flop enhancement' pattern in renal infarction. Materials and methods: Retrospective review of a total of 41 renal infarction in 37 patients were done. These patients underwent initial CT and the diagnosis of renal infarction was confirmed with either follow up CT or at surgery. Results: Twenty-three patients had wedge-shaped focal infarcts, nine patients had global and five patients had multifocal infarcts of the kidneys. Cortical rim sign was seen predominantly with global infarcts. In five patients, a 'flip-flop enhancement' pattern was observed. In two patients, planned renal biopsies due to tumefactive renal lesions were cancelled because of 'flip-flop enhancement' pattern on follow up CTs. Conclusion: Although most of our cases were straightforward for the diagnosis of renal infarction, cases with tumefactive lesions and global infarctions without the well-known cortical rim sign were particularly challenging. We describe a new sign, flip-flop enhancement pattern, which we believe solidified the diagnosis of renal infarction in five of our cases. The authors recommend further investigations for association of flip-flop enhancement and renal infarction.

  5. Bilateral renal infarction following atrial fibrillation and thromboembolism and presenting as acute abdominal pain: a case report

    Directory of Open Access Journals (Sweden)

    Bouassida Khaireddine

    2012-06-01

    Full Text Available Abstract Introduction Renal infarct is rare and often misdiagnosed because the symptoms are misleading. The mechanisms are various, mainly thrombotic and embolic. Case presentation In this review, we report the case of a 61-year-old Tunisian woman presented to the emergency unit with a 4-hour history of abdominal pain diffused at both flanks, ultrasounds was performed to remove a surgical emergency, showed a peri-renal fluid collection with heterogeneous parenchyma. We followed by a CT scan, which confirmed the diagnosis of renal infarct. The patient was treated by heparin at a curative dose, and the outcome was favorable. Conclusion Diagnosis is difficult and should be considered in patients with inexplicable flank or abdominal pain and with risk factors to this disease. Our purpose is to raise clinician’s awareness for this condition so that they will be more likely to diagnose it. This will facilitate prompt diagnosis and treatment. A review of the literature was performed and the case is discussed in the context of the current knowledge of this condition.

  6. Renal transplantation experience in a patient with factor V Leiden homozygous, MTHFR C677T heterozygous, and PAI heterozygous mutation.

    Science.gov (United States)

    Gülhan, Bora; Tavil, Betül; Gümrük, Fatma; Aki, Tuncay F; Topaloglu, Rezan

    2015-08-01

    Vascular complications are important causes of allograft loss in renal transplantation. A two and a half-month-old boy was diagnosed with posterior urethral valve and progressed to end-stage renal disease at eight yr of age. During the HD period, a central venous catheter was replaced three times for repeated thrombosis. The boy was found to be homozygous for FVL and heterozygous for both MTHFR (C677T) and PAI. At the age of 12, renal transplantation was performed from a deceased donor. Postoperative anticoagulation therapy was initiated with continuous intravenous administration of heparin at the dose of 10 IU/kg/h. HD was performed for the first three days. By the fourth day of transplantation, his urine output had increased gradually. Heparin infusion was continued for 18 days during hospitalization at the same dosage. Thereafter, he was discharged with LMWH. On the third month after transplantation, his serum creatinine level was 1.1 mg/dL and eGFR was 75.7 mL/min/1.73 m(2). He has still been using LMWH, and his eGFR was 78.7 mL/min/1.73 m(2) eight months after transplantation. Postoperative low-dose heparin treatment is a safe strategy for managing a patient with multiple thrombotic risk factors. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Hemorrhagic Fever with Renal Syndrome (HFRS)

    Science.gov (United States)

    ... this page: About CDC.gov . Share Compartir Hemorrhagic Fever with Renal Syndrome (HFRS) On this Page What ... is HFRS prevented? Suggested Reading What is hemorrhagic fever with renal syndrome? Hemorrhagic fever with renal syndrome ( ...

  8. Renal cirsoid arteriovenous malformation masquerading as neoplasia.

    Science.gov (United States)

    Silverthorn, K; George, D

    1988-12-01

    A woman with renal colic and microscopic hematuria had filling defects in the left renal collecting system detected on excretory urography. A nephrectomy, performed because of suspected malignancy, might have been averted by renal angiography.

  9. Tumor Seeding With Renal Cell Carcinoma After Renal Biopsy

    OpenAIRE

    M.F.B. Andersen; Norus, T.P.

    2016-01-01

    Tumor seeding following biopsy of renal cell carcinoma is extremely rare with an incidence of 1:10.000. In this paper two cases with multiple recurrent RRC metastasis in the biopsy tract following biopsy of renal tumor is presented and the current literature is shortly discussed.

  10. Renal blood flow in experimental septic acute renal failure

    NARCIS (Netherlands)

    Langenberg, C.; Wan, L.; Egi, M.; May, C. N.; Bellomo, R.

    2006-01-01

    Reduced renal blood flow (RBF) is considered central to the pathogenesis of septic acute renal failure (ARF). However, no controlled experimental studies have continuously assessed RBF during the development of severe septic ARF. We conducted a sequential animal study in seven female Merino sheep. F

  11. Renal vein oxygen saturation in renal artery stenosis

    DEFF Research Database (Denmark)

    Nielsen, K; Rehling, M; Henriksen, Jens Henrik Sahl

    1992-01-01

    Renal vein oxygen-saturation was measured in 56 patients with arterial hypertension and unilateral stenosis or occlusion of the renal artery. Oxygen-saturation in blood from the ischaemic kidney (84.4%, range 73-93%) was significantly higher than that from the 'normal' contralateral kidney (81...

  12. The renal scan in pregnant renal transplant patients

    Energy Technology Data Exchange (ETDEWEB)

    Goldstein, H.A.; Ziessman, H.A.; Fahey, F.H.; Collea, J.V.; Alijani, M.R.; Helfrich, G.B.

    1985-05-01

    With the greater frequency of renal transplant surgery, more female pts are becoming pregnant and carrying to term. In the renal allograft blood vessels and ureter may be compressed resulting in impaired renal function and/or, hypertension. Toxemia of pregnancy is seen more frequently than normal. Radionuclide renal scan monitoring may be of significant value in this high risk obstetrical pt. After being maintained during the pregnancy, renal function may also deteriorate in the post partum period. 5 pregnant renal transplant pts who delivered live babies had renal studies with Tc-99m DTPA to assess allograft perfusion and function. No transplanted kidney was lost during or after pregnancy as a result of pregnancy. No congenital anomalies were associated with transplant management. 7 studies were performed on these 5 pts. The 7 scans all showed the uterus/placenta. The bladder was always distorted. The transplanted kidney was rotated to a more vertical position in 3 pts. The radiation dose to the fetus is calculated at 0.024 rad/mCi administered. This study demonstrates the anatomic and physiologic alterations expected in the transplanted kidney during pregnancy when evaluated by renal scan and that the radiation burden may be acceptable in management of these pts.

  13. Renal posttransplant's vascular complications

    Directory of Open Access Journals (Sweden)

    Bašić Dragoslav

    2003-01-01

    Full Text Available INTRODUCTION Despite high graft and recipient survival figures worldwide today, a variety of technical complications can threaten the transplant in the postoperative period. Vascular complications are commonly related to technical problems in establishing vascular continuity or to damage that occurs during donor nephrectomy or preservation [13]. AIM The aim of the presenting study is to evaluate counts and rates of vascular complications after renal transplantation and to compare the outcome by donor type. MATERIAL AND METHODS A total of 463 kidneys (319 from living related donor LD and 144 from cadaveric donor - CD were transplanted during the period between June 1975 and December 1998 at the Urology & Nephrology Institute of Clinical Centre of Serbia in Belgrade. Average recipients' age was 33.7 years (15-54 in LD group and 39.8 (19-62 in CD group. Retrospectively, we analyzed medical records of all recipients. Statistical analysis is estimated using Hi-squared test and Fischer's test of exact probability. RESULTS Major vascular complications including vascular anastomosis thrombosis, internal iliac artery stenosis, internal iliac artery rupture obliterant vasculitis and external iliac vein rupture were analyzed. In 25 recipients (5.4% some of major vascular complications were detected. Among these cases, 22 of them were from CD group vs. three from LD group. Relative rate of these complications was higher in CD group vs. LD group (p<0.0001. Among these complications dominant one was vascular anastomosis thrombosis which occurred in 18 recipients (17 from CD vs. one from LD. Of these recipients 16 from CD lost the graft, while the rest of two (one from each group had lethal outcome. DISCUSSION Thrombosis of renal allograft vascular anastomosis site is the most severe complication following renal transplantation. In the literature, renal allograft thrombosis is reported with different incidence rates, from 0.5-4% [14, 15, 16]. Data from the

  14. [Renal abnormalities in ankylosing spondylitis].

    Science.gov (United States)

    Samia, Barbouch; Hazgui, Faiçal; Abdelghani, Khaoula Ben; Hamida, Fethi Ben; Goucha, Rym; Hedri, Hafedh; Taarit, Chokri Ben; Maiz, Hedi Ben; Kheder, Adel

    2012-07-01

    We will study the epidemiologic, clinical, biological, therapeutic, prognostic characteristics and predictive factors of development of nephropathy in ankylosing spondylitis patients. We retrospectively reviewed the medical record of 32 cases with renal involvement among 212 cases of ankylosing spondylitis followed in our service during the period spread out between 1978 and 2006. The renal involvement occurred in all patients a mean of 12 years after the clinical onset of the rheumatic disease. Thirty-two patients presented one or more signs of renal involvement: microscopic hematuria in 22 patients, proteinuria in 23 patients, nephrotic syndrome in 11 patients and decreased renal function in 24 patients (75%). Secondary renal amyloidosis (13 patients), which corresponds to a prevalence of 6,1% and tubulointerstitial nephropathy (7 patients) were the most common cause of renal involvement in ankylosing spondylitis followed by IgA nephropathy (4 patients). Seventeen patients evolved to the end stage renal disease after an average time of 29.8 ± 46 months. The average follow-up of the patients was 4,4 years. By comparing the 32 patients presenting a SPA and renal disease to 88 with SPA and without nephropathy, we detected the predictive factors of occurred of nephropathy: tobacco, intense inflammatory syndrome, sacroileite stage 3 or 4 and presence of column bamboo. The finding of 75% of the patients presented a renal failure at the time of the diagnosis of renal involvement suggests that evidence of renal abnormality involvement should be actively sought in this disease. Copyright © 2011 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

  15. Renal metabolism of calcitonin

    Energy Technology Data Exchange (ETDEWEB)

    Simmons, R.E.; Hjelle, J.T.; Mahoney, C.; Deftos, L.J.; Lisker, W.; Kato, P.; Rabkin, R.

    1988-04-01

    The kidneys account for approximately two-thirds of the metabolism of calcitonin, but relatively little is known regarding the details thereof. To further characterize this process, we examined the renal handling and metabolism of human calcitonin (hCT) by the isolated perfused rat kidney. We also studied the degradation of radiolabeled salmon calcitonin (sCT) by subcellular fractions prepared from isolated rabbit proximal tubules. The total renal (organ) clearance of immunoreactive hCT by the isolated kidney was 1.96 +/- 0.18 ml/min. This was independent of the perfusate total calcium concentration from 5.5 to 10.2 mg/dl. Total renal clearance exceeded the glomerular filtration rate (GFR, 0.68 +/- 0.05 ml/min), indicating filtration-independent removal. Urinary calcitonin clearance as a fraction of GFR averaged 2.6%. Gel filtration chromatography of medium from isolated kidneys perfused with /sup 125/I-labeled sCT showed the principal degradation products to be low molecular weight forms eluting with monoiodotyrosine. Intermediate size products were not detected. In the subcellular fractionation experiments, when carried out at pH 5.0, calcitonin hydrolysis exclusively followed the activities of the lysosomal enzyme N-acetyl-beta-glucosaminidase. Typically, at pH 7.5, 42% of total degradation occurred in the region of the brush-border enzyme alanyl aminopeptidase and 29% occurred in the region of the cytosolic enzyme phosphoglucomutase. Although 9% of the calcitonin-degrading activity was associated with basolateral membrane fractions, most of this activity could be accounted for by the presence of brush-border membranes.

  16. Citrato y litiasis renal

    Directory of Open Access Journals (Sweden)

    Elisa E. Del Valle

    2013-08-01

    Full Text Available El citrato es un potente inhibidor de la cristalización de sales de calcio. La hipocitraturia es una alteración bioquímica frecuente en la formación de cálculos de calcio en adultos y especialmente en niños. El pH ácido (sistémico, tubular e intracelular es el principal determinante de la excreción de citrato en la orina. Si bien la mayoría de los pacientes con litiasis renal presentan hipocitraturia idiopática, hay un número de causas para esta anormalidad que incluyen acidosis tubular renal distal, hipokalemia, dietas ricas en proteínas de origen animal y/o dietas bajas en álcalis y ciertas drogas, como la acetazolamida, topiramato, IECA y tiazidas. Las modificaciones dietéticas que benefician a estos pacientes incluyen: alta ingesta de líquidos y frutas, especialmente cítricos, restricción de sodio y proteínas, con consumo normal de calcio. El tratamiento con citrato de potasio es efectivo en pacientes con hipocitraturia primaria o secundaria y en aquellos desordenes en la acidificación, que provocan un pH urinario persistentemente ácido. Los efectos adversos son bajos y están referidos al tracto gastrointestinal. Si bien hay diferentes preparaciones de citrato (citrato de potasio, citrato de sodio, citrato de potasio-magnesio en nuestro país solo está disponible el citrato de potasio en polvo que es muy útil para corregir la hipocitraturia y el pH urinario bajo, y reducir marcadamente la recurrencia de la litiasis renal.

  17. CHILDHOOD BODY MASS INDEX AND ADULT PRO-INFLAMMATORY AND PRO-THROMBOTIC RISK FACTORS; DATA FROM THE NEW DELHI BIRTH COHORT

    Science.gov (United States)

    Lakshmy, Ramakrishnan; Fall, Caroline HD; Sachdev, Harshpal Singh; Osmond, Clive; Prabhakaran, Dorairaj; Biswas, Sushant Dey; Tandon, Nikhil; Ramji, Siddharth; Reddy, K Srinath; Barker, David JP; Bhargava, Santosh K

    2012-01-01

    Objective Weight gain and growth in early life may influence adult pro-inflammatory and pro-thrombotic cardiovascular risk factors. Methods Follow-up of a birth cohort in New Delhi, India, whose weight and height were measured 6-monthly until age 21 years. BMI at birth, during infancy (2 years), childhood (11 years) and adulthood (26-32 years) and BMI gain between these ages were analyzed in 886 men and 640 women in relation to adult fibrinogen, high-sensitivity C-reactive protein (hsCRP) and plasminogen activator inhibitor (PAI-1) concentrations. Results All the pro-inflammatory/pro-thrombotic risk factors were higher in participants with higher adiposity. In women, BMI at birth and age 2 years was inversely related to fibrinogen (p=0.002 and 0.05) and, after adjusting for adult adiposity, to hsCRP (p=0.02 and 0.009). After adjusting for adult adiposity, BMI at 2 years was inversely related to hsCRP and PAI-1 concentrations (p<0.001 and p=0.02) in men. BMI gain between 2-11 years and/or 11 years to adulthood was positively associated with fibrinogen and hsCRP in women and with hsCRP and PAI-1 in men. Conclusions Thinness at birth or during infancy, and accelerated BMI gain during childhood/adolescence are associated with a pro-inflammatory/pro-thrombotic state in adult life. An altered inflammatory state could be one link between small newborn/infant size and adult cardiovascular disease. Associations between pro-inflammatory markers and childhood/adolescent BMI gain are probably mediated through adult adiposity. PMID:20660641

  18. Renal calculus disease.

    Science.gov (United States)

    Schulsinger, D A; Sosa, R E

    1998-03-01

    We have seen an explosion in technical innovations for the management of urolithiasis. Today, the endourologist possesses an assortment of minimally invasive tools to treat renal stones. Most patients receive fast, safe and effective treatment in the outpatient setting. Despite the many technical advances, however, anatomical malformations and complex stones still provide significant challenges in diagnosis, access to a targeted stone, fragmentation, and clearance of the resulting fragments. This review examines a variety of urinary stone presentations and treatment strategies for cost-effective management.

  19. [Pulmonary-renal syndrome].

    Science.gov (United States)

    Risso, Jorge A; Mazzocchi, Octavio; De All, Jorge; Gnocchi, César A

    2009-01-01

    The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbidity-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  20. Role of Erythropoietin in Renal Anemia Therapy

    African Journals Online (AJOL)

    Keywords: Chronic renal failure, Renal anemia, Erythropoietin resistance. Tropical Journal of ... gastrointestinal reaction, which can increase the iron utilization and improve iron reserves, overcoming the reticuloendothelial system iron.