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https://www.readbyqxmd.com/read/28718268/oxymorphone-induced-thrombotic-microangiopathy-mimicking-atypical-haemolytic-uremic-syndrome
#1
Amibhen Gandhi, Saad Ullah, Shani Kotadia, Samer Nasser
Atypical Haemolytic Uremic Syndrome (aHUS) is a rare life threatening entity characterized by thrombocytopenia, haemolytic anaemia and renal dysfunction. It is a thrombotic microangiopathy related to genetic mutations in the alternate complement pathway and has a distinct pathophysiology which makes it harder to distinguish from other microangiopathies. We present a case of a 25-year-old male patient with history of polysubstance abuse who presented with chest pain and dyspnoea. He admitted to using injectable oxymorphone (Opana) two weeks before presentation...
April 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28701203/discovery-of-a-novel-dominant-mutation-in-the-ren-gene-after-forty-years-of-renal-disease-a-case-report
#2
Rhian L Clissold, Helen C Clarke, Olivera Spasic-Boskovic, Kim Brugger, Stephen Abbs, Coralie Bingham, Charles Shaw-Smith
BACKGROUND: Heterozygous mutations in the gene encoding renin (REN) cause autosomal dominant tubulointerstitial kidney disease (ADTKD), early-onset anaemia and hyperuricaemia; only four different mutations have been described in the published literature to date. We report a novel dominant REN mutation discovered in an individual after forty years of renal disease. CASE PRESENTATION: A 57 year old Caucasian woman with chronic kidney disease stage five was reviewed in a regional joint renal genetics clinic...
July 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28684442/impact-of-the-left-ventricular-mass-index-on-the-outcomes-of-severe-aortic-stenosis
#3
Eri Minamino-Muta, Takao Kato, Takeshi Morimoto, Tomohiko Taniguchi, Moriaki Inoko, Tetsuya Haruna, Toshiaki Izumi, Shoichi Miyamoto, Eisaku Nakane, Kenichi Sasaki, Moritoshi Funasako, Koji Ueyama, Shinichi Shirai, Takeshi Kitai, Chisato Izumi, Kazuya Nagao, Tsukasa Inada, Eiji Tada, Akihiro Komasa, Katsuhisa Ishii, Naritatsu Saito, Ryuzo Sakata, Kenji Minatoya, Takeshi Kimura
OBJECTIVE: To elucidate the factors associated with high left ventricular mass index (LVMI) and to test the hypothesis that high LVMI is associated with worse outcome in severe aortic stenosis (AS). METHODS: We analysed 3282 patients with LVMI data in a retrospective multicentre registry enrolling consecutive patients with severe AS in Japan. The management strategy, conservative or initial aortic valve replacement (AVR), was decided by the attending physician. High LVMI was defined as LVMI >115 g/m(2) for males and >95 g/m(2) for females...
July 6, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28646482/clinical-serological-and-histological-determinants-of-patient-and-renal-outcome-in-anca-associated-vasculitis-with-renal-involvement-an-analysis-from-a-referral-centre
#4
Matija Crnogorac, Ivica Horvatic, Luka Toric, Danica Galesic Ljubanovic, Miroslav Tisljar, Krešimir Galesic
PURPOSE: To evaluate significance of clinical and histopathological prognostic factors for renal and patient outcome in AAV patient cohort. METHODS: Retrospective study included consecutive patients diagnosed with pauci-immune crescentic glomerulonephritis from January 2003 to December 2013. Primary outcome was combined endpoint patient death or progression to end-stage renal disease (ESRD). Secondary outcomes were patient survival and progression to ESRD (renal survival) singularly and disease relapse...
August 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28645776/selective-inhibition-of-flt3-by-gilteritinib-in-relapsed-or-refractory-acute-myeloid-leukaemia-a-multicentre-first-in-human-open-label-phase-1-2-study
#5
Alexander E Perl, Jessica K Altman, Jorge Cortes, Catherine Smith, Mark Litzow, Maria R Baer, David Claxton, Harry P Erba, Stan Gill, Stuart Goldberg, Joseph G Jurcic, Richard A Larson, Chaofeng Liu, Ellen Ritchie, Gary Schiller, Alexander I Spira, Stephen A Strickland, Raoul Tibes, Celalettin Ustun, Eunice S Wang, Robert Stuart, Christoph Röllig, Andreas Neubauer, Giovanni Martinelli, Erkut Bahceci, Mark Levis
BACKGROUND: Internal tandem duplication mutations in FLT3 are common in acute myeloid leukaemia and are associated with rapid relapse and short overall survival. The clinical benefit of FLT3 inhibitors in patients with acute myeloid leukaemia has been limited by rapid generation of resistance mutations, particularly in codon Asp835 (D835). We aimed to assess the highly selective oral FLT3 inhibitor gilteritinib in patients with relapsed or refractory acute myeloid leukaemia. METHODS: In this phase 1-2 trial, we enrolled patients aged 18 years or older with acute myeloid leukaemia who either were refractory to induction therapy or had relapsed after achieving remission with previous treatment...
June 20, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28638625/early-eculizumab-use-in-atypical-haemolytic-uraemic-syndrome-in-a-jehovah-s-witness-refusing-blood-products
#6
May Al-Shaghana, Andrew Bentall, Mark D Jesky, William Lester, Graham Lipkin
Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah's Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28620089/development-and-validation-of-risk-prediction-equations-to-estimate-survival-in-patients-with-colorectal-cancer-cohort-study
#7
Julia Hippisley-Cox, Carol Coupland
Objective To develop and externally validate risk prediction equations to estimate absolute and conditional survival in patients with colorectal cancer. Design Cohort study.Setting General practices in England providing data for the QResearch database linked to the national cancer registry.Participants 44 145 patients aged 15-99 with colorectal cancer from 947 practices to derive the equations. The equations were validated in 15 214 patients with colorectal cancer from 305 different QResearch practices and 437 821 patients with colorectal cancer from the national cancer registry...
June 15, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28602181/health-outcomes-and-costs-for-injured-young-people-hospitalised-with-and-without-chronic-health-conditions
#8
Rebecca J Mitchell, Kate Curtis, Jeffrey Braithwaite
BACKGROUND: The prevalence of chronic health conditions such as diabetes among young people is increasing. Limited information is known about the impact of these conditions on young people who have been traumatically injured. Injury is the global leading cause of death and disability in young people. The aim of this study is to compare health outcomes for injured young people with and without chronic health conditions. METHOD: A retrospective examination of injury in young people aged≤25years with and without a chronic health condition using linked hospitalisation and mortality records during 1 January 2010 to 30 June 2014 in New South Wales, Australia...
June 8, 2017: Injury
https://www.readbyqxmd.com/read/28596204/malignant-hypertension-as-a-rare-cause-of-thrombotic-microangiopathy
#9
Guramrinder Thind, Karthik Kailasam
Malignant hypertension can occasionally be associated with microangiopathic haemolytic anaemia. A 38-year-old male presented with nausea, vomiting, loss of appetite and oliguria for 2 weeks. He was diagnosed with hypertensive emergency with cardiac and renal dysfunction. Interestingly, further workup was diagnostic for the presence of thrombotic microangiopathy (TMA): haemoglobin =12.7 g/dL, indirect bilirubin =2.0 mg/dL, haptoglobin ≤6 mg/dL, platelet count =121 000/μL and schistocytes on peripheral smear...
June 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28592199/diffuse-alveolar-haemorrage-as-initial-presentation-of-systemic-lupus-erythematosus-a-case-report
#10
M E Cucuzza, S D Marino, L Schiavone, P Smilari, F Filosco, P Barone
Diffuse alveolar haemorrhage (DAH) is a rare life-threatening complication of systemic lupus erythematosus (SLE), associated with high mortality rates. It usually occurs in patients with an established diagnosis of SLE. It has been reported as the initial presentation of SLE in 11-20% of cases. It occurs most frequently in females. We describe the case of a child, aged 14 years, with fever, asthenia, haemoptysis, dyspnea, anaemia, increased inflammatory markers, positivity to ANA, nDNA, direct Coombs tests, anticardiolipin antibodies and complement factors consumption...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28582636/living-with-the-burden-of-relapse-in-multiple-myeloma-from-the-patient-and-physician-perspective
#11
Cyrille Hulin, Timon Hansen, Louise Heron, Rachel Pughe, Matthew Streetly, Ananda Plate, Sue Perkins, Kate Morgan, Antoine Tinel, Filipe Rodrigues, Karthik Ramasamy
Multiple myeloma (MM) is a progressive plasma cell malignancy, with a range of clinical features including bone lesions, renal insufficiency, anaemia, and hypercalcaemia. Novel agents have significantly improved patient survival, however most patients will suffer multiple relapses. Although clinical challenges and economic costs of relapse are recognised, the psychological impact of relapse is not fully appreciated. Additionally, there is little information on how physicians perceive the impact of relapse on their patients' emotional state and how this might affect patient management...
May 31, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28466968/clinical-and-genetic-predictors-of-renal-dysfunctions-in-sickle-cell-anaemia-in-cameroon
#12
Amy Geard, Gift D Pule, Bernard Chetcha Chemegni, Valentina J Ngo Bitoungui, Andre P Kengne, Emile R Chimusa, Ambroise Wonkam
Micro-albuminuria and glomerular hyperfiltration are primary indicators of renal dysfunctions in Sickle Cell Disease (SCD), with more severe manifestations previously associated with variants in APOL1 and HMOX1 among African Americans. We have investigated 413 SCD patients from Cameroon. Anthropometric variables, haematological indices, crude albuminuria, albumin-to-creatinine ratio (ACR) and estimated glomerular filtration rate (eGFR) were measured. Patients were genotyped for 3·7 kb alpha-globin gene (HBA1/HBA2) deletion, and for variants in APOL1 (G1/G2; rs60910145, rs73885319, rs71785313) and HMOX1 (rs3074372, rs743811)...
May 3, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28462544/efficacy-of-c-e-r-a-in-routine-clinical-practice-for-correction-of-anaemia-and-maintenance-of-the-haemoglobin-levels-in-ckd-patients-not-on-dialysis
#13
P J Promod, R Deshpande, N K Mohanty, S Kulkarni, H A Shah, A Ganju, A Kukreja, S Joshi
INTRODUCTION: C.E.R.A. reported effective correction of anaemia and was well tolerated in International studies on CKD patients not on dialysis. OBJECTIVE: The study aimed to describe the management of renal anaemia in CKD patients not on dialysis with C.E.R.A. in routine clinical practice in India. METHODS: This was a prospective, single-arm, open-label, multi-centre, non-interventional, Phase IV study which followed 108 CKD Stage III-IV patients, not on dialysis with Hb < 10 g/dL for correction of anaemia with C...
March 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28438219/adverse-drug-reactions-and-kinetics-of-cisplatin-excretion-in-urine-of-patients-undergoing-cisplatin-chemotherapy-and-radiotherapy-for-head-and-neck-cancer-a-prospective-study
#14
Marília Berlofa Visacri, Eder de Carvalho Pincinato, Graziele Baldan Ferrari, Júlia Coelho França Quintanilha, Priscila Gava Mazzola, Carmen Silvia Passos Lima, Patricia Moriel
BACKGROUND: Cisplatin is a high-potency anticancer agent; however, it causes significant adverse drug reactions (ADRs). Potential pharmacokinetic markers must be studied to predict or prevent cisplatin-induced ADRs and achieve better prognosis. This study was designed to investigate the relationship between ADRs and kinetics of cisplatin excretion in the urine of patients undergoing high-dose cisplatin chemotherapy and radiotherapy for head and neck cancer. METHODS: Outpatients with head and neck cancer received a first cycle of high-dose cisplatin chemotherapy (80-100 mg/m(2)) concurrent to radiotherapy...
April 24, 2017: Daru: Journal of Faculty of Pharmacy, Tehran University of Medical Sciences
https://www.readbyqxmd.com/read/28421565/iron-homeostasis-in-inflammation-a-single-centre-prospective-observational-study-in-medical-inpatients
#15
Carl Chrobak, Jan Adam Sidler, Alix O'Meara, Sabine Schaedelin, Balthasar L Hug
AIMS OF THE STUDY: We aimed to assess a potential association of iron status with mortality and morbidity of inpatients with systemic inflammation. METHODS: This was a single centre prospective observational study. From April 2014 to October 2014, all consecutive medical inpatients aged >=18 years with a C-reactive protein value >5 mg/l on hospital admission were eligible for the study. We excluded pregnant women and patients with terminal renal insufficiency or past allogeneic stem cell transplantation...
April 19, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28395880/treatment-of-metastatic-uveal-melanoma-with-adoptive-transfer-of-tumour-infiltrating-lymphocytes-a-single-centre-two-stage-single-arm-phase-2-study
#16
Smita S Chandran, Robert P T Somerville, James C Yang, Richard M Sherry, Christopher A Klebanoff, Stephanie L Goff, John R Wunderlich, David N Danforth, Daniel Zlott, Biman C Paria, Arvind C Sabesan, Abhishek K Srivastava, Liqiang Xi, Trinh H Pham, Mark Raffeld, Donald E White, Mary Ann Toomey, Steven A Rosenberg, Udai S Kammula
BACKGROUND: Uveal melanoma is a rare tumour with no established treatments once metastases develop. Although a variety of immune-based therapies have shown efficacy in metastatic cutaneous melanoma, their use in ocular variants has been disappointing. Recently, adoptive T-cell therapy has shown salvage responses in multiple refractory solid tumours. Thus, we sought to determine if adoptive transfer of autologous tumour-infiltrating lymphocytes (TILs) could mediate regression of metastatic uveal melanoma...
June 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28374163/carboplatin-instead-of-cisplatin-in-combination-with-dexamethasone-high-dose-cytarabine-with-or-without-rituximab-dhac-r-is-an-effective-treatment-with-low-toxicity-in-hodgkin-s-and-non-hodgkin-s-lymphomas
#17
B Tessoulin, P Thomare, E Delande, J Moynard, T Gastinne, A Moreau, C Bossard, B Mahé, N Blin, V Dubruille, C Touzeau, J S Boudreault, F Perrin, A Lok, T Guillaume, A Garnier, P Peterlin, P Gallas, P Chevallier, P Moreau, Steven Le Gouill
The DHAP regimen (high-dose cytarabine in combination with dexamethasone and cisplatin) with or without rituximab (DHAP+/-R) is one of the most common regimens in daily practice. It is considered the standard treatment for relapse or refractory Hodgkin's and non-Hodgkin's lymphoma (NHL). Cisplatin nephrotoxicity is a major concern, and other platinum compounds are being tried. We performed a monocentric retrospective analysis to evaluate the use of carboplatin, so-called DHAC+/-R regimen. The purpose was to assess the toxicity of the DHAC+/-R regimen in real-life...
April 3, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28361568/heterogeneous-impact-of-body-mass-index-on-in-hospital-mortality-in-acute-heart-failure-syndromes-an-analysis-from-the-attend-registry
#18
Akiomi Yoshihisa, Takamasa Sato, Katsuya Kajimoto, Naoki Sato, Yasuchika Takeishi
BACKGROUND: Although the obesity paradox may vary depending upon clinical background factors such as age, gender, aetiology of heart failure and comorbidities, the reasons underlying the heterogeneous impact of body mass index (BMI) on in-hospital cardiac mortality under various conditions in patients with acute heart failure syndromes (AHFSs) remain unclear. METHODS: Among 4617 hospitalised patients with AHFSs enrolled in the Acute Decompensated Heart Failure Syndromes (ATTEND) registry, the patient characteristics and in-hospital cardiac mortality rates in those with low BMI (BMI <25 kg/m(2), n = 3263) were compared to those with high BMI (BMI ⩾25 kg/m(2), n = 1354)...
March 1, 2017: European Heart Journal. Acute Cardiovascular Care
https://www.readbyqxmd.com/read/28357112/immunoglobulin-g4-related-disease-a-rare-steroid-responsive-disease
#19
Vorawut Thanthitaweewat, Poonchavist Chantranuwatana, Naricha Chirakalwasan
A 70-year-old man presented with progressive dyspnoea and weight loss. Physical examination revealed only mild pale conjunctiva. The workup showed mild anaemia, mild impaired renal function, and high globulin level. Multiple myeloma was excluded by normal serum protein electrophoresis. The chest radiography and computed tomography (CT) revealed bilateral multifocal patchy infiltration with mediastinal adenopathy. Bronchoscopy was performed. Bronchoalveolar lavage (BAL) fluid examination was negative for infection and malignancy...
May 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28347544/ocular-involvement-in-atypical-haemolytic-uraemic-syndrome
#20
A Sampedro López, B Domínguez Moro, J M Baltar Martin, C Garcia Monteavaro, J J Barbón García
CASE REPORT: The case is presented of a young man with an atypical haemolytic-uraemic syndrome (aHUS), complicated with bilateral serous retinal detachment, cotton wool spots, and a branch artery occlusion. Treatment with plasmapheresis, haemodialysis and systemic eculizumab led to the blood and urine parameters returning to normal, as well as resolution of the retinal anomalies. Genetic analysis show both mutations in complement factor H and C3. DISCUSSION: Haemolytic-uraemic syndrome (HUS) is a thrombotic microangiopathy characterised by microangiopathic haemolytic anaemia, thrombocytopenia, and acute renal failure...
March 24, 2017: Archivos de la Sociedad Española de Oftalmología
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