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https://www.readbyqxmd.com/read/28339660/eculizumab-in-secondary-atypical-haemolytic-uraemic-syndrome
#1
Teresa Cavero, Cristina Rabasco, Antía López, Elena Román, Ana Ávila, Ángel Sevillano, Ana Huerta, Jorge Rojas-Rivera, Carolina Fuentes, Miquel Blasco, Ana Jarque, Alba García, Santiago Mendizabal, Eva Gavela, Manuel Macía, Luis F Quintana, Ana María Romera, Josefa Borrego, Emi Arjona, Mario Espinosa, José Portolés, Carolina Gracia-Iguacel, Emilio González-Parra, Pedro Aljama, Enrique Morales, Mercedes Cao, Santiago Rodríguez de Córdoba, Manuel Praga
Background.: Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. Methods.: We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 10 9 /L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration...
February 20, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28291603/proliferative-kidney-disease-in-rainbow-trout-oncorhynchus-mykiss-under-intensive-breeding-conditions-pathogenesis-and-haematological-and-immune-parameters
#2
Miroslava Palikova, Ivana Papezikova, Zdenka Markova, Stanislav Navratil, Jan Mares, Lukas Mares, Libor Vojtek, Pavel Hyrsl, Eva Jelinkova, Heike Schmidt-Posthaus
Proliferative kidney disease (PKD) is an endoparasitic disease of salmonid fish caused by Tetracapsuloides bryosalmonae (Myxozoa: Malacosporea). This study presents a comprehensive view on PKD development in rainbow trout (Oncorhynchus mykiss) reared at an intensive fish breeding facility, with focus on mortality, pathology/histopathology, haematological findings and immune functions. Diseased and reference fish were sampled monthly and time course of natural infection was followed up from the onset of clinical signs (September 2014) to full recovery (January 2015)...
March 6, 2017: Veterinary Parasitology
https://www.readbyqxmd.com/read/28272834/urate-oxidase-for-the-prevention-and-treatment-of-tumour-lysis-syndrome-in-children-with-cancer
#3
REVIEW
Daniel Kl Cheuk, Alan Ks Chiang, Godfrey Cf Chan, Shau Yin Ha
BACKGROUND: Tumour lysis syndrome (TLS) is a serious complication of malignancies and can result in renal failure or death. Previous reviews did not find clear evidence of benefit of urate oxidase in children with cancer. This review is the second update of a previously published Cochrane review. OBJECTIVES: To assess the effects and safety of urate oxidase for the prevention and treatment of TLS in children with malignancies. SEARCH METHODS: In March 2016 we searched CENTRAL, MEDLINE, Embase, and CINAHL...
March 8, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28237973/modulatory-effects-of-melatonin-and-vitamin%C3%A2-%C3%A2-c-on-oxidative-stress-mediated-haemolytic-anaemia-and-associated-cardiovascular-dysfunctions-in-rats
#4
Temitayo Olabisi Ajibade, Ademola Adetokunbo Oyagbemi, Ladoke A Durotoye, Temidayo Olutayo Omóbòwálé, Ebunoluwa Racheal Asenuga, Funsho Olakitike Olayemi
Background Phenylhydrazine (PHE) in experimental animal models has been widely reported to cause haemolytic anaemia, via the induction of oxidative stress and thus causing deleterious cardiovascular complications. Hence, this study was designed to evaluate the possible modulatory role of melatonin (MLT) or vitamin C when co-administered with PHE. Methods Anaemia was established with PHE administration. MLT or vitamin C was co-administered with PHE. Haematological parameters, markers of oxidative stress, enzymic and non-enzymic antioxidants, blood pressure and electrocardiograms were assessed...
March 1, 2017: Journal of Complementary & Integrative Medicine
https://www.readbyqxmd.com/read/28237970/clinical-predictors-of-length-of-stay-in-adults-with-congenital-heart-disease
#5
Ari Cedars, Lawrence Benjamin, Sara V Burns, Eric Novak, Amit Amin
OBJECTIVE: Length of stay (LOS) is a major driver of inpatient care costs. To date, few studies have investigated risk factors associated with increased LOS in patients with adult congenital heart disease (ACHD). In the present work, we sought to address this knowledge gap. METHODS: We conducted an analysis of the State Inpatient Databases from Arkansas, California, Florida, Hawaii, Nebraska and New York. We analysed data on admissions in patients with ACHD and constructed a series of hierarchical regression models to identify the clinical factors having the greatest effects on LOS...
February 25, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28218952/glomerular-hyperfiltration-is-strongly-correlated-with-age-in-congolese-children-with-sickle-cell-anaemia
#6
Michel Ntetani Aloni, René Makuala Ngiyulu, Pépé Mfutu Ekulu, Fiston IkwaNdol Mbutiwi, Jean Robert Makulo, Jean Lambert Gini-Ehungu, Nazaire Mangani Nseka, François Bompeka Lepira
AIM: Glomerular hyperfiltration is an early marker of sickle cell nephropathy and can lead to microalbuminuria and renal failure. Our aim was to identify the associated risk factors, as these could be of preventative importance. METHODS: We recruited 150 children with sickle cell anaemia (SCA), aged two to 18 years and living in Kinshasa, the Democratic Republic of Congo. Hyperfiltration and microalbuminuria were defined as an estimated glomerular filtration rate of less than 140 mL/min/1...
February 20, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28210512/porphyria-cutanea-tarda-in-a-patient-with-end-stage-renal-disease-a-case-of-successful-treatment-with-deferoxamine-and-ferric-carboxymaltose
#7
Natacha Rodrigues, Fernando Caeiro, Alice Santana, Teresa Mendes, Leonor Lopes
Porphyria cutanea tarda (PCT) is a rare disease, with a strong association with hepatitis C virus. PCT is particularly problematic in end-stage renal disease patients as they have no renal excretion of porphyrins and these are poorly dialyzed. Also, conventional treatment of PCT is compromised in these patients as hydroxychloroquine is contraindicated, phlebotomies with the stipulated frequency are poorly tolerated in already anaemia-prone patients, and iron-chelating agents are less efficient in removing iron and contribute to worsening anaemia...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28209604/birth-size-risk-factors-across-life-and-cognition-in-late-life-protocol-of-prospective-longitudinal-follow-up-of-the-mynah-mysore-studies-of-natal-effects-on-ageing-and-health-cohort
#8
Murali Krishna, G Mohan Kumar, S R Veena, G V Krishnaveni, Kalyanaraman Kumaran, Samuel Christaprasad Karat, Patsy Coakley, Clive Osmond, John R M Copeland, Giriraj Chandak, Dattatray Bhat, Mathew Varghese, Martin Prince, Caroline Fall
INTRODUCTION: For late-life neurocognitive disorders, as for other late-life chronic diseases, much recent interest has focused on the possible relevance of Developmental Origins of Health and Disease (DOHaD). Programming by undernutrition in utero, followed by overnutrition in adult life may lead to an increased risk, possibly mediated through cardiovascular and metabolic pathways. This study will specifically examine, if lower birth weight is associated with poorer cognitive functioning in late life in a south Indian population...
February 16, 2017: BMJ Open
https://www.readbyqxmd.com/read/28191814/the-pathophysiologic-basis-of-anaemia-in-patients-with-malignant-diseases
#9
Umma A Ibrahim, Aminu A Yusuf, Sagir G Ahmed
Cancer patients frequently present with anaemia that may result from the direct or indirect effects of the tumor or its treatment. Anaemia is an independent adverse prognostic factor that exerts negative influence on quality of life and survival of cancer patients. Anaemia in malignant disorders often arises from an interplay of multiple aetiological and pathophysiologic mechanisms. Understanding these mechanisms will help the oncologist identify and treat specific causes of the anaemia thereby minimizing the use of blood transfusion, which is associated with many adverse effects...
September 2016: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28176477/absence-of-thrombocytopaenia-and-or-microangiopathic-haemolytic-anaemia-does-not-reliably-exclude-recurrence-of-atypical-haemolytic-uraemic-syndrome-after-kidney-transplantation
#10
REVIEW
Anoushka R Krishnan, Brian Siva, Aron Chakera, Germaine Wong, Daniel Wong, Wai H Lim
A 54-year-old man was diagnosed with atypical haemolytic uraemic syndrome (aHUS) with confirmed complement H mutation in 2012, requiring ongoing dialysis. He was commenced on eculizumab in 2014 once the pharmaceutical board approved this drug. After 4 months, he received a live unrelated donor renal transplant from his wife and continued eculizumab post-transplant. Three months later, there was a rise in his creatinine with no laboratory features of haemolysis and a kidney biopsy confirmed rejection, which was treated with increased immunosuppression...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28176474/monoclonal-gammopathy-of-renal-significance-triggering-atypical-haemolytic-uraemic-syndrome
#11
REVIEW
Usman Mahmood, Nicole Isbel, Peter Mollee, Andrew Mallett, Sridevi Govindarajulu, Ross Francis
Haemolytic uraemic syndrome is a rare condition with an overall incidence of one to two cases in a population of 100 000 and approximately 10% of these cases are classified as atypical. Atypical haemolytic uraemic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and acute kidney injury. aHUS can be genetic, acquired or idiopathic (negative genetic screening and no environmental triggers). We describe a case of aHUS triggered by monoclonal gammopathy of renal significance (MGRS) successfully treated with plasmapheresis and a bortezomib-based chemotherapy regimen, resulting in marked improvement in renal function and other markers of haemolysis...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28176472/suspected-atypical-haemolytic-uraemic-syndrome-in-two-post-partum-patients-with-foetal-death-in-utero-responding-to-eculizumab
#12
REVIEW
Justin Chua, Kathy Paizis, Simon Z He, Peter Mount
BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) is a rare condition with the triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute kidney injury. Other conditions that present in a similar manner peri-partum include thrombotic thrombocytopaenic purpura, and pregnancy associated conditions including HELLP syndrome (haemolysis, elevated liver enzymes and low platelets), severe pre-eclampsia and less commonly acute fatty liver of pregnancy. CASE REPORTS: We describe two cases of suspected aHUS, who presented post-partum with foetal death-in-utero at 33 and 37 weeks respectively...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28176199/effects-of-antirheumatic-drug-underutilization-on-rheumatoid-arthritis-disease-activity
#13
Mohammad A Y Alqudah, Sayer Al-Azzam, Karem Alzoubi, Mohammad Alkhatatbeh, Khaldoon Alawneh, Ola Alazzeh, Bayan Ababneh
BACKGROUND: Following the recommended guidelines is crucial for achieving patient remission in rheumatoid arthritis. The aim of this study was to assess the effect of proper drug utilization of antirheumatic drugs on disease activity and drug safety in Jordan. METHODS: In a retrospective cross-sectional study, patient's demographics, clinical variables, drug regimens and side effects were recorded and the 28-joint disease activity scores were calculated. Patients were stratified into high, moderate, low disease activity or remission group...
February 8, 2017: Inflammopharmacology
https://www.readbyqxmd.com/read/28174184/multiple-myeloma-presenting-with-bilateral-ankle-pain-microangiopathy-and-complicated-by-streptococcal-meningitis-and-pneumocystis-carinii-pneumonia
#14
Louise Dunphy, Neeraj Singh, Elizabeth Keating
Multiple myeloma is characterised by the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin. This clone of plasma cells proliferates in the bone marrow, resulting in extensive skeletal destruction with osteolytic lesions, osteopenia and pathological fractures. Additional disease-related complications include hypercalcaemia, renal insufficiency, anaemia and infection. We present the case of a 64-year-old woman presenting with rapid onset, painful distal symmetrical lower limb weakness and an acute kidney injury...
February 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28146275/multiple-myeloma-routes-to-diagnosis-clinical-characteristics-and-survival-findings-from-a-uk-population-based-study
#15
Debra Howell, Alexandra Smith, Simon Appleton, Timothy Bagguley, Una Macleod, Gordon Cook, Russell Patmore, Eve Roman
Prompt cancer diagnosis may align UK survival with European averages. We examined the impact of route to diagnosis on survival for multiple myeloma patients diagnosed 2012-2013 using data from our population-based patient cohort that links to national death notifications and collects details on treatment and response (n = 441). Emergency presentation was associated with advanced disease and poorer outcomes, and was the commonest route to diagnosis (28·1%) followed by General Practitioner urgent (19·0%) and two-week wait (17·2%) referrals...
February 1, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28127421/pre-operative-anaemia-is-associated-with-total-morbidity-burden-on-days-3-and-5-after-cardiac-surgery-a-cohort-study
#16
Julie Sanders, Jackie A Cooper, Daniel Farrar, Simon Braithwaite, Updeshbir Sandhu, Michael G Mythen, Hugh E Montgomery
BACKGROUND: Pre-operative anaemia is associated with mortality and red blood cell (RBC) transfusion requirement after cardiac surgery. However, the effect on post-operative total morbidity burden (TMB) is unknown. We explored the effect of pre-operative anaemia on post-operative TMB. METHODS: Data were drawn from the Cardiac Post-Operative Morbidity Score (C-POMS) development study (n = 442). C-POMS describes and quantifies (0-13) TMB after cardiac surgery by noting the presence/absence of 13 morbidity domains on days 3 (D3), 5 (D5), 8 (D8) and 15 (D15)...
2017: Perioperative Medicine
https://www.readbyqxmd.com/read/28058668/impact-of-gender-and-dialysis-adequacy-on-anaemia-in-peritoneal-dialysis
#17
Alicja Ryta, Michal Chmielewski, Alicja Debska-Slizien, Piotr Jagodzinski, Malgorzata Sikorska-Wisniewska, Monika Lichodziejewska-Niemierko
PURPOSE: In the general population, haemoglobin (Hb) concentration is higher in men than in women. However, target Hb levels in dialysis patients are set constant regardless of the patient's sex. The aim of this study was to evaluate Hb concentration and the use of erythropoiesis-stimulating agents (ESA) in peritoneal dialysis (PD) patients taking gender and dialysis adequacy into account. METHODS: The study comprised two parts. The first was a cross-sectional analysis of Hb and ESA in 2180 prevalent PD patients...
January 5, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28050464/senior-loken-syndrome
#18
Amarpreet Kaur, Shashi Kant Dhir, Geetika Goyal, Naveen Mittal, R K Goyal
Senior Loken Syndrome (SLS) is a rare genetic disorder having juvenile nephronophthisis and retinal degeneration progressing to blindness and end stage renal disease. The present case report is about two sisters who presented with decreased visual acuity and end stage renal disease. Both had decreased vision, pallor, deranged renal function test and chronic malnutrition. Investigations revealed anaemia, uraemia, raised creatinine, low Glomerular Filteration Rate (GFR). Ophthalmology examination revealed nystagmus, retinal examination depicted pale optic disc and pigmentary changes in the retina...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28034708/influence-of-age-and-gender-on-clinical-outcomes-following-percutaneous-coronary-intervention-for-acute-coronary-syndromes
#19
Hanan Idris, John K French, Ibrahim M Shugman, Andrew P Hopkins, Craig P Juergens, Liza Thomas
BACKGROUND: Gender and age are non-modifiable factors influencing clinical outcomes in acute coronary syndromes (ACS). There is evidence that coronary artery disease pathophysiology varies in women. We therefore evaluated the effect of age and gender on clinical outcomes in patients with ACS undergoing percutaneous coronary interventions (PCI). METHODS: Among 3178 (25% female) consecutive ACS patients who underwent PCI at Liverpool Hospital, Sydney from 2003 to 2010, using femoral access in 98% of cases, we determined late events including mortality, myocardial infarction and bleeding according to Bleeding Academic Research Consortium (BARC) criteria...
December 2, 2016: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28017406/bortezomib-with-lenalidomide-and-dexamethasone-versus-lenalidomide-and-dexamethasone-alone-in-patients-with-newly-diagnosed-myeloma-without-intent-for-immediate-autologous-stem-cell-transplant-swog-s0777-a-randomised-open-label-phase-3-trial
#20
Brian G M Durie, Antje Hoering, Muneer H Abidi, S Vincent Rajkumar, Joshua Epstein, Stephen P Kahanic, Mohan Thakuri, Frederic Reu, Christopher M Reynolds, Rachael Sexton, Robert Z Orlowski, Bart Barlogie, Angela Dispenzieri
BACKGROUND: Lenalidomide plus dexamethasone is a reference treatment for patients with newly diagnosed myeloma. The combination of the proteasome inhibitor bortezomib with lenalidomide and dexamethasone has shown significant efficacy in the setting of newly diagnosed myeloma. We aimed to study whether the addition of bortezomib to lenalidomide and dexamethasone would improve progression-free survival and provide better response rates in patients with previously untreated multiple myeloma who were not planned for immediate autologous stem-cell transplant...
February 4, 2017: Lancet
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