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https://www.readbyqxmd.com/read/28901454/%C3%AE-%C3%A2-thalassemia-caused-by-compound-heterozygous-mutations-and-cured-by-bone-marrow-transplantation-a-case-report
#1
Liusong Wu, Zhiyu Peng, Sen Lu, Mei Tan, Ying Rong, Runmei Tian, Yuhang Yang, Yan Chen, Jindong Chen
In the present study, a rare familial case of severe thalassemia with compound spontaneous mutations is reported. A 2.5‑year‑old boy, who suffered from severe anemia with yellowish skin, enlarged liver and spleen, was provided with a blood transfusion every 20 days to maintain hemoglobin levels between 90 and 100 g/l. Sanger sequencing combined with reverse transcription‑quantitative polymerase chain reaction (RT‑qPCR) and Gap‑PCR revealed that the proband was a carrier of 4 compound heterozygous mutations: Hemoglobin subunit β (HBB):IVS‑II‑654(C>T)β+; Southeast Asian‑type‑hereditary persistence of fetal hemoglobin (SEA‑HPFH); HBB:c316‑148G>T; hemoglobin subunit α2 (HBA2):c...
September 12, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28878585/selective-upper-body-perfusion-technique-for-removal-of-renal-cell-carcinoma-extending-into-the-inferior-vena-cava-and-right-atrium
#2
Selim Aydin, Bora Cengiz, Banu Vural Gokay, Anar Mammadov, Remzi Emiroglu, Saadettin Eskicorapci, Ersin Erek
Invasion of a renal cell carcinoma thrombus into the inferior vena cava and right atrium is infrequent. Reaching and completely excising a tumor from the inferior vena cava is particularly challenging because the liver covers the surgical field. We report the case of a 61-year-old man who underwent surgery for a renal cell carcinoma of the right kidney that extended into the inferior vena cava and right atrium. During dissection of the liver to expose the inferior vena cava, transesophageal echocardiograms revealed right atrial mass migration into the tricuspid valve...
August 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28876654/ileosigmoid-knotting-a-zimbabwean-case-series
#3
C Mbanje, S Mungazi, A J V Maunganidze
BACKGROUND: Ileosigmoid knotting is a rare cause of intestinal obstruction with a rapidly progressive course, for which expedient surgical intervention is required to avoid mortality. METHOD: A combined retrospective and prospective analysis was performed on 16 patients operated on at PGH with an operative diagnosis of ileosigmoid knotting between February 2011 and April 2016. Data inclusive of demographics, time to presentation and surgery, preoperative diagnostic accuracy, incidence of septic shock and mortality was collected...
June 2017: South African Journal of Surgery. Suid-Afrikaanse Tydskrif Vir Chirurgie
https://www.readbyqxmd.com/read/28862718/clinical-impact-of-regional-citrate-anticoagulation-in-continuous-renal-replacement-therapy-in-critically-ill-patients
#4
Maria Huguet, Lida Rodas, Miquel Blasco, Luis F Quintana, Jordi Mercadal, Jose T Ortiz-Pérez, Irene Rovira, Esteban Poch
BACKGROUND: Regional citrate anticoagulation (RCA) is being used increasingly in continuous renal replacement therapy (CRRT) as a safer alternative to heparin. However, complex metabolic control to avoid side effects have generated discrepancies about its introduction into everyday practice. We aimed to compare both anticoagulation techniques in terms of efficacy, safety and feasibility. METHODS: Observational retrospective study performed in 3 specialized ICUs in patients receiving CVVHDF with RCA between January 2013 and May 2016...
August 17, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28838449/successful-blood-transfusion-management-of-a-living-donor-liver-transplant-recipient-in-the-presence-of-anti-jr-a-a-case-report
#5
N Kurata, Y Onishi, H Kamei, T Hori, M Komagome, C Kato, T Matsushita, Y Ogura
A 48-year-old Japanese woman was diagnosed with Budd-Chiari syndrome and transferred for possible living donor liver transplantation (LDLT). Examinations before LDLT revealed that the recipient had anti-Jr(a) and preformed donor-specific anti-human leukocyte antigen (HLA) antibodies (DSA). Rituximab was administrated at 16 days prior to the patient's scheduled LDLT for the prophylaxis of antibody-mediated rejection by DSA. The clinical significance of anti-Jr(a) has not been clearly established because of the rarity of this antibody, so we discussed blood transfusion strategy with the Department of Blood Transfusion Service and prepared for Jr(a)-negative packed red blood cells (RBCs)...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28833178/expediting-red-blood-cell-transfusions-by-syringing-causes-significant-hemolysis
#6
Willem Lambertus De Villiers, Adriaan Albertus Murray, Andrew Ian Levin
BACKGROUND: Techniques commonly used to expedite blood transfusions include pneumatically pressurizing red blood cell (RBC) bags or manual syringing its contents. We compared these techniques on RBC hemolysis using a simulated transfusion model. STUDY DESIGN AND METHODS: Fifteen warmed RBC units that were 12.3 ± 4.3 (95% confidence interval [CI], 10.1-14.5) days old were each subjected to two experimental rapid transfusion techniques. RBCs from each technique were directed through 18- and 22-gauge cannulas attached to blood administration sets...
August 20, 2017: Transfusion
https://www.readbyqxmd.com/read/28819631/effect-of-tranexamic-acid-on-hematologic-values-and-blood-loss-in-reverse-total-shoulder-arthroplasty
#7
Sae Hoon Kim, Whan Ik Jung, Young Jun Kim, Do Hyeon Hwang, Young Eun Choi
PURPOSE: Use of tranexamic acid (TXA) in the setting of arthroplasty of the lower extremity has been previously described. The aim of this study was to evaluate the benefit of a single dose of TXA (500 mg vial) administered intravenously just prior to RTSA in an Asian population. METHODS: The records of 48 patients (no TXA, n = 24, versus TXA, n = 24) that underwent RTSA for cuff tear arthropathy were retrospectively reviewed. All patients had a Hemovac drain positioned for 2 days after surgery...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28818213/the-natural-history-and-outcomes-of-line-associated-upper-extremity-deep-venous-thromboses-in-critically-ill-patients
#8
Joshua Underhill, Matthew A Sherman, Ryan Howard, Anthony Hage, Andrea Obi, Lena Napolitano, Dawn M Coleman
OBJECTIVE: Anticoagulation remains the standard of care for line-associated upper extremity deep venous thrombosis (UEDVT). This treatment carries the risk of hemorrhagic complications, possibly more so in surgical patients. Considering the low-risk profile of UEDVT-which is associated with fewer, less severe pulmonary emboli than lower extremity deep venous thrombosis-current UEDVT treatment guidelines may be overly aggressive. The goal of this study was to review outcomes of line-associated UEDVT in critically ill patients and to define the efficacy of current treatment protocols in pulmonary embolism (PE) prevention while avoiding hemorrhagic complications...
September 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28811051/patient-blood-management-in-the-intensive-care-unit
#9
REVIEW
Aryeh Shander, Mazyar Javidroozi, Gregg Lobel
Patient Blood Management underscores a fundamental shift from a product-centered approach to a patient-centric approach through timely application of evidence-based medical and surgical concepts designed to maintain hemoglobin concentration, optimize hemostasis, and minimize blood loss in an effort to improve patient outcome. In this concept, allogeneic blood transfusion is not viewed as the treatment of default for anemic patients, but one among many treatment modalities that should be weighed based on its merits-potentials risks and benefits-for the individual patient in the context of other alternatives...
July 31, 2017: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/28801322/portal-vein-aneurysm-in-thalassaemia
#10
Simi Das, Mousam Dey, Vinay Kumar, Hira Lal
Arterial aneurysms are more common than visceral venous aneurysms. Portal vein aneurysms being the most common type of visceral venous aneurysms. Here, we present an 18-year-old young woman with thalassaemia major, who presented with headache, palpitation, shortness of breath and a recent increase in blood transfusion rate. On clinical examination, she had hepatosplenomegaly. Ultrasonography revealed hepatosplenomegaly with fusiform dilatation of extrahepatic portal vein, which was confirmed to be portal vein aneurysm on contrast enhanced CT...
August 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28801130/prospective-implementation-of-enhanced-recovery-after-surgery-protocols-to-radical-cystectomy
#11
Karl H Pang, Ruth Groves, Suresh Venugopal, Aidan P Noon, James W F Catto
BACKGROUND: Multimodal enhanced recovery after surgery (ERAS) regimens have improved outcomes from colorectal surgery. OBJECTIVE: We report the application of ERAS to patients undergoing radical cystectomy (RC). DESIGN, SETTING, AND PARTICIPANTS: Prospective collection of outcomes from consecutive patients undergoing RC at a single institution. INTERVENTION: Twenty-six components including prehabilitation exercise, same day admission, carbohydrate fluid loading, targeted intraoperative fluid resuscitation, regional local anaesthesia, cessation of nasogastric tubes, omitting oral bowel preparation, avoiding drain use, early mobilisation, chewing gum use, and audit...
August 8, 2017: European Urology
https://www.readbyqxmd.com/read/28795966/the-coagulation-profile-of-end-stage-liver-disease-and-considerations-for-intraoperative-management
#12
Katherine T Forkin, Douglas A Colquhoun, Edward C Nemergut, Julie L Huffmyer
The coagulopathy of end-stage liver disease results from a complex derangement in both anticoagulant and procoagulant processes. With even minor insults, cirrhotic patients experience either inappropriate bleeding or clotting, or even both simultaneously. The various phases of liver transplantation along with fluid and blood product administration may contribute to additional disturbances in coagulation. Thus, anesthetic management of patients undergoing liver transplantation to improve hemostasis and avoid inappropriate thrombosis in the perioperative environment can be challenging...
August 8, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28766056/-anesthesia-management-in-microsurgical-reconstructions
#13
A Rand, M Ayoub, C H Meyer-Frießem, P K Zahn, M Bauer
Microsurgical procedures for construction of anastomoses present new challenges for anesthetists in the perioperative setting. Despite their increasing importance, so far no perioperative management guidelines for these patients existed. Anesthetists can influence the success of surgery (e. g. successful perfusion of a flap) via an optimal preoperative, intraoperative and postoperative approach. Patients should be carefully evaluated preoperatively for increased risks to avoid poor postoperative outcomes...
August 1, 2017: Der Anaesthesist
https://www.readbyqxmd.com/read/28764694/predictors-of-haematocrit-in-lumbar-fusion-for-lumbar-disc-herniation-a-surgical-assessment
#14
Qingchun Cai, Sixiang Zeng, Liqiang Zhi, Junlong Wu, Wei Ma
BACKGROUND: Low haematocrit (Hct) is associated with a higher rate of post-operative complications, increased mortality, and additional medical costs following cardiac surgery. Predictors of post-operative Hct in lumbar fusion are unclear and may be beneficial in avoiding adverse surgical outcomes. METHODS: A total of 704 lumbar disc herniation patients (385 males, 319 females) who underwent primary lumbar fusion surgery were reviewed in this retrospective study...
August 1, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28760470/improved-approach-with-subcostal-exchange-of-the-heartmate-ii-left-ventricular-assist-device-difference-in-on-and-off-pump
#15
Ann C Gaffey, Carol W Chen, Jennifer J Chung, Emily Phillips, Joyce Wald, Matthew L Williams, David W Low, Michael A Acker, Pavan Atluri
BACKGROUND: The HeartMate II (St. Jude Medical, Inc, St. Paul, MN [previously Thoratec]) left ventricular assist device (LVAD) exchange has traditionally involved a redo sternotomy. Alternate minimally invasive subcostal approaches have the advantage of avoiding sternal reentry, excessive bleeding, and prolonged recovery. METHODS: This retrospective review included patients who underwent an exchange from May 2009 to March 2016. The patients were divided into three cohorts: (1) redo sternotomy, (2) subcostal approach involving cardiopulmonary bypass (CPB) (ON-CPB SC), and (3) subcostal approach off the CPB pump (OFF-CPB SC)...
July 28, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28756471/the-research-agenda-for-trauma-critical-care
#16
REVIEW
Karim Asehnoune, Zsolt Balogh, Giuseppe Citerio, Andre Cap, Timothy Billiar, Nino Stocchetti, Mitchell J Cohen, Paolo Pelosi, Nicola Curry, Christine Gaarder, Russell Gruen, John Holcomb, Beverley J Hunt, Nicole P Juffermans, Mark Maegele, Mark Midwinter, Frederick A Moore, Michael O'Dwyer, Jean-François Pittet, Herbert Schöchl, Martin Schreiber, Philip C Spinella, Simon Stanworth, Robert Winfield, Karim Brohi
In this research agenda on the acute and critical care management of trauma patients, we concentrate on the major factors leading to death, namely haemorrhage and traumatic brain injury (TBI). In haemostasis biology, the results of randomised controlled trials have led to the therapeutic focus moving away from the augmentation of coagulation factors (such as recombinant factor VIIa) and towards fibrinogen supplementation and administration of antifibrinolytics such as tranexamic acid. Novel diagnostic techniques need to be evaluated to determine whether an individualised precision approach is superior to current empirical practice...
July 29, 2017: Intensive Care Medicine
https://www.readbyqxmd.com/read/28756046/a-preoperative-risk-score-for-transfusion-in-infrarenal-endovascular-aneurysm-repair-to-avoid-type-and-cross
#17
Thomas F X O'Donnell, Katie E Shean, Sarah E Deery, Thomas C F Bodewes, Mark C Wyers, Kerry L O'Brien, Robina Matyal, Marc L Schermerhorn
OBJECTIVE: Preoperative type and cross are often routinely ordered before elective endovascular aneurysm repair (EVAR), but the cost of this practice is high, and transfusion is rare. We therefore aimed to stratify patients by their risk of transfusion to identify a cohort in whom a type and screen would be sufficient. METHODS: We queried the targeted vascular module of the National Surgical Quality Improvement Program (NSQIP) for all elective EVARs from 2011 to 2015...
July 26, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28750609/hemodialysis-patients-preferences-for-the-management-of-anemia
#18
Brett Hauber, John Caloyeras, Joshua Posner, Deborah Brommage, Spiros Tzivelekis, Allan Pollock
BACKGROUND: Patient engagement in end-stage renal disease (ESRD) is expected to result in a more patient-centered approach to care that aligns with patients' values, preferences, and goals for treatment. Nevertheless, no previous studies of which we are aware have evaluated patients' benefit-risk preferences for the management of anemia associated with ESRD. The primary objective of this study was to quantify the tradeoffs patients are willing to make between cardiovascular risks associated with some anemia medicines and red blood cell (RBC) transfusions...
July 28, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28743152/-prevention-of-perioperative-hypothermia-guidelines-for-daily-clinical-practice
#19
Timo Iden, Jan Höcker
Inadvertent perioperative hypothermia (body core temperature < 36 °C) is a serious complication leading to increased rates of wound infection, higher blood loss associated with increased transfusion requirements as well as patient dissatisfaction among others. Body core temperature is a vital parameter and needs constant monitoring just like heart rate, blood pressure and arterial oxygen saturation. Patient-, anesthesia-, surgery- and environment-related risk factors were identified for occurring perioperative hypothermia...
July 2017: Anästhesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie: AINS
https://www.readbyqxmd.com/read/28720606/re-using-blood-products-as-an-alternative-supplement-in-the-optimisation-of-clinical-grade-adipose-derived-mesenchymal-stem-cell-culture
#20
J Phetfong, T Tawonsawatruk, K Seenprachawong, A Srisarin, C Isarankura-Na-Ayudhya, A Supokawej
OBJECTIVES: Adipose-derived mesenchymal stem cells (ADMSCs) are a promising strategy for orthopaedic applications, particularly in bone repair. Ex vivo expansion of ADMSCs is required to obtain sufficient cell numbers. Xenogenic supplements should be avoided in order to minimise the risk of infections and immunological reactions. Human platelet lysate and human plasma may be an excellent material source for ADMSC expansion. In the present study, use of blood products after their recommended transfusion date to prepare human platelet lysate (HPL) and human plasma (Hplasma) was evaluated for in vitro culture expansion and osteogenesis of ADMSCs...
July 2017: Bone & Joint Research
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