Effect of restrictive versus liberal red cell transfusion strategies on haemostasis: systematic review and meta-analysis

Red cells play a key role in normal haemostasis in vitro but their importance clinically is less clear. The objective of this meta-analysis was to assess if correction of anaemia by transfusing red cells at a high haemoglobin threshold (liberal transfusion) is superior to transfusion at a lower haemoglobin threshold (restrictive transfusion) for reducing the risk of bleeding or thrombotic events. We searched for randomized controlled trials in any clinical setting that compared two red cell transfusion thresholds and investigated the risk of bleeding. We searched for studies published up to 19th October 2016 in The Cochrane Central Register of Controlled Trials, MEDLINE, PubMed, Embase, and the Transfusion Evidence Library and ISI Web of Science. Relative risks (RR) or Peto Odds Ratios (pOR) were pooled using a random-effect model. Nineteen randomized trials with 9852 participants were eligible for inclusion in this review. Overall there was no difference in the risk of any bleeding between transfusion strategies (RR 0.91, 95% confidence interval [CI] 0.74 to 1.12). The risk of severe or life-threatening bleeding was lower with a restrictive strategy (RR 0.75, 95% CI 0.57 to 0.99). There was no difference in the risk of thrombotic events (RR 0.83, 95% CI 0.61 to 1.13). The risk of any bleeding was not reduced with liberal transfusion and there was no overall difference in the risk of thrombotic events. Data from the included trials do not support aiming for a high haemoglobin threshold to improve haemostasis

Successful Live Birth following Preimplantation Genetic Diagnosis for Phenylketonuria in Day 3 Embryos by Specific Mutation Analysis and Elective Single Embryo Transfer

Phenylketonuria (PKU) is an autosomal recessive inherited metabolic disorder caused by a complete or near-complete deficiency of the liver enzyme phenylalanine hydroxylase (PAH), which converts the amino acid phenylalanine to tyrosine, leading to the increase of blood and tissue concentration of phenylalanine to toxic levels. PKU is not life threatening but is treated through lifelong dietary management. If untreated, it can lead to severe learning disability, brain function abnormalities, behavioural and neurological problems. The non-life threatening nature of PKU has until now caused some debate on whether to licence its detection by preimplantation genetic diagnosis (PGD). We report the first successful live birth in the UK following single cell embryo biopsy and PGD for the detection of two different mutations in the (PAH) gene. This case highlights both an important scientific development as well as the ethical challenge in offering couples who carry PKU this new reproductive option when starting their family

Successful live birth following preimplantation genetic diagnosis for phenylketonuria in day 3 embryos by specific mutation analysis and elective single embryo transfer

Phenylketonuria (PKU) is an autosomal recessive inherited metabolic disorder caused by a complete or near-complete deficiency of the liver enzyme phenylalanine hydroxylase (PAH), which converts the amino acid phenylalanine to tyrosine, leading to the increase of blood and tissue concentration of phenylalanine to toxic levels. PKU is not life threatening but is treated through lifelong dietary management. If untreated, it can lead to severe learning disability, brain function abnormalities, behavioural and neurological problems. The non-life threatening nature of PKU has until now caused some debate on whether to licence its detection by preimplantation genetic diagnosis (PGD). We report the first successful live birth in the UK following single cell embryo biopsy and PGD for the detection of two different mutations in the (PAH) gene. This case highlights both an important scientific development as well as the ethical challenge in offering couples who carry PKU this new reproductive option when starting their family

Patient satisfaction following carpal-tunnel decompression: a comparison of patients with and without osteoarthritis of the wrist

The aim of this study was to assess whether surgical decompression for carpal-tunnel syndrome (CTS) in the presence of primary or secondary osteoarthritis of the wrist is associated with poorer patient satisfaction. We did a retrospective matched cohort study. Twenty-four patients who underwent surgical decompression for CTS secondary to osteoarthritis were identified by reviewing the notes and the radiographs. A control group consisted of 24 patients without osteoarthritis of the wrist who underwent carpal-tunnel decompression. The control group was matched for age, sex, side, and neuro-physiological severity of the nerve compression. In the group with osteoarthritis of the wrist, 17 (71%) patients reported their symptom relief as satisfactory, and 7 (29%) reported the results as unsatisfactory. In the control group, 23 (96%) patients reported their symptom relief as satisfactory, and 1 (4%) reported the results as unsatisfactory (P=0.0325). In conclusion, patient satisfaction following surgical decompression in patients with secondary CTS due to osteoarthritis was significantly lower compared to patients without osteoarthritis of the wrist