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Background: Congenital red blood cell (RBC) disorders, such as hemoglobinopathies, are frequent worldwide but with large geographical variation. Growing migration has increased the number of patients with RBC disorders in formerly low prevalence countries, eg, Denmark. However, accurate prevalences are unknown. Methods: Patients with a registered diagnosis of congenital hemolysis in the Danish National Patient Register between 1977 and 2016 were linked to a national laboratory database of RBC disorders and the Danish civil registration system. We calculate annual age-and sex-specific prevalences of the congenital hemolytic disorders from 2000 to 2016. Results: Prevalences of all subtypes of congenital hemolytic disorders increased during the study period. The prevalence of hereditary spherocytosis increased 1.73 times between 2000 and 2015, from 10.2/105 persons to 17.7/105 persons. Alpha thalassemia trait had a prevalence of 0.5/ 105 persons in 2000, but increased 41 times to 19.2/105 persons in 2015. Beta thalassemia minor increased eightfold from 4.5/105 persons in 2000 to 34.9/105 persons in 2015. Likewise, sickle cell trait increased 11 times from 0.7/105 persons in 2000 to 8.1/105 persons in 2015, whereas sickle cell disease increased from 0.5/105 persons to 2.7/105 persons in 2015, a fivefold increase. Conclusion: The prevalence of congenital RBC disorders in Denmark is increasing. The hemoglobinopathy traits now have prevalences as high as hereditary spherocytosis. These estimates of congenital hemolytic disorders in Denmark emphasize that inborn hemoglobin disorders are a public health concern, even in some formerly low prevalence countries.

Objective Lesotho has one of the highest maternal mortality rates in the world. While at primary health care (PHC) level maternity care is free, at hospital level co‐payments are required from patients. We describe service utilisation and delivery outcomes before and after removal of user fees and quality of delivery care, and associated costs, at St Joseph's Hospital (SJH) in Roma, Lesotho. Methods We compared utilisation of delivery services, stillbirths and maternal and neonatal mortality for the periods before (1 July 2012 to 31 December 2013) and after (1 January 2014 to 30 June 2015) user fee removal through a retrospective chart review and estimated additional costs attributed to user fee removal from provider (hospital) and patient perspectives. Results Of 4715 deliveries 3855 were at SJH and 860 at PHC centres. Of women delivering at SJH 684 (18.5%) were ≤19 years and 894 (23.6%) were HIV positive. After user fee removal hospital deliveries increased by 49% — from 1547 to 2308 — and neonatal mortality decreased from 4.8 to 1.3 per 1000 live births (P = 0.033). Extrapolating costs to the entire country, 1 USD per capita per year would allow user fee removal at hospital level, the provision of free transport to/from and accommodation at hospital. Conclusion Removing user fees for hospital delivery care in Lesotho is feasible and affordable, and has the potential to improve maternal and neonatal outcomes by removing financial barriers to skilled birth attendants and increasing coverage of institutional deliveries.

Hereditary spherocytosis (HS) is a common anemia caused by germline mutations in red blood cell cytoskeleton proteins. The flow cytometry-based eosin-5'-maleimide (EMA) binding test is most frequently employed for reliable diagnostics. To perform this test, a number of healthy and ideally also age-matched controls are required, which can be challenging and complicates interlaboratory comparisons. To overcome this limitation, we modified the EMA binding test by replacing healthy controls with commercially available fluorescent beads. Blood samples from 289 individuals with suspected HS were analyzed using the EMA binding test with fluorescent beads and benchmarked against regular EMA binding test using two control samples. Using osmotic gradient ektacytometry as validation, 112 individuals (38.8%) were diagnosed with HS. Performance of the modified EMA binding test was not compromised (accuracy 90.3%) compared to EMA binding test using matched controls (accuracy 88.6%). Based on these findings, we conclude that the modified EMA binding test with fluorescent beads is an attractive alternative, especially in laboratories without easy access to matched controls. Furthermore, as fluorescent beads are stable and easily commutable, they could facilitate both interlaboratory comparisons and quality assessment programs.

Dennis Lund Hansen,1,2 Andreas Glenthøj,3 Sören Möller,1,4 Bart J Biemond,5 Kjeld Andersen,1,6 David Gaist,1,7 Jesper Petersen,3 Henrik Frederiksen1,2 1Department of Clinical Research, University of Southern Denmark, Odense, Denmark; 2Department of Hematology, Odense University Hospital, Odense, Denmark; 3Department of Hematology, Center for Hemoglobinopathies, Herlev and Gentofte Hospital, Herlev, Denmark; 4OPEN, Odense University Hospital, Odense, Denmark; 5Department of Hematology, Amsterdam University Medical Centre’s, Amsterdam, Netherlands; 6Department of Mental Health - Odense, Region of Southern Denmark, Odense, Denmark; 7Neurology Research Unit, Department of Neurology, Odense University Hospital, Odense, DenmarkCorrespondence: Dennis Lund Hansen Email dlh@dadlnet.dkBackground: Congenital red blood cell (RBC) disorders, such as hemoglobinopathies, are frequent worldwide but with large geographical variation. Growing migration has increased the number of patients with RBC disorders in formerly low prevalence countries, eg, Denmark. However, accurate prevalences are unknown.Methods: Patients with a registered diagnosis of congenital hemolysis in the Danish National Patient Register between 1977 and 2016 were linked to a national laboratory database of RBC disorders and the Danish civil registration system. We calculate annual age- and sex-specific prevalences of the congenital hemolytic disorders from 2000 to 2016.Results: Prevalences of all subtypes of congenital hemolytic disorders increased during the study period. The prevalence of hereditary spherocytosis increased 1.73 times between 2000 and 2015, from 10.2/105 persons to 17.7/105 persons. Alpha thalassemia trait had a prevalence of 0.5/105 persons in 2000, but increased 41 times to 19.2/105 persons in 2015. Beta thalassemia minor increased eightfold from 4.5/105 persons in 2000 to 34.9/105 persons in 2015. Likewise, sickle cell trait increased 11 times from 0.7/105 persons in 2000 to 8.1/105 persons in 2015, whereas sickle cell disease increased from 0.5/105 persons to 2.7/105 persons in 2015, a fivefold increase.Conclusion: The prevalence of congenital RBC disorders in Denmark is increasing. The hemoglobinopathy traits now have prevalences as high as hereditary spherocytosis. These estimates of congenital hemolytic disorders in Denmark emphasize that inborn hemoglobin disorders are a public health concern, even in some formerly low prevalence countries.Keywords: hemolytic anemia, thalassemia, sickle cell disease, hereditary spherocytosis, hemoglobinopathies, sickle cell anemia

Ambient Intelligence systems use many sensors and actuators, with a diversity of networks, protocols and technologies which makes it impossible to access the devices in a common manner. This paper presents the HomePort software, which provides an open source RESTful interface to heterogeneous sensor networks, allowing a simple unified access to virtually any kind of protocol using well known standards. HomePort includes means to provide event notification, as well as a tracing mechanism. The software is implemented and we report on initial experiments and provide an evaluation that shows the feasibility and scalability of the approach.

BACKGROUND: The combination of protein supplementation with exercise is successful in increasing weight and energy intake, as well as exercise capacity and health--related quality of life in sarcopenic patients diagnosed with chronic obstructive disease (COPD). However, the potential benefit of protein supplementation for non--sarcopenic patients with COPD has yet not previously been examined.AIM: The aim of this trial was to evaluate the effect of protein supplementation on quality of life, physical function, muscle strength and biochemical blood markers in patients diagnosed with COPD undergoing nine weeks of pulmonary rehabilitation.DESIGN: A prospective, parallel group randomised clinical trial. Setting: Patients referred from their general practitioners to the COPD rehabilitation outpatient programme at the local community rehabilitation centre.POPULATION: Patients (n=53) with stable moderate to severe COPD diagnosed with COPD, 40 years or older and with a BMI < 30. Methods: The participants were assigned to one of two groups to receive either twice daily protein supplementation (9,3g of protein/566,4KJ) plus exercise or exercise only. Before and after nine weeks of rehabilitation, mental state was measured by means of St George Respiratory Questionnaire, physical performance was evaluated by shuttle walking test and maximal muscle strength test, and fasting blood samples were analysed.RESULTS: Supplementing exercise with protein had no additional effect on any of the outcome measures. However, shuttle walk time, St George total score and subscore for impact improved as effect of time.CONCLUSION: This trial was unable to provide evidence for the effect of protein supplementation on quality of life, physical function, and muscle strength in non--sarcopenic patients with moderate to severe COPD.CLINICAL REHABILITATION IMPACT: The role of protein supplementation in COPD--rehabilitation should focus on identifying patients to receive supplement with protein and from those who will not benefit.