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Hepatitis in children with haemophilia was historically most often associated with transfusion-transmitted infections. However, with the use of recombinant clotting factor concentrates, acquisition of such infections has now become rare. We studied the profile of hepatitis in North-American children with haemophilia in the modern era of safe blood products and excess childhood obesity. A total of 173 boys (<18 years) registered in the Pediatric Comprehensive Care Haemophilia Program were included in this retrospective study. Hospital records were reviewed for baseline data, serial height and weight measurements and serial alanine aminotransferase (ALT) levels. A body mass index (BMI) ranking was available for 170 boys, of whom 25 (14.7%, 95% CI 9.7-20.9%) were obese. The rate of obesity was higher in severe haemophilic boys. Compared with the general childhood population, the rate of obesity trended towards being higher in young haemophilic boys (2-5 years), but was similar in other age groups. A persistently high ALT (≥80 U L(-1) ) was documented in 5 boys and was associated with obesity. Three boys had clinical and imaging studies compatible with non-alcoholic fatty liver disease (NAFLD). Overweight and obesity are common among haemophilic boys, especially those who are younger and with severe disease. In this large group of haemophilic boys, chronic viral hepatitis was rare and NAFLD was a more common cause of liver disease. Overweight and obese haemophilic boys should be evaluated for NAFLD and interventional programmes should be designed to reduce the potential complications associated with obesity.

The prevalence, alloimmunization risk factors, antigenic exposure, and evaluation of antigen-matched red blood cells for thalassemia transfusions: a 10-year experience at a tertiary care hospital BACKGROUND: Hemoglobin E-β 0 thalassemia and homozygous β 0 -thalassemia are the most common chronic transfusion-dependent thalassemias in Thailand. Patients with these conditions can experience clinical complications such as RBC alloimmunization. In this study we aimed to determine the prevalence, alloimmunization risk factors, antigenic exposure, and evaluation of antigen-(C, c, E, e, Mi a ) matched RBC transfusion. STUDY DESIGN AND METHODS: Thalassemia patients were recruited from a tertiary care hospital for 10 years from 2008 to 2017. The medical records of transfusion history were reviewed for red cell phenotype both of patients and donors, number of units transfused, and type of alloantibodies. RESULTS: A total of 383 thalassemia patients were identified (178 males and 205 females). The frequency of RBC alloantibodies was 19.3%. Some patients tested positive for more than one antibody type. Autoantibodies were detected in nine individuals. Anti-E (49 [39.5%]), anti-Mi a (24 [19.4%]), and anti-c (19 [15.3%]) were the most common antibodies detected. A high rate of alloimmunization was found in splenectomized patients. Risk of alloimmunization increased when more total units of blood had been transfused. A trend toward low alloimmunization rates was noted in the antigen-matched RBC group, where 3.5% (5/143) of patients were alloimmunized. Anti-E and anti-Mi a , which may be naturally occurring, were identified in this group.CONCLUSION: Thai patients are more prone to develop antibodies against the Rh and Mi a than to the Kell blood group antigens.

Frequent infusion of factor concentrates may be challenging in young boys with haemophilia, especially if their disease is complicated by inhibitors. A central venous access device (CVAD) is often placed in young patients in need of repeated infusions for prophylaxis or immune tolerance induction. Although user friendly and capable of providing reliable venous access, these devices are associated with a high complication rate over time. In the haemophilia population, major complications include CVAD-associated infections and deep venous thrombosis, which is most often silent. Established risk factors for catheter-related infection include age less than 6 years at the time of CVAD placement and use of an external CVAD when compared with a totally implantable device such as a port. Avoidance of CVAD-related infections is facilitated by strict adherence to aseptic technique. The risk of deep venous thrombosis appears related to the duration for which the catheter is in place, with the risk increasing beyond 4 years. The promotion of a strict clinic policy in which CVADs are left in place for as short a time as possible should decrease the risk of complications. In rare cases where a totally implantable CVAD cannot be placed for technical reasons, an arteriovenous fistula may provide reliable venous access. In all cases, however, venous access via peripheral veins is preferred over CVADs.

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