A novel nonsense variant in RHAG underlies a Nordic Rhnull phenotype.

BACKGROUND AND OBJECTIVES: The extremely rare Rhnull phenotype is characterized by the absence of all Rh antigens on erythrocytes. It is divided into the regulator and amorph types based on the underlying genetic background. The more common regulator type depends on critical variants silencing RHAG, which encodes RhAG glycoprotein, necessary for RhD/RhCE expression. Rhnull cells have altered expression of glycophorin B and LW glycoprotein. MATERIALS AND METHODS: Four unrelated Rhnull individuals were investigated. Serological testing was performed according to standard blood bank practice. RHD/RHCE and S/s allele-specific Polymerase chain reaction (PCR) genotyping was done on genomic DNA using in-house PCR assays. RHAG, and in some cases also RHD/RHCE, were sequenced. Initial s phenotyping results triggered additional serological investigation. RESULTS: Anti-Rh29 was identified in all four individuals. Extended typing with anti-S and anti-s showed that the three samples predicted to type as s+ failed to react with 2 of 5 anti-s. Sequence analysis of all 10 RHAG exons and the immediate intron/exon boundaries revealed a single nucleotide variant in the 3'-end of intron 6, c.946 -2a>g in all samples. RHD/RHCE showed no alterations. CONCLUSION: A novel Nordic Rhnull allele was identified. In addition, it was shown that s+ Rhnull red blood cells are not only U- but also have qualitative changes in their s antigen expression.

Whole blood transfusion and paroxysmal nocturnal haemoglobinuria meet again: Minor incompatibility, major trouble.

BACKGROUND AND OBJECTIVES: The field of transfusion medicine started out with whole blood transfusion to treat severe anaemia and other deficiencies, and then transitioned to component therapy, largely leaving the practice, and experiences, of whole blood transfusions behind. Currently, the field is circling back and whole blood is gaining ground as an alternative to massive transfusion protocols. MATERIALS AND METHODS: Herein we describe a severely anaemic paroxysmal nocturnal haemoglobinuria (PNH) patient initially suspected of suffering from renal haemorrhage, receiving a standard low-titre group O whole blood transfusion during pre-hospital transportation. RESULTS: Following the transfusion, the patient suffered a clinically unmistakable haemolytic transfusion reaction requiring supportive treatment in the intensive care unit. Clinical observations are consistent with an acute haemolytic reaction. The haemolysis was likely due to minor incompatibility between the plasma from the transfused whole blood and the patient's PNH red cells. Recovery was uneventful. CONCLUSION: This revealed an unappreciated contraindication to minor incompatible whole blood transfusion, and prompted a discussion on the distinction between whole blood and erythrocyte concentrates, the different indications for use and the importance of emphasizing these differences. It also calls attention to patient groups where minor incompatibility can be of major importance.

Changes in self-efficacy, collective efficacy and patient outcome following interprofessional simulation training on postpartum haemorrhage.

AIMS AND OBJECTIVES: To examine whether interprofessional simulation training on management of postpartum haemorrhage enhances self-efficacy and collective efficacy and reduces the blood transfusion rate after birth. BACKGROUND: Postpartum haemorrhage is a leading cause of maternal morbidity and mortality worldwide, although it is preventable in most cases. Interprofessional simulation training might help improve the competence of health professionals dealing with postpartum haemorrhage, and more information is needed to determine its potential. DESIGN: Multimethod, quasi-experimental, pre-post intervention design. METHODS: Interprofessional simulation training on postpartum haemorrhage was implemented for midwives, obstetricians and auxiliary nurses in a university hospital. Training included realistic scenarios and debriefing, and a measurement scale for perceived postpartum haemorrhage-specific self-efficacy, and collective efficacy was developed and implemented. Red blood cell transfusion was used as the dependent variable for improved patient outcome pre-post intervention. RESULTS: Self-efficacy and collective efficacy levels were significantly increased after training. The overall red blood cell transfusion rate did not change, but there was a significant reduction in the use of ≥5 units of blood products related to severe bleeding after birth. CONCLUSION: The study contributes to new knowledge on how simulation training through mastery and vicarious experiences, verbal persuasion and psychophysiological state might enhance postpartum haemorrhage-specific self-efficacy and collective efficacy levels and thereby predict team performance. The significant reduction in severe postpartum haemorrhage after training, indicated by reduction in ≥5 units of blood transfusions, corresponds well with the improvement in collective efficacy, and might reflect the emphasis on collective efforts to counteract severe cases of postpartum haemorrhage. RELEVANCE TO CLINICAL PRACTICE: Interprofessional simulation training in teams may contribute to enhanced prevention and management of postpartum haemorrhage, shown by a significant increase in perceived efficacy levels combined with an indicated reduction of severe postpartum haemorrhage after training.

Blood donor Borrelia burgdorferi sensu lato seroprevalence and history of tick bites at a northern limit of the vector distribution.

In order to study the antibody seroprevalence of the causal agent of Lyme borreliosis, Borrelia burgdorferi sensu lato (s.l.), and the history of tick bites at a geographical distribution limit of Ixodes ricinus, we compared healthy blood donors in geographically extreme regions: the borreliosis-endemic Vestfold County (59°N) and the region of northern Norway. Blood samples were screened using IgG/VlsE ELISA, and positive/borderline samples were confirmed using C6 ELISA and immunoblot assays. Also, donors completed a questionnaire consisting of several items including the places they have lived, and whether they owned any pets. The seroprevalence was 0.48% (5/1048) in northern Norway and 9.25% (48/519) in Vestfold County. Seven donors (of 1048) had experienced a single tick bite in the southern part of Nordland County (65°N) in northern Norway. This first study on B. burgdorferi s.l. antibody seroprevalence and tick bites on humans and pets in northern Norway showed that the seroprevalence of B. burgdorferi s.l. infection and the risk of tick bite in northern Norway are insignificant; the fact that only five positive IgG samples were detected underscores the very low background seroprevalence. These results suggest that so far I. ricinus has not expanded north of the previously established geographical distribution limit.