Translation, validation, and usability of the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (Self-ISTH-BAT).

BACKGROUND: Bleeding questionnaires are effective and recommended screening tools for potential bleeding disorder, but healthcare practitioner-administered bleeding assessment tools (expert-ISTH-BATs) are time-consuming. A patient-administered ISTH-BAT (self-ISTH-BAT) has been developed and validated. We translated, validated, and evaluated the usability of self-ISTH-BAT. METHODS: We conducted a forward-backward translation of self-ISTH-BAT from English to Danish. Expert-ISTH-BAT and Danish self-ISTH-BAT were administered to 106 random individuals aged ≥18 years attending Odense University Hospital between August and November 2020 for elective blood sampling. Results comprise a score of bleeding symptoms. RESULTS: Mean age of included individuals were 49 years (range: 18-83), and 59% were female. Median self-ISTH-BAT score was 2 (range: 0-18) and 1 (range: 0-22) for expert-ISTH-BAT (P = .09). All organ systems had ≥90% exact score agreement between expert-ISTH-BAT and self-ISTH-BAT, except gastrointestinal bleeding (77%) and other bleedings (72%). We found an acceptable correlation (r2  = .80) between expert-ISTH-BAT and self-ISTH-BAT. The self-ISTH-BAT had 82% sensitivity and 89% specificity at the recommended cutoff for expert-BAT (female:<6; male:<4). At this cutoff, 10 had abnormal self-ISTH-BAT scores with normal expert-ISTH-BAT. Three (3%) had normal self-ISTH-BAT with abnormal expert-ISTH-BAT. CONCLUSION: Self-ISTH-BAT can replace expert-ISTH-BAT as a screening tool for bleeding disorders in Danish individuals as only 3% were not identified with the self-ISTH-BAT tool.

How do I establish a stool bank for fecal microbiota transplantation within the blood- and tissue transplant service?

Worldwide, there is a rising demand for thoroughly screened, high-quality fecal microbiota transplantation (FMT) products that can be obtained at a reasonable cost. In the light of this evolving therapeutic area of the intestinal microbiota, both private and public stool banks have emerged. However, some of the larger difficulties when establishing stool banks are caused by the absence of or international disagreement on regulation and legislative formalities. In this context, the establishment of a stool bank within a nonprofit blood and tissue transplant service has several advantages. Especially, this setting can ensure that every step of the donation process, laboratory handling, and donor-traceability is in agreement with the current expert guidelines and meets the requirements of the European Union's regulative directives on human cells and tissues. Although safety and documentation are the top priority of the stool bank setup presented here, cost-effectiveness of the production is possible due to a high donor screening success rate and the knowhow, infrastructure, facilities, personnel, and laboratory- and quality-management systems that were already in place. Overall, our experience is that a centralized, nonprofit, blood and tissue transplant service is an ideal and safe facility to run a stool bank of high quality FMT products that are based on stool donations from volunteer, unpaid, healthy, blood donors.

Time-related variation in IgG subclass concentrations in a group of healthy Danish adults.

INTRODUCTION: Immunoglobulin G subclass measurements are important for the diagnostic work-up of immunodeficiencies and immunoglobulin G4 (IgG4) related diseases. It is currently unknown whether a single sampling is truly representative for an individual's IgG subclass concentrations. This study aimed to investigate whether IgG and IgG subclass concentrations in healthy individuals are stable over time. METHOD: With a span of median 42 weeks, four samples from each of 54 (34M, 20F) healthy adult volunteers (24-66 years) were analyzed for IgG and IgG1-4 using turbidimetry. Concentrations were compared within and between individuals. RESULTS: IgG and IgG subclass concentrations followed either a normal (IgG, IgG1, and IgG3) or log normal (IgG2 and IgG4) distribution. Immunoglobulin 4 demonstrated by far the widest range of concentrations between individuals (670-fold: 0.004-2.68 g/L). Immunoglobulin G subclass variations within individuals were expressed as pooled standard deviations (PSD). These ranged from 0.056 (IgG4) to 0.955 g/L (IgG) and correlated with mean concentration of IgG or the particular IgG subclass. As a consequence, the relative PSDs (i.e., PSD divided by mean IgG or IgG subclass concentration) fell within a narrow range: 5.82%-10.1%. Based on these numbers, the 95%-upper one-sided confidence limits for intraindividual IgG and IgG subclass variation was calculated to range from 9.82% (IgG2) to 16.9% (IgG4). CONCLUSION: The study documents that IgG or IgG subclass concentrations within healthy individuals are very stable over at least 42 weeks. The expected variation for IgG4 concentrations at a 95% confidence level does not exceed ±16.9%.

Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report.

BACKGROUND: In neonates, rhesus D alloimmunization despite anti-D immunoglobulin prophylaxis is rare and often unexplained. Rhesus D alloimmunization can lead to hemolytic disease of the newborn with anemia and unconjugated hyperbilirubinemia. In past reports, transient congenital hyperinsulinism has been described as a rare complication of rhesus D alloimmunization. Our case report illustrates that rhesus D alloimmunization can result in a pseudosyndrome with severe congenital hyperinsulinism, anemia, and conjugated hyperbilirubinemia, despite correctly administered anti-D immunoglobulin prophylaxis. CASE PRESENTATION: We report of a 36-year-old, Caucasian gravida 1, para 1 mother with A RhD negative blood type who received routine antenatal anti-D immunoglobulin prophylaxis. Her full term newborn boy presented with severe congenital hyperinsulinism, anemia, and conjugated hyperbilirubinemia up to 295 µmol/L (ref. < 9), accounting for 64% of the total bilirubin. Syndromic congenital hyperinsulinism was suspected. Examinations showed a positive direct antiglobulin test, initially interpreted as caused by irregular antibodies; diffuse congenital hyperinsulinism by 18F-DOPA positron emission tomography/computed tomography scan; normal genetic analyses for congenital hyperinsulinism; mildly elevated liver enzymes; delayed, but present bile excretion by Tc99m-hepatobiliary iminodiacetic acid scintigraphy; and cholestasis and mild fibrosis by liver biopsy. The maternal anti-D titer was 1:16,000 day 20 postpartum. Y-chromosome material in the mother's blood could not be identified. This could, however, not exclude late intrapartum fetomaternal hemorrhage as the cause of immunization. No causative genetic findings were deetrmined by trio whole exome sequencing. The child went into clinical remission after 5.5 months. CONCLUSION: Our case demonstrates that rhesus D alloimmunization may present as a pseudosyndrome with transient congenital hyperinsulinism, anemia, and inspissated bile syndrome with conjugated hyperbilirubinaemia, despite anti-D immunoglobulin prophylaxis, possibly due to late fetomaternal hemorrhage.