Purpose In the critically ill, hospital-acquired bloodstream infections (HA-BSI) are associated with significant mortality. Granular data are required for optimizing management, and developing guidelines and clinical trials. Methods We carried out a prospective international cohort study of adult patients (≥ 18 years of age) with HA-BSI treated in intensive care units (ICUs) between June 2019 and February 2021. Results 2600 patients from 333 ICUs in 52 countries were included. 78% HA-BSI were ICU-acquired. Median Sequential Organ Failure Assessment (SOFA) score was 8 [IQR 5; 11] at HA-BSI diagnosis. Most frequent sources of infection included pneumonia (26.7%) and intravascular catheters (26.4%). Most frequent pathogens were Gram-negative bacteria (59.0%), predominantly Klebsiella spp. (27.9%), Acinetobacter spp . (20.3%), Escherichia coli (15.8%), and Pseudomonas spp . (14.3%). Carbapenem resistance was present in 37.8%, 84.6%, 7.4%, and 33.2%, respectively. Difficult-to-treat resistance (DTR) was present in 23.5% and pan-drug resistance in 1.5%. Antimicrobial therapy was deemed adequate within 24 h for 51.5%. Antimicrobial resistance was associated with longer delays to adequate antimicrobial therapy. Source control was needed in 52.5% but not achieved in 18.2%. Mortality was 37.1%, and only 16.1% had been discharged alive from hospital by day-28. Conclusions HA-BSI was frequently caused by Gram-negative, carbapenem-resistant and DTR pathogens. Antimicrobial resistance led to delays in adequate antimicrobial therapy. Mortality was high, and at day-28 only a minority of the patients were discharged alive from the hospital. Prevention of antimicrobial resistance and focusing on adequate antimicrobial therapy and source control are important to optimize patient management and outcomes. Supplementary Information The online version contains supplementary material available at 10.1007/s00134-022-06944-2.

Thrombotic thrombocytopenic purpura (TTP) either occurs in a congenital form caused by ADAMTS13 gene mutations or it is acquired and most often due to ADAMTS13 inhibitory autoantibodies. In congenital TTP siblings are often affected, while acquired TTP occurs sporadically and familial clustering has not been described so far. We report identical twin sisters suffering from acquired TTP due to immunoglobulin G (IgG) autoantibodies inactivating ADAMTS13, suggesting an important role of hitherto unidentified genetic determinants of ADAMTS13 inhibitor formation. These cases also demonstrate that familial clustering is not sufficient for unambiguously diagnosing hereditary ADAMTS13 deficiency and congenital TTP. IntroductionThe metalloprotease ADAMTS13 (A disintegrin and metalloprotease with thrombospondin type 1 domains-13) 1-5 specifically cleaves the von Willebrand factor (VWF) subunit at the peptide bond Tyr842-Met843. 6,7 A severely deficient ADAMTS13 activity (Ͻ 5% of that in normal plasma) was found to be a strong risk factor for thrombotic thrombocytopenic purpura (TTP). 8,9 There are 2 fundamental mechanisms known to cause severe ADAMTS13 deficiency. Homozygous or compound heterozygous mutations of the ADAMTS13 gene lead to hereditary ADAMTS13 deficiency in congenital TTP, 3,10-14 often affecting siblings. In contrast, most cases of acquired TTP are caused by autoantibodies inactivating ADAMTS13. 8,9,15 Acquired TTP occurs sporadically, and familial clustering has not been described so far. Distinction between both forms is important as their management may differ. While regular infusion of limited amounts of fresh frozen plasma (FFP) usually reverts or prevents disease manifestations in congenital TTP, plasma exchange with FFP replacement, often combined with corticosteroids or other immunosuppressants, is the current therapy of choice in acute acquired TTP. 16 We report identical twin sisters suffering from acquired TTP due to severe ADAMTS13 deficiency caused by circulating inhibitory immunoglobulin G (IgG) autoantibodies. Study design PatientsPreviously healthy identical twin sisters suffered from a first episode of acute TTP at 23 (sister 1) and 24 (sister 2) years of age. Sister 1 presented with fever, neurologic symptoms, thrombocytopenia, and microangiopathic hemolytic anemia. Since therapy was initiated promptly, symptoms were less pronounced in sister 2. These episodes resolved under plasma exchange with FFP replacement and corticosteroids; sister 1 received vincristine, in addition. Follow-up for now 37 (sister 1) and 25 (sister 2) months was uneventful except for a short relapse in sister 1, which occurred 13 months after the initial episode and was treated by plasma exchange. Since then, no plasma has been administered to either of the sisters.Both sisters are otherwise healthy without indication of another autoimmune disease, especially systemic lupus erythematosus (SLE), and have the same living and working conditions. No pregnancy has so far occurred in either sister. No drugs, including ora...

Adaptive rowing is rowing or sculling for rowers with a physical disability. It debuted at the Paralympic Games in 2008. In order to ensure an equitable playing field, rowers with similar levels of physical function and disability are classified into different sport classes for competition. Rowers with an inability to use a sliding seat and impaired trunk function resulting in an inability to perform trunk forward and backward lean via hip flexion/extension are assigned to the Arms and Shoulders (AS) class. AS rowers have to use a chest strap set immediately below the chest in order to localize any trunk movement in AS class. Conditions created by adaptations of rowing equipment and technique within the AS class create unique stresses on the upper thoracic region. The following case report demonstrates how etiology and management of a rib stress fracture in an AS rower differs in comparison to able-body rowers. Of significant importance were the limitations imposed on the rower’s ability to maintain rowing-specific fitness, due to the nature of the rib stress fracture and requirement to decrease force transmission through the ribs for several weeks. The rower’s gradual return to full training was further impacted by obligatory use of the chest strap, which directly applied pressure over the injured area. Protective orthosis for the chest was designed and applied in order to dissipate pressure of the chest strap over the thorax during rowing (most importantly at the catch position) both on the ergometer and in the boat.