In patients with pulmonary arterial hypertension (PAH), chronic use of digoxin is linked to greater all-cause mortality and hospitalization for heart failure, according to findings from the single-center Minnesota Pulmonary Hypertension Repository (MPHR) published in the Journal of the American Heart Association. Among individuals with PAH, increased right ventricular (RV) afterload is associated with RV…
In individuals with pulmonary arterial hypertension (PAH), mild changes in right ventricular (RV) glucose metabolism may be associated with patient prognosis, according to findings from a positron emission tomography/magnetic resonance imaging (PET/MRI) hybrid imaging follow-up study published in EJNMMI Research. Among those with undiagnosed or poorly managed PAH, progressive increases in RV afterload that result…
In individuals with paroxysmal nocturnal hemoglobinuria (PNH), treatment with eculizumab is not associated with optimal clinical outcomes, with patients continuing to experience an ongoing burden of disease, according to findings from a retrospective study published in the European Journal of Haematology. Patients with PNH suffer hemolysis, with an elevated risk for thrombosis, along with a…
In individuals with severe hemophilia B, the use of recombinant factor IX Fc fusion protein (rFIXFc) was associated with sustained clinical benefits, including complete resolution of target joints and long-term bleed prevention, according to findings from a post hoc analysis of pooled longitudinal data published in Blood Advances. In patients with severe hemophilia B, the…
In patients with acquired hemophilia A (AHA), clearance of factor VIII (FVIII) inhibitors is needed with treatment, particularly in those with refractory disease, according to findings from 4 case reports published in the Journal of Thrombosis and Haemostasis. Individuals with AHA are prone to experiencing episodes of bleeding, which are associated with a decrease in…
In children 2 years of age or older with cystic fibrosis (CF), treatment with lumacaftor/ivacaftor (LUM/IVA) early in the disease course may modify disease progression, according to findings from a phase 2, placebo-controlled trial published in Annals of the American Thoracic Society. Among individuals with CF, mutations in the gene that encodes the CF transmembrane conductance…
Adult patients with sickle cell disease (SCD) who undergo a splenectomy are at increased risk for venous thromboembolic events (VTEs), according to findings from an observational, retrospective study published in the British Journal of Haematology. About 30% of individuals with SCD may require a surgical splenectomy because of such unpredictable complications as acute splenic sequestration…
Among patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD-PAH), 10-year survival rates are poor, according to findings from a study derived from a Chinese national multicenter, prospective registry and published in Rheumatology. The researchers sought to accomplish the following: Individuals who had undergone right heart catheterization within 1 month prior to…
Pediatric liver transplantation is performed for a variety of indications, including Alagille syndrome (ALGS), biliary atresia, hepatoblastoma, and metabolic liver disease, researchers wrote in a retrospective study published in the journal Digestive and Liver Diseases. They sought to examine data on how posttransplant complications affected long-term survival and quality of life among children who had…
In patients with hemophilia A or B who undergo dental extractions, the use of hemostatic treatments and factor prophylaxis is associated with decreased rates of bleeding, according to findings from a retrospective analysis published in the Journal of Clinical Medicine. Among patients with hemophilia, dental extractions are frequently associated with bleeding and the need for…
