Low-Dose Emicizumab Versus Low-/Intermediate-Dose Factor VIII Secondary Prophylaxis for Noninhibitor Haemophilia A Patients With Severe Bleeding Phenotype.

Background: Subcutaneous emicizumab, a factor VIII (FVIII)-mimicking bispecific monoclonal antibody, can effectively prevent bleeds in haemophilia A (HA) patients with/without inhibitors; however, its standard-dose regimens are financially burdensome. Low-dose emicizumab prophylaxis may alternatively be applied to noninhibitor HA patients in resource-limited settings.

Methods: During 2023, Thai patients with noninhibitor severe HA or moderate HA with severe bleeding phenotype (historical annualized bleeding rate [ABR] >5 bleeds/year before regular FVIII prophylaxis) who received low-/intermediate-dose FVIII secondary prophylaxis ≥8 months were enrolled.

Nontransferrin-bound iron in the plasma of haemodialysis patients after intravenous iron saccharate infusion.

Background: Many haemodialysis patients treated with recombinant human erythropoietin (r-HuEPO) receive intravenous iron supplementation on a regular basis. It has been shown previously that this may result in a transient "oversaturation" of transferrin.

Methods: Ten stable haemodialysis patients on r-HuEPO treatment received 100 mg iron saccharate in 60 min, and 1 week later 100 mg in 6 min.

Biliary and urinary metabolic profiles of 1,2-diethyl-3-hydroxypyridin-4-one (CP94) in the rat.

This study compares the biliary and urinary metabolic profiles of 1,2-diethyl-3-hydroxypyridin-4-one (CP94), an orally active iron chelator, in the normal rat. Surprisingly, CP94 was found to form two phase II metabolites, the 3-O- and 4-O-glucuronides. These glucuronides accounted for 38 and 28% of the administered CP94 dose, in bile and urine, respectively.

Determination of non-transferrin-bound iron in genetic hemochromatosis using a new HPLC-based method.

Background/aims: Non-transferrin-bound iron may play a major pathogenic role in iron overload diseases due to its high hepatic uptake and potential damaging effect. The aim of this study was to evaluate the relevance of measuring serum non-transferrin-bound iron levels in genetic hemochromatosis using a new high performance liquid chromatography-based method.

Methods: This method includes a presaturation step of transferrin with cobalt(II) in order to avoid secondary deplacement of non-transferrin-bound iron toward transferrin during the assay.

Non-transferrin-bound iron is present in serum of hereditary haemochromatosis heterozygotes.

Background: Hereditary haemochromatosis (HH) is a common autosomal recessive disease. Recently, HH heterozygosity has been identified as an independent risk factor for myocardial infarction and cardiovascular mortality. Iron may play an important role in atherogenesis by catalyzing peroxidation of low-density-lipoprotein (LDL), an essential step in atherogenesis.