Effect of acute bleeding on daily quality of life assessments in patients with congenital hemophilia with inhibitors and their families: observations from the dosing observational study in hemophilia.

Introduction: Quality-of-life (QOL) assessments in frequently bleeding patients with congenital hemophilia with inhibitors and their families are confounded by preexisting arthropathy and family circumstances. Periodic QOL assessments typically made on nonbleed days may not provide complete reflections of the burden on patients/families.

Aim: To evaluate the impact of bleeding episodes on patients/caregivers/families and the association between monthly QOL scores and patients' average diary experiences.

Evidence for the transmission of parvovirus B19 in patients with bleeding disorders treated with plasma-derived factor concentrates in the era of nucleic acid test screening.

Background: Parvovirus B19 (B19V) is a small, nonenveloped virus that typically causes a benign flu-like illness that occurs most frequently in childhood. The virus is resistant to current viral inactivation steps used in the manufacture of antihemophilic factor concentrates and B19V transmission through these products has been documented. Since 2000, B19V nucleic acid test (NAT) screening of plasma pools has been implemented to further decrease the viral burden in these products, but no study has examined populations using these products to assess the impact of the screening on B19V transmission.

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure.

The most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial, we sought to identify risk factors associated with SCI. In this cross-sectional study, we evaluated the clinical history and baseline laboratory values and performed magnetic resonance imaging of the brain in participants with SCA (HbSS or HbSβ° thalassemia) between the ages of 5 and 15 years with no history of overt stroke or seizures.

Thalassemia-like phenotype in a novel complex hemoglobinopathy with α, β, δ globin chain abnormalities.

The occurrence of multiple abnormalities of α, β, δ, and γ globin genes may lead to unusual and complex phenotypes when they arise simultaneously in the same individual. Here, we report the findings of an African American boy who coinherited 3 heterozygous globin gene abnormalities: the unstable β-globin chain variant; hemoglobin (Hb) Showa-Yakushiji [β110(G12) Leu→Pro], the δ-globin chain variant; HbB2 [δ16(A13) Gly→Arg] and α-thalassemia (α-thal); (α-/αα). Hb Showa-Yakushiji had been previously described in Japanese, Indian, and European populations.

Effects of a single sickling event on the mechanical fragility of sickle cell trait erythrocytes.

Hemolysis contributes to the pathology associated with sickle cell disease. However, the mechanism of hemolysis or relative contribution of sickling due to hemoglobin (Hb) polymerization vs. oxidative damage remains unknown.

Comparison of glycated albumin and hemoglobin A1c concentrations in diabetic subjects on peritoneal and hemodialysis.

Background: Relative to hemoglobin A(1c) (HbA(1c)), percentage of glycated albumin (GA%) more accurately reflects recent glycemic control in diabetic hemodialysis (HD) patients.

Methods: To determine the accuracy of glycemic assays in a larger sample including patients on peritoneal dialysis (PD), HbA(1c) and GA% were measured in 519 diabetic subjects: 55 on PD, 415 on HD, and 49 non-nephropathy controls.

Results: Mean +/- SD serum glucose levels were higher in HD and PD patients relative to non-nephropathy controls (HD 169.

Rapid quantitation of hemoglobin S in sickle cell patients using the Variant II Turbo analyzer.

A rapid ( approximately 90 sec), fully automated method is described for quantifying hemoglobin S (HbS) by high performance liquid chromatography (HPLC) using the Bio-Rad Variant II Turbo analyzer. Although this instrument is designed to quantify only blood hemoglobin A1c (HbA1c), we show that it can also quantify accurately, without modification, HbS levels in sickle cell patients, provided the blood samples meet certain conditions. The samples should contain detectable hemoglobin F (HbF), but should not contain hemoglobin C (HbC).

Dynamic vascular analysis shows a hyperemic flow pattern in sickle cell disease.

Background: By the age of 20 years, 10% of sickle cell disease (SCD) patients have experienced a stroke. It is unclear if SCD stroke is due primarily to hemodynamic effects of intracranial stenosis, or metabolic failure from anemia. Transcranial Doppler ultrasound (TCD) identifies a SCD subgroup with high stroke risk, but high mean flow velocity (MFV) can be due to stenosis or high flow rate, as with metabolic hyperemia of severe anemia.