hemophilia a/phosphatase

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Correction by factor VIII of decreased neutrophil alkaline phosphatase activity in classic haemophilia and von Willebrand's disease.

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Neutrophil alkaline phosphatase (NAP) score was estimated in ten patients with hereditary disorders in which antihaemophilic factor (factor VIII) is functionally deficient. NAP scores were estimated immediately before cryoprecipitate was administered, and following correction of patients' clinical

Correction by factor VIII of decreased neutrophil alkaline phosphatase activity in classic haemophilia and von Willebrand's disease.

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[Alkaline phosphatase activity in the granulocytes of patients with hemophilia].

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Neutrophil alkaline phosphatase activity in haemophilia A.

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Association of severe haemophilia A with osteoporosis: a densitometric and biochemical study.

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Following a femoral neck fracture and vertebral compression fractures in two patients with severe haemophilia A, bone density and turnover were measured in 19 males with severe haemophilia A (all HIV negative, 18/19 hepatitis C antibody positive) and in 19 age/sex matched controls. Bone density at

Antibodies to factor VIII in plasma of patients with hemophilia A and normal subjects.

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Non-neutralizing factor VIII (FVIII) antibodies (FVIII-Ab) in hemophilia A may be associated with an abnormal clinical response to FVIII concentrates. Patients with FVIII inhibitors may develop noncoagulation FVIII-Ab after the induction of immunotolerance. Natural FVIII-Ab may be detected in the

Phenotypic correction of murine hemophilia A using an iPS cell-based therapy.

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Hemophilia A is caused by mutations within the Factor VIII (FVIII) gene that lead to depleted protein production and inefficient blood clotting. Several attempts at gene therapy have failed for various reasons-including immune rejection. The recent generation of induced pluripotent stem (iPS) cells

Sustained phenotypic correction of canine hemophilia B after systemic administration of helper-dependent adenoviral vector.

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We have evaluated the potential of liver-directed, helper-dependent adenoviral (HDAd) vector-mediated gene therapy in the hemophilia B dog. Two dogs were injected intravenously with HDAd (3 x 10(12) VP/kg) bearing a liver-restricted canine coagulation factor IX (FIX) expression cassette. After

The risk of elective orthopaedic surgery for haemophilia patients: Japanese single-centre experience.

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Haemophilic arthropathy causes pain and a severely restricted range of motion, and results in a significant reduction in quality of life. When conservative treatments have failed, orthopaedic surgery is recommended for these patients with severe haemophilic arthropathy. However, surgery for

[A Preliminary Exploration on the Pathogenesis of Osteopenia in Patients with Hemophilia].

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OBJECTIVE To investigate the influencing factors and pathogenesis of osteopenia in the patients with hemophilia. METHODS Twenty-three patients with hemophilia were admitted in the hospital affiliated to North China University of Science and technology from March to August 2015, including 13 severe

Impact of Prophylaxis on Bone Mineral Metabolism in Children With Hemophilia.

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In this study, we aimed to investigate changes in calcium (Ca) metabolism in hemophilia patients (PWH). We also aimed to investigate the importance of diagnosis and treatment of factors impairing calcium metabolism and the significance of early diagnosis and prophylaxis with respect to these

Erythrocyte enzyme allotypes in the X-linked recessive disorders, Duchenne muscular dystrophy and haemophilia-A hemizygotes and heterozygotes.

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The erythrocyte enzyme-systems acid phosphatase, phosphoglucomutase, glutamate pyruvate transaminase, adenosine desaminase, adenylate kinase, glyoxase, glucose-6-phosphate dehydrogenase and esterase-D-isoenzyme phenotypes were studied for their percentile distribution and were compared with their

An ELISA assay for the detection of factor VIII antibodies - comparison with the conventional Bethesda assay in a large cohort of haemophilia samples.

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An enzyme-linked immunosorbent assay (ELISA) has been developed to measure factor VIII antibodies in haemophilia patients. The assay utilizes binding of the antibodies in the plasma to solid phase antigen, i.e. recombinant factor VIII which was subsequently detected by a human polyclonal IgG

Evaluation of Bone Mineral Density in Children With Hemophilia: An Observational Case-Control Study.

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The objectives of this study were to study bone mineral content (BMC), bone mineral density (BMD), vitamin D level, and bone turnover markers in children with hemophilia and compare it with their normal counterparts.This was an observational case-control

Hypovitaminosis D and osteopenia/osteoporosis in a haemophilia population: a study in HCV/HIV or HCV infected patients.

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Recent reports show a correlation between haemophilia and osteoporosis. HIV, HCV and their treatments are independently associated with an increased risk of osteoporosis. Vitamin D plays a pivotal role in bone mineralization. The aim of our study was to compare Vitamin D levels, bone metabolism

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