Impact of ABO blood group antigens on residual factor VIII levels and risk of inhibitor development in hemophilia A.

Background: The clinical phenotype of hemophilia A (HA) does not always correlate with severity. Similarly, the presence of inhibitors does not necessarily increase the risk of bleeding. This paradox between clinical and laboratory findings may be partially attributed to non-modifiable factors, such as blood group, which is known to influence FVIII levels in healthy individuals.

Inhibitor; An Uncommon But Vexing Challenge In North Indian Patients With Hemophilia A.

Factor VIII replacement is the mainstay of treatment in hemophilia A but may lead to the development of inhibitors. While a vexing clinical problem, some observations suggest that the presence of inhibitors may not necessarily portend a higher bleeding risk. Our aim was to assess the prevalence and clinicopathological correlates of inhibitors in a well characterized cohort of Indian patients with HA patients.

Ectopic clotting factor VIII expression and misfolding in hepatocytes as a cause for hepatocellular carcinoma.

Hemophilia A gene therapy targets hepatocytes to express B domain deleted (BDD) clotting factor VIII (FVIII) to permit viral encapsidation. Since BDD is prone to misfolding in the endoplasmic reticulum (ER) and ER protein misfolding in hepatocytes followed by high-fat diet (HFD) can cause hepatocellular carcinoma (HCC), we studied how FVIII misfolding impacts HCC development using hepatocyte DNA delivery to express three proteins from the same parental vector: (1) well-folded cytosolic dihydrofolate reductase (DHFR); (2) BDD-FVIII, which is prone to misfolding in the ER; and (3) N6-FVIII, which folds more efficiently than BDD-FVIII. One week after DNA delivery, when FVIII expression was undetectable, mice were fed HFD for 65 weeks.

Emerging application of robust data envelopment analysis for optimization of graft copolymerization of poly(2-hydroxyethyl methacrylate) to Tamarindus indica seed polysaccharide.

A newer application of data envelopment analysis (DEA) model with robust data envelopment analysis (RDEA) was presented for optimization of reaction variables of graft copolymerization of 2-hydroxyethyl methacrylate (HEMA) to Tamarindus indica seed polysaccharide (TSP). It helped to find out the most appropriate reaction conditions and variables (concentrations of HEMA and reaction initiator; temperature and time duration) for copolymerization. The data generated through the experimental work has been analyzed and indexed to predict the maximum %grafting.

Acquired Hemophilia A: Experience of a Tertiary Care Institute from North India.

Acquired hemophilia A (AHA) is an uncommon bleeding disorder infrequently reported among Indians. The present retrospective data comprises eight cases of AHA over a period of 15 years. The mean age of patients was 59.

Cyclophosphamide Treatment for Acquired Factor VIII Inhibitor in a Patient with AIDS-Associated Progressive Multifocal Leukoencephalopathy.

Acquired hemophilia A (AHA) is a severe bleeding disorder with high mortality rates resulting from the development of autoantibodies to factor VIII (FVIII). Patients typically present with hemorrhages in the skin, subcutaneous tissues, and muscles, which are frequently severe. They can also develop life-threatening retroperitoneal hematomas and compartment syndromes.

Antioxidants reduce endoplasmic reticulum stress and improve protein secretion.

Protein misfolding in the endoplasmic reticulum (ER) contributes to the pathogenesis of many diseases. Although oxidative stress can disrupt protein folding, how protein misfolding and oxidative stress impact each other has not been explored. We have analyzed expression of coagulation factor VIII (FVIII), the protein deficient in hemophilia A, to elucidate the relationship between protein misfolding and oxidative stress.

Genetic structure of Plasmodium falciparum field isolates in eastern and north-eastern India.

Background: Molecular techniques have facilitated the studies on genetic diversity of Plasmodium species particularly from field isolates collected directly from patients. The msp-1 and msp-2 are highly polymorphic markers and the large allelic polymorphism has been reported in the block 2 of the msp-1 gene and the central repetitive domain (block3) of the msp-2 gene. Families differing in nucleotide sequences and in number of repetitive sequences (length variation) were used for genotyping purposes.

Elbow arthropathy in hemophilia.

Twenty-one affected elbows in 14 known hemophilic patients undergoing treatment at the Hemophilia Clinic of our institution between 1994 and 1997 were evaluated using the clinical evaluation score of the Orthopaedic Advisory Committee of the World Federation of Hemophilia and radiological examination using the Pettersson score. The mean age of patients was 16.2 years (range 10-25 years), and all patients except 1 had severe hemophilia with factor VIII levels less than 1% of normal.