Isolated congenital factor VII deficiency

Congenital factor VII (FVII) (proconvertin) is a rare autosomal recessive bleeding disorder. Bleeding manifestations and clinical findings vary widely, ranging from being asymptomatic to life-threatening bleeding. Intracranial bleeding is relatively less common with inherited FVII deficiency than with other coagulation disorders. We report a rare case of congenital FVII deficiency in an 11-year-old male child. The patient had recurrent subdural hemorrhages. The prothrombin time was markedly prolonged with a normal bleeding time, normal partial thromboplastin time and normal platelet count. Treatment consists of replacement therapy with fresh frozen plasma, prothrombin complex concentrates or plasma-derived FVII concentrates, and/or recombinant factor VIIa. Clinical heterogeneity is the hallmark of this disorder

Characterization of Copper-based Ayurved Medicine Tamra bhasma produced by various manufacturers and its Pharmacokinetic profiling in Wistar rat

Background: Tamra bhasma (TB) is copper based herbo-metallic preparation which is used extensively by Ayurvedic practitioners. Tamra bhasma is endorsed for different disorders of liver, abdominal pain, heart disease, colitis, tumors, anemia, loss of appetite, tuberculosis, as well as eye problems. Objective: Our aim is to characterize 5 commercial TB preparations from 5 different manufacturers by using modern scientific techniques and to study there bioavailability in Wistar rats. Materials and Methods: Tamra bhasma was characterized by X-ray diffraction (XRD), Scanning electron microscope (SEM), Energy Dispersive X-ray analysis (EDAX), Nanoparticle tracking analyzer (NTA), Inductively coupled plasma optical emission spectroscopy (ICP-OES). Bioavailability of Tamra bhasma was studies using non compartmental rat model with daily dose of 6.45mg/kg according to their body weight. Results: The colour of one of the TB preparation was different from other 4 TB samples. The chemical phase and particle size is significantly different for all the 5 TB’s. Pharmacokinetic model confirms difference in various PK parameters such as peak concentration (Cmax), half-life (t1/2) and terminal elimination slope (λz) for all 5 TB’s. TB-A showed highest Cmax (82.21 mg/L), whereas TB-E showed lowest Cmax (48.69 mg/L). The highest bioavailability of TB is may be due to specific chemical moiety and morphology. Based on XRD and elemental analysis, it was found that manufacturing route followed for one of the preparation is not as per ayurvedic text reference. Conclusions: The morphology as well as chemical phase of the five TB’s studied were different from each other, which might be responsible for different pharmacokinetic profiles in Wistar rat model