Background: Bernard-Soulier syndrome is a rare inherited bleeding disease caused by quantitative or qualitative defect of GPIb/IX/V, a platelet complex that binds the Von Willebrand factor. The expression of GPIb-IX-V complex can be evaluated by flow cytometry and confirmed by the absence of ristocetin-induced platelet aggregation in platelet-rich plasma. The main aim of the present study was t...

background: bleeding events in hemophilic patients with inhibitors are managed by bypassing agents. currently available agents in iran are recombinant activated factor vii (rfviia; aryogen, aryoseven) and feiba (factor eight inhibitor bypassing agent). no standardized and accurate assay is currently available for monitoring the effectiveness of bypassing agents. we suggested that history of the...

background: bernard-soulier syndrome is a rare inherited bleeding disease caused by quantitative or qualitative defect of gpib/ix/v, a platelet complex that binds the von willebrand factor. the expression of gpib-ix-v complex can be evaluated by flow cytometry and confirmed by the absence of ristocetin-induced platelet aggregation in platelet-rich plasma. the main aim of the present study was t...

friedreichs ataxia (frda) is an inherited recessive disorder characterized by progressive neurological disability and heart abnormalities. a deficiency in the protein frataxin causes this disease. frataxin deficiency leads to progressive iron accumulation in mitochondria, excessive free radical production and dysfunction of respiratory chain complexes. the expansion (gaa) repeat in the first in...

multiple sclerosis (ms) is a demyelinating disease of the central nervous system characterized by morphological hallmarks of inflammation, demyelination and axonal loss. to date, little attention has been paid to the contribution of mitochondrial respiratory chain enzyme activities to ms. in this study, kinetic analysis of mitochondrial respiratory chain complex i enzyme (measured as nadh ferri...

disconnects and defects in care – such as duplication, poor integration between services or avoidable adverse events – are costly to the health system and potentially harmful to patients and families. for patients living with multiple chronic conditions, such disconnects can be particularly detrimental. lean is an approach to optimizing value by reducing waste (eg, duplication and defects) and ...

a nested polymerase chain reaction (pcr) assay with two pairs of primers was applied for the detection of m. tuberculosis complex dna in specimens of 51 patients. of these, 31 clinically diagnosed patients (group i) recruited from july 1996 to june 1997 were given antituherculous chemotherapy from one to six months with standard daily regimen (isaniazid, 5 mg/kg; rifampin, 10 mg/kg; pyrazinamit...