Osteoporosis/osteopenia and hemophilic arthropathy in severe hemophilic patients

Types A and B hemophilia are coagulation disorders associated with many complications. Osteoporosis is a skeletal condition characterized by the decreased density of normally mineralized bone. This study aims to determine the relationship between osteoporosis and hemophilic arthropathy in severe hemophilia patients over the age of 20 years in Kerman, Iran. We performed a cross-sectional study of bone density among 40 male patients with severe hemophilia. Lumbar spine and femoral bone mineral density [BMDs] were measured using a Dual Energy X-ray Absorptiometry [DEXA] scan. The T-scores for BMDs were computed and values from -2.5 to -1 were considered as osteopenia. Those less than -2.5 were considered to be osteoporotic. About 42% of patients had normal BMD, 50% were osteopenic, and the rest were osteoporotic. The mean BMI, number of arthropathic joints, and the numbers of joint bleeding during the previous year were significantly higher in osteoporotic patients than osteopenic and normal groups [P = 0.05, = 0.003 and = 0.011, respectively]. The mean for factor replacement, the number of joint bleeding episodes in the past year, and the number of arthropathic joints were significant independent predictors of both spinal and femoral BMD. Their odds ratios [OR] were 1.29 [factor replacement], 1.17 [numbers of joint bleeding episodes], and 1.73 [number of arthropathic joints], which were significant [P < 0.05]. Our results suggest that men with severe hemophilia have reduced BMD. Patients at risk are those with signs of hemophilic arthropathy. Because osteoporosis may complicate the future treatment of patients with hemophilia, screening of patients with hemophilic arthropathy for reduction of BMD and preventive therapies is highly recommended

Perform gene analaysis in an Iranian family with familial hemophagocytic lytnphohistiocytosis

Perform gene [PRF1] mutations have been reported in 20-30% of patients with familial hemophagocytic lymphohistiocytosis [FHL], an immune disorder of infancy and early childhood. Cytotoxic T and natural killer [NK] cell activities are remarkably reduced or absent in FHL patients. We report the first cases of familial hemophagocytic lymphohistiocytosis in an Iranian family with two siblings. Exons 2 and 3 of the PRF1 gene were analyzed by polymerase chain reaction [PCR] amplification and direct sequencing. Perform gene mutation[s] were detected in none of the cases. The result of our study indicates that not much evidence is present concerning a correlation between perforin gene defects and familial hemophagocytic lymphohistiocytosis etiology in these cases

Causes of death registered on death certificates in an Iranian community based on ICD-10

Accurate documentation of the cause of death is critical for health care planning. In this study, the most important causes of death registered on death certificates and the most important issues preventing accurate registration and access to death information in the town of Rafsanjan were surveyed. This was a descriptive study. All death certificates [totally 1197] issued during the year 2004 in Rafsanjan were reviewed. The relevant information was collected on a checklist. Data were analyzed with SPSS. Common issues preventing accurate documentation and access to information about the causes of death were as follows; incongruity between available information in different medical centers, incomplete death certificates, difficult access to death information, inaccurate registration of the real causes of death and illegibility of the registered information. The most common cause of death in males was accident and in females was a cardiovascular disease. The registration system and access to death information requires extensive modification, therefore, information pertaining causes of death must be used and interpreted cautiously

Antioxidant effects of L-serine against fatty streak formation in hypercholesterolemic animals

Peroxidation of blood lipoproteins is regarded as a key event in the development of atherosclerosis. Evidence suggests that oxidative modification of amino acids in low-density lipoprotein [LDL] particles leads to its convert into an atherogenic form, which is taken up by macrophages. Therefore the reduction of oxidative modification of lipoproteins by increasing plasma antioxidant capacity may prevent cardiovascular disease. In this study, the antioxidant and anti-fatty streak effects of L-serine were investigated in hypercholesterolemic rabbits. Rabbits were randomly divided into three groups which were fed high-cholesterol diet [hypercholesterolemic control group], high-cholesterol + L-serine diet [treatment group], and normal diet [control] for twelve weeks and then blood samples were obtained to measure plasma cholesterol, triglyceride [TG], high-density lipoprotein [HDL], low-density lipoprotein [LDL], antioxidant capacity [AC], malondialdehyde [MDA], and conjugated dienes [CDS]. Right and left coronary arteries were also obtained for histological evaluation. No significant difference was observed in plasma cholesterol, TG, HDL, LDL and CDS levels between treatment and hypercholesterolemic control groups [P>0.05]. The levels of plasma MDA and AC were 0.29% micro M and 56%, respectively in the treatment group which showed a significant change in comparison with hypercholesterolemic control groups [P<0.05]. The mean size of produced fatty streak also showed significant reduction in the treatment group compared to the hypercholesterolemic group [P<0.05]. The results showed that L-serine has antioxidant and anti-fatty streak effects without any influence on plasma lipid levels in hypercholesterolemic rabbits