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1.
Blood transfusion can be a life-saving therapy for β-thalassemia major and β-thalassemia/HbE (β-TM) patients with chronic anemia, major caused severe iron overload particularly in β-TM patients received only blood transfusion therapy. We aim to evaluate the association of iron overload with oxidative stress, liver damage, and elevated very low density lipoprotein cholesterol (VLDL-C) in transfusion-dependent β-TM patients. Serum ferritin, malondialdehyde (MDA), liver profiles, triglycerides levels, and VLDL-C were significantly higher while total cholesterol, low-density lipoprotein cholesterol, high density lipoprotein cholesterol and total antioxidant capacity were lower in β-TM than controls. Serum ferritin was significantly correlated with MDA, liver enzymes and lipid profiles (p < 0.05). Multiple forward stepwise linear regression analyses of the significant variables showed that in these β-TM patients, independent predictors of iron overload were MDA (β = 0.410, r 2 = 0.671, p < 0.001), ALT (β = 0.493, r 2 = 0.578, p < 0.001), and VLDL-C (β = 0.253, r 2 = 0.711, p < 0.001). In conclusion, iron overload associated with increased oxidative stress, lipid peroxidation, liver damage, decreased TC, LDL-C, HDL-C and over production of VLDL-C, is significantly problem in transfusion-dependent β-TM patients. These appeared the major cause of future morbidity and mortality in β-TM patients.  相似文献   

2.
Thalassemia is a congenital hemolytic disease which is treated by repeated blood transfusion. Chronic iron overload is currently considered to be the primary cause of mortality in β-thalassemia, mainly due to the induction of left-sided cardiac failure. Iron overload results from a number of mechanisms associated with the disease itself. In addition to chronic iron overload thalassemic patients are more prone for procoagulant status which in turn lead to clinical thrombotic events. The hypercoagulable state in thalassemia is due to multiple elements, a combination of which is often the drive behind a clinical thromboembolic events. PAI-1 study was done in thalassemia major patients receiving multiple blood transfusion as a marker for procoagulant status. Total of 30 thalassemic patients on repeated blood transfusion was included in the study and total of 30 healthy age and sex matched controls were included in the study. It was also found that there was significant differences between cases and controls. The mean level of PAI 1 in controls was 3047 ± 414 pg/ml, the value in cases was 3683 ± 358 pg/ml. The level was significantly increased (p < 0.05) in the cases compared to controls. PAI-1 levels were also compared with the total number of blood transfusion which correlates well.  相似文献   

3.
Alkaline haemoglobin electrophoresis is a useful tool in diagnosing β-thalassemia and sickle-cell anaemia. In this report, using this simple technique, β-thalassemia associated with sickle-cell anaemia is diagnosed. This is the first case we have diagnosed in our laboratory using agarose gel electrophoresis.  相似文献   

4.
Severe hemolytic anemia in β-thalassemia major and β-thalassemias/HbE (β-TM) patients requires giving blood transfusions. Chronic blood transfusions lead to iron overload consequence with organs damage and risk of alloantibody-formation. This study evaluates the prevalence of red cell alloimmunization and estimates the risk of alloantibody-formation in chronic transfusion-dependent β-TM patients. This cross sectional study was conducted on 143 β-TM patients receiving regular transfusions. We tried to determine the frequency, types and factors influencing red cell alloimmunization in these transfusion-dependent β-TM patients. Median age of 25 (17.5 %) alloantibody-formation β-TM patients was 19.0 years (inter quartile 15.5–24.0 years). The alloantibodies were Anti-Rh (E) (13.1 %), Anti-Rh (D) (0.7 %). Thirty-four patients (23.8 %) of the sample had splenectomies of which 10 (29.4 %) had alloantibody-formation. The interval from first transfusion to antibody development varied from 1.5 to 14 years. Alloantibody-formation correlated with splenectomy and splenectomy correlated with number of transfusion (p < 0.005). In multiple logistic regression used to estimate the risk of alloantibodies formation with splenectomy; OR and 95 % CI were 2.88 (1.07–7.80), p = 0.037 after adjusting for other co-variates. The rate of red cell alloimmunization was 17.5 % and splenectomy associated with increased alloantibody-formation in these transfusion-dependent β-TM patients.  相似文献   

5.
The effect of dietary supplementation with Oil sardine (Sardinella longiceps) fish at 2/3 fat replacement, on serum lipids in the cholesterol-stressed copper loaded rats was studied. Hypercholesterolemic state observed during copper toxicity was not alleviated by sardines. Copper loading significantly decreased the triglyceride levels and the activity of β-hydroxy β-methyl glutaryl (HMG) CoA reductase. Fish supplementation was further effective in reducing the triglyceride levels in copper loaded rats. Significant increase in the serum phospholipid was observed in the fish supplemented rats during copper overload. The finding suggests that hypolipidemic potential of fish is certainly altered to some extent in copper toxicity since, it is well known that copper enhances the formation of free radicals. Thus it may be concluded that sardine fish is not effective in reducing the cholesterol levels during copper toxicity.  相似文献   

6.
DNA samples from a family (parents and a son) with hereditary persistence of fetal hemoglobin (HPFH) condition were subjected to amplification of a 1.214 kbp DNA fragment from β-globin gene using polymerase chain reaction (PCR). The aim of this study was to identify the type of HPFH i.e. deletional or non deletional. Non deletional type of HPFH was identified in two samples and moreover, these samples were found to be associated with 619bp β°-thalassemia deletion. This is the first report on the association of non deletional HPFH with 619bp β°-thalassemia deletion.  相似文献   

7.
Hemoglobin (Hb) Grey Lynn is a Hb variant caused by a substitution of Phe for Leu at position 91 of α1-globin chain, originally described in individual of unknown ethnic background. This article addresses the interaction of Hb Grey Lynn with a non-deletional α+-thalassemia found in Thailand, a hitherto un-described condition. The proband was adult Thai woman referred for investigation of mild anemia with Hb 90 g/L. Hb analyses using low pressure liquid chromatography raised a suspicion of abnormal Hb presence, which was failed to demonstrate by cellulose acetate electrophoresis and capillary electrophoresis. DNA sequencing identified a CTT (Leu) to TTT (Phe) mutation at codon 91 corresponding to the Hb Grey Lynn (Vientiane) [α91(FG3)Leu>Phe (α1) on α1-globin gene and a C deletion between codons 36 and 37 on α2-globin gene causing α+-thalassemia. As compared to those observed in a compound heterozygote for Hb Grey Lynn / α0-thalassemia reported previously, higher MCV (81.7 fL) and MCH (26.3 pg) values with a lower level of Hb Grey Lynn (19.7%) were observed in the proband. The normochromic normocytic anemia observed could be due to the interaction of Hb Grey Lynn with α+-thalassemia. The two mutations could be identified using PCR-RFLP and allele-specific PCR assays developed.  相似文献   

8.
Hemoglobin D exist in four form; HbD trait, HbD-thalassemia, HbD sickle cell and HbD homozygous. HbD trait and HbD homozygous generally asymptomatic condition but when HbD co-inherit with thalassemia and sickle cell anemia, produces clinically significant conditions like chronic hemolytic anemia. Here we present a case of HbD Punjab with α 3.7 kb deletion and IVS-1-5 β-thalassemia across a family. Diagnosis of HbD patient was performed by high performance liquid chromatography and complete blood count was measured by automated cell analyzer. Molecular study for common alpha deletions done by Gap-PCR while beta thalassemia mutation identified by ARMS-PCR. Case was clinically significant due to the inheritance of HbD/β+thalassemia genotype. Thus observed case behaved like thalassemia intermedia due to co-existence of α 3.7 deletions with IVS 1-5 β-thalassemia mutation in HbD Punjab patient.  相似文献   

9.
Free iron in serum has been found in several disease conditions including diabetes. In the present work, we studied the relationship between free iron, fasting blood glucose (FBG) and glycated haemoglobin (HbA1c). Study was carried out on 50 type 2 diabetes cases under poor glycemic control associated with complications, 53 type 2 diabetes cases under good glycemic control and 40 healthy controls. We estimated free iron, both ferrous (Fe+2) and ferric (Fe+3) form, protein thiols, lipid hydroperoxides, FBG, HbA1c and serum ferritin levels in serum. There was a significant increase in free iron in Fe+3 state (p <0.01), HbA1c (p<0.01), serum ferritin (p<0.01), lipid hydroperoxides (p<0.01) and significant decrease in protein thiols (<0.01) in diabetes cases under poor glycemic control compared to diabetes cases under good glycemic control and healthy controls. Free iron correlated positively with HbA1c (p<0.01). Poor glycemic control and increase in glycation of haemoglobin is contributing to the increase in free iron pool which is known to increase oxidant generation.  相似文献   

10.
The aim of the present work is to understand the lipid peroxidation of RBC membrane and the spectrin protein content of RBC membrane cytoskeleton of thalassaemic carrier state (trait) of β and hemoglobin E variant (HbE). We have measured the hemoglobin (Hb), malondialdehyde (MDA) and spectrin content of RBC membrane of thalassaemic carrier. The spectrin content (α and β band) of both β and HbE carrier was not changed than normal individuals. However, lipid peroxidation of RBC membrane was significantly increased in both β and HbE trait, and Hb level was also decreased in thalassaemic carrier. It may be assumed that oxidative damage by excess lipid peroxidation may have no role on irreversible membrane damage in β thalassaemia and HbE thalassaemia carrier.  相似文献   

11.
Serum β2-microglubin (β2-m) levels were measured in oral carcinoma patients and compared with normal healthy controls. It was observed that there was a significant rise in serum β2-microglobulin in oral carcinoma patients. Progressively higher values were obtained as the cancer advanced clinically. Therefore the estimation of serum β2-microglobulin may be useful as one of a battery of tests in the assessment of oral carcinoma patients.  相似文献   

12.
Eight weeks of latent iron deficiency in weaned female rats of Sprague Dawley strain maintained on experimental low-iron diet (18–20 mg/Kg) did not significantly change the gross body weight and tissue weights of brain and liver. Packed cell volume (PCV) and hemoglobin concentration remained unaltered. However, non-heme iron content in liver and brain decreased significantly (P<0.001). The activities of glutamate dehydrogenase, glutamic acid decarboxylase, and GABA-transaminase (GABA-T) in brain decreased by 15%, 11.4% and 25.7% respectively. However, this decrease was not statistically significant. Binding of3H Muscimol at pH 7.5 and 1 mg protein/assay increased by 143% (P<0.001) in synaptic vesicular membranes from iron-deficient rats as compared to the controls.3H glutamate binding to the synaptic vesicles was also carried out under similar condition. However, the L-glutamate binding was reduced by 63% in the vesicular membranes of iron deficient animals. These studies in dicate that iron plays an important functional role in both excitatory and inhibitory neurotransmitter receptors.  相似文献   

13.
Eight weeks of latent iron deficiency in weaned female rats of Sprague Dawley strain maintained on experimental low-iron diet (18–20 mg/kg) did not significantly change the gross body, weight and tissue weights of brain and liver. Packed cell volume (PCV) and hemoglobin concentration remained unaltered. However, non-heme iron content in liver and brain decreased significantly (p<0.001). The activities of glutamate dehydrogenase, glutamic acid decarboxylase, and GABA-transaminase (GABA-T) in brain decreased by 15%, 11.4% and 25.7% respectively. However, this decrease was not statistically significant. Binding of3H Muscimol at pH 7.5 and 1 mg protein/assay increased by 143% (p<0.001) in synaptic vesicular membranes from iron-deficient rats as compared to the controls.3H glutamate binding to the synaptic vesicles was also carried out under similar condition. However, the L-glutamate binding was reduced by 63% in the vesicular membranes of iron deficient animals. These studies indicate that iron plays important functional role in both excitatory and inhibitory neurotransmitter receptors.  相似文献   

14.
Little is known of conditions which influence the stability of urinary enzymes upon storage in the bladder at 37°C. Using a continuous flow system simulatingin vivo conditions, we studied the influence of the pH of urine on the stability of two renal parenchymal enzymes N—Acetyl—β—D—Glucosaminidase (2—acetamido—2—deoxy—β—D—glucoside acetamidodeoxy glucohydrolase, NAG, EC 3.2.1.30) and L—Alanine aminopeptidase (Aminopeptidase N, AAP, EC 3.4.11.2). This continuous flow model that we have described can be employed to study the influence of pH on the stability of any renal enzyme excreted in urine. We also studied thein vitro effects of varying concentrations of low molecular weight regulatory metabolites such as urea, creatinine and uric acid and of some drugs excreted in urine, on the assay of these two enzymes. Urinary pH, urea content and some antibiotics seem to influence measured urinary NAG and AAP activities and we therefore express the need for caution before diagnostic interpretation of the urinary enzyme activities are made.  相似文献   

15.
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16.
The amount of content, both on and offline, to which people in reasonably affluent nations have access has increased to the point that it has raised concerns that we are now suffering from a harmful condition of ‹information overload.’ Although the phrase is being used more frequently, the concept is not yet well understood – beyond expressing the rather basic idea of having access to more information than is good for us. This essay attempts to provide a philosophical explication of the concept of information overload and is therefore what philosophers call ‹conceptual analysis’ – a task that, along with normative ethical analysis, is distinctive to Anglo-American style analytic philosophy. I will begin with an analysis of the atomic concepts expressed by the terms ‹information’ and ‹overload’ and then attempt to give a philosophical explanation of the concept of information overload that more precisely identifies exactly what the condition amounts to.  相似文献   

17.
A clinical study was carried out on an antiprogestin, a synthetic steroid, 17 Hyroxy-11 β-(4-dimethyl amino-phenyl-1)—17 (Prop-1-ynyl-estra—4, 9, dien-3-one) for the termination of pregnancies in female subjects within 14 days of missed menstrual period and with positive urine pregnancy test (Gravindex Test). The compound was tested for its efficacy as an abortifacient and its acceptance by human subjects was assessed by measuring various biochemical parameters before and after introduction of the drug. The parameters measured in the subjects included, fasting blood sugar, blood urea, SGOT, SGPT, serum alkaline phosphatase, serum bilirubin, serum creatinine, serum proteins, A/G ratio and serum Na+, K+. Statistically insignificant variation was noted in all the parameters studied. This clearly shows that the drug is very well accepted by human subjects. 70% of the subjects tested in the pilot study aborted within 4 days of introduction of the drug. Overall, 90% of the subjects tested aborted as a result of introduction of the drug.  相似文献   

18.
Multiple myeloma is a group of B-cell disorders resulting in the secretion of a specific and unique monoclonal immunoglobulin (M-protein). Protein electrophoresis is advised whenever multiple myeloma is suspected. The monoclonal protein migrates as a single entity in the electric field and is detected by the non-specific protein stain as a more intensely stained band superimposed on the usual protein pattern. The M-protein usually migrates in the gamma or beta region of the normal protein pattern; very rarely it may appear in the α2 or even in α1 region. Here we have given an atypical case presentation where the patient with multiple myeloma presented with two M-spike one each in α2 and β-globulin region on agarose gel protein electrophoresis with hypoglobulinemia but with reversed A:G ratio.  相似文献   

19.
In this study, we have examined the effect of linking of bloactive fragment of human IL-1β (163–171) or polytuftsin (PT, a synthetic polymer of natural immunomodulator “tuftsin”) with synthetic peptides of HIV on the induction of immune response to the synthetic peptides. A panel of synthetic peptides representing defined region of gp41, gp120 and gag were used as antigens. Immunomodulators linked peptides (i.e. peptide-IL-1β or peptide-PT) or peptide dimers were employed for immunization in Balb/c mice. Mice immunized with the peptide-immunomodulator develop effective T-cell proliferation,in vitro cytokine release and higher antibody production, but not with peptide dimers. We also found that peptide-immunomodulators induced high level of IgG2a antibody production. Furthermore, there was a positive correlation between the levels of cytokine (IL-2 & IFN-γ) and IgG isotype production. Thus it would appear that incorporation of IL-1β fragment or PT selectively enhances the Th1 type response to these peptides and may therefore be important for virus neutralization and clearance. However, the effect of IL-1β fragment was found to be more pronounced than polytuftsin. Such an approach may provide effective vaccination against other infectious diseases.  相似文献   

20.
Iron is an essential trace element and plays a number of vital roles in biological system. It also leads the chains of pathological actions if present in excess and/or present in free form. Major portion of iron in circulation is associated with transferrin, a classical iron transporter, which prevent the existence of free iron. The fraction of iron which is free of transferrin is known as “non transferrin bound iron”. Along with the incidence in iron over loaded patient non transferrin bound iron has been indicated in patients without iron overload. It has been suggested as cause as well as consequence in a number of pathological conditions. The major organs influenced by iron toxicity are heart, pancreas, kidney, organs involved in hematopoiesis etc. The most commonly suggested way for iron mediated pathogenesis is through increased oxidative stress and their secondary effects. Generation of free oxygen radicals by iron has been well documented in Fenton chemistry and Haber–Weiss reaction. Non transferrin bound iron has obvious chance to generate the free reactive radicals as it is not been shielded by the protective carrier protein apo transferrin. The nature of non transferrin bound iron is not clear at present time but it is definitely a group of heterogenous iron forms free from transferrin and ferritin. A variety of analytical approaches like colorimetry, chromatography, fluorimetry etc. have been experimented in different research laboratories for estimation of non transferrin bound iron. However the universally accepted gold standard method which can be operated in pathological laboratories is still to be developed.  相似文献   

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