Search results for "alas"
showing 10 items of 929 documents
Glutathione S Transferase Polymorphisms Influence on Iron Overload in β-Thalassemia Patients
2013
In patients with β-thalassemia iron overload that leads to damage to vital organs is observed. Glutathione S transferase (GST) enzymes have an antioxidant role in detoxification processes of toxic substances. This role is determined genetically. In this study, we correlated GSTT1 and GSTM1 genotypes with iron overload measured with direct and indirect non-invasive methods; in particular, we used serum ferritin and signal intensity of the magnetic resonance image (MRI) in 42 patients with β-thalassemia, which were regularly subjected to chelation and transfusion therapy. Multiplex polymerase chain reaction was used to determine the genotype. The loss of both alleles leads to a decreased valu…
Oxidative Modification of Low-Density Lipoprotein and Atherogenetic Risk in β-Thalassemia
1998
AbstractWe investigated the oxidative state of low-density lipoprotein (LDL) in patients with β-thalassemia to determine whether there was an association with atherogenesis. Conjugated diene lipid hydroperoxides (CD) and the level of major lipid antioxidants in LDL, as well as modified LDL protein, were evaluated in 35 β-thalassemia intermedia patients, aged 10 to 60, and compared with age-matched healthy controls. Vitamin E and β-carotene levels in LDL from patients were 45% and 24% of that observed in healthy controls, respectively. In contrast, the mean amount of LDL-CD was threefold higher and lysil residues of apo B-100 were decreased by 17%. LDL-CD in thalassemia patients showed a str…
Increased susceptibility to lipid peroxidation in skeletal muscles of dystrophic hamsters.
1989
The results showed that the total content of lipids, which could be peroxidized with Fe(2 +)/ascorbate stimulation in vitro, was 45.4% and 53.7% higher than normal in the dystrophic hamster muscle at the age of 1 and 3 months, respectively. Correspondingly, the susceptibility to lipid peroxidation (stimulated by ADP-chelated iron at 37 degrees C) was 38.6-74.3% higher in dystrophic muscles. The increases were not related to necrotic lesions and inflammation observed. The activities of glucose-6-phosphate dehydrogenase, glutathione reductase, thioredoxin reductase and catalase were increased in dystrophic muscles but those of superoxide dismutases and glutathione peroxidase were unaffected.
Induction of gamma-globin gene transcription by hydroxycarbamide in primary erythroid cell cultures from Lepore patients.
2008
Increased expression of fetal haemoglobin (HbF) may ameliorate the clinical course of beta-thalassemia and sickle cell disease. Some pharmacological agents, such as hydroxycarbamide (HC), can increase fetal haemoglobin synthesis during adult life. Cellular selection and/or molecular mechanisms have been proposed to account for this increase. To explore the mechanism of action of HC we focused on homozygous Hb-Lepore patients that presented with high fetal haemoglobin levels and were good responders to HC treatment "in vivo". We performed primary erythroid cultures from peripheral blood of four homozygous Lepore patients. The increase in HBG (gamma-globin) transcription levels and HbF conten…
The toxic and lethal effects of the trehalase inhibitor trehazolin in locusts are caused by hypoglycaemia
2003
SUMMARY The main blood sugar of locusts is trehalose, which is hydrolysed to two glucose units by trehalase. Homogenates of locust flight muscles are rich in trehalase activity, which is bound to membranes. A minor fraction of trehalase is in an overt form while the remainder is latent, i.e. active only after impairing membrane integrity. Trehazolin, an antibiotic pseudosaccharide,inhibits locust flight muscle trehalase with apparent Ki-and EC50 values of 10–8 mol l–1and 10–7 mol l–1, respectively. Trehazolin is insecticidal: 50 μg injected into locusts completely and selectively blocked the overt form of muscle trehalase (with little effect on latent activity) and killed 50% of the insects…
PROMOTION OF INTRATHYMIC T-CELL DEVELOPMENT BY MHC-Ia-POSITIVE THYMIC MACROPHAGES (Ia + Mø)
1985
The generation of the peripheral T-cell pool is attributed to maturation and differentiation events occurring within the thymus. Three thymic compartments, thymic epithelial cells, thymic hormons and thymic Mo are considered to contribute to the intrathymic T-cell development. While, as we have shown earlier, murine as well as human MHC-Ia-negative-Mo (Ia−Mo) regulate thymocyte (TH) differentiation via suppressive effects (Thymus,6:295,1984) and MHC-Ia+-Mo are involved in intrathymic tolerance induction (Pediatr Res,15:800, 1984), now the contribution of Ia+Mo to intrathymic lymphopoiesis was investigated. An isolation method yielding cell suspensions highly enriched for Ia+ thymic Mo was p…
Achalasia with megaesophagus and tracheal compression in a young patient: A case report.
2015
Highlights • This report emphasizes that physicians should be alert and consider airway obstruction and signs of dyspnea as severe and threatening symptoms in extensive cases of achalasia with megaesophagus. • Early surgical treatment provides a therapeutic option to obviate the occurrence of acute respiratory distress and consecutive complications. • In particular, difficulties in intubation prior to surgery must be considered. Due to potential tracheomalacia, the status of “bull frog neck” in achalasia, including severe tracheal compression caused by megaesophagus with concomitant cervical swelling, may also lead to extubation problems and deserves special care in the postoperative period.
Clinical Presentation and Complications of Achalasia
2001
Patients with achalasia often present with atypical symptoms. If these occur in the context of "normal" morphological findings, the diagnosis may be delayed for several years. However, a careful interview and the use of modern methods that concentrate on pathophysiologic aspects always allow an early diagnosis and the initiation of therapy that affects most but rarely all symptoms. Finally, regardless of whether this therapy remains partially or totally effective, patients require some follow-up since serious and late complications may occur.
Achalasie oder Pseudoachalasie? Fallstricke der Diagnostik und Therapieentscheidung
2007
History Patient 1 (female, aged 55 years) had for some time complained of morning nausea. She reported symptoms of reflux with regurgitation of food for two-and-a-half years and also dysphagia with retrosternal bolus obstruction for the last eighteen months. Patient 2 (male, aged 84 years) complained of restrosternal dysphagia with each intake of food for one year, weight loss of 12 kg and occasional regurgitation of food. Investigations The general condition of patient 1 was only slightly impaired but that of patient 2 markedly reduced. Routine laboratory tests were unremarkable in both. Barium meal in patient 1 revealed fixed narrowing in the region of the esophageal hiatus. The inferior …
The Prognostic Role of Hypertrabeculation By Cardiac Magnetic Resonance in Thalassemia Intermedia Patients
2016
Abstract Background. Differentiation of left ventricle non-compaction (LVNC) from hypertrabeculated LV due to a negative heart remodeling in thalassemia intermedia (TI) can depends on the selected CMR criterion. The recently proposed Piga's criterion (NC/C ratio threshold of >2.5, Am J Haem 2012) seems to have a low specificity to identify the true LVNC in TI. Anyway, the Piga's criterion could well detect easy a negative heart remodeling in TI patients. Purpose: To assess prospectively whether the Piga's criterion has a prognostic role for adverse cardiovascular outcomes in TI patients. Methods. We studied prospectively by CMR 168 TI patients (81 males, mean age 38.32 ±11.61 years) cons…