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Biomedical subjects

A Taher

Publications and source records attributed to A Taher.

At least 37 records · Page 2Linked to original sources
PubMed2002

Hepatitis-C-virus genotypes and hepatitis-G-virus infection in Lebanese thalassaemics.

Exposure to hepatitis C virus (HCV), hepatitis G virus (HGV) and the carrier 'rate' for hepatitis B virus (HBsAg) were investigated in thalassaemia patients in Lebanon, a group that has not been studied in the past. The HCV genotypes and their distribution in the 395 thalassaemics, all of whom had been registered at the Chronic Care Center (CCC) in Hazmieh since 1996, were also studied. Of the 55 samples (14%) found positive for anti-HCV, 19 were also positive for HCV RNA. The 19 samples of HCV RNA were mostly of genotype 4 (37%), followed by 1a and 3a (21% each), lb (16%) and 2b (5%). Most (14; 74%) of the 19 HCV-RNA-positive samples, but only 13 (36%) of the 36 samples that were negative for HCV RNA although anti-HCV-positive, were positive for anti-HGV. Among 100 anti-HCV-negative samples, eight (8%) were anti-HGV positive. Only one (0.28%) of all 395 patients investigated was found to be HBsAg-positive. All of the HBV- and HCV-positive patients had initially been found positive in 1996, when they were first registered at the CCC, and none of the remaining patients had seroconverted since. As none of the patients had been checked for anti-HGV until the present study, the history of their exposure to HGV was unknown. These results emphasise the importance of screening all blood donations collected in Lebanon for HBsAg and anti-HCV. This and stringent infection-control measures are necessary steps to limit the spread of HBV, HCV and perhaps HGV to thalassaemics.

Adolescent↗
PubMed2002

Endometrial metastasis from signet-ring breast carcinoma: case report.

Breast cancer rarely metastasizes to the endometrium. The signet-ring variety of breast cancer is very rare and has been reported to metastasize to the endometrium in only three previous reports. We present a patient with signet-ring breast carcinoma with metastasis to the endometrium. The patient presented with vaginal bleeding and was found to have wide spread metastasis. Endometrial metastasis in patients with signet-ring breast cancer usually denotes a poor prognosis.

Breast Neoplasms↗
PubMed2001

Hughes syndrome associated with varicella infection.

We report a case of Hughes syndrome 1 week after the occurrence of chickenpox. The patient presented with the clinical picture of acute right iliofemoropopliteal deep vein thrombosis. IgM and IgG anticardiolipin (aCL) were both positive upon admission. The IgM aCL antibodies were still positive 6 weeks later, which suggests their role as a predisposing factor of deep vein thrombosis.

Adolescent↗
PubMed2001

Comparison between deferoxamine and deferiprone (L1) in iron-loaded thalassemia patients.

INTRODUCTION: Iron-chelating therapy with deferoxamine in patients with thalassemia major has dramatically improved the prognosis of this disease. However, the limitations of this treatment have stimulated the design of alternative orally active iron chelators. OBJECTIVE: To compare the effectiveness and safety of, and compliance with, oral deferiprone (L1), and deferoxamine, in thalassemia major patients. METHODS: All patients were followed up in one center in Lebanon. Sixteen patients were on L1 (75 mg/kg/d), and 40 patients on subcutaneous deferoxamine (20-50 mg/kg/d). Serum ferritin level, urinary iron excretion (UIE) and side effects were monitored over a two year period. RESULTS: Patients on L1 had an initial serum ferritin concentration of 3663+/-566 microg/l (mean+/-SEM), that dropped to 2599+/-314 at 6 months (p<0.02; paired t-test), and stabilised at that level over the 24 months follow up. Patients on deferoxamine had an initial mean serum ferritin concentration of 3480+/-417 (NS compared to the L1 group), which dropped gradually to 3143+/-417 (p<0.05) and 2819+/-292 (p<0.02) at 6 and 24 months, respectively. The most common adverse reactions associated with L1 were arthralgia and nausea, but they did not necessitate stopping the drug. CONCLUSION: L1 had comparable efficacy as deferoxamine with minimal side effects and better compliance. Provided long term side effects are not encountered, L1 seems to be a valuable alternative iron chelator for patients unable or unwilling to use deferoxamine effectively.

Administration, Oral↗
PubMed2001

Campylobacter fetus pericarditis in a patient with beta-thalassemia: case report and review of the literature.

A case of pericardial effusion due to Campylobacter fetus in a patient with thalassemia is presented. The patient failed to respond to ceftriaxone and clarithromycin despite in vitro susceptibility, but improved after pericardiectomy and ampicillin. Pericarditis due to C. fetus has rarely been reported. A high index of suspicion is essential to recognise this organism, because of its special microbiological characteristics.

Adolescent↗
PubMed2001

Extramedullary hematopoiesis in a patient with beta-thalassemia intermedia manifesting as symptomatic pleural effusion.

A case of bilateral paravertebral extramedullary hematopoietic masses with symptomatic pleural effusion in a patient with beta-thalassemia intermedia is reported. The pleural effusion was controlled by pleurodesis using tetracycline. We review the literature on this uncommon complication of beta-thalassemia intermedia and discuss the options for diagnosis and treatment.

Adult↗
PubMed2001

Infant mortality in Louisiana--identifying the risks.

The state of Louisiana consistently has one of the five highest infant mortality rates in the nation and the racial disparity within the state is glaring. The purpose of this paper is to analyze the state's linked birth and death infant data set for the birth cohort of 1990-1998, and to identify the "at risk" population. We have analyzed data from the 1990 to 1998 birth and infant death cohort in Louisiana to determine mothers' characteristics that are associated with infant death. These include extremely young and old ages, unmarried marital status, lower levels of education, and prenatal care. A mother's race is also associated with higher future mortality for her infant, with black mothers and their infants being at greater risk than white ones. Because the infant mortality rate is higher for black infants than for white ones, Louisiana's higher infant mortality rate can be seen as a result of the higher proportion of black births. Main concerns in reducing infant mortality rates include reducing incidence of low birth weights specifically among black births primarily by providing adequate prenatal care.

Adolescent↗
PubMed2000

Mechanical culture conditions effect gene expression: gravity-induced changes on the space shuttle.

Three-dimensional suspension culture is a gravity-limited phenomenon. The balancing forces necessary to keep the aggregates in suspension increase directly with aggregate size. This leads to a self-propagating cycle of cell damage by balancing forces. Cell culture in microgravity avoids this trade-off. We determined which genes mediate three-dimensional culture of cell and tissue aggregates in the low-shear stress, low-turbulent environment of actual microgravity. Primary cultures of human renal cortical cells were flown on the space shuttle. Cells grown in microgravity and ground-based controls were grown for 6 days and fixed. RNA was extracted, and automated gene array analysis of the expression of 10, 000 genes was performed. A select group of genes were regulated in microgravity. These 1,632 genes were independent of known shear stress response element-dependent genes and heat shock proteins. Specific transcription factors underwent large changes in microgravity including the Wilms' tumor zinc finger protein, and the vitamin D receptor. A specific group of genes, under the control of defined transcription factors, mediate three-dimensional suspension culture under microgravity conditions.

Animals↗
PubMed2000

Ticlopidine-induced aplastic anemia and quick recovery with G-CSF: case report and literature review.

We report here a case of ticlopidine-induced aplastic anemia that responded to G-CSF and review the literature. An 83-year-old woman was started on ticlopidine for coronary artery disease after an episode of upper gastrointestinal bleeding secondary to aspirin. She developed aplastic anemia seven weeks after initiation of ticlopidine. She was hospitalised and received empiric antibiotic therapy and granulocyte colony stimulating factor (G-CSF). Her bone marrow started to recover quickly, and white blood cell and platelet counts returned to normal within three weeks. A review of medical literature revealed 20 similar cases of ticlopidine-induced aplastic anemia resulting in death in seven cases. G-CSF has been used previously with variable success. Ticlopidine is associated with serious, sometimes fatal hematological side effects. This risk should be seriously taken into consideration when prescribing ticlopidine. G-CSF may be helpful in the treatment of ticlopidine-induced aplastic anemia.

Aged↗
PubMed2000

Beta-thalassaemia intermedia in Lebanon.

Approximately one third of thalassaemia patients on record in Lebanon have thalassaemia intermedia. We have analysed three factors in a panel of 73 patients with this less severe form of the disease in our population: mild beta-globin gene mutations, deletions in the alpha-globin gene and the presence of a polymorphism for the enzyme Xmn I in the Ggamma-promoter region. The results show that the most important contributing factor is the beta-genotype: 68% of patients have a mild beta+ mutation (IVSI-6, cd29, -88 or -87), while 26% of patients are positive for the Xmn I polymorphism associated with increased production of HbF, which showed strong linkage to particular mutations (IVSII-1, cd8 and cd30). However, the genotype phenotype correlation is difficult, because many patients were initially misdiagnosed as thalassaemia major and were started early on regular blood transfusions, which was stopped later on. This illustrates well the importance of an early accurate diagnosis of thalassaemia intermedia for appropriate clinical management.

Adolescent↗
PubMed2000

Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion.

We studied 36 patients (17 males and 19 females) with thalassemia major by performing pulmonary function testing (PFT), arterial blood gas analysis (ABG), as well as determining the serum ferritin level. In addition, 19 of these patients were transfused with two units of packed cells, and a repeat ABG and PFT were performed. Twenty-three patients had normal PFTs, eleven patients (30.6%) showed a restrictive pattern (significant decrease in both TLC and DLCO), and only two patients (5.6%) showed an obstructive pattern. A significant negative correlation was found between serum ferritin and restrictive parameters, DLCO and TLC (p = 0.01 and p = 0.03, respectively). This correlation was even stronger after transfusion. Controlling for age, ferritin was still negatively correlated with DLCO (p = 0.04), but no longer with TLC. There was no correlation between age and DLCO or age and TLC; however, there was a statistically significant negative correlation between age and FVC (p = 0.003). Analysis of patients who were transfused revealed a significant decrease in forced vital capacity (89 +/- 4% vs. 74 +/- 5% of predicted; p-value < 0.001) and in maximum midexpiratory flow rate (79 +/- 4% vs. 67 +/- 5% of predicted; p-value = 0.004). For patients older than 15 yr of age, there was a statistically significant decrease in FEV1/FVC (84 +/- 2 vs. 83 +/- 2%; p-value = 0.04). The ABGs showed no significant change post-transfusion. In this study, PFT findings in thalassemia major were restrictive and correlated with serum ferritin level. Transfusion had an acute effect on the obstructive parameters of PFT.

Adult↗
PubMed2000

Interferon alpha is an effective therapy for congenital dyserythropoietic anaemia type I.

The efficacy of interferon-alpha (IFN) was reported in three patients with congenital dyserythropoietic anaemia (CDA) type I. We describe two additional cases treated with IFN, which normalized the haemoglobin level in both patients with a dramatic decrease in the size of the spleen in one. Haemoglobin remained stable more than 6 months after discontinuation of treatment. IFN induced more than 50% decrease in the number of BFU-E in both patients' bone marrow cultures, suggesting an indirect effect of IFN on erythropoiesis in vivo. We conclude that a trial of IFN therapy should be considered in patients with CDA type I.

Adult↗
PubMed2000

A survey of patients with acquired haemophilia in a haemophilia centre over a 28-year period.

Data of patients with acquired inhibitors to clotting factors seen in a haemophilia centre from 1970-98 was collected. Twenty-four patients with anti-factor VIII antibodies and four with acquired von Willebrand disease case records were evaluable. Two-thirds of the patients were females and their age ranged from 2 to 92 years (median 69). There was no correlation between the inhibitor titres and the severity or the pattern of bleeding. Porcine factor VIII and activated prothrombin complex were both effective for acute bleeds. Prednisolone and cyclophosphamide proved valuable for suppression of the antibodies without adverse effects even in the elderly group of patients. Three deaths were directly related to bleeding. This review has shown the life-threatening nature of this acquired bleeding disorder, its variable clinical presentation and the importance of early referral to a specialist centre for appropriate therapy.

Adolescent↗
PubMed2000

Beta-thalassemia mutations and haplotype analysis in Lebanon.

The molecular basis of beta-thalassemia in Lebanon reflects the heterogeneity of the Lebanese population. Eighteen different mutations were identified among a total of 277 chromosomes. There is evidence of clustering of some mutations in particular geographic regions or among specific religious groups. Haplotype analysis, using seven restriction sites was performed on a total of 110 samples and 11 different haplotypes were identified. The five most common mutations were each found on two different haplotypes, and most linkages were as previously reported in other Mediterranean populations, with a few exceptions, also showing some clustering.

Genetic Linkage↗
PubMed2000

Relaxation effect of nitric oxide-donor on diabetic penile smooth muscle in vitro.

The primate model has been used for investigations on the physiology and pharmacology of erection. Recent in vitro investigations indicate that nitric oxide (NO)-donor act as the mediator of penile erection, but it is unclear whether NO-donor could enhance the relaxation effect of sildenafil on diabetic penile smooth muscle. To determine the relaxation effect of NO-donor on diabetic penile smooth muscle, we studied strips of corpus cavernosum tissue obtained from 15 diabetic cynomolgus monkey (Macaca fascicularis). Contraction was induced on isolated strips of corporal smooth muscle by norepinephrine; then relaxation was assessed with the administration of two agents: selective phosphodiesterase type 5 (PDE5) inhibitor (sildenafil citrate) and S-nitroso-N-acetylpenicillamine (SNAP), an NO-donor, and combination of both agents. Analysis of variance was used to compare the responses to the different agents under various treatments. It was concluded that NO-donor could not enhance the relaxation effect of sildenafil on corpus cavernosum of diabetic monkey.

3',5'-Cyclic-GMP Phosphodiesterases↗
PubMed1999

Compensatory changes in GroEL/Gp31 affinity as a mechanism for allele-specific genetic interaction.

Previous work has shown that the GroEL-GroES interaction is primarily mediated by the GroES mobile loop. In bacteriophage T4 infection, GroES is substituted by the gene 31-encoded cochaperonin, Gp31. Using a genetic selection scheme, we have identified a new set of mutations in gene 31 that affect interaction with GroEL; all mutations result in changes in the mobile loop of Gp31. Biochemical analyses reveal that the mobile loop mutations alter the affinity between Gp31 and GroEL, most likely by modulating the stability of the GroEL-bound hairpin conformation of the mobile loop. Surprisingly, mutations in groEL that display allele-specific interactions with mutations in gene 31 alter residues in the GroEL intermediate domain, distantly located from the mobile loop binding site. The observed patterns of genetic and biochemical interaction between GroES or Gp31 and GroEL point to a mechanism of genetic allele specificity based on compensatory changes in affinity of the protein-protein interaction. Mutations studied in this work indirectly alter affinity by modulating a folding transition in the Gp31 mobile loop or by modulating a hinged conformational change in GroEL.

Alleles↗
PubMed1999

Combination cisplatin-vinorelbine for relapsed and chemotherapy-pretreated metastatic breast cancer.

The purpose of this study was to evaluate the combination of cisplatin and vinorelbine (PVn) for relapsed and chemotherapy-pretreated metastatic breast cancer. Twenty-three patients with metastatic breast cancer and prior chemotherapy were entered in a phase II study between June 1993 and December 1994. Eleven patients were premenopausal and 12 were postmenopausal. Follow-up data up to June 1997 are presented. All patients received cisplatin at a dose of 90 mg/m2 divided over 3 days as 30 mg/m2 infused over 4 hours. Intravenous vinorelbine 25 mg/m2 was given on days 1 and 8 or 15 according to patients' blood counts. Cycles were given every 3 to 4 weeks. An overall response rate of 61% (16/23 patients) was observed. Complete remission was obtained in six patients (26%) and partial remission was obtained in nine patients (35%). The duration of response ranged from 3 to 9 months, with an average of 4 months. Stable disease was noted in 29.1% and progressive disease in 8.3%. Overall survival at 12 months was 50%, and at 36 months it was 8%. Five of 12 patients (42%) who had prior doxorubicin therapy responded well to cisplatin-vinorelbine. Of those 12, seven were refractory and progressive on a doxorubicin-containing regimen, one had complete remission, and four had partial remission. Hematologic toxicity was acceptable. Treatment was delayed because of neutropenia in nine cycles (9.2%) and grade 2 leukopenia occurred in 54% of cycles. Febrile neutropenia occurred in seven cycles (7.1%), and five cycles were complicated by documented sepsis (5.1%). No treatment-related mortality occurred. Thrombocytopenia (grade 3) was seen in 27% of cycles, with no patient having a platelet count below 50,000 or bleeding episodes. Other toxicities were not major or dose-limiting. In conclusion, the combination of cisplatin and vinorelbine produced good responses: 61% response rate (16 of 23 patients) in relapsed, refractory, and heavily pretreated metastatic breast cancer, with 50% survival at 1 year, 12% at 2 years, and 8% at 3 years. In addition, a response rate of 42% (5 of 12 patients) was seen in patients resistant to anthracyclines.

Adult↗