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A al-Momen

Publications and source records attributed to A al-Momen.

7 recordsLinked to original sources

Effect of recombinant human erythropoietin on lymphocyte phenotyping and phagocyte activity in hemodialysis patients.

The effect of recombinant human erythropoietin (rHmEPO) on lymphocytic phenotyping as well as on the phagocyte activity of polymorphonuclear cells and monocytes was evaluated in 16 patients on maintenance hemodialysis. The mean age of the patients was 38.2 +/- 16.2 years. There were seven men and nine women. All patients were started on 50 U/kg of rHmEPO intravenously three times per week, and the dosage was increased gradually to achieve target haemoglobin of 12 g/dL. Predialysis blood samples were taken monthly for 3 months, and phagocyte respiratory burst as well as lymphocyte subsets were studied. Healthy blood donors were taken as controls. By 3 months of rHmEPO treatment, there was no significant increase in total T and B cells, but there was a significant increase in both CD4 (P < 0.001) and CD8 (P < 0.005): however, there was no significant change in the CD4/CD8 ratio. There was significant reduction in the natural killer cells (P < 0.005). The phagocyte activity studies showed a significant increase in the respiratory burst in whole blood (P < 0.001) and opsonized zymosan (P < 0.001) as well as improvement in the suppressed polymorphonuclear cell and monocyte activity by uremia. Phagocytosis studied by yeast uptake showed significant improvement from the pretreatment suppressed phagocytes to normal activity posttreatment. In conclusion, treatment with rHmEPO increases CD4 and CD8 cell counts without affecting the CD4/CD8 ratio, decreases the natural killer cells, and improves the impaired phagocyte activity in hemodialysis patients.

Adult↗

On the use of hydroxyurea/erythropoietin combination therapy for sickle cell disease.

Seven sickle cell disease (SCD) patients [sickle cell anaemia = 4 (males 2, females 2, age range 18-40 years), and sickle cell beta (0)-thalassaemia = 3 (all females, age range 20-47 years)], suffering from a severe form of the disease were enrolled in a treatment protocol using hydroxyurea (HU) for up to 12 months followed by a combination therapy with HU and human recombinant erythropoietin (rHuEpo; using 400 U/kg/week i.v.) for 3-4 weeks. Following the withdrawal of rHuEpo the patients were maintained on HU alone. The patients were characterised on the basis of the 'severity index' prior to the initiation of the therapy. Haematological and relevant biochemical parameters, Hb A2 fetal haemoglobin (HbF), HbF cells, reticulocytes and platelet counts were estimated at least at three occasions to determine the mean and range of the parameters. During the treatment period the patients were followed every 2-4 weeks where the haematological and biochemical parameters were assessed. The results were separately analysed and mean +/- SD were obtained for each parameter at the end of each protocol. The statistical significance of the difference in the results obtained on treatment and the baseline results was examined using the paired t test. No toxic side effects of HU and rHuEpo (as judged from reduction in platelet and white blood cell count) were documented during and after the whole period of treatment. The patients showed a significant clinical improvement. Total haemoglobin, haematocrit, red cell count, HbF and HbF cells increased, while white blood cells, reticulocyte counts and bilirubin level decreased. Platelet count decreased but remained within the normal range. The results revealed that 5 of the patients on HU treatment showed a significant increase in the HbF level and HbF cells, while 2 patients (1 sickle cell anaemia and 1 Hb S/beta(0)-thalassaemia patient) did not and were considered as 'non-responders'. The rHuEpo and HU combination therapy elevated the HbF level, with a varying degree, in all patients except 2, who had already reached a high HbF level and showed a decrease in HbF during the rHuEpo protocol. Variable individual response to both HU and rHuEpo therapy was a common feature. We recommend the use of HU for the treatment of SCD and a combination therapy using HU and rHuEpo for the non-responders.

Adolescent↗

The pharmacological manipulation of fetal haemoglobin: trials using hydroxyurea and recombinant human erythropoietin.

Hydroxyurea (HU) and recombinant human erythropoietin (rHuEpo) have been used in several studies to elevate Hb F level in sickle cell disease (SCD) patients and hence to ameliorate the clinical presentations of the disease. The treatment protocol and doses have varied in the different studies. We studied the effects of HU+rHuEpo combination therapy in sickle cell anaemia (SCA patients) to investigate the Hb F manipulation and hence treatment of SCA. Six patients with severe SCA were selected for treatment with HU (20-25 mg/kg body weight) and rHuEpo (400-800 U/kg body weight) combination therapy for 4 weeks followed by HU (20-25 mg/kg body weight) maintenance therapy for 6 months to 1 year. Iron and folic acid were administered during HU+rHuEpo treatment. Signs, symptoms and complications were recorded to obtain the severity index. Only patients with a severity index > or = 6 were included in the study. Haematological and biochemical parameter values, Hb A2, Hb F, Hb F distribution, Hb F cells, bilirubin level and reticulocyte count were assessed at least on 2-3 occasions prior to initiation of the therapy protocol to establish baseline values. During the treatment period, the clinical presentations were monitored and the estimation of the laboratory parameters was carried out every 4-8 weeks. The results of these parameters during HU and rHuEpo combination therapy and HU maintenance therapy were compared with baseline values using paired t test. The elevation in the level of Hb F, Hb F cells, total haemoglobin, red cell count and MCV were significant (p < 0.005), while reticulocyte count and total bilirubin were significantly decreased (p < 0.05). Each patient showed an individual pattern of Hb F elevation. The increase in Hb F level was correlated with the haematological and biochemical parameters using the General Linear Model Programme of Statistical Analysis System. In general, the clinical presentation improved as Hb F level increased in each patient. In addition, the positive correlation with the haematological parameters and negative correlation with reticulocytes and total bilirubin confirmed the beneficial effect of elevated Hb F level on reducing red cell haemolysis. No correlation could be demonstrated between the pretreatment Hb F level and the increase in Hb F during the treatment period. Daily doses of HU with a single intravenous rHuEpo and iron supplementation elevate Hb F and Hb F cells in SCA patients. The Hb F level can be maintained high on HU therapy alone.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗

Does G gamma/A gamma ratio and Hb F level influence the severity of sickle cell anaemia.

Sickle cell anaemia (SCA) exhibits significant variations in clinical presentation in different populations for which several genetic factors including SCA-associated alpha- and beta-thalassaemias, G-6-PD deficiency and elevated Hb F level have been implicated as possible ameliorating factors. Saudi Arabia is unique in that mild and severe forms of the disease occur at a high frequency. We investigated the G gamma/A gamma ratio and Hb F level and correlated these values with the severity of SCA. The results showed that Hb F level varies significantly in both groups of patients with no evident correlation with the mild clinical manifestations. However, G gamma/A gamma ratio correlated significantly with the disease severity where a high ratio was observed in patients with the mild and a low ratio in patients with the severe disease. The results are evaluated and discussed in the light of correlation studies and regression analysis.

Anemia, Sickle Cell↗

Hydroxyurea for the treatment of sickle cell disease.

In this study 21 adults with severe form of sickle cell disease (SCD; sickle cell anaemia, n = 15; Hb S/beta degree-thal, n = 6) were treated with hydroxyurea (HU) to assess the effectiveness of the drug in managing SCD. The individual dose was selected for each patient. The dose selection was based on the HU clearance study. Thereafter, the patients received daily doses of 15-20 mg/kg body weight. An evaluation data form was filled out at the monthly visit. The severity index (SI) of the disease was determined and haematological parameters including red cell indices, platelet counts, reticulocyte counts, irreversibly sickled cells, red cell deformability, Hb F, Hb F cells, total and direct bilirubin levels were measured prior to treatment, at follow-up intervals during treatment and after cessation of treatment. The trial period lasted 3 months. Statistically significant improvement was observed in the clinical presentation, haematological and biochemical parameters. Hb F level and F cells showed a significant increase in most patients, but to a variable degree. A major resultant effect was an increase in mean cell volume. Our experience shows that HU can be used for the treatment of severe forms of SCD with no major side effects, provided that the doses are monitored and that laboratory investigations are regularly undertaken.

Administration, Oral↗