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Biomedical subjects

Ali Taher

Publications and source records attributed to Ali Taher.

At least 37 records · Page 2Linked to original sources

Moyamoya syndrome with intraventricular hemorrhage in an adult with factor V Leiden mutation.

OBJECTIVE: To report a case of proximal occlusion of 2 major cerebral vessels associated with moyamoya network circulation that manifested by spontaneous intraventricular hemorrhage. DESIGN: Case report. PATIENT AND RESULTS: A 36-year-old Syrian man presented with symptoms of sudden-onset headache, neck stiffness, and confusion. The computed tomography scan of his brain showed intraventricular bleeding, and the subsequent 4 vessel angiographies revealed occlusion of the left middle and anterior cerebral arteries with moyamoya appearance in the terminal branches. The coagulation profile showed the presence of heterozygous factor V Leiden mutation. The patient was treated conservatively until resolution of his blood clot, and later he was started on oral anticoagulation. CONCLUSION: Factor V Leiden mutation may cause large cerebral vessel occlusion with moyamoya syndrome in adults.

Adult↗

The role of therapeutic plasma exchange in the catastrophic antiphospholipid syndrome.

The catastrophic antiphospholipid syndrome (CAPS) is a life-threatening sub-entity of the antiphospholipid syndrome (APS) resulting in multiorgan failure and a mortality rate that may reach 50%. The optimal treatment regimen for CAPS is unknown. To evaluate the benefit of therapeutic plasma exchange (TPE) therapy in CAPS, we undertook a computer-assisted (MEDLINE, National Library of Medicine, Bethesda, MD) search of the literature to locate all cases of CAPS (case reports and reviews) treated with TPE. In view of the published literature so far, TPE has shown reasonable therapeutic benefits and improved survival for patients with CAPS. The use of this technique, along with anticoagulation and if needed pharmacological immunosuppression, should be considered for the acute management of patients who present with this life-threatening condition.

Antiphospholipid Syndrome↗

A clinical phase II study of cisplatinum and vinorelbine (PVn) in advanced breast carcinoma (ABC).

OBJECTIVES: The effectiveness of cisplatinum and vinorelbine (PVn) as a salvage regimen in patients with metastatic breast cancer was reported in previous studies. This report is a pilot study assessing the antitumor efficacy and safety of this regimen as first line therapy for advanced breast cancer patients. METHODS: Thirty-five patients were enrolled: 22 with metastatic breast carcinoma and 13 with locally advanced breast carcinoma (stage III). A total of 4 cycles of PVn were planned. After the 4th cycle, patients with metastatic breast cancer received vinorelbine biweekly until disease progression or for a total of 12 cycles, whereas those with locally advanced breast cancer who showed complete or partial response underwent curative surgery. RESULTS: The overall response rate of our whole population was 74.29%. For the metastatic breast cancer group, the overall response rate was 64%, with a median survival of 19 months (range 2-36). For the locally advanced breast cancer group, the overall response rate was 92.3% with a median time to disease progression of 26 months (range 25-27). Toxicity was acceptable, and no treatment-related mortality was encountered. CONCLUSIONS: PVn is effective as first line treatment of advanced breast cancer with overall response rate of 64% in metastatic breast cancer and 92.3% in locally advanced breast cancer, and acceptable toxicity.

Adenocarcinoma↗

Efficacy and tolerability of peginterferon alpha-2a with or without ribavirin in thalassaemia major patients with chronic hepatitis C virus infection.

Thalassaemia patients with genotype 1 or 4 chronic hepatitis C virus (HCV) infection were randomised to receive peginterferon alpha-2a 180 mg/week ribavirin for 48 weeks. Primary efficacy variable was sustained viral response (SVR) at 72 weeks. Thirty-two patients were evaluated; 20 enrolled. Baseline characteristics were comparable. SVR occurred in four of 12 and five of eight patients in the monotherapy and combination groups (30% and 62.5%; P=0.19), respectively. Undetectable RNA at 12 weeks and age <18 years were associated with improved SVR (P<0.05). Transfusion requirements rose by 34% in the combination arm (P=0.08). Peginterferon/ribavirin was effective in thalassaemics with HCV and moderate iron overload.

Adolescent↗

Safety and effectiveness of 100 mg/kg/day deferiprone in patients with thalassemia major: a two-year study.

Deferiprone at a dose of 75 mg/kg/day is not sufficiently effective to maintain iron stores at a level which has been considered safe in all patients with iron overload. Our main aim was to determine the safety of long-term therapy with high-dose (100 mg/kg/day) deferiprone. A secondary aim was to determine the efficacy of this high dose. Twelve thalassemia major patients received deferiprone at a dose of 100 mg/kg/day over 2 years. Transient aspartate aminotransferase increase (8 patients), gastrointestinal discomfort (3 patients) and arthralgia (2 patients) were the most commonly reported side effects. None of the patients discontinued therapy. The mean serum ferritin level fell from 3,901 +/- 3,618 to 1,790 +/- 2,205 microg/l after 2 years (p < 0.05). Five of the 12 patients continued to receive deferiprone for an additional 3 years. No new side effects were encountered. The mean serum ferritin level in this subgroup was initially 2,510 +/- 332 microg/l and dropped to 1,511 +/- 664 microg/l after 5 years (p < 0.05). Liver iron levels at the end of the 2-year study ranged from 1.0 to 30.9 mg/g dry weight, 3 of the patients having levels above 15 mg/g.

Adolescent↗

Lupus anticoagulant, factor V Leiden, and methylenetetrahydrofolate reductase gene mutation in a lupus patient with cerebral venous thrombosis.

We describe the case of a young Lebanese woman with systemic lupus erythematosus (SLE) and a positive lupus anticoagulant (LAC) who developed right internal jugular vein and sigmoid sinus thrombosis. Coagulation studies showed that in addition to the LAC the patient was heterozygous for the factor V (FV) Leiden mutation, and C677T mutation of the methylenetetrahydrofolate reductase gene. The high prevalence of FV Leiden in the eastern Mediterranean region suggests that we should probably screen our SLE patients in this area, especially those with anticardiolipin antibodies and/or LAC who have no history of thrombosis, for this and other thrombophilia markers. The detection of such abnormalities may have major practical consequences for the long-term management of these patients to prevent further thrombotic episodes.

Adolescent↗

Hydroxyurea-induced hemolytic anemia in a patient with essential thrombocythemia.

Hydroxyurea (HU) has been used in patients with essential thrombocythemia (ET) to reduce the frequency and severity of thrombotic complications of the disease. It acts by causing bone marrow depression, resulting in a decrease in platelet and leukocyte counts and in anemia with megaloblastosis. Herein we report a case of an 80-year-old man with ET who developed severe hemolytic anemia requiring multiple packed RBC transfusions while being treated with HU. The hemolysis persisted until discontinuation of the drug. This case suggests hemolysis as a potential side effect of HU and raises the clinical concern that not all HU-induced anemia is secondary to erythropoiesis depression. With the increasing use of HU in the management of ET, hemoglobinopathy, and other diseases, the importance of monitoring for hemolysis must be further emphasized in these patients.

Aged↗

Thrombotic thrombocytopenic purpura and pregnancy: report of four cases and literature review.

Thrombotic thrombocytopenic purpura (TTP) is a severe life-threatening hematological disorder affecting the microcirculation of multiple organ systems. Infection, pregnancy, cancer, drugs, and surgery are frequently associated with the initial episodes and relapses. Women who are either pregnant or in the postpartum period make up 10-25% of TTP patients, suggesting the interrelationship between TTP and pregnancy. The introduction of aggressive treatment with plasma transfusion and plasmapheresis has improved maternal and fetal survival rates. We report four cases of pregnancy-related TTP, describing the clinical course of patients, including response to therapy and pregnancy outcomes. Three out of four (75%) patients were treated by daily single session of plasmapheresis for a period ranged between 3 and 23 days. One patient had complete response to treatment with no sequelae, the second patient had resistant disease and died due to multiorgan failure, while the third patient had complete response after 2 episodes of TTP, which was complicated by intrauterine fetal growth retardation and death. Review of the previously reported cases of pregnancy-related TTP and the current treatment options for this rare condition are discussed also.

Adult↗

Thrombotic thrombocytopenic purpura: 24 years of experience at the American University of Beirut Medical Center.

Thrombotic thrombocytopenic purpura (TTP) is a hematological syndrome defined by the presence of thrombocytopenia and microangiopathic hemolytic anemia without a clinically apparent etiology. Patients may also suffer from fever in addition to neurological and renal impairment. Treatment should be initiated as soon as possible, otherwise this rare disease can be fatal. The main treatment options include therapeutic plasma exchange, fresh frozen plasma infusion, and adjuvant agents such as steroids and antiplatelet drugs. A search of patient records was carried out at the American University of Beirut Medical Center looking for patients who developed TTP over a 24-year period extending from 1980 to 2003. Relevant information was collected and analyzed. A total of 47 records were found. All presented with anemia and thrombocytopenia, 83% had neurological symptoms, 61.7% had fever and 34% had renal impairment. All patients were treated with a multimodality regimen including therapeutic plasma exchange, FFP infusion, steroids, antiplatelet agents, vincristine and others. 38 (81%) cases achieved complete remission. Out of these, 12 (31.6%) relapsed and responded to treatment. Patients who did not receive plasma exchange were more likely to relapse (P = 0.032). A second relapse was observed in 6 cases. The overall mortality rate from TTP over 24 years was 21.3%. TTP remains a fatal disease. A high index of suspicion should, therefore, always be present. Treatment options should be further developed and patients should directly be referred to tertiary care centers.

Adolescent↗

Pregnancy outcome in women with prosthetic heart valves.

OBJECTIVE: This study was undertaken to evaluate the risks and pregnancy outcome in women with prosthetic heart valves on different anticoagulent regimens. STUDY DESIGN: A retrospective chart review of 82 pregnancies in 33 women with mechanical valve prostheses at a tertiary referral center from 1987 to 2002. The main outcome measures were major maternal complications and perinatal outcome. RESULTS: The valve replaced was mitral (60.6%), aortic (18.2%), and both (21.2%). Fifty-four pregnancies (65.9%) resulted in live births, 9 (11.0%) had stillbirths (all on warfarin), and 12 (14.6%) had spontaneous and 7 (8.5%) therapeutic abortions (all on warfarin). The rate of spontaneous abortion was highest in women on warfarin throughout pregnancy (P < .01). The live birth rate was higher in women on heparin compared with those on warfarin (P < .01), and in those on heparin/warfarin compared with warfarin alone (P < .01). There were no maternal deaths; however, 3 patients had mitral valve thrombosis (2 on heparin and 1 on warfarin) necessitating surgery in 1 patient and medical thrombolysis in 2 patients. Hemorrhagic complications occurred in 5 patients, 4 of whom required transfusion. CONCLUSION: No single anticoagulant regimen confers complete protection from thromboembolic phenomena in pregnancy. Despite a high maternal morbidity rate, the perinatal outcome is acceptable when pregnancy progresses beyond the first trimester.

Abortion, Spontaneous↗

Cisplatin plus vinorelbine (PVn) as a salvage regimen for refractory breast cancer.

Breast cancer is regarded as a systemic disease even when tumors are completely resected. In patients with advanced breast cancer the overall prognosis is poor, but the disease is not uniformly fatal. Vinorelbine has proved to be effective when given as a single agent in this setting. Since it has a moderate to severe myelotoxic effect, a combination of vinorelbine with cisplatin, which is a weak myelotoxic drug, is ideal for the treatment of patients with advanced disease. In this paper, we report on a patient with advanced breast cancer who attained complete response of 14 months duration to a cisplatin/vinorelbine combination after progression during treatment with paclitaxel and doxorubicin.

Antineoplastic Combined Chemotherapy Protocols↗

Improvement of cardiac function in thalassemia major treated with L-carnitine.

INTRODUCTION: Heart disease secondary to chronic anemia and hemosiderosis remains the major cause of morbidity and mortality in thalassemic patients. Chronic anemia and the tissue hypoxia it induces impair free fatty acid oxidation and ATP production in myocardial cells. The use of L-carnitine, a butyric acid derivative, may help overcome some of these defects. OBJECTIVE: To investigate the effect of L-carnitine therapy on cardiac function in thalassemia major patients. MATERIALS AND METHODS: Cardiac function was evaluated in 30 patients attending our clinic. The mean (+/-SD) age was 15.87 +/- 3.19 years. The studies we performed included echocardiography, Doppler and multigated equilibrium radionuclide angiography (MUGA). Systolic and diastolic function was evaluated before starting L-carnitine treatment and after 6 months of oral L-carnitine (50 mg/kg/day). RESULTS: Echocardiography studies revealed no significant changes in systolic and diastolic function after L-carnitine therapy (p > 0.05). Analysis of the data taken by MUGA performed in 20 of the patients, however, showed a significant improvement of diastolic function after 6 months of L-carnitine therapy. The mean peak filling rate (end-diastolic volume/s) increased from 3.15 +/- 1.06 to 3.61 +/- 1.68 (p < 0.03). The time to peak (during filling) decreased significantly from 143.45 +/- 42.04 to 117.70 +/- 24.40 s (p < 0.02). Systolic function showed a significant increase in the left ventricular ejection fraction from 58.25 +/- 9.92 to 63.95 +/- 10.11% (p = 0.0001). CONCLUSION: L-carnitine may be an effective drug for improving the cardiac status of thalassemic patients. MUGA is the most accurate technique of those used here for assessing left ventricular function in these patients.

Adolescent↗

Successful treatment of B-cell prolymphocytic leukemia with monoclonal anti-CD20 antibody.

We report a patient with B-cell prolymphocytic leukemia (PLL) who was treated successfully with the monoclonal anti-CD20 antibody (rituximab). The patient had recurrent infections due to relative neutropenia, secondary to bone marrow infiltration. After treatment with monoclonal anti-CD20 antibodies (rituximab) 375 mg/m(2) weekly for 4 weeks, complete remission was obtained. It was documented by normalization of peripheral blood counts, disappearance of organomegaly, and by molecular cytogenetics-fluorescent in situ hybridization (FISH) on bone marrow cells. She remains in complete remission 8 months following the discontinuation of treatment. This is the second reported case of successful treatment of B-cell PLL with rituximab.

Antigens, CD20↗

Hypertransfusion: a successful method of treatment in thalassemia intermedia patients with spinal cord compression secondary to extramedullary hematopoiesis.

SUMMARY OF BACKGROUND DATA: Extramedullary hematopoiesis is a common compensatory phenomenon to chronic hemolytic anemias including thalassemia. Several sites can be involved including the liver, the spleen, the lymph nodes, and other less common locations. Spinal cord compression may result in the rare cases wherein the hematopoietic develops intraspinally. Treatment of such conditions still is controversial. OBJECTIVE: This article reviews the literature and reports two cases of thalassemia intermedia involving patients who presented with neurologic symptoms after acute spinal cord compression secondary to extramedullary hematopoiesis. METHODS: The diagnosis was established by magnetic resonance imaging. Hypertransfusion therapy was used as our first-line treatment method. RESULTS: Complete neurologic recovery was achieved. Improvement in the neurologic status started as soon as the first week of treatment. CONCLUSIONS: Clinical awareness is important for early diagnosis and prevention of irreversible neurologic complications in such cases. Magnetic resonance imaging is the radiologic method of choice for diagnosing extramedullary hematopoietic masses and for delineating the extent of spinal cord involvement. Hypertransfusion seems to be a promising treatment method that should be recommended as a first-line approach or as an adjuvant therapy to other methods.

Adult↗