Search PubMed⌕ Search

Biomedical subjects

Hussain Isma'eel

Publications and source records attributed to Hussain Isma'eel.

8 recordsLinked to original sources
PubMed2006

Thalassemia intermedia: revisited.

Thalassemia intermedia encompasses a wide clinical spectrum of beta-thalassemia phenotypes. Some thalassemia intermedia patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with thalassemia intermedia are rarely seen in thalassemia major, including extramedullary hematopoiesis, leg ulcers, gallstones and thrombophilia. Prevention of these complications, possibly with blood transfusion therapy, is ideal since they may be difficult to manage. Currently, many patients with thalassemia intermedia receive only occasional or no transfusions, since they are able to maintain hemoglobin levels between 7-9 g/dl; the risk of iron overload, necessitating adequate chelation therapy, is also a contributing factor. At present, there are no clear guidelines for initiating and maintaining transfusions in thalassemia intermedia for the prevention or treatment of complications. Here, we review the major clinical complications in thalassemia intermedia and suggest some therapeutic strategies based on retrospective clinical observations.

Blood Transfusion↗
PubMed2006

Screening for inherited thrombophilia might be warranted among Eastern Mediterranean sickle-beta-0 thalassemia patients.

BACKGROUND: The role of genetic thrombophilia in the development of both micro and macro vascular complications in patients with hemoglobinopathies (Sickle cell disease and thalassemia) have been investigated with some studies negating its role while others suggesting it. Lebanon is known to harbor sickle cell disease, thalassemia and sickle beta-thalassemia hemoglobinopathy patients along with a documented high prevalence of genetic thrombophilia mutations. METHODS: Twelve sickle beta-0-thalassemia patients with no pervious history of thrombotic events were selected. These patients underwent a physical examination with history, echo Doppler, along with blood withdrawal for complete blood count and PCR analysis of a sample of DNA for Factor V Leiden G1691A, Factor II G20210A, and MTHFR C677T. Results were compared to a historical control of 50 Lebanese controls and 50 LebaneseThalassemia Intermedia (TI) patients. RESULTS: The results showed that 42%, 59%, and 8% of patients carried heterozygous Factor V Leiden, abnormal (homozygous & heterozygous) MTHFR, and heterozygous Factor II mutations respectively. The sickle-thalassemia patients were 5.24 and 4.39 times more likely to have Factor V Leiden as compared to the normal controls and TI patients respectively (p < 0.05). DISCUSSION: The increased prevalence of more than one prothrombotic genetic mutation among the group indicates a probable clustering phenomenon, unknown to us to which the high consanguinity rate (77%) may have contributed. The role of the specific MTHFR and Factor V Leiden double heterozygous combination in incidence, recurrence, and guidance of duration of therapy in VTE is not well defined in the literature despite the recognized higher risk of thrombosis among this patient population. Our findings suggest that genetic thrombophilia workup is necessary in patients with sickle-beta zero thalassemia presenting with thrombotic events and studies that include a larger number of patients are necessary in order to define specific guidelines.

Adolescent↗
PubMed2006

A review of the off-label use of recombinant activated factor VII in a developing country tertiary care center.

Recombinant activated factor VII (rFVIIa) was first approved for treatment of congenital hemophilia. It could, however, also have a role in management of patients without pre-existing coagulopathies who undergo surgical procedures, have life-threatening hemorrhages, or sustain traumas associated with major blood loss. A retrospective chart review was performed for all cases given rFVIIa at American University of Beirut Medical Center (AUB MC). Patients with a previous medical history of thrombophilia were excluded. There were four pediatric patients with a mean age younger than 1 year. Adult patients' mean age was 64.5 +/- 17.4 years. The most common off-label uses for rFVIIa are control of hemorrhage during the repair of aortic dissection (4/17 cases) or following intracerebral hemorrhage (4/17 case). One trauma patient received the medication. Complications included cerebral ischemia in one patient. Three of the patients died but their death was not related to the bleeding or the medication. Based on the prognostic score proposed by Biss and Hanley, seven patients were low risk, four intermediate risk, and six high risk. Although off-label use of rFVIIa at AUB MC was supported by published reports, and associated with few complications, guidelines are required to control use of this medication.

Academic Medical Centers↗
PubMed2006

Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran.

Beta-thalassaemia is a congenital haemolytic anaemia characterized by partial (intermedia, TI) or complete (major, TM) deficiency in the production of beta-globin chains. The primary aim of this study was to determine the prevalence of thromboembolic events in patients with beta-thalassaemia. To achieve this, a multiple choice questionnaire was sent to 56 tertiary referral centres in eight countries (Lebanon, Italy, Israel, Greece, Egypt, Jordan, Saudi Arabia and Iran), requesting specific information on patients who had experienced a thromboembolic event. The study demonstrated that thromboembolic events occurred in a clinically relevant proportion (1.65%) of 8,860 thalassaemia patients (TI - 24.7% or TM - 75.3%) from the Mediterranean and Iran. Thromboembolism occurred 4.38 times more frequently in TI than TM (p < 0.001), with more venous events occurring in TI and more arterial events occurring in TM. Thrombosis in thalassaemia was also more common in females, splenectomized patients and those with profound anaemia (haemoglobin <9 g/dl). Due to the increased risk of thromboembolic events, the rationale for splenectomy should perhaps be re-assessed and the role of transfusion therapy for the prophylaxis of thrombosis, among other complications, be evaluated prospectively.

Adult↗