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Examines hepatitis-C-virus (HCV) genotypes and hepatitis-G-virus infection in thalassemia patients in Lebanon. Seroprevalence of antibody in hepatitis G virus; Causes of HCV infection; Illustration of the value of the routine screening of blood donations.

The article discusses the case of a female patient with generalized status epilepticus possibly induced by the fluoroquinolone antibiotic, gatifloxacin. Gatifloxacin was used to treat the patient for non-febrile urinary tract infection. Tonic-clonic seizures occurred on the fourth day of treatment. Treatment with gatifloxacin stopped and she was given diazepam.

Abstract: 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 β-globin gene mutations, deletions in the α-globin gene and the presence of a polymorphism for the enzyme Xmn I in the G γ-promoter region. The results show that the most important contributing factor is the β-genotype: 68% of patients have a mild β+ 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. [ABSTRACT FROM AUTHOR]

Multiple sclerosis (MS), a chronic neurological disorder, causes demyelination of spinal cord and brain cells resulting in progressive disability. Cladribine Tablets is an effective and budget saving treatment option for the treatment of HDA-RMS patients in Lebanon from the NSSF perspective. [Extracted from the article]

With evolving diagnostic criteria and the advent of new oral and parenteral therapies for MS, most current diagnostic and treatment algorithms need re-evaluation and updating. The diagnosis of MS relies on incorporating clinical and paraclinical findings to prove dissemination in space and in time, and exclude alternative diseases that can explain the findings at hand. The differential diagnostic workup should be guided by clinical and laboratory red flags to avoid unnecessary tests. Appropriate multiple sclerosis (MS) therapy selection is critical to maximize patient benefit. The current guidelines review the scientific evidence supporting treatment of acute relapses, radiologically isolated syndrome, clinically isolated syndrome, relapsing remitting MS, secondary progressive MS, and primary progressive MS. The purpose of these guidelines is to provide practical recommendations and algorithms for the diagnosis and treatment of MS based on current scientific evidence and clinical experience. [ABSTRACT FROM AUTHOR]

Background Leg ulcers in β-thalassaemia intermedia (TI) patients are a relatively common occurrence that have an 8% prevalence. Both the pathophysiology and treatment of this condition have not been well-elucidated. This is mainly because of the rarity of the disease and the lack of well-structured studies. The goal of this study was to better explore the risk factors for the development of this condition along with the treatment options available. Methods We present 11 such cases that have occurred in 6 β-TI patients over the course of 19 years who are followed up at the Chronic Care Center of Lebanon. Results Our patient population comprised three men and three women aged between 25 and 58, most of whom had iron overload and with an average lifetime haemoglobin ranging between 49 g/L and 77 g/L. Most of the patients were treated with blood transfusions with varying degrees of success. Nonetheless, some received Hydroxyurea, granulocyte macrophage colony-stimulating factor ( GM- CSF) or topical antibiotics. Conclusion Our results show that chelation therapy, hydroxyurea use and blood transfusions are beneficial in the treatment of this condition. Whether foetal haemoglobin is directly related to the development of the ulcers is not clear based on our results. Larger studies are needed to better explore the risk factors that predispose patients to this condition. [ABSTRACT FROM AUTHOR]

β-Thalassemia major is a debilitating disease with a considerable incidence in Lebanon (around 2–3% carriership). The present article describes our experience to this day with 214 patients, emphasizing the survival of β-thalassemia major and development of complications among patients with different parameters. Fifteen deaths were reported. The most common cause of death was heart failure (60%). Patients with a ferritin level of 3,000 ng/ml showed better survival than those with a level >3,000 ng/ml (p < 0.006). In addition, patients with a ferritin level of 1,500 ng/ml showed less complication-free survival than those with a level >1,500 ng/ml (p < 0.024). High level of ferritin (1,500 ng/ml) is associated with increased risk of heart failure. Overall and complication-free survival were statistically different among patients classified according to birth cohort or ferritin level. The Chronic Care Center, a multidisciplinary center located in the suburbs of Beirut, led to an increase in complication-free as well as overall survival. Although patients are being diagnosed earlier and chelation therapy is being initiated at an earlier age, complications due to iron overload still persist. The introduction of new oral iron chelators and better iron overload quantitation methods will most likely modify this picture, and a follow-up study will examine their impact. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

β-Thalassemia is a hereditary anemia that is quite prevalent in Lebanon. Most patients with β-Thalassemia are treated and followed up mostly at a multidisciplinary center, located in the suburban area of Beirut: the Chronic Care Center (CCC), operational since 1994. We will review the experience with β-Thalassemia accumulated through this institution. Four hundred and twenty five patients, aged 2 to 68 years are followed up at the CCC. Sixty four percent have thalassemia major (TM) while 36% have thalassemia intermedia (TI). Lebanese patients with TM receive periodic packed red cell transfusions to maintain a pre-transfusional hemoglobin level of 10 gm/dl at all times and desferrioxamine is the standard iron chelator in use. Since 1994, 12 patients with TM have died from complications of their disease, with heart failure being responsible for the majority of deaths. The incidence of cardiac, endocrinologic, and infectious complications will be reviewed. Finally, both current and prospective preventive measures will be discussed, specifically educational campaigns and premarital screening. The effects of prevention are starting to show as the number of newly diagnosed disease is diminishing. [ABSTRACT FROM AUTHOR]

Twelve thalassaemia major patients have been given deferiprone 75 mg/kg body weight daily as iron chelation therapy for 5 years. Their ages ranged from 18 to 34 years (mean 24.2) at the end of the study. Two patients were hepatitis C virus (HCV) mRNA positive and a further 5 were positive for HCV antibody. The mean serum ferritin level fell significantly from 4,302 ± 2,245 μg/l SD at baseline to 3,032 ± 1,155 μg/l at 2 years (p = 0.037) and 2,229 ± 1,070 μg/l (p = 0.007) at 5 years. At the end of the study, liver iron ranged from 3.59 to 23.7 mg/g dry weight (mean 11.9 ± 5.4), 3 patients having levels >15 mg/g. There was no significant change in serum AST levels, but ALT levels fell significantly at 2 years (p = 0.019) and 5 years (p = 0.001). Liver biopsy at the end of the study showed no evidence of hepatic fibrosis caused by deferiprone. Cardiac studies showed no overall change in left ventricular ejection fraction but a significant improvement in isovolumic relaxation time (p = 0.045). We conclude that in this albeit small group of thalassaemia major patients, deferiprone was a safe long-term method of iron chelation. In a minority, higher doses of deferiprone or a combination with desferrioxamine would be needed to lower liver iron below 15 mg/g. Copyright © 2004 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Abstract: Sickle cell disease (SCD) is an inherited autosomal recessive disorder of the β -globin chain. Despite the fact that all subjects with SCD have the same single base pair mutation, the severity of the clinical and hematological manifestations is extremely variable. This study examined for the first time in Lebanon the correlation between the clinical manifestation of SCD and the β -globin gene haplotypes. The haplotypes of 50 patients diagnosed with SCD were determined using polymerase chain reaction amplification of fragments containing nine polymorphic restriction sites around and within the ε –Gγ –Aγ –ψ β –δ –β -globin gene complex. Most reported haplotypes were found in our population with the Benin haplotype as the most prevalent one. When the patients were divided according to their HbF levels into three groups (Group A: HbF < 5%, Group B: HbF between 5 and 15%, and Group C: HbF > 15%), surprisingly, the highest levels of HbF were associated with the most severe clinical cases. Our findings suggest that fetal hemoglobin levels are important but not the only parameters that affect the severity of the disease. In addition, the high levels of HbF in patients with CAR haplotypes did not seem to ameliorate the severity of symptoms, suggesting that genetic factors other than haplotypes are the major determinants of increased HbF levels in Lebanon. [ABSTRACT FROM AUTHOR]

Provides information on a study that evaluated the effect of the change in chelation therapy, from desferrioxamine to deferiprone, on the psychosocial status of a series of thalassaemic patients. Methodology of the study; Results and discussion on the study.

Abstract: Pregabalin, a molecule with similar structure of GABA neurotransmitter, initially developed as an antiepileptic, is now commonly used in the treatment of painful peripheral neuropathies. We report an 82-year-old man who presented with confusion, urinary incontinence and gait instability. He was receiving pregabalin for a left L5 radicular pain. Laboratory tests at admission revealed a profound hyponatremia (117 mmol/L) that was corrected by fluid restriction and pregabalin withdrawal. According to the patient and laboratory outcome, we established the diagnosis of inappropriate antidiuretic hormone secretion due to pregabalin. The test of Naranjo demonstrated a probable imputability of pregabalin. [Copyright &y& Elsevier]

Abstract: Many neurologic and psychiatric manifestations have been associated with systemic lupus erythematosus. Narcolepsy, currently hypothesized as related to an autoimmune process, has been rarely associated with systemic lupus erythematosus. We report a 36-year-old woman who presented with narcolepsy and who subsequently developed systemic lupus erythematosus. Excessive daytime sleepiness resolved after the administration of four intravenous bolus of cyclophosphamide and methylprednisolone followed by maintenance therapy with hydroxychloroquine, aspirine and prednisone. Narcolepsy should be included in the neuropsychiatric manifestations of systemic lupus erythematosus and it may have a parallel clinical course to the activity of the lupus. [Copyright &y& Elsevier]

Background: This study evaluated the benefits and impact of ICU therapeutic interventions on the survival and functional ability of severe cerebrovascular accident (CVA) patients.Methods: Sixty-two ICU patients suffering from severe ischemic/haemorrhagic stroke were evaluated for CVA severity using APACHE II and the Glasgow coma scale (GCS). Survival was determined using Kaplan-Meier survival tables and survival prediction factors were determined by Cox multivariate analysis. Functional ability was assessed using the stroke impact scale (SIS-16) and Karnofsky score. Risk factors, life support techniques and neurosurgical interventions were recorded. One year post-CVA dependency was investigated using multivariate analysis based on linear regression.Results: The study cohort constituted 6% of all CVA (37.8% haemorrhagic/62.2% ischemic) admissions. Patient mean(SD) age was 65.8(12.3) years with a 1:1 male: female ratio. During the study period 16 patients had died within the ICU and seven in the year following hospital release. The mean(SD) APACHE II score at hospital admission was 14.9(6.0) and ICU mean duration of stay was 11.2(15.4) days. Mechanical ventilation was required in 37.1% of cases. Risk ratios were; GCS at admission 0.8(0.14), (p = 0.024), APACHE II 1.11(0.11), (p = 0.05) and duration of mechanical ventilation 1.07(0.07), (p = 0.046). Linear coefficients were: type of CVA - haemorrhagic versus ischemic: -18.95(4.58) (p = 0.007), GCS at hospital admission: -6.83(1.08), (p = 0.001), and duration of hospital stay -0.38(0.14), (p = 0.40).Conclusion: To ensure a better prognosis CVA patients require ICU therapeutic interventions. However, as we have shown, where tests can determine the worst affected patients with a poor vital and functional outcome should treatment be withheld? [ABSTRACT FROM AUTHOR]