ABSTRACT
OBJECTIVES: Neurocognitive dysfunction has been established in several studies in children with beta-thalassemia major (TM). However, despite its wide occurrence in populations across the Arabian Peninsula, scant attention has been paid to shedding light on neuropsychological functioning among adults with TM. This study aimed to examine the level of neuropsychological functioning among Omani adults with TM regularly followed-up at a tertiary care hospital in Oman. A related aim was to examine the factors associated with neuropsychological performance. METHODS: Standard neuropsychological tests were used to measure attention and concentration, learning and remembering, verbal fluency, and executive functioning. Participants were also gauged on indices of intellectual ability and affective range. As normative data for neuropsychological functioning in Oman is scarce, healthy age- and sex-matched controls underwent the same testing procedure. The log-linear model was used to identify factors associated with TM patients on demographic and neuropsychological performance. RESULTS: This study recruited 28 adult patients with TM (age 30.0±6.5) and 39 healthy controls (age 29.2±6.1). Findings suggested that having a diagnosis of TM was significantly associated with symptoms of depression (p < 0.001) and anxiety (p < 0.001), indices of executive functioning (verbal fluency) (p =0.003), working memory (digit span) (p < 0.001), and verbal and auditory attention scores (California Verbal Learning, p = 0.002). CONCLUSIONS: The data appears to suggest that short-term memory capacity, verbal fluency, and verbal and auditory attention may be impaired in TM compared to controls. Studies on a larger cohort are therefore warranted.
ABSTRACT
Due to the recent alarming increase in the incidence of hepatocellular carcinoma (HCC) in thalassemias, the present report reviews briefly the frequency, the major risk factors, and the surveillance of HCC in ß-thalassemias. Over the past 33 years, 153 cases of HCC were reported in patients with thalassemia, mainly in Italy and Greece. Among HCV-infected patients, additional factors promoting the development of HCC included: advanced age, male sex, chronic hepatitis B (CHB) co-infection, and iron overload. For early diagnosis of HCC, sequential ultrasound screening is recommended especially for thalassemia patients with chronic hepatitis C (CHC), which coincides with (one or more) additional risk factors for HCC. Here we report also the preliminary data from thalassemic patients, above the age of 30 years, followed in 13 ICET-A centers. The total number of enrolled patients was 1,327 (males: 624 and 703 females). The prevalence of HCC in thalassemia major patients [characterized by transfusion-dependency (TDT)] and thalassemia intermedia [characterized by nontransfusion dependency (NTDT)] was 1.66 % and 1.96 %, respectively. The lowest age at diagnosis of HCC was 36 years for TDT and 47 years for NTDT patients. We hope that this review can be used to develop more refined and prospective analyses of HCC magnitude and risk in patients with thalassemia and to define specific international guidelines to support clinicians for early diagnosis and treatment of HCC in thalassemic patients.
