Rituximab therapy for chronic lymphocytic leukemia-associated autoimmune hemolytic anemia

Autoimmune hemolytic anemia (AIHA) is a well-known complication of chronic lymphocytic leukemia (CLL). In recent years the anti-CD20 monoclonal antibody rituximab has been used for the therapy of steroid-refractory AIHA and autoimmune thrombocytopenia, either idiopathic or in association with CLL. We report the results of rituximab treatment for 14 patients suffering from CLL-associated AIHA. They developed a direct antiglobulin test positive AIHA at a mean time of 47 months (range 0-135 months) from the diagnosis of CLL. In 3 cases AIHA was diagnosed at the same time as CLL. Only 1 patient had fludarabine-related AIHA. All patients received steroids as first-line treatment. At a mean time of 46 days (range 1-210 days) from the diagnosis of AIHA all patients received rituximab at a dosage of 375 mg/m(2)/weekly for 4 weeks. All patients except 3 (2 died of cardiac failure or sepsis soon after the third cycle and 1 HCV-positive patient experienced a rise in serum amino transferases) completed the scheduled four programmed cycles. First injection side effects of rituximab were minimal. All but 2 patients showed an increase in hemoglobin levels in response to rituximab (mean value 3.6 g/dl; range 0.7-10 g/dl) and a reduction in the absolute lymphocyte count and lymph nodes and spleen volume. Nine patients required packed red cell transfusions before starting rituximab; 5 no longer needed transfusions just after the second cycle and another patient after the fourth cycle. Three patients (22%) were considered to fully respond and 7 (50%) only responded partially. At a mean follow-up of 17 months, 8 patients were still alive, 6 of them transfusion-free. Our results prove that the anti-CD20 monoclonal antibody is an effective and well-tolerated alternative treatment for CLL-associated AIHA.     

Common carotid artery pseudoaneurysm after neck dissection: colour Doppler ultrasound and multidetector computed tomography findings

Common carotid artery pseudoaneurysm is a rare disease, which has been previously unreported in association with neck dissection. We describe the Doppler ultrasound and multidetector computed tomography (CT) findings of a case of carotid pseudoaneurysm, one month after pharyngolaryngectomy with bilateral neck dissection. Multidetector CT confirmed the diagnosis made on the basis of Doppler ultrasound; the high image quality of axial and three-dimensional reconstructions avoided the need for pre-operative conventional angiography. In the presence of a pulsatile cervical mass after neck surgery, pseudoaneurysm of the carotid artery should be included in the differential diagnosis, and multidetector CT can be the sole pre-operative diagnostic imaging modality.     

Are early grammatical and phonological working memory abilities affected by preterm birth?

There have been few investigations of the effects of very immature preterm birth on specific linguistic competencies and phonological working memory at preschool age. Study 1 aimed to investigate early grammatical abilities in very immature healthy preterms, taking into account their cognitive development and biological and social factors. The linguistic and cognitive differences found between preterms and fullterms led to investigate in Study 2 the role of phonological working memory on preterms' grammatical development. Very immature preterm birth resulted to affect grammatical, cognitive and phonological working memory abilities until 3.5 years leading to persisting difficulties in comparison with fullterms, albeit not severe deficits. Tight relations between phonological working memory and grammar were found both in preterms and fullterms, that highlights the reciprocal support of these abilities in development. A partial compensatory effect by the maternal level of education on preterms' grammatical and cognitive abilities was also found. LEARNING OUTCOMES: The reader will become familiar with the relations between grammatical and phonological working memory abilities in typical and preterm 3.5-year-old children.     

Changes in the expression of hypothalamic lipid sensing genes in rat model of intrauterine growth retardation (IUGR)

Intrauterine growth retardation (IUGR) has been linked to the development of type 2 diabetes in later life. The mechanisms underlying this phenomenon are unknown. Recent data suggest that some of the molecular defects underlying type 2 diabetes reside in the CNS. The enzyme carnitine palmitoyltransferase-1 (CPT1) regulates long-chain fatty acid (LCFA) entry into mitochondria, where LCFA undergo beta-oxidation. Hypothalamic inhibition of CPT1 decreases food intake and suppresses endogenous glucose production. Our aim was to investigate the effects of uterine artery ligation, a procedure that mimics uteroplacental insufficiency, on the CNS expression of CPT1 and other key enzymes of LCFA metabolism. Bilateral uterine artery ligation was performed on d 19 of gestation in the pregnant rat; sham-operated pregnant rats served as controls. Hypothalamus, cerebellum, hippocampus, and cortex were dissected and analyzed at birth by real-time PCR. Nonesterified fatty acid (NEFA) serum levels were significantly higher in IUGR pups (p<0.0001). In IUGR rats, the hypothalamic expression of CPT1 isoform C (p=0.005) and acetyl-CoA carboxylase (ACC) isoforms alpha (p<0.05) and beta (p=0.005) were significantly decreased. The data presented here support the hypothesis that an abnormal intrauterine milieu can induce changes in hypothalamic lipid sensing.     

Health-related quality of life in dialysis patients. A report from an Italian study using the SF-36 Health Survey. DIA-QOL Group.

BACKGROUND: Interest in measuring health-related quality of life (HRQoL) has increased together with an awareness that such humanistic outcomes require valid and reliable measures. In the last decade short, simple and multidimensional generic and disease-specific questionnaires have been developed. Among the several generic questionnaires available, the Short Form 36 Items Health Survey (SF-36) was translated and validated in several languages, and applied to different settings and diseases. METHODS: Within the framework of a larger, prospective, multicentre study (DIA-QOL project) the SF-36 was administered to 304 patients to test its characteristics in terms of patient acceptability, and psychometric and clinical validity. Standard psychometric techniques were used to evaluate its validity in terms of convergence, divergence and internal consistency reliability (Cronbach's alpha). Correlations between clinical variables and HRQoL scores were performed to test the questionnaire's capability to capture differences across patients groups. RESULTS: Overall, the findings show that, in this sample, the SF-36's performance was very good. Acceptability was satisfactory, with a response rate higher than 80%. All the questionnaire scales met the psychometric standards suggested in terms of grouping and scaling assumptions. The internal reliability coefficients actually replicate the satisfactory findings reported previously for the original SF-36. In terms of the ability of the questionnaire scales to discriminate between groups expected to differ in a given health concept in relation to clinical variables, the results were also good. On average, females reported lower scores, the impact of ageing was more evident for physical scales. Diabetic patients score significantly worse on the physical function scale and patients with mental health problems score significantly lower on the mental health scale. No significant association was found with the index KtV, haemoglobin levels, body mass index, parathyroid hormone and type of dialysis. A strong association was indeed found between SF-36 scales measuring physical health concepts and the serum albumin level. This association held after adjusting for the confounding effect of age. Comparison of the health profile of the present sample with others from the US and UK and from a representative sample of the Italian general population highlights the potential of such questionnaire in dialysis setting. CONCLUSIONS: The SF-36 questionnaire is easy to use in Italian dialysis patients and SF-36 scores are related to important clinical aspects. This approach can help in caring for dialysis patients and can be useful in outcome assessment programmes.     

A 58‐year‐old man with B‐cell chronic lymphocytic leukemia and multiple strokes

A 58‐year‐old male with B‐cell chronic lymphocytic leukemia presented with fever, chest pain, and acute‐onset neurological deficits suggestive of multiple strokes (A). Brain autopsy revealed softening areas in the brain parenchyma (B, C) corresponding to extensive necrosis (D) caused by neuroinvasion by Aspergillus hyphae (E, F) necrosis (D) caused by neuroinvasion by Aspergillus hyphae (E, F).     

A rare case of congenital atresia of the left main coronary artery

Congenital atresia of the left main coronary artery is a rare coronary anomaly, in which the left coronary ostium is absent and the blood supply to the entire heart is provided by the right coronary artery. We show the images of the coronary CT angiography performed by a 56-year-old man, with evidence of this vascular abnormality. Coronary CT Angiography has gained a major role in coronary anomalies assessment, thank to high spatial and temporal resolution.