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Alpha thalassemia and beta-globin haplotype are considered classical genetic disease modifiers in sickle cell anemia (SCA) causing clinical heterogeneity. Nevertheless, their functional impact on SCA disease emergence and progression remains elusive. To better understand the role of alpha thalassemia and beta-globin haplotype in SCA, we performed a retrospective study evaluating the clinical manifestations of 614 patients. The univariate analysis showed that the presence of alpha-thalassemia -3.7-kb mutation (αα/-α and -α/-α) decreased the risk of stroke development (p = 0.046), priapism (p = 0.033), and cholelithiasis (p = 0.021). Furthermore, the cumulative incidence of stroke (p = 0.023) and cholelithiasis (p = 0.006) was also significantly lower for patients carrying the alpha thalassemia -3.7-kb mutation. No clinical effects were associated with the beta-globin haplotype analysis, which could be explained by the relatively homogeneous haplotype composition in our cohort. Our results reinforce that alpha thalassemia can provide protective functions against hemolysis-related symptoms in SCA. Although, several genetic modifiers can impact the inflammatory state of SCA patients, the alpha thalassemia mutation remains one of the most recurrent genetic aberration and should therefore always be considered first.

Background: Acute basilar artery occlusion is a devastating life-threatening condition. Early recanalization is the therapeutic goal in patients with acute ischemic stroke. Despite the high rates of recanalization achieved with modern devices for basilar occlusions, many patients have had poor clinical outcomes. This study aimed to assess the predictors of good and poor outcomes among patients with basilar artery occlusion treated with thrombectomy., Methods: A consecutive registry of 80 patients was included in this retrospective study. The primary end point was to access variables associated with neurologic outcomes defined by a modified Rankin Scale (mRS) score of 0-2, symptomatic intracranial hemorrhage (sICH), and mortality at 3 months follow-up., Results: Recanalization was achieved in 86.2%, and the sICH rate was 8.7%. A good neurologic outcome (mRS score 0-2) was observed in 26.2% and a moderate outcome (mRS score 0-3) in 32.5% of patients. The mortality was 38.7% at 3 months follow-up., Conclusions: After thrombectomy for posterior circulation strokes, young patients, V4-proximal basilar occlusion, (high) baseline posterior circulation Alberta Stroke Program Early CT Score, and complete recanalization were independent predictors of good neurologic outcomes. Failure to recanalize was strongly related to sICH and mortality. In addition, diabetes, atrial fibrillation, and baseline National Institutes of Health Stroke Scale scores ≥10 had an independent association with mortality. This study contributes to the knowledge required to optimize recanalization treatments for posterior circulation strokes and may help to improve future clinical studies., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Liver retransplantation (LReTx) is the therapeutic option for hepatic graft failure. Survival after LReTx is poorer than after primary liver transplantation. Given the organ shortage, it is essential to optimize the use of this resource., Objective: To evaluate rates, indications and patient survival after LReTx and identify factors associated with mortality following LReTx., Material and Methods: We conducted a retrospective cohort study of all adults undergoing LReTx based on registry data from the Liver Transplantation Group (Complexo Hospitalar Santa Casa de Porto Alegre), southern Brazil., Results: Between June 16, 1991 and July 19, 2011, 824 patients underwent 866 liver transplants. Forty-two procedures corresponded to LReTx (4.8% of all liver transplants performed). Thirty-eight patients who underwent a single LReTx procedure were included in this study. The leading indication for LReTx was hepatic artery thrombosis (HAT) (31.6%), followed by primary nonfunction (PNF) (18.4%). The main indication for early LReTx was PNF (58.3%) and for late LReTx was HAT (38.5%). During the follow-up period, 26 patients (68.4%) died after LReTx. Patient survival at 1 and 3 years after LReTx was 44.7% and 44.7%, respectively. Patients infected with hepatitis C virus, serum albumin < 2.5 g/dL and receiving mechanical ventilation immediately before LReTx had a significantly lower survival rate than the other patients., Conclusion: Considering the increased mortality when the graft loss is delayed, it is necessary to define the minimum acceptable results to indicate LReTx and identify the patients who would most benefit from this treatment.

Sickle cell disease has a worldwide distribution and is a public health problem in Brazil. Although vaso-occlusive crisis (VOC) is one of the most important clinical features of the disease, there are still several steps of its pathogenesis which are unknown. The increase of the chemotactic factor interleukin 8 (IL-8) has been reported to be involved in sickle cell disease crisis, but this has not been demonstrated conclusively. In the present study we analyzed serum IL-8 levels by ELISA and hematological parameters and hemoglobin patterns by standard techniques in 23 (21 SS and 2 SC) Brazilian patients with sickle cell syndromes during VOC caused by different inducing factors, 22 (21 SS and 1 SC) sickle cell patients out of crisis, and 11 healthy controls. Increased IL-8 levels were observed in 19 of 23 VOC patients (79.2%), 3 of them with more than 1,000 pg/ml. Seventeen of 22 (77.3%) non-crisis patients showed low IL-8 levels (less than 15 pg/ml). Healthy controls had low IL-8 levels. A significant difference in serum IL-8 levels was observed between crisis and non-crisis sickle cell patients (P<0.0001). There was no correlation between IL-8 levels and hematological data or hemoglobin patterns. High serum IL-8 levels were observed in VOC patients independently of the crisis-inducing factor. We conclude that in the studied population, IL-8 concentration may be a useful VOC marker, although the mechanism of the pathogenic process of sickle cell VOC syndromes remains unclear.

Adventitial cystic disease of the popliteal artery is an uncommon condition. Previous reports show a grouping of cases of this disease in Europe and Australasia. With the purpose of widening the knowledge of the geographical distribution of the disease, 3 cases from Brazil are described, and reference is made to 1 previously reported. It is suggested that not recognizing or not reporting the disease could be the cause for this grouping and that the number of diagnosed cases may increase in different parts of the world due to the greater awareness of the disease.