Acidente vascular cerebral na hemoglobinopatia SC(HBB glu6val e glu6lys): avaliação de marcadores de prognóstico / hemoglobinopathy (HBB glu6val and glu6lys): evaluation of prognostic markers

O acidente vascular cerebral (AVC) é uma complicação clínica grave da doença falciforme (DF). Poucos estudos avaliaram a velocidade do fluxo sanguíneo cerebral utilizando o Doppler transcraniano (DTC) e marcadores preditores do AVC na hemoglobinopatia SC (HbSC) e, desta forma, as velocidades consideradas de risco para os indivíduos com esta hemoglobinopatia são baseadas em velocidades descritas para a anemia falciforme (AF) e para a Sβ talassemia (HbS/β). Assim, o objetivo do presente estudo foi identificar marcadores preditores do AVC em indivíduos com HbSC, estabelecendo subfenótipos da doença pela associação de biomarcadores genéticos, hematológicos, bioquímicos e imunológicos com o valor da velocidade do fluxo sanguíneo cerebral. Para tanto, foi realizado um estudo transversal, onde foram investigados 68 indivíduos com HbSC. A velocidade média máxima do fluxo sanguíneo cerebral nas artérias cerebral média, carótida anterior e cerebral anterior foi determinada utilizando o DTC...

Stroke is a serious clinical complication of sickle cell disease (SCD). Only few studies have evaluated the rate of cerebral blood flow by transcranial Doppler (TCD) and stroke predictor markers on hemoglobinopathy SC (HbSC), thus, velocity considered as risk for stroke that is used to diagnose HbSC individuals are based on velocities described for the sickle cell anemia (SCA) and Sβ thalassemia. The objective of this study was to identify predictors markers of stroke in individuals with HbSC, establishing subphenotypes disease by the association of genetic biomarkers, hematological, biochemical and immunological with the value of the velocity of cerebral blood flow. For that, we conducted a cross-sectional study, which were investigated 68 HbSC individuals. The average maximum rate of cerebral blood flow in the middle cerebral artery, anterior cerebral artery and anterior carotid artery was determined using the DTC...

Mortality rate in Sickle Cell Disease Patients in Crisis at a Haematology Day Care Unit (HDCU) in Nigeria

The Haematology Day Care Unit (HDCU) of the University College Hospital; Ibadan; Nigeria was established in 1975 with the main goal of providing immediate and specialized care to haematological emergencies; particularly sickle cell disease (SCD) patients. Since inception; a systematic analysis of its effectiveness has not been done; hence this study. A retrospective study of all registered patients attending the Haematology Day Care Unit of the University College Hospital; Ibadan; over a one-year period was conducted and analyzed. Demographic data; diagnosis; treatment received; outcome of such treatment as well as laboratory parameters were extracted from HDCU register and the data were then analysed using descriptive statistics. A total of 890 patients were seen during the period; January and December 2001; out of which 520 were sickle cell disease patients (HbSS accounted for 508 (92.7) cases and HbSC; 40 (7.3) cases). The mean age of the SCD patients was 25.8years; the median; 23years and the mode; 18years. The mean PCV was 21.2; median; 21.0 and mode; 20. Majority (246 or 47.3) of the patients were between 20 and 30 years; the lowest frequency being in the 50years group (14 or 2.7). One patient died during the period under review (31year old female with HbSC disease); giving a mortality rate of 2 per 1000 patients