Navegando por Palavras-chave "sickle cell disease"
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- ItemSomente MetadadadosChanges in Transcranial Doppler Flow Velocities in Children with Sickle Cell Disease: The Impact of Hydroxyurea Therapy(Elsevier Science Bv, 2018) Adegoke, Samuel Ademola [UNIFESP]; Macedo-Campos, Rejane de Souza [UNIFESP]; Pellegrini Braga, Josefina Aparecida [UNIFESP]; Figueiredo, Maria Stella [UNIFESP]; Silva, Gisele Sampaio [UNIFESP]Background and Objectives: Hydroxyurea (HU) was recently described as a substitute for chronic transfusion for children with sickle cell disease (SCD) and abnormal transcranial Doppler (TCD) velocities who have received at least 1 year of transfusions. However, the role of HU in reverting elevated TCD velocities in patients not treated with transfusion is still debatable. The objective of the study was to examine whether HU influences the progression of TCD velocities in children with SCD. Patients and Methods: Children with SCD with at least 2 TCDs not less than 6 months apart were evaluated over 51 months. Time-averaged maximum mean (TAMM) velocities for the initial and the last transcranial Doppler examinations were noted and differences compared between HU and HU-naive groups. Results: Overall, 68.8% of the HU-group with elevated TCD velocities compared with 40.0% of the HU-naive experienced TCD reversal (P = .047). A higher proportion of the HU-naive group, 7 (14.3%) versus 9.8% of the HU group experienced TCD conversion. Those with initial conditional velocities in the HU-group experienced a significant reduction in TAMM velocities (from 176.8 +/- 5.3 to 162.7 +/- 13.9 cm/s, difference of 14.1 cm/s
- ItemSomente MetadadadosDepression and anxiety in adults with sickle cell disease: the PiSCES project(Lippincott Williams & Wilkins, 2008-02-01) Levenson, James L.; McClish, Donna K.; Dahman, Bassam A.; Bovbjerg, Vjktor E.; Citero, Vanessa de A. [UNIFESP]; Penberthy, Lynne T.; Aisiku, Imoigele P.; Roberts, John D.; Roseff, Susan D.; Smith, Wally R.; Virginia Commonwealth Univ; Univ Virginia; Universidade Federal de São Paulo (UNIFESP)Objective: Depression and anxiety are common in sickle cell disease (SCD) but relatively little is known about their impact on SCD adults. This study measured prevalence of depression and anxiety in SCD adults, and their effects on crisis and noncrisis pain, quality-of-life, opioid usage, and healthcare utilization. Methods: the Pain in Sickle Cell Epidemiology Study-is a prospective cohort study in 308 SCD adults. Baseline variables included demographics, genotype, laboratory data, health-related quality-of-life, depression, and anxiety. Subjects completed daily diaries for up to 6 months, reporting sickle cell pain intensity, distress, interference, whether they were in a sickle cell crisis, as well as health care and opioid utilization. Results: Two hundred thirty-two subjects who completed at least 1 month of diaries were studied; 27.6% were depressed and 6.5% had any anxiety disorder. Depressed subjects had pain on significantly more days than nondepressed subjects (mean pain days 71.1% versus 49.6%, p < .001). When in pain on noncrisis days, depressed subjects had higher mean pain, distress from pain, and interference from pain. Both depressed and anxious subjects bad poorer functioning on all eight SF-36 subscales, even after controlling for demographics, hemoglobin type, and pain. the anxious subjects had more pain, distress from pain, and interference from pain, both on noncrisis pain days and on crisis days, and used opioids more often. Conclusions: Depression and anxiety predicted more daily pain and poorer physical and mental quality-of-life in adults with SCD, and accounted for more of the variance in all domains of quality-of-life than hemoglobin type.
- ItemSomente MetadadadosEmbolization of intracranial aneurysms and sickle cell disease(Wiley-Blackwell, 2004-05-01) Vicari, Perla [UNIFESP]; Choairy, Ana Cristina Cardinalli [UNIFESP; Siufi, Grazziella Curado [UNIFESP]; Arantes, Adriano de Moraes [UNIFESP]; Fonseca, José Roberto Falco [UNIFESP]; Figueiredo, Maria Stella [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)The association of intracranial aneurysms and sickle cell disease (SCD) has been described in the English-language literature. Treatment strategies have included anglography and craniotomy. However, with advancement of non-operative interventions, much controversy surrounds the treatment of these patients. Endovascular embolization has been used in many patients with cerebral aneurysms, but it had been reported in the literature in only two sickle cell patients. the authors present a case of endovascular embolization of multiple intracranial aneurysms in a patient with sickle cell anemia with good outcome. (C) 2004 Wiley-Liss, Inc.
- ItemSomente MetadadadosFrequency and origins of hemoglobin S mutation in African-derived Brazilian populations(Wayne State Univ Press, 2007-12-01) Auricchio, Maria Teresa Balester De Mello; Vicente, Joao Pedro; Meyer, Diogo; Mingroni-Netto, Regina Celia; Universidade Federal de São Paulo (UNIFESP)Africans arrived in Brazil as slaves in great numbers, mainly after 1550. Before the abolition of slavery in Brazil in 1888, many communities, called quilombos, were formed by runaway or abandoned African slaves. These communities are presently referred to as remnants of quilombos, and many are still partially genetically isolated. These remnants can be regarded as relicts of the original African genetic contribution to the Brazilian population. In this study we assessed frequencies and probable geographic origins of hemoglobin S (HBB*S) mutations in remnants of quilombo populations in the Ribeira River valley, Sao Paulo, Brazil, to reconstruct the history of African-derived populations in the region. We screened for HBB*S mutations in 11 quilombo populations (1,058 samples) and found HBB*S carrier frequencies that ranged from 0% to 14%. We analyzed beta-globin gene cluster haplotypes linked to the HBB*S mutation in 86 chromosomes and found the four known African haplotypes: 70 (81.4%) Bantu (Central Africa Republic), 7 (8.1%) Benin, 7 (8.1%) Senegal, and 2 (2.3%) Cameroon haplotypes. One sickle cell homozygote was Bantu/Bantu and two homozygotes had Bantu/Benin combinations. The high frequency of the sickle cell trait and the diversity of HBB*S linked haplotypes indicate that Brazilian remnants of quilombos are interesting repositories of genetic diversity present in the ancestral African populations.
- ItemSomente MetadadadosInfluence of serum 25-hydroxyvitamin D on the rate of pain episodes in Nigerian children with sickle cell anaemia(Taylor & Francis Ltd, 2017) Adegoke, Samuel Ademola [UNIFESP]; Oyelami, Oyeku Akibu; Adekile, Adekunle; Figueiredo, Maria Stella [UNIFESP]Background: In sickle cell disease (SCD), symptoms of vitamin D deficiency (VDD) and chronic pain can overlap. Aim: To examine the relationship between serum vitamin D levels and the frequency of acute pain episodes. Methods: In this cross-sectional study, serum 25-hydroxyvitamin D (25-OHD) was assayed by high-performance liquid chromatography, and its influence on the number of significant pain episodes was examined by bivariate and logistic regression analyses. Results: The mean (SD) serum 25-OHD level in 123 children with SCD (HbSS) was 105.8 (24.1) nmol/L (range 37.5-155.8). Fourteen patients (11.4%) either had a deficient (1.6%) or insufficient (9.8%) level. None had severe VDD. All the children with sub-optimal vitamin D experienced pain, as did 69.7% of those with normal vitamin D [14 (100%) vs 76 (69.7%), 95% CI 0.7-0.9, p=0.04]. The mean serum vitamin D level in the 90 patients with at least one pain episode [103.1 (25.2) nmol/L] was significantly lower than the 113.1 (19.3) nmol/L in the 33 without a pain episode (95% CI 1.3-7.8, p=0.04). The frequency of pain correlated inversely with the serum 25-OHD level. Serum levels of vitamin D (OR 1.2, 95% CI 1.3-1.7, p=0.04) and fetal haemoglobin concentration (OR 1.6, 95% CI 1.1-1.4, p=0.02) predicted significant pain episodes. Conclusion: In children with SCD, there is a possible association between depressed serum vitamin D levels and increased frequency of acute pain episodes. Vitamin D supplements should be considered in patients with low levels and frequent episodes of pain.
- ItemSomente MetadadadosMigraine-mimicking headache and sickle cell disease: a transcranial Doppler study(Sage Publications Ltd, 2006-06-01) Silva, G. S.; Vicari, P.; Figueiredo, M. S.; Junior, H. C.; Idagawa, M. H.; Massaro, A. R.; Universidade Federal de São Paulo (UNIFESP)Headache occurs in sickle cell disease (SCD), but its characteristics and frequency have not previously been studied. Our aim was to study patterns of headache in adults with SCD and to correlate its presence with blood flow velocities measured by transcranial Doppler (TCD) and with brain magnetic resonance imaging (MRI) abnormalities. We studied 56 adults with SCD. Twenty-eight patients (50%) had severe and frequent headaches. in 20 patients (35.7%) the headache met the International Headache Society criteria for migraine without aura. Patients with frequent and severe headache presented TCD velocities significantly higher than those without headache, or with milder headache. No correlation was found between headache and abnormalities in brain MRI. A migraine-mimicking headache occurs in SCD but we should not understand it as a primary headache because the blood flow abnormalities secondary to SCD detected by TCD seem to play an important role in these patients.
- ItemSomente MetadadadosMultiple Primary Choledocholithiasis in Sickle Cell Disease(Japan Soc Internal Medicine, 2008-01-01) Vicari, Perla [UNIFESP]; Gil, Marco Vinicio Fanucchi [UNIFESP]; Cavalheiro, Rita de Cassia Rosario [UNIFESP]; Figueiredo, Maria Stella [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Choledocholithiasis can be caused by either primary or secondary bile duct stones. Although patients with sickle cell disease (SCD) are at high risk of development of pigmented gallstones due to chronic hemolysis, primary choledocholithiasis in SCD is very uncommon. the delay in the diagnosis of biliary tract pathology can occur in patients who had a prior cholecystectomy. There is a possible relationship between prior cholecystectomy and the subsequent occurrence of primary common bile duct stones, due to the higher probability of infection of the biliary system. Here, we report an unusual and severe case of multiple primary choledocholithiasis, associated with pancreatitis, in a patient with SCD, fourteen years after cholecystectomy.
- ItemAcesso aberto (Open Access)Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence(Assoc Arquivos Neuro- Psiquiatria, 2017) Rodrigues, Daniela Laranja Gomes [UNIFESP]; Adegoke, Samuel Ademola [UNIFESP]; Campos, Rejane de Souza Macedo [UNIFESP]; Braga, Josefina Aparecida Pellegrini [UNIFESP]; Figueiredo, Maria Stella [UNIFESP]; Silva, Gisele Sampaio [UNIFESP]Transcranial doppler (TCD) is a strategic component of primary stroke prevention in children with sickle cell disease (SCD). This study was conducted to examine the TCD characteristics of children with SCD in nine different medical centers in Brazil. Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121 +/- 23.83cm/s and 124 +/- 27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9.19 +/- 5.92 years) were evaluated with TCD for the first time. Conclusions: The SCD patients were evaluated with TCD at an older age, representing an important missed opportunity for stroke prevention. Since TCD screening in patients with SCD is important to detect those at high risk for stroke, it is recommended that this screening should be made more readily available.
- ItemSomente MetadadadosPeriodic limb movement in sleep and sickle cell disease: a neglected association?(Wiley, 2017) Cabanas-Pedro, Ana C. [UNIFESP]; Roizenblatt, Suely [UNIFESP]; de Souza, Altay A. L. [UNIFESP]|Tufik, Sergio [UNIFESP]; Figueiredo, Maria S. [UNIFESP]High frequency of periodic limb movements in sleep (PLMS) has been described among children with sickle cell disease (SCD), but there is little information about PLMS among adults with SCD. We aim to determine the frequency of PLMS among adults with SCD and to identify possible associations with iron status and haemolytic parameters. We analysed polysomnography on 99 adults: 74 with sickle cell anaemia (HbSS), 19 with HbSC (double heterozygosis HbS and HbC) and 6 with HbS-beta thalassaemia. Laboratory data were collected close to the time of the polysomnography examination. The prevalence of PLMS > 5/h was 70% and of PLMS > 15/h 36%, in the total group of patients. No differences were observed regarding gender, use of hydroxyurea and iron parameters. Logistic regression showed an association between PLMS > 15/h and hemolytic parameters: absolute reticulocyte count (p = 0.03) and unconjugated bilirubin (p = 0.01). Our data suggest that PLMS may be associated with manifestations of greater severity in SCD.
- ItemSomente MetadadadosRed blood cell alloimmunization in sickle cell disease: the influence of racial and antigenic pattern differences between donors and recipients in Brazil(Wiley-Blackwell, 1996-07-01) Moreira Junior, Gilberto [UNIFESP]; Bordin, Jose Orlando [UNIFESP]; Kuroda, Akemi [UNIFESP]; Kerbauy, José [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Red blood cell (RBC) transfusions are widely used in the management of patients with sickle cell disease (SCD). However, repeated RBC transfusions are often complicated by RBC alloimmunization. To investigate whether the frequency of RBC alloimmunization could be accounted for by racial and RBC phenotype differences between donors and recipients in Brazil, in this study we compared the RBC phenotype of 100 SCD patients with that observed in 120 randomly selected blood donors. A comparison of the RBC phenotype between the two groups revealed a statistically significant increase in the frequency of the C antigen in the donor population (P < 0.01), but no significant difference was observed for the A, B, D, c, E, e, K, k, Fy(a), M, N, S, s, and Jk(a) antigens. Using standard techniques (indirect antiglobulin test, enzyme treatment, and low-ionic-strength solution) we observed an RBC alloimmunization rate of 12.9% (11/85) in the SCD patients. Fifteen alloantibodies were detected in 11 patients, and most (80%) involved antigens in the Rhesus and Kelt systems. This observed RBC alloimmunization rate in SCD patients in Brazil is lower than that reported by studies from North America, suggesting that the requirement for extended antigen-matched RBC transfusion for SCD patients in the setting of a RBC phenotype concordant donor-recipient population may not be cost-effective in some countries. (C) 1996 Wiley-Liss, Inc.
- ItemAcesso aberto (Open Access)Renal dysfunction in patients with sickle cell anemia or sickle cell trait(Associação Brasileira de Divulgação Científica, 1998-10-01) Sesso, Ricardo de Castro Cintra [UNIFESP]; Almeida, M.a.; Figueiredo, Maria Stella [UNIFESP]; Bordin, Jose Orlando [UNIFESP]; A01; Universidade Federal de São Paulo (UNIFESP)Patients with sickle cell anemia (Hb SS) or sickle cell trait (Hb AS) may present several types of renal dysfunction; however, comparison of the prevalence of these abnormalities between these two groups and correlation with the duration of disease in a large number of patients have not been thoroughly investigated. In a cross-sectional study using immunoenzymometric assays to measure tubular proteinuria, microalbuminuria, measurement of creatinine clearance, urinary osmolality and analysis of urine sediment, we evaluated glomerular and tubular renal function in 106 adults and children with Hb SS (N = 66) or Hb AS (N = 40) with no renal failure (glomerular filtration rate (GFR) >85 ml/min). The percentage of individuals with microalbuminuria was higher among Hb SS than among Hb AS patients (30 vs 8%, P<0.0001). The prevalence of microhematuria was similar in both groups (26 vs 30%, respectively). Increased urinary levels of retinol-binding protein or ß2-microglobulin were detected in only 3 Hb SS and 2 Hb AS patients. Urinary osmolality was reduced in patients with Hb SS or with Hb AS; however, it was particularly evident in Hb SS patients older than 15 years (median = 393 mOsm/kg, range = 366-469) compared with Hb AS patients (median = 541 mOsm/kg, range = 406-722). Thus, in addition to the frequently reported early reduction of urinary osmolality and increased GFR, nondysmorphic hematuria was found in 26 and 30% of patients with Hb SS or Hb AS, respectively. Microalbuminuria is an important marker of glomerular injury in patients with Hb SS and may also be demonstrated in some Hb AS individuals. Significant proximal tubular dysfunction is not a common feature in Hb SS and Hb AS population at this stage of the disease (i.e., GFR >85 ml/min).
- ItemSomente MetadadadosThe role of catastrophizing in sickle cell disease - the PiSCES project(Elsevier B.V., 2007-12-15) Citero, Vanessa de A. [UNIFESP]; Levenson, James L.; McClish, Donna K.; Bovbjerg, Viktor E.; Cole, Portia L.; Dahman, Bassam A.; Penberthy, Lynne T.; Aisiku, Imoigele P.; Roseff, Susan D.; Smith, Wally R.; Virginia Commonwealth Univ; Universidade Federal de São Paulo (UNIFESP); Univ VirginiaIn several types of chronic pain, catastrophizing has been related to higher pain intensity, and health care utilization but it has not been explored extensively in sickle cell disease (SCD). the objective of the study was to identify the role of catastrophizing in SCD, specifically in relation to painful crises, non-crisis pain, and responses to pain. Two hundred and twenty SCD adults were enrolled in a prospective cohort study of pain and completed between 30 and 188 daily diaries in 6 months. the Catastrophizing subscale (CAT) of the Coping Strategy Questionnaire (CSQ) was administered at baseline and at study exit. Depression and quality of life were measured by the Patient Health Questionnaire and SF-36, respectively, at baseline. the CAT mean was 13.6 (SD = 8.4) and higher CAT was correlated with greater depression severity (r = 0.48; p < 0.001) and poorer quality of life in all domains (r = -0.24 to -0.47; p < 0.001). There was no significance difference between CAT mean baseline and exit scores, and the measures were strongly correlated within patients (r = 0.69; p < 0.001). No difference was found between higher and lower catastrophizers in intensity of pain, distress, interference, and health service utilization, both on crisis or non-crisis SCD-related pain days, after controlling for depression. Adults with SCD had a higher mean catastrophizing score than found in studies of other chronic pain conditions that are not lifelong and life-threatening. CAT scores were not correlated with pain parameters or utilization. the role of catastrophizing in other conditions cannot be generalized to SCD. (c) 2007 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.
- ItemSomente MetadadadosTranscranial Doppler in adult patients with sickle cell disease(Karger, 2006-01-01) Silva, G. S. [UNIFESP]; Vicari, P. [UNIFESP]; Figueiredo, M. S.; Castelo, A. [UNIFESP]; Valadi, N.; Massaro, A. R. [UNIFESP]; Universidade Federal de São Paulo (UNIFESP); Med Coll GeorgiaBackground: Transcranial Doppler (TCD) is the key test in determining the need for prophylactic blood transfusion to prevent stroke in children with sickle cell disease (SCD). the role of TCD in assessing stroke risk in adults with SCD as well as the pattern of TCD velocities in these patients are still undetermined. Methods: the authors compared TCD velocities in SCD adults (n = 56) with those of healthy controls (n = 56). Results: Velocities in SCD adults were lower than those found in children and higher than in controls and negatively correlated to hematocrit in both groups. Conclusion: the pattern of TCD velocities in adults with SCD is different from that described in children. Age-specific TCD criteria may assist the detection of stroke risk in these patients. Copyright (c) 2006 S. Karger AG, Basel.