Emilia Matos do Nascimento

The effect of hydroxcarbamide therapy on survival of children with sickle cell disease.

Although evidence is accumulating that hydroxycarbamide decreases mortality among adults with sickle cell disease (SCD), there are no published data regarding the effect of hydroxycarbamide on mortality among children. The Paediatric Hydroxycarbamide Program was established to treat children with SCD aged 3–18 years if they met disease severity criteria. Mortality data and clinical/laboratorial effects of hydroxycarbamide were retrospectively collected for the first 9 years of the Program. Mortality among those who received hydroxycarbamide was compared to that of untreated children. Among 1760 subjects, 267 received hydroxycarbamide at a median dose of 20·8 mg/kg/d (range 10–32) for a median of 2 years (range 0·1–6·5). Survival among hydroxycarbamide‐treated children was significantly greater than that among untreated ones (99·5% vs. 94·5%, P = 0·01), due primarily to fewer deaths from acute chest syndrome and infection. Hydroxycarbamide therapy was significantly associated with increases in haemoglobin concentration, fetal haemoglobin, mean corpuscular volume, and reduction in platelet counts, reticulocytes and neutrophils. Toxicity was minimal and predominantly mild reversible neutropenia. Significantly fewer hospitalizations and emergency room visits, and shorter admissions were observed among hydroxycarbamide‐treated subjects, when compared to the 12‐month period prior to treatment initiation. Hydroxycarbamide therapy reduces disease severity and is probably associated with decreased mortality among children with SCD.

Newborn screening program for hemoglobinopathies in Rio de Janeiro, Brazil.

Newborn screening for hemoglobinopathy in Brazil has been decentralized until 2001 when the Health Ministry of Brazil established the National Newborn Hemoglobinopathy Screening Program. The State of Rio de Janeiro started a program in collaboration with the State Health Department and the Institute of Hematology in Rio (HEMORIO). The goal of this study was to evaluate the effectiveness of the first 10 years of the Newborn Hemoglobinopathy Screening Program in identifying and managing infants with Sickle cell disease (SCD) in the State of Rio de Janeiro.

Prognostic impact of clinic and ambulatory blood pressure components in high-risk type 2 diabetic patients: the Rio de Janeiro Type 2 Diabetes Cohort Study.

Background: The prognostic importance of tight clinic blood pressure (BP) control is controversial in diabetic patients. The objective was to investigate the prognostic impact of clinic and ambulatory BPs for cardiovascular morbidity and mortality in type 2 diabetes. Methods: In a prospective cohort study, 565 type 2 diabetic patients had clinical, laboratory and ambulatory BP monitoring (ABPM) data obtained at baseline and during follow-up. The primary endpoints were a composite of fatal and nonfatal cardiovascular events and all-cause mortality. Multivariable Cox survival and splines regression analyses assessed associations between each BP component [SBP, DBP and pulse pressure (PP)] and the endpoints. Results: After a median follow-up of 5.75 years, 88 total cardiovascular events and 70 all-cause deaths occurred. After adjustments for cardiovascular risk factors, clinic SBP and DBPs were predictive of the composite endpoint but not of all-cause mortality, whereas all ambulatory BP components were predictors of both endpoints. Ambulatory systolic and PPs were the strongest predictors and achieved ambulatory BPs during follow-up improved risk prediction in relation to baseline values. When categorized at clinically relevant cut-off values, risk began only at clinic BPs at least 140/90 mmHg, whereas for ambulatory BPs it began at lower values (≥120/75 mmHg for the 24-h period). Conclusion: ABPM provides more valuable information regarding cardiovascular risk stratification than office BPs and should be performed, if possible, in every high-risk type 2 diabetic patient. Achieved 24-h ambulatory BPs less than 120/75 mmHg are associated with significant cardiovascular protection and, if confirmed by other studies, may be considered as BP treatment targets.

Is Poststroke Depression a Major Depression

Background: Poststroke depression (PSD) is the most common neuropsychiatric consequence of stroke. A large number of studies have focused on the pathogenesis of PSD, but only a few aimed to characterize its psychopathology; these studies yielded results that are difficult to compare because of the different methods utilized. The current study aimed to characterize the symptom profile of PSD in an attempt to better understand the disease and allow a more accurate diagnosis. Methods: The study sample comprised 64 patients divided into three groups: stroke patients without diagnosis of depression (n = 33), stroke patients diagnosed with PSD (PSD group, n = 14) and patients diagnosed with major depression (MD) but with no clinical comorbidity (MD group, n = 17). All patients were diagnosed using the Structured Clinical Interview for DSM-IV Axis I Disorders (SCID-I). The initial diagnostic interview was complemented by the Mini Mental State Examination (MMSE), the Rankin Scale, and four scales for the assessment of the intensity of symptoms of anxiety and depression: the Beck Depression Inventory (BDI), the Hospital Anxiety and Depression General Scale (HADS), the Hamilton Depression Rating Scale (HAM-D) and the Hamilton Rating Scale for Anxiety (HAM-A). The Star Plot, a graphical method of data visualization, was used to analyze the results. The t test was used for independent samples (two-tailed analysis). Results: As measured by the BDI, HAM-D and HAM-A scales and HADS depression subscale, the average total scores of symptoms for the sample of patients diagnosed with MD without clinical comorbidity was significantly higher than that of the PSD patients (p < 0.05). Similar results were obtained by plotting the BDI data on Star Plot. The PSD patients showed mild typical depressive symptoms such as less depressed mood, anhedonia, disinterest, guilt, negative thoughts, depreciation, suicidal ideation and anxiety, when evaluated by the HAM-A scale. Moreover, the somatic symptoms of depression did not lead to increased diagnosis of major depression in stroke patients. Conclusions: The results indicate that the PSD clinical picture comprised, in general, symptoms of mild/moderate intensity, especially those considered as pillars for the diagnosis of depression: depressed mood, loss of pleasure and lack of interest. Given the imprecision of boundaries that separate the clinical forms of depression from subclinical and nonpathological forms, or even from the concepts of demoralization and adjustment disorders, we situate PSD in a complex biopsychosocial context in which a better understanding of its psychopathological profile could provide diagnostic and therapeutic alternatives best suited to the difficult reality experienced by stroke patients.

Relationship between Fibrosis and Ventricular Arrhythmias in Chagas Heart Disease Without Ventricular Dysfunction

Background Patients with Chagas disease and segmental wall motion abnormality (SWMA) have worse prognosis independent of left ventricular ejection fraction (LVEF). Cardiac magnetic resonance (CMR) is currently the best method to detect SWMA and to assess fibrosis. Objective To quantify fibrosis by using late gadolinium enhancement CMR in patients with Chagas disease and preserved or minimally impaired ventricular function (> 45%), and to detect patterns of dependence between fibrosis, SWMA and LVEF in the presence of ventricular arrhythmia. Methods Electrocardiogram, treadmill exercise test, Holter and CMR were carried out in 61 patients, who were divided into three groups as follows: (1) normal electrocardiogram and CMR without SWMA; (2) abnormal electrocardiogram and CMR without SWMA; (3) CMR with SWMA independently of electrocardiogram. Results The number of patients with ventricular arrhythmia in relation to the total of patients, the percentage of fibrosis, and the LVEF were, respectively: Group 1, 4/26, 0.74% and 74.34%; Group 2, 4/16, 3.96% and 68.5%; and Group 3, 11/19, 14.07% and 55.59%. Ventricular arrhythmia was found in 31.1% of the patients. Those with and without ventricular arrhythmia had mean LVEF of 59.87% and 70.18%, respectively, and fibrosis percentage of 11.03% and 3.01%, respectively. Of the variables SWMA, groups, age, LVEF and fibrosis, only the latter was significant for the presence of ventricular arrhythmia, with a cutoff point of 11.78% for fibrosis mass (p < 0.001). Conclusion Even in patients with Chagas disease and preserved or minimally impaired ventricular function, electrical instability can be present. Regarding the presence of ventricular arrhythmia, fibrosis is the most important variable, its amount being proportional to the complexity of the groups.

Deep venous thrombosis in children with sickle cell disease

Deep venous thrombosis (DVT) is rare in children compared to adults. Its incidence and risk factors in children are not well known. This study determined these aspects of DVT in children with sickle cell disease (SCD).

Treatment of the acute sickle cell vaso-occlusive crisis in the Emergency Department: a Brazilian method of switching from intravenous to oral morphine

Describe the treatment of patients with vaso‐occlusive crises (VOC) in a Brazilian emergency department (ED) and the successful switch from intravenous to oral morphine.

The Mean Corpuscular Volume and Hydroxyurea in Brazilian Patients with Sickle Cell Anemia: A Surrogate Marker of Compliance

The suppression of erythropoiesis by Hydroxyurea (HU) therapy is associated with increase in mean corpuscular volume, in addition to the increase in Hb F. Monitoring the mean corpuscular volume values and the presence of macrocytosis are effective tools of adherence to the treatment with HU in patients with sickle cell anemia. The aim of this study is to monitor the mean corpuscular volume values after starting treatment with HU to determine if macrocytosis can be used as a surrogate marker of compliance with therapy. We conducted a prospective cohort study over one year with measurements of blood counts and mean corpuscular volume after starting therapy with HU in 95 patients with sickle cell anemia who were regularly followed in our ambulatory outpatient unit. In one-year of successful use of HU the mean value of the mean corpuscular volume increased significantly. The Andersen and Gill model demonstrated that the increase of one unit of MCV implies a 5% reduction in the risk of visiting the emergency room. Monitoring mean corpuscular volume values after prescribing HU alerts the provider of noncompliance in order to counsel the patient in question for better adherence to the use of HU that could improve the quality of care and to reduce morbidity and the frequency of acute pain crises and associated healthcare costs.

Alterações contráteis segmentares e sua associação com arritmias ventriculares complexas, em pacientes chagásicos com eletrocardiograma normal ou borderline

INTRODUCTION: Sudden death is the major cause of death among chagasic patients. A significant number of fatal events in patients without apparent heart disease and borderline electrocardiogram, but with contractile ventricular dysfunction, have been documented. This work aimed to determine the association between regional dysfunction and the presence of ventricular arrhythmia in chagasic patients without apparent heart disease. METHODS: Forty-nine patients with normal or borderline electrocardiogram were submitted to echocardiogram, exercise stress test and Holter. The presence of cardiac contractile alterations and complex ventricular arrhythmia was analyzed. Statistic analysis used the general Log-Linear model. RESULTS: Mean age 56 years-old; 55% women. Regional ventricular dysfunction was verified in 24.5% of patients; positive Holter in 12% and exercise stress test in 18%. An association between complex ventricular arrhythmia and contractile abnormalities in the presence of mild left ventricle dysfunction was verified. CONCLUSIONS: Regional contractile abnormalities with mild left ventricle dysfunction in Chagasic patients indicate a group with higher risk of complex ventricular arrhythmias, who require specific follow-up.

Impact of the Use of Benznidazole Followed by Antioxidant Supplementation in the Prevalence of Ventricular Arrhythmias in Patients With Chronic Chagas Disease: Pilot Study.

Patients with chronic Chagas disease have a higher prevalence of premature ventricular contraction (PVC) because of immunoinflammatory response magnified by the increased oxidative stress. Thus, the sequential treatment with benznidazole (BZN) and antioxidants can reduce the prevalence of PVC. We wish to establish whether the etiological treatment of Chagas disease followed by supplementation with the antioxidant vitamins E and C decreases the prevalence of PVC in these patients. A sample of 41 patients with chronic Chagas disease at different stages of the heart disease was selected for the treatment against the causative agent using BZN (5 mg·kg−1·d−1, minimum dose 300 mg daily) for 2 months followed by supplementation with antioxidants such as vitamins E (800 UI/d) and C (500 mg/d) for 6 months. The prevalence of PVC was observed by conducting 24-hour Holter. To evaluate the oxidative status of the patients, serum markers of oxidative stress like glutathione peroxidase, superoxide dismutase, catalase, glutathione reductase, and glutathione S-transferase were measured, and also reduced glutathione, vitamin E, and markers of tissue damage like thiobarbituric acid reactive substances and protein carbonyl. A decrease in the prevalence of PVC in patients with advanced Chagas heart disease was observed (5391 vs. 1185, P = 0.0068). This reduction was followed by decrease of serum markers of oxidative stress. In patients with a lower degree of cardiac damage, the reduction on prevalence of PVC was not significant. The etiological treatment with BZN followed by supplementation with antioxidant vitamins E and C reduces episodes of PVC in patients with severe Chagas heart disease.