Sandra Fátima Menosi Gualandro

Brazilian Guidelines for transcranial doppler in children and adolescents with sickle cell disease

Background Sickle cell disease is the most common monogenic hereditary disease in Brazil. Although strokes are one of the main causes of morbidity and mortality in these patients, the use of transcranial Doppler to identify children at risk is not universally used. Objective To develop Brazilian guidelines for the use of transcranial Doppler in sickle cell disease children and adolescents, so that related health policies can be expanded, and thus contribute to reduce morbidity and mortality. Methods The guidelines were formulated in a consensus meeting of experts in transcranial Doppler and sickle cell disease. The issues discussed were previously formulated and scientific articles in databases (MEDLINE, SciELO and Cochrane) were carefully analyzed. The consensus for each question was obtained by a vote of experts on the specific theme. Results Recommendations were made, including indications for the use of transcranial Doppler according to the sickle cell disease genotype and patients age; the necessary conditions to perform the exam and its periodicity depending on exam results; the criteria for the indication of blood transfusions and iron chelation therapy; the indication of hydroxyurea; and the therapeutic approach in cases of conditional transcranial Doppler. Conclusion The Brazilian guidelines on the use of transcranial doppler in sickle cell disease patients may reduce the risk of strokes, and thus reduce the morbidity and mortality and improve the quality of life of sickle cell disease patients.

Resultados maternos e perinatais em gestações complicadas por doenças falciformes

PURPOSE: the aim of this study was to describe perinatal and maternal outcomes of pregnancies complicated by sickle cell disease (SCD), comparing to pregnancies of women with sickle cell trait (SCT). METHODS: this was a retrospective cohort study, covering the period from March 2001 to April 2008, which included all pregnant women with SCD (n=42) followed up at a university hospital in the Southeast region of Brazil. The maternal and perinatal outcomes were compared to those of pregnant women with SCT (n=56) who were followed up at the same service. RESULTS:SCD-SS was diagnosed in 42 (82.4%) pregnant women and SC in 9 (17.6%). Mean (±SD) maternal age was significantly lower in the SCD group (26.0 years) compared to SCT women (28.7±7.1 years; p=0.018). The following maternal complications were more common among women with SCD in comparison to SCT: urinary tract infection (25.5 versus 8.9%; p=0.04), pneumonia (23.5 versus 1.8%; p=0.002), pulmonary hypertension (15.7 versus 0%; p=0.002), and blood transfusion during delivery or postpartum (33.3 versus 5.4%; p=0.001). Adverse perinatal outcome was more frequent in the SCD group compared to the SCT group: prematurity (49 versus 25%, p=0.01); mean gestational age at delivery (35.2 versus 37.9 weeks, p<0.001); fetal distress (56.9 versus 28.6%, p=0.006); birth weight <2,500 g (62.7 versus 17.9%, p<0.001); mean birth weight (2,183 versus 2,923 g, p<0.001), and small for gestational age infants (29.4 versus 10.7%, p=0.029). Two maternal deaths (3.9%) occurred in the group with SCD. CONCLUSION: Pregnant women with SCD are at greater risk for maternal morbidity and for adverse perinatal outcomes than women with SCT.

Temporomandibular joint arthritis in sickle cell disease: a case report

We report a rare case of aseptic arthritis in the temporomandibular joint of a patient with sickle cell anemia. A 22-year-old woman with sickle cell disease, in the 18th week of gestation, was referred by her hematologist to investigate a sudden mouth opening limitation and severe pain on her left cheek. The patient received a standard pain assessment protocol, clinical examination, and complementary exams (complete blood count, hemoglobin electrophoresis, blood solubility test, panoramic radiograph, and magnetic resonance imaging [MRI]). The blood results were consistent with a sickle cell crisis and the MRI showed an inflammatory process around the left temporomandibular joint. Treatment with opioid analgesics and blood transfusion provided good results. Sickle cell anemia is a disease that can cause arthritis of the temporomandibular joint, and although it is rare, clinicians should be attentive to the differential diagnosis in patients with this disease.

Risk factors for deferral due to low hematocrit and iron depletion among prospective blood donors in a Brazilian center

Objective Deferral of blood donors due to low hematocrit and iron depletion is commonly reported in blood banks worldwide. This study evaluated the risk factors for low hematocrit and iron depletion among prospective blood donors in a large Brazilian blood center. Method A case–control study of 400 deferred donors due to low hematocrit and 456 eligible whole blood donors was conducted between 2009 and 2011. Participants were interviewed about selected risk factors for anemia, and additional laboratory tests, including serum ferritin, were performed. Bivariate and multivariate analyses were performed to assess the association between predictors and deferral due to low hematocrit in the studied population and iron depletion in women. Results Donors taking aspirins or iron supplementation, those who reported stomachache, black tarry stools or hematochezia, and women having more than one menstrual period/month were more likely to be deferred. Risk factors for iron depletion were repeat donation and being deferred at the hematocrit screening. Smoking and lack of menstruation were protective against iron depletion. Conclusion This study found some unusual risk factors related to gastrointestinal losses that were associated with deferral of donors due to low hematocrit. Knowledge of the risk factors can help blood banks design algorithms to improve donor notification and referral.

Cardiovascular autonomic dysfunction in sickle cell anemia.

Sickle cell anemia (SCA) is associated to increased cardiac output, normal heart rate (HR), abnormal QT dispersion and lower diastolic blood pressure (DBP). The mechanisms are still unknown. The objective of this study was to test the hypothesis that there is cardiovascular autonomic dysfunction (CAD) in SCA. The secondary objectives were to distinguish the roles of chronic anemia and hemoglobinopathy and to evaluate the predominance of the sympathetic or parasympathetic systems in the pathogenesis of CAD. Sixteen subjects with SCA, 13 with sickle cell trait (SCT), 13 with iron deficiency anemia (IDA), and 13 healthy volunteers (HV) were evaluated. All subjects were submitted to 24h-electrocardiogram (24h-ECG), plasma norepinephrine (NE) measurement before and after isometric exercise (IE), and also Valsalva maneuver (VM), diving maneuver (DV), and tilt test (TT). Baroreflex sensitivity (BRS) was also evaluated. The minimum, average and maximum HR as well as the percentage of bradycardia and tachycardia at 24-h ECG were similar in all groups. NE at baseline and after IE did not differ between groups. The SCA group showed less bradycardia at phase IV of VM, less bradycardia during DV, and also less tachycardia and lower DBP during TT. BRS for bradycardia and tachycardia reflex was decreased in the SCA and SCT groups. In conclusion, 1) there is CAD in SCA, and it is characterized by the reduction of BRS and the limitation of HR modulation mediated by the parasympathetic system; 2) cardiovascular sympathetic activity is preserved in SCA; and 3) hemoglobinopathy is the preponderant ethiopathogenic factor.

Acute chest syndrome in pregnant women with hemoglobin SC disease

discomfort, hypotension, and shortness of breath (oxygen saturation 90%). An emergency cesarean was performed. Placental abruption was detected, and a stillborn weighing 1,630 g was delivered. The laboratory test results showed hemoglobin electrophoresis of S 51%, C 47.5%, and F 1.5%, hemoglobin 8.4 g/dL, white blood cell count (WBCC) 34.3x10 9 /L, platelet count 142,000/mm 3 , alanine aminotransferase (ALT) 35 U/L, aspartate aminotransferase (AST) 54 U/L, total bilirubin 1.5 mg/dL, and lactate dehydrogenase (LDH) 791 U/L. The patient was transferred to the ICU immediately post-cesarean, where examination revealed that the uterus was contracted and without abnormal bleeding. At the fifth hour post-partum, she presented with bradycardia and hypotension. An intravenous infusion of vasoactive drugs and broad-spectrum antibiotics were administered. A red blood cell transfusion was performed. At the eighth hour post-partum, she was hemodynamically unstable, and echocardiography was used to diagnose diffuse hypokinesis of the right ventricle with dilatation of the right chambers. At the tenth hour post-cesarean, she presented with cardiovascular shock, bradycardia, hypotension, and cardiac arrest. The autopsy revealed bone marrow pulmonary emboli.

Guidelines on the treatment of anemia of chronic renal failure using recombinant human erythropoietin: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines Project: Associação Médica Brasileira – 2014

pecial article uidelines on the treatment of anemia of chronic enal failure using recombinant human rythropoietin: Associacao Brasileira de ematologia, Hemoterapia e Terapia Celular uidelines Project: Associacao Medica Brasileira – 014 derson da Silva Araujoa, Clarisse Lopes de Castro Lobob, Dimas Tadeu Covasc, ernando Ferreira Costad, Leticia Medeirose, Rodolfo Delfini Cancadof,∗, andra Fatima Menosi Gualandrog, Sara Teresinha Olalla Saadd Universidade Federal de Pernambuco (UFPE), Recife, PE, Brazil Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti (HEMORIO), Rio de Janeiro, RJ, Brazil Faculdade de Medicina de Ribeirao Preto (FMRP), Universidade de Sao Paulo (USP), Ribeirao Preto, SP, Brazil Universidade de Campinas (Unicamp), Campinas, SP, Brazil Associacao Medica Brasileira (AMB), Sao Paulo, SP, Brazil Faculdade de Ciencias Medicas da Santa Casa de Sao Paulo (FCMSCSP), Sao Paulo, SP, Brazil Universidade de Sao Paulo (USP), Sao Paulo, SP, Brazil