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  • Rev Bras Hematol Hemoter. 2011;33(1) 43

    Brazilian Guidelines for transcranial doppler in children and adolescents withsickle cell disease

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

    Keywords: Ultrasonography, doppler, transcranial/methods; Anemia, sickle cell/diagnosis;Anemia, sickle cell/therapy; Hemoglobin, sickle; Stroke/prevention & control; Child; Adolescent;Guideline

    Conflict-of-interest disclosure: The authorsdeclare no competing financial disclosure

    Submitted: 9/20/2010Accepted: 11/23/2010

    Correspondence: Clarisse LoboInstituto Estadual de Hematologia Arthur deSiqueira Cavalcanti, HEMORIORua Frei Caneca, 08 Centro20211-030 Rio de Janeiro ( RJ), Brazildiretoria@hemorio.rj.gov.br

    www.rbhh.org or www.scielo.br/rbhh

    DOI: 10.5581/1516-8484.20110014

    1Instituto Estadual de Hematologia Arthur

    de Siqueira Cavalcanti (HEMORIO),Rio de Janeiro (RJ), Brazil2Faculdade de Cincias Mdicas da Santa

    Casa de So Paulo (SP), Brazil3Fundao de Hematologia e Hemoterapia

    de Pernambuco (HEMOPE), Recife (PE), Brazil4Hospital das Clnicas, Faculdade de Medicina

    de Ribeiro Preto da Universidade deSo Paulo USP, Ribeiro Preto (SP), Brazil5Fundao Hemocentro Ribeiro Preto

    (SP), Brazil6Fundao Centro de Hematologia e

    Hemoterapia de Minas Gerais HEMOMINAS, Belo Horizonte (MG), Brazil7Servio de Hematologia e Transplante de

    Medula ssea, Hospital de Clnicas de PortoAlegre da UFRGS, Porto Alegre (RS), Brazil8Universidade Federal de So Paulo

    UNIFESP, So Paulo (SP), Brazil9Servio de Neurologia do Hospital de

    Clnicas, Universidade Federal do Paran UFPR, Curitiba (PR), Brazil10

    Ecodopler Laboratrio Rio de Janeiro(RJ), Brazil11

    Instituto de Puericultura e PediatriaMartago Gesteira IPPMG UniversidadeFederal do Rio de Janeiro UFRJ, Rio deJaneiro (RJ), Brazil12

    Medical University of South CarolinaMUSC Charleston, USA13

    Faculdade de Medicina, Universidade deSo Paulo USP, So Paulo (SP), Brazil

    Clarisse Lopes de Castro Lobo1

    Rodolfo Delfini Canado2

    Ana Claudia Celestino Bezerra Leite1

    Ana Claudia Mendona dos Anjos2

    Ana Cristina Silva Pinto3

    Andre Palma da Cunha Matta4

    Clia Maria Silva5

    Gisele Sampaio Silva6

    Joo Ricardo Friedrisch7

    Josefina Aparecida Pellegrini Braga8

    Marcos Christiano Lange9

    Maria Stella Figueiredo8

    Marlia lvares Rugani1

    Orlando Veloso10

    Patrcia Gomes Moura1

    Paulo Ivo Cortez11

    Robert Adams12

    Sandra Ftima Menosi Gualandro13

    Shirley Lopes de Castilho1

    Ursula Thom1

    Viviane Flumignan Zetola9

    Introduction

    Sickle cell disease (SCD) is the most common monogenic hereditary disease inBrazil occurring predominantly among African descents.(1) (A) The term SCD includessickle cell anemia (SCA) the form of the disease that occurs in homozygotes ofhemoglobin S (Hb SS), as well as pathological conditions caused by combinations ofthe hemoglobin S gene with other hereditary hemoglobin disorders such as sickle cellSC disease, Hb S/+ thalassemia and Hb S/0 thalassemia. Complications of SCD, suchas renal failure, vaso-occlusive crisis, acute chest syndrome and strokes result in areduction of 25 to 30 years of life expectancy of SCD patients compared to the generalpopulation.(2) (B)

    Recently, several aspects have contributed significantly to the reduction in mortalityin SCD patients. These include the control of infections through immunization and theprophylactic use of antibiotics during early life; watchfulness and appropriate guidancefor parents or caregivers to recognize early signs of splenic sequestration; the diagnosisand treatment of acute chest syndrome; and the identification of children at risk fordeveloping strokes using transcranial doppler (TCD), combined with early use of packedred blood cell (RBC) transfusions when test results are abnormal.(1) (D)

    HEMORIO, with the support of the Ministry of Health in Brazil, the AssociaoBrasileira de Hematologia e Hemoterapia, and the Pro-HEMORIO Foundation, decided todevelop the Brazilian guidelines on the use of transcranial doppler in sickle cell diseasepatients with the intention of expanding health policies for SCD and to contribute in reducingmorbidity and mortality resulting from this pathology. This work was developed in line withthe National Newborn Screening Program which guarantees universal access of newbornsto screening for hemoglobinopathies, and Government decree 1391, which establishesguidelines for the National Policy of Comprehensive Care for People with Sickle Cell Diseaseand other Hemoglobinopathies in the Brazilian national healthcare service.(1) (D)

    Special Article

  • 44 Rev Bras Hematol Hemoter. 2011;33(1)

    Lobo CL, Canado RD, Leite AC, Anjos AC, Pinto AC, Matta AP, et al

    Methods

    A committee of specialists was formed and theguidelines were formulated in two parallel consensusmeetings. At a consensus meeting of experts on TCD, nineregional facilitators, reference physicians in their regions onthis theme, discussed the technical issues of the procedure.Four national facilitators, physicians who are recognized asexperts in these sub-themes, presented the issues and thesupporting scientific evidence. Records of the debate and afinal summary of the findings for each question wereentrusted to two other specialists in TCD. The consensusmeeting was attended by experts in SCD, 26 regionalfacilitators who discussed technical matters involving theindication of TCD and the treatment to be adopted dependingon the different findings of TCD.

    The issues discussed at both consensus meetings werepreviously formulated by the national facilitators and twocoordinators of the event, all nationally renowned physicians.Before the meeting, the national facilitators and coordinatorswere also responsible for the identification of regionalfacilitators assigned to each theme as well as the selectionand distribution of publications to all participants. Thescientific papers were identified from the MEDLINE, SciELOand Cochrane databases and papers were classifiedaccording to the degree of recommendation and level ofevidence. The following designations were adopted: (A)highly consistent experimental or observational studies; (B)less consistent experimental or observational studies; (C)case reports (uncontrolled studies); (D) opinions withoutcritical assessment based on consensus, physiologic studiesor animal models. The consensus for each question wasobtained by voting and proposals were accepted even if therewas a maximum of three opposing votes. Abstentions werenot considered opposing votes. All participants had the rightto vote, except for five participants of organizations thatrepresent SCD carriers.

    Results

    Genotype of hemoglobinopathiesRecommendation: TCD should be used for primary

    prevention of strokes in SCD patients, regardless of thegenotype of the disease, but with priority to those with theHb SS and Hb S/0talassemia genotypes.

    Although the incidence of strokes is higher in patientswith the Hb SS genotype, it should be noted that strokesalso occur in patients with other genotypes. According to apublication of the Cooperative Study of Sickle Cell DiseaseGroup, the incidence of strokes (number of acute events/100patients/year) is 0.61 for patients with Hb SS, 0.17 for Hb SC,0.11 for Hb S/+ thalassemia and 0.10 for Hb S/0 thalassemia.It is estimated that 11%, 15% and 24% of SCA patients and2%, 4% and 10% of Hb SC patients develop symptomaticstrokes up to the ages of 20, 30 and 45 years old, respectively.

    The acute event may occur spontaneously or after any acutecomplication such as, for example, infections. Additionally,the study showed that the estimated prevalence of strokesadjusted for age was 4.01% in patients with Hb SS; 2.43% inHb S/0 thalassemia, 1.29% for Hb S/+ thalassemia and 0.84%for Hb SC.(3) (B)

    Given these data, it was deemed appropriate torecommend TCD as a method of primary prevention for allSCD patients regardless of genotype. The long-termmonitoring of the po

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