Results from transcranial Doppler examination on children and adolescents with sickle cell disease and correlation between the time-averaged maximum mean velocity and hematological characteristics
a cross-sectional analytical study
Keywords:
Ultrasonography, Doppler, transcranial, Anemia, sickle cell, Hemoglobin SC disease, Stroke, Child, AdolescentAbstract
CONTEXT AND OBJECTIVE: Transcranial Doppler (TCD) detects stroke risk among children with sickle cell anemia (SCA). Our aim was to evaluate TCD findings in patients with different sickle cell disease (SCD) genotypes and correlate the time-averaged maximum mean (TAMM) velocity with hematological characteristics. DESIGN AND SETTING: Cross-sectional analytical study in the Pediatric Hematology sector, Universidade Federal de São Paulo. METHODS: 85 SCD patients of both sexes, aged 2-18 years, were evaluated, divided into: group I (62 patients with SCA/Sß0 thalassemia); and group II (23 patients with SC hemoglobinopathy/Sß+ thalassemia). TCD was performed and reviewed by a single investigator using Doppler ultrasonography with a 2 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. The hematological parameters evaluated were: hematocrit, hemoglobin, reticulocytes, leukocytes, platelets and fetal hemoglobin. Univariate analysis was performed and Pearson’s coefficient was calculated for hematological parameters and TAMM velocities (P < 0.05). RESULTS: TAMM velocities were 137 ± 28 and 103 ± 19 cm/s in groups I and II, respectively, and correlated negatively with hematocrit and hemoglobin in group I. There was one abnormal result (1.6%) and five conditional results (8.1%) in group I. All results were normal in group II. Middle cerebral arteries were the only vessels affected. CONCLUSION: There was a low prevalence of abnormal Doppler results in patients with sickle-cell disease. Time-average maximum mean velocity was significantly different between the genotypes and correlated with hematological characteristics.
Downloads
References
Manual de diagnóstico e tratamento de doenças falciformes. Brasília: Anvisa; 2002. Avai- lable from: http://www.anvisa.gov.br/divulga/public/sangue/hemoglobinopatia/diagnos- tico.pdf. Accessed in 2011 (Jan 24).
Cançado RD, Jesus JA. A doença falciforme no Brasil (editorial) [Sickle cell disease in Brazil: (editorial)]. Rev Bras Hematol Hemoter. 2007;29(3):204-6.
Huttenlocher PR, Moohr JW, Johns L, Brown FD. Cerebral blood flow in sickle cell cerebrovas- cular disease. Pediatrics. 1984;73(5):615-21.
Zago MA, Pinto ACS. Fisiopatologia das doenças falciformes: da mutação genética à insu- ficiência de múltiplos órgãos [The pathophysiology of sickle cell disease: from the genetic mutation to multiorgan dysfunction]. Rev Bras Hematol Hemoter. 2007;29(3):207-14.
Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-94.
Balkaran B, Char G, Morris JS, et al. Stroke in a cohort of patients with homozygous sickle cell disease. J Pediatr. 1992;120(3):360-6.
Russell MO, Goldberg HI, Hodson A, et al. Effect of transfusion therapy on arteriographic ab- normalities and on recurrence of stroke in sickle cell disease. Blood. 1984;63(1):162-9.
Adams RJ, Nichols FT, Figueroa R, McKie V, Lott T. Transcranial Doppler correlation with cere- bral angiography in sickle cell disease. Stroke. 1992;23(8):1073-7.
Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 1998;339(1):5-11.
Adams RJ. Stroke prevention and treatment in sickle cell disease. Arch Neurol. 2001;58(4):565-8.
Goldstein LB, Adams R, Becker K, et al. Primary prevention of ischemic stroke: A statement for healthcare professionals from the Stroke Council of the American Heart Association. Stroke. 2001;32(1):280-99.
Sacco RL, Adams R, Albers G, et al. Guidelines for prevention of stroke in patients with ische- mic stroke or transient ischemic attack: a statement for healthcare professionals from the American Heart Association/American Stroke Association Council on Stroke: co-sponsored by the Council on Cardiovascular Radiology and Intervention: the American Academy of Neurology affirms the value of this guideline. Stroke. 2006;37(2):577-617.
Department of Health & Human Services. Clinical alert from the National Heart, Lung and Blood Institute. Bethesda: National Institutes of Health; 2004. Available from: http://www. nhlbi.nih.gov/health/prof/blood/sickle/clincial-alert-scd.pdf. Accessed in 2011 (Jan 24).
Riddington C, Wang W. Blood transfusion for preventing stroke in people with sickle cell disease. Cochrane Database Syst Rev. 2002;(1):CD003146.
Adams R, McKie V, Nichols F, et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med. 1992;326(9):605-10.
Aaslid R, Markwalder TM, Nornes H. Noninvasive transcranial Doppler ultrasound recording of flow velocity in basal cerebral arteries. J Neurosurg. 1982;57(6):769-74.
Arnolds BJ, von Reutern GM. Transcranial Doppler sonography. Examination technique and normal reference values. Ultrasound Med Biol. 1986;12(2):115-23.
Adams RJ, Nichols FT, Stephens S, et al. Transcranial Doppler: the influence of age and hematocrit in normal children. J Cardiovasc Ultrasonog. 1988;7(3):201-5.
Adams RJ, Nichols FT, McKie VC, et al. Transcranial Doppler: influence of hematocrit in chil- dren with sickle cell anemia without stroke. J Cardiovasc Technol. 1989;8:97-101.
Ringelstein EB, Kahlscheuer B, Niggemeyer E, Otis SM. Transcranial Doppler sonography: anatomical landmarks and normal velocity values. Ultrasound Med Biol. 1990;16(8): 745-61.
Adams RJ, Nichols FT 3rd, Aaslid R, et al. Cerebral vessel stenosis in sickle cell disease: criteria for detection by transcranial Doppler. Am J Pediatr Hematol Oncol. 1990;12(3): 277-82.
Kinney TR, Sleeper LA, Wang WC, et al. Silent cerebral infarcts in sickle cell anemia: a risk fac- tor analysis. The Cooperative Study of Sickle Cell Disease. Pediatrics. 1999;103(3):640-5.
Park MVF, Pádua SS, Passos RA, et al. Avaliação do Doppler transcraniano em pacientes com doença falciforme. Rev Bras Hematol Hemoter. 2006;28(2):14. [tema livre].
Melo HA, Barreto-Filho JAS, Prado RC, Cipolotti R. Doppler transcraniano em portadores de anemia falciforme: estudo dos parâmetros de fluxo sangüíneo cerebral em crianças de Ara- caju, Sergipe [Transcranial doppler in sickle cell anaemia: evaluation of brain blood flow pa- rameters in children of Aracaju, Northeast-Brazil]. Arq Neuropsiquiatr. 2008;66(2b):360-4.
Silva C, Borato M, Freitas A, et al. Rastreamento de crianças com anemia falciforme pelo Doppler transcraniano para detecção de risco de acidente vascular cerebral – ex- periência do hemocentro de Belo Horizonte - Hemominas. Rev Bras Hematol Hemoter. 2008;30(4):237-8. [tema livre].
Graido-Gonzales E, Doherty JC, Bergreen EW, et al. Plasma endothelin-1, cytokine, and prostaglandin E2 levels in sickle cell disease and acute vaso-occlusive sickle crisis. Blood. 1998;92(7):2551-5.
Kirkham FJ, Prengler M, Hewes DK, Ganesan V. Risk factors for arterial ischemic stroke in children. J Child Neurol. 2000;15(5):299-307.
Telen MJ. Red blood cell surface adhesion molecules: their possible roles in normal human physiology and disease. Semin Hematol. 2000;37(2):130-42.
Solovey AA, Solovey AN, Harkness J, Hebbel RP. Modulation of endothelial cell activation in sickle cell disease: a pilot study. Blood. 2001;97(7):1937-41.
McIntyre TM, Prescott SM, Weyrich AS, Zimmerman GA. Cell-cell interactions: leukocyte- endothelial interactions. Curr Opin Hematol. 2003;10(2):150-8.
