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Rosário Perry da Câmara1; Teresa Paínho1; Manuel Manita2; Carla Conceição3; Sara Batalha1 Raquel Maia1; Paula Kjollerstrom1; Rita Lopes da Silva4

1-Pediatric Hematology Unit, Department of Pediatrics, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE
2-Neurosonology Unit, Hospital de São José, Centro Hospitalar de Lisboa Central, EPE
3-Neuroradiology Department, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE
4-Pediatric Neurology Department, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE

Excellence in Pediatrics 8th edition, Londres, 9 de Dezembro de 2016. (comunicação oral)

Introduction: Cerebral vascular disease is the main cause of chronic morbidity in pediatric sickle cell disease (SCD) patients. Systematic screening using transcranial doppler (TCD) allows clinicians to stratify the risk of stroke and initiate effective primary prevention measures in high risk patients.
Purpose: Evaluation of Cerebral Vascular Disease in SCD Prevention and Surveillance Program implemented since 2008 in a pediatric tertiary care center in Portugal.
Methods: Inclusion of children and adolescents with SCD and at least one appointment in the Pediatric Hematology clinic from January 2013 to December 2015. All children over 2 y.o. were scheduled a Pediatric Neurology appointment and follow-up TCD according to guidelines. MRI was requested if the patient had atypical headaches, abnormal neurological exam or previous pathological TCD results. Analyzed variables were demographic data, current therapy, pediatric neurology assessment (neurological exam, headaches and learning difficulties), TCD and MRI results.
Results: A total of 110 patients were included, median age of 10, 47% were female. Eighty-one patients (74%) attended the Pediatric Neurology appointment, 10 (12%) had an abnormal neurological exam. In clinic 32 (40%) reported regular headaches and 40 (49%) had learning difficulties. Six patients (5.5%) had a stroke (5 ischemic and 1 hemorrhagic) – only 3 occurred after 2008, 2 of which diagnosed after findings of mild hemiparesis in patients with normal TCD. From the group of 97 patients older than 2 y.o., 95 (98%) had at least one TCD performed and 63 (65%) done in the past year. In the last TCD performed, only 1 (1%) had a time-averaged maximum mean (TAMM) velocity ≥ 200 cm/sec and 4 (4%) had velocities in the conditional range (170–199cm/sec). Thirty-six patients (33%) performed cranial MRI, 16 (44%) had vascular pathological findings - 5 ischemic strokes, 1 hemorrhagic stroke, 10 silent cerebral infarcts (5 of which isolated), 6 intracranial stenosis or moya-moya pattern and 3 showed other changes such as fused basal ganglia.
Discussion: The follow up of the patients is being done according to International Guidelines and the majority performs TCD regularly with a reduced number of pathological findings. In the last few years the clinical presentation of ischemic strokes was mild limb paresis diagnosed in the pediatric neurology appointment. This multidisciplinary prevention and surveillance program has contributed to improve health care in SCD patients.

Palavras Chave: sickle cell disease, cerebral vascular disease, prevention, transcranial doppler