Menéndez VA, Fernández PMA, Svarch E, Scherle MC, Gil AM, Valdés CF, Machín GS, González OA, Serrano MJ

Language: Spanish
References: 0
Page: 55-64
PDF size: 216.80 Kb.

ABSTRACT

Introduction: Sickle cell disease is the most common genetic disease in the world. Neurological complications and their sequelae have been known almost since the first disease description. Stroke is the most frequent, especially in the first decade of life, perhaps as consequence of blood flow velocity of cerebral arteries is higher in children. The stroke risk could be monitorized by non-invasive technics such as transcranial Doppler ultrasound (TCD) of cerebral arteries and related with clinical and laboratorial parameters.
Objective: To characterize risk factors related to stroke in children with sickle cell disease.
Methods: A retrospective descriptive study was conducted in 104 patients diagnosed with sickle cell disease and treated in Pediatric Service of the Institute of Hematology and Immunology (IHI) in Havana from 2003 to 2011. The patients were divided into two groups: more symptomatic genotypes (HBSS and HbSβ0); and less symptomatic (HbSC and HbSβ+). Alterations of cerebral blood flow (CBF) were obtained TCD and were related to clinical and hematological elements.
Results: 48 % of patients were aged between 3-11 years. The most symptomatic group prevailed and the arteries in it showing greater speed FSC were right middle cerebral artery (MCA) (115.8 ± 42,5 cm/s) and left MCA (113.7 ± 40,8 cm/s). Also in this group prevailed significantly: painful vaso-occlusive crises (136.4 ± 20, 1.3 per 100 patients/year), admissions (90.9 ± 14.3, 0.9 per 100 patients/year), transfusions (72.7 ± 16.3, 0.7 per 100 patients/year), and acute chest syndrome (27.3 ± 8.2; 2.8 100 patients/year). There was an inverse correlation between hemoglobin and CBF velocity; and lactate dehydrogenase LDH had statistical significance 1211,8 ± 503.9 IU / L, establishing a significant difference between groups (p= 0.027).