Introduction: Sickle-cell anaemia is the severe homozygotic form of drepanocytosis, a genetic disorder that often occurs among black people and which is characterised by the production of haemoglobin S, chronic hemolytic anaemia and tissue ischaemia due to alterations in blood flow. A quarter of the patients presented neurological manifestations; 8-10% of children will have a stroke. AIM. To analyse the cases of stroke in children with sickle-cell anaemia in our centre.
Patients and methods: We conducted a retrospective descriptive study of children with sickle-cell anaemia and stroke.
Results: Five patients (two Dominicans and three Guineans) with sickle-cell anaemia and stroke; one patient suffered two episodes of stroke. The mean age was 27 months. Five of the episodes were ischaemic infarctions. Stroke was the initial form of presentation of drepanocytosis on three occasions. Two of the strokes occurred within a context of pneumococcal meningitis. Four of the patients had previously reported fever. The initial clinical picture was hemiparesis in four cases. Mean haemoglobin on diagnosing the stroke was 6.5 g/dL. Transcranial ultrasound imaging revealed alterations in three patients and, in all the patients, magnetic resonance imaging revealed lesions, which were bilateral in half the cases. Following the stroke, a hypertransfusion regimen protocol was established and only one patient presented a new stroke. This same patient went on to develop moya-moya disease and was submitted to an indirect revascularisation; the patient progressed well, without presenting any new ischaemic events.
Conclusions: Drepanocytosis is a disease that is emerging in our setting as a result of immigration. It should be suspected in cases of paediatric strokes associated to anaemia, above all in black children under the age of five who were not submitted to neonatal screening.
Title: Ictus en pacientes pediatricos con anemia falciforme.
Introduccion. La anemia falciforme es la forma homocigota, grave, de drepanocitosis, un trastorno genetico, frecuente en raza negra, caracterizado por la produccion de hemoglobina S, anemia hemolitica cronica e isquemia tisular por alteracion del flujo sanguineo. Una cuarta parte de los pacientes presenta manifestaciones neurologicas; el 8-10% de los niños sufrira un ictus. Objetivo. Analizar los casos de ictus en niños con anemia falciforme en nuestro centro. Pacientes y metodos. Estudio descriptivo retrospectivo de niños con anemia falciforme e ictus. Resultados. Se recogieron cinco pacientes (dos dominicanos y tres guineanos) con anemia falciforme e ictus; un paciente sufrio dos episodios ictales. La edad media fue de 27 meses. Cinco episodios fueron infartos isquemicos. El ictus fue la forma de inicio de la drepanocitosis en tres ocasiones. Dos de los ictus ocurrieron en un contexto de meningitis neumococica. En cuatro pacientes hubo fiebre previa. La clinica inicial fue hemiparesia en cuatro casos. La hemoglobina media al diagnostico de ictus fue de 6,5 g/dL. En tres pacientes se hallaron alteraciones en la ecografia transcraneal y, en todos los pacientes, lesiones en la resonancia magnetica, que en la mitad eran bilaterales. Tras el ictus se inicio un protocolo de regimen hipertransfusional, y solo un paciente presento un nuevo ictus, que desarrollo un sindrome moya-moya y fue sometido a una revascularizacion indirecta, con buena evolucion, sin presentar nuevos eventos isquemicos posteriores. Conclusiones. La drepanocitosis es una enfermedad emergente en nuestro medio debido a la inmigracion. Debe sospecharse en ictus pediatricos asociados a anemia, sobre todo en menores de 5 años de raza negra no sometidos a cribado neonatal.