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Crisis vaso-oclusivas, una complicación frecuente de la drepanocitosis Vaso-occlusive crisis, a frequent complication of drepanocytosis

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Author(s): M. González Sánchez | J. Mayordomo Colunga | E. Larrea Tamayo | S. González Muñiz | A. Sariego Jamardo

Journal: Boletín de Pediatría
ISSN 0214-2597

Volume: 50;
Issue: 214;
Start page: 281;
Date: 2010;
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ABSTRACT
Introducción: Existen diferentes formas clínicas de drepanocitosis, siendo la homocigosis la más grave. Es especialmente frecuente en la raza negra. Los actuales movimientos migratorios hacen que su manejo sea cada vez más frecuente en nuestro ámbito.  Presentamos dos casos de crisis vasooclusivas en niños afectos de drepanocitosis homocigota. Caso clínico 1: Paciente con drepanocitosis homocigota, que acude por cuadro febril, asociando dolor lumbar y en ambas rodillas, que no responde a analgesia oral. En la analítica, se objetivan anemia, leucocitosis con neutrofilia y elevación de los niveles de proteína C reactiva. Se instaura hidratación intravenosa abundante y tratamiento intravenoso con cefotaxima y morfina, con mejoría rápida. Caso clínico 2: Paciente con drepanocitosis homocigota, alérgico a cefalosporinas, consulta por fiebre y dolor óseo de 24 horas de evolución. Se realiza analítica, observándose anemia, leucocitosis y elevación de los reactantes de fase aguda. Se instaura cloruro mórfico y se cubre con clindamicina intravenosa. Ante la persistencia de la fiebre tras 48 horas, se solicitan pruebas de imagen, que resultan compatibles con síndrome torácico agudo. Se añade azitromicina oral, con buena evolución posterior. Conclusiones: La drepanocitosis es un problema emergente en nuestro medio, que obliga a conocer sus complicaciones, ya que su pronóstico depende de un diagnóstico y tratamiento precoz. Las complicaciones agudas más frecuentes son las crisis de dolor vaso-oclusivo óseo, que suelen requerir tratamiento analgésico agresivo. Otra complicación frecuente es el síndrome torácico agudo causante, en ocasiones, de hipoxemia refractaria. Introduction: There are different clinical forms of drepanocytosis, the most severe being homozygosis. It is especially frequent in the black race. The current migratory movements have caused its management to be increasingly more frequent in our setting. We present two cases of vaso-occlusive crises in children suffering from homozygous drepanocytosis. Clinical case 1: Patient with homozygous drepanocytosis who came due to fever symptoms, with associated lumbar pain and pain in both knees, who did not respond to oral analgesics. In the laboratory analyses, anemia, leukocytosis with neutrophilia and elevation of the C-reactive protein levels were observed. Abundant intravenous hydration and intravenous treatment with cefotaxime and morphine were established, with rapid improvement. Clinical case 2: Patient with homozygous drepanocytosis, allergic to cephalosporins, consulted due to fever and bone pain of 24 hours evolution. Laboratory analyses were performed, observing anemia, leukocytosis and elevation of the acute phase reactants. Morphine sulfate was established and covered with intravenous clindamycin. Due to the persistence of fever after 48 hours, imaging tests were requested. They were consistent with the acute thoracic syndrome. Oral erythromycin was added, with good subsequent evolution. Conclusions: Drepanocytosis is an emerging problem in our setting, which makes it necessary to know its complications, since its prognosis depends on early diagnosis and treatment. The most frequent acute complications are bone vasoocclusive pain crisis, which generally requires aggressive analgesic treatment. Another frequent complication is the acute forensic syndrome, sometimes causing refractory hypoxemia.
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