RESUMO
BACKGROUND: Sickle cell disease is a common genetic disease in Saudi Arabia; it is an autosomal recessive disorder characterized by production of abnormal hemoglobin S and is associated with high morbidity and mortality. Acute splenic sequestration is a life-threatening complication for this disease. Prophylactic splenectomy is the only effective strategy for preventing future life-threatening episodes. AIM: The aim of this study was to study hospital records for all children aged 2 to 12 year old with Sickle cell disease who underwent splenectomy in Tabuk in Saudi Arabia. METHODS: Records of 24 children (13 males, 11 females) who underwent splenectomy in surgery department of King Salman North West Armed Hospital, Tabuk, Saudi Arabia between 2008 and 2015 were reviewed retrospectively and analyzed for age, sex, indications for splenectomy, surgical technique, preoperative and postoperative length of stay, operative and postoperative complications, acute chest syndrome, painful crises, blood transfusion and fever (preoperative and postoperative). RESULTS: We stressed on the information about the details of operation, the frequency of blood transfusion, fever, acute chest syndrome and painful crisis before and after operation. CONCLUSION: Here we found that blood transfusion frequency decreased after splenectomy.
RESUMO
La anemia de células falciformes o drepanocítica es una enfermedad común en la edad pediátrica, con alta mortalidad en menores de 5 años de edad. Cuando un niño hereda esta condición de sus padres quienes son portadores, los glóbulos rojos sufren un cambio de forma o falciformación, alterando el flujo de sangre y provocando enfermedad, dolor y daño de órganos. Es necesario el despistaje universal a todos los recién nacidos, para identificar los afectados e iniciar tempranamente su cuidado y educación a los padres para prevenir ytratar las complicaciones, permitiéndoles tener una mejor calidad de vida. Se presentan unas guías y opciones terapéuticas para laprevención y el manejo de las complicaciones
Sickle cell disease (SCD) is a common condition in childhood with a high mortality rate, especially under 5 years of age. When children inherit SCD from their carrier parents, the red blood cells form an abnormal sickled shape. As a result, blood does not flow well, causingdisease, pain and organ damage. Universal newborn screening is necessary to identify and treat early those affected babies, thus preventing complications and allowing them to have a healthier life. Here is a set of guidelines and treatment options to prevent and manage sickle cell related complications
