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Abstract：
Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions. Our cohort included 123 patients (82 males, 41 females), with a median age of 27 (Interquartile Range 21-35 years). SCD related complications included acute chest syndrome (ACS) in 52.8%, splenic sequestration in 21.1%, right upper quadrant syndrome in 19.5%, more than > 6 VOC/year in 17.9%, and stroke in 13.8%. At the terminal admission, patients had cough, reduced O2 saturation, crepitation and fever in 24.4%, 49.6%, 53.6% and 68.3% respectively. Abnormal chest X-ray and chest CT scan were seen in 57.7%, and 76.4% respectively. Laboratory parameters showed a significant drop in hemoglobin (Hb) and platelet counts from baseline, with a significant rise in WBC, LDH and CRP from baseline (p < 0.05, Wilcoxon Signed Ranks test). All patients received antibiotics, whereas, 95.9% and 93.5% received simple blood transfusions, and exchange transfusions respectively, and 66.6% required non-invasive ventilation. Among the causes of death, ACS is seen in 32 (26%), sepsis in 49 (40%), and miscellaneous in 42 (34%). Sudden death was seen in 32 (26%) of patients. Male gender, with low HbF, rapid drop in Hb and platelet, and increased in WBC, LDH, ferritin, and CRP, correlated significantly with mortality in this cohort.
摘要：
镰状细胞病（SCD）是全球范围内的主要公共卫生负担，发病率和死亡率不断增加。该研究评估了与SCD患者死亡率相关的危险因素，2006年至2020年在阿曼的三家医院。分析包括临床表现，血液学，生物化学，和放射学参数，使用抗生素，血液和交换输血。我们的队列包括123例患者(82例男性，41名女性），年龄中位数为27岁（四分位距21-35岁）。SCD相关并发症包括急性胸部综合征（ACS）占52.8%，脾隔离率为21.1%,右上腹综合征占19.5%,17.9%超过>6VOC/年，中风占13.8%。在终点站入场时，病人咳嗽,降低O2饱和度，起病和发烧占24.4%，49.6%,分别为53.6%和68.3%。胸部X线和胸部CT扫描异常占57.7%，和76.4%。实验室参数显示血红蛋白（Hb）和血小板计数从基线显著下降，随着白细胞的大幅上升，来自基线的LDH和CRP(p<0.05,Wilcoxon签名等级检验)。所有患者都接受了抗生素治疗,然而,95.9%和93.5%接受简单输血，和交换输血，66.6%需要无创通气。在死亡原因中，ACS见于32例（26%），脓毒症49例（40%），和杂项42（34％）。32例（26%）患者出现猝死。男性,HbF低,血红蛋白和血小板快速下降，白细胞增加，LDH,铁蛋白,CRP,该队列中与死亡率显著相关.
