Hereditary Blood Diseases Center, King Fahad Hospital, Hofuf, Saudi Arabia.
Ann Thorac Med. 2007 Oct;2(4):158-62. doi: 10.4103/1817-1737.36550.
Genetic studies suggest that the sickle cell mutation has arisen on at least four separate occasions in Africa and as a fifth independent mutation in the Eastern Province of Saudi Arabia or India. The pathophysiology of sickle cell disease (SCD) is essentially similar in these different areas although the frequency and severity of complications may vary between areas. The aim of this study was to evaluate the prevalence and outcome of acute chest syndrome (ACS) in SCD patients from Eastern province of Saudi Arabia in comparison with patients with African haplotypes.
This was a retrospective study involving 317 SCD patients who were two years or older, admitted to King Fahad Hospital Hofuf between January-May 2003 for different etiologies. Twenty six patients presented with different causes of ACS; 11 patients presented with different pathologies other than ACS, but had past history of ACS; 280 patients presented with different pathologies and never presented with ACS. Clinical features, CBC, Hb-electrophoresis, G6PD activity, cultures, chest X-ray, arterial oxygen saturation, blood transfusion rates and outcome were studied. Univariate and multiple regression analysis were carried out to evaluate influence on ACS. Comparison between SCD patients with ACS from this study and from Eastern province of Saudi Arabia to patients with African haplotypes were carried out, using data reported in the literature.
During the period of this study, 37 patients with new or previous episodes of ACS were studied (accounting for 11.67% of admitted SCD patients). Most of the patients with ACS had only one episode, but five patients (13.51%) had had episodes or more. One patient died giving an in-hospital mortality rate of 1/26 (3.85%). Comparison of recurrence of ACS and mortality between SCD patients in Eastern province of Saudi Arabia to that of patients with African haplotype showed that recurrence is significantly lower (P<0.025) in patients from Eastern province compared to patients with African haplotype, mortality also is lower but not statistically significant.
Acute chest syndrome in SCD patients in Eastern province of Saudi Arabia is relatively uncommon, but causes significant morbidity and mortality. Its prevalence and recurrence is low if compared to that of patients with African haplotypes.
遗传研究表明，镰状细胞突变至少在非洲发生了四次，在沙特阿拉伯东部省份或印度发生了第五次独立突变。镰状细胞病（SCD）的病理生理学在这些不同地区基本相似，尽管并发症的频率和严重程度可能因地区而异。本研究旨在评估沙特阿拉伯东部省份 SCD 患者急性胸部综合征（ACS）的患病率和结局，并与具有非洲单倍型的患者进行比较。
这是一项回顾性研究，涉及 317 名年龄在 2 岁及以上的 SCD 患者，他们于 2003 年 1 月至 5 月期间因不同病因入住哈夫夫的法赫德国王医院。26 例患者因不同原因出现 ACS；11 例患者因 ACS 以外的不同病理表现就诊，但有 ACS 病史；280 例患者因不同病理表现就诊且从未出现 ACS。研究了临床特征、CBC、Hb 电泳、G6PD 活性、培养物、胸部 X 射线、动脉血氧饱和度、输血率和结局。进行了单变量和多变量回归分析，以评估对 ACS 的影响。对本研究中患有 ACS 的 SCD 患者与沙特阿拉伯东部省份和具有非洲单倍型的患者进行了比较，并使用文献中报道的数据。
在本研究期间，研究了 37 例新发或既往 ACS 患者（占入院 SCD 患者的 11.67%）。大多数 ACS 患者只有一次发作，但 5 例（13.51%）有一次或多次发作。1 例患者死亡，院内死亡率为 1/26（3.85%）。沙特阿拉伯东部省份 SCD 患者 ACS 复发和死亡率与非洲单倍型患者的比较表明，与非洲单倍型患者相比，东部省份患者的复发率明显较低（P<0.025），死亡率也较低，但无统计学意义。
沙特阿拉伯东部省份 SCD 患者的急性胸部综合征相对少见，但会导致显著的发病率和死亡率。与具有非洲单倍型的患者相比，其患病率和复发率较低。
