Alkindi Said Y, Pathare Anil, Al Zadjali Shoaib, Panjwani Vinodhkumar, Wasim Fauzia, Khan Hammad, Chopra Pradeep, Krishnamoorthy Rajagopal, Alkindi Salam
Ministry of Health, Muscat, Oman & McMaster University, Canada.
Sultan Qaboos University Hospital, Muscat, OMAN.
Mediterr J Hematol Infect Dis. 2015 Nov 1;7(1):e2015060. doi: 10.4084/mjhid.2015.060. eCollection 2015.
We explored the potential relationship between steady state serum bilirubin levels and the incidence of cholelithiasis in the context of UGT1A1 gene A(TA)nTAA promoter polymorphism in Omani sickle cell anemia (SCA) patients, homozygotes for African (Benin and Bantu) and Arab-Indian β(S) haplotypes, but sharing the same microgeographical environment and comparable life style factors.
136 SCA patients were retrospectively studied in whom imaging data including abdominal CT scan, MRI or Ultrasonography were routinely available. Available data on the mean steady state hematological/biochemical parameters (n=136), β(s) haplotypes(n=136), α globin gene status (n=105) and UGT1A1 genotypes (n=133) were reviewed from the respective medical records.
The mean serum total bilirubin level was significantly higher in the homozygous UGT1A1(AT)7 group as compared to UGT1A1(AT)6 group. Thus, not cholelithiasis but total serum bilirubin was influenced by UGT1A1 polymorphism in this SCA cohort.
As observed in other population groups, the UGT1A1 (AT)7 homozygosity was significantly associated with raised serum total bilirubin level, but the prevalence of gallstones in the Omani SCA patients was not associated with α thalassaemia, UGT1A1 polymorphism, or β(s) haplotypes.
我们在阿曼镰状细胞贫血(SCA)患者中,探讨了UGT1A1基因A(TA)nTAA启动子多态性背景下稳态血清胆红素水平与胆石症发病率之间的潜在关系。这些患者为非洲(贝宁和班图)和阿拉伯 - 印度β(S)单倍型的纯合子,但处于相同的微观地理环境且生活方式因素相近。
对136例SCA患者进行回顾性研究,这些患者常规可获得包括腹部CT扫描、MRI或超声检查在内的影像数据。从各自的病历中查阅了平均稳态血液学/生化参数(n = 136)、β(s)单倍型(n = 136)、α珠蛋白基因状态(n = 105)和UGT1A1基因型(n = 133)的可用数据。
与UGT1A1(AT)6组相比,UGT1A1(AT)7纯合组的平均血清总胆红素水平显著更高。因此,在这个SCA队列中,受UGT1A1多态性影响的不是胆石症,而是血清总胆红素。
正如在其他人群中观察到的那样,UGT1A1(AT)7纯合性与血清总胆红素水平升高显著相关,但阿曼SCA患者的胆结石患病率与α地中海贫血、UGT1A1多态性或β(s)单倍型无关。