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吉尔伯特综合征中轻度溶血的一氧化碳呼气试验评估

Carbon monoxide breath test assessment of mild hemolysis in Gilbert's syndrome.

作者信息

Kang Ling-Ling, Ma Yong-Jian, Zhang Hou-De

机构信息

Department of Gastroenterology, Nanshan Hospital, Guangdong Medical University.

Guangdong Breath Test Engineering and Technology Research Center.

出版信息

Medicine (Baltimore). 2020 Feb;99(7):e19109. doi: 10.1097/MD.0000000000019109.

Abstract

BACKGROUND

Mild hemolysis is difficult to determinate by traditional methods, and its role in Gilbert's syndrome (GS) is unclear. The main aims were to inspect the erythrocyte (RBC) survival in GS by using Levitt's carbon monoxide (CO) breath test and to assess its contribution to unconjugated hyperbilirubinemia.

METHODS

Fifty subjects with GS and 1 with type-II Crigler-Najjar syndrome (CN2) received RBC lifespan measurement with Levitt's CO breath test. Mean RBC lifespan was compared with normal referral value. Correlations of serum total bilirubin (TB) with RBC lifespan, blood panel data, demographic factors, and uridine diphosphate glucuronosyltransferase (UGT1A1) mutation load were calculated by Spearman analysis. Susceptibility factors for mild hemolysis were analyzed by multivariate regression analysis.

RESULTS

The mean RBC lifespan of the GS subjects was significantly shorter than the normal reference value (95.4 ± 28.9 days vs 126 days; t = -7.504, P < .01), with 30.0% below the lower limit of the normal reference range (75 days). The RBC lifespan of the participant with CN2 was 82 days. Serum TB correlated positively with UGT1A1 mutation load (γ = 0.281, P = .048), hemoglobin (γ = .359, P = .010) and hematocrit (γ = 0.365, P = .010), but negatively with RBC lifespan (γ = -0.336, P = .017). No significant susceptibility factors for mild hemolysis were found.

CONCLUSIONS

The results indicate that mild hemolysis indeed, exists in a portion of patients with GS and might serve as an important contributor to unconjugated hyperbilirubinemia in addition to UGT1A1 polymorphism. Further studies on the mechanism and the potential risks in various medical treatments might be wanted.

摘要

背景

传统方法难以确定轻度溶血,其在吉尔伯特综合征(GS)中的作用尚不清楚。主要目的是通过使用莱维特一氧化碳(CO)呼气试验检测GS患者的红细胞(RBC)生存期,并评估其对非结合性高胆红素血症的影响。

方法

50例GS患者和1例II型克里格勒-纳贾尔综合征(CN2)患者接受了莱维特CO呼气试验测量红细胞寿命。将平均红细胞寿命与正常参考值进行比较。通过Spearman分析计算血清总胆红素(TB)与红细胞寿命、血液检查数据、人口统计学因素和尿苷二磷酸葡萄糖醛酸转移酶(UGT1A1)突变负荷之间的相关性。通过多因素回归分析分析轻度溶血的易感因素。

结果

GS患者的平均红细胞寿命显著短于正常参考值(95.4±28.9天对126天;t=-7.504,P<.01),30.0%低于正常参考范围下限(75天)。CN2患者的红细胞寿命为82天。血清TB与UGT1A1突变负荷(γ=0.281,P=.048)、血红蛋白(γ=.359,P=.010)和血细胞比容(γ=0.365,P=.010)呈正相关,但与红细胞寿命呈负相关(γ=-0.336,P=.017)。未发现轻度溶血的显著易感因素。

结论

结果表明,部分GS患者确实存在轻度溶血,除UGT1A1多态性外,可能是导致非结合性高胆红素血症的重要因素。可能需要对各种医学治疗中的机制和潜在风险进行进一步研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd3b/7035016/b369454fe972/medi-99-e19109-g002.jpg

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