To investigate the clinical features and prognosis of biliary atresia (BA) with normal or minimally elevated gamma-glutamyl transpeptidase (GGT).

The clinical data of patients with BA in our hospital between 2012 and 2017 were retrospectively studied. The patients were divided into a low-GGT group (GGT ≤ 300 IU/L) and a high-GGT group (GGT > 300 IU/L) according to the preoperative GGT level. The perioperative clinical parameters, the postoperative jaundice clearance within 6 months, and the 2-year native liver survival were compared among the groups.

A total of 1,998 children were included in this study, namely, 496 in the low-GGT group and 1,502 in the high-GGT group. The ages and weights at the surgery in the low-GGT group were significantly lower than those in the high-GGT group (64.71 ± 21.35 vs. 68.64 ± 22.42 days, P = 0.001; 4.67 ± 1.03 vs. 4.89 ± 0.98 kg, P < 0.001). The levels of serum ALP, ALT, and AST in the low-GGT group were significantly higher than those in the high-GGT group before and 2 weeks after the surgery (ALP: 647.52 ± 244.10 vs. 594.14 ± 228.33 U/L, P < 0.001; ALT: 119.62 ± 97.14 vs. 96.01 ± 66.28 U/L, P < 0.001; AST: 218.00 ± 173.82 vs. 160.71 ± 96.32 U/L; P < 0.001). The INR of the low-GGT group was higher than that of the high-GGT group (1.05 ± 0.34 vs. 0.98 ± 0.20, P < 0.001), while FIB was lower than the high-GGT group (2.54 ± 0.67 vs. 2.73 ± 1.44 g/L; P = 0.006). The decreasing amplitude of TB and DB within 2 weeks after surgery in the low-GGT group was smaller than those in the high-GGT group (TB: 51.62 ± 71.22 vs. 61.67 ± 53.99 μmol/L, P = 0.003; DB: 33.22 ± 35.57 vs. 40.20 ± 35.93 μmol/L, P < 0.001). The jaundice clearance rate in the low-GGT group was significantly lower than that in the high-GGT group at 1, 3, and 6 months after surgery (17.70 vs. 26.05%; 35.17 vs. 48.58%; 38.62 vs. 54.64%, P < 0.001). In addition, the 2-year native liver survival rate in the low-GGT group was significantly lower than that of the high-GGT group (52.5 vs. 66.3%, P < 0.001 HR 1.80, 95% CI 1.38–2.33).

Compared to patients with high GGT, patients with normal or minimally elevated pre-operative GGT in BA were found to have poorer pre-operative liver function parameters, and post-operatively had lower jaundice clearance rates and worse 2-year native liver survival. This suggests a lower GGT at presentation in biliary atresia could be a sign of more severe liver injury.

探讨γ-谷氨酰转肽酶（GGT）正常或轻微升高的胆道闭锁（BA）的临床特征和预后。

回顾性研究我院2012-2017年BA患者的临床资料。根据术前GGT水平将患者分为低GGT组（GGT≤300 IU/L）和高GGT组（GGT>300 IU/L）。比较各组围手术期临床参数、术后6个月内黄疸清除率和2年自然肝生存率。

本研究共纳入 1,998 名儿童，即低 GGT 组 496 名，高 GGT 组 1,502 名儿童。低 GGT 组的手术年龄和体重显着低于高 GGT 组（64.71 ± 21.35 vs. 68.64 ± 22.42 天，磷= 0.001; 4.67 ± 1.03 对 4.89 ± 0.98 公斤，磷< 0.001)。术前和术后2周，低GGT组血清ALP、ALT、AST水平显着高于高GGT组（ALP：647.52±244.10 vs. 594.14±228.33 U/L，磷< 0.001; ALT：119.62 ± 97.14 与 96.01 ± 66.28 U/L，磷< 0.001; AST：218.00 ± 173.82 与 160.71 ± 96.32 U/L；磷< 0.001)。低 GGT 组的 INR 高于高 GGT 组（1.05 ± 0.34 vs. 0.98 ± 0.20，磷< 0.001)，而 FIB 低于高 GGT 组 (2.54 ± 0.67 vs. 2.73 ± 1.44 g/L;磷= 0.006)。低 GGT 组术后 2 周内 TB 和 DB 下降幅度小于高 GGT 组（TB：51.62 ± 71.22 vs. 61.67 ± 53.99 μmol/L，磷= 0.003; DB：33.22 ± 35.57 与 40.20 ± 35.93 μmol/L，磷< 0.001)。术后1、3、6个月，低GGT组黄疸清除率明显低于高GGT组（17.70 vs. 26.05%；35.17 vs. 48.58%；38.62 vs. 54.64%，磷< 0.001)。此外，低 GGT 组的 2 年天然肝存活率显着低于高 GGT 组（52.5% vs. 66.3%，磷< 0.001 HR 1.80，95% CI 1.38–2.33）。

与 GGT 高的患者相比，BA 中术前 GGT 正常或轻度升高的患者术前肝功能参数较差，术后黄疸清除率较低，2 年天然肝生存率较差。这表明胆道闭锁时 GGT 较低可能是更严重肝损伤的迹象。