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婴儿先天性高胰岛素血症再次胰腺切除术的发生率、预测因素和结局:16 年的三级中心经验。

Incidence, predictors and outcomes of redo pancreatectomy in infants with congenital hyperinsulinism: a 16-year tertiary center experience.

机构信息

Department of Pediatric Surgery, King Fahad Medical City, PO Box 59046, Riyadh, 11525, Kingdom of Saudi Arabia.

Department of Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, 11211, Kingdom of Saudi Arabia.

出版信息

Pediatr Surg Int. 2023 Apr 20;39(1):183. doi: 10.1007/s00383-023-05470-6.

Abstract

PURPOSE

Recurrent severe hypoglycemic attacks often persist even after performing pancreatectomy for medically unresponsive congenital hyperinsulinism (CHI). In this study, we present our experience with redo pancreatectomy for CHI.

METHODS

We reviewed all children who underwent pancreatectomy for CHI between January 2005 and April 2021 in our center. A comparison was made between patients whose hypoglycemia was controlled after primary pancreatectomy and patients who required reoperation.

RESULTS

A total of 58 patients underwent pancreatectomy for CHI. Refractory hypoglycemia after pancreatectomy occurred in 10 patients (17%), who subsequently underwent redo pancreatectomy. All patients who required redo pancreatectomy had positive family history of CHI (p = 0.0031). Median extent of initial pancreatectomy was lesser in the redo group with borderline level of statistical significance (95% vs. 98%, p = 0.0561). Aggressive pancreatectomy at the initial surgery significantly (p = 0.0279) decreased the risk for the need to redo pancreatectomy; OR 0.793 (95% CI 0.645-0.975). Incidence of diabetes was significantly higher in the redo group (40% vs. 9%, p = 0.033).

CONCLUSION

Pancreatectomy with 98% extent of resection for diffuse CHI, especially with positive family history of CHI, is warranted to decrease the chance of reoperation for persistent severe hypoglycemia.

摘要

目的

对于药物治疗无反应性先天性高胰岛素血症(CHI),即使进行胰腺切除术,反复发作的严重低血糖仍经常持续存在。本研究介绍了我们在 CHI 患者中进行再次胰腺切除术的经验。

方法

我们回顾了 2005 年 1 月至 2021 年 4 月期间在我们中心接受胰腺切除术治疗 CHI 的所有儿童患者。比较了初次胰腺切除术后低血糖得到控制的患者和需要再次手术的患者。

结果

共有 58 例患者因 CHI 接受胰腺切除术。10 例(17%)患者在胰腺切除术后出现难治性低血糖,随后行再次胰腺切除术。所有需要再次胰腺切除术的患者均有 CHI 的阳性家族史(p=0.0031)。再次手术组的初始胰腺切除术范围中位数较小,但无统计学意义(95% vs. 98%,p=0.0561)。初次手术时进行积极的胰腺切除术显著降低了再次胰腺切除术的必要性(p=0.0279);OR 0.793(95%CI 0.645-0.975)。再次手术组的糖尿病发生率显著更高(40% vs. 9%,p=0.033)。

结论

对于弥漫性 CHI,特别是有 CHI 阳性家族史的患者,进行 98%范围的胰腺切除术,以降低持续性严重低血糖再次手术的机会。

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