Abstract：
BACKGROUND: Total pancreatectomy with islet autotransplantation (TPIAT) is a potentially curative treatment for patients with chronic pancreatitis (CP) refractory to medical and endoscopic therapies. Patients often receive the initial follow-up medical care at the surgery-performing center, but then may follow up closer to where they live. We sought to describe the characteristics and outcomes of pediatric patients who underwent TPIAT at a national surgical referral center and were subsequently followed at our regional subspecialty center, the Children\'s Hospital Colorado.
METHODS: We performed a retrospective analysis of baseline and outcomes data for the 10 pediatric patients who underwent TPIAT from 2007 to 2020 and received follow-up care at our institution.
RESULTS: All patients had a diagnosis of CP, and nine of 10 patients had an identified underlying genetic risk factor. Insulin usage was common immediately following TPIAT, but at 1 year of follow-up, five of nine patients (55.6%) were insulin-independent and nine of nine had an HbA1c below 6.5%. For the four patients on insulin 1 year after TPIAT, total daily insulin dose ranged from 0.06 to 0.71 units/kg/day. All patients who underwent mixed meal tolerance testing had a robust peak C-peptide response at 1 year. There were significant improvements in nausea, school/work absences, narcotic dependence, and pancreas-related hospital admissions 1 year after TPIAT.
CONCLUSIONS: Patients followed at our center had long-term improvements with low-insulin usage, detectable C-peptide, and improved pancreatitis-related outcomes after TPIAT. Pediatric patients who undergo TPIAT can be successfully co-managed in conjunction with the original surgery-performing center.
METHODS: We performed a retrospective analysis of baseline and outcomes data for the 10 pediatric patients who underwent TPIAT from 2007 to 2020 and received follow-up care at our institution.
RESULTS: All patients had a diagnosis of CP, and nine of 10 patients had an identified underlying genetic risk factor. Insulin usage was common immediately following TPIAT, but at 1 year of follow-up, five of nine patients (55.6%) were insulin-independent and nine of nine had an HbA1c below 6.5%. For the four patients on insulin 1 year after TPIAT, total daily insulin dose ranged from 0.06 to 0.71 units/kg/day. All patients who underwent mixed meal tolerance testing had a robust peak C-peptide response at 1 year. There were significant improvements in nausea, school/work absences, narcotic dependence, and pancreas-related hospital admissions 1 year after TPIAT.
CONCLUSIONS: Patients followed at our center had long-term improvements with low-insulin usage, detectable C-peptide, and improved pancreatitis-related outcomes after TPIAT. Pediatric patients who undergo TPIAT can be successfully co-managed in conjunction with the original surgery-performing center.
摘要：
背景：胰岛自体移植全胰腺切除术（TPIAT）对于药物和内镜治疗难治性慢性胰腺炎（CP）患者是一种潜在的治愈性治疗方法。患者通常在手术中心接受初步的后续医疗护理，但可能会跟进更接近他们居住的地方。我们试图描述在国家外科转诊中心接受TPIAT的儿科患者的特征和结果，随后在我们的地区亚专科中心进行随访。科罗拉多州儿童医院。
方法：我们对2007年至2020年接受TPIAT并在我们机构接受随访护理的10例儿科患者的基线和结果数据进行了回顾性分析。
结果：所有患者均诊断为CP，10例患者中有9例具有确定的潜在遗传风险因素。TPIAT后立即使用胰岛素是常见的，但在1年的随访中，9例患者中有5例(55.6%)为胰岛素非依赖性患者,9例患者中有9例HbA1c低于6.5%.对于TPIAT后1年接受胰岛素治疗的四名患者，每日胰岛素总剂量为0.06~0.71单位/kg/天.所有接受混合餐耐受性测试的患者在1年时都有强烈的C肽峰值反应。恶心有显著改善，学校/工作缺勤，麻醉依赖，TPIAT后1年与胰腺相关的住院。
结论：在我们中心随访的患者在低胰岛素使用率下长期改善，可检测的C肽，TPIAT术后胰腺炎相关结局改善。接受TPIAT的儿科患者可以与原始手术中心成功地共同管理。
方法：我们对2007年至2020年接受TPIAT并在我们机构接受随访护理的10例儿科患者的基线和结果数据进行了回顾性分析。
结果：所有患者均诊断为CP，10例患者中有9例具有确定的潜在遗传风险因素。TPIAT后立即使用胰岛素是常见的，但在1年的随访中，9例患者中有5例(55.6%)为胰岛素非依赖性患者,9例患者中有9例HbA1c低于6.5%.对于TPIAT后1年接受胰岛素治疗的四名患者，每日胰岛素总剂量为0.06~0.71单位/kg/天.所有接受混合餐耐受性测试的患者在1年时都有强烈的C肽峰值反应。恶心有显著改善，学校/工作缺勤，麻醉依赖，TPIAT后1年与胰腺相关的住院。
结论：在我们中心随访的患者在低胰岛素使用率下长期改善，可检测的C肽，TPIAT术后胰腺炎相关结局改善。接受TPIAT的儿科患者可以与原始手术中心成功地共同管理。
