Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children
Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children
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DOI：
10.1016/j.jpedsurg.2018.10.030
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发表时间：
2019-01-01
影响因子：
2.4
通讯作者：
Stanley, Charles A.
中科院分区：
文献类型：
--
作者：
Adzick, N. Scott;De Leon, Diva D.;Stanley, Charles A.
背景：先天性高胰岛素血症（HI）在新生儿和儿童中引起严重的低血糖。我们回顾了胰切除术治疗各种类型HI的经验。方法：从1998年到2018年，500例HI患者接受了胰腺切除术：局灶性HI 246例，弥漫性HI 202例，非典型HI 37例（局限性胰岛核扩大16例，贝克威氏综合征21例），胰岛素瘤15例。局灶性HI新生儿行部分胰腺切除术。治疗失败的弥漫性HI患者接受了近全（98%）胰腺切除术。非典型HI患者根据PET扫描和活检结果进行胰腺切除术。结果：局灶性HI绝大多数胰腺切除术发生率< 50%，多数为2% ~ 10%。97%的局灶性HI患者治愈。对于弥漫性疾病患者，31%为血糖正常，20%为血糖过高，49%需要治疗低血糖；糖尿病的发病率随着长期随访而增加。15例胰岛素瘤患者均治愈。结论：我们对局灶性HI患者的治疗方法可以区分局灶性HI和弥漫性HI，定位局灶灶病变，并允许几乎所有局灶性患者进行部分胰腺切除术并治愈。手术不能治愈弥漫性疾病，但可以帮助预防严重的低血糖和脑损伤。手术可以治愈胰岛素瘤和一些非典型HI病例。(C) 2018 Elsevier Inc版权所有
Background: Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI.Methods: From 1998 to 2018,500 patients with HI underwent pancreatectomy: 246 for focal HI, 202 for diffuse HI, 37 for atypical HI (16 for Localized Islet Nuclear Enlargement [LINE], 21 for Beckwith-Wiedemann Syndrome), and 15 for insulinoma. Focal HI neonates were treated with partial pancreatectomy. Patients with diffuse HI who failed medical management underwent near-total (98%) pancreatectomy. Atypical HI patients had pancreatectomies tailored to the PET scan and biopsy findings.Results: The vast majority of pancreatectomies for focal HI were < 50%, and many were 2%-10%. 97% of focal HI patients are cured. For diffuse disease patients, 31% were euglycemic, 20% were hyperglycemic, and 49% required treatment for hypoglycemia; the incidence of diabetes increased with long-term follow-up. All 15 insulinoma patients were cured.Conclusions: Our approach to patients with focal HI can distinguish focal from diffuse HI, localize focal lesions, and permit partial pancreatectomy with cure in almost all focal patients. Surgery does not cure diffuse disease but can help prevent severe hypoglycemia and brain damage. Surgery can be curative for insulinoma and for some cases of atypical HI. (C) 2018 Elsevier Inc All rights reserved.