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胰腺源性高胰岛素血症性低血糖症的病理基础——三例报告

Pathological aspects underlying pancreatogenous hyperinsulinemic hypoglycemia--report of three cases.

作者信息

Mihai Bogdan Mircea, Lăcătuşu Cristina Mihaela, Arhire Lidia Iuliana, Graur Mariana, Scripcariu Viorel, AniŢei Maria Gabriela, Radu Iulian, Ferariu Dan, Danciu Mihai

机构信息

Discipline of Diabetes, Nutrition and Metabolic Diseases, First Medical Department, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy; Clinic of Diabetes, Nutrition and Metabolic Diseases, "Sf. Spiridon" Clinical Emergency Hospital, Iassy, Romania;

出版信息

Rom J Morphol Embryol. 2015;56(1):251-6.

PMID:25826512
Abstract

Pancreatogenous hyperinsulinemic hypoglycemia (PHH) is a rare disorder determined by an abnormally high secretion of insulin in the pancreas, in the absence of other medical or pharmacological factors. Either β-cell tumors (insulinomas) or β-cell hyperplasia (nesidioblastosis) can determine this pathology. Most publications on insulinomas or nesidioblastosis approached these subjects from a clinical point of view. This paper aims to analyze pathological aspects underlying pancreatogenous hyperinsulinemic hypoglycemia. We present two cases of insulinomas with unusual pancreatic localization and size, one of them showing amyloid deposits in the stroma. In both cases, immunohistochemistry confirmed the clinical and imagistic supposition. The third reported case refers to a 57-year-old patient with nesidioblastosis with isolated disposition of endocrine cells and areas of focal organization, both morphological aspects being extremely rare in adults. Although clinical and laboratory data are usually identical in the two forms of PHH, histopathological and immunohistochemical diagnosis is essential in differentiating insulinomas from nesidioblastosis, as the surgical management is different: enucleation for insulinomas and total or subtotal pancreatectomy for nesidioblastosis.

摘要

胰腺源性高胰岛素血症性低血糖症(PHH)是一种罕见的疾病,由胰腺中胰岛素分泌异常增多引起,且不存在其他医学或药理学因素。β细胞肿瘤(胰岛素瘤)或β细胞增生(胰岛细胞增殖症)均可导致这种病理状态。大多数关于胰岛素瘤或胰岛细胞增殖症的出版物都是从临床角度探讨这些主题的。本文旨在分析胰腺源性高胰岛素血症性低血糖症的病理特征。我们报告了两例胰岛素瘤,其在胰腺中的定位和大小均不寻常,其中一例在间质中可见淀粉样沉积物。在这两例病例中,免疫组织化学均证实了临床和影像学的推测。第三例报告病例是一名57岁患有胰岛细胞增殖症的患者,其内分泌细胞呈孤立分布且存在局灶性组织区域,这两种形态学特征在成年人中极为罕见。尽管在PHH的两种形式中临床和实验室数据通常相同,但组织病理学和免疫组织化学诊断对于区分胰岛素瘤和胰岛细胞增殖症至关重要,因为手术治疗方式不同:胰岛素瘤采用摘除术,而胰岛细胞增殖症则采用全胰腺或次全胰腺切除术。

相似文献

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Pathological aspects underlying pancreatogenous hyperinsulinemic hypoglycemia--report of three cases.胰腺源性高胰岛素血症性低血糖症的病理基础——三例报告
Rom J Morphol Embryol. 2015;56(1):251-6.
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Surgical treatment of persistent hyperinsulinemic hypoglycemia (PHH) (insulinoma and nesidioblastosis).持续性高胰岛素血症性低血糖症(PHH)(胰岛素瘤和胰岛细胞增殖症)的外科治疗
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Adult onset nesidioblastosis treated by subtotal pancreatectomy.成人隐匿性自身免疫性糖尿病行胰腺次全切除术治疗。
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Selective Arterial Calcium Stimulation With Hepatic Venous Sampling Differentiates Insulinoma From Nesidioblastosis.选择性动脉钙刺激试验联合肝静脉采血可鉴别胰岛素瘤与胰岛细胞增殖症。
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Synchronous Nesidioblastosis, Endocrine Microadenoma, and Intraductal Papillary Mucinous Neoplasia in a Man Presenting With Hyperinsulinemic Hypoglycemia.一名患有高胰岛素血症性低血糖症的男性患者同时存在胰岛细胞增殖症、内分泌微腺瘤和导管内乳头状黏液性肿瘤。
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Metastatic insulinoma in an adult patient with underlying nesidioblastosis.一名患有潜在胰岛细胞增殖症的成年患者的转移性胰岛素瘤。
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Diffuse nesidioblastosis as a cause of hyperinsulinemic hypoglycemia in adults: a diagnostic and therapeutic challenge.成人弥漫性胰岛细胞增殖症作为高胰岛素血症性低血糖的病因:诊断与治疗挑战
Surgery. 2007 Feb;141(2):179-84; discussion 185-6. doi: 10.1016/j.surg.2006.04.015.

引用本文的文献

1
Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia.弥漫性成人起病的胰岛细胞增殖症/非胰岛素瘤性胰源性低血糖综合征(NIPHS):高胰岛素血症性低血糖罕见病因的文献综述
Biomedicines. 2023 Jun 16;11(6):1732. doi: 10.3390/biomedicines11061732.