THE DIAGNOSIS AND MANAGEMENT OF CONGENITAL AND ADULT-ONSET HYPERINSULINISM (NESIDIOBLASTOSIS) - LITERATURE REVIEW

被引：2
作者：
Kowalewski, Adam M. [1 ]
Szylberg, Lukasz [1 ]
Kasperska, Anna [1 ]
Marszalek, Andrzej [1 ,2 ,3 ]
机构：
[1] Nicolaus Copernicus Univ Torun, Coll Med Bydgoszcz, Chair & Dept Clin Pathomorphol, Boruckiego 16, PL-85790 Bydgoszcz, Poland
[2] Poznan Univ Med Sci, Chair & Dept Oncol Pathol & Prophylact, Poznan, Poland
[3] Greater Poland Canc Ctr, Dept Oncol Pathol, Poznan, Poland
来源：
POLISH JOURNAL OF PATHOLOGY | 2017年 / 68卷 / 02期
关键词：
congenital; adult; hyperinsulinism; nesidioblastosis; diagnosis; management; DIFFUSE FORMS; FOCAL FORM; HYPOGLYCEMIA; GENETICS; ABCC8; TERM; LOCALIZATION; INSULINOMA; DISORDERS; MUTATION;
D O I：
10.5114/PJP.2017.69684
中图分类号：
R36 [病理学];
学科分类号：
100104 ;
摘要：
Congenital and adult-onset hyperinsulinism (CHI) must be taken under consideration in the differential diagnosis of hypoglycaemia symptoms with endogenous hyperinsulinism, especially in cases in which there was failure to find an insulinoma. Histological examination is necessary for a definitive diagnosis. CHI is a disorder with three histopathological variants: focal CHI, diffuse CHI, and atypical CHI. These variants are clinically indistinguishable. According to published statistics, 0.5 to 5% of nesidioblastosis cases occur in adults. Clinical manifestation ranges from mildly symptomatic up to life-threatening hypoglycaemia. Early diagnosis and treatment are important in young and very young patients because early treatment accounts for favourable mental outcomes.
引用
收藏
页码：97 / 101
页数：5
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