非胰島素瘤胰原性低血糖症候群

Noninsulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS)

杜業豐(Ye-Fong Du) ； 歐弘毅(Horng-Yih Ou) ； 吳達仁(Ta-Jen Wu) ； 黃士銘(Shih-Ming Huang)

《內科學誌》 24卷3期 (2013/06) Pp. 181-188

https://doi.org/10.6314/JIMT.2013.24(3).03

摘要

非糖尿病成人低血糖當中最為人熟知的疾病為胰島素瘤（insulinoma）。非胰島素瘤造成的內源性高胰島素低血糖症在成人則較為少見，其中主要以餐後低血糖表現的稱為非胰島素瘤胰原性低血糖症候群（noninsulinoma pancreatogenous hypoglycemia syndrome; NIPHS）。由於72小時空腹試驗結果常為陰性，因此NIPHS常被延誤診斷，造成病人常在反覆多次發作神經性低血糖症狀後才被診斷。此類症候群的胰臟表現是胰島細胞的肥大，胰島細胞核大而濃染，並可能合併胰島的增生或增大。診斷常需要混合性食物試驗以誘發低血糖，以及選擇性動脈鈣輸注試驗診斷胰島素過度分泌的區域。目前並無標準的治療方式，一般選擇是胰臟亞全切除。術中使用快速胰島素監測，或合併術中選擇性動脈鈣輸注試驗，可能可使開刀範圍更完整。

關鍵字

低血糖 ； 非胰島素瘤胰原性低血糖症候群 ； 選擇性動脈鈣輸注試驗 ； 混合性食物試驗

並列摘要

The most common endogenous hyperinsulinemic hypoglycemia in non-diabetic adult is insulinoma. Non-insulinoma functional β cell disorders are less common in adult. Endogenous hyperinsulinemic hypoglycemia predominantly presented as postprandial hypoglycemia is termed noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS). NIPHS was easily delayed diagnosed despite repeated episodes of neuroglycopenia due to negative 72hour fast test. The pathologic features are islet cell hypertrophy, enlarged and hyperchromatic β cell nuclei, sometimes are coexistent with increased islets number or size. Diagnosis is often made when endogenous hyperinsulinemic hypoglycemia is provoked by mixed meal test. Selective intraarterial calcium stimulation test (IACS) often confirms the presence of endogenous hyperinsulinism and localizes the area of hyperfunctioning β-cells. There is no standard treatment for NIPHS to date. The most acceptable strategy is subtotal pancreatectomy guided by IACS. Application of rapid insulin assay with IACS performed intra-operatively may be more precise in determining the extent and completeness of subtotal pancreatectomy.

並列關鍵字

Hypoglycemia ； Noninsulinoma pancreatogenous hypoglycemia syndrome ； NIPHS ； Selective intraarterial calcium stimulation test ； IACS ； Mixed meal test