Lozano-Melendez Eduardo, Aguilar-Soto Mercedes, Graniel-Palafox Luis Eugenio, Ceceña-Martínez Laura Elena, Valdez-Ortiz Rafael, Solis-Jimenez Fabio
Faculty of Medicine and Nutrition of the Juarez University of the State of Durango, Durango, Durango, Mexico.
Department of Internal Medicine, ABC Medical Center, Mexico City, Mexico.
Case Rep Endocrinol. 2019 Feb 14;2019:7640384. doi: 10.1155/2019/7640384. eCollection 2019.
Nesidioblastosis is a rare cause of hyperinsulinemic hypoglycemia in adults. The diagnosis is further complicated in patients with kidney failure, since impaired renal function can cause hypoglycemia by itself and diagnostic criteria for this clinical scenario have not been developed yet.
We present the case report of a 36-year-old patient with end stage chronic kidney disease who presented to the emergency department because of hypoglycemia. However, the patient's hypoglycemia did not respond well to medical treatment; the diagnosis of hyperinsulinemic hypoglycemia was made due to the presence of inappropriately high levels of insulin, proinsulin, and C-peptide during an episode of hypoglycemia. Imaging studies were performed without any conclusive findings; so selective intra-arterial pancreatic stimulation with hepatic venous sampling (SACTS) was done. Based on the results of this study the patient was referred for subtotal pancreatectomy. Classic criteria for the diagnosis of insulinoma with SACTS required a 2-fold increase in insulin levels but newer criteria suggest thresholds that are useful in the differential diagnosis of insulinoma and nesidioblastosis. In our patient, the former criteria were positive; however, the new criteria were not compatible with insulinoma but with nesidioblastosis, which was the final histopathological diagnosis.
This seems to be the first case report of a patient with end stage chronic kidney disease and nesidioblastosis, as well as the first case of hyperinsulinemic hypoglycemia in the context of kidney failure diagnosed by SACTS. We consider this method to be very useful in patients with renal impairment because peripancreatic insulin levels do not depend on the renal function.
胰岛细胞增殖症是成人高胰岛素血症性低血糖症的罕见病因。对于肾衰竭患者，诊断会更加复杂，因为肾功能受损本身就可导致低血糖，且针对这种临床情况的诊断标准尚未制定。
我们报告一例36岁终末期慢性肾病患者，因低血糖就诊于急诊科。然而，该患者的低血糖对药物治疗反应不佳；低血糖发作期间，胰岛素、胰岛素原和C肽水平异常升高，据此诊断为高胰岛素血症性低血糖症。影像学检查未发现任何确诊结果；因此进行了选择性肝静脉采血的胰腺动脉内刺激试验（SACTS）。根据该研究结果，患者被转诊接受胰腺次全切除术。SACTS诊断胰岛素瘤的经典标准要求胰岛素水平升高2倍，但新的标准提出了有助于胰岛素瘤和胰岛细胞增殖症鉴别诊断的阈值。在我们的患者中，前一标准呈阳性；然而，新标准不符合胰岛素瘤，而是符合胰岛细胞增殖症，这也是最终的组织病理学诊断结果。
这似乎是首例终末期慢性肾病合并胰岛细胞增殖症的病例报告，也是首例在肾衰竭情况下通过SACTS诊断的高胰岛素血症性低血糖症病例。我们认为这种方法对肾功能受损患者非常有用，因为胰腺周围胰岛素水平不依赖于肾功能。
