Valentim Cleomar Ana de Souza, Arimura Vivien Suemi, Pompei Isabella De Melo, Bonvechio Denis, Silva Marcio Shimabuku, Turrini Fabio José, Jorgino Mariana Soares Dalla Mariga, Valentim Andre Silva
Postgraduate Program in Health Sciences, Faculty of Medicine of Jundiaí (FMJ), Jundiaí, SP, Brazil.
Faculty of Medicine of Jundiaí (FMJ), Jundiaí, SP, Brazil.
Am J Case Rep. 2025 Apr 18;26:e945453. doi: 10.12659/AJCR.945453.
BACKGROUND Nesidioblastosis is an uncommon cause of hypoglycemia and is part of a group of diseases known as non-insulinoma pancreatogenic hypoglycemia syndrome (NIHPS). The objective of this report is to provide a comprehensive discussion on the diagnostic and therapeutic management of a case of nesidioblastosis, a rare and potentially fatal condition. CASE REPORT A 42-year-old non-diabetic man with a history of coronary artery disease and systemic arterial hypertension underwent extensive diagnostic investigation because he had been hospitalized several times over a period of approximately 2 years with a clinical history compatible with persistent refractory hypoglycemia. Despite laboratory and imaging tests, the underlying cause of the hypoglycemia remained unclear. Comprehensive investigation included computed tomography (CT), magnetic resonance imaging (MRI), upper endoscopy, PET/CT, and selective pancreatic arteriography. Persistent hypoglycemia associated with high insulin levels led to the suspicion of nesidioblastosis. After exhausting all clinical therapeutic options and after multidisciplinary discussion considering risks and benefits, we decided to perform total pancreatectomy and splenectomy. Anatomopathological and immunohistochemical examination confirmed the diagnosis of nesidioblastosis. CONCLUSIONS The diagnosis of nesidioblastosis is complex and requires a multidisciplinary approach. The decision to perform a total pancreatectomy was essential to control severe hypoglycemia and improve the patient's quality of life. This case report describes the diagnostic and therapeutic management of persistent endogenous hyperinsulinemic hypoglycemia and highlights the importance of diagnostic accuracy and early therapeutic intervention.
胰岛细胞增殖症是低血糖症的一种罕见病因，属于非胰岛素瘤胰源性低血糖综合征（NIHPS）这一疾病组的一部分。本报告的目的是对一例胰岛细胞增殖症病例的诊断和治疗管理进行全面讨论，该病症罕见且可能致命。病例报告：一名42岁的非糖尿病男性，有冠状动脉疾病和系统性动脉高血压病史，因在大约2年的时间里因与持续性难治性低血糖相符的临床病史而多次住院，接受了广泛的诊断性检查。尽管进行了实验室和影像学检查，但低血糖的根本原因仍不清楚。全面检查包括计算机断层扫描（CT）、磁共振成像（MRI）、上消化道内镜检查、正电子发射断层扫描/计算机断层扫描（PET/CT）和选择性胰腺动脉造影。与高胰岛素水平相关的持续性低血糖导致怀疑为胰岛细胞增殖症。在穷尽所有临床治疗选择并经过考虑风险和益处的多学科讨论后，我们决定实施全胰切除术和脾切除术。解剖病理学和免疫组织化学检查证实了胰岛细胞增殖症的诊断。结论：胰岛细胞增殖症的诊断复杂，需要多学科方法。实施全胰切除术的决定对于控制严重低血糖和改善患者生活质量至关重要。本病例报告描述了持续性内源性高胰岛素血症性低血糖的诊断和治疗管理，并强调了诊断准确性和早期治疗干预的重要性。
