Incidence, predictors and outcomes of redo pancreatectomy in infants with congenital hyperinsulinism: a 16-year tertiary center experience.

PURPOSE: Recurrent severe hypoglycemic attacks often persist even after performing pancreatectomy for medically unresponsive congenital hyperinsulinism (CHI). In this study, we present our experience with redo pancreatectomy for CHI. METHODS: We reviewed all children who underwent pancreatectomy for CHI between January 2005 and April 2021 in our center. A comparison was made between patients whose hypoglycemia was controlled after primary pancreatectomy and patients who required reoperation. RESULTS: A total of 58 patients underwent pancreatectomy for CHI. Refractory hypoglycemia after pancreatectomy occurred in 10 patients (17%), who subsequently underwent redo pancreatectomy. All patients who required redo pancreatectomy had positive family history of CHI (p = 0.0031). Median extent of initial pancreatectomy was lesser in the redo group with borderline level of statistical significance (95% vs. 98%, p = 0.0561). Aggressive pancreatectomy at the initial surgery significantly (p = 0.0279) decreased the risk for the need to redo pancreatectomy; OR 0.793 (95% CI 0.645-0.975). Incidence of diabetes was significantly higher in the redo group (40% vs. 9%, p = 0.033). CONCLUSION: Pancreatectomy with 98% extent of resection for diffuse CHI, especially with positive family history of CHI, is warranted to decrease the chance of reoperation for persistent severe hypoglycemia.

Diazoxide during pregnancy and lactation: drug levels in maternal serum, cord blood, breast milk, and infant serum: a case report.

Safety information on diazoxide for pregnant and lactating women with hypoglycemia is limited. In this case report, we assessed diazoxide concentrations in maternal and infant blood, cord blood, and breast milk. We described a 30-year-old pregnant woman diagnosed with hypoglycemia due to nesidioblastosis at 4 months of age. Before becoming pregnant, she was treated with oral diazoxide (75-375 mg). All medications were discontinued after she was discovered to be pregnant. During gestational week 25, diazoxide treatment was resumed at 150-175 mg daily for repeated hypoglycemic episodes. Diazoxide administration was continued in combination with diet treatment until delivery. Glucose levels were well controlled. During gestational week 40, a male infant weighing 3069 g was delivered via spontaneous vaginal delivery with no pregnancy or neonatal complications. Diazoxide concentrations detected in maternal serum at 2.5-11.6 h after oral treatment ranged from 12.4 to 32.7 µg/mL. In cord blood, the diazoxide concentration was 18.5 µg/mL at 7.2 h after the last dose. During lactation, no hypoglycemia or hyperglycemia was observed. The approximate calculated ratio of diazoxide in breast milk and maternal serum was 0.09. The calculated daily infant dose was 0.47 mg/kg/day. The relative infant dose via breast milk ranged from 3.1% to 5.9%. Diazoxide transferred from maternal blood to the fetus across the placenta. It also transferred into breast milk, but there were no harmful effects on the infant.

[Mature teratoma of the anterior mediastinum with a predominance of the pancreatic component and signs of focal nesidioblastosis]. / Zrelaya teratoma perednego sredosteniya s preobladaniem pankreaticheskogo komponenta i priznakami fokal'nogo nezidioblastoza.

A case of a mature anterior mediastinal teratoma with a predominance of the pancreatic component (80% of the tumor) and signs of nesidioblastosis in a 46-year-old woman is presented. During histological examination, all components of the pancreatic parenchyma were determined - acini with interlobular and intralobular ducts, endocrine cells and islets of Langerhans of various sizes and shapes, as well as islet-duct complexes scattered in the acinar tissue. In addition to pancreatic tissue, cartilage, bronchial respiratory epithelium, small intestine tissue, hair follicles, and sebaceous glands were found in the neoplasm. Immunohistochemical examination revealed signs of focal nesidioblastosis. In the islets, insulin-positive ß-cells (80.0% of the volume of the islets), as well as endocrine cells expressing glucagon and somatostatin (10.0% of the volume of the islets) were determined. By 2020, only 4 such cases have been published in the English-language literature.

Utility of 68 Ga-DOTA-Exendin-4 positron emission tomography-computed tomography imaging in distinguishing between insulinoma and nesidioblastosis in patients with confirmed endogenous hyperinsulinaemic hypoglycaemia.

BACKGROUND: Because management is very different, it is important to differentiate between small focal insulinomas and diffuse pancreatic dysplasia (nesidioblastosis) in patients with confirmed endogenous hyperinsulinaemic hypoglycaemia (EHH). Most insulinomas highly express glucagon-like peptide-1 receptors enabling positron emission tomography-computed tomography imaging with its radiolabelled analogue; 68 Ga-DOTA-Exendin-4 (Exendin). AIM: To determine: (i) the utility of Exendin in EHH patients in a clinical setting; and (ii) whether the degree of Exendin uptake differentiates non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS) from post-gastric bypass hypoglycaemia (PGBH). METHODS: This retrospective study reviewed the clinical, biochemistry and prior imaging findings in confirmed EHH patients referred for Exendin. Accuracy of Exendin was based on surgical findings and treatment outcomes. Finally, average Exendin uptake (SUVmax) of five PGBH studies was compared with the SUVmax of a key NIPHS case report. RESULTS: Twenty of 25 consecutive patients had confirmed EHH. Exendin located insulinomas in eight of nine patients enabling successful surgical excision with rapid and durable cure. Exendin correctly identified diffuse nesidioblastosis in two of three cases requiring partial pancreatectomy for hypoglycaemia control. All three relapsed within 1.7 years with one needing completion pancreatectomy. Establishing the cause in the remainder relied on other investigations, clinical correlation and response to empirical treatment. Finally, Exendin SUVmax could not distinguish between NIPHS and PGBH. CONCLUSION: In EHH patients, Exendin accurately identifies the site of insulinoma and thereby differentiates it from nesidioblastosis but negative findings should not be ignored. Exendin is unlikely to differentiate between normal pancreatic uptake, NIPHS and PGBH.

Hypoglycemia Worsened by Glucose Administration: A Case of Hypoglycemia Years After Gastric Surgery.

BACKGROUND: Noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is a rare syndrome characterized by postprandial hypoglycemia with neuroglycopenic symptoms occurring 1 to 3 h after a meal. Diagnosis can be elusive, as the vast majority of patients have normal fasting blood glucose levels, and onset of hypoglycemic episodes can be a late complication of gastric surgery. CASE REPORT: We report the case of a 45-year-old woman presenting to the Emergency Department (ED) with new-onset seizures and hypoglycemia worsened by glucose administration. Surgical history is pertinent for a Roux-en-Y gastric bypass approximately 10 years prior to presentation. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Although rare, it is important for emergency physicians to be vigilant of this disease process as a traditional treatment approach for hypoglycemia may be detrimental. Although cases of NIPHS have been documented in literature, its presence in emergency medicine-specific literature is seemingly nonexistent. Noninvasive imaging techniques will be normal, and diagnosis is dependent on awareness of this disease entity coupled with a detailed history.

Critical role for GLP-1 in symptomatic post-bariatric hypoglycaemia.

AIMS/HYPOTHESIS: Post-bariatric hypoglycaemia (PBH) is a rare, but severe, metabolic disorder arising months to years after bariatric surgery. It is characterised by symptomatic postprandial hypoglycaemia, with inappropriately elevated insulin concentrations. The relative contribution of exaggerated incretin hormone signalling to dysregulated insulin secretion and symptomatic hypoglycaemia is a subject of ongoing inquiry. This study was designed to test the hypothesis that PBH and associated symptoms are primarily mediated by glucagon-like peptide-1 (GLP-1). METHODS: We conducted a double-blinded crossover study wherein eight participants with confirmed PBH were assigned in random order to intravenous infusion of the GLP-1 receptor (GLP-1r) antagonist. Exendin (9-39) (Ex-9), or placebo during an OGTT on two separate days at the Stanford University Clinical and Translational Research Unit. Metabolic, symptomatic and pharmacokinetic variables were evaluated. Results were compared with a cohort of BMI- and glucose-matched non-surgical controls (NSCs). RESULTS: Infusion of Ex-9 decreased the time to peak glucose and rate of glucose decline during OGTT, and raised the postprandial nadir by over 70%, normalising it relative to NSCs and preventing hypoglycaemia in all PBH participants. Insulin AUC and secretion rate decreased by 57% and 71% respectively, and peak postprandial insulin was normalised relative to NSCs. Autonomic and neuroglycopenic symptoms were significantly reduced during Ex-9 infusion. CONCLUSIONS/INTERPRETATION: GLP-1r blockade prevented hypoglycaemia in 100% of individuals, normalised beta cell function and reversed neuroglycopenic symptoms, supporting the conclusion that GLP-1 plays a primary role in mediating hyperinsulinaemic hypoglycaemia in PBH. Competitive antagonism at the GLP-1r merits consideration as a therapeutic strategy. TRIAL REGISTRATION: ClinicalTrials.gov NCT02550145.

The treatment of hyperinsulinemic hypoglycaemia in adults: an update.

BACKGROUND: Treatment of hyperinsulinemic hypoglycaemia (HH) is challenging due to the rarity of this condition and the difficulty of differential diagnosis. The aim of this article is to give an overview of the recent literature on the management of adult HH. METHODS: A search for reviews, original articles, original case reports between 1995 and 2016 in PubMed using the following keywords: hyperinsulinemic hypoglycaemia, insulinoma, nesidioblastosis, gastric bypass, autoimmune hypoglycaemia, hyperinsulinism, treatment was performed. RESULTS: One hundred and forty articles were selected and analysed focusing on the most recent treatments of HH. CONCLUSIONS: New approaches to treatment of HH are available including mini-invasive surgical techniques and alternative local-regional ablative therapy for benign insulinoma and everolimus for malignant insulinoma. A correct differential diagnosis is of paramount importance to avoid unnecessary surgical operations and to implement the appropriate treatment mainly in the uncommon forms of HH, such as nesidioblastosis and autoimmune hypoglycaemia.

The diagnosis and management of congenital and adult-onset hyperinsulinism (nesidioblastosis) - literature review.

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.

Role of (68)Ga somatostatin receptor PET/CT in the detection of endogenous hyperinsulinaemic focus: an explorative study.

PURPOSE: To explore the role of (68)Ga-DOTATATE/DOTATOC PET/CT (SR PET/CT) in patients with suspicion of or histopathologically proven pancreatogenic hyperinsulinaemic hypoglycaemia. METHODS: We included 13 patients with histopathologically proven or a high clinical suspicion of pancreatogenic hyperinsulinaemia. All the patients underwent a SR PET/CT scan. The results were correlated with histopathological findings. Normalization of blood glucose levels after resection of the pancreatic lesion, as well as a cytological and/or pathological diagnosis of insulinoma, was considered the diagnostic gold standard for insulinoma. The diagnosis of nesidioblastosis was based on exclusion of an insulinoma and conclusive pathological examination of a segment of the pancreas. Malignant insulinoma was defined as the presence of locoregional or distant metastases. RESULTS: Based on histopathology, 13 patients were found to have pancreatic hyperinsulinaemia: two patients had malignant insulinoma, eight had nonmetastasized insulinoma, and three had nesidioblastosis. SR PET was positive in 11 of the 13 patients (84.6 %) with a final diagnosis of endogenous pancreatic hypoglycaemia. Histopathological staining confirmed 16 foci of hyperinsulinism (insulin positivity). SR PET detected 14 of the 16 lesions, resulting in a sensitivity of 87 %. One intrapancreatic spleen was falsely diagnosed as insulinoma focus on SR PET, resulting in positive predictive value of 93.3 %. Immunohistochemical staining of somatostatin receptor (SSR) subtype 2a was available in ten specimens: two nesidioblastosis, and seven benign and one malignant insulinoma. Eight out of the ten specimens (80 %) stained strongly to moderately positive. Seven of the eight SSR2a-positive lesions were picked up on SR PET. Based on the results of SR PET/CT, nine patients achieved complete remission of the hypoglycaemic events during follow-up. CONCLUSION: This explorative study suggests that SR PET in combination with CT may play a significant role in the detection and management of patients with pancreatogenic hyperinsulinaemic hypoglycaemia. A large proportion of insulinomas express SSR2a, and a larger study is needed to fully assess the diagnostic accuracy of SR PET in patients with insulinoma and nesidioblastosis compared with current localizing studies used in clinical practice.

Post-Gastric Bypass Hypoglycemia.

Obesity is a major public health problem worldwide. Obesity-related illnesses, such as coronary heart disease, type 2 diabetes, hypertension, dyslipidemia, stroke, sleep apnea, and several forms of cancer (endometrial, breast, and colon), contribute to a significant number of deaths in the USA. Bariatric surgery, including the Roux-en-Y gastric bypass (RYGB) procedure, has demonstrated significant improvements in obesity and obesity-related co-morbidities and is becoming more popular as the number of obese individuals rises. Despite the reported benefits of bariatric surgery, there are potential complications that physicians need to be aware of as the number of patients undergoing these procedures continues to increase. One challenging and potentially life-threatening complication that to date is not well understood is post-RYGB surgery hypoglycemia (PGBH). In this review, we will present the definition, historical perspective, diagnostic approach, currently available treatment options, and anecdotal assessment and treatment algorithm for this disorder.

Clinical characteristics and long-term outcome of Taiwanese children with congenital hyperinsulinism.

BACKGROUND/PURPOSE: Congenital hyperinsulinism (CHI) is a rare condition causing severe hypoglycemia in neonates and infants due to dysregulation of insulin secretion. This study aimed to review 20 years' experience in the management of Taiwanese children with CHI. METHODS: Between 1990 and 2010, children diagnosed with CHI and followed up at the Pediatric Endocrine Clinic of the National Taiwan University Hospital were enrolled. Their medical records were thoroughly reviewed. RESULTS: In total, 13 patients (8 boys and 5 girls) were enrolled, including six patients with onset of hypoglycemia within 1 month of age and seven patients at 4.0 ± 2.1 months of age. The birth weight standard deviation scores of these two age groups were 4.6 ± 1.8 and 1.4 ± 1.3 standard deviation score, respectively (p < 0.01). Initial intravenous glucose infusion at rates of 22.9 ± 5.3 mg/kg/min and 13.4 ± 5.6 mg/kg/min, respectively, were mandatory to maintain euglycemia in these two groups (p < 0.05). All received pancreatectomy after failure of initial medical treatment. Twelve patients were followed up for a period of 2.5-19.8 years. Eight of them remained euglycemic without any medication and three patients developed diabetes mellitus. Seven of the nine patients who underwent intelligence evaluation had normal mental outcomes. Mental retardation of two patients was too severe to be evaluated. All four patients with mental retardation had a delay in the maintenance of euglycemia, and three of them also had seizure disorder. CONCLUSION: The age at onset of hypoglycemia reflects the severity of CHI. Early diagnosis and appropriate treatment are important for favorable mental outcomes.

Endogenous hyperinsulinemic hypoglycemia syndrome: surgical treatment.

BACKGROUND: The endogenous hyperinsulinemic hypoglicemia syndrome (EHHS) can be caused by an insulinoma, or less frequently, by nesidioblastosis in the pediatric population, also known as non insulinoma pancreatic hypoglycemic syndrome (NIPHS) in adults. The aim of this paper is to show the strategy for the surgical treatment of ehhs. MATERIAL AND METHODS: A total of 19 patients with a final diagnosis of insulinoma or NIPHS who were treated surgically from january 2007 until june 2012 were included. We describe the clinical presentation and preoperative work-up. Emphasis is placed on the surgical technique, complications and long-term follow-up. RESULTS: All patients had a positive fasting plasma glucose test. Preoperative localization of the lesions was possible in 89.4% of cases. The most frequent surgery was distal pancreatectomy with spleen preservation (9 cases). Three patients with insulinoma presented with synchronous metastases, which were treated with simultaneous surgery. There was no perioperative mortality and morbidity was 52.6%. Histological analysis revealed that 13 patients (68.4%) had benign insulinoma, 3 malignant insulinoma with liver metastases and 3 with a final diagnosis of SHPNI. Median follow-up was 20 months. All patients diagnosed with benign insulinoma or NIPHS had symptom resolution. CONCLUSION: The surgical treatment of EHHS achieves excellent long-term results in the control of hypoglucemic symptoms.

Nesidioblastosis in Pregnancy: Navigating the Diagnostic and Therapeutic Challenges of a Rare Condition.

Nesidioblastosis, a non-neoplastic proliferation of the pancreatic islet cells of Langerhans, is a rare cause of endogenous hyperinsulinemic hypoglycemia. Although initially thought of as a congenital disease affecting pediatric patients, it is well known nowadays to affect adults as well. In addition, it is increasingly documented as a rare sequela of bariatric surgeries. Management options include medical and surgical therapies, with little known about the beneficial effects of both. Nesidioblastosis affecting pregnant patients is even rarer, with scarce literature known about the optimal treatment. Herein, we present a 22-year-old woman in the 20th week of gestation who experienced symptomatic episodes of severe life-threatening hypoglycemia. The management of this case required a multidisciplinary team approach to navigate the complexities of diagnosis and treatment. The complexity of this case was further heightened by the differential diagnosis, including conditions like insulinoma. The scarcity of literature on nesidioblastosis in pregnancy further complicated the case, underscoring the need for more research and case studies to guide clinical practice.

Noninsulinoma Pancreatogenous Hypoglycemia Syndrome in a Patient With 1p36 Deletion Syndrome.

The 1p36 deletion syndrome involves a phenotypic presentation that includes central nervous system, cardiac, and craniofacial anomalies. We report the case of a 21-year-old female patient with 1p36 deletion syndrome who was found to have noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) after hospitalization for persistent falls. On admission, vital signs were normal and physical examination revealed a thin, nonverbal patient. During hospitalization and prolonged fasting (14-18 hours), she persistently developed hypoglycemia (serum glucose nadir 57 mg/dL [3.2 mmol/L] [70-100 mg/dL; 3.9-5.6 mmol/L]). Subjective symptoms of hypoglycemia were not confirmed due to patient's cognitive impairment. Hypoglycemic events continued despite feeding and dextrose-containing fluids. Further workup included a critical sample that revealed a serum glucose 59 mg/dL (3.3 mmol/L), insulin 20.6 µIU/mL (123.6 pmol/L [5-15 µIU/mL; 30.0-90 pmol/L]), proinsulin 33 pmol/L (3.6-22 pmol/L), C-peptide 1.74 ng/mL (0.58 nmol/L [0.8-3.85 ng/mL; 0.27-1.28 nmol/L]) and beta-hydroxybutyrate < 1.04 mg/dL (< 0.10 mmol/L; [< 4.2 mg/dL; < 0.4 mmol/L]). Insulin antibodies were negative. After confirmed insulin-mediated hypoglycemia, imaging studies followed. Pancreatic protocol abdominal computed tomography (CT), Ga-68 DOTATATE PET/CT scan, and endoscopic ultrasound found no pancreatic mass. Selective arterial calcium stimulation test showed a two-fold increase in insulin levels in 3/3 catheterized pancreatic territories. The patient started octreotide injections with resolution of hypoglycemia and was discharged on monthly lanreotide injections. To our knowledge, this is the first case reported of noninsulinoma pancreatogenous hypoglycemia in a patient with 1p36 deletion syndrome.

Spontaneous Hypoglycemia in a Non-diabetic Patient: A Diagnostic and Therapeutic Conundrum.

Hypoglycaemia is a medical emergency requiring an immediate intervention to prevent neuroglycopenic symptoms such as confusion, seizures, and coma. While evaluating for the cause of hypoglycemia, after excluding common causes like insulin use or sepsis, other causes involving endogenous hypoglycemia need to be evaluated. A cause to be considered is nesidioblastosis. This rare entity is also known as non-insulinoma pancreatogenous hypoglycemia syndrome. There have been instances where this disorder has been mistaken as insulinoma due to the characteristics shared by the two. Here, we present a case of a non-diabetic male experiencing symptoms of giddiness and palpitations for the past two years who had been extensively evaluated to rule out insulinoma and was diagnosed with nesidioblastosis.

Refractory Hyperinsulinemic Hypoglycemia as a Complication of Roux-en-Y Gastric Bypass Surgery.

This case involves a 45-year-old woman with severe obesity who underwent Roux-en-Y gastric bypass (RYGB) surgery. After one year, she developed daily episodes of severe hypoglycemia, presenting with symptoms of palpitations, diaphoresis, and syncope. The patient was diagnosed with endogenous hyperinsulinemic hypoglycemia, a condition characterized by abnormally high insulin levels leading to low blood glucose, commonly associated with insulinoma. In rare instances, this can be due to nesidioblastosis, an overgrowth of pancreatic beta cells, which is more prevalent in individuals who have undergone bariatric surgery. Diagnostic evaluations included blood tests, abdominal computed tomography and magnetic resonance imaging, continuous glucose monitoring, and hepatic venous sampling to exclude insulinoma. This report details the diagnosis and unsuccessful treatment of endogenous hyperinsulinemic hypoglycemia following RYGB surgery. Interventions included dietary modifications (small, frequent, low-carbohydrate meals), medical management with acarbose 100 mg three times daily, diazoxide 150 mg three times daily, verapamil 40 mg twice daily, and surgical reversal of the RYGB. Ultimately, a percutaneous gastrostomy tube was placed for 24-hour continuous parenteral feeding. Despite these extensive treatment efforts, the patient continues to experience frequent hypoglycemic episodes four years after the bariatric procedure.

Trends in Presentation and Management of Endogenous Hyperinsulinaemic Hypoglycaemia Over the Last Three Decades at a Tertiary Care Centre (1992-2022).

Introduction: Endogenous hyperinsulinaemic hypoglycaemia (EHH) is characterized by inappropriate insulin secretion from pancreatic beta cells despite low blood glucose concentrations. We aimed to evaluate the secular changes in presentation and management of EHH due to insulinoma/non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS) at our centre. Methods: This was a single-centre ambispective study (2014-2022). The clinical, biochemical, hormonal and radiological parameters (n = 63) collected as part of this study were compared with our earlier studies (1992-2005, n = 31; and 2006-2013, n = 35) and with other centres across the world. Results: A total of 63 patients (39 males) with a preoperative diagnosis of EHH (insulinoma, n = 58; and NIPHS, n = 5) and a mean age of 40.7 years were studied. The mean lag time from the onset of symptoms to diagnosis decreased from 4.6 years during the first study period to 1.9 years during this study period. However, the majority presented with fasting hypoglycaemia of 98.4%, and both fasting and postprandial hypoglycaemia of 32%. Exclusive postprandial hypoglycaemia was present in 1.7% of insulinoma. A histopathological diagnosis of insulinoma was made in 52 patients and nesidioblastosis in two patients. Intraoperative ultrasonography (IOUS) and intraoperative palpation (IOP) yielded 100% sensitivity, while endoscopic ultrasonography (EUS) and 68Ga-DOTA-Exendin-4 positron emission tomography/computed tomography (PET/CT) yielded sensitivity of 86% and 85%, respectively, for localizing insulinoma. Resolution of hypoglycaemia was noted in 53 of 57 (93%) patients who underwent surgery with a preoperative diagnosis of insulinoma. Conclusion: We observed a trend towards earlier diagnosis of EHH, increased patient numbers and availability of nuclear imaging techniques for preoperative localization in the last decade compared to earlier.

Use of Translational Science, Continuous Glucose Monitoring in the Primary Care Setting for Management of Nesidioblastosis: A Case Report and Literature Review.

Nesidioblastosis is a term used to describe histologic changes in the pancreatic cell, which are defined by beta cell hypertrophy and the formation of ductoinsular complexes. It is a disease previously most extensively identified in neonates and is a rare cause of endogenous hypoglycemia in the adult population. However, with increasing numbers of gastric bypass surgeries for the management of obesity in recent years, there has been a growing number of populations with post-gastric bypass surgery-related nesidioblastosis. Here, we will present a case of a 60-year-old female with a history of Roux-en-Y gastric bypass (RYGB) surgery who initially presented with loss of consciousness and episodes of suspected hypoglycemia. Insulinoma was ruled out, supporting the diagnosis of adult onset RYGB-associated nesidioblastosis.This article was previously presented as a poster presentation at the 2023 Research Symposium, International Conference on Health Disparities, on September 8, 2023.

Diagnosis of pancreatic focal nesidioblastosis assisted by dual­nuclide tracer positron emission tomography/computed tomography: A case report.

Nesidioblastosis is a rare cause of hyperinsulinemic hypoglycemia in adults and its clinical features are similar to those of insulinoma with recurrent hypoglycemic attacks. The present study reports the case of a 48-year-old man who visited the Affiliated Hospital of Zunyi Medical University (Zunyi, China) with a 5-year history of recurrent hypoglycemic symptoms such as dizziness and palpitations. Abdominal magnetic resonance imaging (MRI) showed a mass of ~1.2x1.0 cm in the head of the pancreas, which was suspected to be an insulinoma. For confirmation, the patient underwent both fluorine-18-fluorodeoxyglucose (18F-FDG) and gallium-68-labeled 1,4,7,10-tetraazacyclododecane-1,4,7,10-teraacetic acid-d-Phel-Tyr3-Thr8-OC (68Ga-DOTATATE) positron emission tomography/computed tomography (PET/CT), which showed a moderately increased uptake of 18F-FDG but no uptake of 68Ga-DOTATATE in the corresponding lesion. The patient subsequently underwent surgery to remove the lesion, which was pathologically confirmed as a pancreatic nesidioblastosis. This case showed that nesidioblastosis should be considered a differential diagnosis for insulinoma and that dual nuclear tracer PET/CT imaging is helpful for differentiating between the two. If conventional imaging techniques such as ultrasound, CT and MRI cannot identify the cause of hypoglycemia in future cases, dual-nuclide tracer PET/CT imaging should be considered.

Nesidioblastosis. A case of hyperplasia of the islets of Langerhans in the adult.

INTRODUCTION: Nesidioblastosis is a rare disease caused by hyperplasia of pancreatic islets, developing a state of hypoglycemia due to an increase in the insulin production. It is the leading cause of hyperinsulinic hypoglycemia in childhood, whereas in adults it only represents the 0.5-5% of cases. The pathogenesis is still unknown. We have studied several genetic mutations associated with dependent potassium channel of ATP present in the beta cells of the pancreas, as well as in patients underwent bariatric surgery because of the metabolic changes involved. REPORT: Woman (38 years old) attends consultation of General Surgery derived from Endocrinology before symptoms of persistent hypoglycemia. Factitious hypoglycemia and syndromes of neuroendocrine origin were ruled out. Imaging tests failed to identify space-occupying lesions. The medical treatment failed, persisting hypoglycemia symptoms. Before the given analytical and radiological findings obtained, and the persistence of symptoms affecting the quality of life of the patient, we opted for surgical treatment performing a pancreatectomy of the 80% of the gland. The final pathologic diagnosis was nesidioblastosis. DISCUSSION: Nesidioblastosis is a rare pathology, but it must be present in the differential diagnosis of hypoglycemia symptoms with endogenous hyperinsulinism in adults, once the intake of sulfonylureas and possible pancreatic neoformations have been ruled out.