Nesidioblastosis in an Adult with Short Gut Syndrome and Type 2 Diabetes

Abstract

Objective: Adult nesidioblastosis is characterized by endogenous hyperinsulinemia typically causing post-prandial hypoglycemia, and most commonly occurs post-Roux-en-Y gastric bypass. Methods: We report a unique case of nesidioblastosis occurring in a 67-year-old female. Results: A 5-year history of symptomatic hypoglycemia occurred in a patient with short bowel syndrome and type 2 diabetes mellitus (T2DM) managed previously with a glucagon-like peptide 1 (GLP-1) agonist, which achieved significant weight loss. Continuous glucose monitoring captured 42 hypoglycemia episodes in a 2-week period, and following an oral glucose tolerance test there was the suggestion of a hyperinsulinemia state. She was managed with an open distal pancreatectomy, and subsequently required medical therapy to maintain euglycemia. Conclusion: We present the first case of nesidioblastosis occurring in a patient with short bowel syndrome, pre-existing T2DM managed with a GLP-1 agonist which achieved significant weight loss, all of which we speculate could have predisposed to hypoglycemia and development of nesidioblastosis.

Caso de hipoglicemia severa posterior a desviación gástrica en una mujer con enfermedad por esteatosis hepática no alcohólica (EHGNA)

“…Además, la EHGNA puede estar asociada con una mayor variabilidad glucémica a través del aumento del estrés oxidativo, que es un determinante fundamental de la hipoglucemia (4) . Después de una desviación gástrica, se ha propuesto que el aumento persistente del tamaño de la masa de células β que se desarrolla en el contexto de la EHGNA, la obesidad y la secreción alterada de la insulina y del glucagón posiblemente predispone a los pacientes a la hipoglucemia (5) .…”

Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia

“…Of interest, nesidioblastosis has been reported as coexisting disease in diabetic patients. Thus far, 13 cases have been described in the literature [ 17 , 27 , 57 , 65 , 66 , 67 , 75 , 101 , 132 , 180 , 233 ]. In some of the patients, the nesidioblastosis-related hyperinsulinemia led to a reversal of the diabetic syndrome, and insulin therapy could be discontinued.…”

“…Single case reports documenting islet cell hyperplasia/nesidioblastosis with the following diseases can also be found in the medical literature: Choristoma (no reported hyperinsulinism) [ 304 ]; fibrocystic pancreatic atrophy with concomitant pancreatic carcinoma (no reported hyperinsulinism) [ 321 ]; familial adenomatous polyposis (FAP, with hyperinsulinemic hypoglycemia) [ 66 ]; after pancreas transplantation for diabetes mellitus type I (with hyperinsulinemic hypoglycemia) [ 67 ]; orbital lymphoma, hypopituitarism, and secondary adrenal insufficiency (with hyperinsulinemic hypoglycemia) [ 70 ]; Sheehan syndrome (with hyperinsulinemic hypoglycemia) [ 198 ]; Hashimoto thyroiditis with chronic renal failure, incidental adrenal mass, and ovarian thecal metaplasia (no reported hyperinsulinism) [ 76 ]; and a patient with short bowel syndrome with GLP1-agonist treatment for diabetes mellitus type II (with hyperinsulinemic hypoglycemia) [ 233 ].…”

Functional Localization of Adult-Onset Idiopathic Nesidioblastosis

“…Selected previously reported cases of idiopathic nesidioblastosis with follow-up after surgery[7,11,[19][20][21][22][23][24][25][26][27]. Distal pancreatectomy refers to the removal of the body and tail of the pancreas, typically no more than 60%.…”