Hyperinsulinemic hypoglycemia in adolescents: case report and systematic review

Abstract

Background: Hyperinsulinemic hypoglycemia is the most common cause of severe and persistent hypoglycemia in neonates and children. It is a heterogeneous condition with dysregulated insulin secretion, which persists in the presence of low blood glucose levels.

Case presentation: We report a case of a 15 year-old male with hyperinsulinemic hypoglycemia, who underwent a subtotal pancreatectomy after inadequate response to medical therapy. Pathological examination was positive for nesidioblastosis (diffuse β-cell hyperplasia by H-E and immunohistochemical techniques). The patient's blood glucose levels normalized after surgery and he remains asymptomatic after 1 year of follow-up. The systematic review allowed us to identify 41 adolescents from a total of 205 cases reported in 22 manuscripts, from a total of 454 found in the original search done in PubMed and Lilacs.

Conclusions: Although very well reported in children, hyperinsulinemic hypoglycemia can occur in adolescents or young adults, as it happens in our reported case. These patients can be seen, treated and reported by pediatricians or adult teams either way due to the wide age range used to define adolescence. Most of them do not respond to medical treatment, and subtotal distal pancreatectomy has become the elected procedure with excellent long-term response in the vast majority.

Keywords: Hyperinsulinemic hypoglycemia (HH); Nesidioblastosis; Whipple triad.

Fig. 1

PET-Scan: white arrows show the increased radiotracer uptake distributed all along the pancreatic tissue

Fig. 2

Pathological report of the surgical sample. A and B TECHNIQUE: HEMATOXILINA- EOSNA. 10x magnification Pancreatic parenchyma of globally preserved histoarchitecture is observed, with the presence of islets of Langerhans cells of different sizes, some with a tendency to cluster (arrow in fig. 2a), and in sectors with a certain tendency to adopt a periductal disposition (cross in fig. 2b). C and D IMMUNOHISTOCHEMICAL TECHNIQUE WITH CHROMOGRANINE. 4X INCREASE Positive staining (cytoplasmic and nuclear) is observed in pancreatic islet cells, which show different sizes and irregular shapes (fig c, arrow)

Fig. 3

Systematic Review Flow-Chart