Hyperinsulinaemic hypoglycaemia —an overview of a complex clinical condition

Conclusion: Advances in the fields of genetic testing, imaging techniques and medical treatment are beginning to provide novel insights into earlier detection, less invasive treatment approaches and fewer complications associated with the complex entity of hyperinsulinaemic hypoglycaemia.What is Known:•HH is caused by dysregulated insulin release from the β cell due to genetic mutations and carries a risk for complications, such as neurocognitive impairment. 18F-DOPA PET/CT scanning is presented as the gold-standard imaging technique currently in children with hyperinsulinaemic hypoglycaemia.•Clinical presentation is heterogeneous and treatment options include medical therapy and pancreatectomy.What is New:•18F-DOPA PET/CT is indicated in suspected focal CHI due to paternal transmitted mutations in ABCC8 or KCNJ11.•Novel treatment options have been introduced, such as long-acting octreotide, lanreotide, sirolimus and selective nonpeptide somatostatin receptor subtype 5 (SSTR5) agonists. Future medical treatments include exendin, a GLP-1 antagonist, and glucagon infusion viaa pump. However, all these options are off-label at present.
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research