Can I read Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism on EtoBox?

Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism by Purificación Ros-Pérez; Luz Golmayo; María L. Cilleruelo; Carolina Gutiérrez; Patricia Celaya; Nerea Lacámara; Itziar Martínez-Badás; María Güemes; Jesús Argente is a Medicine article available to read on EtoBox.

What is Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism about?

Background: Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn. Octreotide, a long-acting somatostatin receptor analog (SSRA), is a second line treatment for diazoxide unresponsive CH patients. Although it has been found to be a safe and effective treatment, long-term benefits and side effects, have not been thoroughly evaluated. Case presentation: Some authors have indicated that exocrine pancreatic insufficiency (EPI) is a common but under-recognized adverse reaction in adults treated with octreotide. However, no pediatric patient with SSRA-induced EPI has been reported to date. Here we report a case of an infant with diazoxide unresponsive, diffuse CH, caused by a heterozygous pathogenic paternally inherited mutation in the ABCC8 gene (NM\_000352.4:c.357del), that developed exocrine pancreatic insufficiency and secondary vitamin K deficiency associated to chronic octreotide therapy. Conclusions: We point out the atypical clinical onset with a cutaneous hemorrhagic syndrome, emphasizing the clinical relevance of this potential side effect.

Who reads Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism?

Author

Purificación Ros-Pérez; Luz Golmayo; María L. Cilleruelo; Carolina Gutiérrez; Patricia Celaya; Nerea Lacámara; Itziar Martínez-Badás; María Güemes; Jesús Argente

Publisher

Walter de Gruyter GmbH

Published

2020

Language

EN

Field

Medicine (Health Sciences)