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|Title:||Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism.|
congenital hyperinsulinism (CH)
exocrine pancreatic insufficiency (EPI)
somatostatin receptor analogs (SSRAs)
Exocrine Pancreatic Insufficiency
|Citation:||J Pediatr Endocrinol Metab.2020;(33)7:947-950|
|Abstract:||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.|
|Appears in Collections:||Fundaciones e Institutos de Investigación > FIB H. Infantil U. Niño Jesús > Artículos|
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