Exocrine Pancreatic Insufficiency in Children – Challenges in Management

Abstract

Cystic fibrosis (CF) is the leading etiology for exocrine pancreatic insufficiency (EPI) in children, followed by chronic pancreatitis, Shwachman-Diamond syndrome, and other genetic disorders. Management of EPI in children poses several unique challenges such as difficulties in early recognition, lack of widespread availability of diagnostic tests and limited number of pediatricspecific pancreatic centers. Pancreatic enzyme replacement therapy is the cornerstone of EPI management and in young children difficulties in administering pancreatic enzymes are frequently encountered. Patients with EPI also should be screened for fat-soluble vitamin deficiencies and receive appropriate supplementation. Among disorders with EPI in children, CF is the relatively well-studied condition, and most management recommendations for EPI in children come from expert consensus and conventional practice guidelines. The impact of EPI can be greater in children given their high metabolic demands and rapid growth. Early diagnosis and aggressive management of EPI prevent consequences of complications such as malnutrition, fat-soluble vitamin deficiencies, and poor bone health and improve outcomes. Management by multi-disciplinary team is the key to success.

The Therapeutic Effect of Pancreatic Enzyme Replacement on Anthropometric Parameters in Children with Malnutrition: A Pilot Clinical Trial

“…Conditions associated with inflammation or atrophy in the proximal small intestine, such as celiac disease, can further complicate the health of malnourished individuals. Mucosal atrophy in the intestine is identified as a consequence of malnutrition, affecting enteropancreatic stimulation and impairing liver and pancreas function (8,(13)(14)(15). Notably, the enterokinase enzyme is secreted through the intestinal mucosa and its decreased level may reduce the activation of other pancreatic enzymes (16).…”