Challenges in the management of pancreatic exocrine insufficiency

Abstract

Pancreatic exocrine insufficiency (PEI) occurs when the insufficient secretion or function of pancreatic enzymes leads to maldigestion, most commonly as a result of chronic pancreatitis and pancreatic cancer. The condition is associated with significant morbidity and reductions in quality of life, even in milder forms. The challenges in approaching this condition include the non-specific presentation of mild to moderate PEI, and the lack of a convenient, accurate diagnostic test in this cohort. Classical symptoms appear late in the disease, and the diagnosis should be considered before steatorrhoea develops. Direct pancreatic function tests are the reference standard for diagnosis, but are invasive and not widely available. The faecal elastase-1 (FE-1) stool test is widely available and has been shown to be as effective as the 13C-mixed triglyceride breath test in more advanced disease. We recommend a pragmatic diagnostic approach that combines clinical history, assessment of nutritional status and measurement of FE-1. The critical first step is to consider the diagnosis. Once the diagnosis is confirmed, pancreatic enzyme replacement therapy should be initiated. The variety of enzyme preparations and recommended dosing regimens can present a challenge when selecting an adequate initial dose. Non-response should be actively sought and addressed in a systematic manner. This article discusses these challenges, and presents a practical approach to the diagnosis and management of PEI.

Management of Exocrine Pancreatic Insufficiency in Children

“…Secondary EPI is due to nonpancreatic reasons such as small‐bowel inflammation and asynchrony in the digestive process (Table ). Inflammatory bowel disease, celiac disease, and other conditions with proximal small‐bowel mucosal inflammation can result in EPI due to decreased enteropancreatic stimulation by CCK . Postcibal asynchrony is the dissociation between delivery of chyme to the proximal small bowel and discharge of pancreatic secretions mostly due to altered anatomy of the upper GI tract .…”

“…Inflammatory bowel disease, celiac disease, and other conditions with proximal small‐bowel mucosal inflammation can result in EPI due to decreased enteropancreatic stimulation by CCK . Postcibal asynchrony is the dissociation between delivery of chyme to the proximal small bowel and discharge of pancreatic secretions mostly due to altered anatomy of the upper GI tract . Because of an increase in obesity prevalence, this etiology of EPI is increasingly recognized after bariatric surgeries such as sleeve gastrectomy and gastric bypass surgery .…”

“…Exocrine pancreatic insufficiency (EPI) is defined as a decrease in the secretion of pancreatic enzymes, bicarbonate, or both, resulting in the malabsorption of nutrients . The pancreas has a large physiological reserve, and a reduction of secretion < 10% of the normal output results in the characteristic symptoms of chronic diarrhea, steatorrhea, and poor weight gain .…”

“…The diagnosis of EPI can be challenging, as symptoms may not be recognized. EPI can negatively impact health and well‐being through subclinical maldigestion and malabsorption of both macronutrients and micronutrients, particularly the fat‐soluble vitamins . The presenting symptoms of EPI are often nonspecific and overlap with other gastrointestinal (GI) conditions, with maldigestion or malabsorption resulting in diagnostic difficulties .…”

“…EPI can negatively impact health and well‐being through subclinical maldigestion and malabsorption of both macronutrients and micronutrients, particularly the fat‐soluble vitamins . The presenting symptoms of EPI are often nonspecific and overlap with other gastrointestinal (GI) conditions, with maldigestion or malabsorption resulting in diagnostic difficulties . Unlike in adulthood, during which the most common cause of EPI is chronic pancreatitis (CP), cystic fibrosis (CF) is the most common etiology of EPI in childhood and adolescence .…”