Edit concept Question Editor Create issue ticket

Pancreatic Insufficiency

Pancreatic insufficiency is a term describing the inability of the pancreas to produce enzymes necessary for the digestion of nutrients, resulting in malabsorption. The diagnosis is made based on clinical and laboratory criteria.


Presentation

The symptoms of pancreatic insufficiency are primarily related to the progressive destruction of the exocrine pancreas. In the adult population, it is most frequently encountered in association with acute or chronic pancreatitis, pancreatic resection or neoplastic diseases of this organ [1]. In children, on the other hand, the most common cause is cystic fibrosis [2]. As the pancreas is unable to perform its function of releasing digestive enzymes into the duodenal lumen, the resultant malabsorption causes foul-smelling, bulky and loose stools abundant with fat (steatorrhea) that are difficult to flush away [3] [4]. Steatorrhea can occur up to 2-3 times per day, usually after meals, despite normal intake of dietary fat, and is accompanied by abdominal pain of varying severity [4] [5]. Over time, weight loss, anorexia, muscle wasting and flatulence ensue, while severe cases suffer from profound hypoalbuminemia that can manifest with leukonychia [4]. In children, malabsorption causes poor growth and failure to thrive [2]. Although the majority of symptoms are related to exocrine insufficiency, the decreasing output of insulin and glucagon, which are produced by the endocrine pancreas, can lead to diabetes mellitus and there is a very high risk of hypoglycemia, known as "brittle" diabetes [5]. Apart from malabsorption-related complaints, signs of liposoluble vitamin deficiency can appear such as ecchymoses due to vitamin K deficiency, xerophthalmia and night blindness as a result of impaired vitamin A absorption, and ataxia or peripheral neuropathy in the setting of vitamin E deficiency [4] Muscle spasms, osteomalacia and osteoporosis may be related to vitamin D deficiency and consequent hypocalcemia [4].

Weight Loss
  • Weight loss prior to randomisation was numerically greater in the pancreatic extract group (mean 0.7 vs 2.2 kg). Weight loss was numerically greater in the placebo group, however not significantly. No differences in BMI or nutrition score were seen.[ncbi.nlm.nih.gov]
  • The first patient had weight loss arising from steatorrhea secondary to severe pancreatic insufficiency (lipase output result[ncbi.nlm.nih.gov]
  • Currently, our practice is to measure fecal elastase-1 in any patient with unexplained weight loss or symptoms of malabsorption.[ncbi.nlm.nih.gov]
  • A 1.8-year-old intact female Maltese dog was presented because of a history of chronic diarrhea, polyphagia, weight loss, and coprophagia. The patient was severely emaciated and evacuated very moist and four-smelling, yellow feces.[ncbi.nlm.nih.gov]
  • EPI is largely diagnosed clinically, and is often identified by symptoms such as steatorrhea, weight loss, abdominal discomfort, and abdominal bloating.[ncbi.nlm.nih.gov]
Malnutrition
  • Abstract This report describes a 15-year-old girl with anorexia nervosa whose sweat electrolytes and pancreatic exocrine function were abnormal during a state of malnutrition. Both findings normalized when nutrition improved.[ncbi.nlm.nih.gov]
  • INTRODUCTION: Malnutrition is a frequent problem associated with detrimental clinical outcomes in critically ill patients.[ncbi.nlm.nih.gov]
  • Clinical manifestations include abdominal cramps, steatorrhea and malnutrition.[ncbi.nlm.nih.gov]
  • Timely and accurate diagnosis of EPI is important, as delays in treatment prolong maldigestion and malabsorption, with potentially serious consequences for malnutrition, overall health and quality of life.[ncbi.nlm.nih.gov]
  • If left untreated, this leads to nutrient malabsorption, malnutrition and failure to thrive. As a consequence, many sufferers with CF are reliant on enzyme replacement therapy.[cftr.info]
Multiple Congenital Anomalies
  • Abstract Toriello-Carey syndrome is characterized by multiple congenital anomalies. Pancreatic insufficiency is suspected when patients present with poor weight gain, diarrhea, or maldigestion.[ncbi.nlm.nih.gov]
Atrial Septal Defect
  • Less common clinical features were also present, such as atrial septal defect and biventricular hypertrophy, clotting disorders, abnormal liver function tests and nephrocalcinosis.[ncbi.nlm.nih.gov]
Diarrhea
  • CONCLUSION: EPI is a potential adverse event following CRS and HIPEC and might be largely responsible for refractory diarrhea. In our patients with refractory diarrhea and low FE1, PERT provided immediate symptomatic relief.[ncbi.nlm.nih.gov]
  • METHODS: The study included patients who met the Rome II criteria for D-IBS, patients with chronic diarrhea, and subjects without diarrhea (controls).[ncbi.nlm.nih.gov]
  • Only 1/11 reported no symptomatic benefit and 8/19 patients had discontinued supplementation because their diarrhea had improved.[ncbi.nlm.nih.gov]
  • Abstract Diarrhea is a difficult diagnostic problem in patients with chronic graft-versus-host disease (cGVHD) because there are many causes of it.[ncbi.nlm.nih.gov]
  • Pancreatic insufficiency is suspected when patients present with poor weight gain, diarrhea, or maldigestion. The diagnosis is confirmed by low stool elastase and pancreatic stimulation testing.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Chronic pancreatitis was correlated with age younger than 55 years and abdominal pain.[ncbi.nlm.nih.gov]
  • After enzyme supplementation, improvements in stool frequency (P .001), stool consistency (P .001), and abdominal pain (P .003) were observed in patients in group 1, but not in group 2.[ncbi.nlm.nih.gov]
  • Abstract We report a 13-yr-old boy with Crohn's disease in the upper gastrointestinal tract presenting with abdominal pain, failure to thrive, recurrent fever, iron-deficient anemia, and exocrine pancreatic insufficiency.[ncbi.nlm.nih.gov]
  • Chronic pancreatitis can also manifest with abdominal pain, and diabetes. Diagnosis of pancreatic insufficiency Pancreatic insufficiency is suspected in a patient who develops diabetes , upper abdominal pain and features of malabsorption.[ddc.musc.edu]
  • Stomach Pain When partially digested food has to pass through the digestive system it causes abdominal pain or discomfort.[healthline.com]
Chronic Diarrhea
  • METHODS: The study included patients who met the Rome II criteria for D-IBS, patients with chronic diarrhea, and subjects without diarrhea (controls).[ncbi.nlm.nih.gov]
  • A 1.8-year-old intact female Maltese dog was presented because of a history of chronic diarrhea, polyphagia, weight loss, and coprophagia. The patient was severely emaciated and evacuated very moist and four-smelling, yellow feces.[ncbi.nlm.nih.gov]
  • It is also indicated in patients with celiac disease, who have chronic diarrhea (in spite of gluten-free diet), and in patients with cystic fibrosis with proven EPI.[ncbi.nlm.nih.gov]
  • Abstract A 17-year-old white adolescent had a history of chronic diarrhea, delayed puberty, and growth failure. Investigations excluded cystic fibrosis, Shwachman syndrome, and endocrine causes of growth failure.[ncbi.nlm.nih.gov]
  • Chronic diarrhea and excessive weight loss are the most common mal absorption symptoms, however these are not specific for EPI.[medcraveonline.com]
Chronic Abdominal Pain
  • If there is a history of chronic pancreatitis, patients will likely have chronic abdominal pain.[healthcentral.com]
  • These patients often have steatorrhea with foul-smelling, high volume fatty stools, chronic abdominal pain, bloating, and weight loss. Steatorrhea typically does not occur until 90% of the pancreatic function has been compromised.[clinicaladvisor.com]
Foul Smelling Stool
  • Pancreatic insufficiency usually presents with symptoms of malabsorption, malnutrition, vitamin deficiencies, and weight loss (or inability to gain weight in children) and is often associated with steatorrhoea (loose, fatty, foul-smelling stools).[labtestsonline.org.au]
  • This leads to steatorrhea, which is the excretion of abnormal quantities of fat in the feces, leading to pale and very foul-smelling stool. Consequently, the feces oftentimes floats at the very top of the toilet bowl.[study.com]
  • Unabsorbed food in the intestines can lead to oily, loose, foul-smelling stools, bloating, gas, unexplained weight loss, diarrhea, and stomach pain. 2,4 Learn More About EPI Symptoms[creon.com]

Workup

To diagnose pancreatic insufficiency, it is necessary to perform a thorough workup, starting with a detailed patient history that can reveal the presence of comorbidities or pre-existing pancreatic conditions that might be considered as risk factors (alcoholism, acute/chronic pancreatitis, or tumors). After a meticulous physical examination, the obtained data should be sufficient to make a preliminary diagnosis, but further laboratory studies are necessary to support clinical suspicion [6]. Initial blood work includes a complete blood count (CBC), glucose levels, ferritin, lipase, amylase, albumin and total protein content, and pancreatic tests, which may be indirect or direct. [4]. 72-hour stool collection and determination of the coefficient of fat absorption (CFA) was one of the main indirect tests used, but inadequate patient compliance and introduction of novel techniques have displaced it from regular use [1] [4]. Determination of fecal elastase, on the other hand, is a very reliable procedure that requires only one stool sample, but its sensitivity in early and mild insufficiency is limited [1] [4]. Moreover, degradation of triglycerides through the 13C-mixed triglycerides breath test is recommended as well [3], which comprises ingestion of a small amount of 13C-marked triglycerides with a toasted bread and subsequent measurement of CO2 exhaled air, as 13C-triglycerides should be degraded in the intestinal lumen and metabolized in the liver [3]. In the presence of decreased lipase activity, these fatty acids will not be degraded and a decreased CO2 content in exhaled air will be observed [3]. Other notable noninvasive tests include fecal chymotrypsin determination and secretin-enhanced magnetic resonance cholangiopancreatography [4]. The cholecystokinin (CCK) test, which requires specialized laboratory conditions, the secretin test, and the Lundh test are more invasive procedures that are chosen only if indirect tests fail to confirm the diagnosis [1] [3] [4].

Treatment

  • This case report describes treatment of a 9-year-old patient with chronic pancreatic insufficiency, highlighting the diagnostic aspects involved and clinical implications of this condition with respect to dental treatment.[ncbi.nlm.nih.gov]
  • RESULTS: A total of 35 patients were randomized into the study and 22 patients completed both treatment periods.[ncbi.nlm.nih.gov]
  • Appropriate use of diagnostics and treatment approaches using pancreatic enzymes in EPI is essential for patients.[ncbi.nlm.nih.gov]
  • Abstract Conventional treatment of pancreatic steatorrhoea in man has been unsatisfactory because 90% of the lipase content of therapy is inactivated by acid in the stomach and large doses of replacement treatment are needed to provide adequate supplementation[doi.org]
  • Treatment was rated 'easy' to administer by 95% caregivers and acceptance 'good'/'very good' by 90%. CONCLUSIONS: Creon Micro was well tolerated. Growth development parameters increased over the 3-month treatment period.[ncbi.nlm.nih.gov]

Prognosis

  • What is the prognosis for a person with exocrine pancreatic insufficiency (EPI)? The prognosis of patients with exocrine pancreatic insufficiency depends upon the underlying cause.[rxlist.com]
  • The diagnostic methods for detection both early and end‐stage exocrine pancreatic dysfunction are described, as well as the treatment options and prognosis.[doi.org]
  • Prognosis and Patient Counseling. The prognosis is generally good with compliance of pancreatic enzyme supplementation. Patients need counselling and education about the right way to take enzymes and titration of doses according to symptoms.[clinicaladvisor.com]
  • Improved respiratory prognosis in cystic fibrosis patients with normal fat absorption. J Pediatr 1982; 100: 857–862. PubMed CrossRef Google Scholar 3. DiMagno EP. Medical treatment of pancreatic insufficiency. Mayo Clin Proc 1979; 54, 435–442.[link.springer.com]

Etiology

  • In addition to other etiologies, pancreatic insufficiency should also be considered in patients with cGVHD who demonstrate malabsorption. The pathogenesis of pancreatic insufficiency in these patients is unknown.[ncbi.nlm.nih.gov]
  • Numerous conditions account for the etiology of EPI, with the most common being diseases of the pancreatic parenchyma including chronic pancreatitis, cystic fibrosis, and a history of extensive necrotizing acute pancreatitis.[ncbi.nlm.nih.gov]
  • Abstract In assessing exocrine pancreatic insufficiency (EPI), its diverse etiologies and the heterogeneous population affected should be considered.[ncbi.nlm.nih.gov]
  • The alleviation of symptoms was reflected in GIQLI score improvements at 1 year in both cohorts (P 0.001), independent of CP severity and etiology. Improvements in GIQLI score were more pronounced in cohort 2 (P 0.001).[ncbi.nlm.nih.gov]
  • We have postulated that the etiology of Cbl malabsorption in pancreatic insufficiency is an inability to partially degrade R protein because of a lack of pancreatic proteases.[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiology of chronic pancreatitis: burden of the disease and consequences. United European Gastroenterol J. 2014 Oct;2(5):345-54. Available online at . Accessed on 3/19/17. Schneider, A. et. al. (2005 May).[labtestsonline.it]
  • Part I: Epidemiology, etiology, risk factors, genetics, pathophysiology, and clinical features , Disease-a-Month , 10.1016/j.disamonth.2014.11.002 , 60 , 12 , (530-550) , (2014) .[doi.org]
  • Epidemiology [ edit ] It is thought to have an estimated incidence of 1 in 75,000 people. [8] History [ edit ] The disease was first described as a coherent clinical entity in May 1964 by Bodian, Sheldon, and Lightwood. [9] It was subsequently described[en.wikipedia.org]
  • Lankisch PGAssmus CMaisonneuve PLowenfels AB Epidemiology of pancreatic diseases in Luneburg County: a study in a defined German population. Pancreatology 2002;2 (5) 469- 477 PubMed Google Scholar Crossref 5.[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiological mechanism for both phenomena remains obscure.[ncbi.nlm.nih.gov]
  • Another pathophysiological concept proposes the functional and morphological alterations as a consequence of diabetic neuropathy.[ncbi.nlm.nih.gov]
  • Pathophysiology of malabsorption The lack of pancreatic amylase, lipase and proteases results in maldigestion and hence malabsorption.[vin.com]
  • Part I: Epidemiology, etiology, risk factors, genetics, pathophysiology, and clinical features , Disease-a-Month , 10.1016/j.disamonth.2014.11.002 , 60 , 12 , (530-550) , (2014) .[doi.org]
  • Pathophysiology involved in pancreatic exocrine insufficiency The pathophysiology involved in pancreatic exocrine insufficiency is nothing but alteration of normal physiology or the diseased pancreas per se secreting less enzymes.[ispub.com]

Prevention

  • In Shwachman-Diamond syndrome (SDS), a defective SDBS protein prevents eIF6 eviction, inhibiting its recycle to the nucleus and subsequent formation of the active 80S ribosome.[ncbi.nlm.nih.gov]
  • To avoid malnutrition, most studies focus on the prevention of inadequate nutrition delivery, whereas little attention is paid to the potential role of exocrine pancreatic insufficiency (EPI).[ncbi.nlm.nih.gov]
  • In addition to protein and carbohydrate, EN formulas contain fats as a calorie source, as well as vitamins and minerals to help prevent nutritional deficiencies related to malabsorption.[ncbi.nlm.nih.gov]

References

Article

  1. Friess H, Michalski CW. Diagnosing exocrine pancreatic insufficiency after surgery: when and which patients to treat. HPB (Oxford). 2009;11(S 3):7-10.
  2. Trapnell BC, Strausbaugh SD, Woo MS, et al. Efficacy and safety of PANCREAZE® for treatment of exocrine pancreatic insufficiency due to cystic fibrosis. J Cyst Fibros. 2011;10(5):350-356.
  3. Lindkvist B. Diagnosis and treatment of pancreatic exocrine insufficiency. World J Gastroenterol. 2013;19(42):7258-7266.
  4. Pezzilli R, Andriulli A, Bassi C, et al. Exocrine pancreatic insufficiency in adults: A shared position statement of the Italian association for the study of the pancreas. World J Gastroenterol. 2013;19(44):7930-7946.
  5. Hammer HF. Pancreatic exocrine insufficiency: diagnostic evaluation and replacement therapy with pancreatic enzymes. Dig Dis. 2010;28(2):339-343
  6. Fieker A, Philpott J, Armand M. Enzyme replacement therapy for pancreatic insufficiency: present and future. Clin Exp Gastroenterol. 2011;4:55-73.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2018-06-21 22:15