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.
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 . In children, on the other hand, the most common cause is cystic fibrosis . 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  . 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  . Over time, weight loss, anorexia, muscle wasting and flatulence ensue, while severe cases suffer from profound hypoalbuminemia that can manifest with leukonychia . In children, malabsorption causes poor growth and failure to thrive . 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 . 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  Muscle spasms, osteomalacia and osteoporosis may be related to vitamin D deficiency and consequent hypocalcemia .
Entire Body System
- Weight Loss
Weight loss following esophagectomy is a management challenge for all patients. It is multifactorial with contributing factors including loss of gastric reservoir, rapid small bowel transit, malabsorption, and adjuvant chemotherapy. [ncbi.nlm.nih.gov]
Weight loss and anorexia may also develop over time due to malnutrition. Physical examination Chronic malabsorption results in weight loss, such as temporal scalloping, interosseous wasting, and lack of subcutaneous fat. [dx.doi.org]
Weight Loss Even when you’re eating a normal amount of food, EPI can lead to weight loss. This happens because your body isn’t breaking down food into the smaller forms your digestive system can use. [healthline.com]
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]
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]
New research concludes that the association between celiac disease and exocrine pancreatic insufficiency (EPI) is strong enough that physicians should check for EPI when celiac patients suffer malnutrition or gastric distress despite adherence to prescribed [mdmag.com]
An episode of severe small intestinal hemorrhage occurred in a cystic fibrosis patient having pancreatic insufficiency and receiving timed-release aspirin therapy for disabling hypertrophic pulmonary osteoarthropathy. [ncbi.nlm.nih.gov]
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Type of pain, pain-associatedcomplications, quality of life, disability and resource utilisation in chronic pancreatitis: a prospective cohort study. Gut 2011; 60:77–84. 9. Ammann RW, Muellhaupt B. [ekjm.org]
Sputum and bronchoalveolar lavage samples (n = 492) derived from 275 patients were investigated. E. dermatitidis was isolated in respiratory specimens from 19% (18/97) of the CF patients but in none of the other patient categories. [ncbi.nlm.nih.gov]
Sputum cultures in past reportedly grew out Pseudomonas. Sputum cultures recently c/w normal respiratory flora, enteric gram negative rods, fungal culture negative, AFB smear and culture negative. [aaaai.org]
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]
On day 121 post BMT, she was reevaluated because of persistent diarrhea. [nature.com]
- Abdominal Pain
Chronic pancreatitis was correlated with age younger than 55 years and abdominal pain. [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]
- 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]
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]
[…] that have a very bad smell A full or bloated feeling Weight loss Other symptoms that may develop due to the severity of the disease or its underlying cause occur somewhat less frequently than those listed above include: Chronic abdominal pain Bleeding [rxlist.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]
Medical treatment of chronic pancreatitis focuses on chronic abdominal pain, pancreatic exocrine insufficiency and also pancreatic endocrine insufficiency. [ekjm.org]
- Foul-Smelling Stools
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]
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]
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]
The presentation of steatorrhea, foul-smelling stools, and weight loss despite adequate caloric intake is typical. [nature.com]
The patient did not have any evidence of disorders known to be associated with an abnormal sweat test, including most importantly cystic fibrosis. [ncbi.nlm.nih.gov]
I would not repeat CF genetic screening or obtain another sweat test. I would monitor lymphocyte count and lung function. [aaaai.org]
The pancreas, along with the salivary and sweat glands, is one of the main exocrine glands. Exocrine glands release substances into another organ or the surface of the body through an opening called a duct. [medicalnewstoday.com]
In this latter case, a mild pruritus persisted (pruritus score 1/5), as well as a few focal lesions of erythema and plaque with crusts and mild hyperpigmentation. [jn.nutrition.org]
She had significant dryness and pruritus. A repeat skin biopsy revealed both acute and chronic GVHD. She was placed on high-dose parenteral steroid, the skin rash resolved and liver function tests improved significantly. [nature.com]
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 . 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. . 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  . 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  . Moreover, degradation of triglycerides through the 13C-mixed triglycerides breath test is recommended as well , 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 . 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 . Other notable noninvasive tests include fecal chymotrypsin determination and secretin-enhanced magnetic resonance cholangiopancreatography . 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   .
A patient with pancreatic insufficiency and cyclical neutropenia is described who also has evidence of hepatic dysfunction. He and 3 other patients whose findings are given emphasize the wide range of abnormalities seen in this syndrome. [ncbi.nlm.nih.gov]
Only the first two of these are included in the clinical diagnostic criteria.  Hematologic abnormalities: neutropenia may be intermittent or persistent and is the most common hematological finding. [en.wikipedia.org]
[…] idiopathic) (localized) (perforative) (primary) (with adhesions) (with effusion) 567.9 pancreatic 577.8 Rupture, ruptured 553.9 pancreas 577.8 Sclerosis, sclerotic pancreas 577.8 Syndrome - see also Disease Burke's (pancreatic insufficiency and chronic neutropenia [icd9data.com]
Over time, weight loss, anorexia, muscle wasting and flatulence ensue, while severe cases suffer from profound hypoalbuminemia that can manifest with leukonychia. In children, malabsorption causes poor growth and failure to thrive. [symptoma.com]
Nail leukonychia due to hypoalbuminemia may be present in the late stages of chronic malabsorption. [dx.doi.org]
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]
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]
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]
Berlin, itd: Springer Verlag [ PM ] [ EČ ][ GS ] Lowenfels, A.B., Maisonneuve, P., Cavallini, G., Ammann, R.W., Lankisch, P.G., Andersen, J.R., DiMagno, E.P., Andren-Sandberg, A., Domellof, L., di Francesco, V. (1994) Prognosis of chronic pancreatitis [scindeks.ceon.rs]
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]
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]
Depending on the underlying genetic etiology, it can be inherited in autosomal dominant, autosomal recessive or in a polygenic manner. [cincinnatichildrens.org]
Epidemiology of chronic pancreatitis: burden of the disease and consequences. United European Gastroenterol J. 2014 Oct;2(5):345-54. Available online at https://www.ncbi.nlm.nih.gov/pubmed/25360312. Accessed on 3/19/17. [labtestsonline.it]
The epidemiology and impact of pancreatic diseases in the United States. Curr Gastroenterol Rep. 2005 May. 7(2):90-5. [Medline]. de la Iglesia D, Vallejo-Senra N, Lopez-Lopez A, et al. [emedicine.medscape.com]
Epidemiology [ edit ] It is thought to have an estimated incidence of 1 in 75,000 people.  History [ edit ] The disease was first described as a coherent clinical entity in May 1964 by Bodian, Sheldon, and Lightwood.  It was subsequently described [en.wikipedia.org]
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]
Having correlated our findings with current knowledge of SPINK1's role in exocrine pancreas pathophysiology, we propose that complete and partial functional losses of the SPINK1 gene are associated with quite distinct phenotypes, the former causing a [ncbi.nlm.nih.gov]
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]
Pathophysiology of malabsorption The lack of pancreatic amylase, lipase and proteases results in maldigestion and hence malabsorption. [vin.com]
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]
- Friess H, Michalski CW. Diagnosing exocrine pancreatic insufficiency after surgery: when and which patients to treat. HPB (Oxford). 2009;11(S 3):7-10.
- 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.
- Lindkvist B. Diagnosis and treatment of pancreatic exocrine insufficiency. World J Gastroenterol. 2013;19(42):7258-7266.
- 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.
- Hammer HF. Pancreatic exocrine insufficiency: diagnostic evaluation and replacement therapy with pancreatic enzymes. Dig Dis. 2010;28(2):339-343
- Fieker A, Philpott J, Armand M. Enzyme replacement therapy for pancreatic insufficiency: present and future. Clin Exp Gastroenterol. 2011;4:55-73.