Exocrine pancreatic insufficiency

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Classification according to ICD-10
K90.3 Pancreatogenic steatorrhea
ICD-10 online (WHO version 2019)

As exocrine pancreatic insufficiency (short: EPI ) or pancreas weakness is a disease of the pancreas called that with insufficient production of digestive enzymes associated. The result is a pronounced indigestion with weight loss or insufficient weight gain. The diagnosis can be made by determining the activity of digestive enzymes in the stool. Replacement with food is possible for treatment.

Pathogenesis

Various causes can contribute to the development of the disease. In childhood, two monogenic diseases , cystic fibrosis and Shwachman-Bodian-Diamond syndrome, are the most common causes of exocrine pancreatic insufficiency. In adults, the likelihood of developing exocrine pancreatic insufficiency after acute inflammation of the pancreas ( pancreatitis ) increases with the severity of the inflammation and the extent of tissue destruction ( necrosis ). In addition to acute or chronic pancreatitis, resection of the head of the pancreas or total pancreatectomy as well as genetic or idiopathic diseases that lead to the destruction of the acinar cells in the pancreas can cause an EPI. In addition, exocrine pancreatic insufficiency is common in diabetics. Based on the study data collected with fecal elastase-1, the prevalence is around 51% (type 1) and 32% (type 2 diabetes). This causes a restricted production of the enzymes or enzyme precursors trypsinogen , chymotrypsinogen , carboxypeptidase , α-amylase and pancreatic lipase . The lack of these enzymes in turn causes a considerable disturbance of the digestion of nutrients ingested with food ( maldigestion ). Secondary changes in the intestinal mucosa occur : intestinal villi atrophy , local inflammation and changes in enzyme activity occur within the intestinal mucosa. Incorrect bacterial colonization (incorrect colonization of the small intestine ) of the intestine is often an aggravating factor.

Clinical symptoms

Those affected by the disease show chronic weight loss or, in children, poor weight gain ("failure to thrive") despite increased food intake . The stool is often very light, foul-smelling and voluminous (“fatty stool”, steatorrhea ), sometimes with diarrhea. Rarely, if the intake of vitamin K is reduced, there is an increased tendency to bleeding.

diagnosis

In humans, the most reliable method (“ gold standard ”) for detecting exocrine pancreatic insufficiency is the secretin-pancreozymin test . However, this is quite invasive, time-consuming and is not used in everyday diagnostics. As a rule, the concentration of the pancreatic enzyme elastase-1 in the stool is measured; this method is currently recognized as the non-invasive “gold standard”. Another method is the measurement of chymotrypsin via fluorescein dilaurate test in the stool. A determination of the fat excretion in the stool over 24 hours also indicates a disturbed fat digestion. Because of the low sensitivity , this test should be repeated three times. The pancreolauryl test is no longer common due to its lack of sensitivity and specificity.

treatment

For treatment, digestive enzymes of the pancreas are available as drugs with the active ingredient pancreatin to be taken with meals. The necessary enzymes are packaged in an enteric coating so that they are only released in the small intestine and take effect. Furthermore, the diet should consist of small meals rich in carbohydrates (5–7 per day). If necessary, the fat-soluble vitamins A, D, E, and K should be substituted. Treatment with pancreatin is controversial, however, as the active ingredient is produced from pig slaughterhouse waste. Parts of the Islamic world are critical of the extraction process, as are German approval authorities.

As an alternative there are amylase , protease and lipase , which are extracted from vegetable sources. The basis is provided by the Japanese rice mushroom cultures Rhizopus oryzae and Aspergillus oryzae , from whose mycelium highly purified and highly effective digestive enzymes can be obtained. A gastric juice-resistant acid protection is not necessary for the digestive enzymes from rice mushrooms called rizoenzymes , as these are naturally acid-stable. In this way, they develop their effect in the acidic environment of the stomach.

The University Medical Center Münster and the University Medical Center of the Ruhr University Bochum as well as the biotech company CILIAN AG are currently researching a new process based on the production of digestive enzymes by single-cell organisms .

Veterinary medicine

Signalement and symptoms

The occurrence of exocrine pancreatic insufficiency has been described for dogs and cats , but occurs much more frequently in dogs than in cats. The most common underlying disease in exocrine pancreatic insufficiency in dogs is pancreatic acinar atrophy (PAA); pancreatitis and tumors (neoplasias) are also causes.

Among the dog breeds, the German shepherd and the long-haired collie are often affected; in these breeds it is caused by PAA and is an autosomal recessive inherited disease. In the German Shepherd, the EPI usually occurs in young adulthood, in other dog breeds and in cats it is mostly middle-aged to older animals.

The main symptoms of EPIs are weight loss with increased appetite and accompanied by high-fat diarrhea ( steatorrhea ). Watery diarrhea, vomiting and refusal to feed occur less frequently, although the latter can be an indication of further illnesses.

diagnosis

The most specific test for the diagnosis of EPI is these species of TLI test ( T rypsin- l ike I mmunoreactivity). Since the body's defense reaction to the trypsin formed is measured here, the values ​​are usually reduced when the disease is present. Also, the for Pankreatitisdiagnostik used PLI-test ( P ancreatic L ipase I mmunoreactivity) appears to be suitable, the TLI-test with respect to the sensitivity inferior to (An exception is the rare special case of an isolated deficiency of the activity of pancreatic lipase). The determination of the serum cobalamin , the triglyceride challenge test and quantitative fecal analyzes to determine the activity of digestive enzymes are also used. However, these three methods have a lower sensitivity and specificity than the TLI test.

Therapy and prognosis

In animals, as in humans, therapy consists of the oral replacement of digestive enzymes with pancreatin or rizoenzymes . In addition, feeding raw pork or beef pancreas from slaughterhouse waste can also be an effective treatment. The adaptation of the feeding in the form of a highly digestible diet can decisively support the drug treatment and help to reduce the enzyme dose.

Web links

Individual evidence

  1. ↑ a b J. Walkowiak et al .: Fecal Elastase-1 Is Superior to Fecal Chymotrypsin in the Assessment of Pancreatic Involvement in Cystic Fibrosis In: Pediatrics 2002; 110: e7 PMID 12093988 full text online (English)
  2. ^ GW Hall, P. Dale, JA Dodge. Shwachman-Diamond syndrome: UK perspective. In: Arch Dis Child . 2006 Jun; 91 (6), pp. 521-524. PMID 16714727
  3. ↑ B. Boreham, BJ Ammori: A prospective evaluation of pancreatic exocrine function in patients with acute pancreatitis: correlation with extent of necrosis and pancreatic endocrine insufficiency. In: Pacreatology 2002; 3: pp. 303-308, PMID 12890992
  4. ↑ Birgit Kaufmann, Jutta Girke: Paradigm shift in dietetics: nutrition and enzyme substitution in pancreatic insufficiency . (PDF) In: Diet & Information , Edition 5/2012, pp. 14–24.
  5. ↑ Philip D. Hardt, Michael Erbach, Oliver Schnell: Does he have exocrine pancreatic insufficiency? Weight loss and fat stools in a diabetic . In: MMW - progress in medicine , edition 9/2012.
  6. ↑ a b Gerd Herold : Internal Medicine . Cologne 2007, p. 448, 454 .
  7. ^ Ernst-Albert Meyer: Digestive complaints: Plant enzymes as a therapy option . In: PTA-Forum , issue 04/2008.
  8. ^ Markus Köller: ciliates instead of slaughterhouse waste. (No longer available online.) December 11, 2013, archived from the original on November 12, 2014 ; Retrieved December 12, 2013 . Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.nachrichten-ticker.com
  9. ↑ Münster residents develop therapies for the pancreas. (No longer available online.) Münstersche Zeitung, December 11, 2013, formerly in the original ; Retrieved December 12, 2013 .  ( Page no longer available , search in web archives )  Info: The link was automatically marked as defective. Please check the link according to the instructions and then remove this notice.@1@ 2Template: Toter Link / www.muensterschezeitung.de  
  10. ↑ ME Wiberg: Pancreatic acinar atrophy in German shepherd dogs and rough-coated collies. Etiopathogenesis, diagnosis and treatment. A review. In: The Veterinary quarterly. Volume 26, Number 2, June 2004, pp. 61-75, ISSN  0165-2176 . doi: 10.1080 / 01652176.2004.9695169 . PMID 15230051 . (Review).
  11. ↑ a b Exocrine Pancreatic Insufficiency ( Memento of the original from March 22, 2003 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. in the Merck Veterinary Manual @1@ 2Template: Webachiv / IABot / www.merckvetmanual.com
  12. ↑ Griffin et al .: Acid resistant lipase as replacement therapy in chronic pancreatic exocrine insufficiency: a study in dogs. In: Gut , 1989; 30: pp. 1012-1015 ( doi: 10.1136 / gut.30.7.1012 )
  13. ↑ Vincent C. Biourge, Jacques Fontaine: Exocrine Pancreatic Insufficiency and Adverse Reaction to Food in Dogs: A Positive Response to a High-Fat, Soy Isolate Hydrolysate-Based . In: Journal of Nutrition , 2004, 134, Suppl., Pp. 2166S-2168S. PMID 15284428