Exocrine pancreatic insufficiency
Encyclopedia
Exocrine pancreatic insufficiency (EPI) is the inability to properly digest food due to a lack of digestive enzyme
s made by the pancreas
. This disease is found frequently in dog
s. EPI is also found in humans afflicted with cystic fibrosis
and Shwachman-Diamond Syndrome. EPI is caused by a progressive loss of the pancreatic cells that make digestive enzymes. Most commonly in dogs, this is caused by pancreatic acinar atrophy. The atrophy in turn can be caused by previous infections, a blocked pancreatic duct
, or genetics
. Chronic pancreatitis
is the most common cause of EPI in humans and cat
s, but it is an uncommon cause in dogs. Loss of digestive enzymes leads to maldigestion and malabsorption
of nutrients.
, which is a hereditary recessive disease of Europeans and Ashkenazi Jews involving a chloride channel
called CFTR, and chronic pancreatitis
. Shwachman-Diamond Syndrome is the second most common cause of pancreatic insufficiency in humans.
EPI is also fairly common in diabetes - both type 1 and type 2. Studies have shown that about 35% of type 2 diabetics and 50% of type 1 diabetics exhibit symptoms and characteristics of EPI. However, treatment is normally only initiated once the patient complains of problems with steatorrhea
, bulky stools, abdominal pain, and/or flatulence. Some clinicians refer to this phenomenon as "diabetic diarrhea", however this term is rarely explained as a symptom of EPI possibly because it could also be linked to GI motility problems. Limited and preliminary studies have indicated that treatment of EPI with products such as Pancrelipase
have an effect on glucose control. Also, there have been only small differences in the fat soluble vitamin status of diabetics treated with products such as Pancrelipase, as steatorrhea can lead to a decrease in the absorption of fat soluble vitamins.
s in Finland, in which it is inherited. In the German Shepherd Dog the method of inheritance is through an autosomal recessive gene. In these two breeds, at least, the cause appears to be immune-mediated
as a sequela to lymphocytic
pancreatitis. The German Shepherd Dog makes up about two-thirds of cases seen with EPI. Other breeds reported to be predisposed to EPI include terrier
breeds, Cavalier King Charles Spaniel
s, Chow Chow
s and Picardien Shepherd.
, increased appetite, coprophagia
, and diarrhea. Feces are often yellow-gray in color with an oily texture. There are many concurrent diseases that mimic EPI and severe pancreatitis is one that is allowed to continue unabated can lead to EPI.
levels may also be used for diagnosis in dogs.
In dogs, the best treatment is to supplement its food with dried pancreatic extracts. There are commercial preparations available, but chopped bovine pancreas from the butcher can also be used (pork pancreas should not be used because of the rare transmission of pseudorabies
). Symptoms usually improve within a few days, but lifelong treatment is required in most cases. A rare side effect of use of dried pancreatic extracts is oral ulceration and bleeding.
Because of malabsorption, serum levels of cyanocobalamin
(vitamin B12) and tocopherol
(vitamin E) may be low. These may be supplemented, although since cyanocobalamin contains the toxic chemical cyanide
, dogs which have serious cobalamin issues should instead be treated with hydroxocobalamin
or methylcobalamin. Cyanocobalamin deficiency is very common in cats with EPI because about 99 percent of intrinsic factor
(which is required for cyanocobalamin absorption from the intestine) is secreted by the pancreas. In dogs this figure is about 90 percent, and only about 50 percent of dogs have this deficiency. Cats may suffer from Vitamin K
deficiencies. If there is bacterial overgrowth
in the intestine, antibiotics should be used, especially if treatment is not working. In dogs failing to gain weight or continuing to show symptoms, modifying the diet to make it low fiber and highly digestible may help. Despite previous belief that low fat diets are beneficial in dogs with EPI, more recent studies have shown that a high fat diet may increase absorption of nutrients and better manage the disease. However, it has been shown that different dogs respond to different dietary modifications, so the best diet must be determined on a case by case basis.
Digestive enzyme
'Digestive enzymes' are enzymes that break down polymeric macromolecules into their smaller building blocks, in order to facilitate their absorption by the body. Digestive enzymes are found in the digestive tract of animals where they aid in the digestion of food as well as inside the cells,...
s made by the pancreas
Pancreas
The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as a digestive organ, secreting pancreatic juice containing digestive enzymes that assist...
. This disease is found frequently in dog
Dog
The domestic dog is a domesticated form of the gray wolf, a member of the Canidae family of the order Carnivora. The term is used for both feral and pet varieties. The dog may have been the first animal to be domesticated, and has been the most widely kept working, hunting, and companion animal in...
s. EPI is also found in humans afflicted with cystic fibrosis
Cystic fibrosis
Cystic fibrosis is a recessive genetic disease affecting most critically the lungs, and also the pancreas, liver, and intestine...
and Shwachman-Diamond Syndrome. EPI is caused by a progressive loss of the pancreatic cells that make digestive enzymes. Most commonly in dogs, this is caused by pancreatic acinar atrophy. The atrophy in turn can be caused by previous infections, a blocked pancreatic duct
Pancreatic duct
The pancreatic duct, or duct of Wirsung , is a duct joining the pancreas to the common bile duct to supply pancreatic juices which aid in digestion provided by the "exocrine pancreas"...
, or genetics
Genetics
Genetics , a discipline of biology, is the science of genes, heredity, and variation in living organisms....
. Chronic pancreatitis
Chronic pancreatitis
Chronic pancreatitis is a long-standing inflammation of the pancreas that alters its normal structure and functions. It can present as episodes of acute inflammation in a previously injured pancreas, or as chronic damage with persistent pain or malabsorption....
is the most common cause of EPI in humans and cat
Cat
The cat , also known as the domestic cat or housecat to distinguish it from other felids and felines, is a small, usually furry, domesticated, carnivorous mammal that is valued by humans for its companionship and for its ability to hunt vermin and household pests...
s, but it is an uncommon cause in dogs. Loss of digestive enzymes leads to maldigestion and malabsorption
Malabsorption
Malabsorption is a state arising from abnormality in absorption of food nutrients across the gastrointestinal tract.Impairment can be of single or multiple nutrients depending on the abnormality...
of nutrients.
Causes
In humans, the common causes of EPI are Cystic FibrosisCystic fibrosis
Cystic fibrosis is a recessive genetic disease affecting most critically the lungs, and also the pancreas, liver, and intestine...
, which is a hereditary recessive disease of Europeans and Ashkenazi Jews involving a chloride channel
Chloride channel
Chloride channels are a superfamily of poorly understood ion channels consisting of approximately 13 members.Chloride channels display a variety of important physiological and cellular roles that include regulation of pH, volume homeostasis, organic solute transport, cell migration, cell...
called CFTR, and chronic pancreatitis
Chronic pancreatitis
Chronic pancreatitis is a long-standing inflammation of the pancreas that alters its normal structure and functions. It can present as episodes of acute inflammation in a previously injured pancreas, or as chronic damage with persistent pain or malabsorption....
. Shwachman-Diamond Syndrome is the second most common cause of pancreatic insufficiency in humans.
EPI is also fairly common in diabetes - both type 1 and type 2. Studies have shown that about 35% of type 2 diabetics and 50% of type 1 diabetics exhibit symptoms and characteristics of EPI. However, treatment is normally only initiated once the patient complains of problems with steatorrhea
Steatorrhea
Steatorrhea is the presence of excess fat in feces. Stools may also float due to excess lipid, have an oily appearance and be especially foul-smelling. An oily anal leakage or some level of fecal incontinence may occur. There is increased fat excretion, which can be measured by determining the...
, bulky stools, abdominal pain, and/or flatulence. Some clinicians refer to this phenomenon as "diabetic diarrhea", however this term is rarely explained as a symptom of EPI possibly because it could also be linked to GI motility problems. Limited and preliminary studies have indicated that treatment of EPI with products such as Pancrelipase
Pancrelipase
Pancrelipase is a form of enzyme therapy for the treatment of various digestive disorders, primarily exocrine pancreatic insufficiency. It is a mixture of three digestive enzymes, amylase, trypsin, and lipase, which are normally produced by the pancreas...
have an effect on glucose control. Also, there have been only small differences in the fat soluble vitamin status of diabetics treated with products such as Pancrelipase, as steatorrhea can lead to a decrease in the absorption of fat soluble vitamins.
Treatment
Often this is treated with Pancreatic Enzyme Products (PEPs), such as pancrelipase, that are used to breakdown fats (lipases), proteins (proteases) and carbohydrates (amylases) into units that can be digested by those with EPI.Pathogenesis
In dogs, EPI is most common in young German Shepherd Dogs, and Rough CollieRough Collie
The Rough Collie is a long coated breed of medium to large size dog that in its original form was a type of collie used and bred for herding in Scotland. Originating in the 1800's, it is now well known through the works of author Albert Payson Terhune, and through the Lassie novel, movies, and...
s in Finland, in which it is inherited. In the German Shepherd Dog the method of inheritance is through an autosomal recessive gene. In these two breeds, at least, the cause appears to be immune-mediated
Immune-mediated disease
Immune-mediated diseases are conditions which result from abnormal activity of the body's immune system. The immune system may over-react or start attacking the body . Autoimmune diseases are a subset of immune-mediated diseases....
as a sequela to lymphocytic
Lymphocyte
A lymphocyte is a type of white blood cell in the vertebrate immune system.Under the microscope, lymphocytes can be divided into large lymphocytes and small lymphocytes. Large granular lymphocytes include natural killer cells...
pancreatitis. The German Shepherd Dog makes up about two-thirds of cases seen with EPI. Other breeds reported to be predisposed to EPI include terrier
Terrier
A terrier is a dog of any one of many breeds or landraces of terrier type, which are typically small, wiry, very active and fearless dogs. Terrier breeds vary greatly in size from just a couple of pounds to over 70 pounds and are usually categorized by size or function...
breeds, Cavalier King Charles Spaniel
Cavalier King Charles Spaniel
The Cavalier King Charles Spaniel is a small breed of Spaniel-type dog, and is classed as a toy dog by most kennel clubs. It is one of the most popular breeds in the United Kingdom. Since 2000, it has been growing in popularity in the United States. It is a smaller breed of spaniel, and Cavalier...
s, Chow Chow
Chow Chow
Chow chow is a breed of dog that was developed in China, where it is referred to as Songshi Quan , which literally means "puffy-lion dog".- Appearance :...
s and Picardien Shepherd.
Symptoms
In animals, signs of EPI are not present until 85 to 90 percent of the pancreas is unable to secrete its enzymes. In dogs, symptoms include weight loss, poor hair coat, flatulenceFlatulence
Flatulence is the expulsion through the rectum of a mixture of gases that are byproducts of the digestion process of mammals and other animals. The medical term for the mixture of gases is flatus, informally known as a fart, or simply gas...
, increased appetite, coprophagia
Coprophagia
Coprophagia or coprophagy is the consumption of feces, from the Greek κόπρος copros and φαγεῖν phagein . Many animal species practice coprophagia as a matter of course; other species do not normally consume feces but may do so under unusual conditions...
, and diarrhea. Feces are often yellow-gray in color with an oily texture. There are many concurrent diseases that mimic EPI and severe pancreatitis is one that is allowed to continue unabated can lead to EPI.
Diagnosis and treatment
The most reliable test for EPI in dogs and cats is serum trypsin-like immunoreactivity (TLI). A low value indicates EPI. Fecal elastaseElastase
In molecular biology, elastase is an enzyme from the class of proteases that break down proteins.- Forms and classification:There exist eight human genes for elastase:Bacterial forms: Organisms such as P...
levels may also be used for diagnosis in dogs.
In dogs, the best treatment is to supplement its food with dried pancreatic extracts. There are commercial preparations available, but chopped bovine pancreas from the butcher can also be used (pork pancreas should not be used because of the rare transmission of pseudorabies
Pseudorabies
Pseudorabies is a viral disease in swine that is endemic in most parts of the world. It is caused by Suid herpesvirus 1 , which is also called Pseudorabies virus and is also known as Aujeszky's disease, and in cattle as mad itch. PRV is considered to be the most economically important viral...
). Symptoms usually improve within a few days, but lifelong treatment is required in most cases. A rare side effect of use of dried pancreatic extracts is oral ulceration and bleeding.
Because of malabsorption, serum levels of cyanocobalamin
Cyanocobalamin
Cyanocobalamin is an especially common vitamer of the vitamin B12 family. It is the most famous vitamer of the family, because it is, in chemical terms, the most air-stable...
(vitamin B12) and tocopherol
Tocopherol
Tocopherols are a class of chemical compounds of which many have vitamin E activity. It is a series of organic compounds consisting of various methylated phenols...
(vitamin E) may be low. These may be supplemented, although since cyanocobalamin contains the toxic chemical cyanide
Cyanide
A cyanide is a chemical compound that contains the cyano group, -C≡N, which consists of a carbon atom triple-bonded to a nitrogen atom. Cyanides most commonly refer to salts of the anion CN−. Most cyanides are highly toxic....
, dogs which have serious cobalamin issues should instead be treated with hydroxocobalamin
Hydroxocobalamin
Hydroxocobalamin is a natural form, or vitamer, of vitamin B12, a basic member of the cobalamin family of compounds. Hydroxocobalamin is the form of vitamin B12 produced by many bacteria which are used to produce the vitamin commercially. Like other forms of vitamin B12, hydroxocobalamin has an...
or methylcobalamin. Cyanocobalamin deficiency is very common in cats with EPI because about 99 percent of intrinsic factor
Intrinsic factor
Intrinsic factor also known as gastric intrinsic factor is a glycoprotein produced by the parietal cells of the stomach. It is necessary for the absorption of vitamin B12 later on in the small intestine...
(which is required for cyanocobalamin absorption from the intestine) is secreted by the pancreas. In dogs this figure is about 90 percent, and only about 50 percent of dogs have this deficiency. Cats may suffer from Vitamin K
Vitamin K
Vitamin K is a group of structurally similar, fat soluble vitamins that are needed for the posttranslational modification of certain proteins required for blood coagulation and in metabolic pathways in bone and other tissue. They are 2-methyl-1,4-naphthoquinone derivatives...
deficiencies. If there is bacterial overgrowth
Small bowel bacterial overgrowth syndrome
Small bowel bacterial overgrowth syndrome , or small intestinal bacterial overgrowth , also termed bacterial overgrowth; is a disorder of excessive bacterial growth in the small intestine. Unlike the colon , which is rich with bacteria, the small bowel usually has less than 104 organisms per...
in the intestine, antibiotics should be used, especially if treatment is not working. In dogs failing to gain weight or continuing to show symptoms, modifying the diet to make it low fiber and highly digestible may help. Despite previous belief that low fat diets are beneficial in dogs with EPI, more recent studies have shown that a high fat diet may increase absorption of nutrients and better manage the disease. However, it has been shown that different dogs respond to different dietary modifications, so the best diet must be determined on a case by case basis.