What Is Exocrine Pancreatic Insufficiency?

Written by Stephanie Watson

Medically Reviewed by Neha Pathak, MD on September 17, 2021

• What Causes EPI?
• What Are the Symptoms of EPI?
• How Is EPI Diagnosed?
• Questions for Your Doctor
• How Is EPI Treated?
• How to Treat Conditions That Cause EPI
• Is Surgery an Option?
• Taking Care of Yourself
• What to Expect
• Getting Support
• More

Exocrine pancreatic insufficiency (EPI) causes problems in how you digest food. Your pancreas doesn’t make enough of the enzymes that your body needs to break down and absorb nutrients.

Enzymes speed up chemical reactions in your body. The enzymes made by your pancreas move into your small intestine, where they help break down the food you eat.

When you have EPI, you don’t get the nutrition you need because your body can’t absorb fats and some vitamins and minerals from foods. You might lose weight or have pain in your belly.

There are drugs that work for most people that give you a new supply of enzymes, so you can go back to digesting food the right way.

Besides taking medicine, you can manage your symptoms by making sure you follow the right diet. Your doctor will recommend foods that will help you get enough nutrients and protein that you might be missing.

Damage to your pancreas causes EPI. There are many reasons this can happen but some of the most common are:

Your pancreas gets inflamed often. Doctors call this chronic pancreatitis. It happens when the enzymes made by the pancreas start working while they’re still inside it, before they get to the small intestine. You’re at risk for this if you’re a heavy drinker, though there can be other causes as well. For instance, your pancreas could get inflamed if some passageways in it are blocked or if you have very high levels of triglycerides (a type of blood fat) or an immune system disorder.

You’ve had surgery on your pancreas, stomach, or intestines.

You have one of these inherited diseases:

• Cystic fibrosis
• Shwachman-Diamond syndrome

If you have cystic fibrosis, your body makes unusually thick and sticky mucus. This mucus blocks passageways in your pancreas and stops enzymes from getting out.

If you have Shwachman-Diamond syndrome, you may be missing cells in your pancreas that make enzymes.

Crohn’s disease and celiac disease can also lead to EPI in some people.

You may not have any symptoms at first. But once your pancreas gets so damaged that it starts to hurt your ability to absorb fat, you may get some symptoms, such as:

• Pain or tenderness in your belly
• Bad-smelling bowel movements
• Diarrhea
• Gas
• Feeling full

You might also lose weight and get other problems, because your body doesn’t absorb enough vitamins. For instance, you could develop a bleeding disorder if you’re not getting enough vitamin K. Or you could get bone pain if you don’t get enough vitamin D.

Your doctor may diagnose your condition by checking your symptoms. They may ask you questions such as:

• Do you have pain in your upper belly?
• Have you had bad-smelling bowel movements that are oily and hard to flush down the toilet?
• Do you have gas or diarrhea?
• Have you lost weight?

Several tests can help diagnose EPI. First, you may need some blood tests that check to see if you’re getting enough vitamins and that your pancreas is making enough enzymes. Other blood tests can check for things that can lead to EPI, like celiac disease.

You may also need to take the “3-day fecal test.” It checks for the amount of fat in your bowel movements. You’ll need to collect samples of your stool in special containers for 3 days.

Your doctor may also ask you to take a test called “fecal elastase-1.” For this, you also need to collect a sample of your bowel movement in a container. It will be sent to a lab to look for an enzyme that’s important in digestion. The test can tell you if your pancreas is making enough of it.

You may also need to get some tests that check to see if your pancreas is inflamed, including:

• CT scan. This uses a powerful X-ray to make detailed pictures inside your body.
• MRI. It uses strong magnets and radio waves to make pictures of organs and structures inside your body.
• Endoscopic ultrasound. This test uses sound waves to take pictures inside your digestive system. The sound waves are sent out by a thin tube that your doctor places through your mouth into your digestive system.

When you find out you’ve got EPI, you’ll probably have a lot of questions. You may want to start by asking your doctor:

• What treatments do you recommend?
• Do I need to follow a special diet?
• Are there vitamins I should take?
• Can I drink alcohol?
• What can I do if I’m losing weight?

Apart from a healthy diet, the main treatment for EPI is pancreatic enzyme replacement therapy (PERT). You take prescription pills that replace the enzymes your pancreas isn’t making.

These enzymes break down your food so you can more easily digest and absorb it. You have to take them during your meals. If you take them before you eat, the replacement enzymes may move through your stomach before your food gets there. If you take the pills after you eat, you have the opposite problem.

You may also need to take an antacid to keep your stomach from breaking down pancreatic enzymes before they can start to work.

There are six FDA-approved pancreatic enzyme products that are only available by prescription:

• Creon
• Pancreaze
• Pertzye
• Ultresa
• Viokace
• Zenpep

If your doctor prescribes this medication, take it at the start of meals or before you eat snacks, along with a liquid like water. Don’t dissolve the pill in a liquid like milk or take it with any over-the-counter stomach acid medicine that has calcium or magnesium. These products can break down the coating and enzymes in your pills.

The amount you take depends on your body weight. You’ll start with the lowest possible dosage and take more if you need it.

You may also take drugs to lower stomach acid along with your PERT. Your doctor can prescribe these, and they’re also available over the counter:

• Proton pump inhibitors like esomeprazole or omeprazole
• h3 blockers like cimetidine, famotidine, or ranitidine

You may also need medicine to treat pain. If your doctor says it’s okay, you may take over-the-counter pain medicines such as acetaminophen (Tylenol) or ibuprofen (Advil, Motrin) on occasion. If those don’t bring you relief, your doctor may prescribe stronger pain drugs, such as hydrocodone and oxycodone. Keep in mind that ibuprofen may lead to internal bleeding in the digestive tract and that drugs such as hydrocodone and oxycodone should be used with caution because of addiction potential.

Emotional stress can also trigger pancreas inflammation. Tricyclic antidepressants like amitriptyline or nortriptyline may help ease pain. Gabapentin (Gralise, Neurontin), a drug that helps control seizures, can also help fight EPI pain. Pregabalin (Lyrica), which is used to treat seizures and nerve pain, also shows promise.

You also can treat the health problem that causes your EPI, like cystic fibrosis, Shwachman-Diamond syndrome, or chronic pancreatitis.

Cystic fibrosis. Treatments include enzyme replacement therapy, antibiotics, laxatives, and enemas. You can also eat a high-calorie, high-fat diet or take supplements to get the nutrition you need.

If you have cystic fibrosis and EPI, you may also get diabetes. Keep your blood sugar levels under control, and take insulin or other medications if your doctor prescribes them.

Shwachman-Diamond syndrome. Your doctor may prescribe PERTs, a high-fat and high-calorie diet, and vitamins and supplements. Scientists are also working to see if stem cell transplants will treat this genetic disease.

Chronic pancreatitis. If you have alcoholic pancreatitis, it’s important to stop drinking. You may need to enter a treatment program or work with a counselor to stop. If you smoke, stop. If there are stones blocking your ducts, the doctor can remove them. If you have a systemic illness like lupus or cystic fibrosis, treatment for the underlying condition may help the chronic pancreatitis.

Surgery can open ducts that are clogged or blocked by gallstones, and decompression can widen a main pancreatic duct that’s too narrow.

Another option is to remove your pancreas and give you an autologous islet cell transplant. These are cells from your own body that make insulin. The doctor will get them into your body through a vein in your liver.

This surgery can ease severe chronic pancreatitis pain or prevent or ease diabetes caused by chronic pancreatitis. But it’s only used if other treatments haven’t worked.

If other treatments fail, the doctor might remove a portion of your pancreas, but this is usually a last resort.

The right diet is very important for managing EPI. A dietitian can help you choose the foods that keep your energy level up and give you the nutrition you need. Here are a few tips:

• Eat six small meals per day. Try that instead of the traditional three. A big meal might not be appealing if you have digestion troubles from EPI.
• Don’t drink or smoke. Alcohol can make it even harder for your body to absorb fat, and can damage your pancreas over time. Alcoholism is one possible cause of EPI. Smoking can lead to calcium buildup in your pancreas.
• Take vitamins. You may need to take vitamins A, D, E, and K to replace ones that aren’t getting absorbed from your diet. EPI makes it hard for you to get the right nutrients because your body can’t break down your foods. You also probably don’t take in enough fats. Your doctor can prescribe vitamin supplements to help you get the right levels of these nutrients. They can also tell you which over-the-counter supplements and what dosage to take.

You can manage the symptoms of EPI by taking enzyme replacements and following an eating plan that gives you the right nutrition. Make sure you get the advice of a dietitian or nutritionist. One of your big challenges is to make sure you don’t lose weight. A nutritionist can help you choose foods that have enough protein and nutrients.

Also, talk with your family and friends to get the support you need while you’re getting treatment. Ask your doctor about support groups, which let you talk to others who are going through the same things you are.

You can find more information about pancreas problems by visiting the web site of the National Pancreas Foundation.

You can also learn more by visiting the web sites of the Cystic Fibrosis Foundation and the Shwachman-Diamond Syndrome Foundation.

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What Is Exocrine Pancreatic Insufficiency?

Written by Stephanie Watson

Medically Reviewed by Neha Pathak, MD on September 17, 2021

• What Causes EPI?
• What Are the Symptoms of EPI?
• How Is EPI Diagnosed?
• Questions for Your Doctor
• How Is EPI Treated?
• How to Treat Conditions That Cause EPI
• Is Surgery an Option?
• Taking Care of Yourself
• What to Expect
• Getting Support
• More

Exocrine pancreatic insufficiency (EPI) causes problems in how you digest food. Your pancreas doesn’t make enough of the enzymes that your body needs to break down and absorb nutrients.

Enzymes speed up chemical reactions in your body. The enzymes made by your pancreas move into your small intestine, where they help break down the food you eat.

When you have EPI, you don’t get the nutrition you need because your body can’t absorb fats and some vitamins and minerals from foods. You might lose weight or have pain in your belly.

There are drugs that work for most people that give you a new supply of enzymes, so you can go back to digesting food the right way.

Besides taking medicine, you can manage your symptoms by making sure you follow the right diet. Your doctor will recommend foods that will help you get enough nutrients and protein that you might be missing.

Damage to your pancreas causes EPI. There are many reasons this can happen but some of the most common are:

Your pancreas gets inflamed often. Doctors call this chronic pancreatitis. It happens when the enzymes made by the pancreas start working while they’re still inside it, before they get to the small intestine. You’re at risk for this if you’re a heavy drinker, though there can be other causes as well. For instance, your pancreas could get inflamed if some passageways in it are blocked or if you have very high levels of triglycerides (a type of blood fat) or an immune system disorder.

You’ve had surgery on your pancreas, stomach, or intestines.

You have one of these inherited diseases:

• Cystic fibrosis
• Shwachman-Diamond syndrome

If you have cystic fibrosis, your body makes unusually thick and sticky mucus. This mucus blocks passageways in your pancreas and stops enzymes from getting out.

If you have Shwachman-Diamond syndrome, you may be missing cells in your pancreas that make enzymes.

Crohn’s disease and celiac disease can also lead to EPI in some people.

You may not have any symptoms at first. But once your pancreas gets so damaged that it starts to hurt your ability to absorb fat, you may get some symptoms, such as:

• Pain or tenderness in your belly
• Bad-smelling bowel movements
• Diarrhea
• Gas
• Feeling full

You might also lose weight and get other problems, because your body doesn’t absorb enough vitamins. For instance, you could develop a bleeding disorder if you’re not getting enough vitamin K. Or you could get bone pain if you don’t get enough vitamin D.

Your doctor may diagnose your condition by checking your symptoms. They may ask you questions such as:

• Do you have pain in your upper belly?
• Have you had bad-smelling bowel movements that are oily and hard to flush down the toilet?
• Do you have gas or diarrhea?
• Have you lost weight?

Several tests can help diagnose EPI. First, you may need some blood tests that check to see if you’re getting enough vitamins and that your pancreas is making enough enzymes. Other blood tests can check for things that can lead to EPI, like celiac disease.

You may also need to take the “3-day fecal test.” It checks for the amount of fat in your bowel movements. You’ll need to collect samples of your stool in special containers for 3 days.

Your doctor may also ask you to take a test called “fecal elastase-1.” For this, you also need to collect a sample of your bowel movement in a container. It will be sent to a lab to look for an enzyme that’s important in digestion. The test can tell you if your pancreas is making enough of it.

You may also need to get some tests that check to see if your pancreas is inflamed, including:

• CT scan. This uses a powerful X-ray to make detailed pictures inside your body.
• MRI. It uses strong magnets and radio waves to make pictures of organs and structures inside your body.
• Endoscopic ultrasound. This test uses sound waves to take pictures inside your digestive system. The sound waves are sent out by a thin tube that your doctor places through your mouth into your digestive system.

When you find out you’ve got EPI, you’ll probably have a lot of questions. You may want to start by asking your doctor:

• What treatments do you recommend?
• Do I need to follow a special diet?
• Are there vitamins I should take?
• Can I drink alcohol?
• What can I do if I’m losing weight?

Apart from a healthy diet, the main treatment for EPI is pancreatic enzyme replacement therapy (PERT). You take prescription pills that replace the enzymes your pancreas isn’t making.

These enzymes break down your food so you can more easily digest and absorb it. You have to take them during your meals. If you take them before you eat, the replacement enzymes may move through your stomach before your food gets there. If you take the pills after you eat, you have the opposite problem.

You may also need to take an antacid to keep your stomach from breaking down pancreatic enzymes before they can start to work.

There are six FDA-approved pancreatic enzyme products that are only available by prescription:

• Creon
• Pancreaze
• Pertzye
• Ultresa
• Viokace
• Zenpep

If your doctor prescribes this medication, take it at the start of meals or before you eat snacks, along with a liquid like water. Don’t dissolve the pill in a liquid like milk or take it with any over-the-counter stomach acid medicine that has calcium or magnesium. These products can break down the coating and enzymes in your pills.

The amount you take depends on your body weight. You’ll start with the lowest possible dosage and take more if you need it.

You may also take drugs to lower stomach acid along with your PERT. Your doctor can prescribe these, and they’re also available over the counter:

• Proton pump inhibitors like esomeprazole or omeprazole
• h3 blockers like cimetidine, famotidine, or ranitidine

You may also need medicine to treat pain. If your doctor says it’s okay, you may take over-the-counter pain medicines such as acetaminophen (Tylenol) or ibuprofen (Advil, Motrin) on occasion. If those don’t bring you relief, your doctor may prescribe stronger pain drugs, such as hydrocodone and oxycodone. Keep in mind that ibuprofen may lead to internal bleeding in the digestive tract and that drugs such as hydrocodone and oxycodone should be used with caution because of addiction potential.

Emotional stress can also trigger pancreas inflammation. Tricyclic antidepressants like amitriptyline or nortriptyline may help ease pain. Gabapentin (Gralise, Neurontin), a drug that helps control seizures, can also help fight EPI pain. Pregabalin (Lyrica), which is used to treat seizures and nerve pain, also shows promise.

You also can treat the health problem that causes your EPI, like cystic fibrosis, Shwachman-Diamond syndrome, or chronic pancreatitis.

Cystic fibrosis. Treatments include enzyme replacement therapy, antibiotics, laxatives, and enemas. You can also eat a high-calorie, high-fat diet or take supplements to get the nutrition you need.

If you have cystic fibrosis and EPI, you may also get diabetes. Keep your blood sugar levels under control, and take insulin or other medications if your doctor prescribes them.

Shwachman-Diamond syndrome. Your doctor may prescribe PERTs, a high-fat and high-calorie diet, and vitamins and supplements. Scientists are also working to see if stem cell transplants will treat this genetic disease.

Chronic pancreatitis. If you have alcoholic pancreatitis, it’s important to stop drinking. You may need to enter a treatment program or work with a counselor to stop. If you smoke, stop. If there are stones blocking your ducts, the doctor can remove them. If you have a systemic illness like lupus or cystic fibrosis, treatment for the underlying condition may help the chronic pancreatitis.

Surgery can open ducts that are clogged or blocked by gallstones, and decompression can widen a main pancreatic duct that’s too narrow.

Another option is to remove your pancreas and give you an autologous islet cell transplant. These are cells from your own body that make insulin. The doctor will get them into your body through a vein in your liver.

This surgery can ease severe chronic pancreatitis pain or prevent or ease diabetes caused by chronic pancreatitis. But it’s only used if other treatments haven’t worked.

If other treatments fail, the doctor might remove a portion of your pancreas, but this is usually a last resort.

The right diet is very important for managing EPI. A dietitian can help you choose the foods that keep your energy level up and give you the nutrition you need. Here are a few tips:

• Eat six small meals per day. Try that instead of the traditional three. A big meal might not be appealing if you have digestion troubles from EPI.
• Don’t drink or smoke. Alcohol can make it even harder for your body to absorb fat, and can damage your pancreas over time. Alcoholism is one possible cause of EPI. Smoking can lead to calcium buildup in your pancreas.
• Take vitamins. You may need to take vitamins A, D, E, and K to replace ones that aren’t getting absorbed from your diet. EPI makes it hard for you to get the right nutrients because your body can’t break down your foods. You also probably don’t take in enough fats. Your doctor can prescribe vitamin supplements to help you get the right levels of these nutrients. They can also tell you which over-the-counter supplements and what dosage to take.

You can manage the symptoms of EPI by taking enzyme replacements and following an eating plan that gives you the right nutrition. Make sure you get the advice of a dietitian or nutritionist. One of your big challenges is to make sure you don’t lose weight. A nutritionist can help you choose foods that have enough protein and nutrients.

Also, talk with your family and friends to get the support you need while you’re getting treatment. Ask your doctor about support groups, which let you talk to others who are going through the same things you are.

You can find more information about pancreas problems by visiting the web site of the National Pancreas Foundation.

You can also learn more by visiting the web sites of the Cystic Fibrosis Foundation and the Shwachman-Diamond Syndrome Foundation.

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Pancreatic Enzyme Deficiency: Symptoms, Causes, and Treatment

Table of Contents

• 1 Pancreatic Enzyme Deficiency: Causes, Symptoms, and Treatment
  • 1. 1 Overview of the Disease
    • 1.1.1 What is the Disease?
  • 1.2 Pancreatic enzyme deficiency: what are the symptoms?
  • 1.3 Why does pancreatic enzyme deficiency occur?
  • 1.4 How is pancreatic enzyme deficiency diagnosed?
    • 1.4.1 Fecal fat test
    • 1.4.2 Blood enzyme test
    • 1.4.3 Ultrasound of the pancreas
    • 1.4.4 Duodenal intubacja
  • 1.5 Treatment of pancreatic enzyme deficiency
    • 1.5.1 Replacement enzyme therapy
    • 1.5.2 Dietary advice
    • 1.5.3 Management of complications
  • 1.6 Replacement therapy for pancreatic enzyme deficiency
    • 1.6.1 What is substitution therapy?
  • 1.7 Medicines for replacement therapy in pancreatic enzyme deficiency
  • 1.8 How to eat properly in pancreatic enzyme deficiency?
    • 1.8.1 Avoid fatty and heavy foods
    • 1.8.2 Eat small meals
    • 1.8.3 Increase your intake of enzymes
    • 1. 8.4 Avoid high cooked carbohydrates

  90 005 1.9Complications of pancreatic enzyme deficiency

  • 1.10 Prevention of pancreatic enzyme deficiency
  • 1.11 Related videos:
  • 1.12 Q&A:
    • • 1.12.0.1 What Do symptoms accompany pancreatic enzyme deficiency?
      • 1.12.0.2 How does the pancreas work and what does enzyme deficiency mean?
      • 1.12.0.3 What causes pancreatic enzyme deficiency?
      • 1.12.0.4 What diagnostic methods are used to detect pancreatic enzyme deficiency?
      • 1.12.0.5 What are the treatments for pancreatic enzyme deficiency?
      • 1.12.0.6 Can pancreatic enzyme deficiency be completely cured?

Learn about the causes and symptoms of pancreatic enzyme deficiency and what foods and supplements can help treat the condition. Consult a specialist for an accurate diagnosis and treatment.

The pancreas is an important organ in our digestive tract that produces the necessary enzymes to break down food. Pancreatic enzyme deficiency is a condition in which the body does not produce enough enzymes to digest food efficiently, which can lead to various health problems.

Symptoms of pancreatic enzyme deficiency may include abdominal pain, bloating, diarrhea, and diarrhoea. These symptoms may worsen after meals, especially those rich in fat, and can seriously affect a person’s quality of life.

Why does pancreatic enzyme deficiency occur? There are many reasons. Some people are born with genetic disorders that result in enzyme deficiencies. Others may develop this condition due to inflammatory diseases of the pancreas such as pancreatitis or cancer. Also, pancreatic enzyme deficiency can be caused by other diseases that damage the gland and prevent it from working properly.

How to treat pancreatic enzyme deficiency? Treatment may include the use of special preparations that contain the missing enzymes and can help the body digest food better. You may also need to change your diet and take other measures to correct the problem.

Brief overview of the disease

What is this disease?

Pancreatic enzyme deficiency is a disease that occurs when the pancreas does not produce enough enzymes to properly digest food.

Pancreatic enzyme deficiency can lead to serious digestive problems such as diarrhea, abdominal pain, nutritional deficiencies, weight loss, etc. Therefore, it is very important to seek medical help and start treatment in a timely manner.

Pancreatic enzyme deficiency: what are the symptoms?

Pancreatic insufficiency may present with various symptoms related directly to the digestive process. One of the most common symptoms is epigastric pain – pain in the upper abdomen that occurs after eating. Pain can be either painful colic or just discomfort.

Another common symptom is gastrointestinal upset, which manifests itself as diarrhea, constipation, or a combination of both. Patients with enzyme deficiency may also suffer from abdominal tension, flatulence and heartburn.

Other symptoms include:

• Loss of appetite
• Weight loss
• Metabolic disorder
• Lack of vitamins and minerals
• Weakness and fatigue 9 0010

Most of the symptoms are related to digestive disorders and can be easily described. If you notice any of these symptoms, it is important to see a doctor for diagnosis and treatment.

Why does pancreatic enzyme deficiency occur?

The pancreas is responsible for the production of essential enzymes that are needed for proper digestion of food in the intestines. Various causes can lead to the development of pancreatic enzyme deficiency.

1. Pancreatitis: is an inflammatory process that can lead to malfunction of the pancreas.
2. Genetic factors: in rare cases, the disease may occur due to hereditary causes.
3. Alcoholic damage to the pancreas: Frequent and large doses of alcohol can damage the tissues of the organ and lead to the development of enzyme deficiency.
4. Gastrointestinal surgery: After some surgeries, the normal function of the pancreas can be disturbed, which can lead to enzyme deficiency.

This is not a complete list of causes of pancreatic enzyme deficiency. Consulting a doctor and conducting an additional examination can help determine the true cause of the disease.

How is pancreatic enzyme deficiency diagnosed?

Fecal fat test

One of the first steps in diagnosing pancreatic enzyme deficiency is a fecal fat test. This test allows you to determine the presence of fat in the stool, which may remain in it due to the fact that the body cannot properly remove it from food.

Enzyme blood test

The second important test is a blood test for pancreatic enzymes such as amylase and lipase. If the levels of these enzymes are below normal, this may indicate a pancreatic enzyme deficiency.

Ultrasound of the pancreas

Ultrasound examination (ultrasound) of the organ can be performed to visually assess the condition of the pancreas. This method allows you to determine the presence of changes in the size, shape and structure of the pancreas.

Duodenal intubacja

Duodenal intubacja may sometimes be required – from time to time, minimally aggressive diagnostics and laboratory tests are violated and diet does not help to cope with problems, therefore a more complex and painful method is used – insertion of a thin probe through the oral cavity into duodenum, which allows you to control the process of secretion of digestive enzymes and determine the cause of their shortage.

Treatment of pancreatic enzyme deficiency

Enzyme replacement therapy

The main treatment for pancreatic enzyme deficiency is enzyme replacement therapy. This means taking special medicines containing enzymes that help the body digest food.

The form of drugs can be different: capsules, tablets, powders. They are taken with food or immediately after a meal so that the enzymes can mix with the food mass and begin to work.

Nutrition advice

Proper nutrition is required in addition to enzyme replacement therapy. It is recommended to eat often, but in small portions and remove fatty, fried, smoked foods from the diet. You should also limit the consumption of alcohol and strong tea and coffee. Failure to follow nutritional recommendations can worsen the condition and provoke an exacerbation of the disease.

Treatment of complications

In some cases, complications of pancreatic enzyme deficiency occur, such as pancreatitis, diabetes. In such cases, the complications themselves are treated.

Pancreatitis may require hospitalization and medication. In diabetes, an appropriate diet and hypoglycemic drugs are prescribed.

Replacement therapy for pancreatic enzyme deficiency

What is replacement therapy?

Replacement therapy is a treatment for pancreatic enzyme deficiency that involves taking artificial enzymes to compensate for the deficiency in the body.

In this case, replacement therapy allows the person to take the necessary enzymes to help the body process food properly, thus preventing potential complications and improving overall health.

• Replacement therapy is available as tablets, capsules, powders and crystals.
• Enzyme deficiencies can be caused by various causes, including pancreatic disease, chronic stomach and intestinal disorders, and hereditary predisposition.
• The intake of enzymes should be carried out on the recommendation of a doctor, taking into account the individual characteristics of the patient and the degree of impairment of the functionality of the pancreas.

Pancreatic Enzyme Deficiency Medicines

Pancreatic Enzymes are the main type of medicines used for pancreatic enzyme replacement therapy. These medicines contain enzymes necessary for the normal digestion of food. Among the popular brands on the market are Creon, Pancreatin, Mezim Forte, Festal and others.

Dosages and prescriptions for pancreatic enzymes will depend on the specific situation and degree of fermentation deficiency. Some patients may require high doses to compensate for deficiency, while others may be advised to use a lower dosage.

Reserve intake of pancreatic enzymes is recommended after meals. The patient should understand that the period of taking the tablets may be delayed when using medications in an acute form. In each case, it is better to consult a doctor to find out the optimal dosage and method of using the necessary medicines.

Delivery times and contraindications – vary by manufacturer. However, a particular patient may experience side effects such as nausea, dyspepsia, and diarrhea. The occurrence of any side effects, as well as the possibility of using medications in general, should be discussed with your doctor.

How to eat properly with pancreatic enzyme deficiency?

Avoid fatty and heavy foods

In pancreatic enzyme deficiency, the body cannot digest fats and proteins fast enough. Avoid fatty and heavy foods, which increase the load on the pancreas. Limit your intake of fatty dairy products, meats, and fast food. Instead, choose foods rich in carbohydrates and proteins, such as fruits, vegetables, fish, and high-quality dairy products.

Eat small meals

In pancreatic enzyme deficiency, the stomach may not be able to handle large amounts of food. It is recommended to eat in small portions and often throughout the day. Take care of your stomach, reduce portion sizes and eat them slowly. This will help reduce the load on the pancreas and cope with the digestion of food more efficiently.

Increase your enzyme intake

In pancreatic enzyme deficiency, the body does not produce enough enzymes to process food. Taking enzymes can help improve food digestion. Ask your doctor to recommend specific enzymes and dosages for your condition. The use of enzymes is necessary during meals.

Avoid highly cooked carbohydrates

Eat cereals, whole grain breads and vegetables. Starches such as potatoes and white bread should be avoided as they can lead to high blood sugar levels. Highly cooked carbohydrates can lead to elevated blood glucose levels without the proper involvement of the pancreas.

Pay attention to your diet for pancreatic enzyme deficiency. Follow recommendations such as avoiding fatty foods, eating small meals, increasing your enzyme intake, and avoiding highly cooked carbohydrates to keep your pancreas healthy and reduce the risk of complications.

Complications of pancreatic enzyme deficiency

Pancreatic enzyme deficiency can lead to various complications associated with poor digestion. A lack of enzymes produced by the pancreas can lead to malabsorption of nutrients from food, leading to vitamin and mineral deficiencies in the body.

Possible complications of enzyme deficiency include diarrhea, low weight, anemia, metabolic disorders, osteoporosis, and other nutritional deficiencies.

In addition, insufficient digestion can lead to the development of dysbacteriosis, since the intestinal microflora is disturbed in conditions of insufficient digestion. This can lead to the development of infections and inflammatory diseases.

It is important to know that complications of enzyme deficiency can be associated not only with digestive disorders themselves, but also with those diseases that provoked the appearance of enzyme deficiency. For example, people with celiac disease can experience severe nutritional deficiencies, including enzyme deficiencies, as a result of the loss of the ability to absorb gluten.

Prevention of pancreatic enzyme deficiency

Improper diet and lifestyle can lead to pancreatic dysfunction and the development of enzyme deficiency. Therefore, the main step in prevention is the right choice of food.

• Eliminate from the diet fatty, fried, smoked and spicy foods, as well as foods containing a large amount of preservatives and dyes. These foods can cause damage to pancreatic cells.
• Include in the diet foods containing easily digestible proteins, vitamins and minerals. It can be lean meats, fish, dairy products, fruits and vegetables.
• Moderate exercise will help strengthen the immune system and improve the functioning of the whole organism, including the pancreas.
• Give up bad habits – smoking and drinking alcohol. These substances can cause damage to pancreatic tissue.

If there is a genetic predisposition to the development of enzyme deficiency, it is necessary to pay special attention to prevention and regularly undergo examinations by a gastroenterologist.

Related videos:

Q&A:

What are the symptoms associated with pancreatic enzyme deficiency?

The main symptoms of this disease are abdominal pain, vomiting, diarrhea or constipation, signs of weight loss and weakness, etc.

How does the pancreas work and what does enzyme deficiency mean?

The pancreas produces enzymes that help break down food components. Enzyme deficiency means that the pancreas does not produce enough enzymes to properly break down food.

What causes pancreatic enzyme deficiency?

The causes of pancreatic enzyme deficiency can be varied, including alcohol disease, chronic pancreatitis, genetic disorders, gastrointestinal surgery, etc.

What diagnostic methods are used to detect pancreatic enzyme deficiency?

Doctors usually use tests of blood, urine, and feces for enzyme levels, as well as computed tomography and x-rays of the stomach and intestines, to diagnose pancreatic enzyme deficiency.

What are the treatments for pancreatic enzyme deficiency?

The main method of treatment is the replacement of enzymes, which are necessary for the normal functioning of the gastrointestinal tract. Some patients may also need a special diet and treatment for secondary problems such as diabetes.

Can pancreatic enzyme deficiency be completely cured?

No, there is no cure for pancreatic enzyme deficiency. However, with the right treatment and control of the disease state, a significant improvement in the patient’s life can be achieved.

Clinical case of exocrine pancreatic insufficiency in a patient with type 1 diabetes mellitus | Ragimov

RELEVANCE

The pancreas (PG) belongs to the glands of mixed secretion and simultaneously performs both endo- and exocrine functions. There is a close anatomical and physiological interaction between the exocrine and endocrine “parts” of the pancreas, so a violation of one of the functions will always be reflected in the other.

Manifestations of exocrine pancreatic insufficiency (EPI), especially in diabetes mellitus (DM), in foreign literature are characterized by the term exocrine pancreatic insufficiency (EPI). EPI is the general name for the process of malabsorption caused by inadequate production and decreased activation of pancreatic acinar cell enzymes such as amylase, lipase, and protease necessary for food digestion [1].

In DM, there are several possible causes that can explain the occurrence of EPI, – lack of trophic action of insulin and, possibly, glucagon, as well as somatostatin on acinar cells, autoimmune damage to islet cells, causing destruction of both endocrine and exocrine tissue, fatty infiltration pancreatic tissue or decreased exocrine secretion as a complication of diabetic neuropathy (DN).

Most studies have used pancreatic or fecal elastase-1 (PE-1) concentrations to assess EPI. The prevalence of EPI in patients with type 1 DM (DM1), according to many authors, varies from 25 to 59%, which is determined by the level of PE-1 <200 µg/g of stool. The frequency of detection of EPI in patients with type 2 DM (DM2) or in the control group is less and is 18–29% and 4–13%, respectively. In 11–30% of patients with type 1 diabetes, PE-1 levels were <100 μg/g, indicating severe exocrine insufficiency [2–4].

Differential diagnosis of EPI in DM should be performed with gastrointestinal autonomic DN, chronic pancreatitis (CP), cystic fibrosis, and inflammatory bowel disease.

The sequelae of EPI include malabsorption of fat-soluble vitamins (A, D, E, and K), deficiencies in calcium, folic acid, magnesium, thiamine, and zinc, and a higher incidence of postprandial hypoglycemia compared with patients without EPI.

This should take into account the fact that many patients with DM1 are not examined to assess the exocrine function of the pancreas, and perhaps their number is much higher, especially in the cohort of patients with a long history of DM.

We present a clinical case of confirmed EPI in a patient with a 6-year history of T1DM, which became the main cause of episodes of postprandial hypoglycemia.

CASE DESCRIPTION

In a Caucasian patient, DM was diagnosed in 2014 at the age of 33, against the background of polyuria and polydipsia persisting for 1–2 months. During this period, there was a decrease in body weight up to 7 kg within 2 months. Glycemia in the debut was 19.0 mmol/l. By place of residence due to obesity of the 1st degree (at that time body mass index (BMI) 33. 9kg/m ² ) diagnosed with DM2. In connection with the identified ketonuria, insulin therapy was initiated in the basal-bolus regimen with glargine 100 U/ml at a dose of 20 U at 22:00 and glulisine at 4–12 U before the main meals in a fixed regimen. Subsequently, metformin 1000 mg per day was added to the ongoing therapy. Over the next 5 years, there were no changes in the ongoing hypoglycemic therapy. Throughout the entire period of the disease until 2019, there was no compensation for carbohydrate metabolism, the level of glycated hemoglobin (HbA _1c ) varied within 7.9-10.2% against the background of frequent episodes of hypoglycemia (7-9 times a week). In the winter of 2019, the patient applied on an outpatient basis to the National Research Center for Endocrinology, where the level of autoantibodies (AT) to the components of islet cells — AT to zinc transporter 8 (ZnT8), tyrosine phosphatase (IA-2), glutamate decarboxylase (GAD), surface antigens (ICA) and C-peptide (Table 1). Abs to ICA, IA-2 were within the reference values, the level of C-peptide was below normal, a positive level of Abs to GAD, ZnT8 was detected, on the basis of which it was decided to change the diagnosis to DM1. Metformin was cancelled. The patient was trained in self-control of glycemia and counting bread units with titration of the dose of insulin.

Table 1. Patient’s medical data from 2019 to 2020

Note: AT is an autoantibody.

Summer 2019The patient was admitted to the Department of the Reference Training Center of the Institute of Diabetes of the Federal State Budgetary Institution National Research Center for Endocrinology in a state of carbohydrate metabolism decompensation (HbA _1c – 8.2%) due to the development of frequent episodes of hypoglycemia (1-3 times a day) after the main meals. In the department, the dose of ultra-short-acting insulin (IRDI) and long-acting insulin was adjusted, the time of the IUD injection was postponed to a later time (5–15 minutes after the end of the meal), however, all the measures taken did not give the expected result, and the abolition of the IUD injection for the main doses food led to persistent hyperglycemia 1. 5–2 hours after it. Of the accompanying complaints, there were episodes of flatulence and heaviness after eating, frequent and unformed stools. The patient was examined by a gastroenterologist. Of the complaints characteristic of the gastrointestinal form of diabetic autonomic polyneuropathy, only a feeling of fullness of the stomach after meals was noted, but dysphagia, abdominal pain, nausea, alternation of diarrhea and constipation, nocturnal diarrhea, pain and heaviness in the right hypochondrium were not detected, and therefore it was decided to look for other causes of this problem. An ultrasound examination (ultrasound) of the pancreas, a study of clinical analysis of feces, the level of pancreatic enzymes: pancreatic amylase, lipase, alpha-amylase, and PE-1 were carried out. After obtaining laboratory data, a decrease in the level of PE-1 to 101 µg/g of feces was revealed (normal > 200 µg/g, severe degree of EPI – less than 100 µg/g) with a normal level (within the reference values) of other studied enzymes – lipase, pancreatic amylase, alpha-amylase. According to the results of clinical analysis of feces, a violation of the shape and structure of feces, the presence of a small amount of neutral fat, fiber and starch in it was revealed. According to the ultrasound of the pancreas, diffuse changes in the organ were determined, but there were no signs characteristic of the manifestations of chronic pancreatitis. Based on the data obtained, the gastroenterologist established EPI, and it was decided to prescribe enzyme replacement therapy (pancreatin) for each main meal: immediately before a meal – 10,000 U and immediately after a meal – 25,000 U. Intestinal decontamination was also performed using topical intestinal antiseptics in order to eliminate the possible “enzyme escape syndrome”, which had a favorable result – a pronounced reduction in episodes of hypoglycemia. Subsequently, long-term use of pancreatin replacement therapy in the amount of 25,000–40,000 units for main meals and 10,000–25,000 units for snacks was recommended.

In September 2019, additional examination methods were carried out – MRI of the pancreas, determination of antibodies detected in autoimmune exocrinopathy and autoimmune pancreatitis – to lactoferrin (A-LF), IgG4, as well as pancreatic enzymes not used in routine practice, – alpha-amylase-2 (A-A-2), bile salt-dependent lipase (JSZL), carbonic anhydrase-2 (CA-2), vitamin D – 25 (OH) D, antibodies to the parietal cells of the stomach. The volume of the pancreas (PV) filed by MRI was 37.1 ml with a BMI of 29.4 kg / m ² and body surface area (BSA) 1.94 m ² and 1.26 ml/kg/m ² , respectively. The patient had a slightly elevated level of A-LF. AT to IgG4 — within normal limits. A decrease in the level of YSZL and KA-2 enzymes was noted, the level of A-A-2 was within normal limits.

In December 2020, against the background of continuous pancreatin intake for 6 months, the patient was recommended to take enzymes in a maintenance regimen – 10,000–25,000 IU, subject to abundant meals rich in fats. Against the background of satisfactory compensation of carbohydrate metabolism (HbA _1c 6.9%), primarily due to the decrease in episodes of hypoglycemia, repeated control of the level of PE-1 was carried out, which amounted to 216 µg/g feces. Associated symptoms almost completely disappeared.

DISCUSSION

Violation of the exocrine secretion of the pancreas is often associated with the presence of DM, and in particular DM1.

PE-1 is a marker for pancreatic enzyme secretion, not EPI per se. It does not allow to differentiate primary EPI from secondary. However, the PE-1 level can be used to set the degree of EPI. The current diagnostic reference values ​​for PE-1 are 15, 50, 100, and 200 µg/g [5].

Diagnostic testing using PE-1 has some advantages over other tests due to the absence of the need to maintain a special diet, as well as the high sensitivity of this test in asymptomatic manifestations of EPI. However, there are also disadvantages of this method for studying EPI, characterized by low sensitivity in determining from mild to moderate insufficiency. The presence of EPI is determined at an enzyme level of less than 200 µg/g, and a PE-1 level of less than 100 µg/g requires special attention. The result may be false positive in the analysis of watery stools, so the measurement should be performed on a sample of hard or semi-solid stool [6][7]. Some scientists note that the prevalence of EPI, determined by examining the level of PE-1 in patients with type 1 and type 2 diabetes, is not so pronounced and amounts to 13%. However, EPI was more often determined either in individuals with DM1 or with a long history of DM [5]. The progression of EPI often leads to structural and functional atrophy of the pancreas, manifested by enzymatic deficiency and a decrease in life expectancy. Since the islets make up only 1–2% of the total life expectancy, the decrease in this parameter occurs due to a significant loss of the exocrine tissue of the gland. In addition to direct examination of the material of the pancreas, a decrease in life expectancy was confirmed by a variety of non-invasive imaging methods – ultrasound, computed tomography and MRI in adults and children. According to the results of these research methods, the decrease in life expectancy is 35–45% in the population of patients with a long history of DM1 and about 20–25% during the first year of the disease [8–10]. In the case described by us, the level of PE-1, in combination with a decrease in the level of pancreatic enzymes — KA-2 and ZhSZL and reduced life expectancy, which is normally more than 50 ml in the general population [11], indicates the presence of EPI in a patient with DM1. The reasons for this decline are unknown, and it is likely that some of them originate months or even years before the onset of DM. A possible explanation for the decrease in the size and volume of the pancreas in T1DM includes the loss of insulinotrophic effects by acinar cells as a result of endogenous insulin deficiency, as well as the destruction of exocrine pancreatic tissue as a result of autoimmune exocrinopathy (AE) [12]. AE, according to the authors, is manifested by diffuse lymphocytic infiltration of the exocrine part of the pancreas, which is most common in patients with recent DM1. AE detectable antibodies to lactoferrin and IgG4 were found in 66% and 53% of patients with autoimmune pancreatitis (AIP), respectively. They are also found in 20–65% and 7–67% of adult T1DM patients, respectively, but are relatively rare in children, T2DM patients, or controls [13][14]. In our case, only a slight increase in the level of AT-LF was revealed with a normal level of antibodies to IgG4, which cannot fully reflect the presence of AE or AIP in the patient.

A number of symptoms of EPI are similar to manifestations of the gastrointestinal form of autonomic neuropathy. Symptoms such as fullness after meals, nausea, alternating diarrhea and constipation, and nocturnal diarrhea can often be diagnosed in EPI. EPI can be both a cause and a consequence of diabetic neuropathy [15][16].

In the patient examined by us, the gastrointestinal form of autonomic neuropathy was excluded on the basis of a survey and examination by a gastroenterologist, and a decrease in the level of PE-1 to a level of less than 200 μg/g, as well as the volume and size of the pancreas compared with the data in the general populations [17–19]. The same decrease in the volume and size of the organ occurs in CP. Among the signs characteristic of the manifestations of CP, there is an increase in the echogenicity of the organ, an uneven expansion of the main pancreatic duct, calcifications in the parenchyma, stones in the pancreatic duct, an uneven (“jagged”) contour of the pancreas, pain on palpation and pressure in the area of ​​the projection of the pancreas with an ultrasound sensor, compression large vessels or choledochus, retention cysts or pseudocysts were not detected [20]. In our case, according to ultrasound data, only diffuse changes in the pancreas were noted, other signs were not detected. Given the absence of previously diagnosed CP in history, the presence of this disease in the patient is unlikely.

Enzyme replacement therapy is indicated in the presence of EPI, generally improving the quality of life and favorably influencing the course of other diseases, possibly related. So in our case, the violation of the digestive process caused by EPI in the patient led to episodes of hypoglycemia. When prescribing enzymatic therapy with pancreatin, the outcome is a compensation of carbohydrate metabolism, due to a decrease in the frequency of episodes of hypoglycemia, as well as the absence of discomfort after eating.

The relationship between the exocrine function of the pancreas and DM1 remains unclear at present. However, changes in the exocrine function of the pancreas may precede the onset of T1DM, suggesting that exocrine and endocrine dysfunction may have a similar pathogenesis.

CONCLUSION

EPI was detected in a patient with DM1 during examination and identification of the causes of persistent hypoglycemia. The study of generally accepted enzymes, such as pancreatic amylase, lipase, alpha-amylase, cannot fully reflect the presence and development of EPI in the early stages, and the definition of LE, PE-1, LSZL, KA-2, A-A-2 is not included into routine practice. When such episodes of hypoglycemia occur without an established cause in patients with type 1 diabetes, we recommend that an examination of the exocrine function of the pancreas be performed.

ADDITIONAL INFORMATION

Funding source. The study was funded by the Russian Science Foundation grant No. 17-75-30035 “Autoimmune endocrinopathies with multiple organ lesions: genomic, postgenomic and metabolomic markers. Genetic risk prediction, monitoring, early predictors, personalized correction and rehabilitation.

Consent of the patient. Voluntary informed consent was obtained from the patient for the publication of personal health information.

Conflict of interest. The authors declare no apparent or potential conflicts of interest related to the publication of this article.

Participation of authors. Ragimov M.R., Marchenko E.V. – the concept and design of the study, the collection and processing of materials, the analysis of the data obtained, the writing of the text; Malysheva N.M. — performance of laboratory researches; Derevyanko O.S., Nikonova T.V. — research concept and design, text editing.

All authors approved the final version of the article before publication, agreed to be responsible for all aspects of the work, implying the proper study and resolution of issues related to the accuracy or integrity of any part of the work.

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