Malabsorption of nutrients in cystic fibrosis (CF) has a multifactorial origin. The factors involved in malabsorption include malfunction of the exocrine pancreas and liver, bile acid metabolism, and disordered intestinal resorptive processes. Therapeutic measures presently employed are only partially effective.
What are the causes of malabsorption of nutrients in cystic fibrosis (CF)?
Malabsorption of nutrients in cystic fibrosis (CF) has a multifactorial origin. The factors involved in malabsorption include malfunction of the exocrine pancreas and liver, bile acid metabolism, and disordered intestinal resorptive processes.
What causes malabsorption of nutrients?
Some gastrointestinal diseases, such as celiac disease and inflammatory bowel disease, cause general malabsorption of all kinds of nutrients. In other cases, you may have particular difficulties absorbing a particular kind of nutrient. Some of these types include:
What causes cystic fibrosis (CF)?
In those who do have cystic fibrosis, the symptoms of the disease are caused through some kind of mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which is responsible for creating a particular protein in cells.
What is fat malabsorption?
Fat malabsorption also leads to the malabsorption of fat-soluble vitamins (A, D, E and K). Sometimes fat malabsorption results from a lack of bile from gallbladder diseases, bile ducts or liver.
What causes malabsorption in cystic fibrosis?
Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes, but bicarbonate deficiency, abnormalities of bile salts, mucosal transport and motility, and anatomical structural changes are other contributory factors.
How does cystic fibrosis cause maldigestion and malabsorption?
A normal pancreas is decorated with CFTR, but a deficiency of functional CFTR in cystic fibrosis leads to an altered pH and poor chloride ion secretion. As a result, cystic fibrosis patients suffer from exocrine pancreatic insufficiency, which leads to maldigestion and malabsorption.
How does cystic fibrosis affect nutrition?
Cystic fibrosis is a disease that causes thick, sticky mucus to build up in your lungs and digestive tract. You need to eat high-calorie, high-protein foods throughout the day to maintain a normal weight because your disease makes it hard to get enough calories from the foods you eat.
What nutrient deficiencies affect cystic fibrosis?
Background: Patients with cystic fibrosis (CF) are at risk of developing vitamin K deficiency because of pancreatic insufficiency, hepatobiliary disease, or both.
How does CF contribute to malabsorption of nutrients in a teenage?
CF often causes a condition called pancreatic insufficiency. This means the enzymes made in the pancreas that digest fat, carbohydrates, and protein don't pass into the intestines as they should. So the body can't digest food normally. Teens with pancreatic insufficiency might have problems with growth and weight gain.
What causes the body not to absorb nutrients?
Factors that may cause malabsorption syndrome include: damage to the intestine from infection, inflammation, trauma (injury), or surgery. prolonged use of antibiotics. other conditions such as celiac disease, Crohn's disease, chronic pancreatitis, or cystic fibrosis.
How does cystic fibrosis affect digestion?
In CF, the mucus is often thick and sticky. that blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. Undigested food in the intestines can cause pain, cramping, gas and either loose, greasy, floating stools or constipation. and blockages.
How does cystic fibrosis cause vitamin E deficiency?
Approximately 85% to 90% of people with cystic fibrosis do not produce enough enzymes in their pancreas and are not able to absorb fat when digesting food. These individuals are also likely to have problems absorbing the fat‐soluble vitamins A, D, E and K.
What vitamin deficiency causes cystic fibrosis?
Vitamin D deficiency occurs frequently in patients with cystic fibrosis (CF). Vitamin D is important for optimal mineralization of bone and may be important for other comorbidities commonly occurring in patients with CF.
Why does someone with CF need supplemental vitamins such as vitamin K?
In people with cystic fibrosis, the pancreas often does not produce enough enzymes to allow the body to absorb digested food properly and this may also be linked to deficiencies of fat-soluble vitamins like vitamin K. Vitamin K is needed for adequate blood clotting, bone formation and some metabolic functions.
Why does CF cause malnutrition?
Gastrointestinal problems like malnutrition tend to occur in people with CF because the thick mucus generated by cystic fibrosis clogs the ducts (tubes) leading in and out of the pancreas, liver, and intestines. These blockages mean the pancreas cannot push enough enzymes into the intestines for food to be digested properly.
Why is malnutrition bad for cystic fibrosis?
Malnutrition is especially problematic for people with cystic fibrosis because they tend to burn more calories due to the extra work it takes for them to breath and fight infections. In fact, people with CF need up to twice the daily recommended calories of someone of the same age, height and weight. 3.
How to tell if you have cystic fibrosis?
The symptoms of malnutrition in people with cystic fibrosis may be similar or overlap other signs of gastrointestinal problems. These symptoms include: 1 Poor weight gain or slow growth in children 2 Trouble keeping weight on in adults and children 3 Low energy, tiredness 4 Difficultly concentrating 5 Lack of appetite 6 Excessive gas 7 Frequent, large, greasy, bad-smelling stools 8 Abdominal pain or stomachache 3,4
Why does mucus stop the pancreas from sending enzymes into the intestines?
Thick mucus stops the pancreas from sending enzymes into the intestines, which are needed for the body to absorb nutrients in food. A defect in the intestines prevents nutrients from passing into the bloodstream. People with CF mostly have a problem breaking down and using proteins and fats rather than carbohydrates.
Why does my stomach feel full?
The problems of malabsorption are compounded by delayed gastric emptying, which is when the stomach empties more slowly than average. Delayed gastric emptying causes one to feel full more quickly and is quite common among people with CF. Malnutrition is especially problematic for people with cystic fibrosis because they tend to burn more calories ...
What can a dietitian do for cystic fibrosis?
A dietitian who specializes in helping people with cystic fibrosis can look closely at a person’s diet and help make recommendations to increase calorie and nutrition intake. People with CF are also usually encouraged to eat as much as they’d like of high-calorie, high-fat foods, along with plenty of nutrient-dense fruits and vegetables. 3,4
What is it called when the body can't absorb nutrients?
Malabsorption leads to malnutrition. When the body can’t absorb nutrients, it is called malabsorption. Malabsorption means the body is not getting the nutrients it needs, which leads to malnutrition and trouble gaining or keeping on weight.
What causes a CF to be malabsorption?
The factors involved in malabsorption include malfunction of the exocrine pancreas and liver, bile acid metabolism, and disordered intestinal resorptive processes.
What is the best way to improve fat malabsorption?
Improvement of fat malabsorption is attained by using a pancreatic enzyme supplement consisting of pH-sensitive, enteric-coated microspheres ( microsphere preparations) that prevent enzyme degradation in the stomach and travel with the chyme to the small intestine.
Does microsphere help with bile salt?
Microsphere preparations, however, do not improve bile salt deficiency. The detergent Tween-80, given orally to simulate bile salt activity, does not improve fat absorption. The mucus viscosity is probably enhanced in the intestinal epithelium of CF patients and can be decreased by N-acetylcysteine, which breaks down sulfide bonds.
What are the symptoms of malabsorption?
Other symptoms of malabsorption include greasy, bulky stools, stomachache, and excessive gas. 1-4
Why do people with cystic fibrosis burn more calories?
In addition to malabsorption, people with cystic fibrosis often burn more calories due to the extra energy it takes to breathe and fight infections . In fact, people with CF need up to twice the daily recommended calories compared to other people of the same age and weight. 1-3.
What is PERT in CF?
Pancreatic enzyme replacements (PERT) First, pancreatic enzyme replacements (PERT) help the person with CF process their foods more efficiently so they can absorb the nutrients in the food they eat. This allows them to retain more energy from the foods they consume.
What is the side effect of cystic fibrosis?
Poor growth in children and trouble keeping weight on during adulthood are symptoms of malabsorption, a common side effect of cystic fibrosis.
Can cystic fibrosis cause fatigue?
Eating fatigue is a common complaint for people with CF. Even if a person with cystic fibrosis feels hungry and eats large amounts of food, it may still not be enough to combat malabsorption.
What causes malabsorption syndrome?
Factors that may cause malabsorption syndrome include: damage to the intestine from infection, inflammation, trauma, or surgery. prolonged use of antibiotics. other conditions such as celiac disease, Crohn’s disease, chronic pancreatitis, or cystic fibrosis. lactase deficiency, or lactose intolerance.
How does malabsorption affect women?
Malabsorption may affect people based on age or gender. For instance, women may stop menstruating, and children may not grow properly. Their weight or rate of weight gain may be significantly below that of other children of a similar age and gender.
Why is my intestine not able to absorb nutrients?
This makes the intestine less able to absorb nutrients. SBS may be a birth defect, or it may be caused by surgery. Certain diseases may cause malabsorption. These include tropical sprue, a condition most common in the Caribbean, India, and other parts of Southeast Asia.
What is the term for the condition where nutrients are not absorbed properly?
Recognizing the symptoms of malabsorption syndrome. Symptoms of malabsorption syndrome are caused when unabsorbed nutrients pass through the digestive tract. Many symptoms differ depending on the specific nutrient or nutrients that aren’t being absorbed properly.
What is malabsorption?
Malabsorption is difficulty in the digestion or absorption of nutrients from food. Malabsorption can affect growth and development, or it can lead to specific illnesses.
Diagnosis and Treatment
Diagnosis and treatment will depend on the specific disease causing the malabsorption.
What are the mechanisms of malabsorption?
Mechanisms of malabsorption with respect to luminal, mucosal, and mural/transport phases. Malabsorption develops when components of the mucosa are disrupted or damaged by a wide variety of injuries or stimuli including infectious, immunologic and inflammatory, and toxic or chemical causes.
What are the most common disorders in infants with malabsorption?
Among the latter, abeta/hypobetalipoproteinemia and chylomicron retention disease are the most commonly encountered disorders in infants with malabsorption. The patients suffer from diarrhea and fat malabsorption with low serum cholesterol and triglycerides and peripheral acanthocytosis. Duodenal mucosa shows lipid vacuolization of surface enterocytes with preserved villous architecture ( Figure 16 (a) – 16 (c) ). It is an autosomal recessive disease characterized by the absence of apolipoprotein B (apoB), the transport protein of dietary fat from the small intestinal mucosa into the bloodstream. Mutations in microsomal triglyceride transfer protein (MTP) located in 4q22–q24 result in defective lipid transfer from endoplasmic reticulum to apoB ( Figure 17 ). SAR1B gene encoding protein carrier GTPase located in 5q31 is mutated in chylomicron retention disease. Lipid assembly is disorganized in both of these disorders resulting in low levels of serum lipids accompanied by various neurological manifestations due to malabsorption of fat-soluble vitamins, particularly vitamins E and A.
What causes tropical sprue?
Tropical sprue results from a nutritional deficiency or a transmissible microorganism and/or a toxin. Malabsorption is usually initiated by an infection followed by colonization of the small bowel by enterotoxigenic bacteria.
What causes WD in the body?
WD is a rare, relapsing chronic, and systemic bacterial infection caused by an actinomycete, Tropheryma whipplei. There is evidence of a host-related and possibly genetically determined increased susceptibility to Whipple's disease related to innate defects in T cells and mononuclear cells. Persistent macrophage dysfunction may predispose to the infection since macrophages are unable to degrade the phagocytosed bacteria. Patients present with malabsorptive diarrhea, arthralgia, lymphadenopathy, and cardiac and central nervous system symptoms. Malabsorption in Whipple's disease is due predominantly to blockage of nutrient movement in the lamina propria and in the mesenteric lymphatic drainage system by PAS-positive macrophages. Lipid accumulation in the mucosa and mesenteric lymph nodes is a prominent feature in the small intestine and led to Whipple's name for the disorder – ‘intestinal lipodystrophy.’ The characteristic feature in the small intestinal biopsy consists of blunted villi with distended lamina propria resulting from the accumulation of foamy histiocytes containing PAS-positive rod-shaped, rounded, or sickle-shaped bacilli.
What causes a decrease in surface area of the small intestine?
Conditions leading to a decrease in surface area of the small intestine are among the leading causes of malabsorption as they also cause shortened transit time and defective exposure to digestive enzymes.
Why does food cause diarrhea?
Undigested food produces diarrhea, due to its voluminous effect in the bowel lumen. Although all three major nutrients (fat, carbohydrate, and protein) may be malabsorbed, clinical symptoms usually only develop with carbohydrate and fat malabsorption.
