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Fat Malabsorption Syndrome

Fat malabsorption syndrome is caused by functional or organic


causes. Symptoms and signs include fatigue, weight changes, steatorrhea,
abdominal distention, cramps, gas, explosive diarrhea with foul smelling
stools, malnutrition and weight loss, and biochemical abnormalities. Under
normal dietary conditions, less than 5% of the ingested fat is observed in the
feces. An increase in this value might indicate fat malabsorption. The
digestive process of fat in the digestive system involves the pancreatic stage,
the biliary stage and the cellular and delivery stage into the small intestines.

A lingual lipase is responsible for the first partial hydrolysis of


triglycerides; this enzyme becomes active in persons with low gastric pH
levels and is active even in premature infants. However, the largest part of
triglyceride digestion is accomplished in the duodenojejunal lumen because
of a complex of pancreatic enzymes, the most important of which is the
lipase–colipase complex. Like amylase, these enzymes also develop slowly,
and this accounts for the known low capacity of babies to absorb lipids,
termed physiologic steatorrhea of the newborn. Additionally, adequate
concentrations of intraluminal conjugated bile salts are needed to form
micelles, and the secretion of bile acids may also be partially inadequate in
very young patients.

Disorders of these processes can be congenital (cystic fibrosis and


Shwachman–Diamond syndrome, which cause lipase and colipase
deficiency; the uncommon isolated deficiency of lipase and colipase; the
extremely rare congenital primary bile acid malabsorption, which results in
low bile acids concentrations) or acquired (secondary mostly to disorders of
the liver and the biliary tract or to chronic pancreatitis).

Some of the conditions which are associated with the malabsorption


of lipids are tabulated as follows:

Stage Conditions
Pancreatic stage • Isolated pancreatic lipase or colipase
deficiency (inherited)
• Pancreatic insufficiency
o Cystic fibrosis
o Chronic pancreatitis
o Obstruction of pancreatic duct
o Pancreatic cancer
o Pancreatic resection
o Shwachman–Diamond syndrome
o Johnson–Blizzard syndrome
o Pearson syndrome
Biliary stage • Decreased bile salt synthesis in severe
hepatic insufficiency
• Decreased delivery of bile salts in biliary
tract obstruction or cholestatic biliary
disease
• Decreased concentration of conjugated
bile salts due to increased acidity, drugs
affecting micelle formation
• Increased intestinal loss of bile salts due to
surgery or diseased terminal ileum
Cellular and delivery stage • Rapid transit, dumping syndrome
• Improper emulsification, following certain
types of gastrectomy
• Zollinger–Ellison syndrome (altered
duodenal pH)
• Decrease in small intestine lymphatics
Defects in multiple stages of • Decreased CCK release due to mucosal
digestions and absorption destruction as in sprue or regional enteritis
(Crohn’s disease)
Conditions associated with malabsorption of lipids (Modified from Tietz. Textbook of Clinical Chemistry)

Apart from the aforementioned conditions fat malabsorption could


also be resultant of abetalipoproteinemia is a rare disorder with autosomal
recessive inheritance. Absence of the lipoproteins results in cytoplasmic
lipid accumulation in the enterocyte. Lymphatic transport of long-chain fats
is impaired in patients with abetalipoproteinemia, lymphangiectasia, and
protein-losing enteropathy, resulting in moderate fat malabsorption.

Individuals with malabsorption syndrome must be monitored for


dehydration (dry tongue, mouth, and skin, increased thirst, low, concentrated
urine output, feeling weak and dizzy when standing). An individual should
be evaluated for nutrient depletion and should be evaluated for signs
including nausea or vomiting, fissures at the corners of mouth, fatigue or
weakness, and dry pluckable hair.

Individuals who have inflammatory bowel disease have an increased


risk of nephrolithiasis; enteric hyperoxaluria is the major risk factor with fat
malabsorptive states, and use of probiotics to decrease oxalate levels is being
studied and considered.

Long term nutritional monitoring is necessary after gastric bypass


surgery for morbid obesity which can lead to fat malabsorption and vitamin
deficiencies for Vitamin A, D, E, and K. In addition, altered calcium
metabolism can lead to decline in bone health. In addition, malabsorption of
dietary and biliary phosphatidylcholine may result in choline deficiency.

To avoid nutritional deterioration, early screening for fat


malabsorption should be recommended using the acid steatocrit, a reliable
and inexpensive test. Intake of 40g of olestra can cause false positive results
on tests for steatorrhea and can lead to an erroneous diagnosis of
malabsorption syndrome. Low total cholesterol (120mg/dL) or low serum
carotene levels may be typical of fat malabsorption but not necessarily
diagnostic. Altered stools characterize different types of malabsorption.
Fecal fat study may be useful.

The clinical analysis of fat malabsorption can be mapped as follows


(From Modern Nutrition In Health & Disease- M. Shils, 10th ed (pg 1150):
When working with patients with malabsorption syndrome:
• Monitor for malabsorption of fat soluble vitamins A, D, E & K.
• Prevent calcium oxalate stone formation
• Correct other nutrient deficiencies if present
• Alleviate steatorrhea and reduce intake of fat sources that are not
tolerated. Use medium chain triglycerides (MCT’s-liquid or parenteral
solutions). Introduce gradually in an adult. Caution patient about rapid
consumption.
• Limit dietary fat to one egg 4-6 oz meat, poultry, fish. Check
tolerance for LCT. Work up to 30–40g.
• Increase protein.
• Complex carbohydrates may be better tolerated than simple ones.
Lactose may not be tolerated.
• Include a multivitamin supplement
• A source of essential fatty acids may be needed if medium chained
fatty acid (MCT’s are used with a low fat diet.)
• Prescribing pancreatic enzyme supplements such as cotazym, zymase,
ultrase, pancrease, creon, etc., bile acid binding agents like
cholestyramine may help in controlling fat malabsorption. However,
these drugs have their own side-effects with respect to dosage and
hence this must be carefully monitored.
• Promote small and frequent meals
• Herbs and botanicals are to be discussed with a physician.
• Careful food handling is needed to prevent introduction of food borne
pathogens.

References:

Shils et al.: Modern Nutrition in Health and Disease, 10th Ed., Lippincott
Williams & Wilkins 2005
Sylvia Escott-Stump: Nutrition Diagnosis Related Care, 6th Edition,
Lippincott-Williams & Wilkins, 2007
Norbert W. Tietz (ed), Textbook of Clinical Chemistry, Saunders 1986
http://emedicine.medscape.com/article/931041-print

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