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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 Pancreatic stage Conditions • Isolated pancreatic lipase or colipase deficiency (inherited) • Pancreatic insufficiency o Cystic fibrosis o Chronic pancreatitis

Biliary stage

• • • •

Cellular and delivery stage

• • •

• Defects in multiple stages of • digestions and absorption

o Obstruction of pancreatic duct o Pancreatic cancer o Pancreatic resection o Shwachman–Diamond syndrome o Johnson–Blizzard syndrome o Pearson syndrome 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 Rapid transit, dumping syndrome Improper emulsification, following certain types of gastrectomy Zollinger–Ellison syndrome (altered duodenal pH) Decrease in small intestine lymphatics Decreased CCK release due to mucosal 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