You are on page 1of 18

Upload

Log In
Sign up

Browse
Welcome to Scribd, the world's digital library. Read, publish, and share books and
documents. See more
Download
1
of 4

Fat Malabsorption Syndrome

Ratings: (0)|Views: 7,773|Likes: 9


Published by Vytheeshwaran Vedagiri
A short writeup on fat malabsorption syndrome
See More

Fat Malabsorption SyndromeFat malabsorption syndrome is caused by functional


or organiccauses. Symptoms and signs include fatigue, weight changes,
steatorrhea,abdominal distention, cramps, gas, explosive diarrhea with foul
smellingstools, malnutrition and weight loss, and biochemical abnormalities.
Under normal dietary conditions, less than 5% of the ingested fat is observed in
thefeces. An increase in this value might indicate fat malabsorption. Thedigestive
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 pHlevels and is active even in premature infants.
However, the largest part of triglyceride digestion is accomplished in the
duodenojejunal lumen becauseof a complex of pancreatic enzymes, the most
important of which is thelipasecolipase 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,
adequateconcentrations of intraluminal conjugated bile salts are needed to
formmicelles, and the secretion of bile acids may also be partially inadequate
invery young patients.Disorders of these processes can be congenital (cystic
fibrosis andShwachmanDiamond syndrome, which cause lipase and
colipasedeficiency; the uncommon isolated deficiency of lipase and colipase;
theextremely rare congenital primary bile acid malabsorption, which results inlow
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 malabsorptionof lipids are tabulated as follows:
S t a g e C o n d i t i o n s
Pancreatic stage

Isolated pancreatic lipase or colipasedeficiency (inherited)

Pancreatic insufficiency
o
Cystic fibrosis
o
Chronic pancreatitis

o
Obstruction of pancreatic duct
o
Pancreatic cancer
o
Pancreatic resection
o
ShwachmanDiamond syndrome
o
JohnsonBlizzard syndrome
o
Pearson syndromeBiliary stage

Decreased bile salt synthesis in severehepatic insufficiency

Decreased delivery of bile salts in biliarytract obstruction or cholestatic


biliarydisease

Decreased concentration of conjugated bile salts due to increased acidity,


drugsaffecting micelle formation

Increased intestinal loss of bile salts due tosurgery or diseased terminal
ileumCellular and delivery stage

Rapid transit, dumping syndrome

Improper emulsification, following certaintypes of gastrectomy

ZollingerEllison syndrome (alteredduodenal pH)

Decrease in small intestine lymphaticsDefects in multiple stages of digestions and


absorption

Decreased CCK release due to mucosaldestruction as in sprue or regional


enteritis(Crohns disease)
Conditions associated with malabsorption of lipids (Modified from Tietz. Textbook
of Clinical Chemistry)
Apart from the aforementioned conditions fat malabsorption couldalso be resultant
of abetalipoproteinemia is a rare disorder with autosomalrecessive inheritance.
Absence of the lipoproteins results in cytoplasmiclipid accumulation in the
enterocyte. Lymphatic transport of long-chain fatsis 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,
concentratedurine 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 increasedrisk of nephrolithiasis; enteric hyperoxaluria is the major
risk factor with fatmalabsorptive states, and use of probiotics to decrease oxalate
levels is beingstudied and considered.Long term nutritional monitoring is
necessary after gastric bypasssurgery for morbid obesity which can lead to fat
malabsorption and vitamindeficiencies for Vitamin A, D, E, and K. In addition,
altered calciummetabolism 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 fatmalabsorption
should be recommended using the acid steatocrit, a reliableand inexpensive test.
Intake of 40g of olestra can cause false positive resultson tests for steatorrhea and
can lead to an erroneous diagnosis of malabsorption syndrome. Low total
cholesterol (120mg/dL) or low serumcarotene levels may be typical of fat
malabsorption but not necessarilydiagnostic. 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, 10
th
ed (pg 1150):

Activity (8)

Filters
Add to collectionReviewAdd noteLikeEmbed
1 hundred reads
1 thousand reads

sirnormal liked this

Dennis Dkhar liked this

Gloria Gonzales Semblante liked this


Jason E. Ibaez liked this

Icel Hernandez Dedace liked this

walanalang liked this


More From This User
Cushing's Syndrome

Vytheeshwaran Vedagiri

Copper Deficiency Anemia

Vytheeshwaran Vedagiri

Adrenocortical Insufficiency

Vytheeshwaran Vedagiri

Type II Hypersensitivity

Vytheeshwaran Vedagiri

A Level Organic Chemistry Mini Test

Vytheeshwaran Vedagiri

Hormones Summary

Vytheeshwaran Vedagiri

Introduction to Enzymes

Vytheeshwaran Vedagiri

KS3 Test

Vytheeshwaran Vedagiri
GCSE Lower Tier Chemistry Test

Vytheeshwaran Vedagiri

Dispersal and Fair Test

Vytheeshwaran Vedagiri

Key Stage III Biology Question Paper

Vytheeshwaran Vedagiri

Island Bio Geography

Vytheeshwaran Vedagiri

LAMARCKISM Rejected Fixity Proposed A

Vytheeshwaran Vedagiri

LAMARCKISM Rejected Fixity Proposed A

Vytheeshwaran Vedagiri

Minitest in Formula and Equations

Vytheeshwaran Vedagiri

Energetics Test

Vytheeshwaran Vedagiri

Candidate GI Hormones

Vytheeshwaran Vedagiri

Clinical Enzymology

Vytheeshwaran Vedagiri

Metabolism In
Vytheeshwaran Vedagiri

VENTRICLES OF THE BRAIN AND CSF

Vytheeshwaran Vedagiri

NUCLEUS - MORPHOLOGY AND FUNCTIONS

Vytheeshwaran Vedagiri

Viruses & Bacteriophages

Vytheeshwaran Vedagiri

Unconditional Love of a Mother

Vytheeshwaran Vedagiri

Inventions and their truths A Lesson for Life

Vytheeshwaran Vedagiri

Download and print this document

Read and print without ads


Download to keep your version
Edit, email or read offline

Choose a format:

.PDF .DOC
Download

Recommended

Wild Heart: A Life


HarperCollins
Born in 1876, Natalie Barney-beautiful, charismatic, brilliant and wealthy-w...

China to Me
E-Reads, Ltd.

A revolutionary woman for her time, Emily Hahn takes us on an adventure throu...

Talking to the Dead: A Novel


David C Cook

Twentysomething Kate Davis can't seem to get this grieving-widow thing right....

Paris Without End: The True Story of ...


HarperCollins

Hadley Richardson and Ernest Hemingway were the golden couple of Paris in th...

Page 1 of 3

About
Browse
About Scribd

Team
Blog
Join our team!
Contact Us

Subscriptions
Subscribe today
Your subscription

Gift cards
Advertise with us
AdChoices

Support
Help
FAQ

Press
Purchase Help

Partners
Publishers
Developers / API

Legal
Terms
Privacy
Copyright

Get Scribd Mobile

Mobile Site

Copyright 2014 Scribd Inc.


Language:
English

Malabsorption can be described as the bodys inability to absorb certain nutrients from the
gastrointestinal (GI) tract. This problem has become one of the leading health concerns all across
the US today. There are several different types of malabsorption syndromes and the most
common ones include the inability to absorb fat, proteins, vitamins and sugar. The different
factors that could lead to malabsorption exceed 200 in total and so it is not always easy to
pinpoint the main causative factors. Malabsorption of sugar (generally lactose or milk sugar) is
probably one of the most frequent types of malabsorption. Fat malabsorption syndromes, though
less common, can be a lot more serious. Fat malabsorption refers to the impairment of processing
fat, which takes place mainly in the small intestines. In case you are suffering from fat
malabsorption syndromes, it means that your body is not absorbing this nutrient from the foods
that you eat.

Causes: The malabsorption of fat can be caused by organic or functional factors. Under normal
circumstances, less than 5% of the fat you consume is present in your feces. An increase in this
value may be an indication of fat malabsorption. The normal process of fat digestion can be
divided into 3 stages, the pancreatic stage, the biliary stage and the cellular & delivery stage.
Some of the conditions associated with the malabsorption of fats include:

At the Pancreatic Stage


Isolated pancreatic lipase or co-lipase deficiency
Cystic fibrosis

Chronic pancreatitis
Obstruction in the pancreatic duct
Pancreatic cancer
Resection of the pancreas
Shwachmann Diamond syndrome
Johnson Blizzard syndrome
Pearson syndrome

At the Biliary Stage


Decreased bile salt synthesis in hepatic insufficiency
Cholestatic biliary disease or the decrease delivery of bile salts within the biliary tract

Decrease in the concentration of conjugated bile salts because of increased acidity


Increase in the loss of bile salts because of surgeries, diseases or certain illnesses

At the Cellular and Delivery stage


Decrease in the small intestine lymphatics
Altered duodenal pH (Zollinger Ellison syndrome)

Improper emulsification after certain types of gastrectomy


Rapid transit dumping syndrome

Some of the other possible causes of fat malabsorption syndromes could include:

Undergoing a gastric bypass surgery for treating morbid obesity


Acute abnormality in the intestinal lining because of infestations, antibiotics or alcohol
abuse
Presence of a chronic abnormal intestinal lining, as a result of conditions like Crohns
disease or Celiac disease
Improper intestinal environments because of bacterial overgrowth or the presence of
parasites in the digestive system
Inadequate gastric mixing due to factors like a fistula in the gastric environment or after a
gastrostomy
Impaired movement of the enzymes in the body
Intestinal lymphangiectasia
Whipples Disease
Irritable Bowel Syndrome and Inflammatory Bowel Disease

Most of the causes of fat malabsorption are quite serious and should be treated by a doctor
immediately. Therefore, it is important for you to consult a doctor as soon as you notice any of
the fat malabsorption symptoms.

Symptoms: The signs and symptoms of fat malabsorption may vary, depending upon the causes
and the severity of the condition. Some of the most common fat malabsorption symptoms
include:

Absent tendon flexes


Anemia
Bloating and excessive gas
Carpopedal spasms
Changes in the color and texture of the skin
Cramps in the muscles
Cutaneous bruising
Dehydration
Diarrhea
Edema
Excessive thirst
Failure to thrive (in children)
Fatigue
Foul smelling stools
Greasy, oily or bulky stools
Itching, burning and soreness in the perianal skin
Muscle waste and atrophy
Nausea and vomiting
Pain in the abdomen
Presence of undigested food in the stools
Significant weight loss
Weakness and lethargy

If fat malabsorption is left untreated, the symptoms could become more severe, as you body
continues to lose water, electrolytes and other essential nutrients. Some of the fat malabsorption
symptoms that should be checked right away include:

Irregular heart rhythms


Low or highly concentrated urine output
Dryness in the mouth, tongue or skin
Fissures on the corners of the mouth

Make sure that you speak to your doctor immediately, if you experience any of the signs and
symptoms mentioned above.

Diagnosis: Your doctor may order a couple of tests to diagnose the condition accurately. Some of
the tests usually conducted include:

Abdominal CT scan

Hydrogen breath test


Small bowel biopsy
Complete blood count
Stool culture test
X-rays of the abdomen or other imaging tests

These tests help your doctor determine the root cause of the problem, so that the appropriate
treatment path can be adopted.
Treatment: Fat malabsorption treatment is aimed at addressing the underlying cause of the
problem. Your doctor may recommend various medicines, surgical procedures or even home
remedies to treat fat malabsorption. The diet you follow plays a very important role in the
treatment of fat malabsorption. Your doctor will need to review your current diet and if
necessary, prescribe supplements.

References
1. http://www.nlm.nih.gov/medlineplus/ency/article/000299.htm

The GI tract is responsible for digesting and absorbing food.[5] Lipids provide the richest source
of energy for the body, with 9 calories in every gram of fat; in comparison, carbohydrate and
protein contains 4 calories per gram. Whereas protein and carbohydrate begin to undergo
digestion in the stomach, triglycerides remain mostly unchanged until they reach the small
intestine. Intragastric breakdown accounts for approximately 10% of total lipid digestion.[5]

The pancreatic enzymes responsible for lipid digestion are inactivated when the pH drops below
5; thus, before digestion can continue in the duodenum, the acidic contents of the stomach must
be neutralized. Fortunately, the pancreas also secretes bicarbonate, which increases the pH of the
duodenal contents.

The exocrine pancreas produces 3 main types of enzymes: amylase, protease, and lipase.[1] Under
normal physiologic conditions, pancreatic enzymes (specifically, lipase) break the undigested
triglycerides into fatty acids and monoglycerides. Bile salts then solubilize these breakdown
products to form micelles, which are vehicles for absorbing lipid breakdown products.[5] Normal
fat digestion also depends on postprandial synchrony between delivery of nutrients to the
duodenum and discharge of pancreatic enzymes.[1]

Pancreatic secretion is governed by neural and hormonal mechanisms. The hormones responsible
for regulation are secretin and cholecystokinin (CCK). Secretin is secreted in response to acid in
the duodenum, causing duct cells to release water and bicarbonate; CCK is secreted in response
to protein and fat in the small intestine, stimulating acinar cells to release the pancreatic enzymes
(see the image below).

Factors controlling release of pancreatic secretions. Image


courtesy of Wikimedia Commons.
EPI is characterized by a deficiency of these exocrine pancreatic enzymes, which results in
inability to digest food properly (ie, maldigestion). Because pancreatic lipase accounts for up to
90% of fat digestion, maldigestion of fat is more profound in EPI than maldigestion of proteins
and carbohydrates is.[5] Because the exocrine pancreas retains a large reserve capacity for enzyme
secretion,[5] fat digestion is not clearly impaired until lipase output decreases to below 10% of the
normal level.[2]

Fat malabsorption precedes malabsorption of other macronutrients.[6] Bile salt precipitation and
subsequent adsorption to undigested food reduces the bile salt pool, and this reduction further
impairs fat digestion.[7] Undigested fat, rather than being absorbed, is excreted in the feces,
leading to steatorrhea.

Another factor that contributes to pancreatic steatorrhea is the presence of neurohormonal


disturbances, which result in gall bladder hypomotility and accelerated gastric and intestinal
transit.[8] Malabsorption of fat-soluble vitamins A, D, E, and K may accompany EPI.

PERT is the basis of treatment of EPI[22, 23] ; the endpoints of treatment are normalization of gut
absorption and correction of nutritional deficiencies. The typical indications for initiating PERT
are progressive weight loss and steatorrhea.

Approved agents

The pancreatic enzyme products (PEPs) used for PERT are extracts of porcine pancreas that
contain all 3 pancreatic enzymes (ie, amylase, protease, and lipase) in varying proportions.
However, it is lipase that plays the paramount role in therapy. The following 6 PEPs have been
approved by the US Food and Drug Administration (FDA) for the treatment of maldigestion in
patients whose bodies do not produce sufficient pancreatic enzymes:

Creon (Abbott Laboratories, North Chicago, IL)


Zenpep (Eurand Pharmaceuticals, Yardley, PA)
Pancreaze (Janssen Pharmaceuticals, Titusville, NJ)
Ultresa (Aptalis Pharma US, Birmingham, AL)
Viokace (Aptalis Pharma US, Birmingham, AL)
Pertzye (Digestive Care, Bethlehem, PA)

These PEPs are not interchangeable. For example, Viokace, unlike the other 5 products, lacks an
enteric coating and must be taken with a proton pump inhibitor.

Because exogenous pancreatic enzymes should exert their action on the ingested meal and
because gastric emptying of nutrients should occur in parallel with pancreatic enzymes reaching
the duodenum, PEPs are administered together with meals and snacks. When a sufficient enzyme
concentration is delivered into the duodenal lumen simultaneously with a meal, fat absorption is
enhanced.

PEP dosing for PERT is based on the content of lipase units (see Table 1 below). The pancreatic
lipase replacement dose should be adjusted on the basis of body weight, clinical symptoms, and
stool fat content. Several days should be allowed between dose adjustments.

Table 1. Lipase Content of Currently Available Pancreatic Enzyme Products (Open Table in a
new window)

Pancreatic Enzyme Product Lipase Content, units


Creon 1203 3000
Creon 1206 6000
Creon 1212 12,000
Creon 1224 24,000
Zenpep EURAND 3 3000
Zenpep EURAND 5 5000
Zenpep EURAND 10 10,000
Zenpep EURAND 15 15,000
Zenpep EURAND 20 20,000
Zenpep EURAND 25 25,000
Pancreaze MT 4 4200
Pancreaze MT 10 10,500
Pancreaze MT 16 16,800
Pancrease MT 24 21,000
Ultresa 13800UL 13,800
Ultresa 20700UL 20,700
Ultresa 23000UL 23,000
Viokace 9111 10,440
Viokace 9116 20,880
Pertzye 8 8000
Pertzye 16 16,000

The total daily dose is based on the assumption that the patient will have about 3 meals/day and
2-3 snacks/day, with half the mealtime dose given with a snack. Lipase doses larger than 2500
units/kg/meal (or dosages exceeding 10,000 units/kg/day) should be used with caution and only
when supported by documentation of 3-day fecal fat measures. Lipase doses larger than 6000
units/kg/meal are associated with colonic stricture and should be reduced.

Dosing for pancreatic insufficiency is as follows:

Initial oral lipase dose, 500 units/kg/meal


Lipase dose range, 500-2500 units/kg/meal
Maximum lipase dosage, 10,000 units/kg/day or 4000 units per gram of fat daily

Dosing for pancreatic insufficiency due to chronic pancreatitis or pancreatectomy is as follows:

Oral lipase dose, 72,000 units/meal with consumption of at least 100 g of fat daily
Alternatively, lower initial lipase doses (500 units/kg/meal) with individualized dose
titration may be considered

In a double-blind, randomized, multicountry, placebo-controlled, parallel-group trial enrolling 54


patients aged 18 years or older with confirmed EPI due to chronic pancreatitis or pancreatic
surgery, Whitcomb et al found that Creon delayed-release 12,000lipase unit capsules were
effective in treating fat and nitrogen maldigestion, with a treatment-emergent adverse event rate
similar to that of placebo.[24]

In an open-label extension of this study, the investigators determined that pancrelipase was well
tolerated over 6 months and resulted in statistically significant weight gain and reduced stool
frequency in patients with EPI due to chronic pancreatitis or pancreatic surgery who were
previously managed with standard PERT.[25]

Toskes et al carried out a randomized, double-blind, dose-response, crossover study in which the
effect of Zenpep on the coefficient of fat absorption was investigated with a placebo run-in (7-9
days) and 2 treatment periods (9-11 days) consisting of a high dose (7 20,000 lipase units/day)
and a low dose (7 5000 lipase units/day).[26] The results suggested that chronic pancreatitis
patients with EPI benefit from a low dose, whereas a high dose might be needed for patients with
more severe EPI.

In a randomized, placebo-controlled PERT withdrawal study evaluating the efficacy and safety
of Pancreaze in cystic fibrosis patients with EPI, Trapnell et al found that the agent effectively
improved fat absorption and protein absorption in these patients without giving rise to
unexpected adverse events.[27]

A study examining the use of endoscopic therapy in patients with chronic pancreatitis determined
that although this therapy is most useful in patients with pancreatic duct stones and dilation and
should be used as first-line treatment for those patients, it cannot be recommended as the
treatment of choice for all patients with chronic pancreatitis.[28]

Investigational therapy

Liprotamase is a biologically engineered nonporcine PERT agent that has been advocated for the
treatment of EPI associated with cystic fibrosis and pancreatectomy. In January 2011, the FDA
withheld approval of the drug, citing the need for further study.

In a subsequently published phase III 12-month open-label trial designed to assess the safety,
tolerability, and long-term nutritional effects of liprotamase in 215 patients with cystic fibrosis
and EPI aged 7 years and older, Borowitz et al reported that treatment with a mean of 5.5
capsules of liprotamase per day during meals and snacks for up to 12 months was safe and well
tolerated and was associated with age-appropriate growth and weight gain or weight
maintenance.[29]

You might also like