SEMINAR

ON
MALABSORPTION
SYNDROME

SUBMITTED TO, SUBMITTED BY,

Submitted on
16.04.18

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SL CONTENT PAGE NO.
NO.

1 INTRODUCTION

2 DEFINITION

3 ANATOMYN AND PHYSIOLOGY

4 CAUSES

a)Celiac disease

b)short bowl syndrome

c)lactose intolerance

d)cystic fibrosis

5 NURSING MANAGEMENT

6 RESEARCH ABSTRACT

7 SUMMARY

8 CONCLUSION

9 BIBLIOGRAPHY

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INTRODUCTION
Normally the human gastrointestinal tract digests and absorbs dietary

nutrients with remarkable efficiency. If there is impairment of any of the many

steps involved in the complex process of nutrient digestion and absorption,

intestinal malabsorption may ensue. If the abnormality involves a single step in

the absorptive process, as in primary lactase deficiency, or if the disease process is

limited to the very proximal small intestine selective malabsorption of only a

single nutrient may occur. However, generalized malabsorption of multiple dietary

nutrients develops when the disease process is extensive, thus disturbing several

digestive and absorptive processes, as occurs in coeliac disease with extensive

involvement of the small intestine.

ANATOMY AND PHYSIOLOGY

The gastrointestinal tract consists of a hollow muscular tube starting from

the oral cavity, where food enters the mouth, continuing through the pharynx,

oesophagus, stomach and intestines to the rectum and anus, where food is

expelled. There are various accessory organs that assist the tract by secreting

enzymes to help break down food into its component nutrients. Thus the salivary

glands, liver, pancreas and gallbladder have important functions in the digestive

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system. Food is propelled along the length of the GIT by peristaltic movements of

the muscular walls.

The primary purpose of the gastrointestinal tract is to break food down into

nutrients, which can be absorbed into the body to provide energy.

 First food must be ingested into the mouth to be mechanically

processed and moistened.

 Secondly, digestion occurs mainly in the stomach and small intestine

where proteins, fats and carbohydrates are chemically broken down

into their basic building blocks.

 Smaller molecules are then absorbed across the epithelium of the

small intestine and subsequently enter the circulation.

 The large intestine plays a key role in reabsorbing excess water.

 Finally, undigested material and secreted waste products are

excreted from the body via defecation.

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DEFINITION OF MALABSORPTION SYNDROME

Malabsorption syndromes are a group of disorders in which there is diminished

absorption of dietary materials with excessive loss of non absorbed substances in

the stools.

CAUSES

The malabsorption may be caused by

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(1) an intraluminal (digestive) defect, such as cystic fibrosis or biliary or liver

disease with abnormal bile secretions

(2) a mucosal (absorptive) abnormality, such as celiac disease (small intestine

mucosal injury), diarrhoea, and ulcerative colitis leading to rapid intestinal

motility, which causes mucosal injury or megacolon leading to inflammation of the

mucosa of the distended bowel

(3) an anatomic defect such as short bowel syndrome due to small bowel

resection,which leads to rapid transit time of nutrients through the intestine and

thus their reduced absorption.

 Physiological malabsorption occurs during early infancy and is due to

immaturity. Fats and starches are not completely digested because of the

absence of pancreatic amylase and reduced pancreatic lipase and bile acid

concentrations in the duodenal fluid.

 Emotional factors can lead to malabsorption in a condition such as

nonorganic FTT, in which no organic cause can be found for the difficulty

1. CELIAC DISEASE

DEFINITION

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Celiac disease is a serious autoimmune disorder that can occur in genetically

predisposed people where the ingestion of gluten leads to damage in the small

intestine. It is estimated to affect 1 in 100 people worldwide.

Celiac disease is also known as coeliac disease, celiac sprue, non-tropical sprue,

and gluten sensitive enteropathy.

 The most common cause of malabsorption in infants and children is cystic

fibrosis (a digestive disorder)

 the second most common is celiac disease (an absorptive disorder).

CAUSES

Celiac disease occurs as a result of a child's genetics and exposure to a

trigger. A child who develops celiac disease probably inherits the risk from one or

both parents and then develops the disease when exposed to the dietary trigger,

gluten.Celiac disease is different from wheat allergy. Allergies occur when

different parts of the immune system are activated by wheat, causing allergic

symptoms such as hives and wheezing

PATHOPHYSIOLOGY

Celiac disease results from severe atrophy of the intestinal villi

(Multitudinous thread-like projections that cover the of the mucosa of the small

intestine), which serve as the sites of absorption of fluids and nutrients. This

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atrophy is produced by the permanent intolerance of the infant or child to gliad in

fraction of gluten, which is a protein found in wheat and rye. Although gliadin is

also present in oats and barley, most affected children can tolerate a moderate

amount of these grains. The pathological changes in the mucosa may be caused by

either an inborn mucosal enzyme defect that allows undigested toxic portions of

the gluten molecule (amino acid gluta-mine) to remain in the mucosa, resulting in

the death of new cells and atrophy of the villi, or by an immunological response to

the gliad in itself that causes villous atrophy.

CLINICAL MANIFESTATIONS

 The clinical manifestations of celiac disease begin insidiously 3-6 months

after solid foods, usually cereals, are added to the infant's diet.

 The infant then begins to have diarrhoea and possibly steatorrhoea (mushy,

frothy, bulky, pale, fatty, foul smelling stools) because the absorption of fat

is affected.

 The abdomen becomes distended as a result of accumulated secretions and

gas in the intestinal tract, weakened abdominal muscles, abnormal

peristalsis, and oedema due to protein-losing enteropathy.

 The muscles in the proximal groups and the buttocks become wasted

 infant becomes irritable, apathetic, and anorexic.

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  Peripheral oedema in the lower extremities may become evident as the

result of hypoproteinemia.

 Vomiting abdominal pain, and rectal prolapse

 If the peristaltic activity is decreased, constipation and faecal impaction may

be present.

 Severe malnutrition and fail to thrive.

 anaemia due to iron deficiency and inadequate absorption of vitamin B12

and folic acid

 demineralization of bone (osteoporosis) and softening of bone

(osteomalacia) due to low levels of vitamin D, thus impaired calcium

absorption; and problems with blood coagulation manifested by epistaxis,

bruising, and intestinal haemorrhages due to the lack of absorption of

vitamin K.

 Rickets may occur as the child's height increases.

 Tooth eruption may also be delayed.

 Infants and Toddlers

Infants and toddlers tend to have more obvious symptoms which usually manifest

in the gastrointestinal tract. Symptoms include, but are not limited to: Vomiting,

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Bloating, Irritability, Poor growth, Abdominal distention, Diarrhea with very foul

stools.

 Infants and toddlers can suffer from malnutrition, leading to poor growth in

weight and/or height.

DIAGNOSTIC EVALUATION.

A variety of studies can help establish the diagnosis of celiac disease. These

studies include a

 3-day stool collection and examination to determine the presence of faecal

fat and blood examinations to determine the presence of anaemia (usually

iron deficiency), hypoalbuminemia, hypoprothrombinemia, and the levels

of folic acid, calcium, carotene,vitamin Bi2, A, and D, and gamma-globulin.

 X-ray films determine the child's bone age and the presence of osteoporosis

and osteomalacia.

 Barium contrast studies show a dilated small bowel and coarse mucosal

folds.

 The result of an oral glucose tolerance test is usually flat, reflecting mucosal

damage of the snail bowel.

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  A biopsy of the small intestine with the finding of villous atrophy and other

changes suggests celiac disease, but this abnormality can also occur in other

conditions. It is therefore necessary to perform three intestinal biopsies:

while the child is symptomatic, during recovery on a gluten-free diet, and

after are challenge with gluten. In biopsy of the small intestine or jejunum, a

polyethylene tube is passed through the mouth, oesophagus, stomach, and

into the jejunum. The child is given nothing by mouth for several hours

before the test and is not sedated.

 Individuals of various ages who have celiac disease have a wide range of

clinical manifestations that may indicate severe intestinal malabsorption to

near normal health.

 Obvious GI complaints are common in infants and children before the age of

2 years,whereas older children and adults generally have more subtle

symptoms

TREATMENT

Currently, the only treatment for celiac disease is lifelong adherence to a

strict gluten-free diet. People living gluten-free must avoid foods with wheat, rye

and barley, such as bread and beer. Ingesting small amounts of gluten, like crumbs

from a cutting board or toaster, can trigger small intestine damage.

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NURSING MANAGEMENT

The overall goal of care for infants and children who have celiac disease is to

achieve a stable condition for as long as possible.These children require care in the

hospital during the period of diagnosis and during times of acute crises, but the

remainder of their care is given in the home by the parents. The education of the

parents and the older child is of great importance.

Nursing management of affected children includes

(1) assisting with the diagnosis

(2) assisting parents in their adjustment to the diagnosis

(3) providing a nutritious diet

(4) correcting nutritional deficiencies

(5) preventing and providing care during celiac crises

(6) educating the parents and child during long-term follow-up care.

1. Assisting with the diagnosis

 The nurse observes infants for their reactions to the addition of new foods,

especially cereals, to their diet and after several weeks notes any indications

of the signs and symptoms of celiac disease.If they are present, a diagnostic

evaluation is performed.

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 During the diagnostic evaluation, the nurse collects stool specimens for fat

analysis and assists with collection of blood for haematological studies,with

X-ray examinations with jejunal biopsies, and with the glucose tolerance

test.

 The nurse prepares the child for each of the appropriate for the age group

and assists in gaining child's cooperation during the tests.

2. Assisting Parents in Their Adjustment

Parents may not only have difficulty to the diagnosis but may also find

the child's irritability and behavioural changes difficult to manage. The

nurse must helpthem understand the disease and its treatment and must

have an empathetic understanding of their problems and about their child.

3. Providing a Nutritious Diet.

 Dietary management is the major focus in the treatment of infants and

children with celiac disease.

 The diet that is given generally is high in calories and protein, low in fats

and carbohydrates, which are gluten free.

 avoid wheat, rye, barley, and oats containing gluten, it is almost

impossible to remove all of these grains from the diet. It is for this reason

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 that some physicians permit oats to be given in moderation to some of

these children.

 The requirements for grain in the diet can be fulfilled by substituting

rice, corn, and soybeans prepared in various ways.

 Coarse foods such as nuts and raw fruits and vegetables should not be

given at bed time.

 The nurse and the nutritionist are responsible for helping parents

understand the dietary restrictions followed indefinitely.

 Not only are the offending cereals, breads, and other baked goods, but

included in many processed foods that contain thickeners or hydrolysed

vegetable protein. It is essential that parents read the labels of all food

containers carefully each time they shop.

 Maintaining infants and young children on a gluten-free diet is not too

difficult, although they may become angry and frustrated if the diet is

very limited. As they grow older, major problems may be encountered.

 School-age children and adolescents do not want to eat differently from

their peers or the rest of their family members. Tension between these

individuals and their parents may develop over food selections, such as

cookies, hot dogs, and pizza. In some situations, maintaining the whole

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 family on a gluten-free diet may increase the compliance of the affected

child, but such a diet can be very expensive.

4. Correcting Nutritional Deficiencies.

 Because of their lack of absorption by these children, fat-soluble

vitamins must be supplemented.

 Vitamin K parenterally to children who have prolonged clotting time.

 If malabsorption of iron, folate, or vitamin B12 has caused anemia, it

should be treated appropriately.

 Peripheral parenteral alimentation using glucose, amino and fatty

acids may become necessary if these children are very ill.

5. Prevention of and Care During Celiac Crises.

 Exacerbations of this disease (celiac crises) can be prevented to a large

extent by adhering to a gluten-free diet and by preventing the child's

contact with others who have infections. Physicians, surgeons, and

dentists who provide care for the child should be informed so that

anticholinergic drugs, which may precipitate a celiac crisis, can be

avoided.

 The care of the child during a celiac crisis includes observation for

signs of dehydration, metabolic acidosis (increase in the rate and

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 depth of respirations, weakness), and shock. The nurse closely

monitors the intravenous fluids containing appropriate electrolytes

given for the treatment of these conditions.

 If hypo proteinemia is severe, albumin infusions are given. The nurse

administers corticosteroids as ordered to decrease inflammation of

the bowel and observes for the return of any manifestations of celiac

disease when the dose is gradually reduced.

 If the infant or child is permitted nothing by mouth, mouth care is

essential to prevent dryness and the possibility of infection. when

intermittent nasogastric suctioning is used to decrease abdominal

distension, the nurse records the amount and description of the

drainage

Outcome

The clinical response to a gluten-free diet used in the management of celiac

disease is gratifying. No long-term complications from this form of therapy are

known. Affected children who are not treated may die during a celiac crisis. If

untreated children survive or if treated children do not follow the plan of

therapy,may have growth failure and an increased incidence of lymphoma and

carcinoma of the small bowel during adulthood.

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2. SHORT BOWEL SYNDROME

DEFINITION

Short bowel syndrome (SBS, or simply short gut) is a malabsorption disorder

caused by a lack of functional small intestine. Intestinal failure is decreased

intestinal function such that nutrients, water, and electrolytes are not sufficiently

absorbed. Short bowel syndrome is when there is less than 2 m (6.6 ft) of working

bowel and is the most common cause of intestinal failure.

ETIOLOGY

Short bowel syndrome in adults and children is usually caused by surgery. This

surgery may be done for:

 Crohn's disease, an inflammatory disorder of the digestive tract

 Volvulus, a spontaneous twisting of the small intestine that cuts off the blood

supply and leads to tissue death

 Tumors of the small intestine

 Injury or trauma to the small intestine

 Necrotizing enterocolitis (premature newborn)

 Bypass surgery to treat obesity

 Surgery to remove diseases or damaged portion of the small intestine

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Some children are also born with an abnormally short small intestine, known as

congenital short bowel

PATHOPHYSIOLOGY

The length of the small intestine can vary greatly, from as short as 2.75 m (9.0 ft)

to as long as 10.49 m (34.4 ft). On average it is about 6.1 m (20 ft). Due to this

variation it is recommended that following surgery the amount of bowel

remaining be specified rather than the amount removed.Short bowel syndrome

usually develops when there is less than 2 meters (6.6 feet) of the small intestine

left to absorb sufficient nutrients.

In a process called intestinal adaptation, physiological changes to the remaining

portion of the small intestine occur to increase its absorptive capacity. These

changes include:

 Enlargement and lengthening of the villi found in the lining

 Increase in the diameter of the small intestine

 Slow down in peristalsis or movement of food through the small intestine

The acute phase has the following characteristics:

 Starts immediately after bowel resection and lasts 1-3 months

 Ostomy output of greater than 5 L/day

 Life-threatening dehydration and electrolyte imbalances

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  Extremely poor absorption of all nutrients

 Development of hypergastrinemia and hyperbilirubinemia

The adaptation phase has the following characteristics:

 Begins within 48 hours of resection and lasts up to 1-2 years

 Approximately 90% of the bowel adaptation takes place during this phase

 Enterocyte hyperplasia, villous hyperplasia, and increased crypt depth occur,

resulting in increased surface area; intestinal dilatation and lengthening also

occur

 Luminal nutrition is essential for adaptation and should be initiated as early

as possible; parenteral nutrition is also essential throughout this period

The maintenance phase has the following characteristics:

 The absorptive capacity of the intestine is at its maximum

 Nutritional and metabolic homeostasis can be achieved by oral feeding, or

patients are committed to receiving supplemental or complete nutritional

support for life

SIGNS AND SYMPTOMS

 The primary symptom is diarrhea, which can result

in dehydration, malnutrition, and weight loss. Other symptoms may include

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 bloating, heartburn, feeling tired, lactose intolerance, and foul smelling stool.

Abdominal pain

 steatorrhea (oily, bulky stool, which can be malodorous)

 Fluid depletion

 Fatigue

COMPLICATIONS

 Dehydration

 Vitamin, mineral, and/or electrolyte shortage or imbalance

 Malnutrition

 Severe diaper rash caused by frequent diarrhea

 Abnormal eating habits

 Kidney stones or gallstones caused by abnormal calcium or bile absorption

 Bacterial overgrowth (high levels of bacteria in the intestine)

MANAGEMENT

Excessive fluid losses

 Massive fluid and electrolyte losses are usually observed during the first

week after excessive intestinal resection.

 Patients with short bowel syndrome most often require aggressive

resuscitation with fluids or parenteral nutrition, or both. Instituting

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 enteral therapy as soon as possible is very important in order to facilitate

the adaptive intestinal response.

 In the early postoperative period, monitor serum electrolytes and a

comprehensive biochemical pattern daily.

 The hypersecretion noted within the first 12 months postresection is

usually treated with histamine 2 (H2)–receptor antagonists or proton

pump inhibitors

 The provision of adequate parenteral fluid replacement needs may be

ongoing depending on the amount of stool or ostomy output. Indeed,

enteral nutrition can cause significant osmotic diarrhea.

Malabsorption

 Extensive jejunal resection leads to carbohydrate malabsorption. The

undigested foods produce an osmotic diarrhea typical of most patients

with short bowel syndrome.

 Extensive resection of the ileum may lead to severe malabsorption of

bile salt and vitamin B-12. Bile salt malabsorption produces a choleretic

diarrhea.

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  Ileal resection leads to the malabsorption of bile salts and an abnormal

bile acid pool that leads to the formation of a lithogenic bile and

cholelithiasis.

 Treatment is generally aimed at lessening the degree of bacterial

overgrowth with antibiotic therapy, including administration of

metronidazole alternating with either kanamycin or oral gentamicin.

DIETARY MANAGEMENT

1. Small, frequent meals. Five to six small meals a day makes it easier to meet

a person’s caloric and nutrient requirements.

2. Liquid between instead of with meals. Drinking with meals can increase

transit time leading to diarrhea, and may limit a person’s appetite with a

feeling of fullness.

3. High protein, low simple sugars. Protein tends to slow the transit time of

food, whereas simple sugars like juices or sweet foods can contribute to

diarrhea.

4. Low fat (especially for people experiencing steatorrhea or people missing

their terminal ileum). If fat malabsorption is occurring, supplements of

vitamins A, D, E, and K are also recommended.

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 5. Low oxalate (for people missing their terminal ileum). High oxalate foods

include: strawberries, spinach, rhubarb, chocolate, beets, tea, nuts, and

wheat bran.

6. Foods that may control diarrhea are also recommended. These include

bananas, oatmeal, rice, tapioca, applesauce, yogurt, etc.

7. Multivitamin; magnesium, calcium, and iron supplements (if duodenum

removed); Vitamin B12 (if terminal ileum removed).

CONCLUSION

Short bowel syndrome involves the adaptation of the intestine

following a major surgical resection. Many people are initially on total

parenteral nutrition (feeding intravenously) post-surgery. Depending on the

extent of the resection, a person may be able to resume an oral diet within

a few weeks of surgery.

3. LACTOSE INTOLERANCE

DEFINITION

Lactose intolerance means the body cannot easily digest lactose, a type of natural

sugar found in milk and dairy products.

ETIOLOGY

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 Lactose intolerance occurs when the small intestine does not make enough of

an enzyme called lactase.

 Lactose intolerance most commonly runs in families, and symptoms usually

develop during the teen or adult years.

 Most people with this type of lactose intolerance can eat some milk or dairy

products without problems.

 Sometimes the small intestine stops making lactase after a short-term illness

such as the stomach flu or as part of a lifelong disease such as cystic fibrosis

 In rare cases, newborns are lactose-intolerant. A person born with lactose

intolerance cannot eat or drink anything with lactose.

 Some premature babies have temporary lactose intolerance because they are

not yet able to make lactase. After a baby begins to make lactase, the condition

typically goes away.

PATHOPHYSIOLOGY

The individual who has lactase deficiency is unable to digest lactose to form

glucose and galactose. As a result of this deficiency , lactose ferments and causes

intestinal distension, severe cramping, flatulence and explosive diarhheoa.

Because nutrients are not absorbed, the infant or child to thrive.

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 SIGNS AND SYMPTOMS

Symptoms usually begin 30 minutes to 2 hours after you eat or drink milk

products

 Bloating.

 Pain or cramps.

 Gurgling or rumbling sounds in your belly.

 Gas.

 Loose stools or diarrhea.

 Throwing up.

 Breath samples in lactose-deficient individuals will yielda higher percentage of

hydrogen (20 ppm

 In infants lactose malabsorption maybe diagnosed by evaluating fecal pH and

reducing substances; fecal pH in infants is usually lower than in older children,

but an acidic pH may indicate malabsorption Treatment of lactose intolerance

is elimination of offend-ing dairy products; however, some advocate decreasing

amounts of dairy products rather than total elimination, especially in small

children

 In infants, lactose free or low-lactose formula offers no special advantages over

lactose-containingformula except in the severely malnourished

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DIAGNOSTIC EVALUATION

History collection

Physical Examination

Hydrogen breath test is done to test for lactose intolerance. For the test,

child breathes into a container that measures breath hydrogen level before

and after he or she drinks that contains a known amount of lactose.

Normally, only a small amount of hydrogen is detectable in the breath when

a person eats or drinks and digests lactose. An increase in hydrogen levels is

considered a positive test for lactose intolerance.

 check acidity in the stools of infants and young children who may be unable

to properly do the hydrogen breath test. Acidic stool (low pH) may indicate

lactose malabsorption. Another stool test (reducing substances) looks for

the presence of glucose in the stool, which signifies undigested lactose.

MANAGEMENT

Lactose intolerance: This can be improved by dietary manipulation. If the

quantity of lactose is increased slowly over time, lactobacilli are stimulated to

grow in the colon. A greater number of lactobacilli allow the lactose to break

down into monosaccharides. Although this allows much of the sugar to be

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 absorbed, some of the resulting monosaccharides are still fermented by colonic

bacteria however, the relative amount of colonic fermentation is decreased.

NURSING MANNAGEMENT

The manifestations of lactose intolerance can be controlled if infants are given

a nonlactose soy formula and a lactose free diet (elimination of all milk and

milk products) and if children and adults learn toingest only the amount of

lactose that they can tolerate. Forolder children, parents can add a lactase

enzyme product(Lactaid or Lactozyme) to milk or use low-lactose milk and

cheese to compensate for the decrease in enzyme activity .Parents and

affected children and adults should be taught toread all food labels carefully.

Many individuals have only mild evidence of this condition because of their

own method of dietary control.

4. CYSTIC FIBROSIS

Definition

Cystic fibrosis is a disorder that affects many organ systems of children and young

adults. It is chiefly characterized by chronic obstruction and infection of the

pulmonary airways and by a lack of normal digestion in the GI tract.

Incidence

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 Cystic fibrosis (CF) is the most common life limiting recessive genetic

disorder in Caucasians with an incidence of approximately 1 in 2500 children born

in the United Kingdom. It is less common in African Americans (1 in 15000) and in

Asian Americans (1:31000). It also affects other ethnic groups such as black

population with an incidence of 1 in 17,000 and the native American population

with an approximate incidence of 1 in 80,000.The precise incidence of CF among

Indians is unknown.

Etiology

Mutation in the gene encoding the chloride conductance channel, CF

transmembrane, conductance regulator (CFTR) is the underlying cause. The failure

of chloride conductance by epithelial cells leads to dehydration of secretions by

epithelia cells leads to dehydration of secretions that are too viscid and difficult to

clear. The defective gene is located at long arm of chromosome 7; the most

common mutation is delta F508.

Pathophysiology

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 There are two main types of pathological changes that lead to organic

damage in these children. The first is an abnormally in the secretions of most

exocrine glands, resulting in the thickening of secretions in the ductal systems of

these glands. Instead of producing a thin secretion that flows easily, the mucus

secreting glands produce a thick, inspissated mucoprotein because of a deficiency

of water. This substance tends to accumulate and dilate the glands. The small

gland ducts and acini in these organs become plugged as secretions coagulate. The

lungs, the pancreas, the biliary tree in the liver, and the crypts of GI tract are the

structures most affected by this disease. The second abnormality is a defect in the

electrolytes of the endocrine sweat glands, resulting in high sodium and chloride

concentrations in the sweat. The diagnosis of cystic fibrosis depends on the

demonstration of an elevated sodium chloride concentration in the sweat of these

children.

Pathological changes occur in various organs, as discussed in the following

paragraphs.

Lungs. Because of the viscid secretions that cause impaired drainage from the

bronchi, bronchiectasis and repeated respiratory infections occur, leading

ultimately to respiratory failure due to fibrosis and necrosis of the pulmonary

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alveoli. These pulmonary complications constitute a serious threat to the lives of

children with cystic fibrosis.

Gastrointestinal Tract: The esophageal and duo-denal glands may be distended

with mucoid secretions. Minimal changes may be noted in the GI tract. Rectal

biopsies show dilated crypt lumina.

Pancreas: The viscid secretions in the pancreas lead to chronic pancreatitis and

progressive diffuse fibrosis. A deficiency of pancreatic exocrine secretion or

enzymes including trypsin, amylase, and lipase and the inability of what is

produced to reach the duodenum result in impaired digestion and absorption of

nutrients, especially fats. Steatorrhea (excessive amount of fat in the feces)

results. The islets of Langerhans remain intact. The impaired glucose intoleranee,

when it occurs, is probably caused by fibrosis disrupting the ducts. The incidence

of diabetes mellitus is somewhat greater in these children than inothers of the

same age. The pancreas ultimately be-comes smaller and thinner, and it may be

difficult tolocate at postmortem examination.

Liver: A similar process of blockage in the bile passages and fibrosis leads to

secondary biliary cirrhosis with accompanying portal hypertension and esophageal

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varices. Bile is lithogenic, and older children and adults who have cystic fibrosis

have an increased incidence of cholelithiasis.

Genitourinary Tract: Children who have cystic fibrosis also have changes in the

reproductive organs, in addition to the pathologic changes noted above. In

females, large amounts of mucus distend the glands of the uterine cervix and

collect in the cervical canal. In males, the body and tail of the epididymis, the as

deferens, and the seminal vesicles are obliterated because of defective

development of the wolifian duct structures

Salivary Glands: The changes in the salivary glands namely, the viscosity of their

secretions and the plugging and dilatation of their ducts are the same as those in

other exocrine glands. The concentrations of sodium and chloride are significantly

greater than normal in children who have cystic fibrosis.

CLINICAL FEATURES

The features depend on age of diagnosis and treatment received. The common

clinical presentation includes meconium ileus in neonatal period, recurrent

bronchiolitis in infancy and early childhood, recurrent lower respiratory tract

infections, chronic lung disease, bronchiectasis, steatorrhea and with increasing

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age pancreatitis, and azoospermia. Pancreatic insufficiency is present in >85% of

CF patients

DIAGNOSITIC EVALUATION

The diagnosis of cystic fibrosis rests on

(I) a familyhistory of the disease,

(2) demonstration ofan elevated sweat chloride concentration,

(3) exocrine pancreaticin sufficiency,

and (4) typical chronic obstructive pulmonary disease.

 A positive sweat test in the presents of one or more of the major clinical

manifestations provides a basis for the diagnosis.

 Family History. A family history of the disease among older siblings provides

a clue to the diagnosis Generally, the earlier the diagnosis is made and

treatmcnt begun, the better is the prognosis.

 Sweat Electrolytes. Although certain Simple procedures can be used to

collect sweat for screening forth is disease, they are not as reliable as the

sweat test.

 The diagnosis should be suspected by the presence of a typical phenotype

of family history

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  confirmed by the demonstration of a high sweat chloride(>60 mEq/1) on at

least two occasions and/or by identifying CF causing mutations.

 Nasal potential difference measurements can be used as an adjunct to

sweat test but is not widely available

MANAGEMENT

The treatment of cystic fibrosis includes respiratory management, nutritional care,

anticipation and early diagnosis of liver disease, diabetes and other organ

dysfunction.

The principles of respiratory management include airway clearance techniques,

antibiotics and anti-inflammatory agents. The aim of nutrition management is to

achieve normal growth and development. Nutritional management of CF includes

the following measures:

 Increasing caloric intake by encouraging parents to feed the child more

frequently. If appetite is poor due to persistent infection; feeding may be

given by nasogastric route or by gastrostomy

 Supplement fat soluble vitamins at twice the recommended doses. These

should be given along with food and enzymes.

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  Enteric coated tables or spherules of pancreatic enzymes are given with

each feed. Enzymes are started at doses of 1-2000 IU of lipase/kg in divided

doses and increased by observing improvement in malabsorption by noting

weight gain, nature of stool and abdominal symptoms.

 Medications used to treat patients with cystic fibrosis may include the

following:

 Pancreatic enzyme supplements

 Multivitamins (including fat-soluble vitamins)

 Mucolytics

 Nebulized, inhaled, oral, or intravenous antibiotics

 Bronchodilators

 Anti-inflammatory agents

 Agents to treat associated conditions or complications (eg, insulin,

bisphosphonates)

COMPLICATIONS

Surgical therapy may be required for the treatment of the following respiratory

complications:

 Pneumothorax

 Massive recurrent or persistent hemoptysis

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  Nasal polyps

 Persistent and chronic sinusitis

 GI tract complications requiring surgical therapy are as follows:

 Meconium ileus

 Intussusception

 Gastrostomy tube placement for supplemental feeding

 Rectal prolapsed

 Lung transplantation is indicated for the treatment of end-stage lung

DIETARY MANAGEMENT

 In general, a normal diet with additional energy and unrestricted fat intake

is recommended. A high-energy and high-fat diet, in addition to

supplemental vitamins (especially fat soluble) and minerals, is

recommended to compensate for malabsorption and the increased energy

demand of chronic inflammation.

 In children, because of various physical activities and eating habits,

assessment and modification of energy requirements is based on growth

and weight gain. Special consideration is given to female patients with a

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 potential for delayed puberty because of malnutrition, patients with

diabetes mellitus, and patients with liver disease.

 Additional salt intake is recommended for patients living in hot climates,

especially during exercise or activities that cause excessive sweating.

 Nutritional supplements in the form of either high-energy oral preparations

(eg, Scandishake) or enteral feeds (eg, elemental formulas, high-fat

mixtures) via nasogastric tube or gastrostomy may be indicated in some

patients. In one study, gastrostomy tube placement has been shown to

significantly improve percentile body mass index and percent-predicted

FEV1 in male patients and female pediatric patients. Lung function changes

after placement did not depend on the level of lung function at placement

NURSING MANAGEMENT

Give medications as ordered. Administer pancreatic enzymes with meals and

snacks.

Perform chest physiotherapy, including postural drainage and chest percussion

several times a day as ordered.

Administer oxygen therapy as ordered.

Provide a well-balanced, high calorie, high protein diet for the patient.

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 Make sure the patient receives plenty of fluids to prevent dehydration.

Provide exercise and activity periods for the patient to promote health.

Provide the young child with play periods, and enlist the help of physical

therapy department.

Provide emotional support to the parents of children with cystic fibrosis.

Be flexible with care and visiting hours during hospitalization to allow the child

to continue school.

Include the family in all phases of the child’s care

JOURNAL ABSTRACT

[Clinical studies of pediatric malabsorption syndromes].

[Article in Japanese]

Hosoyamada T1.

Author information
Abstract
Multiple cases with various types of pediatric malabsorption syndromes were
evaluated. The clinical manifestations, laboratory findings, pathophysiology, and
histopathological descriptions of each patient were analyzed in an effort to clear
the pathogenesis of the malabsorption syndromes and the treatments were
undertaken. The cases studied, included one patient with cystic fibrosis, two with
lactose intolerance with lactosuria (Durand type), one with primary intestinal
lymphangiectasia, two with familial hypobetalipoproteinemia, one with Hartnup

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disease, one with congenital chroride diarrhea, one with acrodermatitis
enteropathica, one with intestinal nodular lymphoid hyperplasia (NLH), five with
intractable diarrhea of early infancy and four with glycogenosis type Ia. Each case
description and outcome is described below: 1. A 15-year-old Japanese boy with
cystic fibrosis presented with severe symptoms, including pancreatic insufficiency,
bronchiectasis, pneumothorax and hemoptysis. His prognosis was poor. Analysis
of the CFTR genes of this patient revealed a homozygous large deletion from
intron 16 to 17b. 2. In the sibling case of Durand type lactose intolerance, the
subjects'disaccaridase activity of the small bowel, including lactase, were within
normal limits. The results of per oral and per intraduodenal lactose tolerance tests
confirmed lactosuria in both. These observations suggested, not only an abnormal
gastric condition, but also duodenal and intestinal mucosal abnormal permeability
of lactose. 3. In the case of primary intestinal lymphangiectasia, the subject had a
lymphedematous right arm and hand, a grossly coarsened mucosal pattern of the
upper gastrointestinal tract (identified via radiologic examination) and the
presence of lymphangiectasia (confirmed via duodenal mucosal biopsy). The major
laboratory findings were hypoalbuminemia, decreased immunoglobulin levels and
lymphopenia resulting from loss of lymph fluid and protein into the gastro-
intestinal tract. 4. In two cases of heterozygous familial hypobetalipoproteinemia,
serum total cholesterol and betalipoprotein levels were very low. The subjects
presented with symptoms and signs of acanthocytosis and fat malabsorption.
Further, one subject had neurological abnormalities such as mental retardation
and severe convulsions. Treatment with MCT formula diet corrected the lipid
malabsorption. 5. A 5-year-old girl presented with pellagra-like rashes, mental
retardation and cerebellar ataxia. An oral tryptophan (Trp) and dipeptide (Trp-

38
Phe) loading test were conducted and the renal clearance of amino acids was also
evaluated in this patient and in controls. Following the oral Trp loading test,
plasma levels of Trp indicated a lower peak in the case, reaching a maximum at 60
minutes. On the other hand, the oral dipeptide (Trp-Phe) loading test in the
Hartnup patient showed the peak Trp plasma level was the same as the control
subjects. The renal clearance of neutral amino acids in this case increased to levels
5 to 35 times normal. 6. In the case of congenital chloride diarrhea, the subject
had secondary lactose intolerance, dehydration, hyponatremia, hypokalemia,
hypochloremia, hyperreninemia and metabolic alkalosis. The chloride content of
her fecal fluid was very high. The concentrations were 89-103 mEq/l. In contrast,
her urine was chloride-free. The subject's growth and development improved
after treatment with lactose free formura and oral replacement of the fecal loses
of water, NaCl and KCl. Unfortunately, the patient died of a small bowel
intussusception. The kidney histopathological finding was juxtaglomerular
hyperplasia by a necropsy. 7. In the case of acrodermatitis enteropathica, the
subject had characteristic skin lesions, low serum zinc levels and ALPase activity.
An oral ZnSO4 loading test and intestinal mucosal histology by a peroral biopsy
were conducted. The serum zinc peak level was 2 hours after the oral ZnSO4
loading test. Infant formula alone could not maintain normal serum zinc ranges.
Light microscopic studies of the intestinal villous architecture showed a normal
pattern. However, ultrastructual examination of several epithelial cells revealed
numerous intracellular vesicles. After zinc therapy, these changes

were decreased. The lesions were postulated as the secondary result of zinc
deficiency. 8. A 12-year-old girl presented with hypogammaglobulinemia,
recurrent infections, chronic diarrhea and intestinal NLH. A barium meal and

39
follow-through examination showed multiple nodules throughout the stomach
and intestine. The nodules, all uniform in size, were 2 mm diameter. The barium
enema did not show NLH in the colon. Mucosal biopsy of the stomach and
jejunum revealed the typical histology of NLH in the lamina propria. Also,
achlorhydria was present in this patient and her serum gastrin levels were very
high; 315-775 pg/ml. 9. In 4 cases of intractable diarrhea in early infancy (by Avery
G B), a jejunal biopsy showed shortening villi and nonspecific enterocolitis. Some
patients were found with only low lactase or low lactase and sucrase levels. An
electron microscope analysis of the small bowel in 2 cases showed alterations:
increased pinocytosis in microvillus membranes and lysosomes by endocytosis of
undigested macromolecular substances. I postulated that the stated evidence was
causative of this clinical profile. 10. I frequently observed diarrhea as a clinical
manifestation in glycogenosis type Ia and lipid malabsorption in one case. The
light and electron photomicrographs showed intestinal absorption cells with the
glycogen deposits in the inferior devision of nuclei

SUMMARY

Today we had discussed about malabsorption syndromes. Malabsorption

syndromes are a group of disorders in which there is diminished absorption of

dietary materials with excessive loss of non absorbed substances in the stools.

In this celiac diseases, short bowel syndrome and lactose intolerance and cystic

fibrosis .

CONCLUSION

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The small intestine does most of the digesting of the foods you eat. If you have a

malabsorption syndrome, your small intestine cannot absorb nutrients from

foods. The main role of y small intestine is to absorb nutrients from the food you

eat into your bloodstream. Malabsorption syndrome refers to a number of

disorders in which the small intestine can’t absorb enough of certain nutrients and

fluids. These nutrients can be macronutrients (proteins, carbohydrates, and fats),

micronutrients (vitamins and minerals), or both.

BIBILIOGRAPHY

 Wong’s Essentials of pediatric nursing-Marilyn J Hockenberry.David Wilson

–elsevier publication

 Textbook of pediatric nursing”-Sussamma Varghese-jaypee publishers..2012

edition

 Marlow’s textbook of pediatric nursing.Dorothy R Marlow, Barbara A.

Redding.Elsevier publications.

 The short textbook of paediatrics. Suraj Gupte.Jaypee publishers.12th

edition.

 Manipal manual of anatomy.C N Shekhar.Cbs publishers

 Manipal Manual of physiology.C N Shekhar.CBS publishers.

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