Mal-absorption syndrome

1. Mal-absorption syndrome

2. Malabsorption syndrome refers to a number of disorders in which the intestine can't adequately absorb certain nutrients into the bloodstream. • It can impede the absorption of macronutrients (proteins, carbohydrates, and fats), micronutrients (vitamins and minerals), or both.

3. Proteins, carbohydrates, fats, and most fluids are absorbed in the small intestine (small bowel). • Malabsorption syndrome occurs when something prevents the bowel from absorbing important nutrients and fluids. • The problem may be caused by inflammation, intrinsic disease, or injury to the lining of the intestine. • Sometimes, the condition may be the result of the body’s failure to produce enzymes needed to digest certain foods or to adequately mix the food with the enzymes and acid produced by the stomach. 

4. Factors that may affect the digestion of food and cause malabsorption syndrome include: • prolonged antibiotic use • other conditions such as celiac disease, Crohn’s disease, chronic pancreatitis, or cystic fibrosis • lactase deficiency, or lactose intolerance, which is fairly common in people of African or Asian descent and happens when the body lacks the enzyme needed to digest lactose, a sugar present in milk 

5. Contd… • congenital (birth) defects, such as biliaryatresia, which is when the bile ducts don’t develop normally and may become blocked and prevent the flow of bile from the liverdiseases of the gallbladder, liver, or pancreas damage to the intestine (from infection, inflammation, trauma, or surgery) • parasitic diseases • radiation therapy (which may injure the mucosal lining of the bowel)

6. There are some fairly uncommon disorders that result in malabsorption like • short bowel syndrome: This condition may be a birth defect or the result of surgery. The surface area of the bowel decreases, and this hinders the bowel’s ability to absorb nutrients. • Tropical sprue: This condition is most common in Southeast Asia, the Caribbean, and India. Its symptoms may include anemia, diarrhea, sore tongue, and weight loss. The condition may be related to environmental factors, such as toxins in food, infection, or parasites.

7. Pathophysiology • Carbohydrate, fat, or protein malabsorption is caused by a disorder in the intestinal processes of digestion, transport, or both of these nutrients across the intestinal mucosa into the systemic circulation. • Either a congenital abnormality in the digestive or absorptive processes or, more commonly, a secondarily acquired disorder of such processes may result in malabsorption.

8. Disorders of Carbohydrate metabolism • Congenital : cystic fibrosis and Shwachman-Diamond syndrome, which may cause amylase deficiency; the extremely rare congenital lactase deficiency; sucrase-isomaltase deficiency. • Acquired: the most common being lactose intolerance, typically secondary to a damage of the mucosa, such as a viral enteritis; conditions that cause mucosal atrophy, such as celiac disease.

9. Disorders of protein metabolism • Congenital: cystic fibrosis, Shwachman-Diamond syndrome, and enterokinase deficiency, which cause inadequate intraluminal digestion. • Acquired: disorders of protein digestion and/or absorption are nonspecific (ie, they also affect the absorption of carbohydrates and lipids) and are found in conditions that result in damage to the absorptive intestinal surface, such as extensive viral enteritis, milk protein allergy enteropathy, and celiac disease.

10. Disorders of Lipid metabolism • Congenital: cystic fibrosis and ShwachmanDiamond syndrome, which cause lipase and colipase deficiency, congenital primary bile acid malabsorption. • Acquired (secondary mostly to disorders of the liver and the biliary tract or to chronic pancreatitis). Clearly, any condition that results in the loss of small intestinal absorptive surface also causes steatorrhea.

11. Epidemiology Genetically determined syndromes • Celiac disease is by far the most common inherited malabsorption syndrome. • Cystic fibrosis is the second most common malabsorption syndrome. Acquired syndromes • Cow's milk and soy milk protein allergies are common, especially in infants and young children. • A transient and common form of malabsorption in infants results from acute-onset enteritis (mostly viral, specifically rotaviral), which causes transient lactose intolerance.

12. Epidemiology contd… Gender: • Celiac disease is slightly more common in females. • Autoimmune enteropathy is an X-linked disorder that only affects males in familial cases. Age: • Symptoms of a congenital disease are usually apparent shortly after birth or after a short hiatus once a particular substance is ingested in substantial amounts. • Protein sensitivity syndromes to milk or soy protein usually present in infants younger than 3 months. • Solid food protein sensitivity syndromes are known to occur in older patients.

13. Clinical Manifestations: • Chronic Diarrhoea, abdominal distension, failure to thrive. • Flatulence, anorexia, loss of weight, Impaired digestion • Malnutrition and growth failure • In celiac disease stool may be loose and bulky and in exocrine pancreatic insufficiency pasty and yellowish. • On specific chronic diarrhoea in children may present with diarrhoea containing foul smell and mucous.

14. Diagnosis • History • General health and appearance • Stool examination • Intestinal biopsy • Sweat chloride test • Serology • Radiology and barium X-ray

15. Management • Depends upon underlying cause • Promotion of adequate nutrition • Symptomatic control of diarrhoea • In celiac diseases: strict gluten free diet. Rice and maize can be given instead of wheat • In Cystic Fibrosis: Protein and glucose rich food, vegetables instead of fats. • Carbohydrate malabsorption: glucose and galactose free diet.

16. Management • Replacement of nutrients, electrolytes and fluid may be necessary • In severe deficiency, hospital admission may be required for parenteral administration. • Pancreatic enzymes are supplemented orally in pancreatic insufficiency. • Dietary modification is important in some conditions: – Gluten-free diet in coeliac disease. – Lactose avoidance in lactose intolerance. – Food allergic enteropathy need to be on an elimination diet, avoiding offending food antigens. • Antibiotic therapy will treat Small Bowel Bacterial overgrowth (eg, metronidazole, rifaximin). • cholestyramine :In children with chronic diarrhea secondary to bile acid malabsorption, the use of cholestyramine. • Immunosuppressive medications can be used to control autoimmune enteropathy

17. DIET • Carbohydrate intolerance • Initiate treatment in patients with severe acquired carbohydrate intolerance by eliminating all dietary carbohydrates until the diarrhea is resolved. Then, slowly reintroduce carbohydrates. • In infants, use a glucose polymer (Polycose)– based formula (eg, Pregestimil). • In patients with the most severe carbohydrate intolerance, a casein-based formula that contains essential amino acids and medium-chain triglyceride (MCT) oil and no carbohydrates.

18. Fat intolerance • MCT oil is used to treat patients with poor weight gain that results from fat malabsorption. • MCT oil does not require traditional fat metabolism and, thus, is more easily absorbed directly into the enterocyte and is transported through the portal vein to the liver. • Fat-soluble vitamin supplements are required. • Supplements in patients with fat malabsorption should also include linoleic and linolenic fatty acids.

19. Alternative formulas (protein intolerance) • Currently, soy formulas are not considered effective for the prevention or treatment of nutritional allergies. Instead, use hydrolyzed protein formulas. • High-degree protein hydrolysate formulas are used to treat infants with a cow's milk allergy, but these formulas may contain residual epitopes capable of provoking a severe allergic reaction. • In these infants, use formulas with crystalline amino acids (eg, Neocate, EleCare) as the protein source.

20. Reference • Hockenberry, Wilson, Judie. Wong’s essentials of pediatric Nursing. South India Edition. Elsevier publication. • Dorothy R Marlow, Barbara A Redding, Raman Kalia. Marlow’s textbook of pediatric Nursing. Harcourt Brace & Company Asia Pte Ltd. • ParulDatta. Pediatric Nursing. 4th edition. Jaypee Publishers. New Delhi.

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