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1. Malabsorption Syndromes Nursing management of pediatric patients with Genetic disorders | The Child with Gastrointestinal Dysfunction Dr. Moustafa Ali

2. After mastering the contents of this lecture, you should be able to: Identify the common problems affecting the gastrointestinal system during childhood Provide nursing care to a child with malabsorption syndrome by implementing the nursing process Identify nutritional therapies for the child with a malabsorption syndrome O B J E C T I V E S

3. Malabsorption Syndromes These include disorders with impaired digestion and or absorption. Classification is done according to anatomic and or biochemical defect it has: (continued)

4. Four characteristics Seatoria (Fatty, Foul, Frothy bulky stools) General malnutrition Abdominal distention Secondary Vitamin deficiencies Malabsorption Syndromes

5. Include diminished or absent enzymes such as: Cystic fibrosis: Pancreatic enzyme is absent Biliary liver disease: bile flow is affected (Artesia of the bile duct) Lactase deficiency: Congenital or secondary Lactase intolerance Digestive defects

6. Conditions in which the intestinal mucosal transport system is impaired. Primary defect ( Celiac disease ) or Secondary to inflammatory disease in the bowel leading to impaired absorption, because bowel mobility is accelerated such as Ulcerative Colitis Obstructive Disorders such as Hirschsprung's Disease can cause secondary malabsorption from Enterocolitis . Anatomic defects : extensive resection of the bowel or short bowel syndrome. Absorptive defects

7. Celiac disease (CD) Gluten – induced enteropathy, gluten sensitive enteropathy ( GSE ) Celiac sprue. It is secondary to cystic fibrosis to cause malabsorption in children (continued)

8. Incidence 1:4000 but it appears to be declining may be due to environmental factors. ++ in Europe and rare in America and is rarely reported in Asians or blacks Cause is unknown There is no inherited predisposition with an influence by environmental factors Incidence

9. Intolerance of gluten a protein found in Wheat, Barley, Rye and Oats Gluten consists of: Glutenin Gliadin : Susceptible individuals are unable to digest gliadin fraction resulting in accumulation of a toxic substance that damage the mucosal cells leading to villi atrophy , and reducing the surface for absorption in the small intestine Pathophysiology

10. C/M noticed at 3 – 6 months following introduction of gluten – containing grains into diet Age usually 9 -12 months Diagnosis villi of the intestine

11. C/M insidious and chronic they include: Impaired fat absorption: Steatorrhea and exceedingly foul smelling stools Diagnosis

12. Impaired absorption of nutrients leading to: Malnutrition Muscle wasting (legs and Buttocks) Anemia Anorexia Abdominal distention Diagnosis

13. Impaired absorption of nutrients leading to: Behavioral changes: Irritability Fretfulness Uncooperativeness Apathy Diagnosis

14. Celiac crisis (in very young infants): Present as acute, severe episodes of profuse watery diarrhea and vomiting Diagnosis

15. Celiac crisis (in very young infants): May be precipitated by: Infections (especially in the GI) Prolonged fluid and electrolyte depletion Emotional disturbances Diagnosis

16. Celiac crisis (in very young infants): First incidence may be failure to thrive and diarrhea Definitive diagnosis is by Jejunal biopsy that shows atrophy and changes in the small intestine The improvement after gluten free diet is also diagnostic which occur within a day or two Diagnosis

17. Celiac crisis (in very young infants): Change in personality, weight gain, increase in the appetite, and decrease in diarrhea and steatorrhea occur after several days or weeks Histological and or clinical relapse follow gluten reintroduction Diagnosis

18. Dietary management: Restricted Gluten diet Corn and rice are given instead Supplemental vitamins, Iron and Calories are given for severe malnutrition Treatment NO

19. It is a chronic disease Most severe symptoms occur in early childhood and again in adult life Strict dietary avoidance can prevent symptoms and may minimize the risk of developing lymphoma one of the most serious complications Prognosis

20. To help the parents and the child to adhere to the prescribed diet To explain the disease process to them Advice parents to read all ingredients on labels to avoid hidden sources of Gluten as in “Hydrolyzed vegetable protein” e.g. Hotdogs , Pizza and Spaghetti , Instant soup these are not allowed. Nursing considerations

21. In severe cases Disaccharides digestion is impaired especially in relation to Lactose , so temporary Lactose – free diet given, and (milk products) are restricted from the diet Diet should be high in calories and proteins with simple carbohydrate , fruits and vegetables and low fat. The rough vegetables and fruits are avoided until acute inflammation heals Any faulty in the diet will establish the previous signs and symptoms. Nursing considerations

22. Identify the common problems affecting the gastrointestinal system during childhood? Provide nursing care to a child with malabsorption syndrome by implementing the nursing process? Identify nutritional therapies for the child with a malabsorption syndrome? Evaluation

23. Hockenberry , M. J., Wilson, D., (2015) Wong’s Nursing Care of Infants and Children 10th Edition. Mosby, St Louis MO. Pillitteri , A.: Maternal And Child Health Nursing: care of the childbearing and childrearing family, 2010, 6th edition References

24. Thank you You can find me at: [email protected]