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IMPAIRED DIGESTION AND ABSORPTION

IMPAIRED DIGESTION AND ABSORPTION. MALABSORPTION SYNDROMES CELIAC/GLUTEN ENTEROPATHY NONTROPICAL SPRUE CYSTIC FIBROSIS 2009. CELIAC DISEASE. Intolerance to gluten Gluten is a protein found in wheat, barley, rye, and oats

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IMPAIRED DIGESTION AND ABSORPTION

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  1. IMPAIRED DIGESTION AND ABSORPTION MALABSORPTION SYNDROMES CELIAC/GLUTEN ENTEROPATHY NONTROPICAL SPRUE CYSTIC FIBROSIS 2009

  2. CELIAC DISEASE • Intolerance to gluten • Gluten is a protein found in wheat, barley, rye, and oats • Less incidence because of delayed feeding of solids and increased breast feeding

  3. EARLY STAGES OF CELIAC • Fat absorption effected • Result: large quantities of undigested fat in the stool (steatorrhea) • STOOL: frothy, foul odor, excessive quantity

  4. LATER STAGES OF CELIAC Absorption of the following becomes impaired: • Protein • Calcium • Iron • Folic acid • Vitamin D, K, B12

  5. MALABSORPTION OF Fats Proteins and CHO Vit D and calcium Vitamin K Iron, folic acid, vit B12 LEADS TO: 1. Steatorrhea 2. Peripheral edema/malnutrition 3. Osteomalacia and osteoporosis 4. Inadequate blood coagulation 5. anemia RESULTS OF MALABSORPTION

  6. PROGRESSION OF THE DISEASE • If disease process is not stopped GROWTH FAILURE RESULTS • Begins slowly when child starts to ingest grains within 3-6 month of introduction

  7. FIRST EVIDENCE OF DISEASE • Failure to gain weight • Poor appetite • Bout of diarrhea • Steatorrhea • Constipation vomiting • Abdominal pain • Irritability

  8. AS DISEASE PROGRESSES • See signs of general wasting • Some children do not manifest symptoms until 5 years of age

  9. CELIAC CRISIS Acute episodes in which the child has: Profuse, pale, bulky, rancid, poorly formed stools Vomiting Wasted appearance Dependent edema Smooth tongue

  10. CELIAC CRISIS PRECIPITATED BY • GI infections • Prolonged fasting • Eating gluten

  11. DIAGNOSIS • Stool analysis for fat • Serum albumin (for hypoproteinemia) • CBC, Hct, Hgb, for anemia • PT for hypoprothrombinenemia • Serum iron • Folic acid levels • Vit B12 levels • Immunoglobulin levels

  12. DIAGNOSIS CONTINUED • Xrays for bone age • Bowel studies for dilated flaccid bowel loops • Pancreatic function studies • Sweat test to rule out CF • Small bowel bx

  13. TREATMENT • Remove foods from diet having gluten (wheat, rye, barley, oats) • Substitute with corn, rice, potato, hominy • Provide supplements for malnutrition (vitamins, Fe) • High calorie diet • Peripheral hyperal may be required

  14. TREATMENT OF CRISIS • Considered life threatening event • Correct dehydration • Correct metabolic acidosis • NGT to decrease abdominal distention • IV fluids with K, Ca, mg • Albumin infusions to treat hypoproteinemia • IV steroids to decrease inflammation of bowel

  15. AVOID Cereals and baked goods Check food labels for hydrolyzed vegetable protein Check for grains used in processed foods as fillers CANNOT HAVE: Bread, Cake, Cookies, chocolate candy, malts Crackers, Doughnuts, Pies, some ice cream Spaghetti, Pizza Prepared soups, hot dogs Luncheon meats, meat gravy Some prepared hamburgers NURSING CARE: teach about diet

  16. FOODS THEY CAN HAVE • Tacos and Mexican dishes

  17. GENERAL GUIDELINES • Very difficult for adolescents • Must do forever • If diet not followed increased incidence of lymphoma or GI cancer • Anticholinergic drugs precipitate crisis: inform MD’s and dentists

  18. CYSTIC FIBROSIS

  19. DEFINED • Most common serious pulmonary genetic disease in children • Multisymptom disorder affecting the exocrine glands (mucous producing glands) of white children

  20. SURVIVAL • Changed from a short life expectancy in the 1950’s to 50% of patients survive to adulthood, living an average of 33 years

  21. ETIOLOGY • Inherited autosomal recessive trait (inheriting the defective genes from both parents is a 1:4 chance) • Gene responsible is located on chromosome #7

  22. INCIDENCE • 1:1600 births get the disease • 1:20 are carriers; can pass on to children • Equal sex distribution • Seen mostly with whites, rare among African Americans or Asians

  23. PATHOPHYSIOLOGY • Increased viscosity of mucous gland secretions which causes mechanical obstruction in small passages in organs • Elevation of sweat electrolytes: Na and Chloride content of sweat is 2-5 times greater than that of normal children

  24. DIAGNOSIS • Suspected when the child is identified as FTT or suffers frequent repeated URI • Positive family history aids in diagnosis Sweat test: stimulate the production of sweat, collecting & measuring the sweat electrolytes NL SWEAT CHLORIDE: 5-35 mEq/L CHLORIDE greater than 60 mEq/L up to 200 mEq/:: means diagnosis of CF Test is done on two separate occasions

  25. DIAGNOSIS • Chest xray reveals atelectasis and obstructive emphysema • PFT’s indicate abnormally small airway function in CF • Stool analysis for fat • DNA studies are helpful in the 70% of CF carriers; prenatal testing not yet available

  26. RESPIRATORY TRACT CHANGES • Increased viscosity of bronchial mucous leads to slower flow rate of mucous, incomplete expectoration and leads to bronchial obstruction • Retained mucous provides medium for bacterial growth • Reduced O2/CO2 exchange leads to hypoxia, hypercapnia (increased CO2 in blood) acidosis

  27. RESPIRATORY TRACT CHANGES • Atelectasis and emphysema • Repeated infections (s. aureus, h. influenzae, **pseudomonis aeruginosa) • Become resistant to multiple drugs making the bacteria difficult to eradicate • Fibrotic areas develop • Pneumothorax and hemoptysis can occur

  28. GASTROINTESTINAL TRACT CHANGES • Thick secretions in the pancreas block the ducts leading to degeneration and fibrosis • Fibrosis prevents pancreatic enzymes from reaching the duodenum (lipase, trypsin, amylase) which impairs digestion and absorption of fats, proteins and to a lesser degree carbohydrates • Result: excessive stool fat (steatorrhea) and protein (azotorrhea)

  29. GASTROINTESTINAL TRACT CHANGES • Diabetes mellitus develops frequently (may result from the diminished blood supply to the pancreas) • In the liver: biliary obstruction and fibrosis are common leading to biliary cirrhosis leading to portal hypertension • Salivary glands are blocked so this leads to dry mouth and susceptibility to infection

  30. SIGNS AND SYMPTOMS • Vary widely • Could be diagnosed at birth or not until years later • Intensity of involvement varies

  31. RESPIRATORY TRACT SYMPTOMS • Chest congestion, Cough, crackles • Limited exercise tolerance • Sputum production, with hemoptysis • Use of accessory muscles • Decreased pulmonary function • Repeated bronchitis and bronchopneumonia

  32. LABORATORY TESTS RELATED TO RESPIRATORY FUNCTION Arterial blood gas (ABG): acidosis • Decreased PaO2 • Increased PaCO2 • Increased bicarbonate levels • Low pH DECREASED PULSE OX

  33. Progressive disease see over-inflated, barrel shaped chest and cyanosis, clubbing of fingers and toes

  34. GASTROINTESTINAL TRACT SYMPTOMS • IN INFANTS: may be diagnosed as having meconium ileus • SEE signs of intestinal obstruction: abdominal distention, vomiting, failure to pass stools, dehydration

  35. GASTROINTESTINAL TRACT SYMPTOMS • Increased bulk of stools: 2-3 times nl amount, frothy, foul smelling flatus • FTT due to malabsorption • Have good appetite • Abdomen distended from flatus, but extremities are thin • Deficiency fat-soluble vit (A,D,E,K) • Easy bruising (vit K); anemia

  36. REPRODUCTIVE SYSTEM • Females: fertility inhibited by highly viscous cervical secretions • Males: most are sterile

  37. INTEGRUMENTARY SYSTEM: • Parents when kiss infant taste salt • Risk during hyperthermic conditions

  38. TUNE UP • Child hospitalized about every 6 months • Intensive chest PT with vibrating vest • Inhaled antibiotics, IV antibiotics • PURPOSE: prophylactic prevention of serious infections • Done extensively at Dupont Hospital for Children

  39. MANAGEMENT OF PULMONARY PROBLEMS • Prevention of pulmonary infection • Removing secretions • Administering antimicrobials • Administer bronchodilators • Administer antiinflammatory agents • Administer mucolytics

  40. METHODS OF MANAGING PULMONARY PROBLEMS • Daily chest PT twice a day on rising and in evening • Incorporate play (hanging by knees from a bar, somersaults, playing wheelbarrow) • Bronchodilators given before chest PT • Forced expiration (moves secretions)

  41. ANTIBIOTICS • Prophylactic antibiotics • Antibiotics for actual infection • C & S helps guide choice of antibiotics • pseudomonis aeruginosa; this is serious • Inhaled antibiotics helpful: tobramycin • IV antibiotics best: AMINOGLYCOSIDES (class): tobramycin (Nebcin), CARBAPENEMS (class): meropenem (Merrem) • CF metabolize antibiotics rapidly

  42. PULMONARY MANAGEMENT CONTINUED • Oxygen used cautiously because of chronic CO2 retention • Pneumothorax common, may resolve on own or require chest tubes • Hemoptysis with pneumothorax of greater than 300 cc/24 hr is considered to be life threatening

  43. MANAGEMENT OF GASTROINTESTINAL PROBLEMS • Replace pancreatic enzymes with meals and snacks so that when the food reaches the duodenum will have the appropriate enzymes • Use 1-5 with each meal • Comes in capsules, can sprinkle on food

  44. PSYCHOLOGICAL SUPPORT • Dealing with child and family facing a chronic fatal illness • Refer to Cystic Fibrosis Foundation • Child and family may resent the restrictions the disease places on their lives • Constant fear of death

  45. DIET • High in calories (150% of recommended daily allowance) • Fat restriction not necessary • Multivitamins • Vit K • Hyperalimentation for FTT (short term) • Salt supplements in hot weather

  46. SURGICAL MANAGEMENT Lung transplant/pancreatic transplant • Not a cure • Reduces symptoms • Gradual progression of disease • Extends life 10-20 years

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