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Pediatrics 8

  1. Celiac disease = gluten-sensitive enteropathy (GSE) = celiac sprue
    1. Etiology: absorption problem with genetic predisposition, possibly immune abnormality
    2. Pathophysiology
      1. inability to digest gliadin (byproduct of gluten); permanent intolerance of gluten.
      2. increasing levels of glutamine in the intestine, toxic to mucosal cells
      3. atrophy of villi and decreased absorptive surface (illustration)
      4. malabsorption of fats, carbohydrates, vitamins and electrolytes
      5. gluten is found in the grain of wheat, barley, rye, and oats
    3. Findings (most often appears between ages of one and five years)
      1. diarrhea (stools: pale and watery, offensive odor)
      2. abdominal distention
      3. failure to thrive
      4. vomiting
      5. muscle wasting
      6. steatorrhea
      7. anorexia
      8. abdominal pain
    4. Diagnostics:
      1. history (symptoms occur three to six months after infant begins eating grains)
      2. serum anti-gliadin antibody (AGA)
      3. jejunal biopsy
    5. Management
      1. diet: gluten-free with vitamin supplements
      2. crisis: IV fluids, steroids
  2. Hirschsprung's disease
    1. Definition / etiology: congenital aganglionic megacolon
    2. Pathophysiology
      1. absence of autonomic (parasympathetic) ganglion cells, usually at rectum and part of large intestine
      2. intestine does not propel stool
      3. stool builds up; colon dilates, constipation results with risk of intestinal rupture
    3. Findings
      1. newborn: failure to pass stool or merconium in first 24 hours of life, reluctance to ingest fluids, bile-stained vomitus, distended abdomen, "ribbon-like" stools - flat, wide, and wavy
      2. later: failure to thrive, distended abdomen, constipation, signs of fecal impaction
    4. Diagnostics: history and physical exam, radiographic barium enema, rectal biopsy, anorectal manometry
    5. Management
      1. surgical correction: remove aganglionic portion
      2. temporary ostomy for three to six months, then reanastomose
  3. Appendicitis (illustration)
    1. Etiology:
      1. virus, impacted fecal material, parasites, foreign body
      2. most common in children school-age or older
    2. Pathophysiology
      1. trigger obstruction increased mucous accumulation
      2. distention, capillary occlusion and engorgement of capillary walls
      3. eventually, abscesses and fistula form
      4. possible perforation leading to peritonitis
    3. Findings
      1. colicky abdominal pain
        1. generalized but usually localizes to lower right quadrant
        2. most intense pain at McBurney's point
      2. nausea, vomiting
      3. possible fever and chills
      4. decreased bowel sounds
      5. guarding of abdomen
      6. rebound tenderness
    4. Diagnostics: physical exam, laboratory tests (CBC with WBC), MRI
    5. Management
      1. surgical removal
      2. IV fluids
      3. antibiotics
      4. analgesics
    6. Concerns: perforation and peritonitis, fluids
    7. Caution: do not give enemas or cathartics or use heating pad if suspected appendicitis
  4. Genitourinary System (illustration)
  5. Common findngs: renal and urinary tract (UT) disorders
  6. Urinary tract infection
    1. Definition
      1. bacteria in urine and inflammatory response
      2. may involve any structure in urinary system: kidney (pyelonephritis), ureters, bladder (cystitis) or urethra
      3. findings will point to location
      4. peak age: toddler to preschool age
    2. Etiology
      1. bacterial, structural defect, physiological
      2. extrinsic (example: foley catheter, medications)
      3. improper perineal hygiene
    3. Pathophysiology
      1. organism usually ascends through urethra to bladder.
      2. with a structural defect, urine flows back from ureters into kidney.
    4. Findings often depend on age
      1. under two years: often nonspecific resembling GI disturbance
        1. failure to thrive, feeding problems, nausea/vomiting,
        2. dysuria, persistent diaper rash, abdominal distention
      2. over two years:
        1. enuresis, daytime incontinence in toilet-trained, foul smelling urine
        2. frequency and urgency, dysuria
        3. possible pyelonephritis: similar signs but with fever, back pain, and lethargy
    1. Diagnostics: urine culture and serum testing, radiographic testing
    2. Management
      1. antimicrobials
      2. antipyretics
      3. analgesics
      4. fluids (IV and oral)
      5. education
        1. wipe front to back (in girls)
        2. do not delay urination
    3. Concerns
      1. hydration
      2. recurrence
      3. long term complications such as scarring, renal failure
  7. Enuresis
    1. Definition: inability to control bladder functions, often due to maturation; nocturnal, or "bed wetting" very common
    2. Etiology/findings
      1. primary: never dry at night, and due to CNS or psychological reasons
      2. secondary: child has been toilet trained and becomes incontinent again
        1. findings as above
        2. due to infection, medications, trauma
    3. Diagnostics: history, urine tests, serum culture
    4. Management
      1. primary
        1. have the child participate in activities
        2. limit fluid at night, void before bedtime
        3. imagery, behavioral conditioning
          • tricyclic antidepressant; imipramine
          • desmopressin (DDAVP)
        4. secondary: treat underlying cause