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NR 602 Restrictive Airway Diseases Guide

NR 602 Restrictive Airway Diseases Guide

NR 602 Restrictive Airway Diseases Guide

Restrictive Airway Diseases

  • Less common in pediatrics
  • Decreased lung compliance with relatively normal flow rates
  • HALLMARK: tachypnea and decreased tidal volume/capacity
  • Causes:
    • Neuromuscular weakness
    • Lobar pneumonia
    • Pleural effusion or mass
    • Severe pectus excavatum
    • Abdominal distention

Asthma *Know Levels of severity*

NR 602 Restrictive Airway Diseases Guide

 

Cystic Fibrosis (CF)

  • Genetic disorder, autosomal recessive, mutation of CFTR protein on chromosome 7
  • Multisystem, progressive disease: COPD, GI disturbances, *exocrine dysfunction
  • Life expectancy: 41 yrs
  • Symptoms:
    • Respiratory: chronic airway inflammation and lung infections, viscous mucus, *mucociliary transport dysfunction, chronic cough, and *excess sputum production, respiratory failure
    • GI: meconium ileus, pancreatic insufficiency, rectal prolapsed, GI obstruction, failure to thrive, edema, hypoproteinemia, steatorrhea, poor muscle mass, GERD, *vitamin deficiencies (A, K, E, D)
    • Hepatic: biliary cirrhosis, jaundice, ascites, hematemesis, esophageal varices, cholelithiasis
    • Endocrine: recurrent acute pancreatitis, CF related diabetes (CFRD)
    • Musculoskeletal: osteoporosis
    • Reproductive: delayed sexual development, nonfunctional vas deferens (male sterility), undescended testes, hydrocele, demale decreased fertility, cervicitis
    • Sweat: *“taste salty”, hypochloremic alkalosis, dehydration
  • Diagnostic:
    • Newborn screening performed
    • Gold Standard: pilocarpine iontophoresis sweat test
      • Only ordered if child has more than one clinical feature of CF
      • Sweat chloride concentration > 60 mmol/L (age > 6 months), > 30 mmol/L (in infants)
    • PFTs
    • Glycosylated hemoglobin (elevated)
  • Treatment: complicated, require multidisciplinary team
    • Pulmonary: promote airway clearance
      • Inhaled dornase alfa :reduce mucus viscosity
      • Hypertonic saline: thins mucus
      • Postural drainage (cycle: active breathing, autogenic drainage, percussion, positive expiratory pressure, exercise, high frequency chest wall oscillation) BID
      • High dose Ibuprofen: reduce airway inflammation
      • Azithromycin 3x/week (ibuprofen decreases neutrophil mitigation)
      • Lung transplant
    • GI:
      • Pancreatic enzyme supplementation
      • Vitamin replacement and serum monitoring (A, D, E, K)
      • Osmotic laxatives, Gastrografin enemas
    • Endocrine
      • Glucose tolerance test
      • Diabetes management

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Salmonella

 

Clostridium difficile

 

Cryptosporidium

 

Pyloric Stenosis

 

Pinworms

 

Gastric Esophageal Reflux (GERD)

  • Common in young infants: anatomical reasons
    • Spitting up after meals

Foreign Body Ingestion

  • Common in children exploring their environment with mouths and hands
  • Common locations:
    • Thoracic inlet, pyloris, ileocecal junction
  • Common Culprits: Coins
    • Most pass without problem; 10-20% need surgery
  • Symptoms:
    • Dysphagia
    • odynophagia,
    • drooling,
    • regurgitation,
    • abdominal pain,
    • difficulty breathing

 

Urinary Tract Infection

  • More common in females > uncircumcised male > circumcised males
    • Girls who have > 2 UTIs, urology consult is recommended
    • Boys who have >1 UTIs, urology consult is recommended
  • Lower UTI: uncomplicated, bladder and urethra
  • Upper UTI: complicated, urethra, bladder, ureters, kidneys
    • May require hospitalization
      • Fluid stabilization
      • Treatment
      • Monitoring for sepsis
    • Risk Factors:
      • Perineal irritation (soaps, bubble baths, fragrances, wipes)
      • Not wiping front to back
      • uncircumcised
    • Symptoms:
      • Infants:
        • Fever/hypothermia
        • Jaundice
        • Poor feeding
        • Irritability
        • Vomiting
        • Strong smelling urine
        • Failure to thrive
        • Sepsis
      • Children:
        • Abdominal/ flank pain
        • Urinary frequency
        • Dysuria
        • Urgency
        • Enuresis
        • Vomiting
        • Fever
      • Diagnostics:
        • Urinalysis
        • Urine culture and sensitivity
        • Gram stain
        • Hydration status and electrolyte values
      • Most common cause: coli (85%)
        • Others: Klebsiella, Proteus, Enterococcus, Staphylococcus, and Streptococcus
      • Treatment: dependent on culture, child’s age, and clinical guidelines

Primary Enuresis

 

Glomerulonephritis

  • Result of renal insult caused by immunoglobulin damage to the kidney
  • Red Flag: hematuria
  • Types:
    • Post-infection: most common
      • Post-streptococcal infection: occurs 10 to 14 days post-primary infection
      • Sx: edema, renal insufficiency
      • Dark, tea-colored urine
    • Membranoproliferative
    • IgA nephropathy
    • Henoch – Schonlein purpura (HSP):
      • Most common cause of small vessel vasculitis in children 2-7 yrs old
      • Sx: itching, urticaria, maculopapular rash with purpura on legs, buttocks, and elbows
      • Joint pain
      • 50% chance of renal involvement
    • Systemic lupus
    • Alport syndrome

NR 602 Restrictive Airway Diseases Guide

Osgood-Schlatter

 

Juvenile Rheumatoid Arthirits

 

Osteomyelitis

 

Transcient Synovitis of the Hip

 

Legg-Calve’ – Perthes Disease

 

Idiopathic Scoliosis