PRE HOSPITAL
- Transcutaneous pacing for unstable type II 2nd- or 3rd-degree block
- Atropine:
- Avoid with type II 2nd-degree block because it may precipitate 3rd-degree block.
ED TREATMENT/PROCEDURES
- Stabilize airway, breathing, and circulation:
- Correct fluid, respiratory, electrolyte, and glucose abnormalities.
- Bronchodilators/steroids if wheezing.
- Pneumothorax:
- Observe if <5–10%.
- Thoracostomy
- Consultation with the primary CF physician or pulmonary specialist
- Right heart failure:
- Hemoptysis:
- Blood products as indicated (check INR)
- Ventilatory support
- DIOS:
- Hematemesis:
- Packed RBCs
- Blood products for coagulation abnormalities
- Early consultation with endoscopist
- Intussusception:
- Correct with barium/air enema
- May require surgery
- Rectal prolapse:
- Manual reduction
- Consider surgical consult
- Respiratory care:
- Pulmonary toilet/physical therapy
- Mucous thinning inhaled agents
- Antibiotics for pneumonia:
- Based on culture and sensitivity
- S. aureus (MSSA):
- S. aureus (MRSA):
- P. aeruginosa:
- (Tobramycin or amikacin or colistin) + (piperacillin/tazobactam or ticarcillin/clavulanate or ceftazidime or imipenem/cilastatin or meropenem)
- S. aureus (MSSA) and P. aeruginosa:
- (Piperacillin/tazobactam or ticarcillin/clavulanate or cefepime or imipenem/cilastatin or meropenem) + (tobramycin or amikacin or colistin)
- S. aureus (MRSA) and P. aeruginosa:
- (Vancomycin or linezolid) + coverage for Pseudomonas alone
- B. cepacia:
- Trimethoprim–sulfamethoxazole and/or meropenem and/or cipro and/or minocycline and/or chloramphenicol
- H. influenzae
:
- Cefotaxime or ceftriaxone
- Sinusitis
- Based on cultures and sensitivities
Note: Ciprofloxacin may replace the aminoglycoside if sensitive pseudomonas
- CFTR modulation: Ivacaftor
- Repair of protein function
- Restore airway surface liquid:
- Nebulized hypertonic saline
- Mucous alteration:
- Dornase alpha to thin mucus in lungs
- Future directions:
- Gene therapy: Compacted DNA
- Anti-inflammatory: High-dose ibuprofen
- Anti-infective agents:
- Inhaled tobramycin, aztreonam, colistin
- Continuous vancomycin infusion
- Transplantation: Inhaled cyclosporine
- Nutrition and exercise
MEDICATION
- Amikacin: 7.5–10 mg/kg IV q8h
- Cefazolin: 100 mg/kg/d IV (max.: 6 g/d)
- Cefepime: 50 mg/kg IV q8h (max.: 2 g/8hr)
- Ceftazidime: 50 mg/kg IV q8h (max.: 6 g/d)
- Colistin: 2.5–5 mg/kg/d IV, div. BID–QID
- Imipenem/cilastatin: 15–25 mg/kg IV q6h
- Meropenem: 40 mg/kg IV q8h (max.: 2 g/8hr)
- Nafcillin: 25–50 mg/kg IV q6h (max. 2–3 g q6h)
- Piperacillin/tazobactam: 350–450 mg/kg/d IV (max.: 4.5 g q6h)
- Ticarcillin/clavulanate:300–400 mg/kg/d IV q6h
- Tobramycin: 2.5–3.3 mg/kg/dose IV q8h
- TMP–SMX: 5–10 mg/kg IV q12h (max.: 160 mg TMP q12h)
- Vancomycin: 15 mg/kg q6h (max.: 1 g q6h)
- Note: Because many patients are undernourished, pharmacokinetics of antibiotics (especially aminoglycosides, penicillins, and cephalosporins) may be altered, requiring careful monitoring.
FOLLOW-UP
DISPOSITION
Admission Criteria
- Pulmonary exacerbation with significant deterioration from baseline, hypoxemia, resistant bacteria, failure of outpatient therapy
- Pneumothorax
- Hemoptysis
- Hematemesis
- Intussusception or unexplained abdominal pain or bowel obstruction
- Hyperglycemia
Discharge Criteria
- Close follow-up to verify the sensitivities of culture results and change therapy as needed
- Avoid hot weather.
- Oral salt supplement when profuse sweating
Issues for Referral
All patients followed by a pediatric pulmonary center. Consultation during acute exacerbations.
FOLLOW-UP RECOMMENDATIONS
- Team approach of specialists
- Breathing treatments, chest PT, exercise programs, antibiotics, replacement of pancreatic enzymes
PEARLS AND PITFALLS
- With CF patients in respiratory distress, always consider pneumothorax: Obtain CXR.
- For CF patients with abdominal pain/vomiting, always consider DIOS and intussusception
ADDITIONAL READING
- Hoffman LR, Ramsey BW. Cystic fibrosis therapeutics: The road ahead.
Chest.
2013;143:207–213.
- Ryan G, Jahnke N, Remmington T. Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis.
Cochrane Database Syst Rev.
2012
.
- Willey-Courand DB, Marshall BC. Cystic Fibrosis. AAP
: Pediatric Care Online
2013,
www.pediatriccareonline.org
.
CODES
ICD9
- 277.00 Cystic fibrosis without mention of meconium ileus
- 277.02 Cystic fibrosis with pulmonary manifestations
- 277.03 Cystic fibrosis with gastrointestinal manifestations
ICD10
- E84.0 Cystic fibrosis with pulmonary manifestations
- E84.9 Cystic fibrosis, unspecified
- E84.19 Cystic fibrosis with other intestinal manifestations
DACRYOCYSTITIS AND DACRYOADENITIS
Shari Schabowski
BASICS
DESCRIPTION
- Dacryoadenitis and dacryocystitis are inflammatory conditions affecting the lacrimal system of the eye:
- Dacryoadenitis is inflammation or infection of the lacrimal gland from which tears are secreted.
- Dacryocystitis is an infection within the lacrimal drainage system.
- Dacryoadenitis may be a primarily inflammatory condition or an infectious process resulting from contiguous spread from a local source or systemic infection.
- Dacryocystitis is a suppurative infection involving an obstructed lacrimal duct and sac.
EPIDEMIOLOGY
Dacryoadenitis is an uncommon disorder more commonly seen on the left:
Dacryocystitis is a more common disorder most often occurring in adult females >30 yr old but may be seen in infants
Etiology—Dacryoadenitis
- Most commonly caused by systemic inflammatory conditions:
- Autoimmune diseases
- Sjögren syndrome
- Sarcoidosis
- Crohn's disease
- Tumor
- Infectious causes may be primary or may occur secondary to contiguous spread from bacterial conjunctivitis or periorbital cellulites
- Acute, suppurative:
- Bacteria most common cause in adults:
- Staphylococcus aureus
- Streptococci
- Chlamydia trachomatis
- Neisseria gonorrhea
- Chronic dacryoadenitis:
- Nasal flora > ocular flora
Pediatric Considerations
- Viruses most common cause in children:
- Mumps
- Measles
- Epstein–Barr virus
- Cytomegalovirus
- Coxsackievirus
- Varicella-zoster virus
- Slowly enlarging mass may be dermoid
Etiology—Dacryocystitis
- Under normal conditions, tears drain via pumping action at the lacrimal duct, moving tears to lacrimal sac and then into middle turbinate/sinuses.
- Symptoms begin when duct to lacrimal sac becomes partially or completely obstructed:
- In acquired form, chronic inflammation related to ethmoid sinusitis is a commonly implicated cause but many nasal and systemic inflammatory conditions have been correlated with this process:
- May also occur secondary to trauma, a dacryolith, after nasal or sinus surgery or by any local process that might obstruct flow
- Stasis in this conduit results in overgrowth of bacteria and infection.
- Infection may be recurrent and may become chronic:
- Most common bacteria: Sinus > ocular flora
- S. aureus
is the most common organism
Complications may include formation of draining fistulae, recurrent conjunctivitis, and even abscesses or orbital cellulitis
Pediatric Considerations
- In congenital form, presentation occurs in infancy as a result of dacryocystoceles
- High morbidity and mortality associated with this form:
- Caused by systemic spread of infectious process or bacterial overgrowth in a partially obstructed gland
- The most common organism is
Streptococcus pneumonia.