Sleep Problems in Special Pediatric Populations

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					A Challenging Clinical Case

        Holger Link, MD
    Liz Levine, M.D. PGY 2
Doernbecher Pediatric Pulmonary Program
 None
 16 yo teenage boy presented with worsening
  wheezing, coughing, shortness of breath.

 1 mo PTA:
   Fever, coughing, dx with walking PNA, treated with
    Azithromycin for 5 days with some improvement

 Cough gradually worsened with acute onset of
  SOB, wheezing.

 In ED SpO2 82’%. Tx with Duoneb,
  Dexamethasone, and transferred to DCH PICU.
                Further history:
 PMHx: Term infant, no hospitalizations prior to those
  mentioned. No surgeries. No atopy or hx of asthma. 2
  episodes of PNA 2 years ago, both tx as an outpatient.
  Fully vaccinated.
 Fam Hx: No asthma, eczema, allergies. No other
  respiratory problems.
 Social: Junior in HS, quite active usually, plays football.
  Smokes MJ “few times per month” over past 2 yrs, denies
  all other drug use, including tobacco. Has been sexually
  active in past, not in last 8-9 mo.
 Travel: none out of Oregon in last 2 years
 Animals/Exposures: Denies inhaled dust, smoke,
  chemicals, no pets, birds, farm, or other animal exposure
 Meds: Albuterol MDI prn
 Allergies: NKA
 Needing oygen, continuous nebs, mag sulfate,
  IV steroids, bronchodilators gradually weaned,
  transferred to floor after 2 days.

 Pulm consult: recommended CT given unusual
  presentation for asthma.

**Had received 2 days of IV steroids at time of CT
CXR day of admission
Inspiratory chest CT
Inspiratory chest CT
Expiratory chest CT
Expiratory chest CT
Expiratory chest CT
          Clinical course + tests
 CT initial read: significant amount of air trapping, GG
  opacities and concern for a potential bronchiolitis
  obliterans w/cryptogenic pneumonia.

 Workup: AFB neg, Legionella neg, HIV neg, Resp Viral
  panel neg, Hypersensitivity Pneumonitis panel neg,
  Mycoplasma pneumoniae IgM neg, IgG positive.

 He improved from a respiratory standpoint.

 PFT’s with FEV1 102% day of discharge

 Discharged home with a tapering course of oral
  prednisone, albuterol prn and azithromycin.
CBC + Diff
             After discharge:
 Continued to improved while on tapering
  course of Prednisone, just about back to

 F/up in Pulm clinic 3 weeks later: doing well,
  excellent PFT's (FEV1 of 124%), and his
  azithromycin was discontinued
 After finishing course of steroids: increasing
  cough, using albuterol more often, no other
  cold-like sx. Also notes occasional night
  sweats, weight loss of 15-20 lbs over past few

 Cough worsens, increasing SOB, wheezing,
  chest pain.

 Re-admitted to hospital 2 months after initial
  PICU admission.
                Physical exam
 VS: Temp 36.8 °C BP 112/69, HR 98, RR 24, SpO2 94% Wt
  60.6 kg

 Gen: awake, alert, NAD

 HEENT, CV, Abd, Neuro: normal

 Resp: Non-labored, no retractions or nasal flaring,
  currently on 0.5L. Chest shape is normal. Auscultation
  of lungs: slight tachypnea, prolonged expiration with
  wheezing (both insp and exp) in all lung fields.

 Ext: WWP, no clubbing.

 Skin: no rashes, several linear striae noted over lower
                    On admission:
 Initially required 2-5 L O2 via NC to maintain sat’s.
  Albuterol every 2-4 hrs was helping some.

 Labs:
    WBC: 22.8 (Poly 20, lymph 12, mono 4, Eos 64%)

    Lytes, LFT’s ,H/H, plt wnl

    ESR 25, CRP <0.5

    UDS: +Cannabinoids, all others negative

 Imaging: CXR: airway thickening, hyperinflation

 Pulmonology consult requested
CXR second admission
     Bronchoscopy results + course
 Labs: IgE elevated at 867, rest of immunoglobulins wnl.

 Bronchoscopy:

    Mucosa: mild erythema

    Secretions: thick whitish-yellowish secretions in
     bilateral airways. No blood.

 Eosinophilia of 91% on BAL

 Started on Solumedrol x 1 day, followed by Prednisone 60
  mg daily

 On discharge also started on ICS
BAL findings
BAL histology
Pulmonary Eosinophilic Syndromes
Primary                        Secondary
   Acute eosinophilic PNA        Asthma
   Chronic eosinophilic PNA      ABPA
   Churg-Strauss Syndrome
                                  Bronchocentric granulomatosis
   Hypereosinophilic
                                  Collagen vascular diseases
   Eosinophilic granuloma        Drug/toxin reaction

                                  Infection: parasite (Loeffler’s
                                   syndrome), helminthic, TB,
                                   coccidiodes, others

                                  BOOP

                                  Hypersensitivity pneumonitis

                                  Malignancies
Chronic Eosinophilic Pneumonia
 Rare disorder of unclear etiology resulting in
  insidious onset of respiratory symptoms
  accompanied by pulmonary consolidation and
  accumulation of large numbers of eosinophils in
  the lungs, blood and in BAL fluid.
 Based on history, imaging, eosinophilia

 Proposed criteria:
  1) Resp symptoms >2 weeks

  2) Alveolar eosinophilia (>25%, esp >40%) or
    blood eosinophilia (>1000 eos/mm3)

  3) Pulmonary infiltrates on CXR, usually

  4) Exclusion of other causes of eosinophilic
    lung disease
 Unknown initiating stimulus/insult results in
  accumulation of eosinophils in the lungs

 TH2 lymphocytes activatedIL-5, important role in
  recruiting eosinophils, and inhibiting their apoptosis.

 Other chemokines involved: elevated TARC (thymus
  and activation regulated chemokine), eotaxin and
  RANTES act as other chemoattractants of eosinophils
 Female nonsmokers, mean age of 45 yrs

 Female/male ratio 2.1/1 in 1 review article

 Age range 18 to 80 yrs in same article

 Most patients do have asthma (52%),
  atopy in general 63-75%
            Clinical Presentation:
 Gradual , subacute onset of symptoms which can
    Respiratory: cough (+/- productive), wheezing, SOB, chest pain

    Systemic: low grade fevers, weight loss, night sweats, malaise

 Acute respiratory failure, significant hypoxemia seen in
  acute eosinophilic pneumonia

 Rarely, arthralgias, neuropathy, skin findings, GI sx
         Physical findings
 On exam, non specific findings

  Expiratory wheezing in 50%

  Occasionally inspiratory crackles
 Peripheral eosinophilia usually found first
    90% patients had >30% eosinophils

 Elevated inflammatory markers

 Elevated IgE

 PFT’s: inconsistent
    1/3 no abnormality, 1/3 restrictive, 1/3 obstructive

 BAL fluid: >25% eosinophils
    Mean close to 60%

    Normal <2%
             Imaging: CXR

 “Photographic negative of pulmonary edema”
  (though <25% with this finding)

 Bilateral peripheral opacities, often in upper

 Infiltrates can range from ground-glass,
  migratory to dense consolidation
              Imaging: CT

 May help detect small ground glass opacities
  not seen on film

 Study of high resolution CT scan in 81 patients,
  physicians unable to distinguish between CEP
  and bronchiolitis obliterans
 Spontaneous remission in 10%

 Oral corticosteroids: prednisone 0.3-1
   Usually have dramatic response with cessation of

   Marked decrease in peripheral eosinophilia (and
    BAL fluid)

 Recommended course: gradual taper
  between 6-12 months, though some suggest
  trying 6-8 weeks
 >50% relapse, requiring prolonged courses of CS
  for months-years

 Aside from potential for relapse after cessation of
  steroids, general prognosis is quite good with full
  resolution of symptoms and no long-term

 Individual reports of pulmonary fibrosis

 Rare cases have been associated with cutaneous
  T cell lymphoma, sarcoidosis, Churg-strauss
                Final twist…
 “Oil red O stain indicates a 95% positivity for
  lipid laden macrophages. There are numerous
  Charcot-Leyden eosinophilic crystals and a few
  scattered benign goblet cells suggestive of an
  asthma-like respiratory process.”

 No hx of reflux, choking, GERD symptoms

 After this obtained normal MBSS

 Stool O&P obtained, but unable to be processed
  due to presence of barium
BAL histology
 Alam, M. and N. Burki. Chronic eosinophilic pneumonia: a review.
  Southern Medical Journal 2007, 100: 49-53.

 Cakir, C. et al. Chronic eosinophilic pneumonia with mucous plugs in
  a child. Pediatric Pulmonology 2010, 45: 1040-1042.

 Katz, U. and Y. Shoeneld. Pulmonary eosinophilia. Clinic Rev Allergy
  Immunol, 2008, 34:367-371.

 Uptodate

 Weschler, M. Pulmonary eosinophilic syndromes. Immonol Allergy
  Clin N Am, 2007, 27: 477-492.

 Wubbel, C. et al. Chronic eosinophilic pneumonia: a case report and
  national survey. Chest 2003, 123; 1763-1766.

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