1. A 5 yr boy the a with first episode of cough, wheezing and shortness of breath
Giovanni A. Rossi
Genova

2. Case history
A 5-year-old boy with an unremarkable personal history was admitted with shortness of breath and dyspnoea
He was well till December 9th, when he started to complain dry cough with progressive shortness of breath
He was treated at home with chlarytromicin and beclometasone plus albuterol by nebulization, with no improvement in symptoms
He was then brought to the Gaslini Institute Emergency Department
At admission, on December 12th, he was in fair general conditions
The lung auscultation showed inspiratory and expiratory wheezes with rales over both lung fields
The body temperature was = 36.7°C
SaO2 = 97% in room air
WBC 11,690/m3, N 90,5%
CRP = 1.93 mg/dl

3. Chest X-ray at admission, December 12th…

4. In the Respiratory Diseases unit …
Flow
Baseline
After Salbutamol 200 g
He was treated with:
Dexamethasone
Nebulization with albuterol and ipratropium bromide
Chlarytromicin
Oxygen supplementation for dyspnoea relieve
A spirometry was performed on December 13th
Mild-to moderate restriction
Tendency to a paradox response to salbutamol

5. Symptoms and PFTs improved in 5 days …
December 13th
After salbutamol
December 18th
3 days
salbutamol +
systemic CSs
and O2 therapy
Sent home with salbutamol, beclomethasone and tapering doses of dexamethasone

6. Readmission 12 days after on December 30th
Out of dexamethasone treatment on December 27th
December 18th
Rapid progressive deterioration of pulmonary function
Back to the Gaslini Institute, on December 30th
Dyspnoea with inspiratory and expiratory wheeze with flow limitation
SaO2 = 97% in room air

7. CT scan, bronchoscopy and biopsy on December 30th
3:00 p.m.
Tracheostomy
9:00 a.m.
Inflammatory myofibroblastic tumor of the trachea

8. Inflammatory myofibroblastic tumor (IMT)
IMT is a mesenchymal neoplasm characterized by the presence of proliferating myofibroblasts admixed with inflammatory cells
It was thought to represent a benign post-inflammatory process but reports of aggressive features, including local recurrence, vascular invasion and malignant transformation suggest the need for caution about the prognosis
The treatment of choice is the complete surgical resection, while medical treatment options are limited
The question is: can the corticosteroid-induced immunosuppresion favor tumor growth downregulating the immune response?

10. Corticosteroids and fibroblast proliferation
An enhancing effect of corticosteroids on cell proliferation has been previously observed in airway fibroblasts from asthmatics
Stimulation of G1-S phase transition in cell cycle by dexamethasone resulted in increase in DNA synthesis and this effect was associated with hyperphosphorylation of the tumor suppressor retinoblastoma protein, a process involved in cell progression through the G1/S transition into S phase in the cell cycle
Fouty B. ERJ. 2006; 27: 1160–1167.
Similar findings were reported by Dube J, who demonstrated that proliferation of airway fibroblasts isolated from subjects with mild-to-moderate asthma increased 73% above the negative control after exposure to dexamethasone
Dubé J. Lab Invest 1998; 78:

11. Thank you
Genoa, Italy

12. Thank you