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Published byἙκάβη Αλαφούζος Modified over 6 years ago
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A 5 yr boy the a with first episode of cough, wheezing and shortness of breath
Giovanni A. Rossi Genova
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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
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Chest X-ray at admission, December 12th…
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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
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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
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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
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CT scan, bronchoscopy and biopsy on December 30th
3:00 p.m. Tracheostomy 9:00 a.m. Inflammatory myofibroblastic tumor of the trachea
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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?
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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:
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Thank you Genoa, Italy
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Thank you
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