Case 56

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Clinical History:
The patient is a 14 year old African-American male who presented at the age of 3 months with dyspnea, cough, tachypnea and failure to thrive. He had an open lung biopsy at 8 months. Since then, he has had repeated respiratory exacerbations. He was on steroids and prophylactic antibiotics and receives oxygen at night.

Clinical Physical Exam:
The patient was thin and almost emaciated. He became short of breath when speaking. Auscultation revealed respiratory crackles. He has 3+ clubbing.

Clinical Labs:
Non-contributory

Clinical Differential Diagnosis:
Bronchiolitis obliterans

Imaging Findings:
Chest films demonstrated prominent interstitial infiltrates. A chest CT exam showed prominent interstitial fibrosis.

Images 1 and 2

Images 3, 4 and 5

Imaging Differential Diagnosis:
Idiopathic pulmonary fibrosis.

Operative Findings:
An open lung biopsy was performed at the age of 8 months.

Pathological Findings:
Pathological examination of the lung biopsy specimen at 8 months revealed proliferative interstitial pneumonia with focal fibromuscular hyperplasia.

Final Diagnosis:
Idiopathic Pulmonary Fibrosis

Follow-up and Prognosis:
The patient was awaiting lung transplantation.

Similar Cases:
None

References:

• International Thoracic Teaching Resource - http://www.vh.org/adult/provider/radiology/ITTR/DIP/DIP.html

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