Pulmonary Lab. HPI 24 yo male pt w/ CF presents to ER with 4 day hx of fevers, chills, hemoptysis, and thick purulent sputum production He has failed.

Pulmonary Lab

HPI

• 24 yo male pt w/ CF presents to ER with 4 day hx of fevers, chills, hemoptysis, and thick purulent sputum production

• He has failed outpatient abx therapy

• Pt has been hospitalized 4x in the last year for recurrent PNAs

PMH/PSH

• Recurrent PNAs• Pancreatic insufficiency (malabsorption)• GERD

• G-tube• Rt sided port

Social Hx

• Pt is non-compliant with home meds

Results of Testing

• Sputum cultures– Multidrug resistant pseudomonas aeruginosa– MSSA– Acinetobacter

Chest Xray• This diagram is a Chest

Radiograph of Cystic Fibrosis (CF) Diffuse Interstitial Disease. This frontal chest X ray in CF shows diffuse interstitial disease with bronchiectasis and nodular densities of mucoid impaction.

Chest CT

• Multicystic space within the base of the left lower lobe which is continuous with the small airways and is representative of cystic bronchiectasis.

Hospital Course

• Pt progresses to respiratory failure• Placed on ventilator• Becomes septic• Unable to wean from vent• Pt expires from sepsis and respiratory failure

Gross Specimen CF Lungs

Bronchiectasis

H&E Low Power Lung, CF

• 1.5x

H&E High Power CF, Lung

• Section of a dilated bronchi with florid acute on chronic inflammation of the bronchial wall and surrounding interstitial fibrosis.

5xBronchiectasis

Treatments for CF, Lung

• Broad spectrum abx• Duonebs• PFTs• Chest physiotherapy – helps pt expectorate sputum• Bronchodilators• Dornase alpha – cleaves DNA, decreases viscosity• Inhaled hypertonic saline – hydrates lung secretions• Anti-inflammatories • Lung Tansplant