Southwest Journal of Pulmonary, Critical Care and Sleep

Seongseok Yun, MD PhD¹

Juhyung Sun, BS²

Laura Howe, MD¹

Roberto Bernardo, MD¹

Sepehr Daheshpour, MD¹

Department of Medicine¹

College of Medicine²

University of Arizona

Tucson, AZ 85724

History of Present Illness

A 28 year-old woman with a history of cystic fibrosis, presented with worsening shortness of breath and cough associated with productive secretions. She was diagnosed with cystic fibrosis when she was 14 months old, and has a history of multiple inpatient admissions for acute pulmonary exacerbation of cystic fibrosis. Her most recent hospitalization was a month prior to this admission, and sputum culture demonstrated methicillin-resistant Staphylococcus aureus, multidrug-resistant Pseudomonas aeruginosa, and Achromobacter xylosoxidans. She was treated with linezolide, meropenum, colistin, and azithromycin with significant symptom improvement, then, discharged home with ciprofloxacin, linezolide and zosyn. However, she developed worsening respiratory distress again and came back to hospital. In the emergency department she required 10 L/min of oxygen to maintain an SpO₂ above 90 %.

PMH

Cystic fibrosis
Seizure
Kidney stone
Portacath placement
Gastrostomy tube placement

Medications

Azithromycin 500 mg 3 times a day
Dornase alpha 1 mg/ml nebulizer twice a day
Fluticasone-salmeterol 500-50 mcg/dose inhaler twice a day
Lipase-protease-amylase 21,000-37,000-61,000 unit 4 caps a day
Cholecalciferol 2,000 unit capsule daily
Ferrous sulfate 325 mg PO twice a day
Ascorbic acid 250 mg PO twice a day
Oxycodone-acetaminophen 10-325 mg 4 times a day as needed

Social History

No smoking
No alcohol use
No recreational drug use

Physical Examination

Vital signs: Temperature 37.3 °C, heart rate 114 beats/min, respiratory rate 20-24 breaths/min, blood pressure 99/69mmHg, SpO2 88-90 % on 10 L NC

General: Alert and oriented X 3, acutely distressed, tachypneic and dyspneic

Skin: Diaphoretic. No rash or lesions.

HEENT: Unremarkable.

Respiratory: Diffuse rales in all lung fields, no wheezing, no stridor

CVS: Tachycardic, regular rhythm, no murmur.

Abdomen: Soft, non-tender, no tenderness, no guarding, no hepato-splenomegaly, PEG tube placed

Lymphatics: No cervical or axillary lymphadenopathy

Extremities: No clubbing, no cyanosis, no peripheral edema, normal tone, normal range of movement

Neurological: Normal speech, no focal neurologic deficit, CN exam within normal range

Laboratory

CBC: WBC 11.9X 10³ /μL, Hb 9.8 g/dL, Hct 30.7%, Platelets 356,000 /μL.

Chemistries: Na⁺ 137 meq/L, K⁺ 4.1 meq/L, Cl^- 107 meq/L, CO₂ 22 mmol/L, blood urea nitrogen (BUN) 13 mg/dL, creatinine 0.7 mg/dL, glucose 106 mg/dL, calcium 8.0 mg/dL, albumin 2.6 g/dL, liver function tests within normal limits.

Prothrombin time (PT) 14.0 sec, international normalized ratio (INR)1.1, partial thromboplastin time (PTT) 37.2sec

Pulmonary Function Test

FVC 48 % (1.95 L), FEV₁ 36 % (1.25 L), FEF_25-75 14 % (0.55 L/sec)

Radiography

An old chest x-ray and thoracic CT scan were reviewed (Figure 1).

Figure 1. Previous PA (Panel A), lateral (Panel B) chest x-ray and representative image from the thoracic CT scan (Panel C).

Which of the following are findings of cystic fibrosis on chest x-ray? (Click on the correct answer to move to the next panel)

Reference as: Yun S, Sun J, Howe L, Bernardo R, Daheshpour S. June 2014 critical care case of the month: acute exacerbation in cystic fibrosis. Southwest J Pulm Crit Care. 2014;8(6):305-19. doi: http://dx.doi.org/10.13175/swjpcc047-14 PDF