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Cystic Fibrosis Updated 4/2011

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BASICS

  • Description
  • Epidemiology
  • Risk Factors
  • General Prevention
  • Pathophysiology
  • Associated Conditions

DIAGNOSIS

  • Signs and Symptoms
  • Tests
  • Differential Diagnosis

TREATMENT

  • Medication (Drugs)
  • Additional Treatment
  • Surgery
  • In-patient Considerations

Ongoing Care

  • Follow-Up Recommendations
  • Diet
  • Patient Education
  • Prognosis
The following is an excerpt....
BASICS

Description
  • Cystic fibrosis (CF) is an autosomal-recessive genetic condition that most prominently affects the lungs and pancreas.
  • The intestinal tract, liver, endocrine system, reproductive organs, and skin can all be involved.
  • Initially a pediatric disease, CF has become a chronic pediatric and adult medical condition as improvements in medical care have led to a dramatic increase in long-term survival.
Epidemiology

CF is the most common lethal inherited disease in Caucasians and is found in every racial group.

Incidence
  • Prevalence varies according to country and ethnic background.
  • Number of infants born with CF in relation to the total number of live births in the US:
    • 1 in 2,270 Ashkenazi Jewish Caucasian
    • 1 in 3,000 Caucasians
    • 1 ...

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FIGURE 1.26. A 40-year-old man who developed a left pneumothorax while being ventilated for acute respiratory distress syndrome (ARDS). A: Baseline chest radiograph shows diffuse parenchymal airspace opacity compatible with ARDS. B: A chest radiograph obtained during respiratory deterioration shows a new lucency over the region of left hemidiaphragm (deep anterior sulcus). There is also increased depth of the lateral costophrenic sulcus. The thin white line of pneumothorax is evident superiorly (arrows). Credit: From Crapo JD, MD, Glassroth J, MD, Karlinsky JB, MD, MBA, and King TE, Jr., MD. Baum's Textbook of Pulmonary Diseases, 7th Edition. Philadelphia: Lippincott Williams & Wilkins, 2004.
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