Kashani Strom Utley syndrome
Overview
A very rare syndrome characterized mainly by a narrowed pulmonary aorta and urinary obstructive disease.
Symptoms
* Infant feeding problems * Narrow pulmonary aorta * Malabsorption * Chronic diarrhea * Enlarged ureter * Short stature * High blood pressure * Auricular septal defect
Causes
Predisposing factors include cigarette smoking, recurrent or chronic respiratory infections, air pollution, occupational exposure to chemicals, and allergies. Smoking is by far the most important of these factors — it impairs ciliary action and macrophage function, inflames airways, increases mucus production, destroys alveolar septae, and causes peribronchiolar fibrosis. Early inflammatory changes may reverse if the patient stops smoking before lung destruction is extensive. Familial and hereditary factors (such as deficiency of alpha1-antitrypsin) may also predispose a person to COPD. The most common chronic lung disease, COPD (also known as chronic obstructive lung disease) affects an estimated 17 million Americans, and its incidence is rising. It affects more males than females, probably because until recently men were more likely to smoke heavily. COPD occurs mostly in people older than age 40.
Treatment
Treatment is designed to relieve symptoms and prevent complications. Because most patients with COPD receive outpatient treatment, they need comprehensive teaching to help them comply with therapy and understand the nature of this chronic, progressive disease. If programs in pulmonary rehabilitation are available, encourage patients to enroll. Urge the patient to stop smoking. Provide smoking cessation counseling or refer him to a program. Avoid other respiratory irritants, such as secondhand smoke, aerosol spray products, and outdoor air pollution. An air conditioner with an air filter in his home may be helpful. The patient is usually treated with beta-agonist bronchodilators (albuterol or salmeterol), anticholinergic bronchodilators (ipratropium), and corticosteroids (beclomethasone or triamcinolone). These are usually given by metered-dose inhaler, requiring that the patient be taught the correct administration technique. Antibiotics are used to treat respiratory infections. Stress the need to complete the prescribed course of antibiotic therapy.