Internal Medicine

0521779407-C04 CUNY1086/Karliner 0 521 77940 7 June 14, 2007 20:37

Cough Crigler-Najjer Syndrome Type 1 417

specific therapy

■Nasal steroids for allergic rhinitis

■Antihistamines for allergies

■Treat bronchoconstriction with beta-2 agonist and steroid inhalers;

prednisone for severe bronchoconstriction, to break cough cycle,

or as diagnostic test; ipratropium bromide for chronic bronchitis;

leukotriene receptor antagonist for exercise-induced asthma

■Trial of H2 blocker or proton pump inhibitor if GERD present or

suspected

■Prednisone and other immunosuppressive agents for pulmonary

fibrosis

■Antibiotics for URI or pneumonia; intermittent oral and inhaled

antibiotics (to cover Pseudomonas) in bronchiectasis

■Antitussives (dextromethorphan, codeine, hydrocodone) should be

used with caution in patients with impaired mucociliary clearance

follow-up

■Dictated by specific underlying cause

complications and prognosis

■Chronic cough can lead to:

➣Chest wall pain, rib fractures

➣Emesis, esophageal perforation

➣Dizziness, syncope

➣Cardiac arrythmias

➣Pneumothorax, pneumomediastinum, pneumoperitoneum

➣SC, interstitial emphysema

➣Stress incontinence

➣Psychosocial stress

CRIGLER-NAJJER SYNDROME TYPE 1

WILLIAM E. BERQUIST, MD

history & physical

■incidence: rare

■inheritance: autosomal recessive

■newborn jaundice beginning by day 3 of life and persisting

■normal physical exam initially

■possible mental status and general neurologic deterioration to coma

and death (kernicterus)