Cystic fibrosis--a challenging masquerader

CF, a generalized dysfunction of the exocrine glands, is an inherited disorder with variable clinical manifestations and prognosis. Because of the multisystem involvement, CF can masquerade as other disorders, including allergy. A high index of suspicion and a knowledge of the variable manifestations of this complex multisystem syndrome are essential for early diagnosis of this challenging masquerader. Quantitative analysis of sweat electrolyte levels after pilocarpine iontophoresis is the cornerstone of the diagnosis of CF. Because respiratory involvement causes most of the morbidity and mortality, aggressive treatment of pulmonary infection and efforts to reduce obstruction of the airways are essential in the treatment of CF. Until the basic defect that causes CF is identified, control or stabilization of the disease and management of its complications, rather than a cure, are the realistic goals of treatment. A comprehensive approach to management includes education of the patient about the disease and its treatment, supportive counseling, dietary supplementation, and aggressive therapy for pulmonary disease.