Short stature and pubertal delay in cystic fibrosis

Survival of the patient with cystic fibrosis has been considerably extended in the last two decades due to improved therapy of pulmonary infection, nutritional management, and an organized system of centralized care. Psychological and social aspects of cystic fibrosis may be intensified during adolescence: developing independence in the face of required daily care; developing self-esteem in the face of illness-associated problems such as discolored teeth, malabsorption, short stature, and cough; and participating in group activities despite physical limitations and the disruption in peer relationships when recurrent acute illness is interposed. Pubertal delay is more common in the cystic fibrosis population than the general population, and attendant psychologic dysfunction may be particularly common in males. Comparative studies suggest that this pubertal delay is a function of malnutrition rather than intrinsic to the disease. Normal growth potential may be postulated from somatomedin studies and has been demonstrated in patients treated with long-term aggressive nutritional management. Intervention utilizing testosterone enanthate in males with cystic fibrosis and pubertal delay has resulted in improved rate of growth, progression through puberty, and self-image.