MALABSORPTIVE DISEASES OF CHILDHOOD NO LONGER

Cœliac disease and cystic fibrosis are important causes of intestinal malabsorption in childhood. With more prolonged survival in cystic fibrosis and the knowledge that cœliac disease is a lifelong disorder, patients with these conditions will increasingly be cared for by doctors more familiar with diseases of adolescence and adulthood. The clinical features of cœliac disease differ widely in childhood from those in later life, but the inability to properly tolerate gluten is permanent, and the risks of suboptimal health and malignant disease in adulthood suggest that dietary gluten restriction be continued Indefinitely. Better recognition and treatment of cystic fibrosis in recent years have resulted in longer survival, which has in turn revealed a large number of complications after childhood. Chronic pulmonary disease is the most Important clinical feature and the one requiring most careful treatment. Other aspects requiring attention include exocrine pancreatic insufficiency, chronic liver disease, meconium ileus equivalent, diabetes meliltus and reduced fertility. Genetic counselling will become an increasingly important aspect in the management of these patients. The syndrome of pancreatic Insufficiency and chronic neutropenia is the only other significant cause of pancreatic disease in childhood apart from cystic fibrosis. It is suspected that patients with this disorder may have escaped recognition during childhood and survived to adulthood.