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Paediatric Pulmonology and Allergology
2008 October, Vol. XI, No. 2 (3969-3987)
CYSTIC FIBROSIS: NOW A MULTISYSTEM DISEASE
Andrew Bush
Professor of Paediatric Respirology, Imperial School of Medicine at National Heart and Lung Institute and Honorary Consultant Paediatric Chest Physician, Royal Brompton Hospital, London, UK
Twenty years ago, cystic fibrosis (CF) was considered a disease mainly of children, affecting the lungs and the digestive systems. Diagnosis was often late. Recent changes include diagnosis in the newborn period by screening, and specialist care in dedicated multidisciplinary clinics. The aggressive treatment of infections and good nutritional management has lead to an increase in survival, so that shortly there will be more adults than children with the disease. The nature of the disease is now appreciated to affect nearly all systems in the body, including the bones and the genitourinary system. In case of CF liver disease is difficult to detect screening with ultrasound is recommended. Insulin deficiency occurs in young children, and seriously adversely affects prognosis. There should be a low threshold for starting insulin treatment in CF children should be screened for low bone mineral density; in the future, this is likely to start in early childhood, rather than adolescence as currently recommended. Infertility in CF males is virtually universal, and this should be discussed before puberty. Important other manifestations of CF include electrolyte disorders, arthopathies, vasculitis and renal disease. These new complications have lead to searches for new preventative strategies in childhood, as well as posing novel treatment challenges in adults. Finally, it is becoming appreciated that complications which were thought to be the province of the adult CF patient are actually occurring at a much younger age than previously appreciated, and require detection and treatment. Thus insulin deficiency may be found in quite young children, and adversely affect clinical progress. This manuscript reviews recent advances in the identification and treatment of the extra-pulmonary complications of CF.