Cystic fibrosis.

Cystic Fibrosis (fibrocystic disease of the pancreas) is a common inherited disease 
which, to date, still has a fatal outcome. The diversity of 
clinical manifestations and pathological findings has added further to the mysterious 
nature of the disease, as have the tantalizingly conflicting results of 
biochemical, physical and experimental studies in many laboratories of the western 
world. One of the most constant clinical observations is the viscosity 
of the secretion of the mucosal glands in the respiratory and 
alimentary tracts, in the ducts of the biliary system and in 
the pancreas. In approximately 10 to 15 per cent of all 
patients with cystic fibrosis of the pancreas the earliest manifestation, meconium 
ileus, occurs in the neonatal period. In the remaining 85 to 
90 per cent of affected infants the symptoms generally become evident 
within the course of the first year of life. The lung 
symptoms tend to dominate the clinical picture in the vast majority 
of untreated patients and will ultimately decide the outcome. The lower 
respiratory tract is not the only part involved: nasal polyps and 
the accumulation of viscous secretions in the paranasal sinuses with subsequent 
bacterial infection are not unusual. Pancreatic deficiency is present in the 
vast majority of patients with cystic fibrosis and consists of a 
gross reduction or complete absence of exocrine secretion. Although some histological 
damage has been seen in the neonatal period in infants suffering 
from meconium ileus, in general the full-blown picture of extensive cirrhosis, 
with portal hypertension, hepatosplenomegaly, and severe and sometimes fatal haemorrhages from 
oesophageal varices, is seen in the older child and adolescent. Screening 
of the newborn to enable the institution of protective measures before 
lung complications arise has been attempted with various techniques. Management includes 
diet and pancreatic enzyme replacement. The treatment of pulmonary disease is 
the most important aspect of care in patients with cystic fibrosis, 
and its prevention is priority number one in the over-all management. 
The outlook for the child born with cystic fibrosis depends to 
a large extent upon the avoidance, or delay until adult life, 
of the two complications that threaten his existence: cor pulmonale and 
cirrhosis of the liver. 