m Congenital Cystic Fibrosis:
· Inherited condition results in generalized dysfunction of the exocrine glands.
· It causes increase sodium chloride in sweats. The mother may notice that the child is salty when kissed.
· It causes pancreatic dysfunction with malabsorbtion and growth retardation.
· Also viscid meconium may cause meconium ileus.
· Plugging of the bronchioles leads to chronic pulmonary disease and emphysema.
· Plugging of the bile ducts leads to biliary cirrhosis.
· Investigations: Increase level of sodium and chloride ions in sweat > 90 mmol. Confirm diagnosis.
· Treatment: Diet rich in salts to replace loss in sweat. Administration of pancreatic enzyme preparation for malabsorbtion. Physiotherapy and antibiotic for pulmonary disease.
m Annular Pancreas:
· Incomplete rotation which make the pancreas surrounds the second or third pat of the dudenum.
· It is one of the causes of vomiting in neonate.
· Treatment: Dudenojejunostomy to bypass the obstruction.
m Ectopic Pancreas:
Pancreatic tissue may be found in the gastric, duodenal, gall bladder or intestinal mucosa. Some reports found ectopic pancreatic tissues in the hylum of spleen and within the liver.
m Congenital Cystic Pancreas:
Sometimes with polycystic kidney and polycystic liver.
SOURCE: DR. AYMAN SALEM'S BOOK
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