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06-06-2008, 09:52 PM
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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