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Cystic Fibrosis

Cystic fibrosis (CF) is a common autosomal recessive disorder of the sodium and chloride channels, and it is characterized by dysfunction of exocrine glands and secondary failure to thrive, frequent foul stools and pancreatic insufficiency. The damage to

Fig. 4.16a-c. Pancreas divisum. a CT: distinctly seperate masses of pancreatic tissue. b,c MRCP: a slightly deformed part of the pancreatic head with the duct of Wirsung (arrow), as well as the rest of the pancreas containing the duct of Santorini (arrowhead)

Fig. 4.16a-c. Pancreas divisum. a CT: distinctly seperate masses of pancreatic tissue. b,c MRCP: a slightly deformed part of the pancreatic head with the duct of Wirsung (arrow), as well as the rest of the pancreas containing the duct of Santorini (arrowhead)

b a c the pancreas starts in utero, with a progressive dilatation of the acini and pancreatic ducts with secondary fibrosis and fatty replacement. Microscopic findings will include atrophy and cyst formation of the pancreatic stroma before the first year of life.

On US, the pancreas with CF is characteristically of an echogenic texture secondary to fatty infiltration (Fig. 4.17). An enlarged pancreas may be seen initially with a subsequent atrophy later in life. Pancreatic duct dilatation and calcifications may be seen. Small cysts (anechoic areas) without vascular communication can be identified. Although a hyperechogenic pancreas is very typical of CF, some other diseases such as Schwachman-Diamond syndrome (exocrine pancreas insufficiency associated with bone marrow dysfunction, cyclic neutrope-nia, metaphyseal diastasis and growth retardation), hemosiderosis, chronic pancreatitis, and administration of steroids may also reveal this feature (Feigelson et al. 2000).

CT may demonstrate an atrophic, fatty pancreas with heterogeneous attenuation. Areas of low attenuation will correspond to cysts, while areas of high attenuation will represent calcifications. Partial or total fatty and fibrous replacement are also commonly seen in these patients. There may be a correlation between the degree of fatty infiltration and the pancreatic exocrine dysfunction (Feigelson et al. 2000).

MRI of CF can depict four different patterns. In the first pattern, a diffuse, high signal intensity with lobular features will be seen; the second will show a diffuse homogeneous hyperintensity without lobular features; in the third, the pancreas will have high signal intensity with focal areas of no change in signal intensity; and in the fourth, a normal pancreas might be observed. In some cases the multiple cysts can be seen throughout the entire gland, replacing the normal tissue. This feature has been called pancreatic cystosis (Feigelson et al. 2000).

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Constipation Prescription

Constipation Prescription

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