Required Knowledge base to manage patients with pancreatic insufficiency associated with Cystic fibrosis
Dr A.J. Chandrasekhar

Primary manifestations of pancreatic involvement in cystic fibrosis

Fat maldigestion
Protein maldigestion
Fecal losses

Patho-physiology of pancreatic insufficiency in Cystic fibrosis

The disorder is characterized by

Epithelial secretary dysfunction
Ductal obstructive lesions throughout multiple organ systems
Disturbances of mucus and electrolyte secretion
Defective chloride permeability has been described in Pancreas.

Normally, the pancreas secretes in response to secretin.

Chloride
Bicarbonate
Water

In cystic fibrosis this response is greatly diminished, resulting in

Dehydrated secretions
High protein concentrations
Thickened mucus.

The pancreatic lesions are in general caused by obstruction of small ducts by secretions and cellular debris although stenosis of large pancreatic ducts is occasionally seen.

Pathological findings of Pancreas in Cystic fibrosis.

When severely affected, the pancreas is shrunken, cystic, fibrotic, and fatty. Histologically

Hyperplasia and necrosis of ductular and centroacinar cells
Inspissated secretions
Blockage of pancreatic ductules
Encroach upon acini, causing flattening and atrophy of the epithelium
Cystic spaces fill with calcium-rich, eosinophilic concretions.
A mild inflammatory reaction may be present around obstructed acini, and progressive fibrosis gradually separates and replaces the pancreatic lobules.
The islets of Langerhans are spared in most cases until late in the process and are concentrated in the shrinking pancreas.

Radiological features of Pancreatic disease in Cystic fibrosis

Calcification, although rare, may be apparent on radiographs. Ultrasonography, magnetic resonance imaging (MRI), and computed tomographic (CT) scanning all can document the progression of pancreatic disease in cystic fibrosis. There are three general patterns of abnormality seen on MRI:

complete replacement with fatty tissue
a small atrophic pancreas with partial replacement with fat (often a hyperintense rim)
diffuse atrophy without evidence of fatty replacement

ERCP: Duodenal aspirate in patient with pancreatic insufficiency due to Cystic fibrosis Duodenal aspirates from patients with cystic fibrosis and complete pancreatic achylia are of

Small volume
Viscous
Contain low concentrations of pancreatic enzymes and bicarbonate.

CCK and secretin fail to stimulate fluid or enzyme secretion.

Patients without absorptive defects may have normal or even elevated concentrations of pancreatic enzymes, but pancreatic secretions remain scanty and viscid and contain low concentrations of bicarbonate even after stimulation.