Johns Hopkins CF Center | What is CF? | Diagnosis | Presentations

Johns Hopkins CF Center

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The GI tract is frequently affected in CF patients and approximately 80 percent of CF patients have pancreatic insufficiency. In these patients, thick secretions block the passages within the pancreas leading to damage and scarring, and inadequate enzyme excretion into the intestine. Pancreatic insufficiency leads to malabsorption, failure to gain weight and improper digestion of food, especially fats.

Malabsorption

Where?	Symptoms are confined primarily to abdominal bloating, cramping, or abdominal pain; frequent bulky, greasy or oily, and foul-smelling stools, flatulence (excess gas) and poor weight gain.
What?	Streatorrhea, or pancreatic insufficiency and failure to digest dietary fats resulting in stools with an abnormally high fat content.
Treatment?	Pancreatic enzyme replacement.
Quick Fact?	The lack of pancreatic enzymes in CF patients also prevents them from properly absorbing the fat soluble vitamins (A, E, D, and K), resulting in nutritional deficiencies of these vitamins.

Failure to Thrive
Malabsorption of dietary fats leads to the inability to gain weight. In children, this poor weight gain often results in growth below the standard growth curves and is called “failure to thrive.” Children with failure to thrive should be tested for CF, especially if they have abnormal stools or respiratory problems.