Gastrointestinal Manifestations of Cystic Fibrosis (CF)
- Gastroesophageal reflux
- Constipation / Obstipation
- Distal intestinal obstruction syndrome (DIOS)
- SIBO
- Cystic fibrosis related diabetes
- Other (see below)
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Gastroesophageal reflux
-Typical symptoms for age
Tx:
Constipation / Obstipation
-Abnormal Intestinal secretions
-Dehydrated GI tract
-Pancreatic insufficiency
Tx:
- Osmotic Laxative
- Stimulant laxative, only prn
Distal intestinal obstruction syndrome (DIOS)
-Inspissated intestinal contents in the ileocecal area obstruct the
lumen
-Cramping abd Pain RLQ, distension, +/- vomiting
-Possible stool mass in RLQ
-Dx classic triad: abd pain/distension, RLQ mass, Signs of
obstruction on KUB
Tx:
- If able to tolerate fluids, PO or NG golytely clean-out
- If unable to tolerate PO, attempt enema.
- If unsuccessful, consult Surgery.
SIBO
-Sx: Bloating, flatulence, abdominal pain, watery diarrhea,
dyspepsia, and weight loss.
Tx: Based on breath test: Rifaxamin +/- neomycin (Others:
Metronidazole, Cipro, Augmentin), occasionally used in conjunction
with Prokinetics
Cystic fibrosis related diabetes
Other GI manifestations in CF
- Fibrosing colonopathy - fibrosis, strictures, +/-
ascites (possibly due to excess dose of PERT?)
- Intussusception
- Appendiceal disease
- Rectal Prolapse
- C Diff
- Pancreatic disease (insufficiency, pancreatitis, CF-related
DM)
- CF related Liver disease / Gallbladder disease