Cause of symptoms | Mechanism | Typical presentation | Evaluation |
Gastroesophageal reflux disease (GERD) | Lung hyperinflation, medications that reduce LES pressure, delayed gastric emptying. May be associated with constipation and/or generalized dysmotility. | Heartburn, acid brash, increased cough not due to other causes. | Empiric trial of acid-suppressing medication, exclude other causes of symptoms*¶. |
Obstipation/constipation | Abnormal intestinal secretions, dehydrated GI tract, dysmotility, pancreatic insufficiency. Constipation may contribute to GERD by delaying gastric emptying. | Flatulence, poor appetite, stool mass in LLQ. Constipation often occurs in CF despite daily stooling. Difficulty evacuating stool is rare. | History and physical examination; empiric treatment. Abdominal radiograph (if performed) shows stool mass throughout the colon. |
Distal intestinal obstruction syndrome (DIOS) | Inspissated intestinal contents in the ileocecal area, causing complete or incomplete intestinal obstruction. | Acute or subacute onset of abdominal pain and distension with or without vomiting; stool mass in RLQ. | Abdominal radiograph shows stool mass, especially in the RLQ. Often co-occurs with constipation. |
Small intestine bacterial overgrowth (SIBO) | Bacteria in the small intestine cause enterocyte damage and deconjugation of bile salts, causing malabsorption. | Bloating, flatulence, abdominal pain, watery diarrhea, dyspepsia, and weight loss. | Breath hydrogen and methane test, or empiric trial of metronidazole or other anti-SIBO antibiotics. |
Cystic fibrosis-related diabetes (CFRD) | Diabetes is primarily due to impaired insulin secretion; may worsen with pulmonary exacerbations. CFRD may be complicated by neuropathy, which contributes to gastrointestinal dysmotility. | Weight loss, declining pulmonary function, especially in adolescents or young adults. | Oral glucose tolerance testΔ. |
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