The GI System

Abdominal pain is a frequent complaint in CF and the second most common reason for admission. Of all the causes only distal intestinal obstruction syndrome (DIOS) and fibrosing colonopathy are unique to CF. Fibrosing colonopathy is found mostly in the paediatric age group although it has been described in adults taking pancreatic supplements. All other causes of abdominal pain in the general population also present in patients with CF and with the same signs and symptoms [4]. The location of pain may give some indication as to the differential diagnosis.

Learning Bite

DIOS and fibrosing colonopathy are unique to CF – all other causes of abdominal pain in the general population also present in patients with CF and with the same signs and symptoms.

Epigastric pain

Epigastric pain with dyspepsia or heartburn suggests gastro-oesophageal reflux disease (GORD)

Pancreatitis (in pancreatic sufficient patients), symptomatic cholelithiasis and gastritis/peptic ulcer disease may also present with epigastric pain and vomiting.

Periumbilical pain

Periumbilical pain is most often cause by DIOS. Intussusception has also been associated with similar symptoms.

Appendicitis, with classical periumbilical pain which then migrates to the right iliac fossa occurs no more frequently in adults with CF, although the symptoms are sometimes mistakenly attributed to DIOS.

It should be noted that some patients will have an appendectomy in infancy or childhood at the time of other surgery, often to the small bowel or caecum for meconium ileum and obstruction shortly after birth.

Constipation can also present with colicky periumbilical pain.

Hypogastric pain

Hypogastric pain and bloating are common complaints in CF. the most frequent cause is malabsorption, which also results in streatorrhoea.

DIOS is likely if there is also distension and intermittent constipation.

Carley, Clostridium Difficile colitis can also cause hypogastric pain, and a history of recent antibiotics, fever and bloody stools support the diagnosis.

DIOS

presents with recurrent colicky periumbilical or right lower quadrant pain, bloating, nausea/vomiting and anorexia. In addition, signs of partial or complete small bowel obstruction, with a tender mass in the right iliac fossa are also typical [4].

Haematemesis

can result from gastritis/peptic ulcer disease, GORD, or from oesophageal variceal bleeding secondary to portal hypertension associated with cirrhosis.