Focus on gastroesophageal reflux disease in patients with cystic fibrosis

Table 3 Diagnostic and therapeutic management of gastroesophageal reflux disease in pediatric patients with cystic fibrosis

	Infants	Children
Typical symptoms	Excessive crying, back arching, regurgitation, irritability	Heartburn, regurgitation
Diagnostic test
Indication
PPIs trial	Not indicated; Exclude anatomically abnormalities	12 year-old children with typical symptoms. Not use a trial of PPIs as a diagnostic test for GERD in patients presenting with extraesophageal symptoms
Barium swallow	Not indicated, useful to exclude anatomical abnormalities	Not indicated, useful to exclude anatomical abnormalities
Endoscopy	Indicated in the presence of the alarm symptoms or to detect complications of GERD; to diagnose conditions that predispose or mimic GERD	Indicated in the presence of alarm symptoms or to detect complications of GERD, to diagnose conditions that predispose to GERD (such as hiatal hernia) or to diagnose conditions that might mimic GERD (such as eosinophilic esophagitis, infectious esophagitis)
Esophageal manometry	Not indicated
		Not indicated
	Not indicated
Scintigraphy		Not indicated
	Correlate persistent
Ph-MII	Extraesophageal symptoms with acid and non-acid GER events; Determine the efficacy of acid suppression therapy. Differentiate NERD, hypersensitive oesophagus and functional heartburn in patients with normal endoscopy	Correlate persistent extraesophageal symptoms with acid and non-acid GER events. Determine the efficacy of acid suppression therapy. Differentiate NERD, hypersensitive oesophagus and functional heartburn in patients with normal endoscopy
Therapy
Alginate	Absence of evidence	Absence of evidence
PPIs	First-line treatment of reflux-related erosive esophagitis with GERD	First line of treatment in children with typical symptoms of GERD, and erosive esophagitis with GERD
Prokinetics	Not indicated	Not indicated
Fundoplication	Life-threatening complications of GERD after failure of optimal medical treatment; chronic conditions (i.e. neurologically impaired, cystic fibrosis) with a significant risk of GERD-related complications	Life-threatening complications of GERD after failure of optimal medical treatment; Chronic conditions (i.e. neurologically impaired, cystic fibrosis) with a significant risk of GERD-related complications
GERD-related complications	Barrett’s esophagus	Barrett’s esophagus

PPIs: Proton pump inhibitors; GERD: Gastroesophageal reflux disease.