Focus on gastroesophageal reflux disease in patients with cystic fibrosis

Table 1 Gastroesophageal reflux disease in adults with cystic fibrosis

Authors	Year	N pts	N (%) pts with GERD	Acid reflux episodes (%)	Weakly acidic reflux episodes (%)	Alkaline reflux episodes (%)	Prox reflux episodes (%)	Patients typical GERD symptoms (%)
Blondeau et al[6]	2008	33	28	21 (63)	5 (15)	ND	ND	ND
Pauwels et al[5]	2011	42	28 (67)	22 (52)	ND	58	42	ND
Ledson et al[38]	1998	50	40 (80)	40 (80)	ND	ND	ND	ND

N: Number; pts: Patients; GERD: Gastroesophageal reflux disease; Prox: Proximal; ND: Not detected.

Table 2 Diagnostic and therapeutic management of gastroesophageal reflux disease in adult patients with cystic fibrosis

Adult patients
Typical symptoms	Heartburn, regurgitation
Diagnostic tests indications
PPIs Trial	Classical symptoms
Barium Swallow	Not for GERD diagnosis
Endoscopy	In presence of alarm symptoms
Esophageal biopsy	Exclude non-GERD diagnosis
Esophageal manometry
Therapy	PPI for eight weeks
Additional options	Lifestyle modifications
	Alginate or antiacide for symptoms relief
Complications associated to therapy	Clostridium difficile infection
	Risk of community-acquired pneumonia
	with a short-term PPI

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

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.

Table 4 Gastroesophageal reflux disease in children with cystic fibrosis

Ref.	Year	N pts	N (%) pts with GERD	Acid reflux; Episodes (%)	Weakly acidic; Reflux episodes (%)	Alkaline reflux episodes (%)	Prox reflux; episodes (%)	Patients typical; GERD symptoms (%)
Blondeau et al[7]	2010	89	24 (27)	70	30	0	25	37.5
Doumit et al[17]	2012	20	10 ( 50)	63	ND	ND	72	ND
Palm et al[33]	2012	35	25 ( 71)	50	ND	ND	N.D.	31
Woodley et al[34]	2014	16	13 ( 81)	ND1	ND	ND	57.9	ND
Hauser et al[35]	2015	28	13 (46)	63.6	32	ND	ND	ND

N: Number; pts: Patients; GERD: Gastroesophageal reflux disease; Prox: Proximal; ND: Not detected.

Table 5 Impact of gastroesophageal reflux disease on pulmonary disease

Ref.	Year	N pts	Median age (yr)	Pts With GERD (%)	N pts with FEV1 > 90%	N pts with FEV1 70%-89%	N pts with FEV1 40%-69%	N pts with FEV1 < 40%	P value Reflux on; Pulmonary function
Gustaffson et al[3]	1991	12	14.4	66	4	2	4	2	P < 0.05
Stringer et al[40]	1988	57	21.6	31	ND	ND	ND	ND	P < 0.01

N: Number; pts: Patients; GERD: Gastroesophageal reflux disease; FEV1: Forced expiratory volume in 1 second l.