Colombo, C.

Houwen, R.

Hăuser, B.

Munck, A.

Robberecht, E.

Sermet, I.

Sinaasappel, M.

van der Doer, H.

Walkowiak, J.

Wilschanski, M.

Elsevier - Publisher Connector 

Defining DIOS and constipation in cystic fibrosis: a multicenter study on the incidence characteristics and treatment of DIOS 

9. Gastrointestinal and Liver Disease S79
320* Gastroesophageal reﬂux and aspiration in CF patients
K. Blondeau1, L.J. Dupont2,3, V. Mertens1, G. Verleden3, A. Malfroot4,
Y. Vandenplas4, B. Hauser4, D. Sifrim1. 1Center for gastroenterological Research,
KUleuven, Leuven, Belgium; 2Adult CF center, Division of Respiratory Medicine,
University Hospital Gasthuisberg, Leuven, Belgium; 3Lung Transplant Unit,
Division of Respiratory Medicine, University Hospital Gasthuisberg, Leuven,
Belgium; 4Unit of Pediatric Pulmonology and Gastroenterology, AZ, VUB, Brussel,
Belgium
It is known that GER is increased in CF patients. However, its prevalence,
characteristics, association with aspiration and respiratory impact are not well
characterized. We studied acid and weakly acidic (WA) reﬂux, aspiration and
respiratory symptoms/function in adult CF patients.
Methods: 33 CF patients [19men; 29 (18−55) yrs, 10 post-LTx] underwent
impedance-pH monitoring for detection of acid (pH< 4) and WA GER (pH 4−7).
In 16 patients cough was recorded with oesophageal manometry, and the Symtpom
Association Probability (SAP) was calculated to establish the reﬂux-cough rela-
tionship. Saliva and Bronchoalveolair Lavage Fluid (BALF) were tested for bile
acids (BA).
Results: 28 patients had increased GER (21 acid, 5 WA and 2 acid+WA) and 10 had
a +SAP for reﬂux-cough. Cough ‘inducing’ GER was uncommon. 16/38 patients
had BA in saliva and 6/10 in BALF. This was almost exclusively found in patients
with genotype DF508/DF508. Only 12/28 with increased GER and 9/22 with BA in
saliva/BALF had typical GER symptoms. There was a positive correlation (r = 0.53,
p = 0.03) between oesophageal acid exposure and cough. Patients with +SAP had a
lower lung function than −SAP patients.
Conclusion: Increased GER is prevalent in CF and is not secondary to cough.
Acid GER is most common, but WA GER may also occur. CF patients have a high
risk of aspiration and reﬂux seems associated with more cough and worse lung
function. Outcome studies are needed to conﬁrm the deleterious role of GER in the
progression of CF.
321* Enteropathy – a new ﬁnding in cystic ﬁbrosis
S. Werlin1, I. Benuri-Silbiger2, L. Cohen2, E. Kerem2, M. Aviram3, L. Bentur4,
H. Mussafﬁ5, I. Bjarnasson6, M. Wilschanski2. 1Medical College of Wisconsin,
Milwaukee, WI, USA; 2Hadassah University Hospital, Jerusalem, Israel; 3Soroka
Medical Center, Beer Sheva, Israel; 4Rambam Medical Center, Haifa, Israel;
5Schneider Children’s Hospital, Petach Tiqva, Israel; 6King’s College Hospital,
London, United Kingdom
Background: Treatment with pancreatic enzymes usually fails to completely correct
malabsorption and gastrointestinal symptoms in cystic ﬁbrosis (CF) patients. There
have been no detailed studies of the mid and distal small intestine in CF. The aim of
this study was to examine the small intestine of CF patients without overt evidence
of gastrointestinal disease using capsule endoscopy (CE).
Methods: Patients received the agile patency capsule and, depending on the result
of that procedure, then underwent standard CE using the PillCam SB capsule (Given
Imaging, Yokneam, Israel). A stool specimen was taken for calprotectin level.
Results: 30 CF (26 PI) patients aged 10−36 years were included. One patient failed
to excrete the patency capsule after 36 hours and was withdrawn from the study.
FEV1 was 68.5±16% predicted. Most patients had varying degrees of diffuse areas
of inﬂammatory ﬁndings in the small bowel including edema, erythema, mucosal
breaks and frank ulcerations. Fecal calprotectin levels were markedly high with a
mean of 253±97mg/g (normal <50).
Conclusion: Small bowel mucosal pathology may be detected at CE in most
CF patients. The high fecal calprotectin levels found are suggestive of mucosal
inﬂammation which may correlate with the CE ﬁndings. Further study is required
to examine the possible relationship of this newly identiﬁed CF enteropathy with
gastroenterological symptoms.
Supported by: North American Cystic Fibrosis Foundation.
322* Constipation in pediatric cystic ﬁbrosis patients: an
underestimated medical condition
H.P. van der Doef1, J.W. Woestenenk1, R.H. Houwen1. 1University Medical
Center Utrecht, Utrecht, Netherlands
Objectives: Currently, the role of total fat absorption and dietary intake of ﬂuid
and ﬁber in the development of constipation in Cystic Fibrosis (CF) is unclear. The
aims of this study were therefore to determine risk factors for constipation in CF.
Study design: A retrospective cohort study of 230 pediatric CF patients was
performed and prevalence numbers and risk factors were determined. Constipation
was deﬁned according to the criteria approved by the ESPGHAN in Porto 2005
as (1) abdominal pain and/or distension or (2) reduced frequency and/or increased
consistency of the stools, while (3) the symptoms were relieved by the use of
laxatives. Sixteen patients with complete DIOS, according the criteria approved by
the ESPGHAN in Porto 2005, were excluded as controls.
Results: Hundred-seven out of 230 patients (47%) had a history of constipation,
while 46 out of 230 patients (20%) were constipated at January 1st, 2006. A
history of meconium ileus was more frequent in constipated patients than in patients
without constipation (13% vs. 5%; OR 3.07; 95%CI: 1.07−8.86). Secondly, total
fat absorption was lower in patients with constipation than in patients without
constipation (0.86±0.09 vs. 0.90±0.07; OR 0.003; 95%CI: 0.000−0.28). Finally,
ﬁber and ﬂuid intake were not associated with constipation in CF (OR 0.92; 95%CI:
0.14−6.27 and OR 1.88; 95%CI: 0.35−10.0).
Conclusion: Constipation is a signiﬁcant medical issue in CF patients, with a
prevalence of 47%. The association between constipation and meconium ileus in
CF patients suggest a common defect causing slow transit in the whole intestinal
tract. Furthermore, a low total fat absorption is associated with constipation in CF,
while ﬁber and ﬂuid intake were not associated with constipation in CF.
323* Deﬁning DIOS and constipation in cystic ﬁbrosis: a multicenter
study on the incidence, characteristics and treatment of DIOS
H. van der Doef1, R. Houwen1, I. Sermet2, A. Munck3, B. Hauser4,
J. Walkowiak5, E. Robberecht6, C. Colombo7, M. Sinaasappel8, M. Wilschanski9.
1University Medical Center, Utrecht, Netherlands; 2Hoˆpital Necker, Paris, France;
3Hoˆpital Robert Debre, Paris, France; 4Universitair Ziekenhuis Brussel, Brussels,
Belgium; 5Poznan University of Medical Sciences, Poznan, Poland; 6University
Hospital of Ghent, Ghent, Belgium; 7University of Milan, Milan, Italy; 8University
Medical Center, Rotterdam, Netherlands; 9Hadassah University Hospitals,
Jerusalem, Israel
Objectives: Currently, various deﬁnitions for distal intestinal obstruction syndrome
(DIOS) and constipation in Cystic Fibrosis (CF) are used. The aims of this study
were therefore to seek consensus on the deﬁnitions for DIOS and constipation in
CF and to determine the incidence, characteristics and treatment of children with
DIOS.
Methods: The ESPGHAN CF Working Group discussed the deﬁnition of DIOS
and constipation in CF. Subsequently all patients 18 years with complete DIOS
and diagnosed during the years 2001–2005 in 8 CF centers, were studied.
Results: DIOS was deﬁned as an acute complete or incomplete faecal obstruction
in the ileo-caecum, while constipation was deﬁned as gradual faecal impaction of
the total colon. Fifty-one episodes of DIOS in 39 patients were recorded, giving
an incidence of 6.2 episodes per 1000 patient years. Of the 39 DIOS patients,
20% experienced a relapse, 92% were pancreatic insufﬁcient, 44% had a history of
meconium ileus at birth and 82% had a severe genotype. Conservative treatment
was effective in 49 out of 51 DIOS episodes (96%).
Conclusion: The new deﬁnitions of DIOS and constipation in CF are speciﬁc and
make a clear distinction between these two entities. The incidence of DIOS in the
present study was considerably higher than reported previously.


H. Van Der Doef

R. Houwen

I. Sermet

A. Munck

B. Hauser

J. Walkowiak

E. Robberecht

M. Sinaasappel

M. Wilschanski

C. Colombo

AIR Universita degli studi di Milano

Defining DIOS and constipation in cystic fibrosis : a multicenter study on the incidence, characteristics and treatment of DIOS

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Defining DIOS and constipation in cystic fibrosis: a multicenter study on the incidence characteristics and treatment of DIOS

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