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J Cyst Fibros. 2018 Dec 14. pii: S1569-1993(18)30937-8. doi: 10.1016/j.jcf.2018.11.009. [Epub ahead of print]

Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands.

Author information

1
The University Medical Center Groningen, Dept. of Pediatrics, University of Groningen, Groningen, the Netherlands, Hanzeplein 1, 9713 GZ Groningen, The Netherlands. Electronic address: f.h.pals@umcg.nl.
2
The University Medical Center Groningen, Dept. of Pediatrics, University of Groningen, Groningen, the Netherlands, Hanzeplein 1, 9713 GZ Groningen, The Netherlands. Electronic address: h.j.verkade@umcg.nl.
3
Dutch Cystic Fibrosis Foundation (NCFS), Baarn, Doctor Albert Schweitzerweg 3, 3744, MG Baarn, The Netherlands. Electronic address: V.Gulmans@ncfs.nl.
4
Erasmus University Medical Center, Rotterdam, Dr, Doctor Molewaterplein 40, 3015, GD, Rotterdam, The Netherlands. Electronic address: b.dekoning@erasmusmc.nl.
5
Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands. Electronic address: b.g.koot@amc.uva.nl.
6
VU University Medical Center, De Boelelaan 1117, 1081, HV, Amsterdam, The Netherlands. Electronic address: t.demeij@vumc.nl.
7
Haga Hospital, The Hague, Els Borst-Eilersplein 275, 2545, AA Den Haag, The Netherlands. Electronic address: d.hendriks@hagaziekenhuis.nl.
8
Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The Netherlands. Electronic address: N.Gierenz@cukz.umcn.nl.
9
Maastricht University Medical Center, P. Debyelaan 25, 6229, HX, Maastricht, The Netherlands. Electronic address: A.vreugdenhil@mumc.nl.
10
Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands, Lundlaan 6, 3584, EA, Utrecht, The Netherlands. Electronic address: r.houwen@umcutrecht.nl.
11
The University Medical Center Groningen, Dept. of Pediatrics, University of Groningen, Groningen, the Netherlands, Hanzeplein 1, 9713 GZ Groningen, The Netherlands. Electronic address: f.a.j.a.bodewes@umcg.nl.

Abstract

BACKGROUND:

Up to 10% of patients with Cystic Fibrosis develop cirrhotic CF-related liver disease with portal hypertension: CF cirrhosis (CFC). In a nationwide study, we aimed to determine the role of CFC on survival in the Netherlands between 1 and 1-2009 and1-1-2015.

METHODS:

We identified all CFC patients in the Netherlands, based on ultrasonographic liver nodularity and portal hypertension. A non-cirrhotic control group was obtained from the national Dutch CF patient registry. We compared groups with regards to baseline lung function and nutritional status and survival and age at death over a 6-year period. In case of death of CFC patients, the clinical reported cause was recorded.

RESULTS:

At baseline, we found no significant difference in lung function and nutritional status between the CFC patients (N = 95) and controls (N = 980). Both the 6-year survival rate (77 vs. 93%; P < .01) and the median age at death (27 vs. 37 years; P = .02) was significantly lower in CFC compared to controls. In the deceased CFC patients, the reported primary cause of death was pulmonary in 68% of cases, and liver failure related in 18% of cases.

CONCLUSIONS:

In the Netherlands, the presence of CFC is associated with a higher risk for early mortality and an approximately 10-year lower median age at death. This substantial poorer outcome of CFC patients was not reflected in a lower baseline lung function or a diminished nutritional status. However, in the case of mortality, the reported primary cause of death in CFC patients is predominantly pulmonary failure and not end-stage liver disease.

KEYWORDS:

Cause of death; Cirrhosis; Cystic fibrosis; Nutritional status; Pulmonary function; Survival rate

PMID:
30558881
DOI:
10.1016/j.jcf.2018.11.009

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