[Risk factors for cystic fibrosis related liver disease].
OBJECTIVE
To identify the risk factors for cystic fibrosis related liver disease.


METHODS
Ten patients out of a total of 106 patients regularly followed-up during 1999 met the clinical, biochemical and/or ultrasound criteria for liver disease (9.4%). Using information from the admissions records at the service, we collected data on demography, genotype, age and manifestations at diagnosis of cystic fibrosis, nutritional status and laboratory findings. Variables associated with liver disease were initially identified by the Kaplan-Meier method. Those factors that were significant in the univariate analysis were included in the multivariate analysis by means of a Cox regression model.


RESULTS
Under univariate analysis the following factors were associated with liver disease: male sex, age at diagnosis of cystic fibrosis, pancreatic insufficiency, z score for weight at admission, Shwachman score and biochemistry at admission. After adjustment by Cox model, two variables were independently associated with liver disease: Shwachman score (p = 0.0057) and age at diagnosis of cystic fibrosis (p = 0.014).


CONCLUSIONS
The risk of developing liver disease is higher among patients diagnosed at an early age and those with worse clinical status as assessed by the Shwachman score, indicating that liver involvement might be part of a more severe form of the condition. These patients merit greater attention in terms of screening for liver disease and should be given treatment with ursodeoxycholic acid earlier in the event of abnormal findings.
Fagundes
Eleonora D T
ED
Gastroenterologia Pediátrica, Universidade Federal de Minas Gerais (UFMG), Rua Tenente Anastácio de Moura 740/801, Santa Efigênia, CEP 30240-390 Belo Horizonte, Minas Gerais, Brazil. eleonoradruve@uol.com.br
Roquete
Mariza Leitão Valadares
ML
Penna
Francisco J
FJ
Reis
Francisco J C
FJ
Goulart
Eugênio M A
EM
Duque
Cristiano G
CG
D017668:Age of Onset; D001938:Brazil; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D012307:Risk Factors; D012737:Sex Factors