Liver Disease in Children with Cystic Fibrosis: US-Biochemical Comparison in 195 Patients

Patriquin, H; Lenaerts, C; Smith, Lesley; Perreault, Gilles; Grignon, Andrée; Filiatrault, D; Boisvert, Jonathan; Roy, Claude; Rasquin-Weber, Andrée

doi:10.1148/radiology.211.1.r99ap13229

Cited by 56 publications

(32 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Our study (although in a very different population) mirrored these findings; liver function tests fluctuated significantly over the 24-week period of the study. In a similar study evaluating CF children for liver disease, 63% of those children with an abnormal liver ultrasound had abnormal liver function tests compared with 21% of those children with normal architecture on liver ultrasound [9].…”

Section: Discussionmentioning

confidence: 96%

Laboratory parameter profiles among patients with cystic fibrosis

Goss

Mayer-Hamblett

Kronmal

et al. 2007

Journal of Cystic Fibrosis

View full text Add to dashboard Cite

Background: Clinical trials in cystic fibrosis (CF) currently use laboratory-specific reference ranges to evaluate chemistry and hematology measurements. Laboratory-specific normal reference ranges may not accurately reflect what is abnormal but clinically insignificant among CF patients. Methods: To address this concern, data from the Phase III trial of inhaled tobramycin in CF patients was used to describe the distribution and variability of laboratory parameters. The laboratory specimens were analyzed at a central laboratory after being obtained at baseline and throughout the 24-week trial. Results: At the time of entry into the clinical trial, 91% (463 of 508) of patients had at least a single value outside the normal range. Liver function tests (AST, ALT) were above the normal range in 16% and 12% of the patients respectively, with 2.4% of patients having an AST > 2.0 times the upper limit of normal. Of the 243 patients on placebo, 242 (99.6%) had at least one laboratory parameter that changed from normal to abnormal during the 24-week follow-up period. Of those same placebo patients, 11.5% (N = 28) had a laboratory parameter change from a Common Toxicity Criteria (CTC) grade 0 to grade 2 or higher during follow-up. Conclusions: Patients with CF frequently have laboratory values outside the normal range and have significant longitudinal variability of laboratory values. Interpretation of adverse events in the clinical trial setting may be complicated by the underlying high rates of some laboratory abnormalities in the CF population.This data was presented in poster format at

show abstract

Section: Discussionmentioning

confidence: 96%

Laboratory parameter profiles among patients with cystic fibrosis

Goss

Mayer-Hamblett

Kronmal

et al. 2007

Journal of Cystic Fibrosis

View full text Add to dashboard Cite

show abstract

“…Patriquin et al 11 compared ultrasound findings with hepatic biochemistry alterations (AST, ALT and GGT). Nineteen percent of the 195 patients studied exhibited ultrasound abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Validação do escore ultra-sonográfico de Williams para o diagnóstico da hepatopatia da fibrose cística

Fagundes¹,

Silva²,

Roquete³

et al. 2004

J. Pediatr. (Rio J.)

View full text Add to dashboard Cite

Objectives: To describe the hepatic abnormalities revealed by ultrasound examination of cystic fibrosis (CF) patients followed at the CF Outpatient Clinic at the Federal University of Minas Gerais; to compare ultrasound data with clinical and biochemical parameters; to validate the Williams ultrasound score for the diagnosis of liver disease in CF.Methods: Seventy cystic fibrosis patients were followed prospectively and underwent clinical, biochemical and ultrasound examinations. The ultrasound findings were compared to the results of the clinical and biochemical examinations. Clinical and biochemical criteria were used as the gold standard for the validation of the Williams ultrasound score. We calculated the sensitivity, specificity, and positive and negative predictive values for the Williams score. The patients were divided into two groups: normal (score = 3) or abnormal (score > 3) ultrasound examination.

show abstract

“…As razões para esse achado não foram determinadas. Num corte transversal, Patriquin et al 13 encontraram, numa população de 189 fibrocísticos, elevação da AST, da ALT e da GGT em 26%, 15% e 16% dos pacientes, respectivamente. Ling et al 9 encontraram alterações bioquímicas em 42% dos 124 pacientes, sendo que 27% deles não apresentavam qualquer outra alteração sugestiva de hepatopatia.…”

Section: Discussionunclassified

Triagem diagnóstica da hepatopatia da fibrose cística

Fagundes¹,

Roquete²,

Penna³

et al. 2002

J. Pediatr. (Rio J.)

View full text Add to dashboard Cite

2,5cm da reborda costal direita) firme, e/ou esplenomegalia, e/ou aumento persistente e significativo (>1,5 vezes o limite superior da normalidade, por pelo menos seis meses) de pelo menos duas das enzimas hepáticas (AST, ALT, GGT e FA). Resultados: hepatomegalia foi verificada em sete pacientes (6,6%), e esplenomegalia em cinco (4,7%). A atividade da AST mostrou-se alterada em 18,9% dos pacientes, ALT em 9,4%, GGT em 11,3% e FA em 46,2%. Elevações significativas e persistentes das enzimas hepáticas ocorreram em nove pacientes (8,5%). Dez fibrocísticos (9,4%) preencheram os critérios de hepatopatia. Foram excluídas outras causas de doença hepática. A média de idade dos pacientes com alterações sugestivas de hepatopatia foi de 7,7 anos. Houve predominância absoluta do sexo masculino. Todos os pacientes permanecem assintomáticos. Conclusões: a prevalência da hepatopatia associada à fibrose cística foi de 9,4%. A maior freqüência de elevações transitórias e pouco significativas das enzimas hepáticas é bem documentada na literatura. A sua significância como preditor de hepatopatia ainda não foi determinada. No entanto, esses achados ressaltam a necessidade de avaliações seqüenciais para a definição dos casos de hepatopatia na fibrose cística.]]>

show abstract

Liver Disease in Children with Cystic Fibrosis: US-Biochemical Comparison in 195 Patients

Abstract: The relation between abnormal liver architecture at US and results of three liver function tests in children with CF was significant. The most specific US abnormalities related to abnormal function are signs suggestive of portal hypertension and cirrhosis.

Cited by 56 publications

References 26 publications

Laboratory parameter profiles among patients with cystic fibrosis

Laboratory parameter profiles among patients with cystic fibrosis

Validação do escore ultra-sonográfico de Williams para o diagnóstico da hepatopatia da fibrose cística

Triagem diagnóstica da hepatopatia da fibrose cística

Contact Info

Product

Resources

About