Analysis of risk factors for the development of liver disease associated with cystic fibrosis

Colombo, Carla; Apostolo, M G; Ferrari, Maurizio; Seia, Manuela; Genoni, S.; Giunta, Annamaria; Sereni, Lucia Piceni

doi:10.1016/s0022-3476(94)70361-2

Cited by 172 publications

(138 citation statements)

References 23 publications

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“…9,25 Clinically evident cirrhosis was found in 4%, a figure that is similar to or even less than in earlier reports. 1,2,6,26,34 Recurrent pathological LFT results were noted in all patients who developed cirrhosis. In the 2 patients with moderate fibrosis or cirrhosis and normal US, LFT results had been pathological for several years.…”

Section: Discussionmentioning

confidence: 97%

Natural history of liver disease in cystic fibrosis

1999

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The median age of the population with cystic fibrosis (CF) has increased worldwide, which has led to the suggestion that the prevalence of liver disease would increase. The aim of this study was to evaluate the natural history of CF‐associated liver disease over a 15‐year period in a well‐controlled population of patients with CF. During the years 1976 through 1993, 124 patients were followed up by yearly liver function tests (LFTs). Fifteen patients were followed up with liver biopsies throughout the whole study period. More than 50% of the patients had pathological LFTs in infancy, later being normalized. Approximately 25% of children 4 years of age or older had biochemical markers of liver disease during the study period. In about 10% of the patients, cirrhosis or advanced fibrosis was confirmed at biopsy and 4% of patients had cirrhosis with clinical liver disease. Severe liver disease developed mainly during prepuberty and puberty. Of the 15 patients prospectively followed up with liver biopsies, only 3 had progressive fibrosis. No specific risk factor was identified, but deficiency of essential fatty acids was found more often in patients with marked steatosis (P < .05). No patient developed clinical liver disease in adulthood and the histological changes in the liver biopsies were usually not progressive. Liver disease was no more frequent at the end of the study period although the median age of the patient population had increased. Modern treatment might positively influence liver disease because it seemed less common, less progressive, and less serious than previously reported.

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Section: Discussionmentioning

confidence: 97%

Natural history of liver disease in cystic fibrosis

1999

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“…In CF, however, samples obtained by percutaneous puncture biopsy may not be representative because of the focal nature of the condition. [3][4][5][6] Clinical and biochemical diagnostic criteria also present limitations for the diagnosis of liver disease in CF, although they nevertheless continue to be the most often used follow-up methods. According to recommendations by the Cystic Fibrosis Foundation in the USA, 5 patients should be examined carefully at each visit, with liver and spleen measurements being recorded.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 When, however, biochemical indicators and ultrasound are also included, the prevalence increases significantly. 3,4 Accurate and timely diagnosis of hepatic involvement is desirable due to the therapeutic availability of ursodeoxycholic acid (UDCA). While its efficacy has not yet been proven, early identification appears to be important since these patients respond better than do those with advanced cirrhosis.…”

Section: Original Article Introductionmentioning

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.)

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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.

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“…Colombo et al 11 encontraram alterações persistentes da bioquímica hepática em 16,9% dos pacientes. Neste trabalho, a prevalência dessas alterações foi menor (8,5%).…”

Section: Discussionunclassified

“…A doença hepatobiliar tem sido relatada mais freqüen-temente em pacientes com insuficiência pancreática 10,11 . Na série de Colombo et al 11 e Wilschanski et al 8 , todos os pacientes com doença hepática tinham insuficiência pancreática.…”

Section: Discussionunclassified

Triagem diagnóstica da hepatopatia da fibrose cística

Fagundes¹,

Roquete²,

Penna³

et al. 2002

J. Pediatr. (Rio J.)

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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.]]>

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Analysis of risk factors for the development of liver disease associated with cystic fibrosis

Cited by 172 publications

References 23 publications

Natural history of liver disease in cystic fibrosis

Natural history of liver disease in cystic fibrosis

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

Triagem diagnóstica da hepatopatia da fibrose cística

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