Can the Histologic Changes of Cystic Fibrosis-Associated Hepatobiliary Disease be Predicted by Clinical Criteria?

Potter, Carol; Fishbein, Mark; Hammond, Sue; McCoy, Karen; Qualman, Steve

doi:10.1097/00005176-199707000-00005

Cited by 67 publications

(58 citation statements)

References 14 publications

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“…7), indicating that patients with liver disease might be missed by a too liberal definition of pathological liver enzyme levels used by other investigators. 6,28 Although the diagnostic accuracy is increased by a liver biopsy, this has been questioned in patients with CF owing to the focal character of the liver disease. 8 However, a more recent study showed good agreement between needle and wedge biopsies even in patients with CF, 28 and repeated needle biopsies in individual patients have shown similar morphology over time.…”

Section: Discussionmentioning

confidence: 99%

“…6,28 Although the diagnostic accuracy is increased by a liver biopsy, this has been questioned in patients with CF owing to the focal character of the liver disease. 8 However, a more recent study showed good agreement between needle and wedge biopsies even in patients with CF, 28 and repeated needle biopsies in individual patients have shown similar morphology over time. 19,20 These studies suggest that liver biopsies might be as valuable in patients with CF as in patients with other liver diseases.…”

Section: Discussionmentioning

confidence: 99%

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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: 99%

Section: Discussionmentioning

confidence: 99%

Natural history of liver disease in cystic fibrosis

1999

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“…In most cases, as in the two patients reported here, the clinical and biological abnormalities associated with focal biliary cirrhosis are mild [38]. Hepatomegaly, as observed in the two cases reported here, is present in only 30 to 43% of patients at diagnosis [1].…”

Section: Discussionmentioning

confidence: 75%

Unexpected diagnosis of cystic fibrosis at liver biopsy: a report of four pediatric cases

et al. 2007

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We report here four cases of pediatric patients in whom the diagnosis of cystic fibrosis was made only after the histological examination of a liver specimen obtained by biopsy (three cases) or at autopsy (one case). There were two boys and two girls, aged 13 months to 7.5 years. None had a personal or familial history suggestive of cystic fibrosis. One patient, presenting with myocardial lesion and hepatomegaly, died of heart failure; at autopsy, the liver showed a typical aspect of focal biliary cirrhosis. In the three other cases, liver disease was the only manifestation of cystic fibrosis at the time of diagnosis. Liver biopsy examination showed focal biliary cirrhosis in one case and massive steatosis in two. In all four cases, the diagnosis was confirmed by the existence of known pathogenic mutations in the CFTR gene. The evolution was variable; one patient had progressive liver disease with severe portal hypertension after 7 years; another one had lung complications after 1 year. In conclusion, our experience recalls that the diagnosis of cystic fibrosis must be considered in children presenting with unexplained liver disease; its confirmation by molecular techniques makes it possible to set up an appropriate follow-up.

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“…Although liver biopsy has been considered as the gold standard for assessing stage and grade of most chronic liver diseases, recently more noninvasive tests have been investigated (19). Potter et al have described a clinical score including clinical factors and liver enzymes to predict the type of liver disease in CF with a sensitivity of 85% and a specificity of 82%, whereas its score did not evaluated US abnormality (20). It seems that by delineation of the liver architecture by US, the prevalence of liver disease in children with CF will be augmented.…”

Section: Discussionmentioning

confidence: 99%

Liver Involvement in Iranian Children With Cystic Fibrosis: Ultrasonography and Biochemical Findings

et al. 2013

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Excellence in Tehran, Iran. In all, 114 patients with cystic fibrosis (70 boys, 44 girls) were enrolled. Sample blood test including AST, ALT, GGT and abdominal Sonography was obtained from all patients. Abnormal liver function test was defined by two consecutive occasions; ALT and/ or AST levels were ≥ 2 times the upper limit of normal values. GGT normal values were defined by patient age. Data were analyzed using χ2 test and independent T test. Statistical significance was defined as P values of < 0.05 by SPSS ver.19 software. Results: Abnormal liver function test was detected in CF patients. As well, liver sonogram was abnormal in approximately one-third of the patients. This study showed a higher prevalence of biochemical abnormality in patients with abnormal livers ultrasonography. Conclusions: Noninvasive paraclinical evaluation methods could be recommended in the patients with CF for early detection of silent liver abnormalities before progression to end stage liver disease.

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Can the Histologic Changes of Cystic Fibrosis-Associated Hepatobiliary Disease be Predicted by Clinical Criteria?

Abstract: Significant histologic liver disease is common in cystic fibrosis, although the exact nature of the lesion cannot be predicted without liver biopsy. A clinical liver score that was developed for this may be useful in determining which patients require more definitive evaluation.

Cited by 67 publications

References 14 publications

Natural history of liver disease in cystic fibrosis

Natural history of liver disease in cystic fibrosis

Unexpected diagnosis of cystic fibrosis at liver biopsy: a report of four pediatric cases

Liver Involvement in Iranian Children With Cystic Fibrosis: Ultrasonography and Biochemical Findings

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