Fasting Serum Bile Acid Levels in Relation to Liver Histopathology in Cystic Fibrosis

Strandvik, Birgitta; Samuelson, K

doi:10.3109/00365528509091668

Cited by 17 publications

(11 citation statements)

References 19 publications

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“…The etiological theories based on these findings are that the bile causes obstruction of the bile ducts leading to a biliary cirrhosis. Some support for biochemical evidence of cholestasis is sometimes found (17, 18) but this has not been possible to correlate to morphological findings (18)(19)(20). In our patients, we saw bile plugs in the light microscopic sections, but in the thin sections the small bile ducts were rather unaffected in spite of obvious fibrosis around them and had an appearance as has been described previously (21).…”

Section: Discussionsupporting

confidence: 44%

“…One may thus suspect that, whereas a majority of the patients have significant involvement of the liver, they survive and reach adult age (16). A problem with the detection of the liver disease seems to be that the commonly used liver function tests are normal for a long period of time even if the liver is seriously damaged (9, 17,18). This was the case in our material as well, even though at least three patients had obvious fibrosis or even cirrhosis.…”

Section: Discussionmentioning

confidence: 66%

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Morphological findings in the liver of children with cystic fibrosis: A light and electron microscopical study

1986

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Liver tissue from five children with cystic fibrosis, obtained through percutaneous liver biopsies, have been investigated via light and electron microscopy. None of the patients had clinical evidence of liver disorder, and their blood chemistry was mainly normal. Light microscopy showed slight fibrosis in three cases, more advanced fibrosis in one case and focal cirrhotic changes in one case. All patients had fatty infiltration in the hepatocytes and glycogen in the nuclei of these cells. Electron microscopy showed an increase in the number of Ito cells around the portal tracts and also fibrosis in all patients. In the majority of hepatocytes, no evident necrosis was seen. Hypertrophy of the smooth endoplasmic reticulum and the Golgi apparatus were noted. Large lysosomes containing lipofuscin and lipids were also present. No direct evidence of cholestasis could be seen in the hepatocytes. The bile canaliculi were not dilated and did not contain bile plugs. No bile pigment was seen in the cells, and direct evidence of cholestasis was thus not found in the hepatocytes. Other organelles, such as the rough endoplasmic reticulum, peroxisomes and mitochondria, had a normal appearance. Bile ducts, even when seen in fibrotic portal tracts, were not dilated. The ultrastructural findings cannot explain the basis for the liver cell damage. Cholestasis does not seem to be a presumable etiological factor as judged from the findings in the present study.

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Section: Discussionsupporting

confidence: 44%

Section: Discussionmentioning

confidence: 66%

Morphological findings in the liver of children with cystic fibrosis: A light and electron microscopical study

1986

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“…There is limited diagnostic help by routine liver function tests, which often indicate cholestasis without comparative morphological findings and sometimes even show normal levels of transaminases when severe cirrhosis is present. Also in advanced cirrhosis liver function may be well preserved due to the focal character of the liver involvement in CF (14,15).…”

mentioning

confidence: 96%

Cystic Fibrosis Is Treatment with Ursodeoxycholic Acid of Value?

Strandvik

Lindblad

1994

Scandinavian Journal of Gastroenterology

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Strandvik B, Lindblad A. Cystic fibrosis: is treatment with ursodeoxycholic acid of value? Scand J Gastroenterol 1994;29 Suppl 204:65-7 Liver and biliary diseases are common complications in cystic fibrosis (CF) and may even be so severe that liver transplantation is indicated in cases with mild pulmonary disease. The most common complications are steatosis, fibrosis/cirrhosis, micro gallbladder, cholelithiasis, and sclerosing cholangitis. Ursodeoxycholic acid (UDCA) has been used in the treatment of gallstones and pathological liver function in CF. The effect on gallstones has been contradictory, and the effect on liver function tests has been shown to be dose-dependent. A significantly better effect has been shown on liver function tests during treatment over 1 year with doses of 15-20 mg/kg/day. Although supplementation with taurine did not further improve this effect, it has been used in many studies. The biliary bile acid pool was enriched by UDCA from about lo%, to 3 H O % , but a similar increase was not seen in serum. We have studied the long-term effect of UDCA in 10 patients with liver fibrosis or cirrhosis and/or sclerosing cholangitis. Liver function and liver morphology examined on liver biopsies by both light and transmission electron microscopy (TEM) were followed for 2 years. Preliminary results of this study are encouraging. UDCA is an interesting drug which binds to the hepatocyte membranes, stabilizing membrane structure in in vitro experiments and increasing the bicarbonate excretion in bile in pigs. The mechanism of the effect of UDCA in CF might not only be an increased choleresis; further studies will focus on these aspects.

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“…During the period 1976 to 1979, percutaneous liver biopsy was performed in 27 patients (median age 11 years, range 2 to 27 years), in most patients it was performed at least twice with a 1-to 3-year interval. [16][17][18][19] During the years 1980 to 1988 very few biopsies were performed. From 1989 to 1993, liver biopsies were performed in 41 patients, aged 5 years or older (median age 19 years, range 5 to 43 years).…”

mentioning

confidence: 99%

“…[16][17][18][19] During the years 1980 to 1988 very few biopsies were performed. From 1989 to 1993, liver biopsies were performed in 41 patients, aged 5 years or older (median age 19 years, range 5 to 43 years).…”

mentioning

confidence: 99%

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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Fasting Serum Bile Acid Levels in Relation to Liver Histopathology in Cystic Fibrosis

Cited by 17 publications

References 19 publications

Morphological findings in the liver of children with cystic fibrosis: A light and electron microscopical study

Morphological findings in the liver of children with cystic fibrosis: A light and electron microscopical study

Cystic Fibrosis Is Treatment with Ursodeoxycholic Acid of Value?

Natural history of liver disease in cystic fibrosis

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