Cystic Fibrosis Is Treatment with Ursodeoxycholic Acid of Value?

Strandvik, Birgitta; Lindblad, Anders

doi:10.3109/00365529409103628

Cited by 10 publications

(7 citation statements)

References 35 publications

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“…Our results are consistent with those of other authors who observed that children with end‐stage liver disease associated with CF benefit from transplantation as long as pulmonary function is not severely compromised (5, 15–17). Children with severe pulmonary and liver disease may benefit from combined transplantation (18, 19).…”

Section: Discussionsupporting

confidence: 93%

“…Delta F508 is the most frequent CF mutation (3) and is found in many patients with meconium ileus, occlusive intestinal disease, liver disease, cholestasis, and malnutrition (4). The most common hepatic complications of CF are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis (5). Liver transplantation by itself or in combination with lung or kidney has a role in the management of children with CF (6).…”

mentioning

confidence: 99%

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Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population

Molmenti

Squires

Nagata

et al. 2003

Pediatric Transplantation

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The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10-12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78-5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata, and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas, Klebsiella, and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23-19.09, median 9.61). Survivors were transplanted at a younger age than non-survivors (mean of 9.21 yr vs. 10.66 yr), and had shorter waiting times from diagnosis of end-stage liver disease to transplantation (6.87 months vs. 13.83 months). Eighty percentage (n = 8) of patients had pretransplant variceal bleeds (83% of survivors, 75% of non-survivors). While all non-survivors had a history of meconium ileus and preoperative need of pancreatic enzymes, only 67% of those alive experienced these complications. Preoperative forced vital capacity FVC was 103% for survivors and 95% for non-survivors. The corresponding numbers for forced expiratory flow (FEF) 25-75 were 74-84% respectively. Preoperative Aspergillus was identified in 30% of patients (n = 3). Two of these patients are alive. Cystic fibrosis constitutes an indication for 3.5% of pediatric liver transplants. Evaluation and transplantation for end-stage liver disease associated with cystic fibrosis should be undertaken at an early age. Most deaths were associated with pulmonary/septic events, and occurred less than 2 yr after OLTX. Those children who ...

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Section: Discussionsupporting

confidence: 93%

mentioning

confidence: 99%

Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population

Molmenti

Squires

Nagata

et al. 2003

Pediatric Transplantation

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“…Although UDCA has not been proven to be of any benefit in resolving established stones, it is possible that prophylactic implementation decreases the advance of liver fibrosis and cirrhosis by acting as a choleretic and improving the bile acid mix to a less hydrophobic one [115,116]. Although UDCA has not been proven to be of any benefit in resolving established stones, it is possible that prophylactic implementation decreases the advance of liver fibrosis and cirrhosis by acting as a choleretic and improving the bile acid mix to a less hydrophobic one [115,116].…”

Section: Cystic Fibrosis and Biliary Tract Diseasementioning

confidence: 99%

Biliary Disease in Infants and Children

Superina¹

2006

Diseases of the Gallbladder and Bile Ducts

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In judicandus ye are a physician, in curando, a surgeon. The patient asks for cure -surgery -and not for theory -medicine -it is the doctor who needs the latter. That is: there can be no surgeon who is not also a physician; the latter begets the surgeon and the surgeon tests the physician by the result of his work. Paracelsus O B J EC T I V E S• Become familiar with the diagnostic approach to the child with jaundice • Understand the pathogenesis and treatment strategies used in children with biliary atresia • Classify and discuss the treatment strategies in children with choledochal cysts • List the varying types of progressive familial intrahepatic cholestasis (PFIC)

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“…In patients with CF, the administration of UDCA should decrease bile viscosity, improve biliary drainage, and displace cytotoxic bile acids (157). A series of uncontrolled studies have reported the positive effects of UDCA in patients with CF‐associated liver disease; there is consistent and sustained improvement in biochemical indices related to cytolysis and cholestasis (136‐142). Three controlled trials have confirmed the beneficial effects of UDCA; administration of this drug has been reported to reduce cholestasis and improve the nutritional status of patients with CF (143‐145).…”

Section: Udca In Pediatric Hepatobiliary Diseasementioning

confidence: 99%

“…Preliminary reports have indicated the potential for UDCA therapy in various forms of chronic cholestasis in children (1,2,95,136‐156) (Table 3).…”

Section: Udca In Pediatric Hepatobiliary Diseasementioning

confidence: 99%

Bile Acid Therapy in Pediatric Hepatobiliary Disease: The Role of Ursodeoxycholic Acid

Balistreri

1997

J. pediatr. gastroenterol. nutr.

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Cystic Fibrosis Is Treatment with Ursodeoxycholic Acid of Value?

Cited by 10 publications

References 35 publications

Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population

Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population

Biliary Disease in Infants and Children

Bile Acid Therapy in Pediatric Hepatobiliary Disease: The Role of Ursodeoxycholic Acid

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