Validation of the Williams ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis

Fagundes, Eleonora Druve Tavares; Silva, Rogério A. P.; Roquete, Mariza Leitão Valadares; Penna, Francisco José; Reis, Francisco José Caldeira; Goulart, Eugênio Marcos Andrade; Duque, Cristiano G.

doi:10.2223/jped.1222

Cited by 3 publications

(1 citation statement)

References 14 publications

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“…Regular monitoring of hepatic status should be accomplished in all patients with CF, with liver biochemistry and ultrasound scan included in their routine annual schedule. A simple echographic scoring system based on coarseness of liver parenchyma, nodularity of the liver edge and increased periportal echogenicity is available for the hepatic follow‐up of these patients (32,33). In patients with cirrhosis, it seems prudent to determine alpha‐feto protein levels annually to monitor the possible development of hepatocellular carcinoma (34).…”

Section: Introductionmentioning

confidence: 99%

Liver Disease in Cystic Fibrosis

Colombo

Russo

Zazzeron

et al. 2006

J. pediatr. gastroenterol. nutr.

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Liver involvement in Cystic Fibrosis (CF) is much less frequent than both pulmonary and pancreatic diseases that are present in 80-90% of CF patients; liver disease (LD) affects only one third of CF patients, however, because of the decreasing mortality from extrahepatic causes, its recognition and management is becoming a relevant clinical issue. Recent observations suggest that clinical expression of LD in CF may be influenced by genetic modifiers; their identification is an important issue because it may allow recognition of patients at risk for the development of LD at the time of diagnosis of CF and early institution of prophylactic strategies. Oral bile acid therapy, aimed at improving biliary secretion in terms of bile viscosity and bile acid composition, is currently the only available therapeutic approach for CF-associated LD. However, the impact of this therapy on the natural history of LD remains to be defined and long-term effectiveness on clinically relevant outcomes should be further investigated. Liver transplantation should be offered to CF patients with progressive liver failure and/or with life-threatening sequelae of portal hypertension, who also have mild pulmonary involvement that is expected to support long-term survival. The 1-year survival rate after transplantation in CF patients is approximately 80%, with beneficial effects on lung function, nutritional status, body composition and quality of life in most cases.

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Section: Introductionmentioning

confidence: 99%

Liver Disease in Cystic Fibrosis

Colombo

Russo

Zazzeron

et al. 2006

J. pediatr. gastroenterol. nutr.

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A single centre experience of liver disease in adults with cystic fibrosis 1995–2006

Nash

Allison

McKeon³

et al. 2008

Journal of Cystic Fibrosis

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Diffuse liver diseases in neonatal and pediatric liver transplant candidates: a pictorial essay

Caruso¹,

Mamone²,

Marrone³

et al. 2010

Clinical Transplantation

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A wide spectrum of common and uncommon diffuse liver diseases affecting neonatal and pediatric liver transplant candidates is presented and analyzed using 16 and 64 multi-detector row helical CT (MDCT) and 1.5 T MRI fast imaging. Correlation of imaging findings and explanted liver or histology is illustrated in representative cases. Associated uncommon congenital anomalies are shown. In conclusion, in pediatric liver transplant candidates, 16-MDCT and 1.5 T fast MRI are useful for diagnosis and staging of liver disease, as well as for the evaluation of associated congenital anomalies.

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Validation of the Williams ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis

Cited by 3 publications

References 14 publications

Liver Disease in Cystic Fibrosis

Liver Disease in Cystic Fibrosis

A single centre experience of liver disease in adults with cystic fibrosis 1995–2006

Diffuse liver diseases in neonatal and pediatric liver transplant candidates: a pictorial essay

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