Table 3.

Management recommendations for chronic sickle hepatopathy

Clinical scenarioClinical/investigative dataManagement recommendations
Chronic cholestatic liver disease ? History of acute crises
Chronic elevation in bilirubin
Exclude coincidental liver disease 
Longitudinal assessment
Consider regular exchange transfusion
Consider ursodeoxycholic acid 
End-stage chronic liver disease Often biliary type cirrhosis
Sclerosing cholangitis type injury reported
Exclude coincidental liver disease (eg, autoimmune hepatitis) 
Management of complications of cirrhosis
Role of liver transplant not defined
Consider if
• young patient
• predominant end-organ damage liver 
Iron overload Elevated ferritin
MRI for iron concentration 
Chelation when liver iron concentration >7 mg Fe/g dry weight 
Viral hepatitis Chronic hepatitis B virus
Chronic hepatitis C virus 
Treatment as per normal guidelines
? Avoidance of regimens with ribavirin for hepatitis C virus 
Gallstone disease Diagnosis by standard imaging techniques Cholecystectomy-prophylactic controversial ERCP and duct clearance 
Clinical scenarioClinical/investigative dataManagement recommendations
Chronic cholestatic liver disease ? History of acute crises
Chronic elevation in bilirubin
Exclude coincidental liver disease 
Longitudinal assessment
Consider regular exchange transfusion
Consider ursodeoxycholic acid 
End-stage chronic liver disease Often biliary type cirrhosis
Sclerosing cholangitis type injury reported
Exclude coincidental liver disease (eg, autoimmune hepatitis) 
Management of complications of cirrhosis
Role of liver transplant not defined
Consider if
• young patient
• predominant end-organ damage liver 
Iron overload Elevated ferritin
MRI for iron concentration 
Chelation when liver iron concentration >7 mg Fe/g dry weight 
Viral hepatitis Chronic hepatitis B virus
Chronic hepatitis C virus 
Treatment as per normal guidelines
? Avoidance of regimens with ribavirin for hepatitis C virus 
Gallstone disease Diagnosis by standard imaging techniques Cholecystectomy-prophylactic controversial ERCP and duct clearance 

Adapted from Tavabie and Suddle with permission.

ERCP, endoscopic retrograde cholangiopancreatography.

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