Interventions for treating intrahepatic cholestasis in people with sickle cell disease

Review question

We aimed to review the evidence for treating intrahepatic cholestasis (liver diseases where bile is either not formed or excreted properly (or both)) in people with sickle cell disease.

Background

Sickle cell disease is an inherited condition and the most common hemoglobinopathy occurring worldwide. In 2006, the WHO declared sickle cell disease as a major public health problem with an estimated 70% of sufferers living in Africa. It is common among people with sub-Saharan African, Indian, Middle Eastern or Mediterranean ancestry.

Sickle cell disease affects the hepatobiliary system (liver, gall bladder, bile ducts), with repeated reduced blood flow and the formation of bilirubin stones (a type of gallstone). This results from sickled cells blocking blood vessels and from a reduction in the lifespan of these cells. Sickle cell intrahepatic cholestasis or sickle cell hepatopathy (abnormal or diseased liver) is one complication of the disease. When diagnosed, people with this condition may show an intense yellowing of skin and eyes due to an increasing level of serum bilirubin (jaundice), tiredness, generalized itching, increasing pain in the upper right quadrant of the abdomen due to gallstones, an enlarged liver and lower hemoglobin levels.

Sickle cell intrahepatic cholestasis, while uncommon, is a potentially fatal complication with a high death rate. There is no agreement in how to diagnose or treat this condition. We wanted to assess the benefits and harms of treatments for intrahepatic cholestasis in people with sickle cell disease.

Search date

The evidence is current to 10 October 2014.

Study characteristics

We were not able to find any trials assessing any treatment for this condition.

Key results

No trials were identified.

Quality of the evidence

There is no randomised controlled trial evidence of any intervention for treating intrahepatic cholestasis in people with sickle cell disease. Trials are needed to establish the best treatment for this condition.

Authors' conclusions: 

This Cochrane Review did not identify any randomised controlled trials assessing interventions for treating intrahepatic cholestasis in people with sickle cell disease. Randomised controlled trials are needed to establish the optimum treatment for this condition.

Read the full abstract...
Background: 

Sickle cell disease is the most common hemoglobinopathy occurring worldwide and sickle cell intrahepatic cholestasis is a complication long recognized in this population. Cholestatic liver diseases are characterized by impaired formation or excretion (or both) of bile from the liver. There is a need to assess the clinical benefits and harms of the interventions used to treat intrahepatic cholestasis in people with sickle cell disease.

Objectives: 

To assess the benefits and harms of the interventions for treating intrahepatic cholestasis in people with sickle cell disease.

Search strategy: 

We searched the Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings. We also searched the LILACS database (1982 to 7 July 2014) and the WHO International Clinical Trials Registry Platform Search Portal (7 July 2014).

Date of last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register: 10 October 2014.

Selection criteria: 

We searched for published or unpublished randomised controlled trials.

Data collection and analysis: 

Each author intended to independently extract data and assess the risk of bias of the trials by standard Cochrane Collaboration methodologies; however, no trials were included in the review.

Main results: 

There were no randomised controlled trials identified.

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