How we treat sickle hepatopathy and liver transplantation in adults

Gardner, Kate; Suddle, Abid; Kane, Pauline; O’Grady, John; Heaton, Nigel; Bomford, Adrian; Thein, Swee Lay

doi:10.1182/blood-2013-12-542076

Cited by 79 publications

(95 citation statements)

References 39 publications

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“…However, serum albumin and direct bilirubin showed no statistically significant difference. So, our study shares similar findings with Gardner K et al 2014 and Mahera MM et al 2009 [9,10]. These findings suggest that a multifactorial etiology can exist for occurrence of liver disease in SCA patients…”

Section: Discussion:-supporting

confidence: 92%

Liver Involvement in Sickle Cell Trait: A Case Control Study Among Nepalese Indigenous Tharu Community.

Bhatta¹,

Thapa²,

Ranabhat³

et al. 2017

IJAR

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Background: Sickle cell trait is not usually regarded as a disease state because it may present several types of health complications that are either uncommon or mild and have not been thoroughly investigated. This article aims to describe the relationship between sickle cell trait (SCT) and liver function test (LFT) parameters in tharu community of western tarai region of Nepal. Method: Sixty two (62) subjects divided into control subjects (n = 31) and heterozygous sickle cell patients ( n = 31) were recruited for the study and were investigated for a liver function tests including the measurement of total protein, albumin, globulin, total bilirubin, direct bilirubin, indirect bilirubin, aspartate transaminase (AST), alanine transaminase (ALT) and lactate dehydrogenase (LDH) in both groups. Mean, Standard deviation, Students T test analysis were used for analysis of results. Result: The serum total protein, globulin, total bilirubin, indirect bilirubin, aspartate transaminase (AST), alanine transaminase (ALT) and lactate dehydrogenase (LDH) were significantly higher in SCT patients than in controls, with P values of 0.014, <0.001, 0.003, 0.006, <0.001, <0.001 and <0.001 respectively. However, there were no significant difference in the mean value of albumin and direct bilirubin. There was strong correlation noticed between AST and LDH in the test group. Conclusion: It appears that abnormal liver function tests in patients with sickle cell trait poses an uncertain threat for liver disease. The liver functions of the SCT patients were significantly compromised as compared to controls. Studies at molecular level are needed to understand the pathophysiology and progression of liver disease in sickle cell anemia. It is advisable that liver function tests be interpreted with caution in these patients.

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Section: Discussion:-supporting

confidence: 92%

Liver Involvement in Sickle Cell Trait: A Case Control Study Among Nepalese Indigenous Tharu Community.

Bhatta¹,

Thapa²,

Ranabhat³

et al. 2017

IJAR

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“…In addition, our patient presented with multiple predisposing factors for SCIC, including male gender and hepatic fibrosis secondary to iron overload. In haemosiderosis, non-transferrin-bound iron can induce reactive oxygen species, which can result in cellular and endothelial dysfunction; this, in turn, promotes intrahepatic sickling 1. This intrahepatic destruction in the setting of baseline haemosiderosis likely exacerbated the acuity of our patient's clinical failure.…”

Section: Outcome and Follow-upmentioning

confidence: 88%

Acute on chronic liver failure in a patient with sickle cell anaemia (HbSS)

Essien

DePasse

et al. 2015

BMJ Case Reports

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A man in his late 40s with sickle cell anaemia (HbSS) presented to the emergency department with 2 weeks of diffuse oedema, increased abdominal girth and dyspnoea. His anasarca was thought to be indicative of an acute decompensation of his known liver cirrhosis with transfusion-induced haemosiderosis. While his anasarca improved with diuresis, his direct hyperbilirubinaemia suddenly worsened without any signs of haemolysis, biliary disease or obstruction. He also developed an acute worsening in serum creatinine (1.17-7.0 mg/dL in 7 days) despite subsequent treatment for presumed hepatorenal syndrome (HRS). Given his clinical decline, the patient's goals of care were transitioned to comfort measures only. His clinical presentation and rapid liver and renal deterioration were most typical of sickle cell intrahepatic cholestasis (SCIC). SCIC can lead to rapid deterioration in renal function and can be mistaken for HRS. When SCIC is suspected, consideration of exchange transfusions should be made early.

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“…The number of significant annual VOC was determined by discharge summaries averaged over a 5-year period. Sickle hepatopathy, which is a clinical diagnosis, 26 was identified in the CWRU-UH population in five patients who had a persistent elevation in alanine transaminase or direct bilirubin, in the appropriate clinical setting. Estimated glomerular filtration rate was calculated using the 'Modification of Diet in Renal Disease' four-variable equation.…”

Section: Methodsmentioning

confidence: 99%