Intrahepatic sickle cholestasis

By:

Khwaja, J

Contributor(s):

Publication details: 2018Uniform titles:

British Journal of Haematology

Online resources:

Click here to access online

Summary: A 26-year-old male patient with sickle cell anaemia (HbSS) presented with a one-week history of back pain, pruritis and jaundice. He did not drink regular alcohol and denied taking any illicit drugs. On admission he was clinically stable, saturating normally on room air, afebrile with significant icterus, distended, soft abdomen and no signs of chronic liver disease. Admission blood tests revealed a marked isolated conjugated hyperbilirubinaemia: Hb 62 g/l, WCC 13.72, Plt 119, PT 13.3s, APTT 38s, fibrinogen 2.74, total bilirubin 768, conjugated 697, Cr 49, ALT 33, ALP 237, AST 103, Albumin 32, GGT 194, reticulocyte count 257 (12.11%). Ferriscan 4 months prior to admis-sion was normal (liver iron concentration 0.7 mg/g dry tissue). A liver ultrasound demonstrated coarsened echotexture without focal lesion and a patent portal vein. No ascites was present. An MRCP showed an enlarged nodular liver in keeping with cirrhosis. A full liver screen was negative. A N-Acetylcysteine infusion alongside prophylactic antibiotics and antifungal were administered. He was transfused multiple pack red cells which reduced the Haemoglobin S percentage from 68.9 to less than 30% however his bilirubin remained above 800 and he began to spike fevers. A week after admission a transjugular biopsy was attempted however bleeding was noted in the neck after one attempt of micropuncture and he developed significant pain all over. Over the next three days he developed grade 1-2 encephalopathy, acute renal injury (Cr 446) and coagu-lopathy. He was transferred to the Intensive Care Unit after a tran-shepatic biopsy. This confirmed intrahepatic sickle cholestasis. Unfortunately he developed significant post operatively bleeding and subsequent loss of cardiac output which responded to aggressive blood product support. He returned to theatre to pack the biopsy site without suture. Over the following week he developed worsening multiorgan failure and lactic acidosis and treatment was withdrawn. This case highlighted the poor prognosis of intrahepatic sickling and the complications of biopsy and need for specialist management. [Conference abstract]

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A 26-year-old male patient with sickle cell anaemia (HbSS) presented with a one-week history of back pain, pruritis and jaundice. He did not drink regular alcohol and denied taking any illicit drugs. On admission he was clinically stable, saturating normally on room air, afebrile with significant icterus, distended, soft abdomen and no signs of chronic liver disease. Admission blood tests revealed a marked isolated conjugated hyperbilirubinaemia: Hb 62 g/l, WCC 13.72, Plt 119, PT 13.3s, APTT 38s, fibrinogen 2.74, total bilirubin 768, conjugated 697, Cr 49, ALT 33, ALP 237, AST 103, Albumin 32, GGT 194, reticulocyte count 257 (12.11%). Ferriscan 4 months prior to admis-sion was normal (liver iron concentration 0.7 mg/g dry tissue). A liver ultrasound demonstrated coarsened echotexture without focal lesion and a patent portal vein. No ascites was present. An MRCP showed an enlarged nodular liver in keeping with cirrhosis. A full liver screen was negative. A N-Acetylcysteine infusion alongside prophylactic antibiotics and antifungal were administered. He was transfused multiple pack red cells which reduced the Haemoglobin S percentage from 68.9 to less than 30% however his bilirubin remained above 800 and he began to spike fevers. A week after admission a transjugular biopsy was attempted however bleeding was noted in the neck after one attempt of micropuncture and he developed significant pain all over. Over the next three days he developed grade 1-2 encephalopathy, acute renal injury (Cr 446) and coagu-lopathy. He was transferred to the Intensive Care Unit after a tran-shepatic biopsy. This confirmed intrahepatic sickle cholestasis. Unfortunately he developed significant post operatively bleeding and subsequent loss of cardiac output which responded to aggressive blood product support. He returned to theatre to pack the biopsy site without suture. Over the following week he developed worsening multiorgan failure and lactic acidosis and treatment was withdrawn. This case highlighted the poor prognosis of intrahepatic sickling and the complications of biopsy and need for specialist management.&nbsp;[Conference abstract]

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