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Clinical management of sickle cell liver disease in children and young adults
  1. Eirini Kyrana1,
  2. David Rees2,
  3. Florence Lacaille3,
  4. Emer Fitzpatrick4,
  5. Mark Davenport5,
  6. Nigel Heaton6,
  7. Sue Height7,
  8. Marianne Samyn8,
  9. Fulvio Mavilio9,
  10. Valentine Brousse10,
  11. Abid Suddle11,
  12. Subarna Chakravorty7,12,
  13. Anita Verma11,
  14. Girish Gupte13,
  15. Marki Velangi14,
  16. Baba Inusa15,
  17. Emma Drasar16,
  18. Nedim Hadzic8,
  19. Tassos Grammatikopoulos4,17,
  20. Jonathan Hind4,
  21. Maesha Deheragoda17,
  22. Maria Sellars18,
  23. Anil Dhawan19
  1. 1 Children's Liver Unit, Leeds General Infirmary, Leeds, UK
  2. 2 Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK
  3. 3 Gastroenterology, Hepatology, Nutrition Unit, Hopital Universitaire Necker-Enfants Malades, Paris, France
  4. 4 Paediatric Liver, GI and Nutrition Centre, King's College London, London, UK
  5. 5 Department of Paediatric Surgery, King's College Hospital NHS Foundation Trust, London, UK
  6. 6 Adult and Paediatric Lliver Transplantation, Institute of Liver Studies, King's College Hospital NHS Foundation Trust, London, UK
  7. 7 Paediatric Haematology, King's College Hospital NHS Foundation Trust, London, UK
  8. 8 Paediatric Liver, GI and Nutrition Centre, King's College Hospital NHS Foundation Trust, London, UK
  9. 9 Department of Life Sciences, University of Modena and Reggio Emilia Department of Life Sciences, Modena, Italy
  10. 10 Service de Paediatrie Generale et Maladies Infectieuses, Hopital Necker-Enfants Malades, Paris, France
  11. 11 Institute of Liver Studies, King's College Hospital NHS Foundation Trust, London, UK
  12. 12 Department of Life Sciences, Imperial College London, London, UK
  13. 13 Paediatric Hepatology, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK
  14. 14 Department of Haematology, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK
  15. 15 Children's Sickle Cell and Thalassaemia Centre, Evelina London Children's Healthcare, London, UK
  16. 16 Department of Clinical Haematology, University College London Hospitals NHS Foundation Trust, London, UK
  17. 17 Institute of Liver Studies, Liver Histopathology Department, King's College London, London, UK
  18. 18 Department of Radiology, King's College Hospital NHS Foundation Trust, London, UK
  19. 19 Paediatric Liver, GI and Nutrition Centre and MowatLabs, King's College Hospital NHS Foundation Trust, London, UK
  1. Correspondence to Dr Anil Dhawan, Paediatric Liver, GI and Nutrition Centre and MowatLabs, King's College Hospital, London SE5 9RS, UK; anil.dhawan{at}nhs.net

Abstract

Liver involvement in sickle cell disease (SCD) is often referred to as sickle cell hepatopathy (SCH) and is a complication of SCD which may be associated with significant mortality. This review is based on a round-table workshop between paediatric and adult hepatologists and haematologists and review of the literature. The discussion was prompted by the lack of substantial data and guidance in managing these sometimes very challenging cases. This review provides a structured approach for the diagnosis and management of SCH in children and young adults. The term SCH describes any hepatobiliary dysfunction in the context of SCD. Diagnosis and management of biliary complications, acute hepatic crisis, acute hepatic sequestration and other manifestations of SCH are discussed, as well as the role of liver transplantation and haemopoietic stem cell transplantation in the management of SCH.

  • jaundice
  • gastroenterology
  • adolescent health

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Footnotes

  • Contributors EK, DR, FL, EF, NHe, SH, MSa, FM, VB, AS, SC, ED and AD: participated in the round table discussions and presentations and contributed to the writing and editing of the manuscript. MDa, AV, MDe and MSe contributed in the writing and editing of the manuscript. GG, MV, BI, NHa, TG and JH participated in the round table discussions and presentations and reviewed the manuscript.

  • Funding The workshop was supported by grant 31966 from the British Heart Foundation.

  • Competing interests EK, FL, EF, MDa, NHe, SH, MS, FM, VB, AS, AV, MV, BI, ED, NHa, TG, JH, MDe, MS and AD have nothing to disclose. DR is on advisory boards of Novartis and DSMB of AstraZeneca outside the submitted work. GG reports personal fees from Alexion Pharmaceuticals outside the submitted work. SC reports personal fees from Novartis outside the submitted work. VB reports personal fees from Bluebirdbio and from Addmedica, outside the submitted work.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement No data are available. No data are available for this review.

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