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. 2017 Jul 31;7(7):CD010985.
doi: 10.1002/14651858.CD010985.pub3.

Interventions for treating intrahepatic cholestasis in people with sickle cell disease

Affiliations

Interventions for treating intrahepatic cholestasis in people with sickle cell disease

Arturo J Martí-Carvajal et al. Cochrane Database Syst Rev. .

Update in

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. This is an update of a previously published Cochrane Review.

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

Search methods: 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 23 May 2017), the WHO International Clinical Trials Registry Platform Search Portal (23 May 2017) and ClinicalTrials.gov.Date of last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register: 12 April 2017.

Selection criteria: We searched for published or unpublished randomised controlled trials.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 methodologies; however, no trials were included in the review.

Main results: There were no randomised controlled trials identified.

Authors' conclusions: This updated 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.

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Conflict of interest statement

Arturo Martí‐Carvajal: In 2004 he was employed by Eli Lilly to run a four‐hour workshop on 'How to critically appraise clinical trials on osteoporosis and how to teach this'. This activity was not related to his work with the Cochrane Collaboration or any Cochrane Review.

In 2007 he was employed by Merck to run a four‐hour workshop 'How to critically appraise clinical trials and how to teach this'. This activity was not related to his work with the Cochrane Collaboration or any Cochrane Review.

Cristina Martí‐Amarista: none known.

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References

Additional references

Akinsheye 2010
    1. Akinsheye I, Klings ES. Sickle cell anemia and vascular dysfunction: the nitric oxide connection. Journal of Cellular Physiology 2010;224(3):620‐5. [PUBMED: 20578237] - PubMed
Alderson 2000
    1. Alderson P, Roberts I. Should journals publish systematic reviews that find no evidence to guide practice? Examples from injury research. British Medical Journal 2000;320:376‐7. [PUBMED: 10657341] - PMC - PubMed
Alderson 2004
    1. Alderson P. Absence of evidence is not evidence of absence. British Medical Journal 2004;328:476‐7. [PUBMED: 14988165] - PMC - PubMed
Angulo 2000
    1. Angulo P, Patel T, Jorgensen RA, Therneau TM, Lindor KD. Silymarin in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid. Hepatology 2000;32(5):897‐900. [PUBMED: 11050036] - PubMed
Azar 2017
    1. Azar S, Wong TE. Sickle cell disease: a brief update. The Medical Clinics of North America 2017;101(2):375‐93. [PUBMED: 28189177] - PubMed
Azzaroli 2011
    1. Azzaroli F, Turco L, Lisotti A, Calvanese C, Mazzella G. The pharmacological management of intrahepatic cholestasis of pregnancy. Current Clinical Pharmacology 2011;6(1):12‐7. [PUBMED: 21352094] - PubMed
Baichi 2005
    1. Baichi MM, Arifuddin RM, Mantry PS, Bozorgzadeh A, Ryan C. Liver transplantation in sickle cell anemia: a case of acute sickle cell intrahepatic cholestasis and a case of sclerosing cholangitis. Transplantation 2005;80(11):1630‐2. [PUBMED: 16371935] - PubMed
Ballas 2012
    1. Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I, et al. Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management. The Scientific World Journal 2012;2012:949535. [PUBMED: 22924029] - PMC - PubMed
Balshem 2011
    1. Balshem H, Helfand M, Schunemann HJ, Oxman AD, Kunz R, Brozek J, et al. GRADE guidelines: 3. Rating the quality of evidence. Journal of Clinical Epidemiology 2011;64(4):401‐6. [PUBMED: 21208779] - PubMed
Basch 2012
    1. Basch E, Aronson N, Berg A, Flum D, Gabriel S, Goodman SN, et al. Methodological standards and patient‐centeredness in comparative effectiveness research: the PCORI perspective. JAMA 2012;307(15):1636‐40. [PUBMED: 22511692] - PubMed
Brok 2008
    1. Brok J, Thorlund K, Gluud C, Wetterslev J. Trial sequential analysis reveals insufficient information size and potentially false positive results in many meta‐analyses. Journal of Clinical Epidemiology 2008;61(8):763‐9. [PUBMED: 18411040] - PubMed
Brok 2009
    1. Brok J, Thorlund K, Wetterslev J, Gluud C. Apparently conclusive meta‐analyses may be inconclusive‐‐Trial sequential analysis adjustment of random error risk due to repetitive testing of accumulating data in apparently conclusive neonatal meta‐analyses. International Journal of Epidemiology 2009;38(1):287‐98. [PUBMED: 18824466] - PubMed
Brunetta 2011
    1. Brunetta DM, Silva‐Pinto AC, do Carmo Favarin de Macedo M, Bassi SC, Piccolo Feliciano JV, Ribeiro FB, et al. Intrahepatic cholestasis in sickle cell disease: a case report. Anemia 2011;2011:975731. [PUBMED: 21490769] - PMC - PubMed
Caboot 2014
    1. Caboot JB, Allen JL. Hypoxemia in sickle cell disease: significance and management. Paediatric Respiratory Reviews 2014;15(1):17‐23. [PUBMED: 24461342] - PubMed
Carey 2012
    1. Carey EJ, Lindor KD. Current pharmacotherapy for cholestatic liver disease. Expert Opinion on Pharmacotherapy 2012;13(17):2473‐84. [PUBMED: 23094715] - PubMed
Chalmers 2004
    1. Chalmers I. Well informed uncertainties about the effects of treatments. How clinicians and patients respond?. BMJ 2004;328:475‐6. [PUBMED: 14988164] - PMC - PubMed
Chalmers 2014
    1. Chalmers I, Bracken MB, Djulbegovic B, Garattini S, Grant J, Gülmezoglu AM, et al. How to increase value and reduce waste when research priorities are set. Lancet 2014;383(9912):156‐65. [PUBMED: 24411644] - PubMed
Chan 2013
    1. Chan AW, Tetzlaff JM, Gotzsche PC, Altman DG, Mann H, Berlin JA, et al. SPIRIT 2013 explanation and elaboration: guidance for protocols of clinical trials. British Medical Journal 2013;346:e7586. [PUBMED: 23303884] - PMC - PubMed
Chan 2013a
    1. Chan AW, Tetzlaff JM, Altman DG, Laupacis A, Gotzsche PC, Krleza‐Jeric K, et al. SPIRIT 2013 statement: defining standard protocol items for clinical trials. Annals of Internal Medicine 2013;158(3):200‐7. [PUBMED: 23295957] - PMC - PubMed
Chitturi 2002
    1. Chitturi S, George J, Ranjitkumar S, Kench J, Benson W. Exchange transfusion for severe intrahepatic cholestasis associated with sickle cell disease?. Journal of Clinical Gastroenterology 2002; Vol. 35, issue 4:362‐3. [PUBMED: 12352306] - PubMed
Costa 2006
    1. Costa DB, Miksad RA, Buff MS, Wang Y, Dezube BJ. Case of fatal sickle cell intrahepatic cholestasis despite use of exchange transfusion in an African‐American patient. Journal of the National Medical Association 2006;98(7):1183‐7. [PUBMED: 16895293] - PMC - PubMed
Creary 2007
    1. Creary M, Williamson D, Kulkarni R. Sickle cell disease: current activities, public health implications, and future directions. Journal of Women's Health 2007;16:575‐82. [PUBMED: 17627395] - PubMed
CTU 2011
    1. TSA ‐ Trial Sequential Analysis. Copenhagen Trial Unit, Center for Clinical Intervention Research. http://ctu.dk/tsa/ (accessed 29 September 2011).
Danielson 2002
    1. Danielson CF. The role of red blood cell exchange transfusion in the treatment and prevention of complications of sickle cell disease. Therapeutic Apheresis 2002;6(1):24‐31. [PUBMED: 11886573] - PubMed
Delis 2006
    1. Delis SG, Touloumis Z, Bourli A, Madariaga J, Dervenis C. Can exchange transfusions treat postoperative intrahepatic colestasis in patients with sickle cell anemia?. Transplantation Proceedings 2006;38(5):1385‐6. [PUBMED: 16797311] - PubMed
Diringer 2003
    1. Diringer MN. Evidence‐based medicine: what do you do when there's no evidence?. Critical Care Medicine 2003;31(2):659‐60. [PUBMED: 12576991] - PubMed
Donghaile 2013
    1. Donghaile DO, Klein HG. Hemapheresis. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J editor(s). Hematology: Basic Principles and Practice. 6th Edition. Philadelphia: Saunders, 2013:1716‐26. [ISBN: 978‐1‐4377‐2928‐3]
Easterbrook 1991
    1. Easterbrook PJ, Berlin J, Gopalan R, Matthews DR. Publication bias in clinical research. Lancet 1991;337:867‐72. [PUBMED: 1672966] - PubMed
Ebert 2010
    1. Ebert EC, Nagar M, Hagspiel KD. Gastrointestinal and hepatic complications of sickle cell disease. Clinical Gastroenterology and Hepatology 2010;8(6):483‐9; quiz e70. [PUBMED: 20215064] - PubMed
Emre 2000
    1. Emre S, Kitibayashi K, Schwartz ME, Ahn J, Birnbaum A, Thung SN, et al. Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis. Transplantation 2000;69(4):675‐6. [PUBMED: 10708131] - PubMed
Gabriel 2012
    1. Gabriel SE, Normand SL. Getting the Methods Right ‐ The Foundation of Patient‐Centered Outcomes Research. New England Journal of Medicine 2012;367(9):787‐90. [PUBMED: 22830434] - PubMed
Gardner 2014
    1. Gardner K, Suddle A, Kane P, O'Grady J, Heaton N, Bomford A, et al. How we treat sickle hepatopathy and liver transplantation in adults. Blood 2014;123(15):2302‐7. [PUBMED: 24565828] - PubMed
Geenes 2009
    1. Geenes V, Williamson C. Intrahepatic cholestasis of pregnancy. World Journal of Gastroenterology 2009; Vol. 15, issue 17:2049‐66. [PUBMED: 19418576] - PMC - PubMed
Glasziou 2017
    1. Glasziou P, Chalmers I. Can it really be true that 50% of research is unpublished?. http://blogs.bmj.com/bmj/2017/06/05/paul‐glasziou‐and‐iain‐chalmers‐can‐... (accessed 12 June 2017).
Gluud 1998
    1. Gluud C. "Negative trials" are positive!. Journal of Hepatology 1998;28(4):731‐3. [PUBMED: 9566846] - PubMed
Goodman 2013
    1. Goodman SR, Daescu O, Kakhniashvili DG, Zivanic M. The proteomics and interactomics of human erythrocytes. Experimental Biology and Medicine 2013;238(5):509‐18. [PUBMED: 23856902] - PubMed
GRADEPro 2008
    1. GRADE [Computer program]. Brozek J, Oxman A, Schünemann H. Version 3.6 for Windows 2008.
Gutsaeva 2014
    1. Gutsaeva DR, Montero‐Huerta P, Parkerson JB, Yerigenahally SD, Ikuta T, Head CA. Molecular mechanisms underlying synergistic adhesion of sickle red blood cells by hypoxia and low nitric oxide bioavailability. Blood 2014;123(12):1917‐26. [PUBMED: 24429338] - PMC - PubMed
Guyatt 2011a
    1. Guyatt GH, Oxman AD, Kunz R, Brozek J, Alonso‐Coello P, Rind D, et al. GRADE guidelines 6. Rating the quality of evidence‐‐imprecision. Journal of Clinical Epidemiology 2011;64(12):1283‐93. [PUBMED: 21839614] - PubMed
Guyatt 2011b
    1. Guyatt GH, Oxman AD, Kunz R, Woodcock J, Brozek J, Helfand M, et al. GRADE guidelines: 7. Rating the quality of evidence‐‐inconsistency. Journal of Clinical Epidemiology 2011;64(12):1294‐302. [PUBMED: 21803546] - PubMed
Guyatt 2011c
    1. Guyatt GH, Oxman AD, Montori V, Vist G, Kunz R, Brozek J, et al. GRADE guidelines: 5. Rating the quality of evidence‐‐publication bias. Journal of Clinical Epidemiology 2011;64(12):1277‐82. [PUBMED: 21802904] - PubMed
Guyatt 2011d
    1. Guyatt GH, Oxman AD, Kunz R, Woodcock J, Brozek J, Helfand M, et al. GRADE guidelines: 8. Rating the quality of evidence‐‐indirectness. Journal of Clinical Epidemiology 2011;64(12):1303‐10. [PUBMED: 21802903] - PubMed
Guyatt 2011e
    1. Guyatt GH, Oxman AD, Sultan S, Glasziou P, Akl EA, Alonso‐Coello P, et al. GRADE guidelines: 9. Rating up the quality of evidence. Journal of Clinical Epidemiology 2011;64(12):1311‐6. [PUBMED: 21802902] - PubMed
Guyatt 2011f
    1. Guyatt GH, Oxman AD, Vist G, Kunz R, Brozek J, Alonso‐Coello P, et al. GRADE guidelines: 4. Rating the quality of evidence‐‐study limitations (risk of bias). Journal of Clinical Epidemiology 2011;64(4):407‐15. [PUBMED: 21247734] - PubMed
Guyatt 2011g
    1. Guyatt G, Oxman AD, Akl EA, Kunz R, Vist G, Brozek J, et al. GRADE guidelines: 1. Introduction‐GRADE evidence profiles and summary of findings tables. Journal of Clinical Epidemiology 2011;64(4):383‐94. [PUBMED: 21195583] - PubMed
Guyatt 2011h
    1. Guyatt GH, Oxman AD, Kunz R, Atkins D, Brozek J, Vist G, et al. GRADE guidelines: 2. Framing the question and deciding on important outcomes. Journal of Clinical Epidemiology 2011;64(4):395‐400. [PUBMED: 21194891] - PubMed
Guyatt 2011i
    1. Guyatt GH, Oxman AD, Schunemann HJ, Tugwell P, Knottnerus A. GRADE guidelines: a new series of articles in the Journal of Clinical Epidemiology. Journal of Clinical Epidemiology 2011;64(4):380‐2. [PUBMED: 21185693] - PubMed
Guyatt 2012
    1. Guyatt G, Oxman AD, Sultan S, Brozek J, Glasziou P, Alonso‐Coello P, et al. GRADE guidelines 11‐making an overall rating of confidence in effect estimates for a single outcome and for all outcomes. Journal of Clinical Epidemiology 2012;66(2):151‐157. [PUBMED: 22542023] - PubMed
Higgins 2003
    1. Higgins JP, Thompson SG, Deeks JJ, Altman DG. Measuring inconsistency in meta‐analyses. British Medical Journal 2003;327(7414):557‐60. [PUBMED: 12958120] - PMC - PubMed
Higgins 2011
    1. Higgins JPT, Green S, editor(s). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 [updated March 2011]. Available from www.cochrane‐handbook.org.
Hollis 1999
    1. Hollis S, Campbell F. What is meant by intention to treat analysis? Survey of published randomised controlled trials. British Medical Journal 1999;319(7211):670‐4. [PUBMED: 10480822] - PMC - PubMed
Horn 1987
    1. Horn A, Freed N, Pecora AA. Sickle cell hepatopathy: diagnosis and treatment with exchange transfusion. Journal of the American Osteopathic Association 1987;87(2):130‐3. [PUBMED: 3558021] - PubMed
ICH‐GCP 1997
    1. International Conference on Harmonisation Expert Working Group. International conference on harmonisation of technical requirements for registration of pharmaceuticals for human use. ICH harmonised tripartite guideline. Guideline for good clinical practice1997 CFR & ICH Guidelines. Vol. 1, PA 19063‐2043, USA: Barnett International/PAREXEL, 1997.
Im 2015
    1. Im DD, Essien U, DePasse JW, Chiappa V. Acute on chronic liver failure in a patient with sickle cell anaemia (HbSS). BMJ Case Reports 2015; Vol. 2015:Jul 1. [PUBMED: 26135492] - PMC - PubMed
Ioannidis 2004
    1. Ioannidis JP, Evans SJ, Gotzsche PC, O'Neill RT, Altman DG, Schulz K, et al. Better reporting of harms in randomized trials: an extension of the CONSORT statement. Annals of Internal Medicine 2004;141(10):781‐8. [PUBMED: 15545678] - PubMed
Ioannidis 2010
    1. Ioannidis JP. The art of getting it wrong. Research Synthesis Methods 2010;1(3‐4):169‐84. - PubMed
Johnson 1985
    1. Johnson CS, Omata M, Tong MJ, Simmons JF Jr, Weiner J, Tatter D. Liver involvement in sickle cell disease. Medicine 1985;64(5):349‐56. [PUBMED: 4033412] - PubMed
Kaplan 2012
    1. Kaplan A. Complications of apheresis. Seminars in Dialysis 2012;25(2):152‐8. [PUBMED: 22321209] - PubMed
Karunatilake 2009
    1. Karunatilake H, Vithiya K, Malavan R, Natalia H, Ratnayake H. Exchange transfusion for intrahepatic cholestasis due to sickle beta thalassaemia. Ceylon Medical Journal 2009;54(3):95‐6. [PUBMED: 19999791] - PubMed
Kassim 2013
    1. Kassim AA, DeBaun MR. Sickle cell disease, vasculopathy, and therapeutics. Annual Review of Medicine 2013;64:451‐66. [PUBMED: 23190149] - PubMed
Kato 2006
    1. Kato GJ, McGowan V, Machado RF, Little JA, Taylor J 6th, Morris CR, et al. Lactate dehydrogenase as a biomarker of hemolysis‐associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood 2006;107(6):2279‐85. [PUBMED: 16291595] - PMC - PubMed
Kato 2007
    1. Kato GJ, Gladwin MT, Steinberg MH. Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Reviews 2007;21:37‐47. [PUBMED: 17084951] - PMC - PubMed
Kato 2008
    1. Kato GJ, Gladwin MT. Evolution of novel small‐molecule therapeutics targeting sickle cell vasculopathy. JAMA 2008;300(22):2638‐46. [PUBMED: 19066384] - PMC - PubMed
Kato 2009
    1. Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions. American Journal of Hematology 2009; Vol. 84, issue 9:618‐25. [PUBMED: 19610078] - PMC - PubMed
Kato 2014
    1. Kato GJ. No NO means yes to sickle red cell adhesion. Blood 2014;123(12):1780‐2. [PUBMED: 24652963] - PubMed
Khan 2011
    1. Khan MA, Kerner JA. Reversal of hepatic and renal failure from sickle cell intrahepatic cholestasis. Digestive Diseases and Sciences 2011;56(6):1634‐6. [PUBMED: 21267779] - PubMed
Khurshid 2002
    1. Khurshid I, Anderson L, Downie GH, Pape GS. Sickle cell disease, extreme hyperbilirubinemia, and pericardial tamponade: case report and review of the literature. Critical Care Medicine 2002;30(10):2363‐7. [PUBMED: 12394969] - PubMed
Kleinman 1984
    1. Kleinman SH, Hurvitz CG, Goldfinger D. Use of erythrocytapheresis in the treatment of patients with sickle cell anemia. Journal of Clinical Apheresis 1984;2(2):170‐6. [PUBMED: 6543585] - PubMed
Lee 2012
    1. Lee G, Arepally GM. Anticoagulation techniques in apheresis: from heparin to citrate and beyond. Journal of Clinical Apheresis 2012;27(3):117‐25. [PUBMED: 22532037] - PMC - PubMed
Lockwood 2014
    1. Lockwood SY, Erkal JL, Spence DM. Endothelium‐derived nitric oxide production is increased by ATP released from red blood cells incubated with hydroxyurea. Nitric Oxide 2014;38:1‐7. [PUBMED: 24530476] - PubMed
Lottenberg 2005
    1. Lottenberg R, Hassell KL. An evidence‐based approach to the treatment of adults with sickle cell disease. Hematology / The Education Program of the American Society of Hematology. American Society of Hematology. Education Program 2005:58‐65. [PUBMED: 16304360] - PubMed
Macleod 2014
    1. Macleod MR, Michie S, Roberts I, Dirnagl U, Chalmers I, Ioannidis JP, et al. Biomedical research: increasing value, reducing waste. Lancet 2014;383(9912):101‐4. [PUBMED: 24411643] - PubMed
Malik 2015
    1. Malik A, Merchant C, Rao M, Fiore RP. Rare but lethal hepatopathy‐sickle cell intrahepatic cholestasis and management strategies. The American Journal of Case Reports 2015;16:840‐3. [PUBMED: 26613743] - PMC - PubMed
Modell 2008
    1. Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bulletin of the World Health Organization 2008;86:480‐7. [PUBMED: 18568278] - PMC - PubMed
Moher 2010
    1. Moher D, Hopewell S, Schulz KF, Montori V, Gotzsche PC, Devereaux PJ, et al. CONSORT 2010 Explanation and Elaboration: Updated guidelines for reporting parallel group randomised trials. Journal of Clinical Epidemiology 2010;63(8):e1‐37. [PUBMED: 20346624] - PubMed
Morris 2008
    1. Morris CR. Mechanisms of vasculopathy in sickle cell disease and thalassemia. Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program 2008, issue 1:177‐85. [PUBMED: 19074078] - PubMed
Morris 2014
    1. Morris CR. Alterations of the arginine metabolome in sickle cell disease: a growing rationale for arginine therapy. Hematology/Oncology Clinics of North America 2014;28(2):301‐21. [PUBMED: 24589268] - PubMed
Muriel 2008
    1. Muriel P, Rivera‐Espinoza Y. Beneficial drugs for liver diseases. Journal of Applied Toxicology 2008;28(2):93‐103. [PUBMED: 17966118] - PubMed
NACB 2000
    1. Dufour DR (editor). The National Academy of Clinical Biochemistry. Laboratory guidelines for screening, diagnosing and monitoring for hepatic injury. Laboratory Medicine Practice Guidelines 2000; Vol. 12:1‐61.
NIH 2014
    1. Expert panel report. Evidence‐based management of sickle cell disease. www.nhlbi.nih.gov/health‐pro/guidelines/sickle‐cell‐disease‐guidelines/ (Access: 20 November 2014) 2014.
Nishank 2013
    1. Nishank SS, Singh MP, Yadav R, Gupta RB, Gadge VS, Gwal A. Endothelial nitric oxide synthase gene polymorphism is associated with sickle cell disease patients in India. Journal of Human Genetics 2013;58(12):775‐9. [PUBMED: 24088668] - PubMed
O'Callaghan 1995
    1. O'Callaghan A, O'Brien SG, Ninkovic M, Butcher GP, Foster CS, Walters JR, et al. Chronic intrahepatic cholestasis in sickle cell disease requiring exchange transfusion. Gut 1995;37(1):144‐7. [PUBMED: 7672666] - PMC - PubMed
Omata 1986
    1. Omata M, Johnson CS, Tong M, Tatter D. Pathological spectrum of liver diseases in sickle cell disease. Digestive Diseases and Sciences 1986;31(3):247‐56. [PUBMED: 3948629] - PubMed
Patel 2014
    1. Patel DK, Mohapatra MK, Thomas AG, Patel S, Purohit P. Procalcitonin as a biomarker of bacterial infection in sickle cell vaso‐occlusive crisis. Mediterranean Journal of Hematology and Infectious Diseases 2014;6(1):e2014018. [PUBMED: 24678395] - PMC - PubMed
PCORI 2012
    1. Patient‐Centered Outcomes Research Institute (PCORI). Preliminary draft methodology report: “Our questions, our decisions: Standards for patient‐centered outcomes research”. http://www.pcori.org/assets/Preliminary‐Draft‐Methodology‐Report.pdf (accessed 07 July 2014):1‐61.
Piel 2017
    1. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. The New England Journal of Medicine 2017;376(16):1561‐73. [PUBMED: 28423290] - PubMed
RevMan 2014 [Computer program]
    1. Version 5.3. Copenhagen: The Nordic Cochrane Centre: The Cochrane Collaboration. Review Manager (RevMan). Version 5.3. Copenhagen: The Nordic Cochrane Centre: The Cochrane Collaboration, 2014.
Sarode 2006
    1. Sarode R, Altuntas F. Blood bank issues associated with red cell exchanges in sickle cell disease. Journal of Clinical Apheresis 2006;21(4):271‐3. [PUBMED: 17177280] - PubMed
Savafi 2006
    1. Savafi NA, Akbulut S, Koseoglu T, Albayrak L. Chronic liver disease in a patient with sickle cell anemia. Turkish Journal of Gastroenterology 2006;17(2):123‐5. [PUBMED: 16830296] - PubMed
Sheehy 1980
    1. Sheehy TW, Law DE, Wade BH. Exchange transfusion for sickle cell intrahepatic cholestasis. Archives of Internal Medicine 1980;140(10):1364‐6. [PUBMED: 7425771] - PubMed
Simon 2016
    1. Simon E, Long B, Koyfman A. Emergency medicine management of sickle cell disease complications: an evidence‐based update. The Journal of Emergency Medicine 2016;51(4):370‐81. [PUBMED: 27553919] - PubMed
Steinberg 2009
    1. Steinberg MH. Genetic etiologies for phenotypic diversity in sickle cell anemia. Scientific World Journal 2009;9:46‐67. [PUBMED: 19151898] - PMC - PubMed
Sterne 2011
    1. Sterne JA, Sutton AJ, Ioannidis JP, Terrin N, Jones DR, Lau J, et al. Recommendations for examining and interpreting funnel plot asymmetry in meta‐analyses of randomised controlled trials. British Medical Journal 2011;343:d4002. [PUBMED: 21784880] - PubMed
Sun 2013
    1. Sun K, Xia Y. New insights into sickle cell disease: a disease of hypoxia. Current Opinion in Hematology 2013;20(3):215‐21. [PUBMED: 23549375] - PubMed
Swerdlow 2006
    1. Swerdlow PS. Red cell exchange in sickle cell disease. Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program 2006:48‐53. [PUBMED: 17124039] - PubMed
Thakur 2014
    1. Thakur TJ, Guindo A, Cullifer LR, Li Y, Imumorin IG, Diallo DA, et al. Endothelin‐1 but not endothelial nitric oxide synthase gene polymorphism is associated with sickle cell disease in Africa. Gene Regulation and Systems Biology 2014;8:119‐26. [PUBMED: 24932102] - PMC - PubMed
Thorlund 2009
    1. Thorlund K, Devereaux PJ, Wetterslev J, Guyatt G, Ioannidis JP, Thabane L, et al. Can trial sequential monitoring boundaries reduce spurious inferences from meta‐analyses?. International Journal of Epidemiology 2009;38(1):276‐86. [PUBMED: 18824467] - PubMed
Thorlund 2010
    1. Thorlund K, Anema A, Mills E. Interpreting meta‐analysis according to the adequacy of sample size. An example using isoniazid chemoprophylaxis for tuberculosis in purified protein derivative negative HIV‐infected individuals. Clinical Epidemiology 2010;2:57‐66. [PUBMED: 20865104] - PMC - PubMed
Thorlund 2011
    1. Thorlund K, Engstrøm J, Wetterslev J, Brok J, Imberger G, Gluud C. User Manual for Trial Sequential Analysis (TSA). http://ctu.dk/tsa/files/tsa_manual.pdf (accessed 30 April 2012).
Tiftik 2004
    1. Tiftik N, Altintas E, Kiykim A, Ucbilek E, Sezgin O. Long‐term red blood cell exchange can be used to successfully treat sickle cell intrahepatic cholestasis: a case report. Journal of Clinical Apheresis 2004;19(1):17‐9. [PUBMED: 15095397] - PubMed
Tluway 2017
    1. Tluway F, Makani J. Sickle cell disease in Africa: an overview of the integrated approach to health, research, education and advocacy in Tanzania, 2004‐2016. British Journal of Haematology 2017;177(6):919‐29. [PUBMED: 28295224] - PMC - PubMed
Turner 2012
    1. Turner L, Shamseer L, Altman DG, Weeks L, Peters J, Kober T, et al. Consolidated standards of reporting trials (CONSORT) and the completeness of reporting of randomised controlled trials (RCTs) published in medical journals. Cochrane Database of Systematic Reviews 2012, Issue 11. [DOI: 10.1002/14651858.MR000030.pub2] - DOI - PMC - PubMed
Ware 2017
    1. Ware RE, Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet 2017; Vol. Epub ahead of print. [PUBMED: 28159390] - PubMed
Weatherall 2006
    1. Weatherall D, Akinyanju O, Fucharoen S, Olivieri N, Musgrove P. Inherited Disorders of Hemoglobin. Disease Control Priorities in Developing Countries. 2nd Edition. Washington DC: The World Bank and Oxford University Press, 2006.
Wetterslev 2008
    1. Wetterslev J, Thorlund K, Brok J, Gluud C. Trial sequential analysis may establish when firm evidence is reached in cumulative meta‐analysis. Journal of Clinical Epidemiology 2008;61(1):64‐75. [PUBMED: 18083463] - PubMed
Wetterslev 2009
    1. Wetterslev J, Thorlund K, Brok J, Gluud C. Estimating required information size by quantifying diversity in random‐effects model meta‐analyses. BMC Medical Research Methodology 2009;9:86. [PUBMED: 20042080] - PMC - PubMed
Wetterslev 2017
    1. Wetterslev J, Jakobsen JC, Gluud C. Trial Sequential Analysis in systematic reviews with meta‐analysis. BMC Medical Research Methodology 2017;17(1):39. [PUBMED: 28264661] - PMC - PubMed
WHO 2006
    1. World Health Organization. 59th World Health Assembly resolution WHA59.20 on sickle cell anaemia. http://apps.who.int/gb/ebwha/pdf_files/WHA59‐REC1/e/Resolutions‐en.pdf (accessed December 14, 2012):1‐40.
Yawn 2014
    1. Yawn BP, Buchanan GR, Afenyi‐Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, et al. Management of sickle cell disease: summary of the 2014 evidence‐based report by expert panel members.. JAMA 2014;312(10):1033‐48. [PUBMED: 25203083] - PubMed
Zhang 2012
    1. Zhang Y, Xia Y. Adenosine signaling in normal and sickle erythrocytes and beyond. Microbes and Infection 2012;14(10):863‐73. [PUBMED: 22634345] - PMC - PubMed

References to other published versions of this review

Martí‐Carvajal 2015
    1. Martí‐Carvajal AJ, Simancas‐Racines D. Interventions for treating intrahepatic cholestasis in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. [DOI: 10.1002/14651858.CD010985.pub2] - DOI - PubMed

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