TY - JOUR
T1 - Intrahepatic cholestasis in sickle cell disease
T2 - A review of diagnostic criteria, treatments, and case reports
AU - Edwards, Christopher L.
AU - Scott, Sharena
AU - Boggan, Michaela
AU - Meek, Jordan
AU - Alston, Kiera
AU - Pearson, Aiden
AU - McDougald, Alexandria
AU - Broadnax, McKenzie
AU - Wood, Mary
AU - Barker, Camela S.
AU - Miller, Jessica
AU - Whitworth, Elaine
AU - James, Osaffo
AU - Sollers III, John J.
AU - Bryson, W. Jeff
AU - Thorpe, Roland
AU - Byrd, Goldie
AU - Whitfield, Keith E.
AU - Sudhakar, Shiv
AU - Parker, Debra O.
AU - Livingston, Jonathan
AU - Shah, Nirmish
AU - Railey, Kenyon
N1 - Publisher Copyright:
© 2022 National Medical Association
PY - 2023/2
Y1 - 2023/2
N2 - Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbid disease. The literature contains only a small number of case reports involving SCIC and hence limited guidance can be obtained. Methods We reviewed the scientific literature to evaluate the science of SCIC to determine if there were consistencies in presentation, evaluation, treatment, and clinical outcomes. Results We reviewed 6 case reports and a limited number of clinical papers on SCIC. We reported consistencies in clinical presentation and treatment outcomes among cases as well as serological and hematological finding. Conclusions While there is some consistency in the symptom presentation of individuals with SCIC, reliable evaluation and clinical procedures were not demonstrated in what we reviewed. Further research is needed to delineate the attributes of this complicated disease that occurs within SCD.
AB - Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbid disease. The literature contains only a small number of case reports involving SCIC and hence limited guidance can be obtained. Methods We reviewed the scientific literature to evaluate the science of SCIC to determine if there were consistencies in presentation, evaluation, treatment, and clinical outcomes. Results We reviewed 6 case reports and a limited number of clinical papers on SCIC. We reported consistencies in clinical presentation and treatment outcomes among cases as well as serological and hematological finding. Conclusions While there is some consistency in the symptom presentation of individuals with SCIC, reliable evaluation and clinical procedures were not demonstrated in what we reviewed. Further research is needed to delineate the attributes of this complicated disease that occurs within SCD.
KW - Intrahepatic cholestasis
KW - Rare
KW - Sickle cell disease
KW - Thalassemia
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U2 - 10.1016/j.jnma.2022.12.004
DO - 10.1016/j.jnma.2022.12.004
M3 - Review article
C2 - 36599744
AN - SCOPUS:85145999962
SN - 0027-9684
VL - 115
SP - 26
EP - 37
JO - Journal of the National Medical Association
JF - Journal of the National Medical Association
IS - 1
ER -