Intrahepatic cholestasis in sickle cell disease: A review of diagnostic criteria, treatments, and case reports

被引：2

作者：

Edwards, Christopher L. ^{[1
,2
]}

Scott, Sharena ^{[1
,2
]}

Boggan, Michaela ^{[1
,2
]}

Meek, Jordan ^{[1
,2
]}

Alston, Kiera ^{[1
,2
]}

Pearson, Aiden ^{[1
,2
]}

Mcdougald, Alexandria ^{[2
]}

Broadnax, Mckenzie ^{[2
]}

Wood, Mary ^{[3
]}

Barker, Camela S. ^{[4
]}

Miller, Jessica ^{[5
]}

Whitworth, Elaine ^{[5
]}

James, Osaffo ^{[1
]}

Sollers III, John J. ^{[1
,2
]}

Bryson, W. Jeff ^{[6
]}

Thorpe, Roland ^{[7
]}

Byrd, Goldie ^{[8
]}

Whitfield, Keith E. ^{[9
]}

Sudhakar, Shiv ^{[10
]}

Parker, Debra O. ^{[1
,2
]}

Livingston, Jonathan ^{[1
,2
]}

Shah, Nirmish ^{[3
]}

Railey, Kenyon ^{[3
]}

机构：

[1] North Carolina Cent Univ, 1801 Fayetteville St, Durham, NC 27707 USA

[2] NCCU Debra O Parker Res Incubator, Durham, NC USA

[3] Duke Univ, Med Ctr, Durham, VA USA

[4] Duke Univ, Duke, NC USA

[5] Bridges Point Sickle Cell Fdn, Durham, NC, Algeria

[6] Fielding Grad Univ, Arvada, CO USA

[7] Johns Hopkins Univ, Baltimore, MD USA

[8] Wake Forest Baptist Med Ctr, Wake Forest, NC USA

[9] Univ Nevada Las Vegas, Las Vegas, NV USA

[10] Calif Northstate Univ, Coll Med, Internal Med & Infect Dis, 9700 W Taron Dr, Elk Grove, CA 95757 USA

来源：

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION | 2023年 / 115卷 / 01期

关键词：

Intrahepatic cholestasis; Sickle cell disease; Thalassemia; Rare; LIVER-TRANSPLANTATION; HEPATOPATHY; MANAGEMENT; PAIN;

D O I：

10.1016/j.jnma.2022.12.004

中图分类号：

R5 [内科学];

学科分类号：

100201 [内科学];

摘要：

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.

引用

页码：26 / 37

页数：12

共 21 条

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