Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography

Papafragkakis, Haris; Ona, Mel A.; Changela, Kinesh; Sadanandan, Swayamprabha; Jelin, Abraham; Anand, Sury; Duddempudi, Sushil

doi:10.1177/1756283x14530781

Cited by 8 publications

(7 citation statements)

References 36 publications

(44 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…They were young adults. The literature review from 2001 to 2020 has shown that the incidence is higher in the pediatric population, but less aggressive if we compare with the adult one [7]. Congolese patients presented an aggressive course.…”

Section: Discussionmentioning

confidence: 95%

Severe Variant of Sickle Cell Intrahepatic Cholestasis a Difficult Approach Diagnosis Clinical Presentation and Outcome in Adult Population in the Congo

Ngolet¹,

Nkounkou²,

Ibara³

et al. 2020

OJBD

View full text Add to dashboard Cite

Background: There are no consensual diagnosis criteria to make diagnosis of sickle cell intrahepatic cholestasis and identify its 2 variants. Here we delineate the frequency of the complication in its severe form and describe its clinical features. Methods: We included in the study all the patients with sickle cell disease who were presenting fever, jaundice, pain in the upper right quadrant and filling up the biological criteria of the severe sickle cell intrahepatic cholestasis (serum direct bilirubin ≥ 78.5 mg/dL). Patients with evidence of viral hepatitis or gallstones were not included in the study. Results: Sixty-two patients with an average age of 21.5 years (range 18 and 25 years) meet the inclusion criteria, minimum serum direct bilirubin: 82 versus 528 for the maximum. The liver cytolysis was major and ranged from 310 to 1550 UI/L. Forty-nine patients (83.9%) did not develop any organ failure. The symptoms and biological abnormalities resolved with supportive treatment within 7 days. The global lethality was 16.1% associated with liver failure. Conclusion: Severe sickle cell intrahepatic cholestasis is more frequent than we thought and was mainly represented by borderline clinical feature. Early intervention may reduce the progression to organ failure stage and ultimate death.

show abstract

Section: Discussionmentioning

confidence: 95%

Severe Variant of Sickle Cell Intrahepatic Cholestasis a Difficult Approach Diagnosis Clinical Presentation and Outcome in Adult Population in the Congo

Ngolet¹,

Nkounkou²,

Ibara³

et al. 2020

OJBD

View full text Add to dashboard Cite

show abstract

“…While the incidence of SCIC is higher in children than in adults, adults tend to experience a more aggressive disease course with mortality rate as high as 50%. [5] Diagnosis of SCIC calls for primary biliary disease to be ruled out, which is most commonly done using ultrasound which has a positive predictive value of 95% in diagnosing acute cholecystitis. [7] Since our patient had a history of cholecystectomy, cholecystitis was not in the differential diagnosis, and there was no evidence of biliary obstruction on either abdominal ultrasound or CT scan.…”

Section: Discussionmentioning

confidence: 99%

Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

Likhtshteyn

Iqbal

McFarlane

et al. 2020

AJMCR

View full text Add to dashboard Cite

With the advent of hydroxyurea, the sickle cell population has been enjoying a prolonged life span as compared to the pre-hydroxyurea era. Traditionally, acute complications of sickle cell disease includes acute chest syndrome, MI and stroke. In this report we present a case of an elderly man with sickle cell disease who presented with intrahepatic cholestasis (SCIC); a rather rare and fatal complication of sickle cell hemoglobinopathy. The patient presented with jaundice and elevated bilirubin up to 53, his hospital course was complicated by coagulopathy and encephalopathy, and expired on day 43 of presentation after failing multiple therapeutic interventions including exchange transfusion. In this report, we will provide literature review and discuss the underlying pathophysiologic mechanisms of intrahepatic cholestasis in the sickle cell population highlighting the need for immediate recognition and institution of therapy for this fatal complication of sickle cell disease, particularly in elderly populations with low metabolic reserve.

show abstract

“…SCIC has a poor prognosis and carries a high mortality (up to 40%) that has been altered by early use of exchange transfusions. 5 This was attempted in our patient after her liver transplant to decrease graft injury associated with sickle cell anemia. In sickle cell patients in whom this fails, liver transplantation is the only option.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Liver Transplantation in Sickle Cell Anemia

Alder¹,

Vasquez

Reichman

et al. 2016

Clin Pediatr (Phila)

View full text Add to dashboard Cite

Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography

Cited by 8 publications

References 36 publications

Severe Variant of Sickle Cell Intrahepatic Cholestasis a Difficult Approach Diagnosis Clinical Presentation and Outcome in Adult Population in the Congo

Severe Variant of Sickle Cell Intrahepatic Cholestasis a Difficult Approach Diagnosis Clinical Presentation and Outcome in Adult Population in the Congo

Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

Pediatric Liver Transplantation in Sickle Cell Anemia

Contact Info

Product

Resources

About