Acute Hepatic Crisis in Sickle Cell Anemia: Favorable Outcome After Exchange Transfusion

Khalifeh, Hassan K.; Chamoun, Cynthia T.; Elhoujairy, Abir H.; Alkoussa, Wael A.; Lahoud, Chantal I. Zeidan; Masri, Gihan Al

doi:10.14740/jh304w

Cited by 6 publications

(5 citation statements)

References 11 publications

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“…1,6 Systematic evaluation and timely management with exchange transfusion/simple blood transfusion, supportive care and tackling the cause will reduce the progressing liver cell injury and mortality. 7,8 It is also equally important to rule out other causes of chronic liver disease like Wilson, autoimmune hepatitis, systemic sepsis etc. 9…”

Section: Discussionmentioning

confidence: 99%

Hepatopathy in sickle cell disease with osteomyelitis: a challenging diagnosis: case report

Sushmitha

Nazir

Sreekantham

et al. 2023

Int J Contemp Pediatr

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‘Sickle hepatopathy’ is a condition which is seen in only 10% cases of sickle cell disease (SCD). Sickle hepatopathy could be due to acute sickle crisis, sequestration, cholangiopathy, severe intrahepatic cholestasis or as a result of chronic blood transfusion related infection and hemosiderosis. Differentiating the cause for hepatopathy in SCD is challenging. Hepatopathy manifestation can range from mild liver function test abnormality to life threatening fulminant hepatic failure. Hence, demanding prompt recognition and management. Here we reported a one such case of 13 year old male with progressing jaundice and bony pain with swelling of lower limbs was diagnosed as SCD with right proximal tibia sickle osteomyelitis and left knee septic arthritis with hepatopathy who was managed with supportive care and surgical intervention.

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Section: Discussionmentioning

confidence: 99%

Hepatopathy in sickle cell disease with osteomyelitis: a challenging diagnosis: case report

Sushmitha

Nazir

Sreekantham

et al. 2023

Int J Contemp Pediatr

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“…The serum alanine and aspartate aminotransferase are seldom >300 IU/L. Aspartate aminotransferase is also elevated by hemolysis, so serum alanine aminotransferase may be a more accurate indicator of liver injury [17].…”

Section: Discussionmentioning

confidence: 99%

Hyperhemolytic Crisis Following Transfusion in Sickle Cell Disease With Acute Hepatic Crisis: A Case Report

Trivedi¹,

Abbas²,

Kazmi³

et al. 2022

Cureus

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Hyperhemolysis syndrome (HHS) is a catastrophic unpredictable consequence of blood transfusion in sickle cell disease. It leads to further drop in hemoglobin via immune mechanisms complicating a hospital course and prolonging length of stay. Although sickle cell patients receive multiple transfusions throughout their disease course, this condition remains underreported by health care professionals or misinterpreted for other sickle cell crises. We present a similar case highlighting the importance of early recognition of HHS and judicious blood transfusion in sickle cell disease patients to avoid such a complication.

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“…In patients with SCIC, therapy is aimed at aggressive supportive measures, including exchange transfusion to maintain a low level of HbS (<30%) [17, 18]. If supportive measures fail, patients are likely to have a worse prognosis, and liver transplantation remains the only viable therapeutic option.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Acute Liver Failure in Sickle Cell Disease

Rodrigues

Almeida

Salgado

et al. 2021

GE Port J Gastroenterol

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Introduction: Sickle cell intrahepatic cholestasis (SCIC) is one of the rarest and the most severe acute hepatic manifestations of sickle cell disease (SCD) and it can rapidly progress to acute liver failure. It is associated with a high mortality rate, demanding prompt recognition and management. Case Presentation: We report a case of a 7-year-old boy with a history of homozygous HbS SCD who presented to the emergency department with fever, increasing abdominal pain, and jaundice. His course was complicated by acute liver injury (AST 9,472 IU/L, ALT 2,683 IU/L, total bilirubin 15.4 mg/dL; conjugated bilirubin 8.69 mg/dL, hypoalbuminemia 2.6 g/dL, and persistent hypoglycemia), with acute liver failure (coagulopathy not corrected by vitamin K administration with INR 3.26, decreased factors V 10% and VII 28%, and West Haven grade I hepatic encephalopathy associated with mild hyperammonemia of 71 µmol/L). After excluding other causes of acute liver failure, the patient was diagnosed as having SCIC and was successfully treated with manual exchange transfusion. Conclusion: This case reinforces that exchange transfusion is an effective treatment for SCIC and that it should be introduced promptly to prevent fulminant and potentially fatal liver failure.

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Acute Hepatic Crisis in Sickle Cell Anemia: Favorable Outcome After Exchange Transfusion

Cited by 6 publications

References 11 publications

Hepatopathy in sickle cell disease with osteomyelitis: a challenging diagnosis: case report

Hepatopathy in sickle cell disease with osteomyelitis: a challenging diagnosis: case report

Hyperhemolytic Crisis Following Transfusion in Sickle Cell Disease With Acute Hepatic Crisis: A Case Report

Pediatric Acute Liver Failure in Sickle Cell Disease

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