Uncommon Hepatic Sequelae from an Acute Sickle Cell Crisis

Lim, Kimberly; Walter, James S.

doi:10.29046/tmf.014.1.017

Cited by 4 publications

(7 citation statements)

References 7 publications

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“…The level of bilirubin is not a good predictor of outcome [4]. Coagulopathy is common and worsens as hepatocyte necrosis spreads [1].…”

Section: Discussionmentioning

confidence: 99%

“…The cornerstone treatment is rapid Hb S fraction reduction through ET. Additional supportive treatment includes intravenous hydration, analgesia, coagulopathy correction and electrolyte monitoring [1]. In a study, five surviving children's symptoms resolved in few days to 2 weeks.…”

Section: Discussionmentioning

confidence: 99%

“…Although not commonly seen, liver involvement can be life threatening. Sickle cell hepatopathy has been a term used to generally describe varying etiologies of liver dysfunction in sickle cell patients [1].…”

Section: Introductionmentioning

confidence: 99%

“…Hepatic sequestrations can cause hepatic enlargement and rapidly falling hemoglobin. These complications can present similarly to acute sickle crisis and need to be excluded to make a diagnosis [1][2][3]. Herein, we report a case of pediatric sickle cell intrahepatic cholestasis (SCIC) with a favorable outcome after exchange transfusion (ET) of packed red blood cell (PRBC).…”

Section: Introductionmentioning

confidence: 99%

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

Khalifeh

Chamoun²,

Elhoujairy³

et al. 2016

J Hematol

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Pediatric acute liver sickle crisis, also known as sickle cell intrahepatic cholestasis (SCIC), is an uncommon but fatal complication of sickle cell disease observed mainly in patients with homozygous sickle cell anemia. Herein we describe a case of pediatric SCIC treated successfully with manual exchange transfusion (ET). The patient was admitted for jaundice, enlarged liver and signs of hepatic failure, such as hyperbilirubinemia and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. We performed a session of ET in order to reduce the percentage of Hb S to level inferior to 30% which was successfully accomplished. The patient had complete recovery of hepatic function. This case has shown that ET is an effective treatment of pediatric SCIC and should be introduced early on the onset of this severe complication.

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“…The level of bilirubin is not a good predictor of outcome [4]. Coagulopathy is common and worsens as hepatocyte necrosis spreads [1].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Khalifeh

Chamoun²,

Elhoujairy³

et al. 2016

J Hematol

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show abstract

“…Sickle cell disease (SCD) is a qualitative hemoglobinopathy that can cause widespread sickling and vaso-occlusive events in all organ systems [ 1 ]. Sickle cell hepatopathy is a term used for a myriad of acute and chronic pathologies of the liver as a result of sickling in SCD patients.…”

Section: Introductionmentioning

confidence: 99%

Acute Liver Failure From Sickle Cell Hepatopathy Treated With Exchange Transfusion

Kassem¹,

Park²,

Rajkumar³

2021

Cureus

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Sickle cell disease (SCD) is a qualitative hemoglobinopathy that can cause widespread sickling and vaso-occlusive events in all organ systems. Sickle cell hepatopathy is an umbrella term for various acute and chronic pathologies of the liver as a result of sickling in SCD patients. We present below the case of a 49-year-old woman who had an acute liver failure in the setting of a hepatic crisis with recovery after exchange transfusion. Hepatic involvement in SCD may be life-threatening. Understanding the etiology and severity of hepatic involvement by sickling is necessary for appropriate treatment.

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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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Uncommon Hepatic Sequelae from an Acute Sickle Cell Crisis

Cited by 4 publications

References 7 publications

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

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

Acute Liver Failure From Sickle Cell Hepatopathy Treated With Exchange Transfusion

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

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