Fatal type B lactic acidosis in a patient with end-stage liver disease related to homozygous sickle cell disease
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Hepatic involvement by sickle cell disease (SCD) can result in a variety of symptoms ranging from mild to life-threatening. Acute intrahepatic cholestasis is a rare but often fatal condition, with multi-organ failure as a terminal event. The following observation is suggesting that extreme hyperbilirubinemia may be associated with energetic failure.
A 16-year-old girl was admitted to the intensive care unit (ICU) for seizures. She had a complicated medical past history in relation to homozygous SCD. At the age of 3 months, she received a liver transplantation for a fulminant hepatic failure due to neonatal hemochromatosis. At the age of 15, she developed a chronic renal failure with a corticoresistant nephrotic syndrome due to a membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis connected with sickle cell nephropathy. A few months later, she developed a progressive liver disease with jaundice. Liver biopsy could rule out rejection and was in favor of sickle...
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An informed consent was obtained from the relatives.
Conflict of interest
The authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.