Coma secondary to cerebral fat embolism syndrome due to sickle cell disease fully recovering following red cell exchange transfusion.

A 51-year-old woman known for sickle cell disease presented with 2 weeks of headache and bilateral lower limb pain. During admission, she suffered from multiple generalised tonic-clonic seizures but had an unremarkable CT of the brain. Incidentally, she had worsening baseline renal function. She was admitted to the intensive care unit with an acute confusional state. A bedside electroencephalogram showed triphasic waves and diffuse slow activity suggestive of encephalopathy with no epileptiform discharges. She remained obtunded despite appropriate medical therapy of hydration, antiepileptic and pain control. Lumbar puncture failed to identify an infectious cause. An urgent MRI of the brain was done and revealed features compatible with fat embolism syndrome (FES). Her haemoglobin S was 84.2%. Urgent red cell exchange transfusion was done, and within 3 days she fully regained her orientation and motor function. This represents the first case of such profound obtundation due to FES with a complete response to exchange transfusion.

A Rare Cause of Chronic Liver Disease Diagnosed by Endoscopic Ultrasound-Guided Liver Biopsy.

BACKGROUND Elevated liver enzymes is a common clinical problem with many possible etiologies, yet some are rare and can be missed. Patients with sickle cell disease (SCD) may be at risk of liver disease due to recurrent blood transfusion predisposing to viral hepatitis. Furthermore, recurrent transfusions can increase the risk of iron overload, which can create deposits in the liver, eventually resulting in chronic liver disease. Liver biopsy is an essential tool to establish a diagnosis of liver disease in many patients with unexplained elevation of liver enzymes. Recently, endosocpic ultrasound (EUS)-guided liver biopsy has been shown to be safe and effective in obtaining adequate liver tissue. However, the safety and efficacy has not been established in patients with SCD. CASE REPORT A 59-year-old man with SCD and beta-thalassemia minor was evaluated for persistently elevated liver enzymes (mainly cholestatic). He had a background history of treated hepatitis C virus infection. He had multiple blood transfusions in the past for sickle cell crisis. A diagnostic work-up revealed negative viral and autoimmiune serology and no evidence of biliary obstruction on abdominal imaging. The iron profile was elevated, consistent with iron overload. An EUS-guided liver biopsy confirmed a diagnosis hepatic hemosiderosis secondary to long-term blood transfusions. CONCLUSIONS This report emphasizes the importance of careful monitoring of iron levels in patients with hematological conditions requiring long-term blood transfusions. In addition, it highlights the emerging role of EUS-guided liver biopsy as a safe and accurate alternative to percutaneous liver biopsy.