Abstract

INTRODUCTION: Acute liver failure (ALF) is a rare but serious condition defined by severe acute liver injury with coagulopathy (INR ≥ 1.5) and encephalopathy without preexisting cirrhosis. ALF carries a high morbidity and mortality. Liver transplantation (LT) is the only definitive treatment for patients that fail other supportive therapies. CASE DESCRIPTION/METHODS: A 28 year-old female with hyperthyroidism due to Grave disease was transferred to our institution for acute liver failure (ALF) and transplant evaluation. One month prior, she developed jaundice and pruritus. She had been treated with propylthiouracil (PTU) for 1 year but that was stopped 1 week prior to transfer due to elevated liver enzymes. On evaluation she was found to have elevated AST/ALT (2800/1900), hyperbilirubinemia (28.6) and an elevated INR (2.85). The patient progressively worsened and developed encephalopathy thus fulfilling the criteria for ALF. On admission, her MELD-Na was 33 and she had grade III hepatic encephalopathy. Her thyroid studies were found to be TSH <0.010, free T3 17.9 and free T4 >5.6. Her Burch-Wartofsky Point Scale was >45, which is highly suggestive of thyroid storm. She was subsequently started on plasmapheresis, methimazole, hydrocortisone and cholestyramine for thyroid dysfunction. Patient continued to clinically decompensate requiring an emergent thyroidectomy followed by LT. Post-operatively patient had an acute change in her neurological status with loss of reflexes. CT head showed diffuse global cerebral edema with brainstem compression and herniation. Medical care was withdrawn and patient expired. DISCUSSION: This is a case of ALF from PTU drug induced liver injury complicated by thyroid storm. PTU is the second most common non-acetaminophen related cause of DILI that requires liver transplantation. PTU-related ALF can occur at any point of treatment and the onset is usually sudden with rapid progression. While extremely rare, thyroid storm is also a reported cause of ALF. Excess thyroid hormone may cause hepatocyte dysfunction. This case poses a diagnostic dilemma given the coexistence of thyroid storm and PTU therapy. It is possible that the initial hepatic injury due to PTU-related DILI made the liver more susceptible to fulminant hepatic failure from thyroid storm. Despite the rapid listing and transplantation within 48 hours of arrival, the patient did not survive. This case extends the spectrum of the presentation of ALF in the setting of thyroid dysfunction.

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