Acute hepatic steatosis: an unusual manifestation of poorly controlled type 1 diabetes

Field: Medicine (Health Sciences)

Hepatic steatosis (fatty liver) is associated with insulin resistance and is more frequent in obese subjects and patients with type 2 diabetes, whereas it is rather less common in subjects with type 1 diabetes.1 Most patients with hepatic steatosis are asymptomatic and have no signs of liver disease at the time of diagnosis. By contrast, acute hepatic steatosis may present with acute abdominal pain due to fast enlargement of the liver and stretching of the liver capsule.2 A 16-year-old girl with type 1 diabetes presented to our emergency department in February 2008 with a one-day history of osmotic symptoms preceded by right upper quadrant abdominal pain. The pain was progressive, dull in nature, and not associated with constitutional symptoms or weight loss. She has had diabetes for six years and her control had always been unsatisfactory, with frequent admissions for ketoacidosis. There was no previous history of alcohol intake or chronic liver disease. She was taking no medications except for aspart and glargine insulins. On clinical examination, she was haemodynamically stable and mildly dehydrated. Body mass index was 20kg/m2 with no features of insulin resistance. She was icteric and had no stigmata of chronic liver disease. Abdominal examination was normal except for an enlarged smooth and tender liver with a span of 21cm. Systemic examination was unremarkable. HbA1c was raised at 14% (130mmol/mol) and serial liver functions were as shown in Table 1. Viral and hepatic autoimmune serology were negative. Routine chemistry and haematology were normal after correction of ketoacidosis. Serum amylase was normal. Coeliac disease was confirmed on the basis of positive serology and histology. Serum levels of ferritin, alfa-fetoprotein and ceruloplasmin were normal. Fasting triglycerides level was raised at 3.27mmol/L with a total cholesterol of 6.3mmol/L. Abdominal ultrasound showed an enlarged liver with increased echogenicity suggestive of fatty liver. Abdominal CT also confirmed a fatty liver and ruled out splenomegaly and lymphadenopathy. A liver biopsy was suggestive of hepatic steatosis with swollen hepatocytes and micro- and macrovesicular fat vacuoles (Figure 1). Liver biopsy, H&E stain showing swollen hepatocytes with macro- and microvesicular fat vacuoles in keeping with hepatic micro- and macrosteatosis She made a quick recovery from ketoacidosis, with significant improvement in abdominal pain and liver profile with intensive insulin therapy. Hepatic steatosis is a manifestation of excessive triglycerides accumulation in the liver. The major sources of triglycerides are from fatty acids stored in adipose tissue and fatty acids newly made through de novo lipogenesis.3 The pathogenesis of hepatic steatosis differs between type 1 and type 2 diabetes. In type 2 diabetes, insulin resistance leads to an influx of free fatty acids to the liver both by increased lipolysis and increased de novo lipogenesis in the liver resulting in accumulation of fat within the hepatocytes.4 In type 1 diabetes, however, steatosis occurs in the setting of poor diabetic control and reduced insulin levels when the rate of hepatic triglycerides synthesis exceeds its secretion as VLDL.1 In addition, hyperglycaemia may play a role as glucose activates carbohydrate response element-binding protein (ChREBP) which exerts stimulatory effects on the genes involved in lipogenesis and triglycerides synthesis.5 Coeliac disease is more prevalent in people with type 1 diabetes than in the general population, and is a recognised cause of hepatomegaly and severe hepatic steatosis.6,7 The possibility of coeliac disease should always be ruled out as a cause of fatty liver in these patients. Acute hepatic steatosis is a rather unusual complication of uncontrolled type 1 diabetes that can present as an acute abdomen pain, and can resolve with insulin treatment. This diagnosis should be considered in patients with poorly controlled type 1 diabetes who present with abdominal pain with deranged liver enzymes. References are available at www.practicaldiabetesinternational.com.