SFEBES2008 Poster Presentations Diabetes, metabolism and cardiovascular (51 abstracts)
A 19-year-old female presented with DKA. She had developed T1DM aged 5. She had an HbA1c of 12.9%, and numerous previous admissions with DKA. There was no other past medical history of note. She took 162 units of insulin/day, as a basal-bolus regime. She took no recreational drugs or other medications. She consumed up to 10 units of alcohol a week. On clinical assessment and investigation, there was no evidence of sepsis, and she was treated with intravenous insulin and fluids. Over the following days a transaminitis was noted. Her ALT peaked on day 4 at 2107 u/l, having been normal on admission. Synthetic function remained normal. A liver screen was normal, and a paracetamol undetectable on admission bloods. A liver ultrasound was normal. Her ALT resolved over several days, and she was discharged. Nine months later she was re-admitted with DKA. ALT again rose after commencement of treatment, and peaked at 1570 u/l on day 3. Also noted was neutropenia at 0.8×109/l. Ultrasound suggested an enlarged liver. The transaminitis and neutropenia resolved over a few days. Two months later she was re-admitted with DKA. She had a 4 cm liver edge, and hepatomegaly. ALT peaked on day 3 to 1027 u/l. Neutrophils were 0.3×109/l. CT abdomen with contrast revealed uniform hepatomegaly. A liver biopsy was performed and revealed preserved liver architecture, but foamy changes were seen within hepatocyte cytoplasm and focal nuclear vacuolations were present. The findings were consistent with hepatic glycogenosis. A bone marrow biopsy was unremarkable, and her intermittent neutropenia remains unexplained. Her hepatomegaly persists, and her current ALT remains raised at 127 u/l.
Hepatic glycogenosis is well described in children with poorly controlled T1DM, but is rarely documented in adults. Administration of supraphysiological doses of insulin in the context of recurrent hyperglycaemia is thought to be the driving factor.