Endocrine Abstracts

Glycogen Hepatopathy (GH) was initially described in 1930 by Pierre Mauriac. 90 years later, GH remains underrecognized in adults. The clinical or radiological characterization of GH is difficult, further compounded by lack of widespread literature. We present two cases of GH characterized by recurrent lactatemia and transient liver function and radiological abnormalities.

• 19 years old male with Type 1 diabetes admitted with nausea and vomiting. pH 6.9, glucose 33 mmols/l and lactate 7.7 mmol/l (normal 0.5-2 mmol/l). He was started on intravenous (IV) fixed rate insulin. Lactate initially improved however was noted to be rising 8 h into being started on insulin peaking at 9.2 mmol/l. On admission, bilirubin 10 umol/l (normal range 0-21 umol/l), ALP 80 IU/l (normal range 0-390 IU/l) ALT 162 IU/l (normal 10-50 IU/l) Albumin 44 gm/l (normal 35-50 gm/l). ALT worsened during admission peaking at 790 IU/l corresponding to lactate. Liver screen including hepatitis, HIV, EBV, CMV, alpha 1 antitrypsin, caeruloplasmin, anti-smooth muscle antibodies, anti-liver kidney microsomal antibodies and ANA were negative. Liver ultrasound revealed smooth gross hepatomegaly with increased liver reflectivity. Liver function started resolving at Day 5 and ALT came down to 442 IU/l on Day 8. Repeat liver function 2 months later was normal. MRI liver three months later revealed normal sized liver with no abnormal enhancement with a smooth surface and no fatty infiltration or cirrhosis.

• 21 years old female, first presentation of Type 1 diabetes with diabetic ketoacidosis (DKA). pH 7.2 on admission, glucose 20 mmol/l and lactate 7 mmol/l. Lactate initially improved with fluids however at 24 h peaked at 8.6 mmol/l. Liver ultrasound showed echogenic enlarged liver. Liver function on admission was normal (ALT 31 IU/l) but worsened after starting IV insulin to 578 IU/l and peaked to 752 IU/l at Day 4. Liver function normalized after 3 months. GH was first reported when short acting insulin was introduced. Supraphysiologic rapid acting insulin dosages during DKA management may be a potential cause to drive glycogen storage in rapid hypo- and hyper-glycaemia cycle. A dual peak of lactate may signify a change from Type 1 (impaired perfusion) to Type 2 lactatemia (impaired gluconeogenesis). Further work is needed to characterize patients at risk of GH in re-attendances and potential plans to give lower dose of rapid acting and higher dose of long-acting insulin in acute phase may be explored.