Examine a case of presumed hepatic glycogenosis in a poorly controlled Type 1 Diabetes Mellitus, and the discuss the implications and clinical prognosis of glycogenosis.
Methods : This is a case presentation of a single patient with Type 1 Diabetes Mellitus who presented with hepatic glycogenosis. Information was obtained via chart review, with literary search from PubMed.
26 year old lady with history of poorly-controlled type 1 diabetes mellitus presented with complaints of nausea and vomiting. Initial work up was completed, and patient was determined to have DKA from viral gastroenteritis subsequently leading to insulin noncompliance. Patient was given intravenous fluids and started on the hospital insulin protocol. Within 24 hours patient;s DKA resolved and was transitioned to subcutaneous insulin regiment. However, patient had persistent abdominal pain. Liver function tests showed hepatocellular abnormality with the following values, AST 700U/L, ALT 345 U/L, Alkaline phosphatase 185 U/L. Iron panel, ferritin, hepatitis panel, CPK, smooth muscle antibodies and mitochondrial antibodies were all obtained and within normal limits. An abdominal ultrasound revealed a prominent liver with evidence of hepatic steatosis, which was not seen on a prior ultrasound two months prior. Yet with supportive treatment, her liver function tests improved. Patient was discharged home and asked to follow up with her primary care doctor. Two months after her hospitalization a repeat abdominal ultrasound was completed and showed the hepatic steatosis resolved.
Hepatic Glycogenosis, or glycogenic hepatopathy, is a rare finding in the adult population who have poorly controlled type 1 DM. When described it usually presents in the adolescent population with concomitant DKA or with Mauriac syndrome. The pathophysiology arises from insufficient control of hyperglycemia, which stimulates fatty acid synthesis and in turn inhibits glycogenolysis leading to hepatomegaly. Radiographically it appears similar to nonalcholic steatohepatitis. However it is reversible with strict blood glucose control. The prognosis for hepatic glycogenosis is favorable when compared to NASH. Treatment for hepatic glycogenosis is also different when compared to NASH, which solely focuses on adequate glycemic control.
Conclusion : This case presentation of hepatic glycogenosis shows the importance of characterizing this syndrome given its favorable prognosis. The majority of literature describes this process within the adolescent community, however can be present within the adult population. Hence, physicians should be familiar with the pathogenesis of hepatic glycogensis to better treat and educate their patients.
St Marys Hospital
PGY-3 Medical Resident at St Marys Hospital.