Case Report Glycogenic Hepatopathy in Type 1 Diabetes Mellitus Murat Atmaca, 1 Rifki Ucler, 1 Mehmet Kartal, 2 Ismet Seven, 2 Murat Alay, 1 Irfan Bayram, 3 and Sehmus Olmez 4 1 Department of Endocrinology and Metabolism, Yuzuncu Yil University Faculty of Medicine, 65100 Van, Turkey 2 Department of Internal Medicine, Yuzuncu Yil University Faculty of Medicine, 65100 Van, Turkey 3 Department of Pathology, Yuzuncu Yil University Faculty of Medicine, 65100 Van, Turkey 4 Department of Gastroenterology, Yuzuncu Yil University Faculty of Medicine, 65100 Van, Turkey Correspondence should be addressed to Murat Atmaca; drmuratatmaca@hotmail.com Received 7 April 2015; Accepted 28 July 2015 Academic Editor: Mario Pirisi Copyright © 2015 Murat Atmaca et al. is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Glycogenic hepatopathy is a rare cause of high transaminase levels in type 1 diabetes mellitus. is condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible and excessive accumulation of glycogen in hepatocytes. is is a case report on a 19-year-old male case, diagnosed with glycogenic hepatopathy. Aſter the diagnosis was documented by liver biopsy, the case was put on glycemic control which led to significant decline in hepatomegaly and liver enzymes. It was emphasized that, in type 1 diabetes mellitus cases, hepatopathy should also be considered in the differential diagnoses of elevated liver enzyme and hepatomegaly. 1. Introduction Liver enzyme elevation is more common among diabetic patients compared to the general population. is condition is oſten associated with nonalcoholic hepatosteatosis [1, 2]. Another very rare cause of elevated liver enzymes, especially among type 1 diabetic patients, is glycogenic hepatopathy (GH). GH develops due to excessive and irreversible accu- mulation of glycogen in the hepatocytes and causes liver function disorders and hepatomegaly [3, 4]. Mauriac first defined GH in a child with brittle diabetes, as a component of Mauriac syndrome, characterized by delayed development, hepatomegaly, cushingoid appearance, and delayed puberty [5]. Additionally, GH can also be observed in adult type 1 dia- betic individuals without other components of Mauriac syn- drome [6–8]. Hyperglycemia and overinsulinization (poor glycemic control) are believed to be metabolic preconditions in GH. GH therapy is performed via establishing glycemic control. Tight glycemic control via intensive insulin therapy provides full remission of clinical, laboratory, and histological abnormalities [4]. Here, a 19-year-old case diagnosed with GH is presented with a discussion referenced to the medical literature. 2. Case A 19-year-old male patient was admitted to the emergency department due to loss of appetite and nausea complaints that continued for two days. e medical history of the case showed that he was followed up due to type 1 diabetes for 8 years and for hepatosteatosis for 3 years, had poor blood glucose regulation despite insulin analogue and basal insulin therapy, and was hospitalized and followed up 8– 10 times for diabetic ketoacidosis. He did not have any outstanding condition in family history. His arterial blood pressure was measured as 90/50 mmHg and pulse as 92 beat/min and body temperature was 36.9 ∘ C in his physical examination. At his abdominal examination, liver was pal- pable 4 cm below the costal margin, and no splenomegaly or acid was determined. e cardiovascular and respiratory system examination results were normal. e laboratory results of the case obtained in the emergency department were as follows: glucose 350 mg/dL, aspartate aminotrans- ferase (AST) 603 IU/mL, alanine aminotransferase (ALT) 570 IU/mL, alkaline phosphatase (ALP) 921 U/L, and gamma glutamyl transferase (GGT) 379 U/L. Ketone was positive in his full urinalysis but there was no evidence of acidosis in his Hindawi Publishing Corporation Case Reports in Hepatology Volume 2015, Article ID 236143, 4 pages http://dx.doi.org/10.1155/2015/236143