Mauriac’s Syndrome Caused By Munchausen Syndrome-Associated Insulin Abuse in Type 1 Diabetes Mellitus

Presentation Number: MON 607
Date of Presentation: April 3rd, 2017

Suleyman Nahit Sendur1, Selcuk Dagdelen*1, Ugur Unluturk2, Tomris Erbas1 and Miyase Bayraktar3
1Hacettepe University Medical School, Ankara, Turkey, 2Hacettepe University School of Medicine, Ankara, Turkey, 3Hacettepe University, School of Medicine, Ankara, Turkey

Abstract

Introduction: Mauriac’s syndrome is a rare complication of poorly controlled type 1 diabetes mellitus (T1DM) which was firstly was described by Pierre Mauriac, in 1930. It is characterised by varying degrees of dwarfism, hepatomegaly and cushingoid habitus, resulting from hepatic glycogenosis. Here we present an interesting case of Mauriac’s syndrome which was triggered by insulin abuse.

Clinical case: A 19-year-old female, was administered to the hospital with a history of abdominal distension and poor glucose regulation. She was diagnosed to have T1DM at 9 years old. Since then she had been on intensive insulin treatment. During the last two years, she complains abdominal distension which was gradually increasing and abdominal discomfort on right upper quadrant. Her height was 148 cm (<3%) and her weight was 46 kg (3-10%). On physical examination, a large non-tender liver was palpated below subcostal margin. Secondary sexual characters were arrested at Tanner stage 3. There were signs of Cushing’s syndrome. Transamimases were elevated, and her HbA1c level was 9.9%. Abdominal USG showed hepatomegaly ( long axis: 21 cm), hepatosteatosis and a hemangioma. Liver biopsy was performed and revealed megamitochondrias with extensive macrovesicular steatosis. Intranuclear glycogen inclusions in hepatocytes were seen. Despite strict dietary precautions and intensive insulin regimen, blood glucose control was not achieved and frequent hypoglycemia attacks were noted. At one ward visit, incidentally, an insulin injector was found on patient’s bed. The patient was consulted to psychiatry. Munchausen syndrome was diagnosed and the patient was transferred to the psychiatric ward. Detailed psychiatric questioning revealed past history of sexual abuse by step grand-father and domestic violence. Glucose control was succeeded at psychiatric support. Following the good glycemic control, liver size and liver functions were improved.

Discussion: Here we describe a type 1 diabetic patient with Munchausen syndrome, so self-manipulating insülin regimens and then complicating with Mauriac’s syndrome. Inapproprite insulinisations like here in our case, might cause excessive hepatic glycogen storage and Mauriac’s Syndrome which is also known as pseudo-Von Gierke disease. This rare clinical entity should be considered in differential dignosis of delayed puberty and pseudo-Cushing’s syndrome as well.

 

Nothing to Disclose: SNS, SD, UU, TE, MB