May 7, 2000
Question from Fort Wayne, Indiana, USA:
My 14-month-old son was diagnosed with Type 1 diabetes four months ago. He has now developed a large belly. An ultrasound test was performed and the doctor believes it is caused by an enlarged liver. Blood tests indicate the liver is otherwise healthy. Is this a common side effect of diabetes?
As I read this account, a number of possibilities came to mind. My first was to wonder whether indeed the liver was enlarged. Unless the subject is obese or very resistant to examination it is nearly always possible to feel the lower edge of an enlarged liver on breathing in or to percuss it. That it took an ultrasound to produce what sounds like a rather hesitant diagnosis of hepatomegaly makes me wonder if the abdominal distention doesn’t have some other cause. If indeed the liver is enlarged then there are a number of possibilities, all very uncommon, some related to the diabetes, and some distinct occurrences.The latter group would have been accompanied by a large liver before the onset of the diabetes.
To begin with, I would ask the doctor if he/she did an antibody test at the time of diagnosis of the diabetes. If this was positive and I would expect it to have been, then the first step might be to look for other autoimmune explanations for the distended abdomen. The first one to consider in a case of Autoimmune Polyglandular Syndrome Type II would be the Celiac Syndrome (about 10%) and this can be detected by looking for serum anti glutamyl transferase antibodies. The fact that liver function tests were apparently normal favours this possibility.
Liver enlargement is certainly recognised in diabetic children who have been badly controlled or deprived of insulin. This used to be called Mauriac’s syndrome; but it doesn’t sound at all applicable here.
Then there are a number of inherited metabolic disorders that cause a large liver; but are not associated with diabetes; but which could co-exist, conditions like the glycogen storage diseases. If there is no evidence of the Celiac Syndrome and if the liver turns out to be convincingly enlarged and control of the diabetes has been good then it might be appropriate to consider referral to a regional children’s metabolic unit for more elaborate enzyme tests and perhaps a liver biopsy.