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November 2, 2000


Question from Burbank, California, USA:

My 11 year old little sister has type 1A diabetes, diagnosed two and one-half years ago. We have just been told she has Mauriac Syndrome and currently some kidney and liver damage. We were told this is rare and I can obtain little information. We are starting on even a more stringent diet and now three insulin shots a day rather than two. What is the ultimate prognosis? On the one hand the doctors say she is "dying" and yet they think we can help, perhaps stop the damage. They don't know as they have only experienced three other cases of Mauriac Syndrome (the doctors are specialists).


Mauriac Syndrome is now very rarely seen in the U.S. The condition is the result of chronic poor control of diabetes which leads to an enlarged liver due to excessive glycogen deposition, short stature and delayed puberty. There is usually a history of repeated hospitalisations for diabetic ketoacidosis and hemoglobin A1c tests can be as high as twice the upper level of normal. Kidney function is usually not affected, although it may be an additional complication of poor control. Eating disorders are sometimes an accompaniment.

Treatment can be very successful, but requires the combined efforts of an experienced diabetes team that includes not only a physician, but a dietitian, and, perhaps most importantly, a experienced medical social worker (MSW) or clinical psychologist. Because of the relatively rapid onset of this problem, and because of, what I sense to be, a very caring family, I wonder if other diagnoses should be considered. A relatively common one might be that your sister has been withholding insulin because of anxiety over obesity. This is where the MSW can help so much to disentangle the underlying problem.

Alternative medical explanations might include a disorder of iron metabolism called hemochromatosis, but this could be excluded if the original diagnosis of type 1 diabetes had been shown to be due to autoimmunity by and initial positive antibody test, and if serum ferritin levels were normal. The kidney is sometimes involved. Another condition might be non-alcoholic steatohepatitis (NASH) which has been recorded in children, but is more commonly associated with obesity and a type 2 form of diabetes.A needle biopsy of the liver would show fat rather than glycogen accumulation. Liver transaminase tests would be elevated, and, again, early antibody tests would have been negative.