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March 30, 2000

Other Illnesses

Question from Colorado Springs, Colorado, USA:

My daughter has had type 1 diabetes for 7 years now and has been diagnosed with Mauriac syndrome; where do I go for more information?


Mauriac Syndrome is the name which was given to a constellation of findings in children with very poorly controlled diabetes. (A Dr. Mauriac described the syndrome.) Basically these children have poor growth, features of Cushing’s syndrome (excess cortisol), usually have liver abnormalities including an enlarged liver and abnormal liver function tests, and often delayed puberty. These patients were usually treated with only 1 injection a day and very little or no dietary therapy. It is very rare to see a patient with Mauriac Syndrome today now that the importance of stricter blood sugar control with 2 or more shots/day and nutritional counselling is more standard.

If your daughter has been under reasonable control, I would look for other causes of her problems. The treatment is to improve control (though sometimes you have to do this slowly as too rapid an improvement in control in someone severely out of control has been reported to exacerbate eye or kidney problems in teenagers.) If you want to read more about this syndrome, you can go to PubMed and search for Mauriac Syndrome.

Additional comments from Dr. John Schulga:

Mauriac syndrome is a syndrome of diabetes associated with short stature and a glycogen laden liver, and osteopenia in children. It is a very rare syndrome essentially due to inadequate amounts of insulin being given, and used to be more common before the days of long acting insulins.

Additional comments from Dr. Kenneth J. Robertson:

Mauriac syndrome is the term given to the unusual (now) situation of a big liver full of glycogen found in some children with diabetes. Growth is also impaired. The problem is thought to be due to under-insulinisation and resolves slowly if the correct doses are given.