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October 18, 2002

DIDMOAD

Question from Scotland:

My 22 month old daughter was rushed into hospital last week and quickly diagnosed with diabetes mellitus, but about two days later, her kidneys were not working well and soon they also diagnosed diabetes insipidus which is causing the doctors real concern here, but they will not speculate. The doctors are really concerned, she is getting bloods taken every six to right hours with blood sugars every hour, and she has been on two to three drips. The poor little lass is so sick of it all now. Her blood sugar levels are rising up and down like a yo-yo (3.9-24 mmol/L [70-432 mg/dl], and it seems she cannot stabilized for long. She has to go for an MRI, and we have told she may have to have other tests. Any information help and advice would be greatly received, and I would like anyone on the list who has a child with both these types of diabetes to contact me. [email protected]

Answer:

The association between diabetes mellitus and diabetes insipidus is recognised, but very uncommon indeed. I imagine the doctors are considering either Wolfram Syndrome — also called DIDMOAD (central diabetes insipidus, diabetes mellitus, optic atrophy and deafness) — or histiocytosis X. The former is a recessively inherited mitochondrial disorder which leads to a destabilisation of energy in selected vulnerable cells such as the insulin-secreting cells in the pancreas.

In addition to the conventional treatment of the various components of this syndrome, there have been reports of benefit from the use of Thiamine (vitamin B1). In these cases, the deafness and the visual problems are very variable, but they do need to be looked for at intervals.

This is a complicated problem, and you are going to have to talk it over very thoroughly with the Paediatric Consultant to help understand it. In addition, it might be a good idea, if you are planning to have more children, to also talk this over with a geneticist.

I am sorry if I seem to deal with this complex issue so briefly, but I think it would be much better to go over the details with your Scottish team.

DOB

[Editor’s comment: See Worldwide Society of Wolfram Syndrome Families.

SS]