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From Portsmouth, Virginia, USA:

I am a mother of a 3 1/2 year old boy who was diagnosed with hyperinsulinism at birth. His blood sugar levels are regulated by diet and diazoxide. Before he was 1 year old, he was diagnosed with cataracts. I was told that he has probably had them since birth. I was also told that his cataracts are not bad enough to do surgery. In addition, he is receiving speech therapy and has been labelled as developmentally delayed (about a year or less behind). I know that cataracts could affect a child's development, but he appears to see well.

Is it possible that the hyperinsulinism could cause him to be developmentally delayed? If so, what would be the neurologic prognosis? Also, what is the physical prognosis? And do children with hyperinsulinism eventually grow out of it?


I have not personally encountered a situation like this; but I think the most likely common denominator for hyperinsulinism, cataracts and developmental delay is hypoglycemia. The association of cataracts with hypoglycemia was described many years ago and of course the relationship of developmental problems to hypoglycemia is well recognised.

There is not much information about the long term outlook with this group of problems. In part this is because of their rarity; but it is also because it is in reality the end point of several distinct conditions, and because in about half the cases pancreatectomy had been the treatment of choice. In general terms it seems that the hypoglycemia does disappear although an abnormality of insulin metabolism remains and there is a risk of diabetes at around puberty. If you have access to a medical library, you might like to look at a paper by Leibowitz et al. in the Journal of Clinical Endocrinology and Metabolism 1995, vol 80, page 386.

The important goal is to contain the hypoglycemia. It sounds as though this has gone well; but you might like to talk to your son's doctor about the use of 'calcium channel blockers,' a familiar class of drugs in the management of hypertension: the reference is Lindley et al. Archives of Disease in Childhood, 1996, 74:369.


Original posting 31 Aug 1998
Posted to Other Illnesses


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