July 17, 2003
Other Illnesses
Question from the Netherlands:
Our seven year old daughter who has had congenital adrenal hyperplasia (CAH) since birth and type 1 diabetes for about two years, is on hydrocortisone three times per day and a mixture of Insultard (NPH), Actrapid (Regular), and NovoRapid insulin. Last week, she had her first night-time hypo, was unconscious, and it took hours before she was “back”. This was really horrifying, but we knew it would to happen at some point. At least we have experienced it now and will be even more alert on checking her sugar at bedtime.
The combination of type 1 diabetes and CAH seems to be uncommon, and compared to diabetes, treating CAH is far less complex. If medication is taken and regular checks are conducted by the endocrinologist, it is manageable. Even though we are happy with the treatment our diabetes team, I would like to know whether you are familiar with patients who are affected by both diabetes and CAH. Is there any research data/material retrievable that shows whether one disease affects the other negatively or whether the combination of the two can lead to more/other severe conditions? According to the doctors here, there is nothing available, and I would appreciate a second opinion.
Answer:
The concurrence of congenital adrenal hyperplasia in your daughter with a form of diabetes that I assume to be type 1A (autoimmune) is, I believe, quite fortuitous. There are theoretical links between the two states nonetheless.
There are reports, for instance, that in late CAH insulin resistance, with or without obesity, leading to a type 2 form of diabetes, which is not insulin dependent. Another form of the deficiency of the enzyme 21-hydroxylase may occur in association with diabetes in the Autoimmune Polyglandular Syndrome Type II, but this leads to adrenal deficiency with none of the masculinising features of congenital hyperplasia.
DOB
