I have a 8 months old daughter, she is diagnosed with hypopituitarism. Her endoconologist is not sure either if she is missing her pituitary gland or if its not working. She is behind in every body movement, is it because of that? Can her doctor check her pituitary gland if its working now, if she should then what are the test she should do to find out? Please help me out here.
Hypopituitarism is evaluated by imaging such as an mri of the pituitary with and without contrast as well as blood tests, some we can get with one test, others require that we give a medication and measure the hormone response before and after. The most important hormone that the pituitary makes is acth, this tells our adrenal gland to get going during times of stress and make very important cortisol. This can be tested to be sure that signal is working with a 1 microgram acth stimulation test, cortisol is measured before and after giving a copy of the hormone that the pituitary makes, if the adrenal gland makes good cortisol with the acth then we know that there has been communication and that this track is working. The next hormone needed to assess is antidiuretic hormone, which also comes from the pituitary, it is the hormone that helps us hold on to water by acting at the kidneys.
If a child has no adh then they will have what is often called water diabetes, meaning they cannot hold onto water and become severely dehydrated and have high sodium levels with risk of death without treatment.
The thyroid gland in the neck is directed also by the pituitary and tsh which the pituitary hormone makes can be measured as well as free T4 to see if the two are talking.
Lastly growth hormone, it is made also from the pituitary and is critical in growth but also for overall good health, bone density and many other wonderful things we are learning about. So this is also an important one that takes a stimulation test to bring it out and measure to see the brains potential.
Puberty hormones will be addressed later and at times these kids may not generate the right signal to start things at that time(last priority right now)
Hope this helps. Your endocrinologist is very well equipped to talk with you about each and everyone of these issues, set aside time and talk with them and make a list of all your questions.
Thank you Dr. for all the helpful information. I have few more questions regarding hypopituitarism: First of all, can her endoconologist arrange to do another MRI on my daughter to find out if she has a pituitary gland or if its missing completely. Secondly, how can the babies get into this situation, is it something related to the genes or somthing else? Now she has this situation then it means she is going to be on the medication for rest of her life? IS she going to be behind in her growth and all the developmental way too, because she is behind right now, is it going to affect her in the future too?
one mri with and without contrast is enough, so if it has been done this will be able to show anatomy of the pituitary. This is a developmental thing, rare rare to be genetic and we do not know exact form of inheritance, alot of times it is just at the time of development of the nervous system of the fetus. No one did anything to cause this.
Most of the times replacement medications are needed for life if indeed she is deficient. With replacement growth should improve if not follow a pretty normal course provided the rest of her health is good. It is hard to predict development, such a complex process, so with assistance such as PT and OT these skills become learned to make up for what is lacking.
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