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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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Kearns Sayre Syndrome. Begin Him On Hormone Injections? Is Puberty Difficulty?

Hello Dr. Grief, Thank you for taking the time to answer my question. My question is with regard to Kearns Sayre Syndrome and hormone injections . My son is 14yo, he has yet to start (or show signs of starting) puberty . However, this is very common with patients who have KSS. My sons Endocrinologist wants to begin him on hormone injections once a month. I have yet to agree to this. The reason being, (a) I have yet to find any research that proves this is beneficial to my son, (b) his doctor could not support nor refer me to any research that proves this is not going to put my sons disease into progression, (c) the only information that I have found concerning puberty and KSS is that puberty is a difficult period for patients with mitochondrial diseases due to the changes that occur. If you can give me any advice or refer me to a good resource concerning this topic I would truly appreciate it. Again, thank you for your time. Chrissy
Thu, 18 Feb 2021
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General & Family Physician 's  Response
Hi,

Presently, we have no known treatment for Kearns Sayre syndrome. Treatment is just conservative and symptomatic. Of course, any hormonal deficiency should be corrected to bring these to a normal level. In the case of your son, if there is deficiency of Progesterone, then injection of progesterone may be justified. As such there is no specific role of Progesterone in this disorder.

Take care. Hope I have answered your question. Let me know if I can assist you further.

Regards,
Dr. Satnarain Bansal, General & Family Physician
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Kearns Sayre Syndrome. Begin Him On Hormone Injections? Is Puberty Difficulty?

Hi, Presently, we have no known treatment for Kearns Sayre syndrome. Treatment is just conservative and symptomatic. Of course, any hormonal deficiency should be corrected to bring these to a normal level. In the case of your son, if there is deficiency of Progesterone, then injection of progesterone may be justified. As such there is no specific role of Progesterone in this disorder. Take care. Hope I have answered your question. Let me know if I can assist you further. Regards, Dr. Satnarain Bansal, General & Family Physician