Most internists are aware of the disease entity called adrenal hyperplasia 

involving cortex, causing Cushing's syndrome, but never heard of

hyperplasia of adrenal gland medulla which produces catecholamines,

i.e. epinephrine and norepinephrine.

This courageous young man turned out to have hereditary adrenal gland

medulla hyperplasia which afflicted his mother and another member of family.

This young man is some sort of genius who possesses the intelligence,

courage, determination, perseverance and every other remarkable trait and

humbled the entire medical community.

It is just incredible!

Sometimes I wonder how difficult it is to see inside of our body when God 

designed us to see outside of it. Have we had tried to play God? I remember neuroanatomy

professor 성기준 often referred difficult questions to God by saying “God only knows(GOK).

Doug certainly fulfilled the prophecy of  “God helps those who help themselves”.

It is a great story Dr. Lee! Thank you. 

That is quite a moving story and delighted to learn this young man pulled himself through to delivering such a happy ending. However, I am quite baffled with such difficulty poor young man had before reaching to right diagnosis for adrenal medullary hyperplasia (AMH); further he has such unique family Hx with his mother and aunt sharing same clinical condition, alarming strongly suspected hereditary endocrine condition involved to a genetic issue, which cannot escape from the keen eye of the internist/endocrinologist.   

I am NOT an endocrinologist nor endocrine surgeon – I once wanted to keep this specialty as my additional career to a transplant-vascular surgeon but I couldn’t afford to stretch myself too thin! - and further, I do not want to become a Monday morning quarterback. But, AMH is very well known to be associated with a genetic syndrome, mainly Multiple endocrine neoplasias, type II (MEN II) and many endocrine surgeons remain suspicious this unique condition as a precursor of pheochromocytoma to watch like a hawk.

Even as a ‘retired’ vascular surgeon, I am still familiar with this AMH of non-hereditary origin along the ‘renal’ hypertension as well as pheochromocytoma so that I am really annoyed why it took such long way to get right diagnosis. Although AMH is much rarer than pheochromocytoma, it is very well recognized by the vascular surgeon as well, together with Cushing’s for proper D.D. Even if blood tests like serum electrolytes (e.g. potassium), cortisol, and aldosterone, etc could remain normal, there are so many other tests to give a positive clue for AMH with different degrees of elevation among uric and serous catecholamine, vanillylmandelic acid (VMA), uric 17-OH, and 17-KS, etc. Even they can do Regitine suppressing tests to confirm.

Nevertheless, he wrote ‘the fourth scan finally showed his adrenals "glowing brightly in 2006”- which should be ¹³¹I-MIBG while MRI scan and CT scan show negative results” and further he chose the ‘unorthodox’ procedure, which blew my mind and driving me nuts!

Because bilateral subtotal adrenalectomy is the first line to attack such bilateral AMH whether there are masses existing or not. Some institute like Mayo, if I remember correctly, they do remove severer side first for bilateral hyperplasia and if clinical manifestations are not controlled, then do proceed to contralateral partial adrenalectomy.

Anyhow, it certainly interesting case we could learn a lesson.

BB Lee

P.S. My personal experience on the adrenal gland removal is limited to a total 5 cases of pheochromocytoma and 3 cases of AMH including 1 with MEN II; 5 of them were done with laparoscopic surgery through a retroperitoneal approach like we do donor kidney harvest.