Food-dependent Cushing syndrome: a new entity of organic hypercorticism

Matejka G, et al. Rev Med Interne. 1996.

Abstract

Diagnosis of Cushing’s syndrome is quite difficult in endocrinology. Spontaneous Cushing’s syndrome is usually divided into two subgroups, one which is dependent on corticotropin (ACTH) and another one which is not.

In the first class are Cushing’s disease, the ectopic corticotropin syndrome and the rare ectopic corticotropin-releasing hormone (CRH) syndrome; these ACTH-dependent Cushing’s syndrome have usually diffusely enlarged adrenal glands.

In the second class are cortisol producing unilateral adrenocortical adenomas or carcinomas, and the recent Cushing’s syndrome with food dependent periodic hormonogenesis.

This food dependent Cushing’s syndrome is an ACTH-independent Cushing’s syndrome with multinodular enlargement of both adrenal glands. Pathogenesis is an aberrant adrenal sensitivity to physiologic secretion of gastric inhibitory peptide (GIP). Ectopic expression of GIP receptors on adrenal cells involve pathologic food induced cortisol secretion.

Food dependent Cushing’s syndrome is a new cause of Cushing’s syndrome. Food induced cortisol secretion may have to be explored in the ACTH-independent Cushing’s syndrome.

PMID

8758532 [PubMed – indexed for MEDLINE]

Full text

Full text from provider (Elsevier Science) Article in French.

From http://www.ncbi.nlm.nih.gov/m/pubmed/8758532/

Biography of a Food-Dependent Cushing’s patient

Severe psychosis due to Cushing’s syndrome in a patient with a carcinoid tumour in the lung

Severe psychosis in patients with Cushing’s syndrome is a rare occurrence and can be extremely resistant to medical therapy. We describe a case of a 51-year-old Afro-Caribbean female patient, with refractory severe hypertension (initially resistant to polypharmacy) and gradual development of severe psychosis secondary to ectopic Cushing’s syndrome, who was subsequently diagnosed to have a carcinoid tumour in her lung.

Her psychotic episodes – secondary to hypercortisolism and initially refractory to the medical therapy – subsided only after the resection of the carcinoid tumour in her right lower pulmonary lobe. Early localization and appropriate surgical resection of the ectopic ACTH-secreting tumour can be of immense value to the successful alleviation of the psychotic episodes of the patients with ectopic Cushing’s syndrome.

Author: Mohamad BabaDebamalya Ray
Credits/Source: World Journal of Surgical Oncology 2015, 13:165

From http://7thspace.com/headlines/507616/severe_psychosis_due_to_cushings_syndrome_in_a_patient_with_a_carcinoid_tumour_in_the_lung_a_case_report_and_review_of_the_current_management.html

Day Twenty-eight, Cushing’s Awareness Challenge 2015

 

I first saw a similar image to this one with the saying Life. Be in it at a recreation center when my son was little.  At the time, it was “Duh, of course I’m in it”.

The original image was one a couple males, a couple females and a dog walking/running.  No folks in wheelchairs, no older folks and certainly no zebras.

It would be nice to have everyone out there walking or running but that’s not real life, at least in the Cushie world.  It’s been a long time since I’ve really been In My Life – maybe it’s time to get back.

A dear friend who has not one, but two forms of cancer was traveling throughout Europe for the first time after her husband’s death wrote:

Some final words before I turn in for the night. If there is a spark of desire within you to do something which is not contrary to God’s Holy Law, find a way to make it happen. All things are possible and blessings abound for those who love Him. Life is such an adventure. Don’t be a spectator – live every single moment for Him and with Him.

Somedays, it’s hard even getting up in the morning but I’m trying.  I’ve tried Water Aerobics for People with Arthritis and I actually went to class twice a week, I got a new part-time job two years ago, my son and I will play at Steinway Hall in NYC again in June, we have plans for a cruise in June, and a trip to Scotland to cross something off my Bucket List – seeing/hearing the Edinburgh Tattoo.

This is the one and only life I’ll ever have and I want to make the most of it!

 

 

Day Twenty-six, Cushing’s Awareness Challenge 2015

It’s Sunday again, so this is another semi-religious post so feel free to skip it 🙂

I’m sure that many would think that this is a semi-odd choice for all-time favorite hymn.

My dad was a Congregational (now United Church of Christ) minister so I was pretty regular in church attendance in my younger years.

Some Sunday evenings, he would preach on a circuit and I’d go with him to some of these tiny churches.  The people there, mostly older folks, liked the old hymns best – Fanny Crosby and so on.

So, some of my “favorite hymns” are those that I sang when I was out with my Dad.  Fond memories from long ago.

In 1986 I was finally diagnosed with Cushing’s after struggling with doctors and trying to get them to test for about 5 years.  I was going to go into the NIH (National Institutes of Health) in Bethesda, MD for final testing and then-experimental pituitary surgery.

I was terrified and sure that I wouldn’t survive the surgery.

Somehow, I found a 3-tape set of Readers Digest Hymns and Songs of Inspiration and ordered that. The set came just before I went to NIH and I had it with me.

At NIH I set up a daily “routine” of sorts and listening to these tapes was a very important part of my day and helped me get through the ordeal of more testing, surgery, post-op and more.

When I had my kidney cancer surgery, the tapes were long broken, but I had replaced all the songs – this time on my iPod.

Abide With Me was on this tape set and it remains a favorite to this day.  Whenever we have an opportunity in church to pick a favorite, my hand always shoots up and I request page 700.  When someone in one of my handbell groups moves away, we always sign a hymnbook and give it to them.  I sign page 700.

I think that many people would probably think that this hymn is depressing.  Maybe it is but to me it signifies times in my life when I thought I might die and I was so comforted by the sentiments here.

This hymn is often associated with funeral services and has given hope and comfort to so many over the years – me included.

If you abide in Me, and My words abide in you, you will ask what you desire, and it shall be done for you.

~John 15:7

Abide With Me

Words: Henry F. Lyte, 1847.

Music: Eventide, William H. Monk, 1861. Mrs. Monk described the setting:

This tune was written at a time of great sorrow—when together we watched, as we did daily, the glories of the setting sun. As the last golden ray faded, he took some paper and penciled that tune which has gone all over the earth.

Lyte was inspired to write this hymn as he was dying of tuberculosis; he finished it the Sunday he gave his farewell sermon in the parish he served so many years. The next day, he left for Italy to regain his health. He didn’t make it, though—he died in Nice, France, three weeks after writing these words. Here is an excerpt from his farewell sermon:

O brethren, I stand here among you today, as alive from the dead, if I may hope to impress it upon you, and induce you to prepare for that solemn hour which must come to all, by a timely acquaintance with the death of Christ.

For over a century, the bells of his church at All Saints in Lower Brixham, Devonshire, have rung out “Abide with Me” daily. The hymn was sung at the wedding of King George VI, at the wedding of his daughter, the future Queen Elizabeth II, and at the funeral of Nobel peace prize winner Mother Teresa of Calcutta in1997.

Abide with me; fast falls the eventide;

The darkness deepens; Lord with me abide.

When other helpers fail and comforts flee,

Help of the helpless, O abide with me.

Swift to its close ebbs out life’s little day;

Earth’s joys grow dim; its glories pass away;

Change and decay in all around I see;

O Thou who changest not, abide with me.

Not a brief glance I beg, a passing word;

But as Thou dwell’st with Thy disciples, Lord,

Familiar, condescending, patient, free.

Come not to sojourn, but abide with me.

Come not in terrors, as the King of kings,

But kind and good, with healing in Thy wings,

Tears for all woes, a heart for every plea—

Come, Friend of sinners, and thus bide with me.

Thou on my head in early youth didst smile;

And, though rebellious and perverse meanwhile,

Thou hast not left me, oft as I left Thee,

On to the close, O Lord, abide with me.

I need Thy presence every passing hour.

What but Thy grace can foil the tempter’s power?

Who, like Thyself, my guide and stay can be?

Through cloud and sunshine, Lord, abide with me.

I fear no foe, with Thee at hand to bless;

Ills have no weight, and tears no bitterness.

Where is death’s sting? Where, grave, thy victory?

I triumph still, if Thou abide with me.

Hold Thou Thy cross before my closing eyes;

Shine through the gloom and point me to the skies.

Heaven’s morning breaks, and earth’s vain shadows flee;

In life, in death, O Lord, abide with me.

 

Day Twenty-three, Cushing’s Awareness Challenge 2015

 

This is a tough one.  Sometimes I’m in “why me” mode.  Why Cushing’s?  Why cancer?  Unfortunately, there’s not a thing I can do about either.  Cushing’s, who knows the risk factors?  For kidney cancer I found out the risk factors and nearly none apply to me. So why? But why not?  No particular reason why I should be exempt from anything.

Since there’s nothing to be done with the exception of trying to do things that could harm my remaining kidney, I have to try to make the best of things.  This is my life.  It could be better but it could be way worse.

One of the Challenge topics was to write about “My Dream Day” so here’s mine…

I’d wake up on my own – no snooze alarms – at about 8 am, sun streaming through the window.  I’d we well rested and not have had any nightmares the night before.  I remember my son is home for a visit but I let him sleep in for a while.

I’d get out for a bike ride or a brisk walk, come home, head for the hot tub then shower.  I’d practice the piano for a bit, then go out to lunch with friends, taking Michael with me.  While we’re out, the maid will come in and clean the house.

After lunch, maybe a little technology shopping/buying.  Then the group of us go to one of our homes for piano duets, trios, 2-piano music.

When we get home, it’s immaculately clean and I find that the Prize Patrol has visited and left a substantial check.

I had wisely left something for dinner in the Ninja so dinner is ready.  After dinner, I check online and find no urgent email, no work that needs to be done, no bills that need to be paid, no blog challenge posts to write…

I wake up from My Dream Day and realize that this is so far from real life, so I re-read The Best Day of My Life  and am happy that I’m not dealing with anything worse.