Paediatric cyclical Cushing’s disease due to corticotroph cell hyperplasia

Posted on June 11, 2015 by MaryO

Cushing’s disease is very rare in the paediatric population. Although uncommon, corticotroph hyperplasia causing Cushing’s syndrome has been described in the adult population, but appears to be extremely rare in children.

Likewise, cyclical cortisol hypersecretion, while accounting for 15 % of adult cases of Cushing’s disease, has only rarely been described in the paediatric population. Here, we describe a very rare case of a 13-year old boy with cyclical cortisol hypersecretion secondary to corticotroph cell hyperplasia. The case is that of a 13-year old boy, presenting with a long history of symptoms and signs suggestive of hypercortisolism, who was found to have cyclical ACTH-dependent hypercortisolism following dynamic pituitary testing and serial late-night salivary cortisol measurements.

The patient underwent endoscopic transsphenoidal resection of the pituitary. Early surgical remission was confirmed by undetectable post-operative morning plasma cortisol levels.

Histology and immunocytochemistry of the resected pituitary tissue showed extensive corticotroph cell hyperplasia.

Conclusion: This report describes a rare case of cyclical Cushing’s disease secondary to corticotroph hyperplasia in a paediatric patient. This highlights the challenging and varied nature of Cushing’s disease and its diagnosis, and the need to keep a differential diagnosis in mind during the diagnostic process.

Author: E. Noctor S. Gupta T. Brown M. Farrell M. Javadpour C. Costigan A. Agha
Credits/Source: BMC Endocrine Disorders 2015, 15:27

From http://7thspace.com/headlines/510543/paediatric_cyclical_cushings_disease_due_to_corticotroph_cell_hyperplasia.html

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Cushing Syndrome in Children: Growth after Surgical Cure

Posted on October 22, 2014 by MaryO

Cushing syndrome (CS) occurs only rarely in children, but when it does, it causes weight gain and stunting. In young children, adrenal tumors are usually the cause while in adolescents, pituitary tumors are more likely.

The September 2014 issue of Endocrine-Related Cancer examines growth patterns in 19 pediatric patients with ACTH-dependent CS (CD) and 18 patients with a form of ACTH-independent CS, micronodular adrenal hyperplasia (MAH). The researchers gathered data at the time of surgery and also followed up one year later.

Patients in the CD and the MAH groups had similar demographic characteristics, baseline heights and BMI scores before surgery. All patients experienced significant improvements in height and BMI after surgery. Patients with MAH, however, fared significantly better than those with CD and had better post-operative growth.

The researchers propose several reasons:

  • When ACTH-secreting pituitary adenoma requires extensive surgical exploration, remaining pituitary cells often lose some of their function.
  • CD patients tend to be older and have consistent and increased glucocorticoid exposure; they develop vertebral fractures more often leading to compromised skeletal and overall growth potential. MAH patients often have cyclical CS, with intermittent hypercortisolism and an overall milder CS.
  • CD patients often need a longer-than-expected course of therapy with steroids after surgery, which alters metabolism and growth.
  • CD patients have been shown to have advance bone age because of ACTH-induced metabolic changes.

The authors indicate that CS patients are often considered for growth hormone therapy once the underlying problem is corrected. They remind clinicians that MAH patients are less likely to need growth hormone. They recommend close monitoring for CD patients, and early intervention with growth hormone if growth does not meet expectation. –

See more at: http://www.hcplive.com/articles/Cushing-Syndrome-in-Children-Growth-after-Surgical-Cure

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Interview with Stacy, Mom to Possible Cushing’s Patient

Posted on June 8, 2014 by MaryO

Stacy writes in her bio

MacKenna is my daughter.  I am in desperate need of assistance regarding her weight.

She is 6 years old and weighs about 95 pounds.  At four, she was normal to underweight.

First clue – she gained 10 pounds in three months.  Of course doctor’s wanted a food log …. however, as her parent, I knew something was wrong.

So much to say, I NEED to help my baby and am hoping someone on this board can point me to a good pedi endo (I am currently on my second).  I have pictures to show the progression of her changes.  Her growth pattern is abnormal as she does not gain height while she gains weight and vice versa.

I believe it is cyclical cushings.  And if it isn’t, I still need help as this is going to kill her – her little frame cannot hold much more.

 

McKenna’s mom will be interviewed June 11 at 6:00 pm eastern in BlogTalkRadio.  Archives will be available later in the Cushing’s Podcast.

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Quality of Life and Other Outcomes in Children Treated for Cushing Syndrome

Posted on June 25, 2013 by MaryO

Abstract

Journal of Clinical Endocrinology and Metabolism, 05/16/2013  Review Article  Clinical Article

Keil MF – Cushing syndrome (CS) in children is associated with residual impairment in measures of health–related quality of life, even after successful resolution of hypercortisolemia, highlighting the need for early identification of morbidities and improvements in long–term management of these patients.

A review of the literature identifies significant morbidities associated with CS of pediatric onset, which must not be treated in isolation. CS affects children and adolescents in many ways that are different than adults.

Post–treatment challenges for the child or adolescent treated for CS include: optimize growth and pubertal development, normalize body composition, and promote psychological health and cognitive maturation. All these factors impact health–related quality of life, which is an important outcome measure to assess the burden of disease as well as the effect of treatment.

Get this article here

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