Patients Undergoing Adrenalectomy Should Receive Steroid Substitutive Therapy

All patients who undergo removal of one adrenal gland due to Cushing’s syndrome (CS) or adrenal incidentaloma (AI, adrenal tumors discovered incidentally) should receive a steroid substitutive therapy, a new study shows.

The study, “Predictability of hypoadrenalism occurrence and duration after adrenalectomy for ACTH‐independent hypercortisolism,” was published in the Journal of Endocrinological Investigation.

CS is a rare disease, but subclinical hypercortisolism, an asymptomatic condition characterized by mild cortisol excess, has a much higher prevalence. In fact, subclinical hypercortisolism, is present in up to 20 percent of patients with AI.

The hypothalamic-pituitary-adrenal axis (HPA axis) is composed of the hypothalamus, which releases corticotropin-releasing hormone (CRH) that acts on the pituitary to release adrenocorticotropic hormone (ACTH), that in turn acts on the adrenal gland to release cortisol.

To avoid excess cortisol production, high cortisol levels tell the hypothalamus and the pituitary to stop producing CRH and ACTH, respectively. Therefore, as CS and AI are characterized by high levels of cortisol, there is suppression of the HPA axis.

As the adrenal gland is responsible for the production of cortisol, patients might need steroid substitutive therapy after surgical removal of AI. Indeed, because of HPA axis suppression, some patients have low cortisol levels after such surgeries – clinically known as post-surgical hypocortisolism (PSH), which can be damaging to the patient.

While some researchers suggest that steroid replacement therapy should be given only to some patients, others recommend it should be given to all who undergo adrenalectomy (surgical removal of the adrenal gland).

Some studies have shown that the severity of hypercortisolism, as well as the degree of HPA axis suppression and treatment with ketoconazole pre-surgery in CS patients, are associated with a longer duration of PSH.

Until now, however, there have been only a few studies to guide in predicting the occurrence and duration of PSH. Therefore, researchers conducted a study to determine whether HPA axis activity, determined by levels of ACTH and cortisol, could predict the occurrence and duration of PSH in patients who undergo an adrenalectomy.

Researchers studied 80 patients who underwent adrenalectomy for either CS or AI. Prior to the surgery, researchers measured levels of ACTH, urinary free cortisol (UFC), and serum cortisol after 1 mg dexamethasone suppression test (1 mg-DST).

After the surgery, all patients were placed on steroid replacement therapy and PSH was determined after two months. For those with PSH, levels of cortisol were determined every six months for at least four years.

Results showed that PSH occurred in 82.4 percent of CS patients and 46 percent of AI patients. PSH lasted for longer than 18 months in 50 percent of CS and 30 percent of AI patients. Furthermore, it lasted longer than 36 months for 35.7 percent of CS patients.

In all patients, PSH was predicted by pre-surgery cortisol levels after the 1 mg-DST, but with less than 70 percent accuracy.

In AI patients, a shorter-than-12-month duration of PSH was not predicted by any HPA parameter, but was significantly predicted by an absence of pre-surgery diagnosis of subclinical hypercortisolism.

So, this study did not find any parameters that could significantly predict with high sensitivity and specificity the development or duration of PSH in all patients undergoing adrenalectomy.

Consequently, the authors concluded that “the PSH occurrence and its duration are hardly predictable before surgery. All patients undergoing unilateral adrenalectomy should receive a steroid substitutive therapy.”

From https://cushingsdiseasenews.com/2017/12/08/therapy-cushings-patients-adrenalectomy/

Lower health-related quality of life observed in patients with Addison’s disease, Cushing’s syndrome

Patients with hypothalamic-pituitary-adrenal axis dysregulations report health-related quality of life that is far lower than that of the general population, according to findings of a prospective study.

“In most centers, both patients with adrenal deficiency and patients with Cushing’s syndrome are managed by the same team,” Charlotte DeBucy, of the Center for Rare Adrenal Diseases at Cochin Hospital in Paris, and colleagues wrote. “Despite the usual perception that both types of diseases alter quality of life, few studies have similarly investigated the impact of cortisol dysregulations on [health-related quality of life]. Such studies are important, however, to identify meaningful differences that would be important to consider to improve management and outcome.”

De Bucy and colleagues analyzed data from 343 patients with Addison’s disease or Cushing’s syndrome followed in routine practice at a single center in France between September 2007 and April 2014 (78% women; mean age, 48 years; mean length of time since diagnosis, 7.8 years; 61% married). All participants completed the short-form health survey (SF-36), a survey of health-related quality-of-life measures and the 12-item general health questionnaire (GHQ-12), a measure of psychological well-being or distress. Questionnaires were completed at baseline and at 6, 12, 24 and 36 months. Patients with Cushing’s syndrome were also assessed for cortisol status at baseline and at follow-up evaluations.

Within the cohort, 206 had Cushing’s syndrome of pituitary origin, 91 had Cushing’s syndrome of adrenal origin and 46 patients had Addison’s disease; 16% were included in the study before any treatment was initiated.

Researchers found that mean standard deviation scores for psychological and physical dimensions of the SF-36 were “well below” those of the general population, but diagnosis, cortisol status and time since treatment initiation all influenced individual scores. Cushing’s syndrome of pituitary origin was associated with worse health-related quality of life, especially for physical functioning, social functioning and mental health. In Cushing’s syndrome, health-related quality of life was generally worse during periods of hypercortisolism, but scores for these patients were lower than those of patients with Addison’s disease even during periods of hypocortisolism or eucortisolism, according to the researchers.

“The differences were particularly large for physical functioning and role-physical subscales,” the researchers wrote.

They also found that mental health scores for patients with Cushing’s syndrome decreased during periods of hypocortisolism, whereas other adrenal conditions were associated with higher mental health scores.

More than half of patients, regardless of diagnosis and cortisol status, had psychological distress requiring attention, according to the GHQ-12 survey.

“Our findings are important for clinical practice,” the researchers wrote. “The consequences of cortisol dysregulation on [health-related quality of life] should be considered in the management of adrenal insufficiency and even more (in) Cushing’s syndrome patients, and these consequences can be long term, affecting apparently cured patients. Early information on these consequences might be helpful for patients who often perceive a poor quality of life as the result of inadequate disease control or treatment. Even if this possibility exists, knowing that adrenal diseases have long-lasting effects on [health-related quality of life] may be helpful for patients to cope with them.” – by Regina Schaffer

Disclosure: L’association Surrénales supported this study. The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/in-the-journals/%7B842655ce-e710-4476-a3c2-2909b06434ed%7D/lower-health-related-quality-of-life-observed-in-patients-with-addisons-disease-cushings-syndrome

New Diagnostic Criteria for Subclinical Hypercortisolism using Postsurgical Hypocortisolism

Clin Endocrinol (Oxf). 2016 Jun 24. doi: 10.1111/cen.13145. [Epub ahead of print]

 

Abstract

OBJECTIVE:

There is no consensus on the biochemical diagnostic criteria for subclinical hypercortisolism (SH). Using parameters related to the hypothalamic-pituitary-adrenal axis, we aimed to develop a diagnostic model of SH for predicting postsurgical hypocortisolism and metabolic complications.

DESIGN:

Prospective and cross-sectional, observational, multicentre study in Korea.

METHODS:

After exclusion of overt Cushing’s syndrome, adrenal incidentaloma (AI) patients who underwent unilateral adrenalectomy (n = 99) and AI patients (n = 843) were included. Primary outcome was defined as the presence of postsurgical hypocortisolism; secondary outcome was the presence of ≥4 complications (components of the metabolic syndrome and low bone mass). Postsurgical hypocortisolism was determined on the fifth postsurgery day using the ACTH stimulation test.

RESULTS:

Thirty-three of the 99 patients developed postsurgical hypocortisolism. Analysis of the presurgery overnight 1-mg dexamethasone suppression test (1-mg DST) showed that all patients with cortisol levels of >138 nmol/l experienced postsurgical hypocortisolism, whereas those with levels of ≤61 nmol/l did not. The models of (i) 1-mg DST >138 nmol/l or (ii) >61 nmol/l with the presence of one among low levels of ACTH and dehydroepiandrosterone-sulphate had the highest accuracy (89·9%, P < 0·001) and odds ratio [OR 111·62, 95% confidence interval (CI) 21·98-566·74, P < 0·001] for predicting postsurgical hypocortisolism. Finally, patients with the same criteria in the 843 AI patients showed the highest risk for having ≥4 complications (OR 3·51, 95% CI 1·84-6·69, P < 0·001), regardless of gender, age, body mass index and bilaterality.

CONCLUSIONS:

Our proposed model is able to accurately predict subtle cortisol excess and its chronic manifestations in AI patients.

© 2016 John Wiley & Sons Ltd.

Adrenal Diseases During Pregnancy: Pathophysiology, Diagnosis And Management Strategies

Am J Med Sci. 2014 Jan;347(1):64-73. doi: 10.1097/MAJ.0b013e31828aaeee.

Author information

Abstract

: Adrenal diseases-including disorders such as Cushing’s syndrome, Addison’s disease, pheochromocytoma, primary hyperaldosteronism and congenital adrenal hyperplasia-are relatively rare in pregnancy, but a timely diagnosis and proper treatment are critical because these disorders can cause maternal and fetal morbidity and mortality.

Making the diagnosis of adrenal disorders in pregnancy is challenging as symptoms associated with pregnancy are also seen in adrenal diseases. In addition, pregnancy is marked by several endocrine changes, including activation of the renin-angiotensin-aldosterone system and the hypothalamic-pituitary-adrenal axis.

The aim of this article was to review the pathophysiology, clinical manifestation, diagnosis and management of various adrenal disorders during pregnancy.

PMID:
23514671
[PubMed – in process]

From http://www.ncbi.nlm.nih.gov/pubmed/23514671

Doctor’s Notes: Part 2, Adrenal

The adrenal glands sit atop the kidneys.

The adrenal glands sit atop the kidneys. (Photo credit: Wikipedia)

Acronyms or abbreviations for “Adrenal”

AD: adrenal vein
AG: adrenal gland
AdNA: adrenal gland
AC: adrenal cortex
adc: adrenal cortex
ADM: adrenal medulla
AA: adrenal adenoma
AF: adrenal failure
AM: adrenal medulla
AA: adrenal androgen
PA: pituitary-adrenal
AA: adrenal androgens
AAs: adrenal androgens
AM: adrenal medullary
LAV: left adrenal vein
AH: adrenal hypoplasia
AH: adrenal hemorrhage
AE: adrenal enucleation
AG: adrenal glomerulosa
AH: adrenal hyperplasia
HFA: human fetal adrenal
BAC: bovine adrenal cells
ADM: adrenal demedullation
AI: adrenal incidentaloma
AI: adrenal insufficiency
AVS: adrenal vein sampling
AI: adrenal incidentalomas
BAM: Bovine Adrenal Medulla
PAA: pituitary-adrenal axis
AMQD: Adrenal Move Quick Draw
AVS: Adrenal venous sampling
ach: adrenal cortical hormone
ACCs: adrenal chromaffin cells
AZF: adrenal zona fasciculata
BAM: Bovine adrenal medullary
PAL: Primary adrenal lymphoma
Ad4BP: Adrenal 4-binding protein
BAC: bovine adrenal chromaffin
ACC: adrenal cortical carcinoma
acca: adrenal cortical carcinoma
BAG: bovine adrenal glomerulosa
SAM: sympatho-adrenal-medullary
NAH: neonatal adrenal hemorrhage
PAH: primary adrenal hyperplasia
AHC: adrenal hypoplasia congenita
ACA: adrenal cortex autoantibodies
ACTH: adrenal corticotropic hormone
BAH: bilateral adrenal hyperplasia
CAH: congenital adrenal hypoplasia
HPA: hypothalamo-pituitary-adrenal
PAI: primary adrenal insufficiency
SAM: sympathetic-adrenal medullary
cah: congenital adrenal hyperplasia
HPA: hypothalamic-pituitary-adrenal
IAH: idiopathic adrenal hyperplasia
ACTH: adrenal corticotrophic hormone
ahc: adrenal hypoplasia, congenital
BAMC: bovine adrenal medullary cells
H-P-A: hypothalamic-pituitary-adrenal
HPA: hypothalamic-adrenal-pituitary
HPA: hypothalamus-pituitary-adrenal
HPAA: hypothalamic-pituitary-adrenal
IHA: idiopathic adrenal hyperplasia
LOAH: late-onset adrenal hyperplasia
NCAH: nonclassic adrenal hyperplasia
UAH: unilateral adrenal hyperplasia
BACC: bovine adrenal chromaffin cells
BACCs: bovine adrenal chromaffin cells
BCC: Bovine adrenal chromaffin cells
CAH: congenital adrenal hyperplasias
HHA: hypothalamo-hypophyseal-adrenal
BAC: bovine adrenal fasciculata cells
ARH: adrenal regeneration hypertension
HPAA: hypothalamo-pituitary-adrenal axis
ASNA: adrenal sympathetic nerve activity
HPA: hypothalamo-pituitary-adrenal axis
BAMC: bovine adrenal medullary chromaffin
FAH: Functional adrenal hyperandrogenism
HPA: hypothalamic-pituitary-adrenal axis
HPA-axis: hypothalamic-pituitary-adrenal axis
HPAA: hypothalamic-pituitary-adrenal axis
HPAA: hypothalamus-pituitary-adrenal axis
AASH: adrenal androgen stimulating hormone
BAME: bovine adrenal medullary endothelial
HPA: hypothalamus-pituitary-adrenal gland
NADF: National Adrenal Diseases Foundation
PAMC: porcine adrenal medullary chromaffin
CLAH: congenital lipoid adrenal hyperplasia
APA: aldosterone-producing adrenal adenoma
HPA: hypothalamic-pituitary-adrenal system
HPAT: hypothalamus-pituitary-adrenal-thymus
LHPA: limbic-hypothalamic-pituitary-adrenal
PCAI: primary chronic adrenal insufficiency
HHAS: hypothalamo-hypophyseal-adrenal system
HPA: hypothalamo-pituitary-adrenal cortical
HPA: hypothalamic-pituitary-adrenal cortical
RAMEC: rat adrenal medullary endothelial cells
CVAH: congenital virilizing adrenal hyperplasia
CAH: congenital virilizing adrenal hyperplasia
LOCAH: late-onset congenital adrenal hyperplasia
LHPA: limbic-hypothalamic-pituitary-adrenal axis
NC-CAH: non-classical congenital adrenal hyperplasia
AIMAH: ACTH-independent bilateral macronodular adrenal
pre-ASNA: preganglionic adrenal sympathetic nerve activity
AIMAH: ACTH-independent macronodular adrenal hyperplasia
CAHSA: Congenital Adrenal Hyperplasia Support Association
AIMAH: ACTH-independent bilateral macronodular adrenal hyperplasia

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