Adrenal Insufficiency Patients Require More Education on Adrenal Crisis

Greater efforts to educate patients with adrenal insufficiency and their families about prevention of adrenal crisis may be necessary, according to data presented at the American Association of Clinical Endocrinologists (AACE) 24th Annual Scientific & Clinical Congress.

Additionally, the researchers, who looked at patients treated for adrenal insufficiency, found that many are not being adequately trained or equipped to deal with an adrenal crisis.

“These patients can crash and we are not doing enough to help prevent problems,” study investigator Nitika Malhotra, MD, endocrinologist in Lansing, Michigan, said. “We did this study because we think this is a big problem.”

Malhotra, who presented the study findings at the meeting, explained that patients with adrenal insufficiency are at risk for developing adrenal crisis, and it is now estimated that 8% of patients with adrenal insufficiency are hospitalized for adrenal crisis each year.

The problem, according to Malhotra, is that far too many patients are failing to receive crises prevention education. Moreover, they are not receiving emergency glucocorticoid kits.

“All of the families need to be taught and that is not happening,” Malhotra said in an interview with Endocrinology Advisor. “It will reduce the morbidity and mortality and the hospitalization, and it may improve the quality of life of patients too.”

For their study, Malhotra and her colleagues collected data from patients with adrenal insufficiency who were seen at a single institution between March 2009 and March 2014.

The investigators conducted a retrospective chart review and examined age, gender, causes of adrenal insufficiency, glucocorticoid dose, and monitoring for hyponatremia and hyperkalemia. They also looked at postural blood pressure, crises prevention education for glucocorticoid dose adjustments during stress, and whether patients had a Medic Alert ID or a parenteral glucocorticoid kit. 

The researchers identified 85 patients (29 males and 56 females) with adrenal insufficiency. Of these patients, 33 patients had primary adrenal insufficiency (38.8%) and 52 had secondary adrenal insufficiency (61.2%). The mean age of the patients was 55.8 years.

Among the 85 patients, 23 (27%) had postural blood pressures checked — five of whom were positive (21.7%). Seventy-seven patients (90.6%) were monitored for electrolytes, and 41 patients (48.2%) were on steroid doses above 20 mg per day.  

However, the researchers found that only 57 patients (67.1%) had received steroid dose adjustment instructions. In addition, only 29 patients (34.1%) had a Medic Alert ID, and only 17 patients (20%) were setup with emergency parenteral glucocorticoid kits.

Even though this study has many inherent limitations, Malhotra said, it appears that the preventive strategies for adrenal crisis in patients with adrenal insufficiency are not being consistently followed.

Patient education is paramount for achieving a successful prevention strategy for adrenal crisis, and endocrinologists have a responsibility to make sure that all patients with adrenal insufficiency have a Medic Alert ID and access to emergency glucocorticoid kits, according to Malhotra.

Furthermore, she said families should receive adequate education about parenteral steroid administration and steroid dose adjustments in stressful situations.

At her institution, Malhotra said, endocrinologists are introducing an automated electronic alert in their electronic medical records to determine if this electronic prompt will improve adherence.

Reference

1. Malhotra N et al. Abstract #102. Presented at: American Association of Clinical Endocrinologists (AACE) 24th Annual Scientific & Clinical Congress; May 13-17, 2015; Nashville, Tenn.

From http://www.endocrinologyadvisor.com/aace-2015/adrenal-crisis-in-adrenal-insufficiency/article/415123/

Severe fatigue, decreased physical activity in patients with Addison’s disease

van der Valk ES, et al. Clin Endocrinol. 2016;doi:10.1111/cen.13059.

Dutch adults with primary adrenal insufficiency reported abnormal or severe fatigue, reduced physical activity and significantly reduced quality of life vs. healthy controls, according to recent survey results.

In a cross-sectional study, Eline S. van der Valk, MD, of Amphia Hospital in Breda, the Netherlands, and colleagues also found that patients with Addison’s disease reported physical activity levels that were significantly lower than those reported by other Dutch chronically ill patients.

“The clinical relevance of the impaired [quality of life] and increased fatigue found in our study is supported by the size of the differences in scores and the restriction in physical activity in patients with [Addison’s disease], an important activity in daily life,” the researchers wrote. “Physical inactivity could be very detrimental in [Addison’s disease] because the prevalence of other cardiovascular risk factors is already increased, and it has been demonstrated that patients with [Addison’s disease] have an up to twofold increased mortality rate from [CVDs].”

Researchers analyzed survey data from 328 Dutch adults with Addison’s disease on stable glucocorticoid replacement therapy with hydrocortisone or cortisone acetate (mean age, 53 years; 223 women; mean duration of disease after diagnosis, 15.6 years). Participants attended outpatient clinics at University Medical Center Utrecht and Radboud University Nijmegen Medical Centre, or were members of the Dutch Association of Addison and Cushing Patients. They completed general and health-related quality of life (Short Form 36; Checklist Individual Strength) and physical activity questionnaires. Scores were compared with a random sample of 1,718 adults who completed a Dutch National Health Survey (controls).

Within the cohort, 53% of participants had isolated Addison’s disease; 74.1% received hydrocortisone therapy; 25.9% received cortisone acetate therapy; 87.2% received fludrocortisone therapy; and 23.2% received dehydroepiandrosterone replacement therapy.

Researchers found that 45.7% of participants with Addison’s disease met the standard of physical activity (Dutch standard of healthy physical exercise, defined as moderately intensive physical exercise for 30 minutes daily 5 days per week; “Fitnorm,” defined as 20 minutes of intensive physical exercise at least 3 days per week) vs. 67.8% of controls (P < .01). Researchers found 61% of participants with Addison’s disease reported abnormal fatigue, and 43% reported severe fatigue. Mean fatigue scores were significantly higher vs. controls (mean difference, 32.6; 95% CI, 24-41).

In both men and women with Addison’s disease, researchers found that quality of life scores in all component summaries were significantly decreased compared with controls, particularly in participants aged 65 years and younger. – by Regina Schaffer

Disclosure: The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7Ba8914384-d40e-41ab-aa1c-134d856d2edd%7D/severe-fatigue-decreased-physical-activity-in-patients-with-addisons-disease

Addison's disease may cause psychosis, say researchers

Research suggests that chronic adrenal insufficiency, more commonly known as Addison's disease, may be responsible for psychiatric symptoms in those who suffer with it. Unfortunately, these symptoms are poorly understood and inadequately studied. In one case, a 41-year-old construction worker was admitted to a psychiatric clinic complaining of depression. He had trouble sleeping and concentrating and had lost 6 pounds due to a loss of appetite. He was placed on 20mg of fluoxetine but returned 2 weeks later complaining that the therapy did not work, and even reported hallucinating his ex-wife, who had recently died in a car accident. He returned again later 4 months later and was found to have a weak pulse, major hypotension, and hyponatremia and hyperkalemia. It was at this point that he was diagnosed with Addison's disease.

The disease was first described by Thomas Addison in the mddle of the 19th century. It involves inadequate secretion from the adrenal glands, leading to lower secretion of glucocorticoids. Its usual symptom pigmentation involves fatigue, weight loss, nausea, vomiting, weakness and abdominal pain. Among its psychiatric symptoms are psychosis and delirium.

"An array of neuropsychiatric symptoms is associated with AD. Addison is quoted as saying in 1855 that AD patients might present with “attacks of giddiness, anxiety in the face, and delirium.”1 Anglin et al1 also noted four case series published in the 1940s and 1950s that found the prevalence of neuropsychiatric symptoms in AD to be between 64 and 84 percent. Iwata et al6 reported that in some cases, the neuropsychiatric symptoms were the initial and sole presentation of AD, even though such symptoms are more common in the late course of the disease; this might lead to a patient initially being misdiagnosed, as it did in our case, and in turn, incorrectly treated. Neuropsychiatric symptoms of AD include, but are not limited to, depression, lack of energy, and sleep disturbances. During an Addisonian crisis, agitation, delirium, and, in some cases, visual and auditory hallucinations are reported.1 According to Smart,7 neuropsychiatric symptoms might also be the first presentation of an Addisonian crisis, especially in a patient who was previously symptom free while under therapy."

It is not clear exactly why these neuropsychiatric symptoms appear. It is possible that electrolytic and metabolic disturbances could produce these symptoms, and hyponatremia, which is its central expression, can result in brain damage. Other researchers believe that the neuropsychiatric symptoms are the effects of glucocorticoid abnormalities on the brain. Some researchers believe that unusually low amounts of the substance could produce hallucinations. In one report, Addison's disease presented as a psychotic break:

"A Caucasian 63-year-old man presented a five-month history of progressive depressive symptoms with sadness, anhedonia, asthenia, hyporexia, insomnia, limb weakness, and psychotic symptoms. He was a former heavy smoker and had been diagnosed with chronic occupational lung disease (silicosis). After a specialized psychiatric consultation, the patient started taking fluoxetine, risperidone, and nitrazepam. After a short period of time, the patient withdrew his medication by his own because of worsening of the limb weakness. The patient became severely paranoid with persecutory beliefs, delusions, an infantile speech, progressive social isolation, fear of leaving his home, periods of mental confusion and disorientation, and sporadic nausea and vomits. A few days before hospital admission, the patient almost stopped fluid intake and evolved with syncope."

From http://www.examiner.com/article/addison-s-disease-may-cause-psychosis-say-researchers

Cushing's Awareness Day Challenge 2015

The Cushing's Awareness Challenge is almost upon us again!

Do you blog? Want to get started?

Since April 8 is Cushing's Awareness Day, several people got their heads together to create the Fourth Annual Cushing's Awareness Blogging Challenge.

All you have to do is blog about something Cushing's related for the 30 days of April.

There will also be a logo for your blog to show show you've participated.

Please let me know the URL to your blog in the comments area of this post or an email  and I will list it on CushieBloggers ( http://cushie-blogger.blogspot.com/)

The more people who participate, the more the word will get out about Cushing's.

Suggested topics - or add your own!

• In what ways have Cushing's made you a better person?
• What have you learned about the medical community since you have become sick?
• If you had one chance to speak to an endocrinologist association meeting, what would you tell them about Cushing's patients?
• What would you tell the friends and family of another Cushing's patient in order to garner more emotional support for your friend? challenge with Cushing's? How have you overcome challenges? Stuff like that.
• I have Cushing's Disease....(personal synopsis)
• How I found out I have Cushing's
• What is Cushing's Disease/Syndrome? (Personal variation, i.e. adrenal or pituitary or ectopic, etc.)
• My challenges with Cushing's
• Overcoming challenges with Cushing's (could include any challenges)
• If I could speak to an endocrinologist organization, I would tell them....
• What would I tell others trying to be diagnosed?
• What would I tell families of those who are sick with Cushing's?
• Treatments I've gone through to try to be cured/treatments I may have to go through to be cured.
• What will happen if I'm not cured?
• I write about my health because…
• 10 Things I Couldn’t Live Without.
• My Dream Day.
• What I learned the hard way
• Miracle Cure. (Write a news-style article on a miracle cure. What’s the cure? How do you get the cure? Be sure to include a disclaimer)
• Health Madlib Poem. Go to : http://languageisavirus.com/cgi-bin/madlibs.pl#.VPGZQlPF9A8 and fill in the parts of speech and the site will generate a poem for you.
• The Things We Forget. Visit http://thingsweforget.blogspot.com/ and make your own version of a short memo reminder. Where would you post it?
• Give yourself, your condition, or your health focus a mascot. Is it a real person? Fictional? Mythical being? Describe them. Bonus points if you provide a visual!
• 5 Challenges & 5 Small Victories.
• The First Time I…
• Make a word cloud or tree with a list of words that come to mind when you think about your blog, health, or interests. Use a thesaurus to make it branch more.
• How much money have you spent on Cushing's, or, How did Cushing's impact your life financially?
• Why do you think Cushing's may not be as rare as doctors believe?
• What is your theory about what causes Cushing's?
• How has Cushing's altered the trajectory of your life? What would you have done? Who would you have been
• What three things has Cushing's stolen from you? What do you miss the most? What can you do in your Cushing's life to still achieve any of those goals?
• What new goals did Cushing's bring to you?
• How do you cope?
• What do you do to improve your quality of life as you fight Cushing's?
• Your thoughts...?

Exophthalmos and Cushing's Syndrome

A woman experienced red, irritated and bulging eyes. She saw an ophthalmologist who strongly suspected Graves’ ophthalmopathy. However, the patient did not have and never had hyperthyroidism.

Indeed, she had primary hypothyroidism optimally treated with levothyroxine. Her thyroid stimulating hormone level was 1.197 uIU/mL.

An MRI of the orbits showed normal extraocular muscles without thickening, but there was mild proptosis and somewhat increased intraorbital fat content. Both thyroid-stimulating immunoglobulins as well as thyrotropin receptor antibodies were negative.

The patient presented to her primary care physician a few months later. She had experienced a 40-lb weight gain over only a few months and also had difficult-to-control blood pressure.

After failing to respond to several antihypertensive medications, her primary care physician astutely decided to evaluate for secondary causes of hypertension. A renal ultrasound was ordered to evaluate for renal artery stenosis, and the imaging identified an incidental right-sided adrenal mass. A CT confirmed a 3.4-cm right-sided adrenal mass. Her morning cortisol was slightly high at 24.7 ug/dL (4.3 – 22.4) and her adrenocorticotropic hormone was slightly low at 5 pg/mL (10-60).

At this point I saw the patient in consultation. She definitely had many of the expected clinical exam findings of Cushing’s syndrome, including increased fat deposition to her abdomen, neck, and supraclavicular areas, as well as striae. Her 24-hour urine cortisol was markedly elevated at 358 mcg/24hrs (< 45) confirming our suspicions.

She asked me, “Do you think that my eye problem could be related to this?”

“I’ve not heard of it before,” I replied, “but that doesn’t mean there can’t be a connection. Wouldn’t it be wonderful if your eyes got better after surgery?”

The patient underwent surgery to remove what fortunately turned out to be a benign adrenal adenoma.

When we saw her in follow-up 2 weeks later, her blood pressures were normal off medication and her eye symptoms had improved. I had a medical student rotating with me, so I suggested that we do a PubMed literature search.

The first article to come up was a case report titled “Exophthalmos: A Forgotten Clinical Sign of Cushing’s Syndrome.” Indeed, not only did Harvey Cushing describe this clinical finding in his original case series in 1932, but others have reported that up to 45% of patients with active Cushing’s syndrome have exophthalmos.

The cause is uncertain but is theorized to be due to increased intraorbital fat deposition. Unlike exophthalmos due to thyroid disease, the orbital muscles are relatively normal — just as they were with our patient.

Some of you may have seen exophthalmos in your Cushing’s patients; however, this was the first time I had seen it. Just because one has not heard of something, does not mean it could never happen; no one knows everything. “When in doubt, look it up” is a good habit for both attending physicians and their students.

For more information:

Giugni AS, et al. Case Rep Endocrinol. 2013; 2013: 205208.

From http://www.healio.com/endocrinology/adrenal/news/blogs/%7B779bf3e5-e1da-459e-af27-955c9b4274a5%7D/thomas-b-repas-do-facp-face-cde/exophthalmos-and-cushings-syndrome

New Jersey Ambulances Carrying Solu-Cortef

The New Jersey Department of Health passed a waiver in October of last year that allows ambulances to carry Solu- Cortef, for the purposes of treating an adrenal crisis. As a result, New Jersey ambulances can be better prepared to treat adrenal insufficiency.

This news was brought to NADF by Karen Fountain of the CARES Foundation, who has been helping push state health directors to accept protocols to help treat adrenal insufficient patients during an emergency.

Adrenal insufficient people in New Jersey should contact their local EMS to make them aware of the waiver, and encourage them to carry Solu-Cortef in their ambulances.

The hope is that other states, and eventually the entire country and beyond, will start having their ambulances carry the needed medication to treat adrenal crisis.

http://www.nadf.us

Diagnosing and Treating Cortisol Excess and Deficiency

From Day 1 of the 16th International Congress of Endocrinology and the Endocrine Society’s 96th Annual Meeting and Expo »

Chicago, IL - June 21, 2014

A phase 2 study of Chronocort®, a modified release formulation of hydrocortisone, in the treatment of adults with classic congenital adrenal hyperplasia

A Mallappa, L-A Daley, N Sinaii, C Van Ryzin, H Huatan, D Digweed, D Eckland, M Whitaker, LK Nieman, RJ Ross, DP Merke

Summary: Classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by cortisol and aldosterone deficiency and androgen excess. Current conventional glucocorticoid therapy is suboptimal as it cannot replace the normal cortisol circadian rhythm and inadequate or inappropriate suppression of adrenal androgens are common. In the preliminary results of a phase 2 study of Chronocort®, a modified release hydrocortisone capsule formulation, researchers found that Chronocort®, a novel modified release hydrocortisone capsule formulation, approximates physiological cortisol secretion, and improves biochemical control of CAH. Further analyses are underway.
Methods:

• The study objectives were to characterize pharmacokinetics and examine disease control following 6 months dose titration.
• Serial profiling was obtained at baseline (conventional glucocorticoid) and every 2 months.
• Twice-daily Chronocort® was initiated: 20 mg at 2300 h, 10 mg at 0700 h.
• Dose titration was based on clinical status and optimal hormonal ranges (17OHP 300-1200 ng/dL, normal androstenedione (males: 40-150, females: 30-200 ng/dL), with androstenedione prioritized.
• Chronocort® cortisol pharmacokinetic profile was the primary endpoint.
• Secondary endpoints included biomarkers of disease control.

Results:

• A total of 16 adults (8 females; age 29 ±13 years) with classic CAH (12 salt-wasting, 4 simple virilizing) participated.
• Conventional therapy varied (5 dexamethasone, 7 prednisone, 4 hydrocortisone).
• Chronocort® cortisol pharmacokinetic profile approximated physiological cortisol secretion.
• Ten patients required Chronocort® dose adjustments (decrease in 8, increase in 2; mean hydrocortisone equivalent dose conventional vs 6 months: 16.1 ± 6.4 vs 14.7 ± 6.4 mg/m2).
• Serial androstenedione levels were in the normal range in 8 (50%) of patients on conventional therapy compared with 12 (75%) on Chronocort® at 6 months.
• The majority of patients on Chronocort® achieved 17O HP levels within the normal range, rather than within the mildly elevated range currently used for management.
• At 6 months, Chronocort® resulted in lower 24-hr (P=0.02), morning (0700-1500; P=0.008), and afternoon (1500-2300; P=0.03) area-under-the-curve androstenedione compared with conventional therapy.
• No serious adverse events occurred.
• Common adverse events were headache, fatigue, early awakening, and anemia.
• Three patients had unexpected carpal tunnel syndrome, which resolved with wrist splints.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ5BA369FDE9DE4CED82CB6A7CD5BFD1BE/16521/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-ICE/EN2014&nonus=0#

ARMC5 Mutations in Macronodular Adrenal Hyperplasia with Cushing's Syndrome

Guillaume Assié, M.D., Ph.D., Rossella Libé, M.D., Stéphanie Espiard, M.D., Marthe Rizk-Rabin, Ph.D., Anne Guimier, M.D., Windy Luscap, M.Sc., Olivia Barreau, M.D., Lucile Lefèvre, M.Sc., Mathilde Sibony, M.D., Laurence Guignat, M.D., Stéphanie Rodriguez, M.Sc., Karine Perlemoine, B.S., Fernande René-Corail, B.S., Franck Letourneur, Ph.D., Bilal Trabulsi, M.D., Alix Poussier, M.D., Nathalie Chabbert-Buffet, M.D., Ph.D., Françoise Borson-Chazot, M.D., Ph.D., Lionel Groussin, M.D., Ph.D., Xavier Bertagna, M.D., Constantine A. Stratakis, M.D., Ph.D., Bruno Ragazzon, Ph.D., and Jérôme Bertherat, M.D., Ph.D.

N Engl J Med 2013; 369:2105-2114 November 28, 2013 DOI: 10.1056/NEJMoa1304603

BACKGROUND

Corticotropin-independent macronodular adrenal hyperplasia may be an incidental finding or it may be identified during evaluation for Cushing’s syndrome. Reports of familial cases and the involvement of both adrenal glands suggest a genetic origin of this condition.

METHODS

We genotyped blood and tumor DNA obtained from 33 patients with corticotropin-independent macronodular adrenal hyperplasia (12 men and 21 women who were 30 to 73 years of age), using single-nucleotide polymorphism arrays, microsatellite markers, and whole-genome and Sanger sequencing. The effects of armadillo repeat containing 5 (ARMC5) inactivation and overexpression were tested in cell-culture models.

RESULTS

The most frequent somatic chromosome alteration was loss of heterozygosity at 16p (in 8 of 33 patients for whom data were available [24%]). The most frequent mutation identified by means of whole-genome sequencing was in ARMC5, located at 16p11.2. ARMC5 mutations were detected in tumors obtained from 18 of 33 patients (55%). In all cases, both alleles of ARMC5 carried mutations: one germline and the other somatic. In 4 patients with a germline ARMC5 mutation, different nodules from the affected adrenals harbored different secondary ARMC5 alterations. Transcriptome-based classification of corticotropin-independent macronodular adrenal hyperplasia indicated that ARMC5 mutations influenced gene expression, since all cases with mutations clustered together. ARMC5 inactivation decreased steroidogenesis in vitro, and its overexpression altered cell survival.

CONCLUSIONS

Some cases of corticotropin-independent macronodular adrenal hyperplasia appear to be genetic, most often with inactivating mutations of ARMC5, a putative tumor-suppressor gene. Genetic testing for this condition, which often has a long and insidious prediagnostic course, might result in earlier identification and better management. (Funded by Agence Nationale de la Recherche and others.)

Supported in part by grants from Agence Nationale de la Recherche (ANR-10-Blan-1136), Corticomedullosurrénale Tumeur Endocrine Network (Programme Hospitalier de Recherche Clinique grant AOM95201), Assistance Publique–Hôpitaux de Paris (Clinical Research Center Grant Genhyper P061006), Institut National du Cancer (Recherche Translationelle 2009-RT-02), the Seventh Framework Program of the European Commission (F2-2010-259735), INSERM (Contrat d’Interface, to Dr. Assié), the Conny-Maeva Charitable Foundation, and the intramural program of the Eunice Kennedy Shriver National Institute of Child Health and Human Development.

Disclosure forms provided by the authors are available with the full text of this article at NEJM.org.

Drs. Assié, Libé, Espiard, Rizk-Rabin, Ragazzon, and Bertherat contributed equally to this article.

We thank Drs. J. Chelly and M. Delpech of the cell bank of Cochin Hospital and Dr. B. Terris of the tumor bank of Cochin Hospital for their help in sample collection; Dr. E. Clauser of the oncogenetic unit of Cochin Hospital for help in microsatellite analysis; Drs. J. Guibourdenche and E. Clauser of the hormone biology unit of Cochin Hospital for cortisol assays; Drs. F. Tissier and Pierre Colin for pathological analysis; Anne Audebourg for technical assistance; J. Metral and A. de Reynies of the Cartes d’Identité des Tumeurs program of Ligue Nationale contre le Cancer for help in genomics studies and fruitful discussions; Dr. P. Nietschke of the bioinformatics platforms of Paris Descartes University for helpful discussions; all the members of the Genomics and Signaling of Endocrine Tumors team and of the genomic platform of Cochin Institute for their help in these studies; and the patients and their families, as well as the physicians and staff involved in patient care, for their active participation.

SOURCE INFORMATION

From INSERM Unité 1016, Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut Cochin (G.A., R.L., S.E., M.R.-R., A.G., W.L., O.B., L.L., S.R., K.P., F.R.-C., F.L., L. Groussin, X.B., B.R., J.B.), Faculté de Médecine Paris Descartes, Université Paris Descartes, Sorbonne Paris Cité (G.A., S.E., A.G., O.B., L.L., M.S., K.P., F.R.-C., L. Groussin, X.B., J.B.), Department of Endocrinology, Referral Center for Rare Adrenal Diseases (G.A., R.L., O.B., L. Guignat, L. Groussin, X.B., J.B.), and Department of Pathology (M.S.), Assistance Publique–Hôpitaux de Paris, Hôpital Cochin, and Unit of Endocrinology, Department of Obstetrics and Gynecology, Hôpital Tenon (N.C.-B.) — all in Paris; Unit of Endocrinology, Centre Hospitalier du Centre Bretagne, Site de Kério, Noyal-Pontivy (B.T.), Unit of Endocrinology, Hôtel Dieu du Creusot, Le Creusot (A.P.), and Department of Endocrinology Lyon-Est, Groupement Hospitalier Est, Bron (F.B.-C.) — all in France; and the Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics and the Pediatric Endocrinology Inter-Institute Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD (C.A.S.).

Address reprint requests to Dr. Bertherat at Service des Maladies Endocriniennes et Métaboliques, Centre de Référence des Maladies Rares de la Surrénale, Hôpital Cochin, 27 rue du Faubourg St. Jacques, 75014 Paris, France, or at jerome.bertherat@cch.aphp.fr.

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Related articles

• Are you carrying adrenal Cushing’s syndrome without knowing it?(cushieblog.com)
• ARMC5 mutation identified in patients with macronodular adrenal hyperplasia(2minutemedicine.com)

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Perspectives on the management of adrenal insufficiency

Grossman A, Johannsson G, Quinkler M, Zelissen P.

Source

Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Headington, Oxford OX3 7EJ, UK.

Abstract

BACKGROUND:

Conventional glucocorticoid (GC) replacement for patients with adrenal insufficiency (AI) is inadequate. Patients with AI continue to have increased mortality and morbidity and compromised quality of life despite treatment and monitoring.

OBJECTIVES:

i) To review current management of AI and the unmet medical need based on literature and treatment experience and ii) to offer practical advice for managing AI in specific clinical situations.

METHODS:

The review considers the most urgent questions endocrinologists face in managing AI and presents generalised patient cases with suggested strategies for treatment.

RESULTS:

Optimisation and individualisation of GC replacement remain a challenge because available therapies do not mimic physiological cortisol patterns. While increased mortality and morbidity appear related to inadequate GC replacement, there are no objective measures to guide dose selection and optimisation. Physicians must rely on experience to recognise the clinical signs, which are not unique to AI, of inadequate treatment. The increased demand for corticosteroids during periods of stress can result in a life-threatening adrenal crisis (AC) in a patient with AI. Education is paramount for patients and their caregivers to anticipate, recognise and provide proper early treatment to prevent or reduce the occurrence of ACs.

CONCLUSIONS:

This review highlights and offers suggestions to address the challenges endocrinologists encounter in treating patients with AI. New preparations are being developed to better mimic normal physiological cortisol levels with convenient, once-daily dosing which may improve treatment outcomes.

PMID:

 

24031090

 

[PubMed - in process] 

PMCID:

 

PMC3805018

 [Available on 2013/12/1]

From http://www.mdlinx.com/endocrinology/newsl-article.cfm/4829245/ZZ4747461521296427210947/?news_id=2364&newsdt=110713&subspec_id=1509&utm_source=Focus-On&utm_medium=newsletter&utm_content=Top-New-Article&utm_campaign=article-section