Tonight! Interview with Miriam K (Meeks089), Pituitary Success Story

Miriam writes in her bio:

“I suffered for eight long years with Cushings disease . I had surgery on August 1 , 2012 , I look like a different person , and act like a different person. I would love to share my journy . One that was an emotional roller coaster .

It was a long hellish journey .However I would not trade it for anything else in the world.

Although I suffered immensly, Cushings has made me who I am today. I have become strong from this disease. Although I suffered many symptoms, the emotional ones were by far the worst.

I would love to be interveiwed because I vowed when I was ill to help people when I got better.

I want to give people hope .”

MaryONote:  Miriam will be interviewed on BlogTalkRadio podcasts December 4, 2013 at 6:00PM Eastern.

If you want to ask Miriam a question, please use the call-in number: (646) 200-0162

Archives will be available at Cushings-Help on BlogTalkRadio a few hours after the conclusion of this interview.

Are you carrying adrenal Cushing’s syndrome without knowing it?

Genetic research that will be published tomorrow in the New England Journal of Medicine suggests to Dr. André Lacroix, professor at the University of Montreal, that clinicians’ understanding and treatment of a form of Cushing’s syndrome affecting both adrenal glands will be fundamentally changed, and that moreover, it might be appropriate to begin screening for the genetic mutations that cause this form of the disease.

“Screening family members of bilateral adrenal Cushing’s syndrome patients with  may identify affected silent carriers,” Lacroix said in an editorial in the Journal. “The development of drugs that interrupt the defective genetic chemical link that causes the syndrome could, if confirmed to be effective in people, provide individualized specific therapies for hypercortisolism, eliminate the current practice of removing both , and possibly prevent disease progression in genetically affected .”

Adrenal glands sit above the kidneys are mainly responsible for releasing cortisol, a stress hormone. Hypercortiolism means a high level of the adrenal hormone cortisol, which causes many symptoms including weight gain, , diabetes, osteoporosis, concentration deficit and increased cardiovascular deaths.

Cushing’s syndrome can be caused by corticosteroid use (such as for asthma or arthritis), a tumor on the adrenal glands, or a  that releases too much ACTH. The pituitary gland sits under the brain and releases various hormones that regulate our bodies’ mechanisms.

Jérôme Bertherat is a researcher at Cochin Hospital in Paris. In the study he published today, he showed that 55% of Cushing’s Syndrome patients with bilaterally very enlarged adrenal glands have mutations in a gene that predisposes to the development of adrenal tumours. This means that bilateral adrenal Cushing’s is much more hereditary than previously thought. The new knowledge will also enable clinicians to undertake genetic screening. Hervé Lefebvre is a researcher at the University Hospital in Rouen, France. His research shows that the adrenal glands from the same type of patients with two large adrenal glands can produce ACTH, which is normally produced by the pituitary gland. Hormone receptors are the chemical link that cause a cell to behave differently when a hormone is present. Several misplaced hormone receptors cause the ACTH to be produced in the enlarged benign adrenal tissue. Knowing this means that researchers might be able to develop drugs that interrupt the receptors for these hormones and possibly even prevent the benign tissue from developing in the first place.

 Explore further: Scientists discover a curable cause for some cases of high blood pressure

More information: André Lacroix, M.D., Heredity and Cortisol Regulation in Bilateral Macronodular Adrenal Hyperplasia, New England Journal of Medicine 369;22, November 28, 2013

Estelle Louiset, Ph.D., Céline Duparc, Ph.D., Jacques Young, M.D., Ph.D., Sylvie Renouf, Ph.D., Milène Tetsi Nomigni, M.Sc., Isabelle Boutelet, Ph.D., Rossella Libé, M.D., Zakariae Bram, M.Sc., Lionel Groussin, M.D., Ph.D., Philippe Caron, M.D., Antoine Tabarin, M.D., Ph.D., Fabienne Grunenberger, M.D., Sophie Christin-Maitre, M.D., Ph.D., Xavier Bertagna, M.D., Ph.D., Jean-Marc Kuhn, M.D., Youssef Anouar, Ph.D., Jérôme Bertherat, M.D., Ph.D., and Hervé Lefebvre, M.D., Ph.D., Intraadrenal Corticotropin in Bilateral Macronodular Adrenal Hyperplasia, New England Journal of Medicine 369;22, November 28, 2013

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., ARMC5 Mutations in Macronodular Adrenal Hyperplasia with Cushing’s Syndrome, New England Journal of Medicine 369;22, November 28, 2013

The Current Role Of Transcranial Surgery In The Management Of Pituitary Adenomas

Pituitary. 2013 Dec;16(4):419-34. doi: 10.1007/s11102-012-0439-z.

The current role of transcranial surgery in the management of pituitary adenomas.

Source

Section of Neurosurgery, Department of Neurological Sciences, Christian Medical College, Vellore, 632004, Tamil Nadu, India.

Abstract

The aim of this study was to determine the factors influencing the use of a transcranial (TC) approach in pituitary adenomas and suggest a decision-making tree for the surgical strategy.

The data for 23 (4.6 %) patients who underwent TC surgery from amongst 494 pituitary adenomas were retrospectively analyzed. Eight factors on magnetic resonance imaging (MRI) that could predict a difficult transsphenoidal (TS) surgery were noted.

Adverse findings at TS surgery leading to a 2nd stage TC surgery were documented. Eighteen of the 23 cases were giant adenomas. Thirteen patients underwent TC surgery alone or as an initial approach when combined with TS while 10 underwent 2nd stage TC surgery following a TS approach. Most cases in the first group had 3 or more radiological factors in combination with a small sella. The 2nd group had higher sellar tumor volumes and fewer unfavourable radiological factors that led to the initial use of the TS approach.

A hard, fibrous consistency or a significant residue obscured from the surgeon’s view, and difficulty in hemostasis were additional factors prompting the use of a TC approach. Tumor excision ≥90 % could be achieved in 13 cases (56.5 %). Post-operative RT was administered in 12 patients. There were 2 deaths (8.7 %) and the major morbidity rate was 43 %. Despite advances in endoscopic surgery the TC approach may be required in 5 % of cases.

A study of the preoperative MRI for factors that predict difficulty with the TS approach might encourage the surgeon to consider a TC surgery either as an initial approach or combined with a TS surgery.

PMID:
23076713
[PubMed – in process]

Is Diabetes in Cushing’s Syndrome a Consequence of Hypercortisolism?

Eur J Endocrinol. 2013 Nov 19. [Epub ahead of print]

Is Diabetes in Cushing syndrome only a consequence of hypercortisolism?

Source

C Giordano, Dipartimento di Medicina Interna e Specialistica (Di.Bi.Mi.S) Sezione di Endocrinologia e Malattie del Metabolismo, University of Palermo, Palermo, Italy.

Abstract

OBJECTIVE:

Diabetes mellitus (DM) is one of the most frequent complications of Cushing syndrome (CS). Aim of the study was to define the changes in insulin sensitivity and/or secretion in relation to glucose tolerance categories in newly diagnosed CS patients.

DESIGN:

Cross-sectional study on 140 patients with CS.

METHODS:

113 women (80 with pituitary disease and 33 with adrenal disease, aged 41.7±15.7 yr) and 27 men (19 with pituitary disease and 8 with adrenal disease, aged 38.1±20.01 yr) at diagnosis were divided according to glucose tolerance into normal glucose tolerance (CS/NGT), impaired fasting glucose and/or impaired glucose tolerance (CS/prediabetes) and diabetes (CS/DM).

RESULTS:

71 patients belonged to CS/NGT (49.3%), 26 (18.5%) to CS/prediabetes and 43 (30.8%) to CS/DM. Significant increasing trends in the prevalence of family history of diabetes (p<0.001), metabolic syndrome (p<0.001), age (p<0.001) and waist circumference (p=0.043) and decreasing trends in HOMAβ (p<0.001)and Oral Dispositional Index (DIo) (p<0.002) were observed among the groups. No significant trend in fasting insulin, AUC INS, ISI-Matsuda and VAI was detected.

CONCLUSIONS:

Impairment of glucose tolerance is characterized by the inability of β-cells to adequately compensate insulin resistance through increased insulin secretion. Age, genetic predisposition and lifestyle, in combination with duration and degree of hypercortisolism, strongly contribute to the impairment of glucose tolerance in the natural history of CS. A careful phenotypic evaluation of glucose tolerance defects in patients with CS proves useful for the identification of patients at high risk for metabolic complications.

PMID:
24255133
[PubMed – as supplied by publisher]

Interview with Miriam K (Meeks089), Pituitary Success Story

Miriam writes in her bio:

“I suffered for eight long years with Cushings disease . I had surgery on August 1 , 2012 , I look like a different person , and act like a different person. I would love to share my journy . One that was an emotional roller coaster .

It was a long hellish journey .However I would not trade it for anything else in the world.

Although I suffered immensly, Cushings has made me who I am today. I have become strong from this disease. Although I suffered many symptoms, the emotional ones were by far the worst.

I would love to be interveiwed because I vowed when I was ill to help people when I got better.

I want to give people hope .”

MaryONote:  Miriam will be interviewed on BlogTalkRadio podcasts December 4, 2013.