scholarly journals RECOGNIZING ENDOGENOUS CUSHING’S SYNDROME AFTER THERAPY FOR SCLERODERMA

2016 ◽  
Vol 63 (4) ◽  
pp. 334-337
Author(s):  
Ana Valea ◽  
◽  
Dan Nicolae Paduraru ◽  
Adriana Elena Nica ◽  
Maria Iuliana Oprisor ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Tumor ◽  
White Blood Cells ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Serum Cortisol Level ◽  
Adrenal Crisis ◽  
Metabolic Disturbances ◽  
Threatening Condition ◽  
The Moment

Introduction. Scleroderma induces heterogeneous skin changes due to collagen anomalies, including facial. The recommended therapy is, among others, topic cortisol derived products, so an issue of diagnosis differential regarding the etiological type of Cushing’s syndrome (CS) is raised if a patient develops a suggestive phenotype. Case presentation. This is a 64-year female presenting (since the last few months) red face, high blood pressure, central obesity. She has a 6-year history of scleroderma, intermittently treated with local corticotherapy. On admission, she associated metabolic disturbances as lipid profile anomalies, high uric acid, insulin resistance and increased number of white blood cells (which were not connected with a relapse of scleroderma, neither to an inflammatory syndrome). Low baseline plasma ACTH and morning serum cortisol level (not high, yet detectable) with abnormal diurnal rhythm suggested an adrenal source of CS. A 3 cm right adrenal tumor was found on computer tomography and later removed through a classical intervention. Two weeks after, the patient voluntarily reduced her prednisone dose causing an adrenal crisis which required re-admission as an emergency. After discharge, daily oral adrenal replacement therapy is needed for the moment. Conclusions. Scleroderma, especially with skin involvement, and some of associated therapies may mask an endogenous CS as adrenal tumor derivate, thus delaying the adequate diagnosis and therapy. Patient’s education regarding a potential life threatening condition as chronic adrenal insufficiency is necessary since post-operatory recovery of adrenal function is expected within 6 to 24 months.

scholarly journals Efficacy of pasireotide in controlling severe hypercortisolism until cardiac transplantation

2017 ◽  
Vol 2017 ◽  
Author(s):  
Roberto Attanasio ◽  
Liana Cortesi ◽  
Daniela Gianola ◽  
Claudia Vettori ◽  
Fulvio Sileo ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Cushing’S Syndrome ◽  
Cardiac Transplantation ◽  
Serum Cortisol ◽  
Primary Treatment ◽  
Cushing's Syndrome ◽  
Line Treatment ◽  
First Line ◽  
First Line Treatment ◽  
Undergo Heart Transplantation

Summary Cushing’s syndrome is associated with increased morbidity and mortality. Although surgery is the first-line treatment, drugs can still play a role as an ancillary treatment to be employed while waiting for surgery, after unsuccessful operation or in patients unsuitable for surgery. We were asked to evaluate a 32-year-old male waiting for cardiac transplantation. Idiopathic hypokinetic cardiomyopathy had been diagnosed since 6 years. He was on treatment with multiple drugs, had a pacemaker, an implantable cardioverter and an external device for the support of systolic function. Physical examination showed severely impaired general status, signs of hypercortisolism and multiple vertebral compression fractures. We administered teriparatide, and the few evaluable parameters supported the diagnosis of ACTH-dependent hypercortisolism: serum cortisol was 24.2 µg/dL in the morning and 20.3 µg/dL after overnight 1 mg dexamethasone, urinary free cortisol (UFC) was 258 µg/24 h and ACTH 125 pg/mL. Pituitary CT was negative. Pasireotide 300 µg bid was administered and uptitrated to 600 µg bid. Treatment was well tolerated, achieving dramatic improvement of clinical picture with progressive normalization of serum cortisol and ACTH levels as well as UFC. After 4 months, the patient underwent successful heart transplantation. Many complications ensued and were overcome. Pituitary MRI was negative. On pasireotide 300 µg bid and prednisone 2.5 mg/day (as part of immunosuppressive therapy), morning serum cortisol and ACTH were 15.6 µg/dL and 54 pg/mL respectively, UFC was 37 µg/24 h, fasting glucose: 107 mg/dL and HbA1c: 6.5%. In conclusion, primary treatment with pasireotide achieved remission of hypercortisolism, thus allowing the patient to undergo heart transplantation. Learning points: Untreated Cushing’s syndrome is associated with ominous prognosis. First-line treatment is surgery (at pituitary or adrenal, according to disease localization). A few drugs are available to treat hypercortisolism. Pasireotide is a multi-ligand somatostatin analog approved for treatment of hypercortisolism. Primary treatment with pasireotide was effective in a patient with severe Cushing’s syndrome, allowing him to undergo heart transplantation.

CUSHING'S SYNDROME IN INFANCY

PEDIATRICS ◽  
1970 ◽  
Vol 46 (2) ◽  
pp. 217-229
Author(s):  
Michel G. Gilbert ◽  
William W. Cleveland
Keyword(s):  
Surgical Treatment ◽  
Cushing’S Syndrome ◽  
Adrenal Tumor ◽  
Cushing's Syndrome ◽  
Adrenal Steroids ◽  
Careful Attention ◽  
Intravenous Pyelogram ◽  
Reasonable Expectation ◽  
Surgical Cure ◽  
Postoperative Administration

Cushing's syndrome in infancy is usually due to adrenal tumor which is often malignant. Three cases are described in which the disorder was due to adenoma and surgical cure was achieved. The clinical picture is strongly suggestive if not diagnostic. Its hallmark is generalized obesity with typical facies; also present are hypertension and signs of virilization. Striae were not found in our patients. Increased excretion of both 17-hydroxysteroids and 17-ketosteroids is characteristic; this excretion was not suppressed by dexamethasone in two of these patients in whom it was tested. Intravenous pyelogram was distinctly abnormal in all cases and indicated the presence and location of the tumor. The diagnosis can be made efficiently and surgical treatment can be promptly instituted with reasonable expectation of cure. Careful attention must be paid to preoperative and postoperative administration of adrenal steroids.

Serum cortisol concentrations during low dose dexamethasone suppression test to screen for Cushing's syndrome

BMJ ◽  
1985 ◽  
Vol 290 (6462) ◽  
pp. 158-159
Author(s):  
L. Kennedy ◽  
D. Hadden ◽  
B. Atkinson ◽  
B Sheridan ◽  
H. Johnston
Keyword(s):  
Cushing’S Syndrome ◽  
Low Dose ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Dexamethasone Suppression Test ◽  
Suppression Test ◽  
Dexamethasone Suppression

ACTH-independent massive bilateral adrenal disease (AIMBAD): A subtype of Cushing's syndrome with major diagnostic and therapeutic implications

1994 ◽  
Vol 131 (1) ◽  
pp. 67-73 ◽  
Author(s):  
Steven A Lieberman ◽  
T Ross Eccleshall ◽  
David Feldman
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Glands ◽  
Medical Center ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Bilateral Adrenalectomy ◽  
Preoperative Treatment ◽  
Therapeutic Implications ◽  
Adrenal Imaging ◽  
Adrenal Disease

Lieberman SA, Eccleshall TR, Feldman D. ACTH-independent massive bilateral adrenal disease (AIMBAD): a subtype of Cushing's syndrome with major diagnostic and therapeutic implications. Eur J Endocrinol 1994:131:67–73. ISSN 0804–4643 A 49-year-old man with classic manifestations of Cushing's syndrome had undetectable levels of ACTH, lack of suppression of hypercortisolism with dexamethasone in doses of 2, 8, or 16 mg per day, bilaterally enlarged adrenal glands on MRI, and bilateral adrenal uptake of iodocholesterol. Preoperative treatment with ketoconazole lowered blood pressure and serum cortisol and produced symptoms of steroid withdrawal. Bilateral adrenalectomy revealed massively enlarged adrenal glands (left: 199 g, right: 93 g). Sequencing of the gene encoding the stimulatory G protein, Gsα, did not show either of two activating mutations previously reported in patients with McCune–Albright syndrome or acromegaly. Twenty-three previous cases of Cushing's syndrome due to ACTH-independent massive bilateral adrenal disease (AIMBAD) have been reported. AIMBAD may cause confusion in the differential diagnosis of Cushing's syndrome as endocrine testing suggests a unilateral, ACTHindependent process while adrenal imaging demonstrates bilateral abnormalities. Bilateral adrenalectomy is curative and appears to carry little risk of Nelson's syndrome. The pathogenesis of AIMBAD appears to be heterogeneous, as recent reports have demonstrated GIP-mediated hypercortisolism and familial AIMBAD. Transition from Cushing's disease to ACTH-independence is not supported by the available data. Future cases of AIMBAD should be investigated carefully to further elucidate the pathogenesis of this disorder. David Feldman, Division of Endocrinology, Dept. of Medicine, Room S-005, Stanford University Medical Center, Stanford, CA 94305, USA

Presence of a Gsα mutation in an adrenal tumor expressing LH/hCG receptors and clinically associated with Cushing's syndrome

2000 ◽  
Vol 14 (1) ◽  
pp. 50-54 ◽  
Author(s):  
M. J. M. Bugalho ◽  
X. Li ◽  
Ch. V. Rao ◽  
J. Soares ◽  
L. G. Sobrinho
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Tumor ◽  
Cushing's Syndrome ◽  
Gsα Mutation
Sign in / Sign up

Export Citation Format

Share Document