Continuous Etomidate for the Management of Cushing’s Syndrome Complicated by Pulmonary Nocardiosis

2021 ◽  
pp. 089719002110174
Author(s):  
William Blake Hays ◽  
Quinn Czosnowski
Keyword(s):  
Cushing’S Syndrome ◽  
Opportunistic Infections ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Level Of Care ◽  
Pituitary Microadenoma ◽  
Oral Medications ◽  
Advanced Airway ◽  
Slow Onset ◽  
Serum Acth

Severe Cushing’s syndrome is associated with significant complications including opportunistic infections. In the setting of infection, urgent reduction of circulating cortisol is warranted and commonly used oral medications have a slow onset of effect. Here we describe a 29-years old female who presented to an outside hospital with symptoms of untreated Cushing’s syndrome on ketoconazole. Following identification of a pulmonary Nocardia spp infection, she was transferred for a higher level of care. Her initial serum ACTH and cortisol levels after transfer were 106pg/mL and 53.7mCg/dL respectively. Given the severity of her nocardiosis and lack of response to ketoconazole, the patient was placed in the ICU and started on a continuous etomidate infusion. Her serum cortisol rapidly decreased to 5.7mCg/dL. She was transitioned to metyarapone and the etomidate infusion was weaned to off. The etomidate infusion was well tolerated without adverse effects or need for an advanced airway. Following discontinuation of etomidate, she was found to have a pituitary microadenoma that was resected. The patient was discharged from the hospital on antibiotics for the management of her Nocardia spp infection. In patients with severe Cushing’s syndrome, continuous etomidate may be safe and effective for patients who are unresponsive to other therapies.

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.

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

scholarly journals Pheochromocytoma combined with unusual form of Cushing's syndrome and pituitary microadenoma

1999 ◽  
pp. 653-654 ◽  
Author(s):  
WK Khalil ◽  
J Vadasz ◽  
E Rigo ◽  
L Kardos ◽  
L Tiszlavicz ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Pituitary Microadenoma ◽  
Unusual Form

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

scholarly journals Perioperative Endocrine Therapy for Patients with Cushing's Syndrome Undergoing Retroperitoneal Laparoscopic Adrenalectomy

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Xiaobo Cui ◽  
Lu Yang ◽  
Jianwei Li ◽  
Siyuan Bu ◽  
Qiang Wei ◽  
...  
Keyword(s):  
Endocrine Therapy ◽  
Cushing’S Syndrome ◽  
Laparoscopic Adrenalectomy ◽  
Clinical Manifestations ◽  
Perioperative Period ◽  
Serum Cortisol ◽  
Serum Levels ◽  
Cushing's Syndrome ◽  
Adrenocortical Adenoma ◽  
Hospital Days

Objectives. To investigate the efficacy and safety of perioperative endocrine therapy (PET) for patients with Cushing’s syndrome (CS) undergoing retroperitoneal laparoscopic adrenalectomy (RLA).Methods. The novel, simplified PET modality of 82 patients who underwent RLA procedures for CS were studied. Clinical manifestations were observed for all patients on days 1 and 5 postoperatively, and clinical data, such as blood pressure (BP), levels of serum cortisol, adrenocorticotropin (ACTH), blood glucose, and electrolytes, were acquired and analyzed.Results. Supraphysiological doses of glucocorticoid were administered during the perioperative period, and the dosage was reduced gradually. In all 82 cases, the RLAs were performed successfully without any perioperative complication, such as steroid withdrawal symptoms. The patient’s symptoms and signs were improved quickly and safely during the hospital days. The serum cortisol and potassium levels were rather stable on days 1 and 5 postoperatively, and most were within the normal range. The clinical manifestations, serum levels of cortisol, ACTH, and potassium in most patients restored to normal gradually after several months (mean, 6.7 ± 1.2 months), except for one patient undergoing bilateral adrenalectomy.Conclusions. This perioperative endocrine therapy for patients with Cushing’s syndrome (mainly for adrenocortical adenoma) undergoing retro-laparoscopic adrenalectomy is both effective and safe.

scholarly journals Primary thyroid disorders in endogenous Cushing's syndrome

2002 ◽  
pp. 305-311 ◽  
Author(s):  
H Niepomniszcze ◽  
F Pitoia ◽  
SB Katz ◽  
R Chervin ◽  
OD Bruno
Keyword(s):  
Cushing’S Syndrome ◽  
Dynamic Testing ◽  
Imaging Techniques ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Control Group ◽  
Thyroid Disorders ◽  
Thyroid Antibodies ◽  
Autoimmune Thyroid ◽  
Free Cortisol

OBJECTIVE: To study the prevalence of primary thyroid disorders in patients who underwent endogenous hypercortisolism. DESIGN: Retrospective evaluation of 59 patients with Cushing's syndrome (CS) who had, at least, a record of thyroid palpation by expert endocrinologists and basal measurements of TSH by second generation assays. When available, tri-iodothyronine and thyroxine serum levels, TRH-TSH tests and anti-thyroid antibodies were also analyzed. There were two age- and gender-matched control groups. The 'goiter control group' comprised 118 healthy subjects who underwent thyroid palpation. The 'antibody control group' was composed of 40 individuals who attended the blood bank of our hospital. Antibodies against thyroperoxidase and measurements of TSH were analyzed in their blood samples. METHODS: Available files of 83 CS patients admitted to our endocrine unit from 1985 to 1998 were examined. Fifty-nine patients (52 women and 7 men) with a mean age of 36.2 years (range 14-61 years) met the above requirements. Diagnosis of hypercortisolism had been established by a standard 1-mg overnight dexamethasone suppression test and urinary free cortisol (UFC). Etiological diagnosis involved dynamic testing, measurements of ACTH levels and imaging techniques. After treatment, all but one of the patients were cured or controlled of their hypercortisolism. This was established by the finding of subnormal serum cortisol concentrations and/or subnormal 24-h UFC levels. Primary thyroid disorders were defined by the presence of one or more of the following diagnostic criteria: (i) goiter, (ii) positive anti-thyroid antibodies and/or (iii) primary thyroid function abnormalities. RESULTS: Eighteen (30.5%) patients had goiter (diffuse in 78% and nodular in 22%), 14 (23.7%) had primary subclinical hypothyroidism and 5 (8.4%) had hyperthyroidism. In 41 patients evaluated for antithyroid antibodies, it was found that 23 (56.1%) had positive titers. In a group of patients in which thyroid autoantibodies were measured both before and after resolution of hypercortisolism, prevalences of positive titers were 26.7% and 86.7% respectively (P=0.001). The overall frequency of primary thyroid abnormalities in our patients with Cushing's syndrome was 55.9%. CONCLUSIONS: Patients with endogenous Cushing's syndrome exhibit a remarkably high prevalence of primary thyroid disease. Resolution of hypercortisolism seems to trigger the development of autoimmune thyroid disorders in presumably predisposed subjects.

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 SUN-LB34 A Case of BMAH With Aberrant Vasopressin Adrenal Receptors

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Faheem Seedat ◽  
Nazeer Ahmed Mohamed ◽  
Nereshni Lutchman
Keyword(s):  
Cushing’S Syndrome ◽  
Salivary Cortisol ◽  
Incidental Finding ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Screening Tests ◽  
Pathological Examination ◽  
Unilateral Adrenalectomy ◽  
Urine Free Cortisol ◽  
Free Cortisol

Abstract We report on a 42 - year old woman was referred following an incidental finding of bilateral macronodular (nodules > 1cm) adrenal glands after a computed tomography scan for investigation of an unrelated urological problem. Clear features of Cushing’s syndrome were elicited on initial clincal evaluation and screening tests confirmed the diagnosis biochemically: midnight salivary cortisol 15.4nmol/L (0.2 - 3nmol/L), 24 hour urine free cortisol > 662.4nmol/L (8.3 - 118.7nmol/L per 24 hours) and an 8am serum cortisol measured 951nmol/L following 1mg dexamethasone suppression test (< 50nm/L). A serum adrenocorticotropic hormone (ACTH) measured 0.3pmol/L (1.6 - 13.9pmol/L) suggesting, in light of the CT findings, an adrenal source of the elevated cortisol. Screening for aberrant adrenal receptors to catecholamines, Gastric inhibitory peptide, ACTH, vasopressin and Gonadotropin releasing hormone was performed. A positive biochemical response to ACTH and vasopressin was noted as measured serum cortisol increased to 1600pmol/L (upper limit of the assay). Following a unilateral adrenalectomy, were pathological examination of the respected adrenal gland confirmed adrenal hyperplasia, the patient had both clinical and biochemical resolution of hypercortisolemia. 2 weeks following surgery her midnight salivary cortisol measured 2.1nmol/L (0.2 - 3nmol/L) and 24 hour urine free cortisol 218nmol/L (8.3 - 118.7nmol/L per 24 hours). Her 8am ACTH also increased to 2.9pmol/L (1.6 - 13.9pmol/L). Biochemical screening of her sister showed no evidence of Cushing’s syndrome. Bilateral macronodualr hyperplasia (BMAH) represents a rare cause of Cushing’s syndrome may be diagnosed incidentally and is often associated with an insidious onset. We use this case to highlight the complexities of provocative testing for aberrant adrenal receptors which occur with BMAH and the utilization of unilateral adrenalectomy to achieve clinical and biochemical remission of Cushing’s syndrome thus, removing the need for post - operative steroid replacement following bilateral adrenalectomy.

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