Cushing's syndrome of nonpituitary causes

Carroll, Ty B.; Findling, James W.

doi:10.1097/med.0b013e32832d8950

Cited by 11 publications

(9 citation statements)

References 62 publications

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“…Symptoms of Cushing's syndrome include osteoporosis, muscle weakness and easy fatigability, hypertension, emotional lability, truncal obesity, and in some patients, diabetes. Patients with iatrogenic Cushing's syndrome secondary to steroids generally recover to normal function after steroids are withdrawn, although some patients may have persistent symptoms [20]. Patients with adrenal-based hypercortisolism may have increased serum and urine cortisol levels [67].…”

Section: Diagnosis and Treatment Of Diseases Associated With Impairedmentioning

confidence: 99%

Short-term and Long-term Orthopaedic Issues in Patients With Fragility Fractures

Bukata

Kates

O’Keefe

2011

Clinical Orthopaedics &Amp; Related Research

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Background Patients with impaired bone quality who suffer a fragility fracture face substantial challenges in both their short-and long-term care. In addition to poor bone quality, many of these patients have multiple medical comorbidities that alter their surgical risk and affect their ultimate functional recovery. Some medical issues can contribute to the altered bone quality and must be addressed to prevent future fractures. Questions/purposes This review summarizes the modifications in perioperative management and fracture fixation in patients with common fragility fractures who have impaired bone quality. It also summarizes the postoperative diagnosis and treatment of secondary causes of impaired bone quality in these patients. Methods We performed a PubMed search, and literature published after 2000 was prioritized, with the exception of benchmark clinical trial studies published before 2000. Results Patients with altered bone quality require rapid perioperative management of multiple medical comorbidities. Implant selection in patients with poor quality bone should permit early weightbearing, and constructs should maximize surface area contact with the remaining bone. Long-term diagnosis and treatment of other disease states contributing to poor bone quality (vitamin D deficiency/ insufficiency, hypothyroidism, hyperthyroidism, hyperparathyroidism, Cushing's disease, and hypogonadism) must occur to minimize the chances of future fractures. Conclusions Recognition of patients with impaired bone quality and proper treatment of their special needs in both the short and long term are essential for their best opportunity for maximal functional recovery and prevention of future fractures.

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Section: Diagnosis and Treatment Of Diseases Associated With Impairedmentioning

confidence: 99%

Short-term and Long-term Orthopaedic Issues in Patients With Fragility Fractures

Bukata

Kates

O’Keefe

2011

Clinical Orthopaedics &Amp; Related Research

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“…Interpretation of the test results is dependent on the assay method used and the expertise of an endocrine specialist is often necessary to clarify the caveats of each test 1–4 8. After biochemical confirmation of hypercortisolism, further laboratory testing is required to determine the aetiology of Cushing's syndrome (table 1).…”

Section: Clinical and Biochemical Aspects Of Cushing's Syndromementioning

confidence: 99%

“…After biochemical confirmation of hypercortisolism, further laboratory testing is required to determine the aetiology of Cushing's syndrome (table 1). Corticotroph response is assessed by measuring plasma ACTH levels 2–4 8. A plasma ACTH concentration of less than 5 pg/mL (1.1 pmol/L) is in keeping with ACTH-independent Cushing's syndrome, while a concentration of more than 15 pg/mL (3.3 pmol/L) is suggestive of ACTH-dependent Cushing's syndrome 2–4 8.…”

Section: Clinical and Biochemical Aspects Of Cushing's Syndromementioning

confidence: 99%

“…Corticotroph response is assessed by measuring plasma ACTH levels 2–4 8. A plasma ACTH concentration of less than 5 pg/mL (1.1 pmol/L) is in keeping with ACTH-independent Cushing's syndrome, while a concentration of more than 15 pg/mL (3.3 pmol/L) is suggestive of ACTH-dependent Cushing's syndrome 2–4 8. Borderline plasma ACTH values between 5 pg/mL and 15 pg/mL (1.1 pmol/L and 3.3 pmol/L) are equivocal, and a CRH stimulation test may be helpful in differentiating Cushing's disease from other aetiologies 2–4.…”

Section: Clinical and Biochemical Aspects Of Cushing's Syndromementioning

confidence: 99%

“…Rare neuroendocrine tumours (<1%) can also present with Cushing's syndrome when they secrete corticotropin-releasing hormone (CRH) 3 10. In contrast with the diverse aetiologies of ACTH-dependent Cushing's syndrome, ACTH-independent cases are attributed to primary cortisol-producing adrenocortical lesions (20%), which may arise sporadically or in the setting of rare genetic syndromes 8 11–13…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Republished: Clinicopathological correlates of adrenal Cushing's syndrome

Duan

Hernandez

Mete

2015

Postgraduate Medical Journal

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Endogenous Cushing's syndrome is a rare endocrine disorder that incurs significant cardiovascular morbidity and mortality, due to glucocorticoid excess. It comprises adrenal (20%) and non-adrenal (80%) aetiologies. While the majority of cases are attributed to pituitary or ectopic corticotropin (ACTH) overproduction, primary cortisol-producing adrenal cortical lesions are increasingly recognised in the pathophysiology of Cushing's syndrome. Our understanding of this disease has progressed substantially over the past decade. Recently, important mechanisms underlying the pathogenesis of adrenal hypercortisolism have been elucidated with the discovery of mutations in cyclic AMP signalling (PRKACA, PRKAR1A, GNAS, PDE11A, PDE8B), armadillo repeat containing 5 gene (ARMC5) a putative tumour suppressor gene, aberrant G-protein-coupled receptors, and intra-adrenal secretion of ACTH. Accurate subtyping of Cushing's syndrome is crucial for treatment decision-making and requires a complete integration of clinical, biochemical, imaging and pathology findings. Pathological correlates in the adrenal glands include hyperplasia, adenoma and carcinoma. While the most common presentation is diffuse adrenocortical hyperplasia secondary to excess ACTH production, this entity is usually treated with pituitary or ectopic tumour resection. Therefore, when confronted with adrenalectomy specimens in the setting of Cushing's syndrome, surgical pathologists are most commonly exposed to adrenocortical adenomas, carcinomas and primary macronodular or micronodular hyperplasia. This review provides an update on the rapidly evolving knowledge of adrenal Cushing's syndrome and discusses the clinicopathological correlations of this important disease.

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Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing's Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

Raff

Sharma

Nieman

2014

Comprehensive Physiology

121

105

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The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing’s syndrome) or exogenous glucocorticoid therapy (iatrogenic Cushing’s syndrome). Endogenous Cushing’s syndrome can be subdivided into ACTH-dependent and ACTH-independent, the latter of which is usually due to autonomous adrenal overproduction. The former can be due to a pituitary corticotroph tumor (usually benign) or ectopic ACTH production from tumors outside the pituitary; both of these tumor types overexpress the proopiomelanocortin gene. The converse of Cushing’s syndrome is the lack of normal cortisol secretion and is usually due to adrenal destruction (primary adrenal insufficiency) or hypopituitarism (secondary adrenal insufficiency). Secondary adrenal insufficiency can also result from a rapid discontinuation of long-term, pharmacological glucocorticoid therapy because of HPA axis suppression and adrenal atrophy. Finally, mutations in the steroidogenic enzymes of the adrenal cortex can lead to congenital adrenal hyperplasia and an increase in precursor steroids, particularly androgens. When present in utero, this can lead to masculinization of a female fetus. An understanding of the dynamics of the HPA axis is necessary to master the diagnosis and differential diagnosis of pituitary-adrenal diseases. Furthermore, understanding the pathophysiology of the HPA axis gives great insight into its normal control.

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Cushing's syndrome of nonpituitary causes

Abstract: The diagnosis of patients with less profound cortisol excess has increased the prevalence of Cushing's syndrome and made nonpituitary causes more common. As a result, clinicians must be cognizant of such patients and pursue the diagnosis when appropriate.

Cited by 11 publications

References 62 publications

Short-term and Long-term Orthopaedic Issues in Patients With Fragility Fractures

Short-term and Long-term Orthopaedic Issues in Patients With Fragility Fractures

Republished: Clinicopathological correlates of adrenal Cushing's syndrome

Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing's Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

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