Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome.
Summary of "Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome."
Primary aldosteronism (PA), an autonomous aldosterone hypersecretion from adrenal adenoma and/or hyperplasia, and subclinical Cushing syndrome (SCS), a mild but autonomous cortisol hypersecretion from adrenal adenoma without signs or symptoms of Cuhing's syndrome, are now well-recognized clinical entities of adrenal incidentaloma. However, the clinicopathological features of PA associated with SCS (PA/SCS) remain unknown. The present study was undertaken to study the prevalence of PA/SCS among PA patients diagnosed at our institute, and characterize their clinicopathlogical features. The prevalence of PA/SCS was 8 of 38 PA patients (21%) studied. These 8 PA/SCS patients were significantly older and had larger tumor, higher serum potassium levels, lower basal plasma levels of aldosterone, ACTH and DHEA-S as well as lower response of aldosterone after ACTH stimulation than those in 12 patients with aldosterone-producing adenoma without hypercortisolism. All 8 PA/SCS patients showed unilateral uptake by adrenal scintigraphy at the ipsilateral side, whereas the laterality of aldosterone hypersecretion as determined by adrenal venous sampling varied from ipsilateral (3), contralateral (2), and bilateral side (2). 6 PA/SCS patinets who underwent adrenalectomy required hydrocortisone replacement postoperatively. Histopathological analysis of the resected adrenal tumors from 5 PA/SCS patients revealed a single adenoma in 3, and double adenomas in 2, with varying degrees of positive immunoreactivities for steroidgenic enzymes (3β-HSD, P450(C17)) by immunohistochemical study as well as CYP11B2 mRNA expression as measured by real-time RT-PCR. In conclusion, PA/SCS consists of a variety of adrenal pathologies so that therapeutic approach differs depending on the disease subtype.
Affiliation
Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo 113-8519, Japan.
Journal Details
This article was published in the following journal.
Name: Endocrine journal
ISSN: 1348-4540
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21521926
- DOI: http://dx.doi.org/
Medical and Biotech [MESH] Definitions
Glomerulosclerosis, Focal Segmental
A clinicopathological syndrome or diagnostic term for a type of glomerular injury that has multiple causes, primary or secondary. Clinical features include PROTEINURIA, reduced GLOMERULAR FILTRATION RATE, and EDEMA. Kidney biopsy initially indicates focal segmental glomerular consolidation (hyalinosis) or scarring which can progress to globally sclerotic glomeruli leading to eventual KIDNEY FAILURE.
Skin Diseases, Papulosquamous
A group of dermatoses with distinct morphologic features. The primary lesion is most commonly a papule, usually erythematous, with a variable degree of scaling on the surface. Plaques form through the coalescing of primary lesions.
Cushing Syndrome
A condition caused by prolonged exposure to excess levels of cortisol (HYDROCORTISONE) or other GLUCOCORTICOIDS from endogenous or exogenous sources. It is characterized by upper body OBESITY; OSTEOPOROSIS; HYPERTENSION; DIABETES MELLITUS; HIRSUTISM; AMENORRHEA; and excess body fluid. Endogenous Cushing syndrome or spontaneous hypercortisolism is divided into two groups, those due to an excess of ADRENOCORTICOTROPIN and those that are ACTH-independent.
Petrosal Sinus Sampling
Sampling of blood levels of the adrenocorticotropic hormone (ACTH) by withdrawal of blood from the inferior petrosal sinus. The inferior petrosal sinus arises from the cavernous sinus and runs to the internal jugular vein. Sampling of blood at this level is a valuable tool in the differential diagnosis of Cushing disease, Cushing syndrome, and other adrenocortical diseases.
Encephalitis, Japanese
A mosquito-borne encephalitis caused by the Japanese B encephalitis virus (ENCEPHALITIS VIRUS, JAPANESE) occurring throughout Eastern Asia and Australia. The majority of infections occur in children and are subclinical or have features limited to transient fever and gastrointestinal symptoms. Inflammation of the brain, spinal cord, and meninges may occur and lead to transient or permanent neurologic deficits (including a POLIOMYELITIS-like presentation); SEIZURES; COMA; and death. (From Adams et al., Principles of Neurology, 6th ed, p751; Lancet 1998 Apr 11;351(9109):1094-7)
PubMed Articles
We report a first case of lumbar herniation of a kidney after laparoscopic adrenalectomy in a patient with Cushing's syndrome. A 59-year-old woman underwent separate laparoscopic adrenalectomies for r...
Primary aldosteronism: from bench to bedside.
Primary aldosteronism is now thought to be the commonest potentially curable and specifically treatable form of hypertension. The detection of primary aldosteronism is of utmost importance not only be...
Endocrine hypertension represents more than half of the causes of secondary hypertension. This entity encompasses several diseases including primary aldosteronism, paraganglioma/pheochromocytoma and C...
BACKGROUND: Patients with primary aldosteronism experience greater left ventricular hypertrophy and a higher frequency of cardiovascular events than do essential hypertensive patients with comparable...
The diagnosis of Cushing's syndrome.
Spontaneous Cushing's syndrome is well known but unusual clinical disorder. Many of the clinical features (central weight gain, glucose intolerance, hypertension, muscle weakness) are seen in other co...
Clinical Trials
Background: The most common pharmacologic test for diagnosis of primary aldosteronism (PA) is administration of captopril to examine whether abnormal aldosterone to plasma rennin activity...
A Study to Assess SOM230 in Patients With Pituitary Cushing's Disease
The study treatment period is 15 days in length and includes patients with pituitary Cushing's disease who are candidates for surgical intervention as well as and patients who have recurr...
Study of Hypercortisolism in Cushing's Syndrome and Stress-Induced Pseudo-Cushing's Syndrome
OBJECTIVES: I. Determine whether Cushing's syndrome and stress-induced pseudo-Cushing's syndrome can be differentiated by evaluating endogenous corticotropin-releasing hormone activity.
Extension Study to Assess the Safety and Efficacy of Pasireotide in Patients With Cushing's Disease
Cushing's disease is a rare serious condition that is caused by an adrenocorticotropic hormone (ACTH) secreting pituitary adenoma. This study will assess the long-term safety and efficacy...
Study of Depression, Peptides, and Steroids in Cushing's Syndrome
OBJECTIVES: I. Study the relationship between dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis and disorders of mood, vegetative function, and cognition in patients with Cus...