In adolescents and children with PPNAD, the disease frequently presents with periodic CS in which normal cortisol production is interrupted by days or weeks of hypercortisolism. [rarediseases.info.nih.gov]

Discussion PPNAD usually presents along with CNC while 12% of the cases manifest as a sole presentation, known as iPPNAD [ 9 ]. [karger.com]

Her lung and heart auscultation were normal and she presented heart rate of 78 beats/min and arterial blood pressure of 130 x 80 mmHg. Her abdomen was painless, without any palpable masses, and presented purple stretch marks. [scielo.br]

Clinical description Although the majority of cases are diagnosed in the 2nd and 3rd decades of life, a substantial proportion of patients present during early childhood (2-3 years). [orpha.net]

• Buffalo Hump

  All of the cases had moon facies, buffalo humps, plethora, violaceous striaes appeared at the abdomen and medial thighs, and also hairiness, acne, and other typical symptoms of Cushing syndrome. [124.205.33.103]

  On clinical examination, skin atrophy, buffalo hump, moon facies, proximal muscle weakness and elevated blood pressure (145/90 mmHg) were documented. [jmedicalcasereports.biomedcentral.com]

  Physical examination revealed moon face with an excess of acne, buffalo hump, and abdominal obesity. [omicsonline.org]

• Collapse

  No evidence of cortical collapse. [edmcasereports.com]

• Easy Bruising

  Easy bruising, facial plethora and proximal myopathy are the most sensitive features of Cushing’s syndrome. [clinicaladvisor.com]

  Cushing’s syndrome in Carney complex is always due to primary pigmented nodular adrenocortical disease (PPNAD), and symptoms may be a combination of weight gain, high blood pressure, diabetes, and easy bruising, caused by the overproduction of the hormone [cancer.net]

• Thin Skin

  On examination, she had a significant cushingoid appearance with a round face, thin skin, supraclavicular fat pad, and proximal muscle weakness. There were purplish striae on her lower abdomen and numerous acne lesions on her face. [karger.com]

• Round Face

  Her round face, easily bruised skin, and proximal myopathy all improved significantly. The dose of antihypertensive drugs was reduced by 50%. [karger.com]

Further workup was consistent with primary pigmented nodular adrenal disease (PPNAD) without evidence of Carney Complex. A bilateral adrenalectomy was performed and the patient maintained on oral adrenal hormone replacement therapy. [digitalcommons.hsc.unt.edu]

• ACTH Decreased

  The serum and urine free cortisol excretion of PPNAD increased, cortisol secretion without circadian rhythms, serum ACTH decreased or could not be measured. [124.205.33.103]

Management and treatment Bilateral adrenalectomy is the most common treatment for CS due to PPNAD followed by life-long cortisol and mineralocorticoid supplementation. Prognosis Without treatment, CS due to PPNAD can be life-threatening. [rarediseases.info.nih.gov]

Determine the prognosis for carriers and affected individuals. Provide sufficient data for genetic counseling of families with PPNAD and/or Carney Complex. [clinicaltrials.gov]

Prognosis Without treatment, CS due to PPNAD can be life-threatening. Last updated: 9/1/2012 [rarediseases.info.nih.gov]

MRI T1 lower signal intensity than surrounding atrophic adrenal tissue signal drop out demonstrated on out-of-phase imaging T2: lower signal intensity than surrounding atrophic adrenal tissue Treatment and prognosis The treatment of choice is bilateral [radiopaedia.org]

Prognosis Without treatment, CS due to PPNAD can be life-threatening. The documents contained in this web site are presented for information purposes only. [orpha.net]

To further determine the etiology of Cushing’s syndrome and localize the source of excess glucocorticoids production, additional studies were performed. [digitalcommons.hsc.unt.edu]

Etiology More than 90% of reported cases of PPNAD occur as one of the manifestations of Carney complex (CNC; see this term). Although rare, familial cases of isolated PPNAD have also been reported. [rarediseases.info.nih.gov]

(See Pathophysiology and Etiology.) [emedicine.medscape.com]

This respected cardiology reference covers everything you need to know - from epidemiology and pathophysiology through diagnosis, risk stratification, treatment, outcome studies, concomitant diseases, special populations and special situations, and future [books.google.com]

Epidemiology The prevalence of endogenous Cushing syndrome (CS; see this term) is estimated at 1/26,000. PPNAD is responsible for less than 2% of cases. PPNAD is more frequent in females, especially after puberty. [rarediseases.info.nih.gov]

Summary Epidemiology The prevalence of endogenous Cushing syndrome (CS; see this term) is estimated at 1/26,000. PPNAD is responsible for less than 2% of cases. PPNAD is more frequent in females, especially after puberty. [orpha.net]

(See Epidemiology and DDx.) While myxomas are usually sporadic, several familial, autosomal dominant conditions that combine lentiginosis and cardiac myxomas have been described. [emedicine.medscape.com]

This respected cardiology reference covers everything you need to know - from epidemiology and pathophysiology through diagnosis, risk stratification, treatment, outcome studies, concomitant diseases, special populations and special situations, and future [books.google.com]

Molecular studies of the tumors of the patients will provide additional clues for the pathophysiologic mechanisms leading to PPNAD/Carney complex. [clinicaltrials.gov]

(See Pathophysiology and Etiology.) [emedicine.medscape.com]

American Volume 88 1117 – 1132. ( doi:10.2106/JBJS.E.01041 ) Search Google Scholar Export Citation Glueck CJ Freiberg R Tracy T Stroop D Wang P 1997 Thrombophilia and hypofibrinolysis: pathophysiologies of osteonecrosis. [edmcasereports.com]

Groussin L, Cazabat L, Rene-Corail F, Jullian E, Bertherat J (2005) Adrenal pathophysiology: lessons from the Carney complex. Horm Res 64(3):132–139 PubMed CrossRef Google Scholar 5. [link.springer.com]

Learn new methods of aggressive patient management and disease prevention to help ensure minimal risk of further cardiovascular problems. Benefit from the authors’ Clinical Pearls to reduce complications of hypertension. [books.google.com]

There is some evidence suggesting that statins can prevent osteonecrosis, but it is of note that our patient was never exposed to such treatment ( 19 ). [edmcasereports.com]

Women with Carney complex may be at a high risk for recurrent atrial myxomas that lead to multiple strokes. [22] Early identification of a female patient with Carney complex may facilitate stroke prevention. [emedicine.medscape.com]

The curative rate of PPNAD was about 30% by unilateral adrenalectomy, 60% by sub-total adrenalectomy, and nearly 100% by total adrenalectomy. 9 The Nelson syndrome could be prevented by routine dosage of adrenocor- ticosteroid replacement. 10 One case [124.205.33.103]

Appropriate Prophylaxis and Other Measures to Prevent Readmission. None VII. What's the evidence? Boscaro, M, Arnaldi, G. “Approach to the Patient with Possible Cushing's Syndrome”. J Clin Endocrinol Metab. vol. 94. 2009. pp. 3121-3131. [clinicaladvisor.com]