Sunday, May 22, 2011

Overactive adrenal glands (Cushing's syndrome)


If your child’s adrenal glands produce excessive amounts of certain hormones, they are said to be overactive. The symptoms (and treatment) of overactive adrenal glands depends on which hormone is being overproduced.
Some of the most commonly overproduced hormones are:
  • Androgenic steroids (also known as androgen hormones)
    • Testosterone is one of the most well-known androgen hormones. Excessive production of this or other androgen hormones can lead to exaggerated male characteristics in both men and women (like excess hair on the face and body, baldness, acne, a deeper voice and increased muscle mass).
    • If a female fetus is exposed to high levels of androgens early during a mother’s pregnancy, her genitals may develop abnormally. Young boys who experience high levels of androgen levels may grow faster, but their bones may also mature faster and stop growing too soon.
  • Aldosterone hormone
    • Overproduction of aldosterone hormone can lead to high blood pressure and to symptoms associated with low levels of potassium (like weakness, muscle aches, spasms and sometimes paralysis).
  • Corticosteroids
    • An overproduction of corticosteroids leads to the condition known as Cushing’s syndrome. Rare in children, it’s more commonly seen in adults.

What causes Cushing’s syndrome?
Cushing’s syndrome—the overproduction of corticosteroids—may be caused by an overproduction of cortisol (the hormone that controls the adrenal gland) by the pituitary gland. Other causes of Cushing’s syndrome include:
  • certain lung cancers and other tumors outside the pituitary gland
  • benign (non-cancerous) or cancerous tumors on the adrenal gland(s)
What are the symptoms of Cushing’s syndrome?
Children and adolescents with Cushing's syndrome experience weight gain, growth retardation and hypertension (high blood pressure). Other symptoms may include:
How do doctors diagnose an overactive adrenal glands?
In addition to a complete medical history and physical examination, your child’s doctor will order specific blood and/or urine tests to measure hormone levels.

How can doctors tell if my child has Cushing’s syndrome?
In addition to a complete medical history and physical examination, your child’s doctor may request some or all of the following procedures:
  • x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film
  • 24-hour urinary test (urine is collected over a 24-hour period to measure corticosteroid hormones)
  • computerized tomography scan (Also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body
  • magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body
  • dexamethasone suppression test (to differentiate whether the excess production of corticotropins originates from the pituitary gland or tumors elsewhere)
  • corticotropin-releasing hormone (CRH) stimulation test (to differentiate whether the cause is a pituitary tumor or an adrenal tumor)
How are overactive adrenal glands/Cushing’s syndrome treated?
Treatment for overactive adrenal glands may include surgical removal of growths on the adrenal gland(s) or the adrenal gland(s) itself. Your doctor may also prescribe medications that block the excessive production of certain hormones.


Thursday, May 19, 2011

Adrenal venous sampling is crucial before an adrenalectomy whatever the adrenal-nodule size on computed tomography

Journal of Hypertension:
June 2011 - Volume 29 - Issue 6 - p 1196–1202
doi: 10.1097/HJH.0b013e32834666af
Original papers: Aldosterone

Adrenal venous sampling is crucial before an adrenalectomy whatever the adrenal-nodule size on computed tomography

Sarlon-Bartoli, Gabriellea; Michel, Nicolasa; Taieb, Davidb; Mancini, Julienc; Gonthier, Camillea; Silhol, Françoisa; Muller, Cyrild; Bartoli, Jean-Micheld; Sebag, Frédérice; Henry, Jean-Françoise; Deharo, Jean-Claudea; Vaisse, Bernarda


Objective: To assess the additional value of adrenal venous sampling (AVS) to diagnose primary aldosteronism sub-types in patients who have a unilateral nodule detected by computed tomography (CT scan) and who should undergo an adrenalectomy.

Methods: A retrospective study to assess consecutive patients with primary aldosteronism undergoing an adrenal CT scan and AVS. Criterion for selective cannulation was an equal or higher cortisol level in the adrenal vein compared to the inferior vena cava. An adrenal-vein aldosterone-to-cortisol ratio of at least two times higher than the other side defined lateralization of aldosterone production.

Results: Sixty-seven patients (mean age 52 years, 39 men) underwent a CT scan accccand AVS. In nine patients (13%), cannulation of the right adrenal vein led to a technical failure. Both procedures led to diagnosis of 29 patients with adenoma-producing aldosterone (APA; 50%), 23 bilateral adrenal hyperplasias (40%), and six unilateral adrenal hyperplasias (10%). Of the 45 patients with a nodule detected by CT, subsequent AVS showed bilateral secretion in 16 patients (36%). Compared to the strategy of coupling CT scans with AVS to diagnosis APA, a CT scan alone had an accuracy of 72.4% (P < 0.001). Among patients with a macronodule detected by CT, 13 (37%) had bilateral secretion as assessed by AVS. The patients with a macronodule detected by CT alone had the same risk of a discrepancy as those with a small nodule (P = 0.99).

Conclusion: AVS is essential to diagnose the unilateral hypersecretion of aldosterone, even in patients in whom a unilateral macronodule is detected by CT, to avoid unnecessary surgery.


Thursday, May 05, 2011

NIH Adrenal Clinical Trials Updated 5/5/2011

Rank Status Study
1 Completed Cardiovascular Risk in Patients With Non-Functional Adrenal Incidentaloma
Condition: Adrenal Cortex Neoplasms
2 Completed
Has Results
Changes in Adrenal Hormones During Adrenal Radiofrequency Ablation
Condition: Adrenal Gland Neoplasms
Intervention: Procedure: Radiofrequency ablation
3 Completed Adrenal Scans With Radioiodine-Labeled Norcholesterol (NP-59)
Conditions: Adrenal Gland Neoplasms;   Adrenal Malignancies;   Abnormal Hormonal Secretions;   Electrolytes Abnormalities
Intervention: Other: Imaging, Adrenal acans
4 Recruiting Trial of Vasopressin and Epinephrine to Epinephrine Only for In-Hospital Pediatric Cardiopulmonary Resuscitation
Conditions: Cardiopulmonary Arrest;   Cardiac Arrest
Interventions: Drug: Vasopressin;   Drug: Epinephrine
5 Completed Adrenal Suppression and Adrenal Recovery Induced by Megestrol Acetate
Condition: Adrenal Function
Intervention: Drug: megestrol acetate
6 Recruiting Test Predicting Adrenal Insufficiency in Volunteers Under Prednisone Treatment
Condition: Adrenal Gland Hypofunction
Interventions: Procedure: adrenal insufficiency testing;   Drug: prednisone
7 Recruiting The Bupivacaine Dose Sparing Effect of Intrathecal Epinephrine
Condition: Spinal Anesthesia
Interventions: Drug: Placebo;   Drug: epinephrine 25;   Drug: Epinephrine 50;   Drug: epinephrine 100;   Drug: Epi 200
8 Recruiting SPARTACUS: Subtyping Primary Aldosteronism: a Randomized Trial Comparing Adrenal Vein Sampling and Computed Tomography Scan.
Condition: Hyperaldosteronism
Intervention: Other: Ct-scan or adrenal vein sampling
9 Completed Make up for the Epinephrine Autoinjector
Condition: Anaphylaxis
Intervention: Device: Epinephrine autoinjector
10 Completed Epinephrine Inhalation Aerosol USP, a HFA-MDI Study for Assessment of Pharmacokinetics
Conditions: Asthma;   Bronchospasm;   Wheezing;   Shortness of Breath
Interventions: Drug: Epinephrine Inhalation Aerosol, HFA;   Drug: Epinephrine Inhalation Aerosol
11 Completed Once-Daily Oral Modified-Release Hydrocortisone in Patients With Adrenal Insufficiency
Condition: Adrenal Insufficiency
Interventions: Drug: hydrocortisone (modified release), oral tablet 20 and 5 mg;   Drug: Hydrocortisone, oral tablet, 10 mg
12 Completed Pharmacokinetics (PK) Study of Epinephrine Inhalation Aerosol in Healthy Volunteers
Condition: Asthma
Intervention: Drug: epinephrine inhalation aerosol
13 Completed ED50 and ED95 of Intrathecal Bupivacaine With or Without Epinephrine for Total Knee Replacement Arthroplasty
Conditions: Spinal Anesthesia;   Total Knee Replacement Arthroplasty
Interventions: Drug: intrathecal bupivacaine 6 mg with 100 mcg of epinephrine;   Drug: intrathecal bupivacaine 7 mg with 100 mcg of epinephrine;   Drug: intrathecal bupivacaine 8 mg with 100 mcg of epinephrine;   Drug: intrathecal bupivacaine 9 mg with 100 mcg of epinephrine;   Drug: intrathecal bupivacaine 10 mg with 100 mcg of epinephrine;   Drug: intrathecal bupivacaine 11 mg with epinephrine 100 mcg;   Drug: intrathecal bupivacaine 6 mg with 200 mcg of epinephrine;   Drug: intrathecal bupivacaine 7 mg with 200 mcg of epinephrine;   Drug: intrathecal bupivacaine 8 mg with 200 mcg of epinephrine;   Drug: intrathecal bupivacaine 9 mg with 200 mcg of epinephrine;   Drug: intrathecal bupivacaine 10 mg with 200 mcg of epinephrine;   Drug: intrathecal bupivacaine 11 mg with 200 mcg of epinephrine
14 Active, not recruiting Study of UK Adults With Congenital Adrenal Hyperplasia.
Condition: Congenital Adrenal Hyperplasia
15 Not yet recruiting Intranasal Injection Versus Topical Administration of Epinephrin During Endoscopic Sinus Surgery
Conditions: Hypertension;   Hypotension;   Tachycardia;   Bradycardia;   Arrhythmia
Interventions: Drug: Epinephrin (Intranasal injection);   Drug: Epinephrin (Topical administration)
16 Recruiting Adrenal Insufficiency in Septic Shock
Conditions: Septic Shock;   Acute Adrenal Insufficiency
Intervention: Drug: Corticosteroid
17 Recruiting Performance of 18F-Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) in the Diagnosis of Indeterminate Adrenal Tumors on Conventional Imaging: A French Prospective Multicentric Study
Condition: Adrenal Gland Neoplasms
Intervention: Other: FDG-PET scan
18 Unknown  Prospective Study on the Incidence of Adrenal Crisis in Patients With Chronic Adrenal Insufficiency
Condition: Adrenal Insufficiency
19 Completed Dose Response Relationship for Single Doses of Corticotropin Releasing Hormone (CRH) in Normal Volunteers and in Patients With Adrenal Insufficiency
Conditions: Adrenal Gland Hyperfunction;   Adrenal Gland Hypofunction;   Cushing's Syndrome;   Healthy
Intervention: Drug: Ovine Corticotropin-Releasing Hormone (oCRH)
20 Recruiting Adrenal Function in Critical Illness
Condition: Adrenal Insufficiency

Rank Status Study
21 Recruiting Study of Adrenal Gland Tumors
Condition: Adrenal Gland Neoplasm
22 Recruiting The Effects of Epinephrine in Endotoxemia in Normal Volunteers
Condition: Immune System
Interventions: Biological: Endotoxin, Lipopolysaccharide, LPS;   Biological: Endotoxin, Lipopolysaccharide, LPS /Epinephrine
23 Unknown  Does Topical Steroid Treatment Impair the Adrenal Function?
Conditions: Hypothalamus-Pituitary-Adrenal Axis Assessement;   Topical Steroid Therapy in Chronic Skin Diseases
24 Recruiting Combination Local Anesthetics
Condition: Perioperative Pain
Interventions: Drug: 1% Lidocaine with Epinephrine;   Drug: 0.25% Bupivacaine with epinephrine;   Drug: 1% Lidocaine + 0.25% Bupivacaine with Epinephrine;   Drug: 2% Lidocaine + 0.5% Bupivacaine with epinephrine
25 Recruiting Study Comparing Peri-articular Injection of Bupivacaine With and Without Epinephrine
Condition: Osteoarthritis
Interventions: Procedure: Peri-articular injection of marcaine/epinephrine;   Procedure: Peri-articular injection of marcaine alone
26 Not yet recruiting Gluing Lacerations Utilizing Epinephrine
Condition: Lacerations
Intervention: Drug: LET - Lidocaine Epinephrine Tetracaine
27 Recruiting Use of Local Analgesia With Epinephrine During Total Hip Arthroplasty (THA)
Condition: Arthroplasty, Replacement, Hip
Intervention: Drug: ropivacaine, physical serum and adrenalin
28 Recruiting Relative Adrenal Insufficiency in Preterm Very Low Birth Weight Infants With Shock
Condition: Adrenal Insufficiency
29 Unknown  Adrenal Insufficiency in Cirrhotics With Ascites. Effects of Hydrocortisone on Renal and Haemodynamic Function
Condition: Cirrhosis With Ascites
Interventions: Drug: hydrocortisone;   Drug: dextrose solution 5%
30 Recruiting Bronchiolitis, Optimal Treatment in Infants and Prognosis
Condition: Bronchiolitis
Interventions: Drug: Racemic adrenaline;   Drug: Isotonic saline
31 Recruiting RAD001 in Pheochromocytoma or Nonfunctioning Carcinoid
Conditions: Pheochromocytoma;   Extra-Adrenal Paraganglioma;   Non-functioning Carcinoid
Intervention: Drug: RAD001
32 Not yet recruiting Hypoglycemia Associated Autonomic Failure in Type 1 DM, Q4
Condition: Type 1 Diabetes
Intervention: Drug: epinephrine
33 Recruiting Effect of Epinephrine/ Phenylephrine for Preventing the Postreperfusion Syndrome During Reperfusion in Liver Transplantation
Condition: Hypotension After Reperfusion in Liver Transplantation
Interventions: Drug: phenylephrine;   Drug: epinephrine;   Drug: placebo control
34 Recruiting Safety Study Evaluating the Adrenal Suppression Potential of Product 0405 in Pediatric Subjects With Atopic Dermatitis
Condition: Atopic Dermatitis
Intervention: Drug: Product 0405
35 Recruiting Merits of Continuous Paravertebral Block in the Management of Renal/Adrenal Surgery by Laparotomy
Condition: Patient Scheduled for Renal and/or Adrenal Surgery
Intervention: Procedure: Continuous Paravertebral block
36 Not yet recruiting Adrenalectomy Versus Follow-up in Patients With Subclinical Cushings Syndrome
Condition: Adrenal Tumour With Mild Hypercortisolism
Intervention: Procedure: Adrenalectomy
37 Recruiting Role of the Protein Osteoprotegerin in the Bone Health of Women With Congenital Adrenal Hyperplasia
Condition: Adrenal Hyperplasia, Congenital
38 Recruiting Gene Polymorphisms Influencing Steroid Synthesis and Action
Conditions: Disorders of Sex Development;   Congenital Adrenal Hyperplasia;   Congenital Adrenal Hypoplasia;   Adrenal Insufficiency;   Mineralocorticoid Deficiency;   Intersex
39 Recruiting Adrenal Function and Use of Intralesional Triamcinolone Acetonide 10 mg/mL (Kenalog-10) in Patients With Alopecia Areata
Condition: Alopecia Areata
Intervention: Drug: Triamcinolone Acetonide 10 mg/mL (Kenalog-10)
40 Recruiting Assessment of the Efficacy of Nebulised 3% Hypertonic Saline Among Infants Aged 6 Weeks- 24 Months With Bronchiolitis
Condition: Bronchiolitis
Interventions: Drug: L-Epinephrine and 0.9% Normal Saline;   Drug: L-Epinephrine and 3% Hypertonic Saline

Sunday, May 01, 2011

Congenital adrenal hyperplasia (CAH)

PerkinElmer's Neonatal17 a-OH-Progesterone (17OHP) assay for its DELFIA®, AutoDELFIA® and GSP® platforms platforms provides the highest standards of reliability and safety in screening for congenital adrenal hyperplasia (CAH). It is globally the most widely used assay for 1st tier CAH screening, and in 2008 theproduct was used in 47 countries.

No extraction step needed
The assay is a straightforward dried blood spot assay.  No extraction step is needed, which means savings in both total assay time and materials.

DELFIA®-technology stands for quality
The unique fluorescent properties of lanthanide chelatesare the basis for high sensitivity and low assay variation, and these features, in turn, stand for reliable and accurate 17a-OH-Progesterone measurement with low bias.

Early diagnosis for early disease/Intervention
Congenital adrenal hyperplasia is a genetic disorder affecting 1:10,000 to 1:15,000 newborns worldwide and the most severe form of the disease can lead to a life-threatening condition during the first weeks of life.  The disease is caused by enzyme defects in the steroid biosynthesis, the most frequent types being 21- and 11a-hydroxylase deficiency. In both of these the17a-OH-progesterone, a precursor for cortisol, is increased which makes its determination a useful screening method for 95% of all of the CAH cases.

PerkinElmer's Neonatal 17OHP assay is intended for the quantitative determination of 17a-OH-progesterone indried blood spot specimens as an aid in screening newborns for CAH.