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Corticotropin Stimulation Test

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121. Tourette Syndrome and Other Tic Disorders (Treatment)

dyskinesia. Dopamine agonist therapy Paradoxically, several mixed dopamine agonists have also been proven effective in reducing tic frequency. [ , , ] To date, they have been tested exclusively in relatively low doses, partly because of a theory that, at these doses, they must antagonize dopamine function by selective action at presynaptic receptors. Accumulating evidence suggests that this rationale is faulty, however, and trials with higher doses may be expected. Habit reversal therapy Five RCTs have (...) demonstrated the efficacy of a specific form of behavior therapy for tics. [ , , , , , ] The originally tested treatment consisted of a package of interventions called habit reversal therapy, [ ] which comprises monitoring, relaxation, and other nonspecific elements of behavior therapy. The most important element is application of a competing response whenever the patient notices either a tic or the urge to tic. Initially, heavy effort on the part of the patient may be needed. However, in all 4 reported

2014 eMedicine.com

122. Pituitary Disease and Pregnancy (Treatment)

. The ACTH stimulation test cannot be used for at least 3 months, because the adrenal cortex needs time to atrophy and lose responsiveness to low-dose ACTH. In the 3-month postpartum period, -induced hypoglycemia or other stimulation tests, such as glucagon, would be needed for definitive diagnosis of ACTH (and GH) deficiency. Even if a clear temporal association exists with parturition, imaging with MRI (preferably) or CT scanning is indicated to exclude mass lesions. In long-standing Sheehan syndrome (...) are started on therapy for hyperprolactinemia, mechanical contraception can be advised for the first 2-3 cycles so that an intermenstrual period can be established. By doing this, the woman will know when she has missed a period when unprotected intercourse is resumed. As soon as this occurs, a pregnancy test can be obtained and dopamine agonist stopped. This approach limits dopamine agonist exposure to a maximum of 3-4 weeks. In practice, this delay is usually not done. appears to be safe in pregnancy

2014 eMedicine.com

123. Pseudo-Cushing Syndrome (Treatment)

cortisol with an automated immunoassay system. Clinical Chemistry & Laboratory Medicine . 2006. 44(12):1441-5. . Alwani RA, Schmit Jongbloed LW, de Jong FH, van der Lely AJ, de Herder WW, Feelders RA. Differentiating between Cushing's disease and pseudo-Cushing's syndrome: comparison of four tests. Eur J Endocrinol . March/2014. 170(4):477-86. . Yanovski JA, Cutler GB Jr, Chrousos GP, Nieman LK. Corticotropin-releasing hormone stimulation following low-dose dexamethasone administration. A new test (...) cortisol concentrations. [ ] PCS can occur at any age; it can develop in infants exposed to alcohol in breast milk. [ ] Next: Causes and Complications Etiology The mechanism behind PCS is unclear. Most evidence suggests central stimulation of a corticotropin-releasing hormone (CRH), either at the hypothalamic or suprahypothalamic level. [ , ] Rats given 15% alcohol for 3 months showed a greater number of corticotropin-producing cells with increased secretory activity. [ ] A two-hit hypothesis has been

2014 eMedicine.com

124. Pseudohypoparathyroidism (Overview)

PHP type 1a Several other peptide hormones, including thyroid-stimulating hormone (thyrotropin), antidiuretic hormone, gonadotropins, glucagons, adrenocorticotropin, and growth hormone–releasing hormone, use the α subunit of stimulatory G protein to enhance cAMP production. Patients with PHP-1a can present with resistance to the effects of any of these hormones, although in most patients, responses to corticotropin and glucagon are clinically unaffected. The dominant pattern of inheritance of PHP (...) (AHO). These features included short stature, rounded face, shortened fourth metacarpals and other bones of the hands and feet, obesity, dental hypoplasia, and soft-tissue calcifications/ossifications. In addition, administration of PTH failed to produce the expected phosphaturia or to stimulate renal production of cyclic adenosine monophosphate (cAMP). However, the AHO phenotype is not a feature of PHP-1b or PHP-2. The molecular defects in the gene ( GNAS1 ) encoding the α subunit

2014 eMedicine.com

125. Pseudo-Cushing Syndrome (Overview)

cortisol with an automated immunoassay system. Clinical Chemistry & Laboratory Medicine . 2006. 44(12):1441-5. . Alwani RA, Schmit Jongbloed LW, de Jong FH, van der Lely AJ, de Herder WW, Feelders RA. Differentiating between Cushing's disease and pseudo-Cushing's syndrome: comparison of four tests. Eur J Endocrinol . March/2014. 170(4):477-86. . Yanovski JA, Cutler GB Jr, Chrousos GP, Nieman LK. Corticotropin-releasing hormone stimulation following low-dose dexamethasone administration. A new test (...) cortisol concentrations. [ ] PCS can occur at any age; it can develop in infants exposed to alcohol in breast milk. [ ] Next: Causes and Complications Etiology The mechanism behind PCS is unclear. Most evidence suggests central stimulation of a corticotropin-releasing hormone (CRH), either at the hypothalamic or suprahypothalamic level. [ , ] Rats given 15% alcohol for 3 months showed a greater number of corticotropin-producing cells with increased secretory activity. [ ] A two-hit hypothesis has been

2014 eMedicine.com

126. Primary Insomnia (Overview)

insomnia Idiopathic insomnia Paradoxical insomnia [ , ] Next: Treatment Goals and Considerations The pathophysiology of primary insomnia is not well understood, and essential features assist in diagnosis. The focus of management is on symptoms. [ ] However, findings have strengthened the evidence that primary insomnia may be linked with mood disorders and is associated with hypothalamic-pituitary-adrenal (HPA) axis overactivity and excess secretion of corticotropin-releasing factor (CRF (...) as well. Previous Next: Differentials in Primary Insomnia The exclusion of common causes of insomnia is required to make the diagnosis of primary insomnia. Medical causes of insomnia include the following: Chronic pain, especially neuropathic pain Primary sleep disorders (eg, sleep apnea, periodic limb movements, restless legs syndrome) Dyspnea from any cause Pregnancy Drug use or withdrawal (eg, selective serotonin reuptake inhibitors, stimulants, antihistamines, caffeine, diet pills, herbal

2014 eMedicine.com

127. Pituitary Disease and Pregnancy (Overview)

. The ACTH stimulation test cannot be used for at least 3 months, because the adrenal cortex needs time to atrophy and lose responsiveness to low-dose ACTH. In the 3-month postpartum period, -induced hypoglycemia or other stimulation tests, such as glucagon, would be needed for definitive diagnosis of ACTH (and GH) deficiency. Even if a clear temporal association exists with parturition, imaging with MRI (preferably) or CT scanning is indicated to exclude mass lesions. In long-standing Sheehan syndrome (...) are started on therapy for hyperprolactinemia, mechanical contraception can be advised for the first 2-3 cycles so that an intermenstrual period can be established. By doing this, the woman will know when she has missed a period when unprotected intercourse is resumed. As soon as this occurs, a pregnancy test can be obtained and dopamine agonist stopped. This approach limits dopamine agonist exposure to a maximum of 3-4 weeks. In practice, this delay is usually not done. appears to be safe in pregnancy

2014 eMedicine.com

128. Pheochromocytoma (Overview)

hypotension: From volume contraction Hypertensive retinopathy Weight loss Pallor Fever Tremor Neurofibromas Tachyarrhythmias Pulmonary edema Cardiomyopathy Ileus Café au lait spots See for more detail. Diagnosis Diagnostic tests for pheochromocytoma include the following: Plasma metanephrine testing: 96% sensitivity, 85% specificity [ ] 24-hour urinary collection for catecholamines and metanephrines: 87.5% sensitivity, 99.7% specificity [ ] Test selection criteria include the following: Use plasma (...) metanephrine testing in patients at high risk (ie, those with predisposing genetic syndromes or a family or personal history of pheochromocytoma) Use 24-hour urinary collection for catecholamines and metanephrines in patients at lower risk Imaging studies should be performed only after biochemical studies have confirmed the diagnosis of pheochromocytoma. Studies are as follows: Abdominal CT scanning: Has accuracy of 85-95% for detecting adrenal masses with a spatial resolution of 1 cm or greater MRI

2014 eMedicine.com

129. Pregnancy Diagnosis (Overview)

feasible tests that use these hormones have been made available to aid in the diagnosis of pregnancy. Beta-human chorionic gonadotropin hCG is a glycoprotein similar in structure to follicle-stimulating hormone (FSH), luteinizing hormone (LH), and thyrotropin. hCG is composed of alpha and beta subunits. The alpha subunit of hCG is similar to the alpha subunit of FSH, LH, and thyrotropin. The free beta subunit of hCG differs from the others in that it has a 30–amino acid tailpiece at the COOH terminus (...) dilutions to look for linearity, and testing using a different method. The five potential sources of positive hCG results outside of pregnancy are described below: [ , ] Phantom hCG Caused by heterophilic antibodies that bind the capture and labeled antibodies together without hCG being present Antibody production results from exposure to animals used to produce antibodies used in assay Rule out with sensitive urine assay, as these antibodies do not cross into urine Pituitary hCG Stimulated

2014 eMedicine.com

130. Fibromyalgia (Overview)

-stimulating hormone level 25-hydroxy vitamin D level Vitamin B12 level Iron studies, including iron level, total iron binding capacity, percent saturation, and serum ferritin level Magnesium level Erythrocyte sedimentation rate Antipolymer antibody assay: May provide conclusive evidence for a subgroup of people with fibromyalgia; about 50% of fibromyalgia patients have antipolymer antibodies Patient self-report forms, clinical psychometric testing Self-report forms, for assessing patients’ pain, fatigue (...) be explained if platelet ATP levels are also low. ATP is necessary to move and then hold serotonin in platelets. More investigation into ATP and the link to serotonin is needed. Dysfunction of the hypothalamic-pituitary-adrenal axis Studies of the neuroendocrine aspects of fibromyalgia have found dysfunction of the HPA axis. [ ] The HPA axis is a critical component of the stress-adaptation response. The sequence of HPA action is that corticotropin-releasing hormone (CRH) from the hypothalamus stimulates

2014 eMedicine.com

131. Carney Complex (Overview)

See for more detail. Diagnosis Laboratory studies used in the diagnosis of Carney complex include the following: Complete blood count (CBC) Glucose/electrolytes Erythrocyte sedimentation rate Thyroxine/thyroid-stimulating hormone Adrenocorticotropic hormone Growth hormone Twenty-four–hour urinary cortisol excretion test and dexamethasone stimulation test: To evaluate for primary pigmented nodular adrenocortical disease (PPNAD) as part of Carney complex [ ] Echocardiography is the investigation (...) . (See Pathophysiology and Etiology.) Endocrine overactivity is one of the characteristics of this syndrome. [ ] In fact, corticotropin hormone–independent due to primary pigmented nodular adrenocortical disease is an important characteristic of Carney complex. (See Workup and Treatment.) Patient education The family should be aware that this is an autosomal dominant disorder with a 50% chance that the offspring of an affected individual will have the disorder. Previous Next: Pathophysiology Carney

2014 eMedicine.com

132. Carney Syndrome (Overview)

See for more detail. Diagnosis Laboratory studies used in the diagnosis of Carney complex include the following: Complete blood count (CBC) Glucose/electrolytes Erythrocyte sedimentation rate Thyroxine/thyroid-stimulating hormone Adrenocorticotropic hormone Growth hormone Twenty-four–hour urinary cortisol excretion test and dexamethasone stimulation test: To evaluate for primary pigmented nodular adrenocortical disease (PPNAD) as part of Carney complex [ ] Echocardiography is the investigation (...) . (See Pathophysiology and Etiology.) Endocrine overactivity is one of the characteristics of this syndrome. [ ] In fact, corticotropin hormone–independent due to primary pigmented nodular adrenocortical disease is an important characteristic of Carney complex. (See Workup and Treatment.) Patient education The family should be aware that this is an autosomal dominant disorder with a 50% chance that the offspring of an affected individual will have the disorder. Previous Next: Pathophysiology Carney

2014 eMedicine.com

133. Tourette Syndrome and Other Tic Disorders (Overview)

The onset is before age 18 years The disturbance is not due to the direct physiologic effects of a substance (eg, stimulants) or a general medical condition (eg, Huntington disease or postviral encephalitis) Criteria have never been met for Tourette’s disorder or persistent (chronic) motor or vocal tic disorder See for more detail. Management Treatments for tics that have demonstrated efficacy in replicated controlled trials include the following: Dopamine D2 receptor antagonist therapy Dopamine agonist (...) -depleting agents also improve tics, and in some patients, tics may be worsened by neuroleptic withdrawal or, possibly, stimulant use. However, other data do not support a simple hypothesis that dopamine function is hyperactive in individuals with TS. Tics are not abated with the subsequent development of Parkinson disease. [ ] However, in Parkinson disease, dopamine loss is most evident in posterior putamen, [ ] whereas caudate and ventral striatum are more implicated in TS. Furthermore, dopamine

2014 eMedicine.com

134. Conn Syndrome (Overview)

group in 2011. [ ] Workup Screening (first-tier) tests for primary aldosteronism include the following: Serum potassium and bicarbonate levels Sodium and magnesium levels Plasma aldosterone/plasma renin activity ratio Confirmatory (second-tier) tests include the following: Serum aldosterone level 24-hour urinary aldosterone excretion test Salt-loading test Tests for determining the primary aldosteronism subtype (third-tier tests) include the following: Postural stimulation test Furosemide (Lasix (...) ) stimulation test Diurnal rhythm of aldosterone The initial radiologic investigation in the workup of primary aldosteronism is high-resolution, thin-sliced (2-2.5 mm) adrenal computed tomography (CT) scanning with contrast. Other tests include the following: NP-59 iodo-methyl-norcholesterol scintigraphy: Although fairly difficult to set up and not routinely available, this test can be useful in select cases for distinguishing between adenomas and hyperplasia Adrenal venous sampling: Adrenal venous sampling

2014 eMedicine.com

135. Alpha2-Plasmin Inhibitor Deficiency (Overview)

noncoagulation proteins, such as complement, growth hormone, corticotropin, and glucagon, are substrates for plasmin. Therefore, the reasons for the bleeding disorder that develops due to the actions of excess unfettered and unneutralized plasmin are easily comprehended. Alpha 2-plasmin inhibitor (alpha 2-PI, a2-PI) belongs to the serpin family of inhibitors, is synthesized by the liver, and is present in plasma as a single-chain protein in approximately half the concentration of plasminogen. Two forms (...) Very few cases of inherited alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency) have been reported; therefore, data do not exist to determine the true frequency. In the next several years, as widespread high-throughput genomic testing becomes commonplace, the frequency of genetic defects will be known, and the frequency of these rare disorders can then be determined. The frequency of acquired alpha 2-plasmin inhibitor deficiency (alpha 2-PI deficiency, a2-PI deficiency

2014 eMedicine.com

136. Luteinizing Hormone Deficiency (Overview)

Hormone Deficiency Updated: Nov 04, 2016 Author: Jennifer L Eaton, MD, MSCI, FACOG; Chief Editor: Richard Scott Lucidi, MD, FACOG Share Email Print Feedback Close Sections Sections Luteinizing Hormone Deficiency Overview Background An isolated luteinizing hormone (LH) deficiency is an uncommon condition. LH deficiency almost always occurs in conjunction with follicle-stimulating hormone (FSH) deficiency because LH and FSH are secreted by the same pituitary gonadotrope cells. LH deficiency can manifest (...) treatment. Structure and genetics LH is a glycoprotein dimer composed of 2 glycosylated noncovalently-linked subunits designated alpha and beta. The alpha subunit is composed of 92 amino acids and is encoded on the long arm of chromosome 6. The beta subunit is 121 amino acids and is encoded on the long arm of chromosome 19. The alpha subunit of LH is biologically identical to 3 other hormones: FSH, thyroid-stimulating hormone (TSH), and human chorionic gonadotropin (hCG). The beta subunit is unique

2014 eMedicine.com

137. Infertility, Male (Overview)

), pituitary gland, and testicles to secrete gonadotropin-releasing hormone (GnRH) in a pulsatile pattern approximately every 70-90 minutes. The half-life of GnRH is 2-5 minutes. Release of GnRH is stimulated by melatonin from the pineal gland and inhibited by testosterone, inhibin, corticotropin-releasing hormone, opiates, illness, and stress. GnRH travels down the portal system to the anterior pituitary, located on a stalk in the sella turcica, to stimulate the release of the gonadotropins, luteinizing (...) (normal, >40% of sperm having normal movement) Sperm morphology (sample lower limit for percentage of normal sperm is 4%) Signs of infection – An increased number of white blood cells (WBCs) in the semen may be observed in patients with infectious or inflammatory processes Other variables (eg, levels of zinc, citric acid, acid phosphatase, or alpha-glucosidase) Other laboratory tests that may be helpful include the following: Antisperm antibody test Hormonal analysis (FSH, LH, TSH, testosterone

2014 eMedicine.com

138. Hyperaldosteronism, Primary (Overview)

was described by Lifton’s group in 2011. [ ] Workup Screening (first-tier) tests for primary aldosteronism include the following: Serum potassium and bicarbonate levels Sodium and magnesium levels Plasma aldosterone/plasma renin activity ratio Confirmatory (second-tier) tests include the following: Serum aldosterone level 24-hour urinary aldosterone excretion test Salt-loading test Tests for determining the primary aldosteronism subtype (third-tier tests) include the following: Postural stimulation test (...) Furosemide (Lasix) stimulation test Diurnal rhythm of aldosterone The initial radiologic investigation in the workup of primary aldosteronism is high-resolution, thin-sliced (2-2.5 mm) adrenal computed tomography (CT) scanning with contrast. Other tests include the following: NP-59 iodo-methyl-norcholesterol scintigraphy: Although fairly difficult to set up and not routinely available, this test can be useful in select cases for distinguishing between adenomas and hyperplasia Adrenal venous sampling

2014 eMedicine.com

139. Hypopituitarism (Panhypopituitarism) (Overview)

of the pituitary gland can be modulated at many stages. The pituitary hormones, or target organ hormones, can influence the hypothalamus and the pituitary to decrease or increase pituitary hormone secretion through long and short feedback loops. Hormones secreted by the anterior pituitary include the following: Thyrotropin, or thyroid-stimulating hormone (TSH) Gonadotropins, or follicle-stimulating hormone (FSH) and luteinizing hormone (LH) Somatotropin or growth hormone (GH) Corticotropin (...) hormones are often involved, resulting in isolated or partial . [ ] (See Pathophysiology and Etiology.) Pituitary gland physiology The pituitary gland has 2 parts: the anterior pituitary (adenohypophysis) and the posterior pituitary (neurohypophysis). The anterior pituitary receives signals from the hypothalamus that either stimulate or inhibit secretion of pituitary hormones. These hormones are secreted directly into the systemic circulation, where they act on specific organs. The actions

2014 eMedicine.com

140. Osteoporosis in Solid Organ Transplantation (Overview)

are indicated in the pretransplant evaluation of all patients awaiting solid organs [ , ] : Serum calcium level Phosphorus level Bicarbonate level Alkaline phosphatase level BUN/creatinine levels Intact PTH assay, to assess for hyperparathyroidism (primary or secondary) 25-Hydroxyvitamin D value to assess total body vitamin D stores Thyroid function tests (eg, thyroid-stimulating hormone, free levothyroxine) Testosterone level (males only), to ensure eugonadism Estradiol and FSH levels (in females (...) hormone, thereby decreasing gonadal hormone production; may also directly decrease gonadal hormone production Suppress corticotropin, thereby suppressing the adrenal production of androstenedione, a substrate for both testosterone and estrone production Decrease osteoblast-mediated bone formation Increase bone resorption Glucocorticoids result in a disproportionate loss of cancellous or trabecular bone, possibly because trabecular bone has an inherently greater rate of turnover than cortical bone

2014 eMedicine.com

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