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Corticotropin Stimulation Test

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181. Adrenal Glands (Overview)

entirely controlled by the secretion of ACTH by the anterior pituitary gland, which is controlled by corticotropin-releasing hormone (CRH) secreted by the hypothalamus. In normal situations, CRH, ACTH, and cortisol secretory rates demonstrate a circadian rhythm, with a zenith in the early morning and a nadir in the evening. Various stresses also stimulate increased ACTH and, thus, cortisol secretion. A negative feedback effect of cortisol on the anterior pituitary and the hypothalamus help control (...) test for distinguishing between Cushing disease and ectopic ACTH production is the metyrapone stimulation test. Because metyrapone blocks 11-hydroxylase, which is responsible for conversion of 11-deoxycortisol to cortisol, administration at 15 mg/kg (or 750 mg for adolescents) PO every 4 hours for 24 hours decreases plasma cortisol and increases ACTH. The normal response is an increase in plasma 11-deoxycortisol levels to more than 10 μg/dL and an increase in 24-hour urine 17-OHCS levels to twice

2014 eMedicine Pediatrics

182. Adrenal Hypoplasia (Overview)

stimulation. J Clin Endocrinol Metab . 1991 Sep. 73(3):674-86. . Heckmann M, Hartmann MF, Kampschulte B, et al. Cortisol production rates in preterm infants in relation to growth and illness: a noninvasive prospective study using gas chromatography-mass spectrometry. J Clin Endocrinol Metab . 2005 Oct. 90(10):5737-42. . Kazlauskaite R, Evans AT, Villabona CV, et al. Corticotropin tests for hypothalamic-pituitary- adrenal insufficiency: a metaanalysis. J Clin Endocrinol Metab . 2008 Nov. 93(11):4245-53 (...) . . Schurmeyer TH, Avgerinos PC, Gold PW, et al. Human corticotropin-releasing factor in man: pharmacokinetic properties and dose-response of plasma adrenocorticotropin and cortisol secretion. J Clin Endocrinol Metab . 1984 Dec. 59(6):1103-8. . Peter M, Partsch CJ, Dorr HG, Sippell WG. Prenatal diagnosis of congenital adrenal hypoplasia. Horm Res . 1996 Jul. 46(1):41-5. . Media Gallery of 0 Tables Contributor Information and Disclosures Author Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief

2014 eMedicine Pediatrics

183. Glucocorticoid Therapy and Cushing Syndrome (Treatment)

hormone stimulation following low-dose dexamethasone administration. A new test to distinguish Cushing's syndrome from pseudo-Cushing's states. JAMA . 1993 May 5. 269(17):2232-8. . Media Gallery Diagnosis of Cushing syndrome. Etiology of Cushing syndrome. Physical findings in Cushing syndrome. of 3 Tables ] Table 1. Glucocorticoid Equivalencies [ ] Type Drug Dose Relative Glucocorticoid Potency Relative Mineralocorticoid Potency Plasma Half-Life (mg) Biologic Half-Life (h) Short-acting Cortisol 20 1.0 (...) available mineralocorticoid in most countries. Fludrocortisone has some glucocorticoid activity. Medication is also required when the patient has Cushing syndrome due to ectopic corticotropin (ACTH) and the primary source cannot be found or when surgery has not cured the hypercortisolism. In this situation, the aim of treatment is to suppress glucocorticoid production and, in the case of malignancy, to reduce tumor growth. When possible, minimize the dose and duration of glucocorticoid treatment

2014 eMedicine Pediatrics

184. Hypothermia, Circulatory Arrest and Cardiopulmonary Bypass (Treatment)

catecholamines, cortisol, growth hormone, Glucagon, corticotropin (or adrenocorticotropic hormone [ACTH]), thyroid-stimulating hormone (TSH), and endorphins. levels of thyroid hormone decreases the first few days after CPB. Decreased renal and hepatic function after CPB leads to decreased clearance of vasoactive inflammatory mediators from the kidneys and liver. The lung is normally responsible for metabolizing and clearing many of these hormones, particularly catecholamines. Exclusion of the lungs from (...) surfaces of the bypass circuit are associated with activation of inflammatory mediators. Effects include activation of the complement system, including plasma-activated complement 3 (C3a). A potent stimulator of platelet aggregation, C3a causes the release of from mast cells and basophils, increases vascular permeability, and stimulates WBCs to release oxygen free radicals and lysosomal enzymes. Elevated levels of C3a are linked to the duration of CPB. Neutrophil activation has been linked

2014 eMedicine Pediatrics

185. Hypopituitarism (Overview)

produced in the hypothalamus. These regulatory hormones are transported to the anterior pituitary via the pituitary portal system circulation. The release-stimulating hormones produced by the hypothalamus include the following: Growth hormone–releasing hormone (GHRH) Corticotropin-releasing hormone (CRH) Thyrotropin-releasing hormone (TRH) Gonadotropin-releasing hormone (GnRH) PRL secretion is distinct from that of the other anterior pituitary hormones, being inhibited by hypothalamic dopamine (...) from the anterior pituitary include the following: Growth hormone (GH) Adrenocorticotropic hormone (ACTH) Thyroid-stimulating hormone (TSH) Luteinizing hormone (LH) Follicle-stimulating hormone (FSH) Prolactin (PRL) The pathway from embryogenesis to the full differentiation of specific functional cell types within the pituitary is controlled by numerous genes that encode transcription factors. (See the diagram below.) Mutations in these genes are causes of congenital hypopituitarism and have

2014 eMedicine Pediatrics

186. Hyposomatotropism (Overview)

requiring comprehensive clinical and auxologic assessment combined with biochemical testing of the GH-insulinlike growth factor (IGF) axis and radiologic evaluation. Biochemical testing of the GH-IGF axis includes radioimmunoassays (RIAs) of GH, IGF, and insulinlike growth factor binding proteins (IGFBPs) [ , , ] Next: Background Human pituitary-derived growth hormone In the 1950s, growth hormone isolated from the pituitaries of humans and anthropoid apes was discovered to stimulate growth in children (...) , Thompson JQ. Human growth hormone release. Comparison of provocative test procedures. Am J Med . 1974 Feb. 56(2):179-85. . Lanes R, Hurtado E. Oral clonidine-an effective growth hormone-releasing agent in prepubertal subjects. J Pediatr . 1982 May. 100(5):710-4. . Collu R, Leboeuf G, Letarte J, Ducharme JR. Stimulation of growth hormone secretion by levodopa-propranolol in children and adolescents. Pediatrics . 1975 Aug. 56(2):262-6. . Merimee TJ, Rabinowtitz D, Fineberg SE. Arginine-initiated release

2014 eMedicine Pediatrics

187. Hypothermia, Circulatory Arrest and Cardiopulmonary Bypass (Overview)

catecholamines, cortisol, growth hormone, Glucagon, corticotropin (or adrenocorticotropic hormone [ACTH]), thyroid-stimulating hormone (TSH), and endorphins. levels of thyroid hormone decreases the first few days after CPB. Decreased renal and hepatic function after CPB leads to decreased clearance of vasoactive inflammatory mediators from the kidneys and liver. The lung is normally responsible for metabolizing and clearing many of these hormones, particularly catecholamines. Exclusion of the lungs from (...) surfaces of the bypass circuit are associated with activation of inflammatory mediators. Effects include activation of the complement system, including plasma-activated complement 3 (C3a). A potent stimulator of platelet aggregation, C3a causes the release of from mast cells and basophils, increases vascular permeability, and stimulates WBCs to release oxygen free radicals and lysosomal enzymes. Elevated levels of C3a are linked to the duration of CPB. Neutrophil activation has been linked

2014 eMedicine Pediatrics

188. Menkes Kinky Hair Disease (Overview)

cloning has enabled molecular diagnosis of females who carry the gene and at-risk fetuses in certain families, enhancing preventive efforts. Evidence that the gene encodes a highly conserved copper-transporting adenosine triphosphatase (ATPase) has stimulated investigation of the molecule's normal function in prokaryotic and eukaryotic systems. [ ] Knowledge gleaned from such efforts may ultimately suggest the novel therapeutic strategies needed to achieve normal neurologic outcomes in patients (...) abnormality, was excluded from direct consideration by localization to chromosome 16 in somatic cell hybrid studies. Experience with prenatal detection increased, and biochemical tests that used chorionic villus samples were developed to enable earlier diagnosis in at-risk pregnancies. A Menkes parents and professionals association was formed in the United States. In 1987, a female with classic Menkes kinky hair disease caused by an X-autosome chromosomal translocation was reported. This critical

2014 eMedicine Pediatrics

189. Maternal Chorioamnionitis (Overview)

refill time, cool and clammy skin, pale or mottled appearance, and/or oliguria Gastrointestinal: Abdominal distention, vomiting, diarrhea, and/or bloody stools Central nervous system: Thermal regulatory abnormalities, behavioral abnormalities, apnea, and/or seizures Hematologic and/or hepatic: Pallor, petechiae or purpura, and overt bleeding Laboratory tests During the intrapartum period, the diagnosis of chorioamnionitis is usually based on clinical criteria, particularly for pregnancies at term (...) . Laboratory studies for asymptomatic pregnant mothers who present with premature labor or PROM include the following: Examination of amniotic fluid Maternal blood studies Maternal urine studies Maternal group B streptococcal (GBS) screening test Testing in febrile pregnant women with suspected chorioamnionitis may include the following: White blood cell (WBC) counts C-reactive protein (CRP) levels Alpha1-proteinase inhibitor (A1PI) complex measurement Serum interleukin-6 (IL-6) or ferritin levels Studies

2014 eMedicine Pediatrics

190. Congenital Adrenal Hyperplasia (Overview)

, hyperkalemia, and elevated plasma renin activity (PRA), indicating hypovolemia Hypertensive forms of adrenal hyperplasia (ie, 11-beta-hydroxylase deficiency and 17-alpha-hydroxylase deficiency) are associated with suppressed PRA and, often, hypokalemia Subtle forms of adrenal hyperplasia (as in nonclassic forms of 21-hydroxylase deficiency and nonclassic 3-beta-hydroxysteroid dehydrogenase deficiency): Synthetic corticotropin (Cortrosyn) stimulation testing demonstrates the abnormal accumulation (...) (eg, mixed gonadal dysgenesis, Denys-Drash syndrome) Urogenitography is often helpful in defining the anatomy of the internal genitalia A bone-age study is useful in evaluating for advanced skeletal maturation in a child who develops precocious pubic hair, clitoromegaly, or accelerated linear growth Other tests A karyotype is essential in an infant with ambiguous genitalia, to establish the chromosomal sex Genetic testing is essential for genetic counseling and prenatal diagnosis of adrenal

2014 eMedicine Pediatrics

191. Conduct Disorder (Overview)

in the anterior cingulate cortex (ACC), orbitofrontal cortices (OFC), and dorsolateral prefrontal cortex (DLPFC) that worsens over time due to dysphoria activation of brain stress systems and increases in corticotropin-releasing factor (CRF). [ ] Areas deep in the brain, especially the amygdala and insula, appear to exhibit abnormal function reflected in overall decreases in resting state connectivity and smaller overall size. [ ] This decrease in brain structure and functionality is also seen in youth (...) and community. Parents become reluctant to take their child with CD out in public because they fear an uncontrollable incident with the child. After increased negative interaction with the child, parental stimulation may decrease as the parent spends less time with the child. In these situations, children with CD do not have sufficient opportunities to learn to accurately identify their strong emotions or to develop necessary self-control skills. Elementary school progression As the children advance

2014 eMedicine Pediatrics

192. Maternal Chorioamnionitis (Treatment)

of the sepsis calculator in a population of well-appearing neonates (≥34 weeks' gestation) exposed to the clinical maternal diagnosis of chorioamnionitis would have substantially reduced the proportion of neonates undergoing laboratory tests and receiving antimicrobial agents. [ , , , ] Term neonates undergoing an evaluation to exclude sepsis who consistently appear well can probably go home with their mothers within 48 hours after birth. Septic-appearing neonates usually receive antibiotic therapy via (...) . [ ] When the mother is allergic to penicillin, she is given clindamycin if her GBS isolate is documented to be susceptible to clindamycin. Approximately 30% of GBS isolates in the United States were clindamycin resistant in 2010, and the proportion varies by country. If, however, clindamycin susceptibility testing has not been performed, vancomycin should be administered instead. [ ] Obstetric management influencing neonatal outcome When acute chorioamnionitis is evident, delivery must be expedited

2014 eMedicine Pediatrics

193. Glucocorticoid Therapy and Cushing Syndrome (Overview)

. Yanovski JA, Cutler GB Jr, Chrousos GP, Nieman LK. Corticotropin-releasing hormone stimulation following low-dose dexamethasone administration. A new test to distinguish Cushing's syndrome from pseudo-Cushing's states. JAMA . 1993 May 5. 269(17):2232-8. . Media Gallery Diagnosis of Cushing syndrome. Etiology of Cushing syndrome. Physical findings in Cushing syndrome. of 3 Tables ] Table 1. Glucocorticoid Equivalencies [ ] Type Drug Dose Relative Glucocorticoid Potency Relative Mineralocorticoid Potency (...) and industry to maximize their beneficial effects while minimizing their adverse effects. Thus, many synthetic compounds with glucocorticoid activity have been manufactured and tested. Alterations of the basic steroid nucleus and its side groups give rise to the pharmacologic differences between these chemicals. Such changes may affect the bioavailability of these steroid compounds, including their GI absorption; parenteral distribution; plasma half-life; their metabolism in the liver, fat, or target

2014 eMedicine Pediatrics

194. Conduct Disorder (Treatment)

in the anterior cingulate cortex (ACC), orbitofrontal cortices (OFC), and dorsolateral prefrontal cortex (DLPFC) that worsens over time due to dysphoria activation of brain stress systems and increases in corticotropin-releasing factor (CRF). [ ] Areas deep in the brain, especially the amygdala and insula, appear to exhibit abnormal function reflected in overall decreases in resting state connectivity and smaller overall size. [ ] This decrease in brain structure and functionality is also seen in youth (...) and community. Parents become reluctant to take their child with CD out in public because they fear an uncontrollable incident with the child. After increased negative interaction with the child, parental stimulation may decrease as the parent spends less time with the child. In these situations, children with CD do not have sufficient opportunities to learn to accurately identify their strong emotions or to develop necessary self-control skills. Elementary school progression As the children advance

2014 eMedicine Pediatrics

195. Hypopituitarism (Treatment)

, and therefore, hyperpigmentation does not take place. Patients with secondary adrenal insufficiency usually are eukalemic. This differs from primary adrenal insufficiency, in which patients develop hyponatremia and hyperkalemia. Aldosterone secretion is not affected, as it does not depend on corticotropin but instead on the renin-angiotensin axis. Thyroid-stimulating hormone deficiency Secondary due to decreased TSH (also known as thyrotropin) exhibits symptoms identical to those of primary thyroid disease (...) , hypopituitarism is difficult to diagnosis in the acutely decompensated or the stable patient. Laboratory and radiographic tests are necessary to confirm the diagnosis. A variety of diagnostic laboratory tests can be used, but significant controversy exists regarding which tests are ideal. Because many specific endocrine tests are not rapidly available in the emergency department (ED) setting, immediate confirmation of hypopituitarism may not be practical. Clinical suspicion by history and clinical examination

2014 eMedicine Emergency Medicine

196. Hypopituitarism (Overview)

, and therefore, hyperpigmentation does not take place. Patients with secondary adrenal insufficiency usually are eukalemic. This differs from primary adrenal insufficiency, in which patients develop hyponatremia and hyperkalemia. Aldosterone secretion is not affected, as it does not depend on corticotropin but instead on the renin-angiotensin axis. Thyroid-stimulating hormone deficiency Secondary due to decreased TSH (also known as thyrotropin) exhibits symptoms identical to those of primary thyroid disease (...) , hypopituitarism is difficult to diagnosis in the acutely decompensated or the stable patient. Laboratory and radiographic tests are necessary to confirm the diagnosis. A variety of diagnostic laboratory tests can be used, but significant controversy exists regarding which tests are ideal. Because many specific endocrine tests are not rapidly available in the emergency department (ED) setting, immediate confirmation of hypopituitarism may not be practical. Clinical suspicion by history and clinical examination

2014 eMedicine Emergency Medicine

197. Anxiety (Overview)

response to psychologic or pharmacologic interventions. Previous Next: Pathophysiology In the central nervous system (CNS), the major mediators of the symptoms of anxiety disorders appear to be norepinephrine, serotonin, dopamine, and gamma-aminobutyric acid (GABA). Other neurotransmitters and peptides, such as corticotropin-releasing factor, may be involved. Peripherally, the autonomic nervous system, especially the sympathetic nervous system, mediates many of the symptoms. [ ] Positron emission (...) , 22q, 4q31-q34, and probably 9q31 may be associated with the heritability of panic disorder phenotype. [ ] The cognitive theory regarding panic is that patients with panic disorder have a heightened sensitivity to internal autonomic cues (eg, tachycardia). Triggers of panic can include the following: Injury (eg, accidents, surgery) Illness Interpersonal conflict or loss Use of cannabis (can be associated with panic attacks, perhaps because of breath-holding) [ ] Use of stimulants, such as caffeine

2014 eMedicine Emergency Medicine

198. Pheochromocytoma (Diagnosis)

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

199. Pituitary Microadenomas (Diagnosis)

in all cases, especially if early in the course, a serum insulinlike growth factor-1 (IGF-1) level is recommended in all cases. Magnetic resonance imaging (MRI) studies have shown sensitivity and specificity of about 90% for secretory tumors. Enhancement with gadolinium diethylenetriaminepentaacetic acid (DTPA) improves the detection rate. Sensitivity for detection of corticotropin-secreting adenomas is much less (60-75%); diagnosis may require specialized tests such as petrosal sinus sampling (...) , although a variety of other cystic, vascular, neoplastic, hyperplastic, or inflammatory processes may present in a similar manner. Workup and management In the absence of symptoms or signs suggesting excess of specific hormones, the most cost-effective strategy is simply measurement of the prolactin level. [ ] If clinical suspicion of Cushing syndrome, acromegaly, or other hormone excess exists, order appropriate tests. Because excess growth hormone secretion may not produce the clinical phenotype

2014 eMedicine.com

200. Osteoporosis (Secondary) (Diagnosis)

difficulties may be evident, especially in patients with an altered center of gravity from severe kyphosis. [ ] Patients may have difficulty performing tandem gait and performing single limb stance. See for more detail. Diagnosis Baseline laboratory studies include the following: Complete blood count: May reveal anemia Serum chemistry levels: Usually normal in persons with primary osteoporosis Liver function tests Thyroid-stimulating hormone level: Thyroid dysfunction has been associated with osteoporosis (...) rotation) hip joint test Decreased weight-bearing on the fractured side or an antalgic gait pattern Patients with Colles fractures may have the following: Pain on movement of the wrist Dinner fork (bayonet) deformity Patients with pubic and sacral fractures may have the following: Marked pain with ambulation Tenderness to palpation, percussion, or both With sacral fractures, pain with physical examination techniques used to assess the sacroiliac joint (eg, FABER, Gaenslen, or squish test) Balance

2014 eMedicine.com

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