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Urine Vanillylmandelic Acid

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41. Neuroblastoma Screening

Neuroblastoma Screening Neuroblastoma Screening (PDQ®)—Health Professional Version - National Cancer Institute Menu Search Search Search Summary of Evidence Note: Separate PDQ summaries on and are also available. Intervention Screening, usually at age 6 months, for urine vanillylmandelic acid and homovanillic acid, which are metabolites of the hormones norepinephrine and dopamine. Benefits Based on solid evidence, screening for neuroblastoma does not lead to decreased mortality. Description (...) to provide information or advice for the primary prevention of this disease. It is generally thought that many neuroblastomas are present and detectable at birth, thereby allowing for detection of tumors by a single, once-in-a-lifetime screening test, such as those used for neonatal screening for noncancerous conditions (e.g., phenylketonuria). Screening is performed through biochemical tests for metabolites of norepinephrine and dopamine (i.e., vanillylmandelic acid [VMA], and homovanillic acid [HVA

2012 PDQ - NCI's Comprehensive Cancer Database

42. Neuroblastoma, Childhood

with neuroblastoma and a strong family history of neuroblastoma or clearly bilateral/multifocal neuroblastoma. Surveillance consists of the following:[ ] Abdominal ultrasonography. Quantitative, normalized assessment of urinary catecholamines, such as urine vanillylmandelic acid (VMA) and homovanillic acid (HVA), by gas chromatography and mass spectroscopy (can be a random urine collection normalized for urine creatinine). Chest x-ray. Surveillance begins at birth or at diagnosis of neuroblastoma predisposition (...) .) Urine catecholamine metabolites: Urinary excretion of the catecholamine metabolites VMA and HVA per milligram of excreted creatinine is measured before therapy. Collection of urine for 24 hours is not needed. If they remain elevated, these markers can be used to suggest the persistence of disease. In contrast to urine, serum catecholamines are not routinely used in the diagnosis of neuroblastoma except in unusual circumstances. Biopsy: Tumor tissue is often needed to obtain all the biological data

2012 PDQ - NCI's Comprehensive Cancer Database

43. Organic Solvents (Overview)

crosslinking in gamma-diketone neuropathy: in vitro and in vivo studies using the seaworm myxicola infundibulum. Neurotoxicology . 1989 Winter. 10(4):743-56. . Stanosz S, Kuligowski D, Pieleszek A, Zuk E, Rzechula D, Chlubek D. Concentration of dopamine in plasma, activity of dopamine beta-hydroxylase in serum and urinary excretion of free catecholamines and vanillylmandelic acid in women chronically exposed to carbon disulphide. Int J Occup Med Environ Health . 1994. 7(3):257-61. . Suwanlaong K (...) for polyvinylchloride resins for plastics, floor coverings, upholstery, appliances, packaging Xylene Paint, lacquers, varnishes, inks, dyes, adhesives, cements, fixative for pathologic specimens Table 2. Exposure levels Believed Safe for Workers Compound Urine Blood Expired Air Acetone Acetone, formic acid 100 mg/L Acetone Acetone Benzene Total phenol 50 mg/g at the end of the shift, trans-trans- muconic acid Benzene Benzene before shift, 0.08 ppm; end exhaled, 0.12 ppm Carbon disulfide 2-TTCA 5 mg/g * Carbon

2014 eMedicine.com

44. Pheochromocytoma (Overview)

and metabolism of catecholamines: Tyrosine → dihydroxyphenylalanine (DOPA) → dopamine (DA) → norepinephrine + epinephrine → homovanillic acid (HVA) + vanillylmandelic acid (VMA) The biosynthesis and storage of catecholamines in chromaffin cell tumors may differ from the biosynthesis and storage in the normal medulla. However, the granules are morphologically and functionally similar to the granules from the adrenal medulla. The increase in tissue turnover suggests an alteration in the regulation (...) . . Pamporaki C, Därr R, Bursztyn M, et al. Plasma-free vs deconjugated metanephrines for diagnosis of phaeochromocytoma. Clin Endocrinol (Oxf) . 2013 Mar 5. . Hickman PE, Leong M, Chang J, Wilson SR, McWhinney B. Plasma free metanephrines are superior to urine and plasma catecholamines and urine catecholamine metabolites for the investigation of phaeochromocytoma. Pathology . 2009 Feb. 41(2):173-7. . Boyle JG, Davidson DF, Perry CG, Connell JM. Comparison of diagnostic accuracy of urinary free

2014 eMedicine Pediatrics

45. Radiographic Evaluation of the Pediatric Urinary Tract (Follow-up)

is suspected, perform the appropriate urine studies (homovanillic acid [HVA] and vanillylmandelic acid [VMA]). Cloaca Retrograde genitography and VCUG are helpful in outlining the level of confluence of the urethra, vagina, and colon when surgery is being planned. Baseline renal US is also necessary because renal agenesis, reflux, and megaureter are common associated findings. MRI of the pelvis is an important ancillary study in complex cloacal cases. Cystic kidney disease Multicystic dysplastic kidneys (...) . Dimercaptosuccinic acid scanning DMSA is bound in the cortex of the kidney after injection, revealing relative renal function. It is the criterion test for renal scarring. [ ] A bladder catheter is not required for DMSA scanning, because DMSA is not excreted into the urine. The "top-down" approach has suggested that the DMSA scan be used as the primary test in patients with UTI, in that it selects for patients with renal injury. [ ] Limitations of DMSA scanning include intermittent unavailability and the need

2014 eMedicine Pediatrics

46. Horner Syndrome (Diagnosis)

a significant role in the diagnosis and management of Horner syndrome. However, depending on the localization and suspected etiology, certain laboratory tests may be considered, as follows: Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) in pediatric Horner syndrome to rule out neuroblastoma Complete blood count (CBC) Fluorescent treponemal antibody absorption (FTA-ABS) test Venereal Disease Research Laboratory (VDRL) test (for rare cases of syphilitic basilar meningitis or when (...) The drops are relatively expensive The test can yield equivocal results Cocaine metabolites may be detected in urine Topical apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ , , ] Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong alpha2-agonist. [ , , , ] Typically given in a 0.5% or 1% solution

2014 eMedicine.com

47. von Hippel-Lindau Disease (Diagnosis)

catecholamines and urinary catecholamine metabolites (inclusive of vanillylmandelic acid [VMA], fractionated metanephrines [especially normetanephrines], and total catecholamines) is performed to detect pheochromocytomas, even when hypertension is absent. Screening for pheochromocytomas and paragangliomas should include measurements of plasma-free metanephrines or urinary fractionated metanephrines. The 2014 Endocrine Society Guidelines for obtaining sera and urine samples are as follows: For a blood sample (...) for glial fibrillary acid protein and neuron-specific enolase. [ ] Retinal capillary hemangiomas, usually supplied by large dilated feeder vessels, may occur in any part of the retina. Serum leakage from these vessels and hemangiomas leads to retinal exudates. Organized fibroglial bands with traction retinal detachment and vitreous hemorrhage may occur, along with potential complications such as glaucoma or permanent vision loss. [ ] (See the image below.) von Hippel-Lindau disease. Spectral display

2014 eMedicine.com

48. Horner Syndrome (Treatment)

a significant role in the diagnosis and management of Horner syndrome. However, depending on the localization and suspected etiology, certain laboratory tests may be considered, as follows: Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) in pediatric Horner syndrome to rule out neuroblastoma Complete blood count (CBC) Fluorescent treponemal antibody absorption (FTA-ABS) test Venereal Disease Research Laboratory (VDRL) test (for rare cases of syphilitic basilar meningitis or when (...) The drops are relatively expensive The test can yield equivocal results Cocaine metabolites may be detected in urine Topical apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ , , ] Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong alpha2-agonist. [ , , , ] Typically given in a 0.5% or 1% solution

2014 eMedicine.com

49. Organic Solvents (Treatment)

, Chlubek D. Concentration of dopamine in plasma, activity of dopamine beta-hydroxylase in serum and urinary excretion of free catecholamines and vanillylmandelic acid in women chronically exposed to carbon disulphide. Int J Occup Med Environ Health . 1994. 7(3):257-61. . Suwanlaong K, Phanthumchinda K. Neurological manifestation of methyl bromide intoxication. J Med Assoc Thai . 2008 Mar. 91(3):421-6. . Urban P, Lukás E. Visual evoked potentials in rotogravure printers exposed to toluene. Br J Ind Med (...) Believed Safe for Workers Compound Urine Blood Expired Air Acetone Acetone, formic acid 100 mg/L Acetone Acetone Benzene Total phenol 50 mg/g at the end of the shift, trans-trans- muconic acid Benzene Benzene before shift, 0.08 ppm; end exhaled, 0.12 ppm Carbon disulfide 2-TTCA 5 mg/g * Carbon disulfide Carbon disulfide ETO None None None N- hexane 2,5-hexanediol 5 mg/g at the end of the shift, 2-hexanol, total metabolites N- hexane N- hexane Hydrogen sulfide None None None Methane None None None

2014 eMedicine.com

50. von Hippel-Lindau Disease (Treatment)

catecholamines and urinary catecholamine metabolites (inclusive of vanillylmandelic acid [VMA], fractionated metanephrines [especially normetanephrines], and total catecholamines) is performed to detect pheochromocytomas, even when hypertension is absent. Screening for pheochromocytomas and paragangliomas should include measurements of plasma-free metanephrines or urinary fractionated metanephrines. The 2014 Endocrine Society Guidelines for obtaining sera and urine samples are as follows: For a blood sample (...) for glial fibrillary acid protein and neuron-specific enolase. [ ] Retinal capillary hemangiomas, usually supplied by large dilated feeder vessels, may occur in any part of the retina. Serum leakage from these vessels and hemangiomas leads to retinal exudates. Organized fibroglial bands with traction retinal detachment and vitreous hemorrhage may occur, along with potential complications such as glaucoma or permanent vision loss. [ ] (See the image below.) von Hippel-Lindau disease. Spectral display

2014 eMedicine.com

51. Adrenal Carcinoma (Overview)

AC typically present with any of the following: Fever Weight loss Abdominal pain and tenderness Back pain Abdominal fullness Symptoms related to metastases See for more detail. Diagnosis Laboratory studies These include the following: Serum glucose Serum cortisol Serum adrenal androgen Urine adrenal hormone Urine vanillylmandelic acid (VMA) Urine homovanillic acid (HVA) levels Imaging studies Adrenal computed tomography (CT) scanning and magnetic resonance imaging (MRI) are the imaging studies (...) , TP57 , TP16 , H19 , retinoblastoma gene, APC gene, and various deoxyribonucleic acid (DNA) repair ̶ enzyme genes Inhibition of senescence and/or apoptosis: Mutations involving telomerase and/or BCL-2 genes Changes in adrenocortical tissue-specific factors: Mutations involving the genes for StaR, SF-1 (steroidogenic factor), and Dax-1 transcription factor Aberrant expression of receptors to normal adrenocorticotropic agents and ligands: Adrenocorticotropic hormone, angiotensin 2, catecholamines

2014 eMedicine.com

52. Horner Syndrome (Overview)

a significant role in the diagnosis and management of Horner syndrome. However, depending on the localization and suspected etiology, certain laboratory tests may be considered, as follows: Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) in pediatric Horner syndrome to rule out neuroblastoma Complete blood count (CBC) Fluorescent treponemal antibody absorption (FTA-ABS) test Venereal Disease Research Laboratory (VDRL) test (for rare cases of syphilitic basilar meningitis or when (...) The drops are relatively expensive The test can yield equivocal results Cocaine metabolites may be detected in urine Topical apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ , , ] Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong alpha2-agonist. [ , , , ] Typically given in a 0.5% or 1% solution

2014 eMedicine.com

53. Horner Syndrome (Overview)

a significant role in the diagnosis and management of Horner syndrome. However, depending on the localization and suspected etiology, certain laboratory tests may be considered, as follows: Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) in pediatric Horner syndrome to rule out neuroblastoma Complete blood count (CBC) Fluorescent treponemal antibody absorption (FTA-ABS) test Venereal Disease Research Laboratory (VDRL) test (for rare cases of syphilitic basilar meningitis or when (...) The drops are relatively expensive The test can yield equivocal results Cocaine metabolites may be detected in urine Topical apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ , , ] Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong alpha2-agonist. [ , , , ] Typically given in a 0.5% or 1% solution

2014 eMedicine.com

54. von Hippel-Lindau Disease (Overview)

catecholamines and urinary catecholamine metabolites (inclusive of vanillylmandelic acid [VMA], fractionated metanephrines [especially normetanephrines], and total catecholamines) is performed to detect pheochromocytomas, even when hypertension is absent. Screening for pheochromocytomas and paragangliomas should include measurements of plasma-free metanephrines or urinary fractionated metanephrines. The 2014 Endocrine Society Guidelines for obtaining sera and urine samples are as follows: For a blood sample (...) for glial fibrillary acid protein and neuron-specific enolase. [ ] Retinal capillary hemangiomas, usually supplied by large dilated feeder vessels, may occur in any part of the retina. Serum leakage from these vessels and hemangiomas leads to retinal exudates. Organized fibroglial bands with traction retinal detachment and vitreous hemorrhage may occur, along with potential complications such as glaucoma or permanent vision loss. [ ] (See the image below.) von Hippel-Lindau disease. Spectral display

2014 eMedicine.com

55. Multiple Endocrine Neoplasia, Type 2 (Treatment)

occurred in 11 of 717 (1.5%) patients after a mean follow-up of 13 years. [ ] Previous Next: Long-Term Monitoring Patients should be monitored on a lifelong basis for evidence of recurrent disease. After an initial follow-up visit, patients may be evaluated at 6 months, then yearly if they are asymptomatic. Evaluations should include the following: Physical examination 24-hour urine catecholamine, metanephrine and vanillylmandelic acid levels CEA level Calcitonin level Serum calcium level. If recurrent (...) . Deterrence/prevention Start annual 24-hour urine collections for catecholamine concentrations to detect pheochromocytoma at the earliest age possible. Begin annual testing of serum calcium and PTH levels at age 10 years. Outpatient care Monitor patients for recurrence of medullary thyroid carcinoma with calcitonin, and CEA, and consider provocative calcitonin testing. Perform annual screening for hyperparathyroidism with serum calcium and PTH levels in MEN2A patients. Obtain urinary catecholamine levels

2014 eMedicine.com

56. Organic Solvents (Follow-up)

, Rzechula D, Chlubek D. Concentration of dopamine in plasma, activity of dopamine beta-hydroxylase in serum and urinary excretion of free catecholamines and vanillylmandelic acid in women chronically exposed to carbon disulphide. Int J Occup Med Environ Health . 1994. 7(3):257-61. . Suwanlaong K, Phanthumchinda K. Neurological manifestation of methyl bromide intoxication. J Med Assoc Thai . 2008 Mar. 91(3):421-6. . Urban P, Lukás E. Visual evoked potentials in rotogravure printers exposed to toluene. Br (...) . Exposure levels Believed Safe for Workers Compound Urine Blood Expired Air Acetone Acetone, formic acid 100 mg/L Acetone Acetone Benzene Total phenol 50 mg/g at the end of the shift, trans-trans- muconic acid Benzene Benzene before shift, 0.08 ppm; end exhaled, 0.12 ppm Carbon disulfide 2-TTCA 5 mg/g * Carbon disulfide Carbon disulfide ETO None None None N- hexane 2,5-hexanediol 5 mg/g at the end of the shift, 2-hexanol, total metabolites N- hexane N- hexane Hydrogen sulfide None None None Methane None

2014 eMedicine.com

57. Multiple Endocrine Neoplasia, Type 2 (Follow-up)

occurred in 11 of 717 (1.5%) patients after a mean follow-up of 13 years. [ ] Previous Next: Long-Term Monitoring Patients should be monitored on a lifelong basis for evidence of recurrent disease. After an initial follow-up visit, patients may be evaluated at 6 months, then yearly if they are asymptomatic. Evaluations should include the following: Physical examination 24-hour urine catecholamine, metanephrine and vanillylmandelic acid levels CEA level Calcitonin level Serum calcium level. If recurrent (...) . Deterrence/prevention Start annual 24-hour urine collections for catecholamine concentrations to detect pheochromocytoma at the earliest age possible. Begin annual testing of serum calcium and PTH levels at age 10 years. Outpatient care Monitor patients for recurrence of medullary thyroid carcinoma with calcitonin, and CEA, and consider provocative calcitonin testing. Perform annual screening for hyperparathyroidism with serum calcium and PTH levels in MEN2A patients. Obtain urinary catecholamine levels

2014 eMedicine.com

58. von Hippel-Lindau Disease (Follow-up)

catecholamines and urinary catecholamine metabolites (inclusive of vanillylmandelic acid [VMA], fractionated metanephrines [especially normetanephrines], and total catecholamines) is performed to detect pheochromocytomas, even when hypertension is absent. Screening for pheochromocytomas and paragangliomas should include measurements of plasma-free metanephrines or urinary fractionated metanephrines. The 2014 Endocrine Society Guidelines for obtaining sera and urine samples are as follows: For a blood sample (...) for glial fibrillary acid protein and neuron-specific enolase. [ ] Retinal capillary hemangiomas, usually supplied by large dilated feeder vessels, may occur in any part of the retina. Serum leakage from these vessels and hemangiomas leads to retinal exudates. Organized fibroglial bands with traction retinal detachment and vitreous hemorrhage may occur, along with potential complications such as glaucoma or permanent vision loss. [ ] (See the image below.) von Hippel-Lindau disease. Spectral display

2014 eMedicine.com

59. Horner Syndrome (Follow-up)

a significant role in the diagnosis and management of Horner syndrome. However, depending on the localization and suspected etiology, certain laboratory tests may be considered, as follows: Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) in pediatric Horner syndrome to rule out neuroblastoma Complete blood count (CBC) Fluorescent treponemal antibody absorption (FTA-ABS) test Venereal Disease Research Laboratory (VDRL) test (for rare cases of syphilitic basilar meningitis or when (...) The drops are relatively expensive The test can yield equivocal results Cocaine metabolites may be detected in urine Topical apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ , , ] Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong alpha2-agonist. [ , , , ] Typically given in a 0.5% or 1% solution

2014 eMedicine.com

60. Horner Syndrome (Follow-up)

a significant role in the diagnosis and management of Horner syndrome. However, depending on the localization and suspected etiology, certain laboratory tests may be considered, as follows: Urine test (ie, vanillylmandelic acid [VMA] and homovanillic acid [HVA]) in pediatric Horner syndrome to rule out neuroblastoma Complete blood count (CBC) Fluorescent treponemal antibody absorption (FTA-ABS) test Venereal Disease Research Laboratory (VDRL) test (for rare cases of syphilitic basilar meningitis or when (...) The drops are relatively expensive The test can yield equivocal results Cocaine metabolites may be detected in urine Topical apraclonidine test The topical apraclonidine test is a practical and reliable alternative to the topical cocaine test; it is readily available and adequately sensitive (87%) and is currently the test of choice. [ , , ] Apraclonidine is an ocular hypotensive agent that acts as a weak alpha1-agonist and a strong alpha2-agonist. [ , , , ] Typically given in a 0.5% or 1% solution

2014 eMedicine.com

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