Vanillylmandelic acid (VMA) is excreted in urine in which of the following condition?
Correct Answer: Pheochromocytoma
Description: (C) Pheochromocytoma[?]ODOUR:oMisty/Mousy Odour of urine - PhenylketonuriaoFruity/Sweet odour - Presence of ketones.oPungent smell - Presence of bacterial / specimen contaminated with bacteria.oOdour of sweaty feet - Isovaleric acidemiaoMaple syrup - Maple syrup urine diseaseoFishy odour / Rancid butter - HypermethioninemiaMETABOLIC DISORDERS WITH ABNORMAL URINE ODORInborn errors of metabolismUrine odorGlutaric acidemia 2Sweaty feet, acridHawkinsinuriaSwimming PoolIsovaleric acidemiaSweaty feet, acridMSUDMaple syrupHypermethioninemiaBoiled cabbageMultiple carboxylase deficiencyTomcat urineOasthouse urine diseaseBuilding for drying hopsPKUMousy or mustyTrimethylaminuriaRotting fishTyrosinemiaBoiled cabbage, rancid butter INBORN ERRORS OF AMINO ACID METABOLISM ASSOCIATED WITH ABNORMAL ODORInborn error of metabolismUrine odorGultaric AcidemiaSweaty feetMaple syrup urine diseaseMaple syrupHypermethioninemiaBoiled cabbagePhenylketonuriaMousy or mustyTrimethylaminuriaRotten fish OdorObserved InFruity smellDiabetes mellitus Type 1Mousy odorPhenylketonuriaFishyUTIAmmonical odorUTI caused by pseudomonas & proteusBurnt SugarMaple syrup urine disease[?]BIOCHEMICAL TESTING:oPheochromocytomas & paragangliomas synthesize and store catecholamines, which include norepinephrine (noradrenaline), epinephrine (adrenaline), and dopamine.oElevated plasma and urinary levels of catecholamines and the methylated metabolites, metanephrines, are the cornerstone for the diagnosis.oCatecholamines and metanephrines can be measured by using different methodsoIn a clinical context suspicious for pheochromocytoma, when values are increased three times the upper limit of normal, a pheochromocytoma is highly likely regardless of the assay used.[?]VMA is the end product of catabolism of catecholamines.oIn pheochromocytoma and neuroblastoma there is excessive synthesis of catecholamines which causes enhanced synthesis of VMA and its excretion in the urine.oVMA is the urinary product of both epinephrine and nor-epinephrine.oIt is a good screening test for pheochromocytoma, and is also used to diagnose and follow up neuroblastoma and ganglioneuroma.oPheochromocytoma (PCC) is a neuroendocrine tumor of the medulla of the adrenal glands (originating in the chromaffin cells), or extra-adrenal chromaffin tissue that failed to involute after birth, that secretes high amounts of catecholamines,omostly nor-epinephrine, plus epinephrine to a lesser extent.Other Options[?]Alkaptonuria is a rare inherited genetic disorder in which the body cannot process the amino acids phenylalanine and tyrosine, which occur in protein.[?]Phenylketonuria (PKU) is an inherited disorder that increases the levels of a substance called phenylalanine in the blood. Phenylalanine is a building block of proteins (an amino acid) that is obtained through the diet.[?]Diabetic ketoacidosis (DKA) is a life-threatening condition that develops when cells in the body are unable to get the sugar (glucose) they need for energy because there is not enough insulin. When the sugar cannot get into the cells, it stays in the blood.
Category:
Biochemistry
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