Maple syrup urine disease - Study guides, Class notes & Summaries

MT AAB Chemistry Which of the four elements in proteins differentiated this class of substances from carbohydrates and lipids? - ANSWER Nitrogen Which of the proteins is soluble in water? - ANSWER Albumin How are amino acids joined together to form a chainlike structure? - ANSWER Peptide bonding Proteins may become denatured when subjected to mechanical agitation, heat, or chemical treatment. What does denaturation of proteins refer to? - ANSWER Alteration of tertiary structure Whic...

SSS/BETS (small sharp waves / benign epileptiform transients of sleep) Low voltage, short duration, diphasic spikes with a steep descending limb. Usually seen in drowsiness and light sleep. SREDA (subclinical rhythmic electrographic discharges of adults) Sharply contoured theta activity in the posterior head region. A normal variant in older adults during wakefullness. 14 and 6 positive spikes 1-2 seconds of sharply contoured discharges in the posterior head regions in light sle...

IBCLC Exam Flashcards UPDATED 2023 Micrognathia Condition where jaw is undersized; common but can interfere with feeding and breathing PKU (phenylketonuria) Body does not produce enzyme to break-down amino acid phenylalanine and this builds-ups in blood; need low phe formula and CAN breastfeed Maple Syrup Disease Body is unable to break-down certain proteins/amino acids (leucine); sweet smelling urine. Need low protein diet/special formula i.e. Ketonex-1. BF CONTRAINDICATED Gal...

Irene Gold Boards Part 1-Biochemistry Exam Questions with 100% Correct Answers How many a.a.'s are incorporated into proteins? - Answer ️️ -20 What structures make up an AA? - Answer ️️ -Amine group Acid group Hydrogen R-Group = defining group All a.a.'s are in L-form EXCEPT for what? - Answer ️️ -glycine Glycine is not in L-form because? - Answer ️️ -it doesn't contain a chiral carbon define chiral carbon - Answer ️️ -different compounds at each binding site All...

MLS ASCP Body Fluids Exam with Questions and Answers Urine handling ANSWER examine within 1 hour room temp and 2-4 hours refrigerated green/blue urine ANSWER indicate presence of biliverdin, medications containing dye, or pseudomonas infections Dark-brown/black urine ANSWER indicate presence of homogentisic acid (alkaptonuria) melanin Urine smells ANSWER Sulfur: asparagus metabolite Musty/mousy: PKU and some liver diseases Ammonia: old samples Sweet: maple syrup urine disease Pu...

What are the primary organic acid abnormalities that occur in carnitine deficiency? correct answers -adipate -suberate -ethylmalonate What patterns of abnormalities discriminate mitochondrial inefficiency from ammonia challenge as a cause of citric acid elevation? correct answers -high citrate, aconitate, isocitrate + orotate indicates ammonia challenges -wihtout orotate= mitochondrial inefficiency What patterns suggest a deficiency of CoQ10? correct answers -High succinate, fumarate, m...

Spectrophotometer correct answer: Measures concentration in a solution by calculating absorbance from the transmitted light Beer's law correct answer: Concentration of a substance is directly proportional to absorbed light or inversely proportional to the logarithm of transmitted light Fluorescence or fluorometry correct answer: The emited light of some substances is at a longer wavelength than the absorbed light Turbidimetry correct answer: Method that measures the scattered light...

Which of the four elements in proteins differentiated this class of substances from carbohydrates and lipids? Nitrogen Which of the proteins is soluble in water? Albumin How are amino acids joined together to form a chainlike structure? Peptide bonding Proteins may become denatured when subjected to mechanical agitation, heat, or chemical treatment. What does denaturation of proteins refer to? Alteration of tertiary structure Which aminoacidopathy is NOT caused by...

Which of the following aminoacidopathies is NOT caused by an enzyme deficiency? Choose one answer. a. phenylketonuria b. alkaptonuria c. maple syrup disease d. cystinuria d. cystinuria What method is most commonly used to measure total protein? Choose one answer. a. dye binding b. Kjeldahl c. Biuret d. refractometry c. Biuret Which of the following is the preferred reagent for measuring albumin by the dye binding method because there is less interfering substances? Cho...

A mother expresses fear about changing her infant's diaper after he is circumcised. What does the woman need to be taught to take care of the infant when she gets home? - CORRECT ANSWERS Cleanse the penis gently with water and put petroleum jelly around the glans after each diaper change A patient with a history of gonorrheal infection has just delivered a baby. What immediate intervention should the nurse provide to the newborn to ensure safety? 1 Place the newborn in incubator. 2 ...