Phenylketonuria - Study guides, Revision notes & Summaries

1) A nurse is assisting a child who has multiple closed fractures of the lower extremities due to a motor-vehicle crash. The nurse should monitor the child for which of the following complications during the first 24 hr after the injury occurred? a. Osteomyelitis b. Compartment syndrome c. Volkmann ischemic contracture d. Renal calculi - b. Compartment syndrome 2) A nurse is teaching the parent of a toddler who has phenylketonuria about meal planning. Which of the following informat...

ATI Capstone Maternal Newborn Assessment Updated 2023 1. A nurse is performing a vaginal exam on a client who is in active labor. The nurse notes the umbilical cord protruding through the cervix. Which of the following actions should the nurse take? Administer oxytocin to the client via intravenous infusion. Apply oxygen at 2 L/min via nasal cannula. Prepare for insertion of an intrauterine pressure catheter. Assist the client into the knee-chest position. 2. A nurse is assessing a new...

describe what happens in general in phenylketonuria (enzyme involved, aa involved, etc.) correct answers protein from diet or from breakdown of muscle is metabolized into aa one of these aa, phenylalanine, is converted to tyrosine by the PAH enzyme defective PAH enzyme = accumulation of phenylalanine = phenylketonuria (PKU) what are some clinical features of classical untreated PKU correct answers developmental delay and neurological/behavioral symptoms sever mental retardation, hyp...

Autosomal recessive disorder of L-phenylalanine metabolism; Caused by a defect in a single enzyme: phenylalanine hydroxylase (PAH) correct answers what's PKU 1:10,000 worldwide with large national variability correct answers worldwide incidence? Turkey correct answers country with highest pku prevalence L-Phe --(PAH)--> L-Tyr --> L-DOPA --> Dopamine --> NorEpinephrine --> Epinephrine correct answers pathway of intermediates from L-Phe to Epi hydroxylated to tyrosine; ...

MATERNAL NEWBORN ATI COMPREHENSIVE EXAM QUESTIONS AND ANSWERS UPDATED 2024/2025 (100% Verified) Urinary frequency usually disappears at about 12 weeks of gestation but returns near term as the enlarging uterus presses on the bladder. It can also worsen following fetal descent. A nurse in a prenatal clinic overhears a newly licensed nurse discussing conception with a client. Which of the following statements by the newly licensed nurse requires intervention by the nurse? - ANSWER "Implantati...

You're requested to consult on a female newborn whose newborn screen was flagged for elevated phenylalanine. You ordered quantitative plasma amino acid which showed phenylalanine concentration of 1540 µmol/L. Before giving a diagnosis of PKU, which condition should be ruled out? correct answers Defects in biopterin synthesis and recycling. -- LESS TREATABLE 2% individuals have hyperphenylalaninemia due to tetrahdrobiopterin (cofactor of PAH) deficiency which is a less treatable condition....

ABRET 2023 Exam | With Complete Questions & Answers (Graded A+) ABRET 2023 Exam | With Complete Questions & Answers (Graded A+) What info do you need to calculate sensitivity? - ANSWER - Deflection (mm) and Voltage (uV) Normal alpha in an adult should NOT be seen in the... - ANSWER - Frontal head region What disease is caused by a proteinaceous particle or prion? - ANSWER - Creutzfeldt-Jakob disease Syringomyelia is... - ANSWER - An abnormal cavity or cyst in the spinal cord Spin...

What is phenylketonuria? correct answers an inherited inability to metabolised phenylketonuria which if untreated causes brain and nerve damage what is phenylalanine? correct answers an essential amino acid that when injested is transformed to form tyrosine (used in protein synthesis. too little slows physical and intellectual growth but too much is toxic) What does teratogenic mean? correct answers relating to or causing developmental malformations What is microcephaly? correct answers...

Presentation pf PKU correct answers Developmental delay Microcephaly Eczema Unusual smell Enzyme affected in PKU correct answers Lack of phenylalanine hydroxylase Most common inborn error in protein metabolism correct answers PKU What is elevated in PKU correct answers Phenylalanine Why is phenylalanine raised in PKU correct answers PAH deficiency Cofactor deficiency BH4 Liver disease Galactosaemia Heritability of PKU correct answers AR Chromosome involved in PKU correct ...

When preparing to obtain a neonatal screening test for phenylketonuria (PKU), the neonate must have received which of the following to ensure reliable results? 1. A feeding of an iron-rich formula. 2. Nothing by mouth for 4 hours before the test. 3. Initial formula or breast milk at least 24 hours before the test. 4. A feeding of glucose water. correct answers 3. PKU is an autosomal recessive disorder involving the absence of an enzyme needed to metabolize the essential amino acid, phenylal...