Neonatal Hepatitis and Inborn Errors of Metabolism Quiz
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Questions and Answers

What is the recommended glucose screening protocol for asymptomatic at-risk infants?

  • Every 12 hours
  • Every 30 minutes
  • Every 2 hours
  • Every 3-6 hours (correct)
  • When should symptomatic infants be treated with IV glucose?

  • When blood glucose is less than 2.6 mmol/L (correct)
  • When blood glucose is less than 2.0 mmol/L
  • When blood glucose is less than 3.0 mmol/L
  • Only when blood glucose is less than 1.8 mmol/L
  • Why are small for gestational age (SGA) infants and preterm infants at risk for neonatal hypoglycemia?

  • Hyperinsulinemia
  • Overproduction of insulin
  • Inadequate glycogen stores (correct)
  • High blood glucose levels in utero
  • What is the suggested duration for glucose screening in infants of diabetic mothers (IDM) and large for gestational age (LGA) infants?

    <p>12 hours after birth</p> Signup and view all the answers

    What is the recommended frequency for blood glucose checks in infants before feeds during the initial assessment?

    <p>Every 30 minutes</p> Signup and view all the answers

    What intervention is suggested for asymptomatic infants with blood glucose levels between 1.8-2.5 mmol/L?

    <p>Enteral supplementation</p> Signup and view all the answers

    Why are infants of diabetic mothers (IDM) at risk of hypoglycemia?

    <p>Transient hyperinsulinemia</p> Signup and view all the answers

    What is the rationale for continuing glucose screening in large for gestational age (LGA) infants for 12 hours after birth?

    <p>'Vulnerability period' has not elapsed</p> Signup and view all the answers

    What is the most common type of infectious hepatitis that can cause neonatal jaundice?

    <p>Hepatitis B</p> Signup and view all the answers

    Which of the following is NOT an early sign of kernicterus in neonates?

    <p>High pitched cry</p> Signup and view all the answers

    What is the primary excretory route of photooxidation products of bilirubin after phototherapy?

    <p>Urine</p> Signup and view all the answers

    Which of the following is a complication associated with exchange transfusion in neonates?

    <p>Hypoglycemia</p> Signup and view all the answers

    In exchange transfusion for neonatal jaundice, what does the process involve?

    <p>Slowly removing and replacing the baby's blood with fresh donor blood</p> Signup and view all the answers

    Which of the following signs is indicative of severe late-stage kernicterus in neonates?

    <p>Bulging fontanelle</p> Signup and view all the answers

    What treatment modality is considered if neonatal jaundice is severe and does not respond adequately to phototherapy?

    <p>Blood exchange transfusion</p> Signup and view all the answers

    Which source of energy does the fetus primarily depend on?

    <p>Glucose</p> Signup and view all the answers

    What is the main source of energy for cerebral cells in the brain?

    <p>Glucose</p> Signup and view all the answers

    During the 1st day after birth, what percentage of a baby's endogenous glucose comes from glycogenolysis?

    <p>50%</p> Signup and view all the answers

    What is responsible for regulating glucose supply in the brain?

    <p>[Plasma]</p> Signup and view all the answers

    What metabolic shift does the respiratory quotient change from 1 to 0.8 suggest during the 1st hour of life?

    <p>Shift from glucose to a significant contribution by fat</p> Signup and view all the answers

    Which babies are more likely to have decreased glucose reserves, according to risk factors for neonatal hypoglycemia?

    <p>Pre-term babies</p> Signup and view all the answers

    What do breastfed term babies have lower concentrations of compared to formula-fed babies?

    <p>[Glucose]</p> Signup and view all the answers

    Which condition is associated with a higher risk of neonatal hyperbilirubinemia if the mother has O+ blood type?

    <p>ABO incompatibility</p> Signup and view all the answers

    Which transporter is expressed in the cerebellum and responsible for regulating glucose supply in the brain?

    <p>[GLUT3]</p> Signup and view all the answers

    Which enzyme defect is more commonly seen in Asian males and can lead to neonatal hyperbilirubinemia?

    <p>G6PD</p> Signup and view all the answers

    Which membrane abnormality is associated with osmotic fragility and can contribute to neonatal hyperbilirubinemia?

    <p>Spherocytosis</p> Signup and view all the answers

    What is the suggested mechanism by which breast milk may lead to neonatal hyperbilirubinemia?

    <p>Inhibiting Glucuronyl transferase</p> Signup and view all the answers

    Which liver enzyme defect is characterized by a total lack of glucuronyl transferase activity?

    <p>Crigler-Najjar syndrome type 1</p> Signup and view all the answers

    Which condition is associated with hypothyroidism in neonates?

    <p>Inadequate feeds/Breastfeeding jaundice</p> Signup and view all the answers

    Which anatomical condition should not be missed when evaluating neonates with conjugated hyperbilirubinemia?

    <p>Biliary atresia</p> Signup and view all the answers

    What is the darker, hyperpigmented urine and pale, acholic stools indicative of in neonates?

    <p>Biliary atresia</p> Signup and view all the answers

    What is a potential consequence associated with severe hypoglycemia in infants?

    <p>Seizure disorders</p> Signup and view all the answers

    In infants, what symptoms may indicate hypoglycemia and the need for immediate testing?

    <p>Coma and hypothermia</p> Signup and view all the answers

    What is the main difference between unconjugated and conjugated bilirubin with respect to neurotoxicity?

    <p>Unconjugated bilirubin is non-toxic, while conjugated bilirubin is neurotoxic.</p> Signup and view all the answers

    When does physiological hyperbilirubinemia typically occur in infants?

    <p>2-3 days of life</p> Signup and view all the answers

    What may persistent hypoglycemia (>2-3 hours) in infants lead to?

    <p>Neurodevelopmental delay</p> Signup and view all the answers

    What physiological mechanism contributes to the development of physiological hyperbilirubinemia in infants?

    <p>Increased red blood cell (RBC) lifespan</p> Signup and view all the answers

    Which of the following is a symptom associated with jaundice in infants?

    <p><strong>Cyanosis</strong></p> Signup and view all the answers

    What is the significance of unconjugated bilirubin crossing the blood-brain barrier?

    <p><strong>It is neurotoxic</strong></p> Signup and view all the answers

    Study Notes

    Glucose Screening Protocol

    • Asymptomatic at-risk infants: recommended glucose screening protocol
    • Symptomatic infants: IV glucose treatment when necessary

    Risk Factors for Neonatal Hypoglycemia

    • Small for gestational age (SGA) infants: at risk due to limited glycogen stores
    • Preterm infants: at risk due to immature glucose regulation
    • Infants of diabetic mothers (IDM): at risk due to hyperinsulinemia

    Glucose Screening Duration

    • Infants of diabetic mothers (IDM): glucose screening for 12-24 hours
    • Large for gestational age (LGA) infants: glucose screening for 12 hours

    Glucose Management

    • Asymptomatic infants with blood glucose levels between 1.8-2.5 mmol/L: feed and re-check
    • Infants before feeds: blood glucose checks every 1-2 hours during initial assessment

    Neonatal Jaundice

    • Most common type of infectious hepatitis that can cause neonatal jaundice: hepatitis A
    • Early sign of kernicterus in neonates: not irritability
    • Primary excretory route of photooxidation products of bilirubin after phototherapy: bile

    Exchange Transfusion

    • Complication associated with exchange transfusion in neonates: hypocalcemia
    • Process involves: replacing baby's blood with donor blood to remove bilirubin

    Kernicterus

    • Sign indicative of severe late-stage kernicterus in neonates: abnormal muscle tone
    • Treatment modality for severe neonatal jaundice: exchange transfusion

    Energy Metabolism

    • Fetus primarily depends on: glucose as energy source
    • Main source of energy for cerebral cells: glucose
    • 1st day after birth: 40-50% of baby's endogenous glucose comes from glycogenolysis
    • Regulator of glucose supply in the brain: GLUT-1 transporter

    Neonatal Hypoglycemia

    • Metabolic shift during the 1st hour of life: respiratory quotient change from 1 to 0.8 indicates glucose metabolism
    • Risk factors for neonatal hypoglycemia: SGA, IDM, preterm, and LGA infants
    • Breastfed term babies have lower concentrations of: glucose compared to formula-fed babies

    Neonatal Hyperbilirubinemia

    • Condition associated with a higher risk if the mother has O+ blood type: Rh incompatibility
    • Enzyme defect more commonly seen in Asian males: glucose-6-phosphate dehydrogenase (G6PD) deficiency
    • Membrane abnormality associated with osmotic fragility: hereditary spherocytosis
    • Suggested mechanism by which breast milk may lead to neonatal hyperbilirubinemia: increased enterohepatic circulation

    Liver Enzyme Defects

    • Liver enzyme defect characterized by a total lack of glucuronyl transferase activity: Crigler-Najjar syndrome
    • Condition associated with hypothyroidism in neonates: congenital hypothyroidism

    Conjugated Hyperbilirubinemia

    • Anatomical condition that should not be missed: biliary atresia
    • Darker, hyperpigmented urine and pale, acholic stools indicative of: conjugated hyperbilirubinemia

    Hypoglycemia Consequences

    • Potential consequence associated with severe hypoglycemia: brain damage
    • Symptoms that may indicate hypoglycemia: jitteriness, lethargy, and seizures
    • Main difference between unconjugated and conjugated bilirubin: unconjugated bilirubin is more lipophilic and neurotoxic
    • Physiological hyperbilirubinemia typically occurs in infants: 2-3 days after birth
    • Persistent hypoglycemia (>2-3 hours) in infants may lead to: brain damage

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    Description

    Test your knowledge on neonatal hepatitis, inborn errors of metabolism, and Kernicterus. Learn about different causes, symptoms, and treatment options for these conditions.

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