Neonatal Hepatitis and Inborn Errors of Metabolism Quiz

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39 Questions

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

Every 3-6 hours

When should symptomatic infants be treated with IV glucose?

When blood glucose is less than 2.6 mmol/L

Why are small for gestational age (SGA) infants and preterm infants at risk for neonatal hypoglycemia?

Inadequate glycogen stores

What is the suggested duration for glucose screening in infants of diabetic mothers (IDM) and large for gestational age (LGA) infants?

12 hours after birth

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

Every 30 minutes

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

Enteral supplementation

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

Transient hyperinsulinemia

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

'Vulnerability period' has not elapsed

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

Hepatitis B

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

High pitched cry

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

Urine

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

Hypoglycemia

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

Slowly removing and replacing the baby's blood with fresh donor blood

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

Bulging fontanelle

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

Blood exchange transfusion

Which source of energy does the fetus primarily depend on?

Glucose

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

Glucose

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

50%

What is responsible for regulating glucose supply in the brain?

[Plasma]

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

Shift from glucose to a significant contribution by fat

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

Pre-term babies

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

[Glucose]

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

ABO incompatibility

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

[GLUT3]

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

G6PD

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

Spherocytosis

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

Inhibiting Glucuronyl transferase

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

Crigler-Najjar syndrome type 1

Which condition is associated with hypothyroidism in neonates?

Inadequate feeds/Breastfeeding jaundice

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

Biliary atresia

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

Biliary atresia

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

Seizure disorders

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

Coma and hypothermia

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

Unconjugated bilirubin is non-toxic, while conjugated bilirubin is neurotoxic.

When does physiological hyperbilirubinemia typically occur in infants?

2-3 days of life

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

Neurodevelopmental delay

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

Increased red blood cell (RBC) lifespan

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

Cyanosis

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

It is neurotoxic

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

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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