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Medical Science Optional daily answer writing practice for CSE 2024 – Jan 22

Topic: Respiratory distress syndrome, broncho – pneumonias, kernicterus

Question: A term neonate born to Rh negative mother is brought at 22 hours of life with jaundice, fever and one episode of Convulsion. ① what is the most-likely diagnosis and its basis? ② Enumerate the key investigations and principles of management. ③ List four important long term complications of this disease. ④ mention the preventive measures.

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Most Likely Diagnosis and Basis:

The most likely diagnosis is Rh incompatibility and hemolytic disease of the newborn (HDN).
Basis:
The neonate is born to an Rh-negative mother, indicating a potential Rh incompatibility.
The presence of jaundice (hyperbilirubinemia) within the first 24 hours of life suggests hemolysis and rapid bilirubin production.
Fever and convulsion are signs of severe hyperbilirubinemia and potential kernicterus, a condition where bilirubin crosses the blood-brain barrier and affects the brain, causing neurological damage.

  1. Key Investigations and Findings:

Complete blood count (CBC) with differential and reticulocyte count:
Findings: Anemia (low red blood cell count) due to hemolysis. Increased reticulocyte count indicates increased bone marrow activity in response to hemolysis.
Blood type and Rh factor of the neonate and mother:
Findings: Confirms Rh incompatibility (Rh-negative mother and Rh-positive neonate).
Direct antiglobulin test (DAT or Coombs’ test):
Findings: Positive DAT indicates antibodies bound to the neonate’s red blood cells, confirming immune-mediated destruction.
Serum bilirubin levels:
Findings: Elevated total and direct bilirubin levels, reflecting the severity of hyperbilirubinemia.
Liver function tests:
Findings: May show signs of liver dysfunction due to bilirubin overload.
Principles of Management:

Phototherapy:
Mainstay of treatment for jaundice.
Uses blue or white light to convert bilirubin into a form that can be excreted.
Exchange transfusion:
Indicated in severe cases with high bilirubin levels and risk of kernicterus.
Involves removing a portion of the neonate’s blood and replacing it with Rh-negative blood, reducing bilirubin levels and removing antibodies.
Intravenous immunoglobulin (IVIg):
Can be used in conjunction with phototherapy to reduce bilirubin levels and prevent further hemolysis.
Supportive care:
Maintain hydration and electrolyte balance.
Monitor vital signs and neurological status closely.

  1. Long Term Complications and Findings:

Kernicterus:
Findings: Bilirubin-induced damage to the brain, leading to neurological problems such as athetoid cerebral palsy, hearing loss, and intellectual disabilities.
Anemia:
Findings: Chronic hemolysis can result in persistent anemia.
Hepatic dysfunction:
Findings: Severe hyperbilirubinemia can cause liver damage and dysfunction.
Speech and language difficulties:
Findings: May occur as a result of kernicterus.

  1. Preventive Measures:

Rh prophylaxis:
Administration of Rh immunoglobulin (RhIg) to Rh-negative mothers during pregnancy and after delivery prevents Rh sensitization and subsequent HDN in future pregnancies.
Proper prenatal care:
Regular monitoring of maternal and fetal well-being, including blood type and antibody screening, helps identify potential risks and allows for timely intervention.

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