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Phlebotomy
0%
0/4
Partial exchange transfusion with hydration
75%
3/4
Fluid resuscitation
25%
1/4
Hydroxyurea
Interferon alpha
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The baby presents with hemotcrit of 72% and clinical signs consistent with neonatal polycythemia rubra vera (p vera). The optimal treatment includes hydration and partial exchange transfusion (PET). P vera of the newborn is defined as a hematocrit greater than 65%. Symptoms of p vera are related to hyperviscosity and include lethargy, cyanosis, tremors, seizures, apnea, respiratory distress, jitteriness, and irritability. This is more common in infants of diabetic mothers and instances of delayed cord clamping, twin-twin transfusion, Beckwith-Wiedemann syndrome, and excess placental transfusion. Treatment of neonatal p vera includes hydration and PET. Dempsey et al. review PET as a treatment for neonatal hyperviscosity due to p vera. They acknowledge that the standard treatment for neonatal p vera is PET however, they question the long term benefits and outcomes attributed to PET treatment. They conclude that when deciding treatment for p vera in patients with minor symptoms or asymptomatic patients the provider should take into account the lack of evidence of the effectiveness of PET. However, studies have demonstrated benefit in patients with severe neurologic symptoms. Sarkar et al. explain that polycythemia and hyperviscosity cause pathology by changing blood flow in specific organs. Although there is no data that supports the use of PET for treatment it continues to be used as treatment for symptomatic and asymptomatic neonates. Further, they explain that crystalloids are as effective as colloids for use in PET and should be used because of their lower cost and availability. Incorrect Answers: Answer 1: Phlebotomy is a possible treatment for adults with p vera but is not used for neonates. Answer 3: Fluid resuscitation is part of the optimal treatment however, alone will not be enough to reduce the hematocrit to a safe level. Partial exchange transfusion is necessary to reduce the hematocrit to a safe level in the symptomatic neonate. Answer 4: Hydroxyurea is used to treat adult p. vera NOT neonatal p vera. Hydroxyurea treats p. vera by reducing circulating platelets and red blood cells. Answer 5: Interferon alpha is used to treat adult p. vera NOT neonatal p vera. Interferon alpha reduces bone marrow production of red blood cells.
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