Updated: 12/25/2021

Neonatal Polycythemia Rubra Vera

0%
Topic
Review Topic
0
0
N/A
N/A
Questions
3
0
0
0%
0%
Evidence
2
0
0
Topic
Images
https://upload.medbullets.com/topic/120623/images/screen shot 2015-10-29 at 11.33.12 am.jpg
  • Snapshot
    • A 2-day-old newborn presents with cyanosis and irritability. His mother had gestational diabetes, which resulted in a difficult delivery due to the baby’s large size. Delivery was further complicated by delayed clamping of the umbilical cord. On physical exam, the patient has plethora and ruddiness of his face. Heelstick hematocrit was found to be 71%. Venous sampling of hematocrit was found to be 68%.
  • Introduction
    • Venoushematocrit exceeding normal values for gestational and postnatal age
      • typically hematocrit > 65%
    • Most babies are asymptomatic
    • Distinct from adult polycythemia vera, a myeloproliferative neoplastic disorder
  • EPIDEMIOLOGY
    • Risk factors
      • delayed clamping of umbilical cord (most common)
        • ↑ transfer of placental blood to infant
      • twin-to-twin transfusion
      • placental insufficiency
      • maternal diabetes
      • maternal hypoxemia
      • macrosomia
      • maternal hypertension
    • Hyperviscosity thought to play a role in symptoms, if any
  • Presentation
    • Majority are asymptomatic
    • Symptoms/physical exam
      • GI upset
        • poor feeding
        • vomiting
      • hypoglycemia
      • cyanosis/apnea
      • plethora
      • respiratory distress
      • lethargy
  • studies
    • Venous hematocrit > 65%
    • Glucose for hypoglycemia
    • Bilirubin level
      • ↑ RBC can lead to hyperbilirubinemia
    • Observe closely for neurologic and cardiovascularsymptoms
      • may have underlying pathologic causes of polycythemia
  • Differential
    • Other causes of polycythemia vera
      • hypoxemia
  • Treatment
    • Asymptomatic
      • supportive care
      • monitor bilirubin and glucose
    • Symptomatic
      • IV hydration with glucose
        • many have hypoglycemia
      • partial exchange transfusion
        • reduces Hct without hypovolemia
        • may not improve outcomes
        • used only if symptoms worsen
  • Complications
    • Hypoglycemia
  • Prognosis
    • Unclear effect on long-term outcome
Flashcards (0)
Cards
1 of 0
Questions (3)
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(M3.PD.17.21) During the exam of a 2-day-old female neonate you determine that she appears lethargic, cyanotic, and has a coarse tremor of her right arm. The patient's mother explains that she observed what she believed to be seizure-like activity just before you arrived in the room. The mother has a history of type two diabetes mellitus and during childbirth there was a delay in cord clamping. You decide to get electrolytes and a complete blood count to work up this patient. The labs are significant for mild hypoglycemia and a hematocrit of 72%. What is the most effective treatment for this patient's condition?

QID: 102775

Phlebotomy

0%

(0/4)

Partial exchange transfusion with hydration

75%

(3/4)

Fluid resuscitation

25%

(1/4)

Hydroxyurea

0%

(0/4)

Interferon alpha

0%

(0/4)

M 5 D

Select Answer to see Preferred Response

Evidence (2)
EXPERT COMMENTS (0)
Private Note