What is the management for a newborn with burns, hepatomegaly, and bleeding in multiple sites, with normal levels of activated Fresh Frozen Plasma (aFFP), Vitamin K, and factor 12?

Management of Newborn with Burns, Hepatomegaly, and Multi-Site Bleeding

This newborn requires immediate vitamin K administration (1 mg intramuscularly or subcutaneously) followed by fresh frozen plasma (FFP) at 10-20 mL/kg if bleeding persists, as this clinical presentation strongly suggests vitamin K deficiency bleeding (VKDB) with hepatic involvement. 1

Immediate Diagnostic and Therapeutic Approach

First-Line Treatment: Vitamin K Administration

• Administer vitamin K1 1 mg immediately via intramuscular or subcutaneous route - this is both diagnostic and therapeutic for hemorrhagic disease of the newborn 1
• A prompt response (shortening of prothrombin time within 2-4 hours) following vitamin K administration confirms the diagnosis of VKDB 2, 1
• Failure to respond within 2-4 hours indicates another coagulation disorder or underlying pathology requiring further investigation 1, 3

Second-Line Treatment: Fresh Frozen Plasma

• If bleeding is excessive or life-threatening, administer FFP at 10-20 mL/kg immediately while awaiting vitamin K response 4, 5, 6
• FFP provides immediate replacement of vitamin K-dependent coagulation factors (II, VII, IX, X) that are critically depleted 6, 1
• FFP does not correct the underlying vitamin K deficiency and must be given concurrently with vitamin K1 1

Critical Laboratory Monitoring

• Obtain baseline PT/aPTT, fibrinogen, platelet count, and liver function tests before treatment if time permits 4
• PIVKA-II (undercarboxylated vitamin K-dependent proteins) is the most specific biomarker for vitamin K deficiency when locally available 2
• Recheck PT/aPTT at 2-4 hours post-vitamin K administration to confirm response 1, 3

Understanding the Clinical Context

Why This Presentation Suggests VKDB

• Hepatomegaly with multi-site bleeding in a term newborn strongly suggests cholestatic liver disease causing secondary vitamin K deficiency 1, 3
• The combination of burns (suggesting possible sepsis or systemic illness) with hepatomegaly creates risk factors for impaired vitamin K absorption and synthesis 1, 7
• Normal factor XII argues against most inherited coagulation disorders, making acquired vitamin K deficiency more likely 8

Hepatobiliary Disease as a Risk Factor

• Cholestasis, biliary obstruction, and hepatic dysfunction are major causes of secondary VKDB 1, 9
• These conditions impair both vitamin K absorption (fat-soluble vitamin) and synthesis of coagulation factors 1, 7
• Parenteral vitamin K prophylaxis is more effective than oral in patients with hepatobiliary disease 3, 9

Additional Supportive Measures

Addressing Coagulopathy Beyond Vitamin K

• If fibrinogen is <1.0 g/L, administer cryoprecipitate at 5-10 mL/kg or fibrinogen concentrate 4, 5
• Target platelet count should be maintained at ≥75 × 10⁹/L in the setting of active bleeding 4
• Higher doses of vitamin K (up to 5-10 mg) may be necessary if maternal medications interfered with vitamin K metabolism 1, 7

Managing the Burns Component

• Burns increase metabolic demands and may contribute to consumptive coagulopathy 4
• Active warming is essential as hypothermia worsens coagulopathy 4
• Maintain adequate perfusion to prevent the "lethal triad" of hypothermia, acidosis, and coagulopathy 6

Critical Pitfalls to Avoid

• Do not delay vitamin K administration while waiting for laboratory confirmation - empiric treatment is appropriate given the clinical presentation 1, 3
• Do not use oral vitamin K in this sick infant with hepatomegaly - the oral route is inappropriate for infants with cholestasis or impaired intestinal absorption 9
• Do not assume normal factor XII rules out all coagulation disorders - vitamin K deficiency specifically affects factors II, VII, IX, and X while sparing other factors 1, 8
• Do not give FFP alone without vitamin K - this treats the symptom but not the underlying deficiency 1

Ongoing Management After Stabilization

Repeated Dosing Requirements

• Infants at high risk (hepatic disease, prematurity, systemic illness) require repeated vitamin K doses 7, 9
• The rate and route of subsequent doses should be determined by clinical response and coagulation parameters 1, 7
• Monitor for recurrent bleeding as single-dose prophylaxis may be insufficient in hepatobiliary disease 3, 9

Investigation of Underlying Hepatomegaly

• Once bleeding is controlled, investigate the cause of hepatomegaly (biliary atresia, metabolic disease, infection) 1
• Infants with underlying cholestatic conditions require ongoing vitamin K supplementation beyond the neonatal period 1, 9