04th May
2018

Bilirubin encephalopathy

Dr. Gaurav Jawa, Consultant Neonatologist- Apollo Cradle Royale

This case describes a baby who was brought to Apollo Cradle Royale in the middle of the night with yellow discoloration of body till soles and an outside Serum Bilirubin report of 38 mg/dl. Baby had decreased feeding and vomiting after feed. Baby was showing signs of stage 1 Bilirubin encephalopathy, suggestive of bilirubin crossing over into the brain.

Baby was born to G2 mother at 34 week gestation by normal vaginal delivery at a nursing home in Faridabad after an uneventful antenatal period .Baby had cried immediately after birth and was discharged after 1 day. Mother was O negative and baby was B positive.

On examination baby had a weak cry and poor reflexes with yellow discoloration till soles, but other systemic examination was normal. Baby was admitted in NICU and IV fluid and Double surface phototherapy was started after sending relevant investigations. In view of very high Serum Bilirubin (38 mg/dl) and clinical evaluation, decision was taken to arrange for double volume exchange transfusion and a blood sample from the infant and mother both was sent to the blood bank Double Volume exchange Transfusion was done under aseptic precaution through umbilical vein and umbilical artery catheterization. Antibiotic cover was given and later stopped once the blood tests showed no signs of infection. A repeat serum bilirubin post exchange after 6 hrs came down to 22.2 mg/dl, which came down further to 6.9 after 24 hrs. when baby was taken off from phototherapy. Feeds were started through orogastric tube initially I/V/O persistent vomiting which was gradually increased to full paladai and breast feed which the baby accepted well. Baby was discharged in a stable condition.

Blood bank detailed report suggested an incompatibility of Anti D as well as Anti E antigen against red blood cells, which could explain aggravated jaundice levels. On follow up examination now till 6 months age, baby was normal with no signs of neurological impairment.

Introduction
Hemolytic disease of the fetus and newborn (HDFN) affects an estimated 3 in 100,000 to 80 in 100,000 patients annually with less than 10% requiring intrauterine transfusion.

Common causes of hemolytic disease of the newborn

  • Rh system antibodies( Anti D , Anti E)
  • ABO system antibodies
  • Kell system antibodies

HDN due to Rh isoimmunization, or blood group incompatibility, occurs when fetal red blood cells (RBCs), which possess an antigen that the mother lacks, cross the placenta into the maternal circulation, where they stimulate antibody production. The antibodies return to the fetal circulation and result in RBC destruction. Prolonged hemolysis leads to severe anemia, which stimulates fetal erythropoiesis in the liver, spleen, bone marrow, and extra medullary sites, such as the skin and placenta. Destruction of RBCs releases heme that is converted to unconjugated bilirubin.

HDN due to other antibodies
Clinically significant allo-antibodies other than anti-D such as anti-E, anti-K, and anti-c occur in 1:300 pregnancies and risk of hemolytic disease of the fetus and newborn (HDFN) caused by these antibodies is 1:500. HDFN caused by anti-E may be moderate or severe in its presentation and brings to attention the necessity of introducing antibody screening for pregnant women as part of the antenatal care to look for significant alloantibodies other than anti-D.

Clinical presentation of HDN varies from mild jaundice and anemia to hydrops fetalis (with ascites, pleural and pericardial effusions). Risks during labor and delivery include asphyxia and splenic rupture.

Postnatal problems include:

  • Asphyxia
  • Pulmonary hypertension
  • Pallor (due to anemia) Edema (hydrops, due to low serum albumin)
  • Respiratory distress
  • Coagulopathies (platelets & clotting factors)
  • Jaundice and Kernicterus (from hyperbilirubinemia)

Hemolytic disease of newborn if not treated timely can also lead to severe jaundice causing bilirubin encephalopathy

Bilirubin encephalopathy (BE), is a neurological condition that occurs when an infant has severe jaundice. In early stage it may cause symptoms like severe jaundice, poor sucking or feeding, lethargy (extreme sleepiness), and a lack of startle reflexes but later on it causes hyperextended or arched back, shrill crying, seizures, muscle rigidity or arched back, coma.

Prognosis
Overall survival is 85-90% but reduced for hydropic fetuses by 15%. Most survivors of alloimmunized gestation are intact neurologically.

Bilirubin encephalopathy (BE), is a neurological condition that occurs when an infant has severe jaundice.

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