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 discolouration of the body till soles and an outside Serum Bilirubin report of 38 mg/dl. The baby had decreased feeding and vomiting after a feed. The baby was showing signs of stage 1 Bilirubin encephalopathy, suggestive of bilirubin crossing over into the brain.
The baby was born to a G2 mother at 34 weeks gestation by normal vaginal delivery at a nursing home in Faridabad after an uneventful antenatal period. The baby had cried immediately after birth and was discharged after 1 day. Mother was O negative and baby was B positive.
On examination, the baby had a weak cry and poor reflexes with yellow discolouration to the soles, but other systemic examinations were normal. The baby was admitted to NICU and IV fluid and Double surface phototherapy were started after sending relevant investigations. Given the very high Serum Bilirubin (38 mg/dl) and clinical evaluation, the decision was taken to arrange for double volume exchange transfusion and a blood sample from the infant and mother was sent to the blood bank Double Volume exchange Transfusion was done under aseptic precaution through the 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 the baby was taken off from phototherapy. Feeds were started through an orogastric tube initially I/V/O persistent vomiting which was gradually increased to full paladai and breast feed which the baby accepted well. The baby was discharged in a stable condition.
The blood bank's detailed report suggested an incompatibility of Anti D as well as Anti E antigens against red blood cells, which could explain aggravated jaundice levels. On follow-up examination now till 6 months ago, the 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 anaemia, which stimulates fetal erythropoiesis in the liver, spleen, bone marrow, and extramedullary 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 the 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 anaemia to hydrops fetalis (with ascites, pleural and pericardial effusions). Risks during labour and delivery include asphyxia and splenic rupture.
Postnatal problems include:
- Asphyxia
- Pulmonary hypertension
- Pallor (due to anaemia) 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 the 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 all immunized gestation are intact neurologically.
Bilirubin encephalopathy (BE), is a neurological condition that occurs when an infant has severe jaundice.
