Neonatal Jaundice - Introduction and Management

1. NEONATAL JAUNDICE PRESENTED BY: DR. AHMAD ZHAFIR BIN ZULKFLI @ ZULKIFLI DR. NURHAFIZATUL AKMA BINTI HASAN SUPERVISED BY: DR. FAIDZAN HASLENY BINTI HASBOLLAH

2. OUTLINE • INTRODUCTION TO NEONATAL JAUNDICE • RISK FACTORS • METABOLISM OF BILIRUBIN • ASSESSMENT OF NEONATAL JAUNDICE • ETIOLOGY OF NEONATAL JAUNDICE • MANAGEMENT • COMPLICATIONS OF NEONATAL JAUNDICE • REFERENCES • QUIZ

3. INTRODUCTION • Neonatal Jaundice is one of the most common medical conditions in newborn babies. • Jaundice - yellowish of the skin, scleras and mucous membrane due to hyperbilirubinemia and deposition of bile pigments • All babies have a transient rise in serum bilirubin but only about 75% are visibly jaundiced. • Jaundice is clinically detectable when the serum bilirubin levels are >85μmol/L (5 mg/dl).

4. METABOLISM OF BILIRUBIN

6. RISK FACTORS • Prematurity • Low birth weight infants • Mother with Blood Group O or Rhesus Negative • G6PD deficiency • Sepsis • Lactation failure in exclusive breastfeeding • Cephalohaematomaor bruises • Babies of diabetic mothers

7. ASSESSMENT OF JAUNDICE • Gestational age : the incidence of hyperbilirubinemia increases with prematurity • Time of onset : pathological vs physiological , to elicit causes • Antenatal history: Mother’s blood group Infections Diabetes in pregnancy Risks of sepsis • Previous infants with NNJ, neonatal death, G6PD deficiency • Family history • Labour and delivery • Breastfeeding history • Presence of abnormal symptoms such as apnea , difficulty in feeding , feeding intolerance , temperature instability

8. PHYSICAL EXAMINATION • General condition • Severity of jaundice in terms of cephalocaudal progression • Evidence of bleeding • Cephalohaematoma, subaponeurotic hemorrhage • Bruising • Pallor • Evidence of congenital infections • Petechiae • Hepatosplenomegaly • Neurological signs of bilirubin encephalopathy • loss of Moro’s reflex, lethargy, hypotonia, seizure, opisthotonus, high pitch cry

9. INVESTIGATION • Total serum bilirubin • G6PD status • Others as indicated: • Infant’s blood group, maternal blood group, Coombs’ test (indicated in Day 1 jaundice and severe jaundice). • Full blood count + reticulocyte • FBP • Blood culture, UFEME and urine culture (if infection is suspected)

10. (Kramer’s rule)

11. ETIOLOGY

12. ETIOLOGY

13. Breast milk jaundice • Adequate breastfeeding • Increase amount non estrified fatty acids which inhibit bilirubin conjugation due to certain enzymes/genetic problem • Thus increase production of bilirubin • Advise mother to continue breastfeeding Breastfeeding jaundice • Less breastmilk in few days of life • Dehydration esp weight loss > 10% (dehydration worsen the jaundice) • Phototherapy is indicated

15. PROLONGED JAUNDICE • Visible jaundice (or serum bilirubin > 85µmol/L) that persist beyond 14 days of life in a term infant or 21 days in a preterm infant.

17. Unconjugated hyperbilirubinemia: • Important investigation: • TFT • UFEME, Urine C&S • FBC + reticulocyte count • FBP • G6PD screening • Breast milk jaundice is a diagnosis of exclusion.

18. Conjugated hyperbilirubinemia: • Defined as conjugated bilirubin > 25µmol/dL • Investigate for biliary atresia and neonatal hepatitis syndrome • Investigations: • LFT • coagulation profile • lipid profile • Hep B and C virus status • TORCES, VDRL • alpha-1 antitrypsin level • gamma glutamyltranspeptidase (GGT) • US HBS

19. BILIARY ATRESIA • Also known as extrahepatic ductopenia and progressive obliterative cholangiopathy. • Is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. Can be congenital or acquired • Treatment: 1. Kasai procedure (hepatoportoenterostomy) 80% successful within 1st 2 months of life 20%-30% in later surgery 2. Liver transplant

20. MANAGEMENT Criteria for admission/Referral • Onset jaundice within 24 hours of life • Rapidly rising TSB > 6mg/dL/day (103umol/L/day) • Clinical jaundice below umbilicus , corresponding to TSB of 12-15 mg/dL (205-257umol/L) • Clinical jaundice till soles of feet ( urgent referral for possibility of ET) • All G6PD deficiency baby • Clinical symptoms/ signs suggestive of sepsis

21. PHOTOTHERAPY • Mainstay of NNJ treatment • Mechanism: converts unconjugated bilirubin by photodegradation into a harmless water-soluble pigment increase excretion (isomerization) Type of devices • LED (light emitting diode) • Fibreoptic ( egbiliblanket • Halogen bulbs

23. PHOTOTHERAPY Effective phototherapy consist of • Blue light range (400-500 nm) • Irradiance minimum of 15 uW/cm2/nm for conventional phototherapy • Irradiance minimum of 30 uW/cm2/nm for intensive phototherapy • Distance of light source not exceeding 30-50 cm for baby (from top surface of the infants)

24. PHOTOTHERAPY Care of babies during phototherapy • Babies should be regularly monitored for vital signs including temperature & hydration status • Baby should be adequately exposed • Baby eyes should be covered to prevent retinal damage • Breastfeeding should be continued

25. PHOTOTHERAPY • Failure of phototherapy • has been defined as inability to observe a decline a bilirubin 1-2mg/dl (17-34Umol/L) after 4-6 hours & or to keep the bilirubin below the exchange transfusion level • Complications of phototherapy • Phototherapy rash • Lethargy • Diarrhea • Hyperthermia • Retinal damage • Dehydration

26. INTENSIVE PHOTOTHERAPY

29. EXCHANGE TRANSFUSION Indications • To lower serum bilirubin level • Hyperammonimia • To remove bacterial toxins in septicaemia • To correct life-threatening electrolyte and fluid disorders in acute renal failure • To correct polycythaemia with hyperviscosity • To correct severe anaemia without hypovolaemia Method of performing ET • Femoral vein • Umbilical vein • Umbilical artery / Vein • Peripheral artery (radial artery)/peripheral vein

30. Mechanism • Lower the serum bilirubin level (by about 45%) • Remove the sensitized infant’s RBC and also antibodies to reduce degree of hemolysis (will remove estimated 85% of RBCs

31. Video

32. Complications/ risk of ET

37. Immunoglobulin • High dose IVIG 0.5-1gm/kg over 2 hours reduce the need for exchange transfusion in Rh & ABO hemolytic disease • Give as early as possible in hemolytic disease with positive coombs test or when serum bilirubin increasing despite of intensive phototherapy • Dose can be repeated in 12 hours if necessary . If ET is already indicated , IVIG should be given after ET

38. Do not forget to arrange for hearing and neurodevelopmental assessment in babies with severe NNJ!

39. BILIRUBIN ENCEPHALOPATHY • A neurologic syndrome resulting from deposition of unconjugated bilirubin in the basal ganglia& brain stem nuclei. • Clinical features : • Appeared 2-5 days after birth (term) , & 7th day in preterm ** can occur at any time in neonatal period

40. BILIRUBIN ENCEPHALOPATHY • Early signs • Lethargy • Poor feeding • Loss of Moro’s reflex • Ill & prostrated • Decrease tendon reflexes • Opisthotonus • Bulging anterior fontanelles • Twitching of face or limbs • High pitched cry • Convulsion with stiffly extending their arms inward rotation with fists clenched **** rigidity was late sign

41. Complication : Neurological sequeale/ death

42. Source: Nature Scientific Reports 2018 https://www.nature.com/articles/s41598-018-24811-3

43. REFERENCES • CPG Management of Neonatal Jaundice (2ndedition) – Ministry of Healthy, Malaysia, 2015 • Paediatric Protocols for Malaysian Hospitals 3rd edition – Ministry of Healthy, Malaysia • Nelson’s Essentials of Pediatrics, 7th edition, 2015 • Adaptive response of neonatal sepsis-derived Group B Streptococcus to bilirubin, Richard Hansen et. al. (2018) 8:6470 | DOI:10.1038/s41598-018-24811-3 https://www.nature.com/articles/s41598-018-24811-3 (retrieved on June 8th, 2018)