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- 2. OUTLINE Introduction Pathophysiology Classification (Pathological, physiological & prolonged jaundice) Assessment(History and Physical Examination) Investigation Complications Management Prolonged Jaundice
- 3. INTRODUCTION Neonatal Jaundice(NNJ) or neonatal hyperbilirubinaemia is one of the most common medical conditions in newborn babies. All babies have a transient rise in serum bilirubin but only about 75% are visibly jaundiced Jaundice can be detected clinically when the level of bilirubin in the serum rises above 85 μmol/l (5mg/dl) Hyperbilirubinemia is either unconjugated or conjugated
- 4. PATHOPHYSIOLOGY Bilirubin isa yellow-orange compound that is produced by the breakdown of haemoglobin from red blood cells Unconjugated bilirubin: is an end product of heme protein catabolism Conjugated bilirubin: is an end product of unconjugated bilirubin that has gone conjugation in the liver cell by uridine diphosphate (UDP) – glucuronyl transferase
- 6. CAUSES OF NEONATALJAUNDICE AGE OF ONSET <24 HOURS OF LIFE 24 HOURS – 2 WEEKS OF LIFE >2 WEEKS OF LIFE CAUSES Haemolytic disorders: • Rhesus incompatibility • ABO incompatibility • Enzymopathy – G6PD deficieny, pyruvate kinase deficiency • Membranopathy –spherocytosis Congenital infections • Infection - urinary tract infection • Haemolysis - G6PD deficiency • ABO incompatibility • Physiological jaundice • Breastfeeding jaundice • Bruising • Polycythaemia • Crigler-Najjar syndrome Unconjugated: • Breast milk jaundice • Infection-UTI • Hypothyroidism • Haemolytic anaemia • High GI obstruction- pyloric stenosis Conjugated: • Bile duct obstruction • Neonatal hepatitis
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- 8. PATHOLOGICAL JAUNDICE (EARLY ONSETOF JAUNDICE) Serum bilirubin rises > 5mg/dL(85umol/L) in 24 hours Appears within 24 hours of Life Causes: I. Hemolysis – ABO incompatibility, rhesus isoimmunisation, G6PD deficiency II. Sepsis III. Gastrointestinal tract obstruction: increase in entero hepatic circulation. IV. Cephalhaematoma, subaponeuritic haemorrhage V. Infant of diabetic mother VI. Polycythemia (hyperviscosity)
- 9. CAUSES OF PATHOLOGICALJAUNDICE PATHOLOGICAL JAUNDICE HAEMOLYSIS TRAUMA POLYCYTHEMIA INFECTION BREASTFEEDING JAUNDICE BREAST MILK JAUNDICE BILIARY TREE OBSTRUCTION
- 10. PHYSIOLOGICAL JAUNDICE Appears after24 hours Serum bilirubin rises > 5mg/dL(85umol/L) Peaks at day 3-5 of life Resolving after 1-2 weeks (3 weeks if preterm) Is not associated with underlying disease and is usually benign Causes: I. 1. Increased bilirubin production resulting from an increased RBC mass relatively polycythemic II. 2. Shortened RBC lifespan III. 3. Poor bilirubin clearance : hepatic immaturity of ligandin and glucuronosyltransferase
- 11. RISK FACTORS FORSEVERE NEONATAL JAUNDICE prematurity low birth weight jaundice in the first 24 hours of life mother with Blood Group O or Rhesus Negative G6PD deficiency rapid rise of total serum bilirubin sepsis lactation failure in exclusive breastfeeding high predischarge bilirubin level cephalhaematoma or bruises babies of diabetic mothers family history of severe NNJ in siblings
- 12. ASSESSMENT 1. History 2. Physicalexamination
- 13. HISTORY Onset ofjaundice <24 hours 24hr – 2 weeks >2 weeks Symptoms of kernicterus Lethargy Poor feeding Abnormal posture Irritable Inconsolable cry High pitch cry Convulsion Mother’s blood group & Rhesus
- 14. Family history Previous history of other siblings with neonatal jaundice G6PD deficiency Blood disorders Liver diseases History of pregnancy & delivery Antenatal infections Eg: TORCHES, Syphillis, Drug intake/herbal remedies Delayed cord clamping Birth trauma with bruising / fractures HISTORY
- 15. Postnatal history Breastfeeding Use of drug/remedies in lactating mother Greater than average weight loss Symptoms & signs of hypothyroidism Symptoms & signs of metabolic disease Eg: galactosemia Exposure to total parental nutrition HISTORY
- 16. PHYSICAL EXAMINATION Generalcondition, gestation and weight, signs of sepsis, hydration status. Level of jaundice Using Kramers rule To find causes & other associated signs Cephalhematoma Infections of umbilical cord Hepatosplenomegaly Signs of sepsis Signs of intrauterine infection e.g. petechiae, hepatosplenomegaly.
- 17. SIGNS OF KERNICTERU S Decreas e tendon reflex Opisthotonus Bulging anterior fontanel Twitchin gof face and limbs Rigidit y
- 20. TRANSCUTANEOUS BILIRUBINOMETER (TCB) Handheld device that measures the amount of bilirubin in the skin. Used as a screening tool Limitation: 1. If levels exceed 200 μmol/L (12 mg/dL), total serum bilirubin (TSB) should be measured 2. Should not be used in preterm babies 3. Should not be used to monitor bilirubin levels in babies on phototherapy
- 21. COMPLICATIONS Acute Bilirubin encephalopathy ABEresult in changes of mental (behavioral) status and muscle tone during the neonatal period when the baby is having hyperbilirubinemia Signs of acute bilirubin encephalopathy: drowsiness, poor feeding and hypotonia followed by hypertonia affecting particularly extensor muscles - opisthotonus, Retrocollis Risk factors: Preterm infants, small for gestational age, sepsis, acidosis, hypoxic-ischemic encephalopathy, hypoalbuminaemia, jaundice less than 24hours of age
- 22. COMPLICATIONS Kernicterus (Bilirubin Encephalopathy) Neurologic syndrome resulting from deposition of unconjugated bilirubin in basal ganglia and brainstem nuclei May be seen in babies who survive from ABE The earliest clinical manifestations are lethargy, hypotonia, irritability, poor Moro response, poor feeding, a high-pitched cry and emesis Early signs are noted after day 4 of life.
- 23. Signs ofchronic bilirubin encephalopathy (irreversible brain damage to basal ganglia): dystonia, choreo athetoid cerebral palsy, paralysis of upward gaze, sensorineural hearing loss Early signs of kernicterus occasionally may be reversed by immediately instituting an exchange transfusion 10% mortality and 70% morbidity
- 24. BLOOD INVESTIGATION Test Why? Serumbilirubin (SBV/SBC) • Total • Unconjugated • Conjugated 1. Bilirubin level (narrow down dx) 2. Severity 3. Monitoring Further Investigations: G6PD testing G6PD deficiency TSH status Hypothyroidism FBC ± peripheral blood count Reticulocyte count 1. Haemolytic disorders 2. Infections Blood grouping (maternal & baby) Direct Coomb’s test 1. ABO incompatibility 2. Rhesus incompatibility Septic workout (if infection is suspected) • Blood culture & sensitivity • Urine culture & sensitivity, UFEME, Urinalysis Infections/Sepsis
- 25. MANAGEMENT Phototherapy Exchangetherapy Pharmacotherapy
- 26. CPG management ofneontal jaundice 2nd edition Predischarge screening: -clinical risk factor assessment -predischarge bilirubin levels – TCB -G6PD Deficiency should be admitted and monitored for NNJ during first 5 days of life and check TSB if clinical jaundice -Term, BW >2.5kg, may be discharged earlier at day 4 of life if TSB <160umol/L (9mg/dL) and follow up closely -All babies discharged < 48H after birth should be seen by healthcare provider within 24H of discharge -Severe jaundice, early follow up is needed to detect rebound jaundice after discharge
- 27. PHOTOTHERAPY It isthe mainstay of treatment in NNJ No sunlight exposure (risk of dehydration and sunburn) Types of devices : flouroscent tubes, Light Emitting Diode (LED), fibreoptic and halogen bulbs Mechanism of action: bilirubin (water insoluble) is transformed into photoisomers that are water soluble , and are easily excreted in the urine as they do not require hepatic conjugation Commenced when total serum bilirubin reaches the phototherapy threshold
- 32. EFFECTIVE PHOTOTHERAPY Achievedwith optimal irradiance and adequate exposed body surface rather than number of phototherapy units Supine position Blue light range (400-500 nm) Irradiance of minimum of 15uW/cm2/nm for conventional phototherapy Irradiance of minimum of 30uW/cm2/nm for intensive phototharapy Distance of the light source not exceeding 30-50 cm from baby *Visual observation unreliable. Serum bilirubin levels must guide the management
- 33. CARE OF BABIESDURING PHOTOTHERAPY Adequately exposed cover eyes (prevent retinal damage) Babies should be regularly monitored for vital signs including temperature and hydration status Monitor urine output and weight Breastfeeding should be continued. Turn off photolights and remove eyepads during feeding and blood taking
- 34. SIDE EFFECT increasedinsensible water loss, dehydration hyperthermia skin rash Diarrhea hypocalcemia skin bronzing
- 35. TYPES OF PHOTOTHERAPY Conventional 6-12w/m2/nm Lullaby SP20w/m2/nm IP 40w/m2/nm Biliblanket
- 36. • Failure ofphototherapy is defined as an inability of decline in bilirubin of 17-34 umol/L (1-2mg/dL) after 4-6 hours and/or to keep the bilirubin below the exchange transfusion level
- 37. EXCHANGE TRANSFUSION (ET) Indicated for severe hyperbilirubinemia to reduce risk of brain damage associated with kernicterus. Various methods :femoral vein (FV), umbilical vein(UV), umbilical artery/vein (UA/V), peripheral artery (radial artery).
- 38. INDICATIONS DOUBLE VOLUME EXCHANGEPARTIAL EXCHANGE TRANSFUSION 1. To lower serum bilirubin level and reduce risk of brain damage associated with kernicterus 1. To correct polycythemia with hyperviscosity 2. Hyperammonia 2. To correct severe anemia without hypervolemia 3. To remove bacterial toxins in septicemia 4. To correct life threatening electrolyte and fluid disorders in acute renal failure
- 39. • Signed consentfromparents • Ensure resuscitation equipment is ready and available • Stabilise the patient, maintain temperature, pulse, respiration, O2 saturation • Obtain peripheral venous access for IV fluid maintenance • Proper gentle restraint • Omit the last feed before ET (if < 4 hours from last feed, empty gastric contents by NGaspiration) • Types of blood to be used; RH isoimmunisation: ABO compatible, Rh negative blood Other conditions: Cross match with baby and mother’s blood In emergency (unknown blood type): O Rh negative blood • Volume to be exchanged is 2x the infant’s total blood volume (2x80ml/kg)=160ml/kg • Fresh whole blood, less than 5 days old or reconstituted Packed Red blood cells and FFP in a ratio of 3:1 PREPARATIONS
- 41. • Pre-warm bloodto body temperature using water bath • Connect baby to cardiac monitor • Neonatal Exchange Blood Transfusion Sheet Take baseline observation (heart rate, respiration, oxygen saturation) and record down following observations are recorded every 15-30 minutes • Perfom under aseptic technique using a gown, mask and headcap. • Cannulate the umbilical vein not > 5-7cm depth • Aliquot for removal and replacement 5ml/kg per cycle (not more than 5-8% of blood volume): 20ml for term not more than 5ml/kg for ill/preterm • Begin with an initial removal, so that there is always deficit, to avoid cardiac overload PROCEDURE OF ET
- 42. PROCEDURE OF ET •Syringe should be held vertically during infusion ‘in’ to prevent air embolism • Total exchange duration should be 90-120 minutes utilizing 30-35 cycles • Rate of exchange: 3-4 min per cycle (1 min out, 1 min in, 1-2 min pause) • Record the amount of blood given or withdrawn, and medications given • Anemic hb pre-ET < 12: at the end of procedure, give extra aliquot vol of blood 10cc/kg at a rate 5 cc/kg/h
- 43. • Investigations – Pre-ET(the 1st vol. of blood removed) • FBC, blood c+s, HIV ,hep B, hep C,SBV – Post-ET • SBV, FBC, renal profile, capillary blood sugar, blood c+s • Post-ET management –continue Intensive Phototherapy –Monitor vital signs (BP , HR, spo2) hourly x 2, 2 hourly x 2, then 4 hourly ifstable –Monitor dscan hourly x 2 ,then 4 hourly if stable –repeat SBV 2 hours post ET, then 4-6hly –NBM with IVD (can allow feeding if well) -readministered medication after ET -repeat ET may be required in 6H if high rebound serum bilirubin
- 44. COMPLICATION OF ET CATHETERRELATED HEMODINAMIC PROBLEMS ELECTROLYTE/METAB OLIC DISORDERS • Infection • Hemorrhage • Necrotising enterocolitis • Air embolism • Vascular events • Portal, splenic vein thrombosis (late) • Overload cardiac failure • Hypovolemic shock • Arrythmia (catheter tip near sinus node in right atrium) • Hyperkalemia • hypocalcemia • Hypo/hyperglycemia
- 45. • Intravenous Immunoglobulin(IVIG) –high dose IV Immunoglobulin (0.5-1g/kg over 2h) reduces the rate of haemolysis in babies with rhesus haemolytic disease. –give as early as possible in haemolytic disease with +ve Coombs test or when the serum bilirubin is increasing despite intensive phototherapy • Clofibrate • Human albumin • Tin-mesophorphyrin • Phenobarbitone PHARMACOTHERAPY
- 46. FOLLOW UP Acute bilirubinencephalopathy • Long term follow up, to monitor neurodevelopment sequelae TSB > 342umol/L (20mg/Dl)/ done ET • Auditory Brainstem Response (ABR) test (3 month of life) • Referred to audiologist • Neurodevelopmental follow up Prem babies with Jaundice • Follow up for neurodevelopment sequelae (as per prem follow up plan)
- 47.
- 48. PROLONGED JAUNDICE Visiblejaundice that persists beyond : 14 days of life in term infant (≥37 weeks) 21 days in preterm infant (≥35 weeks to < 37 weeks) Causes of prolonged jaundice Conjugated or Unconjugated hyperbilirubinemia conjugated hyperbilirubinemia: direct bilirubin more than 34umol/L(2mg/dL) or >15%of total bilirubin *All babies with conjugated hyperbilirubinaemia must be referred to a paediatrics department urgently to exclude biliary atresia
- 49. Conjugated hyperbilirubinaemia Unconjugated hyperbilirubinaemia Biliary tree abnormalities: Biliaryatresia Choledochal cyst Paucity of bile duct Alagille syndrome Idiopathic neonatal hepatitis syndrome Septicemia UTI Congenital infection (TORCHES) Metabolic disorder Progressive familial intrahepatic cholestasis (PFIC) Alpha-1 antitrypsin deficiency Total parenteral nutrition Septicemia UTI Breast milk jaundice Hypothyroidism Hemolysis: G6PD deficiency Congenital spherocytosis Galactosemia Gilbert syndrome *Early diagnosis and treatment of biliary atresia and hypothyroidism is important for favourable long term outcome of patient
- 50. • Common andremain a diagnosis of exclusion (Infant is well, gaining weight, breast feed well, stool normal yellow with normal physical examination) • Interruption of breastfeeding for 1 to 2 days results in a rapid decline of bilirubinlevels. • Breast milk may contain an inhibitor of bilirubin conjugation • Management : -continue breast feed and review periodically -Increase breastfeeding to 8-12 times/day ,recheck serum bilirubin levels in 12-24 hours. BREAST MILK JAUNDICE
- 51. BREASTFEEDING JAUNDICE • Manifestsin the first 3 days of life ,peaks by 5-15 days of life, dissapears by week 3 of life. • Caused by: -insufficient production or intake of breast milk -Maternal breastfeeding issues (engorgement,cracked nipples, and fatigue) -Neonatal factors (ineffective sucking) • In contrast with breastmilk jaundice, infants generally exhibit mild dehydration and weight loss in the first few days of life • Management: -Increase breastfeeding, make sure proper breastfeeding technique is adheredto.
- 53. • Needs earlydiagnosis •Clinical features ; - persistent or late onset jaundice -conjugated hyperbilirubinaemia -pale stool -firm liver -hepatosplenomegaly • Better prognosis if Kasai procedure (hepatoportoenterostomy) is done within first 2 months of life • Diagnosis -US liver ,raised GGT , palestool BILIARY ATRESIA
- 54. serious, rare,autosomal recessive , permanent deficiency of glucuronyl transferase results in severe unconjugated hyperbilirubinemia Type I : does not respond to phenobarbitol and manifests as persistent indirect hyperbilirubinemia, often leading to kernicterus. Type II : responds to enzyme induction by phenobarbitol , producing an increase in enzyme activity and reduction of bilirubin levels. CRIGLER-NAJJAR SYNDROME
- 55. • mutation ofpromoter region of glucuronyl transferase , resulting in mild indirect hyperbilirubinemia GILBERT DISEASE
- 56. ALAGILLE SYNDROME Considerin infants who have cardiac murmurs or dysmorphism. One of the parents is usually affected (AD inheritance, variable penetrance) Affected infants might not have typical dysmorphic features at birth due to evolving nature of the syndrome. Important screening tests include : Vertebral x ray: To look for butterfly vertebrae Echocardiography: look for branched pulmonary artery stenosis. Slit eye lamp examination: look for posterior embryotoxon May also help to rule out other aetiologies in neonatal hepatitis syndrome, e.g. retinitis in congenital infection, cataract in galactosaemia. Other known abnormalities - ASD, valvular pulmonary stenosis. Gene test: JAG1 gene mutation which can be done at IMR (EDTA container).(Consult Genetist Prior to Testing)
- 57. APPROACH TO PROLONGED JAUNDICE All babies must be screened for prolonged jaundice (clinical jaundice) Clinical assessment : feeding method, weight, stool colour, hepatosplenomegaly Risk stratification: direct and total serum bilirubin Once diagnosed refer to MO on the same day/next working day
- 58. INVESTIGATIONS most important lab investigation: Totalserum bilirubin with direct and indirect Work up for Prolonged Jaundice : - FBP + reticulocyte count - Thyroid Function test, e.g. TSH - Liver function test - Urine FEME & culture and sensitivity * Observe stool colour for 3 days *No Phototherapy needed in Prolonged jaundice
- 59.
- 62. FOLLOW UP FOR PROLONGEDJAUNDICE • Treat any treatable conditions (biliaryatresia, hypothyroidism, UTI, IEM, syphilis) • Follow up 2-8weekly with LFT • Watchout for worsening LFT • No need for regular serum bilirubin monitoring once investigations for prolonged jaundice is done and the serum bilirubin is not increasing in trend.
- 63. • Paediatric protocolsfor Malaysian hospitals 3rd & 4th edition • Nelsonessentials of Pediatrics 7th edition • http://hsibu.moh.gov.my/hsb.bm/wp-content/ uploads/2017/04/Neonatal-Jaundice-Dr-Lee.pdf • www.medscape.com • CPG management of neontal jaundice 2nd edition • https://www.nice.org.uk/guidance/cg98/evidence/full- guideline-245411821 REFERENCES
Editor's Notes
- #51 Associated with unconjugated hyperbilirubinemia without evidence of hemolysis during first to second week of life, persists longer than physiological jaundice (can persist up to 4 months of age) Periodically,during 1 and 2 mo immunisazation
- #59 Phototherapy not needed because of bbb already fully functional by 2 weeks and 3 weeks in preterm babies