Increased neonatal serum bilirubin levels are sufficient enough to result in jaundice. This can be physiological or pathological.
Is the yellow colouration of the skin and sclera caused by deposits of bilirubin The yellow colour becomes visible on newborn skin at >5mg/dl Jaundice is the most common condition that requires medical attention in newborns Definition
BILIRUBIN METABOLISM Bilirubin is made from breakdown of RBCs. Myoglobin,and other heame containing substances in the body.The heame is metabolised to bilirubin:- HEAME → BILIVERDIN → BILIRUBIN ↑ ↑ heame biliverdin oxygenase. Reductase.
METABOLISM contd Uncojugated energy depen conjugat Bilirubin → liver metab →bilirubin transferase enz In serum, unconjugated bilirubin is bound to albumin.↓serum albumin leads to ↑free bilirubin.This is fat soluble and crosses the blood / brain barrier to cause kernicterus if the levels are high.
METABOLISM contd SOME FACTORS DISPLACE BILIRUBIN FROM THE ALBUMIN BINDING SITES. Acidosis, drugs esp. sulphonamides
METABOLISM contd Conjugated water excreted via Bilirubin → soluble → urine/stool Conjugation is an energy dependant activity that can be disrupted by various factors to be mentioned later.
Term – 60% of term neonates Preterm – 80% 0f preterm neonates Incidence
Unconjugated bilirubin ( indirect) Binds to albumin Fat soluble Can cross blood brain barrier Toxic in high level to brain Conjugated bilirubin ( direct) Conjugated with glucoronic acid Water soluble Excreted in urine and stool Non toxic Types of bilirubin
Increased bilirubin load due to a high haemoglobin concentration. The normal newborn infant Hemolysis Cephalohematoma or bruising, polycythemia Mechanisms of Neonatal Jaundice
PHYSIOLOGIC JAUNDICE DEFINITION;- that jaundice that occurs in a full term infant around day 3-4 of life and no pathological cause can be found.
Appears after 24 hours Total bilirubin rises by less than 5mg/dl per day Maximum intensity by 4 th – 5 th day in term and 7 th day in preterm Serum level less than 15mg/dl Clinically not detectable after 14 days Physiological Jaundice
JAUNDICE OF PREMATURITY Jaundice occurring in a preterm infant. Usually caused by;- ↑RBC mass. immature liver metab prone to hypo- glycemia, anoxia, acidosis. p/s premature infants can also have pathological jaundice described ahead
PATHOLOGICAL JAUNDICE DUE TO:- (a) overproduction of bilirubin Such as increased RBC destruction. (b) reduced metab/excretion. Such as enzymatic deficiencies
Appears within 24 hours of age Increase of bilirubin >5mg/dl/day Serum bilirubin >15mg/dl Jaundice persisting after 14 days Stool clay/ white coloured and urine staining yellow Direct bilirubin >2mg/dl Pathological Jaundice
In first 24 hours Hemolytic disorders(G6PD-Spherocytosis) TORCH (congenital infection) 2 nd day – 3 rd week Physiological (disappears after the 1 st week) Breast milk Sepsis polycythemia Causes of Neonatal Jaundice
Cephalohaematoma Crigler – Najjar syndrome Haemolytic disorders Appearance or persistence after 3 rd week Breast milk Hypothyrodism Pyloric stenosis cholestasis
Increased bilirubin load Defective conjugation Increased entero -hepatic function Incidence Term in 60% Preterm in 80% Why Physiological Jaundice develops
BREASTMILK JAUNDICE Jaundice associated with breastfeeding. For some mothers ,their breastmilk contains factors that interfere with bilirubin metabolism.( glucuronidase,NEFFA (NON-ESTERIFFIED FREE FATTY ACIDS ) ) Exclusively breastfed infants have a higher peak of bilirubin that results in jaundice. May be due to dehydration resulting from initial low milk output.=breastfeeding jaundice. RX stoppage of breastfeeding for 24hrs resolves the jaundice!
Common in term infants Caused by prolonged increased enterohepatic circulation of bilirubin Bilirubin peaks at 10 – 15 days of age Unconjugated bilirubin at 10 – 30 mg/dl If nursing is interrupted for 24 hours, the bilirubin level falls Breast milk Jaundice
CLINICAL FEATURES HISTORY----any history of previously affected sibling. --mothers blood group if known. --suggestive pre-disposing factors to sepsis.
Clinical features Jaundice on the skin, sclera of eyes, under the tongue , and palms and soles of feet. Generally jaundice occurring on 1 st or 2 nd day of life is pathological. Jaundice on the 3 rd or 4 th day may be physiological and if physiological it should last less than 2 weeks. Jaundice appearing after day 4 is also most likely pathological.
CLINICAL FEATURES EXAMINATION…jaundice,pallor,hepatomegally,vomiting,lethargy,poor feeding. With onset of kernicterus,there is high pitched cry,hypertonicity,jitteriness convulsions, bulging fontanelles, ophisthotonus posturing.
DIAGNOSTIC EVALUATION HISTORY and CLINICAL FINDINGS LABORATORY WORKUP (a)Total bili and differential..serially to monitor effectiveness of treatment. (b) Heamogram…HB and reticulocyte count. (c) Blood group….mother and baby
LABORATORY INV (d) Coombs test to identify case of HDN that may not involve ABO or RH factors. (e) Peripheral blood film….may show features of infection or hemolysis. (f) Total protein and albumin to determine albumin binding capacity.
No effective treatment exist Phototherapy Exchange transfusion Treatment of the cause Treatment
MANAGEMENT SUPPORTIVE:- (a) maintain hydration (b) ensure nutrition or adequete caloric intake to avoid hypo glycemia. (c) correct acidosis and hypoxia.
MANAGEMENT SPECIFIC:-(1) Phototherapy Works by a process of photo-isomerisation so that isomers are produced which are water soluble and do not require liver metab for excretion.
PHOTOTHERAPY Light of 420-470 nm wavelength is most effective. Blue light is most appropriate. Distance from light source to the baby should be not more than 15-20 cm Turn baby frequently or use photo-optic blanket. May cause loose stools,skin rashes,dehydration,retinal damage,overheating.
When there is increased levels of unconjugated bilirubin Prophylaxis for extreme low birth weight Side effects of phototherapy Dehydration – results from diarrhoea and incensible water loss Retinal damage Indications of phototherapy
Shield eyes with eye patches Keep the baby naked Place the baby close to the light source but watch that the baby is not overheating Do not place anything on the phototherapy devices Promote frequent breastfeeding Periodically change position – prone/supine Precautions for phototherapy
Monitor temperature every 4 hours Monitor weight every 24 hours Perform periodic( 12 – 24 hours)plasma/serum bilirubin test Make sure each light source is working
EXCHANGE TRANSFUSION This is a medical procedure in which a baby’s blood which has high bilirubin content is replaced with fresh blood.
It is used after failed phototherapy to correct anaemia and correct heart failure in hydrops fetalis Severe septicaemia (ill child—sepsis/meningitis) Reduced ongoing haemolysis - Rapid fall in HB Very rapid rise of bilirubin 8-17 umol /dl/hr. Prematurity Previously severe dx in sibs Reticulocyte count > 15 % Indications of exchange transfusion
Hypoglycaemia Hypocalcaemia hypomagnessia Cardiac overload Bleeding Infections – HIV and Hepatitis B Hypothermia incompartibility Side effects of exchange transfusion
EXCHANGE TRANSFUSION PROCEDURE: -- use fresh blood preferably heparinised. -- blood group O-ve most suitable -- can use babies ABO type but RH-ve cross matched against mothers serum. -- warm blood to 35-37 °c -- continuously mix the blood to avoid sedimentation.
PROCEDURE Empty infants stomach with NGT and leave it in situ. Aseptically cannulate infants umbilical vein (up to 7cm for full term baby) Take pre exchange sample for HB and Bilirubin baseline. Exchange over 45-60 minutes using 5-20ml aliquotes depending on baby size and condition.
PROCEDURE Monitor HR, RR by continuous monitor if possible otherwise use observation and stethoscope strapped on infants praecordium. Take post exchange sample for HB and Bilirubin at end of procedure. Continue phototherapy and serial bilirubin monitoring at end of procedure.
EXCHANGE TRANSFUSION DOUBLE VOLUME TRANSFUSION Assumes baby blood vol. of 85 mls /kg body weight. (2 x 85) wt in kg= vol of blood required.
( 1) hypothermia if the blood is not warmed to body temperature, (2) hypocalcemia from the anticoagulant in the donor blood, (3) metabolic acidosis if unbuffered anticoagulant is used in the donor blood, (4) thrombocytopenia if platelet-poor donor blood is used, (5) hyperglycemia from the donor blood followed by reactive hypoglycemia, and (6) graft-versus-host disease if the exchange transfusion is done with non irradiated donor blood. Complications of exchange transfusion
HYPERBILIRUBINEMIA COMPLICATIONS. KERNICTERUS Unconjugated bilirubin crosses the blood/brain barrier and if very high can lead to kernicterus.
KERNICTERUS High unconjugated bilirubin crosses the blood brain barrier and interferes with oxygen uptake of the brain cells. It is deposited in the basal ganglia giving them a yellow coloration. Some pre-disposing factors increase the chance of kernicterus occurring.
KERNICTERUS Lethargy, poor suck, loss of moros response, reduced tendon reflexes. Respiratory distress, bulging fontannelles, shrill high pitched cry, convulsions and spasms eventfully develops ophisthotonic posturing. 75% mortality while survivors have various degrees of brain injury including athetotic cerebral palsy.