Nenatal Jaundice Guideline Guideline Number: 663 Supersedes: Classificatin Clinical Versin N: Date f EqIA: Apprved by: Date Apprved: Date made active: Review Date: 1 Obstetric Written Dcumentatin Review Grup 14/09/2017 21/09/2017 14/09/2018 Brief Summary f Dcument: Scpe T be read in cnjunctin with: Owning grup Obstetric Written Dcumentatin Review Grup 1 f 8 1
Versin n: Summary f Amendments: Reviews and updates Date Apprved: 1 New Guideline 14.9.2017 Glssary f terms Term Definitin Keywrds Nenatal jaundice Dcument ref: 663 Page 2 f 8 Versin 1 Nenatal Jaundice Guideline
CONTENTS 1. INTRODUCTION... 4 2. BILIRUBIN... 4 3. PHYSIOLOGICAL JAUNDICE... 4 4. PROLONGED JAUNDICE... 4 5. CAUSES OF PATHOLOGICAL JAUNDICE... 5 6. BILIRUBIN, ENCEPHALOPATHY AND KERNICTERUS... 5 7. INVESTIGATIONS... 5 8. MANAGEMENT OF JAUNDICE... 5 9. PROLONGED JAUNDICE... 6 10. INVESTIGATIONS OF PROLONGED JAUNDICE... 7 11. BREAST MILK JAUNDICE... 7 12. REFERENCES... 8 13. DEFINITIONS OF PATHOLOGICAL JAUNDICE... 8 Dcument ref: 663 Page 3 f 8 Versin 1 Nenatal Jaundice Guideline
1. INTRODUCTION Apprximately 60% f term and 80% f preterm babies develp jaundice in the first week f life, and abut 10% f breastfed babies are still jaundiced at 1 mnth f age. In mst babies with jaundice there is n underlying disease, and this early jaundice (termed physilgical jaundice ) is generally harmless. Hwever, there are pathlgical causes f jaundice in the newbrn, which, althugh rare, need t be detected. Such pathlgical jaundice may c-exist with physilgical jaundice. Nenatal jaundice refers t yellw cluratin f the skin and the sclera (whites f the eyes) f newbrn babies that results frm accumulatin f bilirubin in the skin and mucus membranes. This is assciated with a raised level f bilirubin in the circulatin, a cnditin knwn as hyperbilirubinaemia. 2. BILIRUBIN Bilirubin is a breakdwn prduct f the red cells in the bld. Red cell breakdwn prduces uncnjugated (r indirect ) bilirubin, which is mstly bund t albumin. Uncnjugated bilirubin is metablised in the liver t prduce cnjugated (r direct ) bilirubin, which then passes thrugh the gut and is excreted in the stl. Bilirubin can be reabsrbed again frm stls remaining in the gut. Newbrn babies red bld cells have a shrter lifespan than thse f adults. The cncentratin f red bld cells in the circulatin is als higher in newbrns than it is in adults, s bilirubin levels are higher than they are later in life. The metablism, circulatin and excretin f bilirubin is als slwer than in adults. Thus a degree f hyperbilirubinaemia ccurring as a result f this nrmal physilgical mechanism is cmmn in newbrn babies and usually harmless. It is difficult t tell which babies are at risk f develping high levels f bilirubin that culd becme dangerus, r wh have a serius prblem as the explanatin fr their jaundice, which is why this guideline has been develped. 3. PHYSIOLOGICAL JAUNDICE Breastfed babies are mre likely than bttle-fed babies t develp physilgical jaundice within the first week f life but the appearance f jaundice is nt a reasn t stp breastfeeding. Physilgical jaundice refers t the cmmn, generally harmless, jaundice seen in many newbrn babies in the first weeks f life and fr which there is n underlying cause. Thereasns fr the assciatin between breastfeeding and nenatal jaundice have nt yet been fully explained but may include inadequate breastfeeding supprt leading t a reduced intake, sluggish gut actin leading t an increase in the enter-hepatic circulatin f bilirubin, r unidentified factrs in breast milk. Finally, it may be that there is a relative reductin f bilirubin levels in frmula-fed babies due t increased clearance f bilirubin frm the gut. Current practice f early pstnatal discharge, ften within 24 hurs, reduces the pprtunity t assess whether successful lactatin has been established and t prvide adequate breastfeeding supprt and advice. 4. PROLONGED JAUNDICE Prlnged jaundice, that is jaundice persisting beynd the first 14 days, is als seen mre cmmnly in term breastfed babies. The mechanism fr this breast milk jaundice is still nt cmpletely understd and the cnditin appears t be generally harmless. Hwever, prlnged jaundice can be a clue t serius underlying liver disease and shuld be assessed carefully. Dcument ref: 663 Page 4 f 8 Versin 1 Nenatal Jaundice Guideline
5. CAUSES OF PATHOLOGICAL JAUNDICE Jaundice may als have ther, nn-physilgical, causes, including bld grup incmpatibility (mst cmmnly Rhesus r ABO incmpatibility), ther causes f haemlysis, sepsis, bruising and metablic disrders. Gilbert syndrme and Crigler Najjar syndrme are rare causes f nenatal jaundice and are caused by liver enzyme prblems. Deficiency f a particular enzyme, glucse-6- phsphate dehydrgenase (G6PD), can cause severe nenatal jaundice. G6PD deficiency is mre cmmn in certain ethnic grups and is familial. Cngenital bstructin and malfrmatins f the biliary system, such as biliary atresia, cause an bstructive jaundice with cnjugated hyperbilirubinaemia. This cnditin needs specialist investigatin and early surgical treatment, preferably befre 8 weeks f life. 6. BILIRUBIN, ENCEPHALOPATHY AND KERNICTERUS High levels f uncnjugated bilirubin (lipid sluble, and easily crsses bld brain barrier) can cause in the shrt-term, temperature instability, fits, apnea, cllapse and death. In the lng-term, high frequency deafness, chre-athetid cerebral palsy and severe learning prblems may ccur. This cnditin is Kernicterus; the name given t the yellw staining f the basal ganglia fund at autpsy in bilirubin txicity. Hpefully it shuld never be seen! Interventin aims t prevent these prblems. The level f uncnjugated bilirubin which can be cnsidered safe depends n gestatinal age and als the general cnditin f the infant. Acidsis and hypxia displace bund bilirubin frm albumin, allwing mre bilirubin t crss the bldbrain- barrier, thus lwering the threshld fr treatment. In the well term infant, prblems are rarely seen belw abut 360 micrmls/l. In the pre-term infant the bld brain barrier is less develped and bilirubin can be pathlgical at lwer levels. 7. INVESTIGATIONS Baseline fr all jaundice Histry & full examinatin, e.g. the spleen is ften palpable in haemlysis r sepsis FBC and film and reticulcyte cunt Grup and Cmbs (interpreted in relatin t mum s bld grup) Ttal bilirubin; split direct & indirect bilirubin requested when baby > 6 days ld Additinal investigatins as suggested by histry Bld cultures and septic screen Urine culture G6PDH screen Mre investigatins are dictated by suspected cause. 8. MANAGEMENT OF JAUNDICE Phttherapy is the main treatment fr uncnjugated hyperbilirubinaemia. Indicatin fr phttherapy: In the absence f firm evidence, empirical guidelines have been set. The frmula: Dcument ref: 663 Page 5 f 8 Versin 1 Nenatal Jaundice Guideline
Gestatin (weeks) x 10 gives the threshld fr exchange transfusin; subtract 100 frm this fr the phttherapy threshld. Hwever, this frmula shuld prbably nly be relied upn fr infants f >3 days ld. Use NICE guideline charts whenever pssible. NOTE: In cases f Haemlytic Anaemia use a chart that is fr babies f 1 week lwer gestatin. Phttherapy needs t be started immediately and exchange level is dependent n the rate f rise rather than abslute level as per guideline fr Management f jaundice in first 24 hurs see belw If the infant is sick (HIE, hypxia, acidsis, hypercapnia, hypglycaemia, infectin) lwer threshlds fr treatment are necessary therefre use a chart f 1 week lwer gestatin e.g. fr a baby 0f 30 weeks gestatin use a chart fr 29 week GA. Managing a baby requiring phttherapy The baby shuld have as much skin expsed as pssible, s lay the baby n an pen nappy with n hat (unless ventilated). The baby will prbably need an incubatr r if using bili-blanket verhead heater t keep warm. The temperature shuld be measured 4-hurly. Explain fully t the mther befre starting. The mther shuld be allwed t handle the baby fr feeding, changing and cuddling the baby ut f the phttherapy with eyes uncvered, as lng as there is n immediate risk f having t d an exchange transfusin. Hwever, baby shuld nt be ut f the phttherapy fr prlnged perids. Phttherapy itself des nt increase fluid requirements; hwever dehydrated babies ften have mre severe jaundice and an assessment f hydratin shuld be made. If the SBR is particularly high, then electrlytes shuld be checked and if dehydratin is suspected, NG feeds r IVI cnsidered. SBR levels can generally be checked nce per day, but if the level is clse t exchange, then repeat them mre frequently. Stpping Phttherapy: This can be dne when the SBR drps 10% belw the treatment threshld. Recheck SBR 24 hurs after stpping phttherapy, except in sick r premature babies, when a level shuld be repeated earlier 12 hurs after stpping phttherapy. 9. PROLONGED JAUNDICE Defined as jaundice persisting beynd 14 days in term and 21 days in preterm babies Causes f prlnged jaundice: Persistence f uncnjugated jaundice frm early nenatal perid: Haemlytic jaundice (f any aetilgy) Infectin, including UTI Rare causes f uncnjugated jaundice: Inbrn errrs f metablism (Galactsaemia, tyrsinaemia, lipid-strage disrders, and thers) Hypthyridism Dcument ref: 663 Page 6 f 8 Versin 1 Nenatal Jaundice Guideline
Drugs Crigler-Najjar Gilbert s Intestinal bstructin Cystic fibrsis Cnjugated jaundice Breast milk jaundice HYWEL DDA UNIVERSITY HEALTH BOARD 10. INVESTIGATIONS OF PROLONGED JAUNDICE Histry & examinatin t elicit cause, remember simple things as clur f stl. Base line investigatins: Split SBR (ttal and direct SBR) - the direct SBR is the cnjugated fractin - a value f >10% f the ttal r f >30 mml/l is suggestive f an bstructive r hepatic cause fr the jaundice. FBC. Grup and Cmb s. Bld film. LFT & cagulatin screen TFTs Urine culture Further septic screen, if unwell Urine fr reducing substances Prlnged Cnjugated jaundice: This is usually picked up because f prlnged jaundice with a high direct fractin. There may be a histry f pale stls and dark urine, which shuld ring alarm bells fr biliary atresia and ther causes f bstructive jaundice. D base line blds/urine and discuss with the cnsultant. Further investigatins in case f cnjugated jaundice: Hepatitis A, B and C Gal-1-put α1 antitrypsin phentype. (NB, since α1 antitrypsin is an acute phase reactant, it may be raised in any intercurrent illness and may be nrmal even if there is a deficiency. Hence the need fr the typing.) TORCH screen Serum amin acids and urine rganic acids, and urine rganic acids X-ray spine (hemivertebra in Alagille syndrme) Liver USS (t exclude chledchal cyst, and lk fr signs f biliary atresia) HIDA scan (radi-istpe scan f the liver t detect biliary atresia) Eye examinatin (psterir embrytxin) 11. BREAST MILK JAUNDICE This is a diagnsis f exclusin. Excessive investigatins may cause anxiety t parents, and lead mther t stp breast feeding. Jaundice is nt a reasn t stp breastfeeding but fluids may need t be increased. If the baby is well and is nrmal n examinatin, and has nrmally pigmented stls, check the baseline investigatins (see abve). If these are nrmal, n further investigatins are needed unless the clinical picture changes r the jaundice clinically becmes darker, in which case, a further split SBR shuld be checked. If the jaundice des nt reslve in a week, then the split SBR shuld again be repeated, but if it remains uncnjugated and the baby is well, n further tests are needed Dcument ref: 663 Page 7 f 8 Versin 1 Nenatal Jaundice Guideline
12. REFERENCES Nenatal Jaundice NICE Clinical Guideline May 2010 13. DEFINITIONS OF PATHOLOGICAL JAUNDICE Visible in first 24 hurs (suggests haemlysis and needs urgent investigatin and treatment) Baby is unwell Ttal Bilirubin >300 mml/l (term infants) Cnjugated jaundice Prlnged jaundice see belw Jaundice which recurs having cleared If any f these criteria are met, the cause(s) fr the jaundice shuld be sught Dcument ref: 663 Page 8 f 8 Versin 1 Nenatal Jaundice Guideline