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HO SIEW CHOK HUI
DefinitionYellow discolouration of the
skin ,sclerae or mucous membrane due to elevation of serum bilirubin
It is usually become mores apparent at a total serum bilirubin level of 5 mg/dl
Jaundice is observed during the 1st wk
in approximately 60% of term infant
and 80% of preterm infant.
Hyperbilirubinemia can be toxic, with
high levels resulting in an
encephalopathy known as kerni-cterus
Increased production in neonate due to larger red cell volume, which produces bilirubin as cells are broken down and shorter RBC life span, so broken down faster.
Heme is catabolized within the reticuloendothelial system by heme oxygenase to form biliverdin.
Biliverdin is metabolized to bilirubin in the presence of biliverdin reductase
Ligandins responsible for transport from plasma membrane to endoplasmic reticulum.
Bilirubin conjugated in presence of UDPGT (uridine diphosphate glucuronyl transferase) to mono and diglucoronides, which are then excreted into bile canaliculi.
Benign Physiologic Breast Milk Breastfeeding
Pathologic Many causes
Icterus neonatorum (physiology jaundice)The most common form of indirect jaundice
in infants under 14 days of age between 24-72 hours of age, peaks by 4-5 days in term and 7th day in
preterm neonates and disappears by 10-14days of life.
It is predominantly unconjugated and levels usually do not exceed 15 mg/dl.
caused by increased bilirubin production with transient limited conjugation abilities
Increased rbc’s
Shortened rbc lifespan
Immature hepatic uptake & conjugation
Increased enterohepaticCirculation
Breast Milk jaundice develops after the first 4-7 days of life
Increased enterohepatic circulation of bilirubin due to (1) increased content of beta glucuronidase activity in breast
A diagnosis of breast milk jaundice should be considered if the serum bilirubin is predominantly unconjugated, other causes of prolonged jaundice have been excluded and the infant is in good health, vigorous and feeding well and gaining weight adequately
Breast feeding jaundice Occures in the first week of life and is
caused by insufficient production or intake of breast milk.
Jaundice in breast-fed babies usually appearsbetween 24-72 hours of age, peaks by 5-15 days of life and disappears by the third weekof life.
Pathologic jaundice Appearance of jaundice within 24 hours, increase in serum bilirubin beyond 5
mg/dl/day, peak levels above the expected normal
range, presence of clinical jaundice beyond 2
weeks and conjugated bilirubin (dark urine staining the clothes)
Features Jaundice in 1st 24 hrs Rapidly rising TSB (> 5
mg/dL per day) TSB > 17 mg/dL
Categories Increased bilirubin load Decreased conjugation Impaired bilirubin
excretion
Hemolytic Disease Features: elevated reticulocytes,
decreased Hgb Coomb’s + Rh incompatibility, ABO
incompatibility, minor antigens Coomb’s - G6PD, spherocytosis,
pyrovate kinase deficiency
Non-hemolytic Disease normal reticulocytes Extravascular sources – I.e.
cephalohematoma Polycythemia Exaggerated enterohepatic circulation –
I.e. CF, GI obstruction
Elevated unconjugated bilirubinGenetic Disorders
Crigler-Najjar▪ 2 types▪ Severe hyperbilirubinemia
Gilbert Syndrome▪ Mild hyperbilirubinemia
Hypothyroidism
Crigler-Najjar syndrome is elicited by a lack or deficiency of the enzyme uridine diphosphate glycosyltransferase (UGT).
Type 1 Crigler-Najjar syndrome is associated with an almost complete absence of the enzyme, which results in very high levels of unconjugated hyperbilirubinemia (up to 50 mg/dL) at birth.
Lower levels of serum bilirubin (up to 20 mg/dL) and markedly depressed activity of hepatic UGT are characteristic of type 2 Crigler-Najjar syndrome
Elevated unconjugated and conjugated bilirubin (> 2 mg/dL or > 20% of TSB)
Biliary Obstruction Structural defects – I.e. biliary atresia Genetic defects – Rotor’s & Dubin-Johnson
syndromes Infection – sepsis, TORCH Metabolic Disorders – I.e. alpha1 antitrypsin
deficiency Chromosomal Abnormalities – Turner’s
syndrome Drugs – I.e. ASA, sulfa, erythromycin
Prolonged jaundiceCauses1)Unconjugated HyperbilirubinemiaHypothyroidismHaemolysis (G6PD/ cogenital
spherocytosis)GalactosemiaGilbert syndromeSepsis / UTIBreast milk Jaundice
Conjugated hyperbilirubinemiaBiliary tree abnormalitiesBiliary atresiaCholedochal cystPaucity of bile ducts (Alagille syndrome )Idiopathic neonatal hepatits syndromeTORCHESMetabolic disease (Citrin
deficiency,galactosemia , PFIC , alpha1 antitrypsin deficiency )
TPNSepsis / UTI
ASPHYXIA TRAUMA Metabolic disorder (acidosis ,
hypoglycemia) Sepsis Prematurity Intestinal obstruction Enzyme deficiency : G6PD deficiency Hypothyroidism
Close surveillance of these patient will ensure of early detection of severe jaundice and effective treatment
Jaundice below umbilicus, corresponding to serum bilirubin of 12-15 mg/dl (200-250 mmol/L).
Jaundice up to level of the sole of the feet - likely to need exchange transfusion.
Jaundice within 24 hours of life. Rapid rise of serum bilirubin of more than 8.5
mmol/L/hour (>0.5 mg/dl/hour). Prolonged jaundice of more than 14 days - other
causes/conditions need to be excluded e.g. neonatal hepatitis, biliary atresia.
Family history of significant haemolytic disease or kernicterus
Clinical symptoms/signs suggestive of other diseases e.g. sepsis.
Prenatal/Perinatal : Pregnancy complications Gestation age Maternal blood type/Rh Infections Drug use Delivery method/Delivery intervention Birth weight Previous miscarriages Previous infants with NNJ, kernicterus,
neonatal death, G6PD deficiency
HPI Onset of jaundice Rate of progress of jaundice Appropriate weight gain Trauma Evidence of bleeding /bruising Presence of symptoms such as
apnoea, difficulty in feeding, feed intolerance
and temperature instability.
Diet history :Breast and /or formula fed , length of
time on each breast. Latch strength
Family history :Bleeding disorder, perinatal deaths ,
endocrinopathies
General condition–whether ill-looking or lethargic, presence of hypotonia, or
abdominal distension.Level of jaundicePallor? Cephalohaematoma/subaponeurotic
haematoma,
Look for signs of sepsisPresence of lethargy, poor feeding, failure to thrive, hepato-splenomegaly, temperature
instability or apnea may be a marker of an underlying serious disease.
Look for signs of intrauterine infection –rashes ,Hepatosplenomegaly , SGA
Look for any signs of kernicterus
When a neonate is jaundiced, the yellow discoloration of skin first appears on the face
and it progresses to the trunk, to the palm of the hand, and then the sole of the feet-ie cephalo-caudal progression.
The intensity of yellow discoloration correspondingly increases from the face caudally as the severity of jaundice increases.
Jaundice can be detected by blanching the skin of the neonate with digital pressure exposing the colour of the underlying skin.
The severity of neonatal jaundice is assessed clinically using Kramer’s chart based on dermal zones of neonatal jaundice, where the levels of serum bilirubin correlate with the area of skin that is jaundiced, as indicated in the table
This is applicable to full term infants with jaundice not due to all hemolytic conditions
included Rh incompatibility.
Zone 1 2 3 4 5
SB(umol/L)
100 150 200 250 >250
Kramer drew attention to the observation that jaundice starts on the head, and extends towards the feet as the level rises. This is useful in deciding whether or not a baby needs to have the SB measured. Kramer divided the infant into 5 zones, the SBR range associated with progression to the zones is as follows:
Table 1: correlation between levels of serum bilirubin with the area of skin that is Jaundiced
Area of body Range of indirect bilirubin mg/dl(mmol/L)
Head & neck 4-8 (68-135) Upper trunk 5-12 (85-204) Lower trunk and thigh 8-16 (136-272) Arms & lower legs 11-18 (187-306) Palms and soles >18 (>306)
(NB: It may be difficult to assess jaundice in dark skinned infants).
Principles :Do TSB stat. Estimates of TSB based solely on clinical examination are not reliable
Admit & investigate :Babies who are UNWELL regardless of the TSB levelBabies with jaundice onset< 24 hrs of lifeBabies requiring phototherapy
Total serum bilirubin - sufficient in most cases Unconjugated & conjugated fractions in
specific conditions eg prolonged NNJ Infant’s blood group, maternal blood group Direct Coomb’s test G6PD status (if not already known) Full blood count Reticulocyte count Peripheral blood film (if hereditary
spherocytosis suspected) Blood culture, Urine microscopy and culture
(if infection is suspected)
Copyright ©2004 American Academy of Pediatrics
Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316
Nomogram for designation of risk in 2840 well newborns at 36 or more weeks' gestational age with birth weight of 2000 g or more or 35 or more weeks' gestational age and birth weight of 2500 g or more based on the hour-specific serum bilirubin
values
Copyright ©2004 American Academy of Pediatrics
Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316
Algorithm for the management of jaundice in the newborn nursery
Copyright ©2004 American Academy of Pediatrics
Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316
Guidelines for phototherapy in hospitalized infants of 35 or more weeks' gestation
Advise for physiological jaundice: The parents should be explained about
the benign nature of jaundice. Encourage more frequent
breastfeeding to stimulate the gut and hence increase excretion of bilirubin via the stool
DO NOT encourage or allow supplementary fluids like glucose or water as it will prolong the jaundice.
Pathologic jaundice baby’s blood group, Rh typing and DCT
(if mother is Rh negative or O group);FBC
packed cell volume (PCV); peripheral blood smear (PBS) for hemolysis and red blood cell morphology; reticulocyte count ; RP ; LFT
Do TSB 6 hrly Phototherapy / ET
If a child has near exchange transfusion or higher TSB level but no signs of kernicterus and is well :
Prepare for exchange transfusion Double phototherapy Put up an IV line and maintainence
fluids Continue breastfeeding Rpt SB at 4 hrs If SB cont’ to rise , proceed with ET
Prolonged jaundice beyond 2 weeks: The common causes include breast milk jaundice,
extravasated blood (cephalhematoma), hemolytic disease, G-6PD deficiency and hypothyroidism.
One should rule out cholestasis by noting the urine color and checking the level of direct bilirubin.
Investigations to rule out cholestasis (stool color, urine color, direct and indirect bilirubin levels)
Investigations to rule out ongoing hemolysis, G-6PD screen
Investigations to rule out hypothyroidism Investigations to rule out urinary tract infection.
InvestigationFBC , PBFRP , LFTHep B, Hep C screenTORCHES Urine Feme and C+SUrine reducing sugar , Ferric chlorideG6PD status , TFTMother & baby blood groupCoombs test
If unconjugated hyperbilirubinemiaAdmit if patient is unwellInvestigationExclude UTI and hypothyrodismBreast milk jaundice is diagnosis of
exclusionChild must be well, ,gaining weight
appropriately ,breastfeed well and stool is yellow
Management is continue breast feeding
If conjugated hyperbilirubinaemiaAdmit and observe colour of stool for 3
consecutive daysStart phenobarbitone on admission at
5mg/kg OD for 5 days to prime the liver for HIDA scan if eventually required
Further IX : LFT , Hep B &C status, TORCHES ,VDRL test , alfa-1 antitrypsin and IEM screen
Consider Alagille syndrome
Alagille syndrome is an autosomal dominant disorder with variable expression.
Associated abnormalities include those of the liver, heart, eye, skeleton, and kidneys and characteristic facial features.
Mild-to-moderate mental retardation also may be present.
Typical facial features of Alagille syndrome. Note broad forehead, deep-set eyes and pointed chin
If the stool is pale over 3 consecutive days, suspect biliary atresia and inform Paediatric surgeon and plan for
1)US of liver2)HIDA scan3)Liver biopsy4)Operative cholangiogram
US of hepatobiliary system- Done after4 hrs of fasting- Dilated intrahepatic bile ducts and
absent gall bladder – raises the suspicious of extrahepatic biliary atresia
HIDA scan after 5 days of phenobarbitone
-Low uptake with normal excretion : neonatal hepatitis syndrome- Normal uptake with absent excretion
: Extrahepatic biliary atresia
Liver biopsy- Ensure PT and APTT normal- If not give Vit K 1 mg IV- Platlet count > 40,000
Biliary atresia -Kasai procedure -must be performed within 2 months of life
Neonatal Hepatitis Syndrome- Followup with LFT fortnightly- Watch out for liver failure and
bleeding tendency (Vit K deficiency)- Repeat Hep B and C screening at 6
weeks- Most infants with neonatal hepatitis
made a complete recovery
To prevent potentially dangerous bilirubin levels
and to decrease the need for exchange transfusion, since phototherapy changes bilirubin into more soluble forms to be excreted in the bile or urine (lumirubin )
The minimum irradiance is 6-12 μW/cm2/nm the distance of the light source from the
baby, the optimum distance being 35 - 50 cm
Expose infant appropriately Baby must be completely naked for full
exposure Eye must be covered Beware of hypo/hyperthermia Ensure adequate hydration Turn off photolights during feeding /blood
taking
COMPLICATIONS Tanning and bronze baby Diarrhea Skin rash or burns Dehydration Haemolysis Hyperthermia/ hypothermia
Purpose1)To lower the SB level and reduce
risk of kernicterus2)To remove the infant’s sensitised
RBC and the circulating ab and reduce the degree of red cell destruction
3)To control the blood volume and relieve potential heart failure
Blood vol to exchange 160ml/kg body weight 1 ml of 4.2 % NaHCo3 given for every 100mls
of blood exchanged 1 ml of 10% calcium gluconate given for
every 160mls of blood exchanged Rate of exchange 3min /cycle and total
exchange should be about 90 min Exchange start with removal of blood If child anaemic (hb< 15) ,give an extra
aliquot vol of blood at the end , leaving +ve balance
Always discard the serum & last portion of blood in the tube to avoid electrolyte imbalance
Feed after3 hrs If initial SB is 25mg % , do not remove the
UVC as ET may need to be repeated
IxPre exchange (1st vol of blood removal ) Blood C+S TSB VDRL , HIV ,Hep B and Hep C screening DCT , HbPost exchange (last vol of blood removed ) TSB FBC blood sugar BUSE, calcium
4- 6 hour post exchange : TSB
Complication
Catheter relatedInfection, Haemorrhage, NEC , Air embolism ,Portal and splenic vein thrombosis
Haemodynamic problemsOverload cardiac failurehypovolemic shockArrhythmia
Electrolyte imbalanceHyperkalemia , hypocalcemiahyper/hypo-glycemiametabolic acidosis / alkalosis
FollowupReview TSB at nearest PKK 2 days
after d/cFollowup for 2 years and d/c if
normalLook for signs of deafness , cerebral
palsy and mental retardationFor hearing and neurodevelopmental
assessment
Management of jaundice in healthy newborns , MOH Clinical practice guidelines
Paediatric Protocol for Hospital in SarawakThird edition
Paediatric Protocols for Malaysian Hospital 2nd edition 2008
American Academy of Pediatrics, Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation