almost all newborn infants develop some degree of hyperbilirubinemia as a normal transition in physiology .
high levels of unbound unconjugated bilirubin can cross the blood brain barrier and cause neurological symptoms .
the association between hyperbilirubinemia and encephalopathy was first described in 1847 by hervieux ; the term kernicterus was first used by schmorl in 1903 to describe yellow staining of the basal ganglia .
the terms  bilirubin induced neurological dysfunction  ( bind ) and kernicterus are often used interchangeably , although some authors consider bind to refer to the clinical presentation and kernicterus to be an anatomical diagnosis . in this paper , the term kernicterus will be used to describe both the neuropathology of bilirubin induced brain injury and the associated clinical presentation .
kernicterus is a preventable condition ; the unconjugated bilirubin levels can be decreased to safe levels by the use of phototherapy or exchange transfusion .
phototherapy is an effective way of decreasing the bilirubin level in neonates , by converting unconjugated bilirubin into isomers such as lumirubin that are water soluble and can be excreted in the urine .
exchange transfusion ( et ) , however , is considered to be the most effective and quickest method to lower the bilirubin level in infants at high risk of kernicterus .
an et is indicated when hyperbilirubinemia remains at dangerous levels despite intensive phototherapy and is particularly useful when there is excessive haemolysis .
the actual bilirubin level at which to implement phototherapy or et has been a subject of controversy for many years . in south africa
, consensus was reached in 2006 on a nationally accepted nomogram for the management of hyperbilirubinemia .
kernicterus is a preventable condition ; however cases still occur , even in high - income countries like the usa .
reasons include early discharge of neonates without predischarge screening , lack of knowledge regarding neonatal hyperbilirubinemia among both health workers and caregivers , inadequate follow - up , and an increase in breastfeeding rates without adequate support .
kernicterus and severe hyperbilirubinemia are even more prevalent in low- and middle - income countries , due to delays , at all levels of care , in babies with severe hyperbilirubinemia receiving adequate treatment .
although et is effective in preventing kernicterus , the procedure has significant complications , including cardiovascular , biochemical , and haematological abnormalities ; the risk of death may be as high as 5% .
the aim of this study was to review neonates undergoing et at charlotte maxeke johannesburg academic hospital ( cmjah . )
objectives included describing the characteristics of babies undergoing et , comparing readmitted babies with babies who were inpatients after birth , and describing complications and outcome of et .
this was a retrospective review of all newborns undergoing et for severe hyperbilirubinemia at cmjah between 01 june 2006 and 30 december 2011 .
babies who underwent et included those who were discharged at birth and readmitted to the general paediatric wards (  readmitted babies  ) and those who were still inpatients after birth (  in - hospital babies  ) .
subjects were identified from the cmjah neonatal database , admission registers for the paediatric wards , and the south african national blood bank database .
neonates were managed according to the south african consensus guidelines   at the discretion of the attending paediatrician .
phototherapy was commenced in all neonates with severe hyperbilirubinemia ; et was done if the bilirubin remained above the et levels in the consensus nomogram .
ethics approval was obtained from the human research ethics committee of the university of the witwatersrand .
results were described using measures of central tendency , mean and standard deviation for continuous data with a normal distribution or median and range for skewed data .
comparisons between continuous variables were done using paired t - tests for data with a normal distribution and wilcoxon signed rank test was used to compare skewed data .
records could not be traced in 5 babies , so there were 64 babies included in the analysis who underwent 67 et .
the median age of discharge after et was 8 days ( range 3 to 71 ) .
 readmitted  and  in - hospital  babies are compared in table 2 . in comparison to the  in - hospital  babies ,  readmitted  babies
were of significantly greater birth weight and gestational age , had a higher initial total bilirubin , were more likely to be breastfed , and had et at a later stage ( see table 2 ) . in most cases
the cause of neonatal hyperbilirubinemia was undetermined ( 19/26 ( 73% ) of readmitted babies compared to 32/38 ( 84% ) in  in - hospital  babies ( p = 0.17 ) ) .
a total of 26/64 ( 40.6% ) babies were admitted to the general paediatric wards for et .
six babies ( 23.1% ) had neurological signs compatible with kernicterus , including hypertonia , hypotonia , opisthotonus , high pitched cry , and seizures .
all six of these babies with neurological signs had a total bilirubin level above 460  mol / l .
changes in haematology and blood chemistry before and after exchange transfusion are shown in table 3 .
the total bilirubin and platelet counts were significantly decreased after the et , whereas calcium and phosphate levels both significantly increased after et ( see table 3 ) .
most babies who underwent et were in - hospital babies ( 38/64 ; 59.4% ) ; 17/38 ( 44.7% ) were very low birth weight ( vlbw ) infants .
there were 6468 neonatal admissions during the study period ; thus there were 5.8 et per 1000 neonatal admissions .
one in - hospital infant required a second et ; this was a term infant with rh incompatibility .
two of the babies died of presumed sepsis at 4 and 6 days of age , respectively ; both had raised c reactive protein with clinical signs of sepsis but negative cultures .
the third infant was premature and died of escherichia coli sepsis with perforated necrotising enterocolitis , three days after exchange transfusion .
changes in haematology and chemistry for in - hospital babies are shown in table 4 .
the total and direct bilirubin levels as well as the platelet count decreased significantly after et .
severe hyperbilirubinemia remains a problem in neonatal care in lmics . in the present study ,
there were 64 babies who underwent et over a period of 4.5 years corresponding to 0.9 et per 1000 live births .
this is considerably less than that reported in lagos , nigeria , where 1.9% of all neonates had an et .
reasons for the lower rate of et in the present study may reflect the fact that the hospital is in a metropolitan area , where most babies are delivered within a health facility and g6pd deficiency is much less prevalent in south africa than in central and west africa . it is worrying to note that six ( 23% ) of the babies readmitted for et had isoimmune haemolysis , suggesting that this condition was overlooked at birth .
isoimmune haemolysis is a definite risk factor for severe hyperbilirubinemia and these babies should have been closely monitored after birth . in the present study ,
most babies with hyperbilirubinemia were male , breastfed , and delivered vaginally , which is in keeping with other reports .
the present study highlights the fact that there are two populations of newborn babies who undergo et , premature and sick babies who remain in hospital after birth and well term babies who are discharged on the day of birth .
for all babies , it is important to know the mother 's blood group and to screen babies for jaundice in the first 48 hours after birth .
the fact that the majority of babies undergoing et were  in - hospital  patients may reflect suboptimal care ; however the rate of et in hospital was relatively low at 5.8 per 1000 neonatal admissions .
reasons for inadequate monitoring of total serum bilirubin ( tsb ) in hospital were not evaluated in this study but may reflect inadequate staffing with high volumes of patients .
prevention of severe hyperbilirubinemia in premature and sick infants in hospital includes implementing a protocol for monitoring tsb , either using a transcutaneous bilirubin meter ( tcb ) or taking blood to determine tsb . house staff must be adequately trained and supervised to ensure that tsb results are obtained timeously and that babies are properly managed .
blood results may often be overlooked in busy , short staffed neonatal units , particularly after hours .
it is also essential to ensure that phototherapy is effective ; for example , the baby needs to be fully exposed to the light ( not covered in a diaper ) , the luminescence of the lights must be adequate , and the lights must be the correct distance away from the baby .
the avoidable factors associated with severe hyperbilirubinemia were unknown in the majority of readmitted babies in the current study .
it is , however , standard practice in the study hospital for healthy newborn babies and their mothers to be discharged within 6 hours of birth .
prevention of severe hyperbilirubinemia in babies discharged on the day of birth should focus more on factors outside the hospital setting .
prevention of severe hyperbilirubinemia includes educating expectant mothers and their families at antenatal clinic , how to detect jaundice , the correct steps to take if their baby is jaundiced , the dangers of haemolytic agents and certain traditional remedies , and the need for follow - up of babies discharged on the day of birth , especially those with risk factors for hyperbilirubinemia [ 7 , 13 ] .
education of primary health care workers about the dangers , signs , and management of neonatal hyperbilirubinemia is also essential .
establishing breastfeeding with proper lactation support at home after hospital discharge , especially in circumstances where most well mothers and babies are discharged on the day of birth , is also very important .
although tcb is ideal for this purpose , these instruments are expensive and may not be freely available in lmics .
there were few complications of et ; these included a relative decrease in platelet count and increase in serum phosphate and calcium levels .
the avoidable factors resulting in severe hyperbilirubinemia and kernicterus were not evaluated in the current study .
the study was conducted in a metropolitan area and tertiary hospital , so it is not necessarily representative of all babies with hyperbilirubinemia in south africa .
unfortunately there is no long term follow - up data available on the babies in the present study , so the rate of neurodevelopmental problems and hearing loss from severe hyperbilirubinemia is not known .
although severe neonatal hyperbilirubinemia is less common in cmjah than in other african countries , six normal term babies were readmitted with signs of kernicterus .
there is still a need for vigilant screening and correct management of babies with severe hyperbilirubinemia to prevent the occurrence of kernicterus .
 
 background . severe 
 hyperbilirubinaemia requiring exchange transfusion has become less common in recent years ; however , kernicterus still occurs . 
 the aim of this study was to review babies undergoing exchange transfusion for severe hyperbilirubinaemia in a johannesburg hospital . methodology . 
 this was a retrospective review of babies who required exchange transfusion in both the neonatal and the paediatric wards from june 1 , 2006 , to december 31 , 2011 . 
 results . 
 there were 64 patients who underwent 67 exchange transfusions . 
 isoimmune haemolysis ( both rh and abo incompatibility ) was the cause of jaundice in 9/64 ( 14% ) . 
 most babies who underwent exchange transfusion were sick or preterm and were admitted in hospital after birth ( 38/64 ; 59.5% ) ; three of these babies died , but not during the exchange transfusion ( 3/38 ; 7.9% ) ; all three had signs suggestive of neonatal sepsis . the remaining 26 babies ( 40.6% ) were readmitted to the paediatric wards for exchange transfusion . 
 six of these babies ( 6/26 ; 23.0% ) had signs of kernicterus . 
 the most significant complication of exchange transfusion was apnoea requiring mechanical ventilation in three patients ( 3/64 ; 4.6% ) . conclusion . despite a relatively low number of babies undergoing exchange transfusion 
 , kernicterus still occurs and must be prevented . 
 proper protocols for screening and management of severe hyperbilirubinaemia need to be enforced . 
1. Background
2. Subjects and Methods
3. Results
4. Discussion
5. Conclusions