A cause of preventable permanent brain damage. Mrs B.A had normal delivery of a full term baby weighing 3.5kilogram on 16th of August 2015 at a Mission Home. She was discharged after 24hours.

The mother noticed yellowness of baby’s eyes two days after delivery. She took the baby to the mission home a day after. She was advised to give ampiclox drop and to take him to hospital if yellowness does not reduce. 

The parents did not present the baby to hospital until the naming ceremony day (8th day of delivery) when they noticed that he was having some abnormal limb movements, yellowness of body and refusal of feeding.

The serum bilirubin done was 40mg/dl. The diagnosis made was kernicterus as a complication of severe neonatal jaundice. He died 30minutes after arrival in the hospital. 

This could have been prevented if the couple had not placed priority on naming ceremony and also if the baby was taken to the right place at the right time. Going to mission home and prescription of ampiclox drop were not right. 

Neonatal jaundice, is a yellowing of the skin and other tissues of a newborn infant. A bilirubin level of more than 85 μmol/l (5 mg/dL) leads to a jaundiced appearance in neonates whereas in adults a level of 34 μmol/l (2 mg/dL) is needed for this to occur. In newborns, jaundice is detected by blanching the skin with pressure applied by a finger so that it reveals underlying skin and subcutaneous tissue. 

Jaundiced newborns have yellow discoloration of the white part of the eye, and yellowing of the face, extending down onto the chest. Neonatal jaundice can make the newborn sleepy and interfere with feeding. Extreme jaundice can cause permanent brain damage from kernicterus. 

In neonates, the yellow discoloration of the skin is first noted in the face and as the bilirubin level rises proceeds caudal to the trunk and then to the extremities. This condition is common in newborns affecting over half (50–60%) of all babies in the first week of life. Infants whose palms and soles are yellow, have serum bilirubin level over 255 μmol/l (15 mg/dL) (more serious level). 

 

Causes 

In neonates, jaundice tends to develop because of two factors - the breakdown of fetal hemoglobin as it is replaced with adult hemoglobin and the relatively immature metabolic pathways of the liver, which are unable to conjugate and so excrete bilirubin as quickly as an adult. This causes an accumulation of bilirubin in the blood (hyperbilirubinemia), leading to the symptoms of jaundice. If the neonatal jaundice does not clear up with simple phototherapy, other causes such as biliary atresia, Progressive familial intrahepatic cholestasis, and other pediatric liver diseases should be considered.

Severe neonatal jaundice may indicate the presence of other conditions contributing to the elevated bilirubin levels, of which there are a large variety of possibilities. These should be detected or excluded as part of the differential diagnosis to prevent the development of complications.

They can be grouped into the following categories: 

A Unconjugated 1 Hemolytic Intrinsic causes of hemolysis; Membrane condition like Spherocytosis and Hereditary elliptocytosis, Enzyme conditions like Glucose-6-phosphate dehydrogenase deficiency (also called G6PD deficiency), Pyruvate kinase deficiency Globin synthesis defect like sickle cell disease Extrinsic causes of hemolysis; Systemic conditions e.g. Sepsis, Hemolytic disease of the newborn (ABO), Rh disease, Other blood type mismatches causing hemolytic disease of the newborn.

 

2. Non-hemolytic causes Breast milk jaundice, Cephalohematoma, Polycythemia, Urinary tract infection, Sepsis, High GI obstruction (Pyloric stenosis, Bowel obstruction) B Conjugated (Direct Liver causes e.g. 

Infections like Sepsis, Hepatitis A, Hepatitis B, TORCH infections C Non-organic causes Breastfeeding failure jaundice intake,[4] "Breastfeeding failure jaundice" or "lack of breastfeeding jaundice," is caused by insufficient breast milk resulting in inadequate quantities of bowel movements to remove bilirubin from the body. This leads to increased enterohepatic circulation, resulting in increased reabsorption of bilirubin from the intestines. Usually occurring in the first week of life, most cases can be ameliorated by frequent breastfeeding sessions of sufficient duration to stimulate adequate milk production. Breast milk jaundice Whereas breast feeding jaundice is a mechanical problem, breast milk jaundice is a biochemical occurrence and the higher bilirubin possibly acts as an antioxidant. 

 

Breast milk jaundice: This occurs later in the newborn period, with the bilirubin level usually peaking in the sixth to 14th days of life. This late onset jaundice may develop in up to one third of healthy breastfed infants.

 

Physiological jaundice: Most infants develop visible jaundice due to elevation of unconjugated bilirubin concentration during their first week. This common condition is called physiological jaundice. This pattern of hyperbilirubinemia has been classified into two functionally distinct periods. Phase one Term infants - jaundice lasts for about 10 days with a rapid rise of serum bilirubin up to 204 μmol/l (12 mg/dL). 

 

Preterm infants - jaundice lasts for about two weeks, with a rapid rise of serum bilirubin up to 255 μmol/l (15 mg/dL). Phase two - bilirubin levels decline to about 34 μmol/l (2 mg/dL) for two weeks, eventually mimicking adult values. Preterm infants - phase two can last more than one month. Exclusively breastfed infants - phase two can last more than one month. 

 

Diagnosis Clinical Assessment: This method is less accurate and more subjective in estimating jaundice. Any of the following features characterizes pathological jaundice: Clinical jaundice appearing in the first 24 hours or greater than 14 days of life. Increases in the level of total bilirubin by more than 8.5 μmol/l (0.5 mg/dL) per hour or (85 μmol/l) 5 mg/dL per 24 hours. Total bilirubin more than 331.5 μmol/l (19.5 mg/dL) (hyperbilirubinemia). Direct bilirubin more than 34 μmol/l (2.0 mg/dL). 

 

Treatment 

The bilirubin levels for initiative of phototherapy varies depends on the age and health status of the newborn. In the 1950s, phototherapy was discovered, and became the standard treatment. 

 

Phototherapy: The use of phototherapy was first discovered, accidentally, at Rochford Hospital in Essex, England. Investigation led to the determination that blue light, wavelength of 420-480 nm (peak 458 nm), caused transformation of the trans bilirubin to cis bilirubin, a soluble product that does not contribute to kernicterus Infants with neonatal jaundice are treated with colored light called phototherapy. Exposing infants to high levels of colored light changes trans-bilirubin to the more water-soluble cis-form which is excreted in the bile. 

In phototherapy, blue light is typically used because it is more effective at breaking down bilirubin. Too, Increased feedings help move bilirubin through the neonate’s metabolic system. The light can be applied with overhead lamps, which means that the baby's eyes need to be covered. 

Exchange transfusions Much like with phototherapy, the level at which exchange transfusion should occur depends on the health status and age of the newborn. Complications Prolonged hyperbilirubinemia (severe jaundice) can result in chronic bilirubin encephalopathy (kernicterus). Quick and accurate treatment of neonatal jaundice helps to reduce the risk of neonates developing kernicterus. 

Infants with kernicterus may have a fever or seizures High pitched crying is an effect of kernicterus Exchange transfusions performed to lower high bilirubin levels are an aggressive treatment.