Newborn Jaundice (Neonatal Jaundice)

Jaundice in newborns most commonly occurs because their livers are not mature enough to remove bilirubin from the blood.

Neonatal jaundice is the most common condition requiring medical evaluation in a newborn. Though it is often a normal and transient physiologic consequence of the newborn's immature liver, there are a variety of other medical conditions that can cause severe neonatal jaundice. Jaundice in the newborn must be carefully monitored, as excessively elevated levels of bilirubin that go untreated for too long, can lead to permanent brain damage (a condition termed kernicterus).

Jaundice is the yellowish discoloration of the skin, mucous membranes, and the whites of the eyes (sclerae) caused by elevated levels of bilirubin in the blood (hyperbilirubinemia). The term jaundice is derived from the French word jaune, which means yellow. Jaundice is not technically a disease, but rather it is a visible sign of an underlying condition causing increased levels of bilirubin in the blood.

Jaundice symptoms in newborns include fever, fussy eating, and looking ill.

Treatment for jaundice in newborns includes phototherapy, tanning booths, and other treatments.

Jaundice in newborns most commonly occurs because their livers are not mature enough to remove bilirubin from the blood.

Jaundice may also be caused by several other medical conditions, such as:

• Physiologic jaundice
• Neonatal jaundice
• Hemolysis
• Polycythemia
• Cephalohematoma, a collection of blood under the scalp
• Infant swallowing during birth
• Mothers who have diabetes
• Crigler-Najjar syndrome
• Lucey-Driscoll syndrome
• Maternal-fetal blood group incompatibility (Rh, ABO)
• Breast milk and breastfeeding
• Red blood cell enzyme and membrane defects

Jaundice is caused by the accumulation of bilirubin in the blood, typically from an increased production of bilirubin or a decreased ability to metabolize and excrete it. Bilirubin is formed when red blood cells break down and are normally metabolized in the liver and excreted in urine and feces.

Neonatal jaundice can be caused by several different conditions, and it is very commonly seen in newborns. The most common cause in newborns is physiologic jaundice, which affects most newborns and is usually benign. However, many other causes of neonatal jaundice can be more serious, and require further medical evaluation and intervention.

A newborn that develops jaundice within the first 24 hours of life requires immediate medical attention.

The causes of jaundice in newborns include the following:

• Physiologic jaundice
  • This form of jaundice is usually evident on the second or third day of life. It is the most common cause of newborn jaundice and it is usually a transient and harmless condition.
  • Physiologic jaundice is caused by the inability of the newborn's immature liver to metabolize (conjugate) and thus excrete bilirubin, which accumulates due to the breakdown of red blood cells which have a shorter life-span (70 to 90 days) than adult red blood cells (120 days). This increase in red blood cell breakdown and decreased ability to metabolize bilirubin overwhelms the newborn's ability to properly process and excrete bilirubin.
  • As the newborn's liver matures, however, the jaundice eventually disappears after 1 to 2 weeks.
• Maternal-fetal blood group incompatibility (Rh, ABO)
  • This form of jaundice occurs when there is an incompatibility between the blood types of the mother and the fetus. This leads to increased bilirubin levels from the breakdown of the fetus' red blood cells (hemolysis).
• Breast milk jaundice
  • This form of jaundice occurs in breastfed newborns and often appears at the end of the first week of life. Certain chemicals in breast milk are thought to be responsible. It is usually a harmless condition that resolves spontaneously. Mothers typically do not have to discontinue breastfeeding.
• Breastfeeding jaundice
  • This form of jaundice occurs when the breastfed newborn does NOT receive adequate breast milk intake. This may occur because of delayed or insufficient milk production by the mother or because of poor feeding by the newborn. This inadequate intake results in dehydration and fewer bowel movements for the newborn, which results in decreased bilirubin excretion from the body.
• Cephalohematoma (a collection of blood under the scalp)
  • Sometimes during the birthing process, the newborn may sustain a bruise or injury to the head, resulting in a blood collection/blood clot under the scalp. As this blood is naturally broken down, sudden elevated levels of bilirubin may overwhelm the processing capability of the newborn's immature liver, resulting in jaundice.
• Red blood cell enzyme defects
  • Inherited genetic enzyme disorders, such as glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) or pyruvate kinase deficiency, for example, can lead to increased hemolysis and subsequent elevation in bilirubin levels.
• Red blood cell membrane defects
  • Abnormalities in the membrane and shape of red blood cells (for example, spherocytosis) can lead to increased hemolysis.
• Other causes
  • Other causes of neonatal jaundice include:
    • Prematurity
    • Maternal diabetes
    • Polycythemia
    • Infection/sepsis
    • Hypothyroidism
    • Biliary atresia
    • Cystic fibrosis
    • Crigler-Najjar syndrome
    • Gilbert syndrome
    • Hepatitis
    • Thalassemia
    • Galactosemia

Approximately 60% of all newborns develop jaundice; however, newborns with certain risk factors are at higher risk for developing neonatal jaundice.

Risk factors for neonatal jaundice include the following:

• Having siblings who have had neonatal jaundice
• Having parents of East Asian or Mediterranean descent
• Preterm babies
• Newborns with feeding difficulties/poor feeding
• Mother with diabetes
• Newborns with bruising/cephalohematoma
• Blood type incompatibility
• Congenital infection

Neonatal jaundice may be associated with various signs and symptoms, depending upon the degree of elevation in bilirubin levels as well as the exact cause of jaundice. The yellow discoloration of the skin will begin on the face and forehead and extend toward the feet as the levels of bilirubin increase. This characteristic progression of jaundice in infants can sometimes allow your healthcare professional to estimate the bilirubin level based on the location and extent of jaundice, though this assessment can often be inaccurate.

The signs and symptoms associated with neonatal jaundice may include the following:

• Yellow discoloration of the skin, mucous membranes, and the whites of the eyes
• Light-colored stool
• Poor feeding
• Lethargy/excessive sleepiness
• Changes in muscle tone (either listless or stiff with arching of the back)
• High-pitched crying
• Seizures

Kernicterus, which is caused by prolonged excessively elevated levels of bilirubin affecting the central nervous system, must be recognized and promptly treated as it can lead to permanent brain damage. Kernicterus is an irreversible and chronic condition that can include cerebral palsy, hearing loss, and intellectual impairment. Kernicterus has likely started to develop if the infant begins to exhibit extreme lethargy, changes in muscle tone, and a high-pitched cry.

You should contact your doctor if your baby has the following signs:

• Develops jaundice on the first day of birth
• Develops jaundice on the whites of the eyes, arms, chest, palms, soles, or abdomen one month after birth
• Jaundice worsens or does not go away after 2 weeks
• Is unusually drowsy or difficult to keep awake
• Has rashes along with jaundice
• Is feeding poorly
• Cries inconsolably
• Develops fever
• Arches their neck or back
• Becomes stiff or floppy
• Has pale bowel movements

Neonatal jaundice can be diagnosed by examining the newborn and obtaining a blood test to check the total serum bilirubin level. Your healthcare professional should closely follow your newborn for the development of jaundice, and many hospitals routinely check total bilirubin levels on newborns before hospital discharge. Though the blood test is the most accurate, in certain instances, a transcutaneous bilirubin reading can be obtained by placing a handheld sensor against the newborn's skin. If this reading is high, a blood test may be ordered to confirm the total bilirubin level. Bilirubin levels are usually highest at 3-5 days of life.

Additional blood tests may be ordered for those newborns who require treatment and monitoring, or for those who may have other underlying conditions thought to be causing neonatal jaundice.

The need to treat jaundice in newborns is determined by interpreting the total bilirubin level and comparing it with the newborn's age in hours. If the level is deemed to be too high, or if it is rising too quickly, treatment is necessary. Sometimes, all that is needed for treatment is close monitoring of the bilirubin levels, and no further interventions may be necessary. In certain instances, treatment for neonatal jaundice can occur at home, while in other cases, admission to a hospital is necessary.

Based on the total bilirubin level, the underlying cause leading to jaundice, and the newborn's clinical condition, various treatment modalities are available to treat jaundice in newborns. These include the following:

• Feeding/hydration: By encouraging frequent feeding with breast milk or formula, newborns will produce more stool and urine, and thus eliminate bilirubin more rapidly from the body. Newborns may need to be fed up to 12 times per day, thus ensuring proper hydration and elimination. In certain instances, newborns may require observation in a hospital to receive intravenous fluids and closer monitoring of bilirubin levels.
• Phototherapy: This treatment is the most common for newborns requiring medical treatment for neonatal jaundice. Phototherapy (light treatment) involves placing the newborn under artificial blue lights with the baby undressed (wearing only a diaper) and using a special mask to protect the eyes. This treatment may occur in an open bassinet or warmer, or sometimes with a phototherapy blanket. These special lights work by converting bilirubin to lumirubin through a chemical reaction that occurs when the newborn's skin is exposed to these special lights. This allows the newborn to more easily eliminate bilirubin from the body. Care needs to be taken to avoid excessive heat and dehydration. Phototherapy is used in hospitals, and in certain cases, newborns may be treated at home with phototherapy. Generally speaking, phototherapy is very safe and effective, and this treatment is successful for almost all newborns who require it. Phototherapy treatment is stopped once the newborn's closely monitored bilirubin levels have decreased to appropriate values.
• Exchange transfusion: This urgent treatment is used in newborns with severe jaundice who may have failed phototherapy treatment or in those who are at higher risk of developing or have signs of kernicterus. During an exchange transfusion, the newborn's blood is replaced with donated blood to rapidly decrease bilirubin levels. It is a more specialized procedure that is used on a case-by-case basis.
• Intravenous immunoglobulin: Administering this treatment may be used in select cases of significant neonatal jaundice due to maternal-fetal blood group incompatibility, and it may in some cases reduce the need for an exchange transfusion.

Generally speaking, the prognosis for newborns with jaundice is excellent if they receive the appropriate monitoring and treatment, and the vast majority of infants with neonatal jaundice will improve with no adverse effects. However, healthcare professionals need to remain vigilant and parents need to be informed and educated about the potential dangers of severe hyperbilirubinemia to prevent the devastating consequences of kernicterus.

The complications associated with neonatal jaundice occur when bilirubin levels reach toxic levels, and the bilirubin gets into the central nervous system and damages the brain. The brain toxicity can either be reversible (early acute bilirubin encephalopathy) or the damage may be permanent and irreversible (kernicterus). Permanent damage may lead to cerebral palsy, deafness, and intellectual impairment.

Some degree of jaundice in newborns is normal and not entirely preventable. However, the prevention of significant hyperbilirubinemia and its complications is possible through proper screening (obtaining bilirubin levels), identifying newborns at high risk, close surveillance, and monitoring of those infants with hyperbilirubinemia, parent education, and prompt treatment when deemed medically indicated.