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Why TEST a baby's jaundice? It mattered when no one told us it did

No one is born with KI. This preventable malady can occur when jaundice (hyperbilirubinemia) is mismanaged in the first days or weeks of life. Athetoid cerebral palsy, auditory neuropathy, cortical visual impairment, dental enamel hypoplasia, and gaze abnormalities are the common neurological sequelae. In 2001, the National Quality Forum issued a “Never Event” ranking kernicterus among 27 medical errors that should never happen. The word kernicterus comes from the root “kern,” meaning core and “icterus,” meaning yellow. When bilirubin reaches a toxic level it crosses the blood-brain barrier in an infant and literally stains a certain part of the brain (the globus pallidus in the basal ganglia)…

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 Kernicterus in the 21st century: frequently asked questions

Acute kernicterus remains a clinical emergency and its delayed management represents an easily preventable neonatal brain injury. Yet, practitioners encounter recurrent questions regarding the risk and timing of bilirubin-related neurotoxicity. These include the following: does bilirubin damage the brain of healthy infants? Is there a re-emergence of kernicterus in the United States? Was kernicterus previously prevented in the United States? What was the public health impact of 1994 American Academy of Pediatrics Guidelines?

Articles of Interest

Baby Has Jaundice: Is That a Problem?

Jaundice can signal risk of a rare but irreversible neurological disorder called kernicterus. Once fairly common, kernicterus virtually disappeared in the U.S. by the 1970s, only to stage a comeback over the past decade. One specialist estimates there are 75 to 100 cases a year in the U.S. Some say the academy of pediatrics fed the comeback with a 1994 recommendation, later modified, that doctors needn't be so vigilant about managing jaundice.

Bilirubin Induced Encephalopathy

Hyperbilirubinemia is one of the most common neonatal disorders. Delayed diagnosis and treatment of the pathologic and progressive indirect hyperbilirubinemia lead to neurological deficits, defined as bilirubin induced encephalopathy (BIE). The incidence of this disorder in underdeveloped countries is much more than developed areas. All neonates with the risk factors for increased the blood level of indirect bilirubin are at risk for BIE, especially preterm neonates which are prone to low bilirubin kernicterus .

Clinicopathological Spectrum of Bilirubin Encephalopathy/Kernicterus

Bilirubin encephalopathy/kernicterus is relatively rare, but continues to occur despite universal newborn screening. What is more interesting is the spectrum of clinical and even neuropathological findings that have been reported in the literature to be associated with bilirubin encephalopathy and kernicterus... Bilirubin-induced neurological complications continue to occur in industrialized countries, but a disproportionately increased burden is reported in low- and middle-income countries, primarily because of delays in delivering treatments that are more readily available in high-income countries.

Kernicterus

The risk of developing kernicterus increases considerably in infants with bilirubin levels >25 mg/dL while levels >30 mg/dL are associated with extremely high risk and irreversible damage.

Kernicterus on the Spectrum

Kernicterus is the potential toxic sequela of extreme neonatal hyperbilirubinemia resulting from the passage of excess free, unconjugated bilirubin across the blood−brain barrier, irreversibly and selectively damaging vulnerable target brain cells including the basal ganglia, the cerebellum, and the auditory system. Kernicterus continues to plague the modern world. Not only does it continue to be uncontrolled in developing countries with underdeveloped medical systems, and health organizations rendered ineffective by the ravages of war, but it also remains prevalent in industrialized countries.

Kernicterus: Past, Present, and Future

The concept of a relationship between severe neonatal hyperbilirubinemia and kernicterus evolved slowly. Jaundice was recognized in ancient as well as biblical times as a manifestation of disease. However, according to Louis K. Diamond, who reviewed the history of neonatal jaundice, the first record concerning jaundice of the newborn appears to be by Barthomomaeus Metlinger in 1473 in his book Ein Regiment der Jungen Kinder.

My Child Has Kernicterus: Why it Could Have Been Caused by Medical Malpractice

According to the Center for Disease Control, about 60% of all babies are born with jaundice. If treated appropriately with phototherapy, jaundice usually resolves unless there is a more pathologic cause which at that point will be investigated and treated depending on the cause. If a provider fails to identify and treat jaundice in a timely manner, the baby could suffer from a life threatening condition called kernicterus, which is the result of untreated jaundice. The bilirubin level continues to rise without treatment, causing brain damage to the newborn. Elevated bilirubin is referred to as hyperbilirubinemia.

Risk of Brain Disorder Rare in Healthy Newborns with Jaundice

Researchers at UCSF Benioff Children’s Hospital San Francisco and the Kaiser Permanente Northern California Division of Research sought to examine the correlation between elevated levels of the liver-produced pigment bilirubin, which causes the yellowing of the skin and eyes associated with jaundice, and cerebral palsy — a range of disorders that impairs control of movement. The team was especially interested in kernicterus, a rare and life-threatening type of cerebral palsy triggered by escalating bilirubin that injures the brain.

‘He could have died’: family calls for better jaundice testing of black and Asian babies

Oddie says that jaundice is much more difficult to detect, especially in south Asian babies, but points out that if parents or clinicians suspect jaundice, National Institute for Health and Care Excellence guidance stipulates that a test should be given.

Resources

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K is for Kernicterus and it’s something that doesn’t have to happen…

Radiopaedia

Kernicterus, also known as chronic bilirubin encephalopathy, describes the chronic, toxic, permanent sequelae of high levels of unconjugated bilirubin on the central nervous system of infants. It is part of the spectrum of bilirubin-induced neurologic dysfunction, which also includes acute bilirubin encephalopathy.

StatPearls

Kernicterus, or bilirubin encephalopathy, is bilirubin-induced neurological damage, which is most commonly seen in infants. It occurs when the unconjugated bilirubin (indirect bilirubin) levels cross 25 mg/dL in the blood from any event leading to decreased elimination and increased production of bilirubin. The unconjugated bilirubin can cross the blood-brain barrier as it is lipid-soluble, unlike the water-soluble conjugated bilirubin. It gets deposited in the brain tissue, mainly the basal ganglia.