what are the signs of kernicterus

Full Answer

How do I know if my baby has kernicterus?

Signs & Symptoms Initial findings associated with kernicterus may vary from case to case, but often include lack of energy (lethargy) or drowsiness, poor feeding habits, fever, a shrill high-pitched cry, and/or absence of certain reflexes (e.g., Moro reflex, etc.).

What are the stages of kernicterus?

In phase 1, the infant demonstrates decreased alertness, hypotonia, and poor feeding. This is followed by a phase of hypertonia of the extensor muscles. Progress to this phase invariably leads to long-term neurologic deficits. Phase 3 usually occurs in infants aged >1 week, and they typically demonstrate hypotonia.

How do you confirm kernicterus?

How is kernicterus diagnosed? Kernicterus is most often diagnosed in babies. One test that may be used to check bilirubin levels is a light meter. A doctor or nurse will check your baby's bilirubin levels by placing the light meter on your baby's head.

At what level does bilirubin cause brain damage?

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.

How common is kernicterus in newborn?

Kernicterus only affects about 1 in 44,000 newborns in developed countries because jaundice is usually treated before it becomes dangerous. Symptoms of kernicterus include excessive sleepiness, high-pitched crying, muscle spasms and arching in the neck and back.

How long does it take jaundice to affect brain?

People should seek immediate medical attention anytime that the symptoms of jaundice or kernicterus occur. It can take less than 24 hours for serious cases of jaundice to progress to kernicterus.

What turns yellow first with jaundice?

The whites of the eyes are often the first tissues that you notice turning yellow when you develop jaundice. If the bilirubin level is only mildly high then this might be the only part of the body where you can detect a yellow colour. With higher levels of bilirubin, the skin also becomes yellow.

Who is at risk for kernicterus?

Infants born to mothers of blood type O negative are at greatest risk, with one series of 249 infants with severe hyperbilirubinemia reporting an odds ratio of 48.6 for infants with Rh incompatibility.

When should I be concerned about my baby's jaundice?

“If the jaundice is noticeable on the first day of your baby's life or it affects the chest or abdomen, it's a sign their level of bilirubin may be higher than normal,” he says. “In this case, your baby should be evaluated by their doctor.”

What color is bilirubin poop?

Stool Colors The brown color is mainly due to bilirubin, which is in the bile your gallbladder secretes. As bilirubin is digested, it becomes brown. There are various shades of brown stool may be, from light yellow-brown to dark brown.

What level of jaundice causes kernicterus?

Kernicterus is likely to occur when serum levels of unconjugated bilirubin are greater than 30 mg/dl and is unlikely to occur when levels are lower than 20 mg/dl.

How long is too long for jaundice?

Jaundice usually clears up within 2 weeks in formula-fed babies. It may last for more than 2 to 3 weeks in breastfed babies. If your baby's jaundice lasts more than 3 weeks, talk to his health care provider.

Is kernicterus progressive?

Kernicterus is a progressive condition that gets worse as the brain damage advances. The specific symptoms of kernicterus differ depending on what the stage of progression of the the condition.

What happens if bilirubin doesn't go down?

Talk to your baby's doctor if the jaundice is prolonged. “Often, elevated bilirubin doesn't lead to any major health problems,” says Dr. Radhakrishnan. “However, very high levels or inadequately treated elevated levels may cause brain damage and the risk is higher for premature babies.

What is increased in kernicterus?

Kernicterus is a type of brain damage that can result from high levels of bilirubin in a baby's blood. It can cause athetoid cerebral palsy and hearing loss. Kernicterus also causes problems with vision and teeth and sometimes can cause intellectual disabilities.

What is the GREY baby syndrome?

Gray baby syndrome is an adverse reaction to chloramphenicol that is characterized by abdominal distention, hemodynamic collapse, and ashen-gray skin discoloration in neonates.

What are the symptoms of kernicterus?

Other symptoms of kernicterus include: high-pitched crying. decreased appetite and less feeding than usual. inconsolable crying. floppy or limp body. missing reflexes. arching of the head and heels back, like a bow. uncontrollable movements.

How do you know if you have kernicterus?

Symptoms of kernicterus. Signs of jaundice can appear within the first few days of a newborn’s life. Jaundice causes the baby’s skin and whites of the eyes to turn a yellowish color. Kernicterus symptoms are more severe. Babies with kernicterus are also lethargic. This means they are unusually sleepy.

Why does my baby have kernicterus?

Kernicterus is caused by severe jaundice that isn’t treated. Jaundice is a common problem in newborns. It happens because a newborn’s liver can’t process bilirubin quickly enough. Bilirubin builds up in the baby’s bloodstream as a result.

What is the drug that kills kernicterus?

Sulfonamides (also called sulfa drugs) are a group of antibiotics that kill bacteria. One common antibiotic combines the sulfonamide sulfamethoxazole with trimethoprim (SMX-TMP) to treat bacterial infections. Studies have raised concerns#N#Trusted Source#N#that sulfonamides might increase the risk for kernicterus.

Is kernicterus rare in adults?

Kernicterus is very rare in adults. The conditions that cause it most often affect infants. It’s possible for adults to develop high bilirubin levels, but almost never kernicterus.

Can a baby with kernicterus be lethargic?

Babies with kernicterus are also lethargic. This means they are unusually sleepy. All babies sleep a lot, but lethargic babies sleep even more than is typical and are very hard to wake up. When they do wake up, they often fall right back to sleep.

Does Gilbert's syndrome cause kernicterus?

This condition does not cause kernicterus. Gilbert’s syndrome: A condition in which the liver can’t properly process bilirubin. Rotor syndrome: An inherited disorder that causes bilirubin levels to build up in the blood. This condition does not cause kernicterus.

What are the symptoms of kernicterus?

Initial findings associated with kernicterus may vary from case to case, but often include lack of energy (lethargy) or drowsiness, poor feeding habits, fever, a shrill high-pitched cry, and/or absence of certain reflexes (e.g., Moro reflex, etc.). Affected infants may eventually experience respiratory distress, mild to severe muscle spasms including those in which the head and heels are bent backward and the body bows forward (opisthotonus), and/or diminished muscle tone (hypotonia).

When is Kernicterus suspected?

In most cases, persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice) is apparent within the first few days of life.

How to treat kernicterus durint?

Treatment for Kernicterus focuses on decreasing the amount of unconjugated bilirubin in the blood. Early treatment is imperative in the attempt to prevent the symptoms and physical findings associated with kernicterus durint the first months of life. Such treatments may include exchange blood transfusions in which small amounts of blood are withdrawn repeatedly and replaced with blood from a donor until most of the blood has been exchanged. In another procedure known as plasmapheresis, unwanted substances (toxins, metabolic substances and plasma parts) are removed from the blood. During this procedure, blood is removed from the affected individual and blood cells are separated from plasma. The plasma is then replaced with other human plasma and the blood is transfused into the affected individual.

Why does kernicterus occur randomly?

Causes. Some cases of kernicterus occur randomly, for no apparent reason (sporadically). According to the medical literature, excess levels of bilirubin (hyperbilirubinemia) alone is not sufficient to produce kernicterus. Potential causes may include Rh disease and/or unknown factors.

Why is phototherapy used for kernicterus?

This helps to speed up the excretion of bilirubin from the skin and aids in its decomposition. As an affected individual ages, body mass increases and the skin thickens; phototherapy becomes less effective against preventing the symptoms and physical findings associated with kernicterus. Therefore, liver transplantation may be performed. Some researchers believe that liver transplantation should be performed at an early age, before brain damage potentially associated with kernicterus can develop.

How long does it take for kernicterus to appear?

In some cases, symptoms and physical findings of kernicterus appear two to five days after birth. Within the first few days of life, affected infants develop abnormally high levels of bilirubin in the blood (hyperbilirubinemia) and persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice).

What is kernicterus type 1?

In some rare cases, kernicterus may result from a rare disorder known as Crigler-Najjar Syndrome Type I. (For more information on this disorder, choose “Crigler Najjar” as your search term in the Rare Disease Database.)

What is the diagnosis of Kernicterus?

Kernicterus is reserved for a diagnosis with a combination of clinical features and MRI findings. The classic diagnosis includes a tetrad of features: (1) dystonia and/or athetosis; (2) hearing impairment or deafness; (3) lack of upward gaze; and (4) dental enamel dysplasia, along with abnormal MRI findings in the globus pallidus and subthalamic nuclei. Other less common accompanying symptoms may include hypotonia or sensorimotor abnormalities, and oculomotor abnormalities may vary and include strabismus or other misalignment of the eyes 34).

What are the long term effects of kernicterus?

Long-term outcomes of infants with kernicterus vary widely based on severity as well as a variety of risk factors described previously. Increased incidence of dyskinetic cerebral palsy occurs following high bilirubin exposure in full-term infants or low–moderate levels of bilirubin exposure in preterm infants 38). Cerebral palsy was most recently defined by an international committee as “a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain 39). The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, behavior, by epilepsy, and by secondary musculoskeletal problems” 40).

What is yellow staining in kernicterus?

Original autopsy studies of kernicterus identified yellow staining and necrosis of the basal ganglia, specifically in the globus pallidus, indicative of “unbound bilirubin” crossing the blood–brain barrier (BBB). Other regions noted to have cellular damage after hyperbilirubinemia include the substantia nigra reticulata, subthalamic nuclei, vestibular and oculomotor nuclei, hippocampus, and cerebellar Purkinje cells. Loss of neurons, decreased myelination, and gliosis can be observed in the internal and external globus pallidus and subthalamic nuclei. These regions all have functions related to movement, balance, and posture regulation and appear to be selectively vulnerable. The globus pallidus may also serve to integrate inputs from multiple systems including somatosensory and motor pathways; thus, integration of this information may be damaged in patients with kernicterus 17). By contrast, regions such as the striatum and thalamus generally appear to be spared following hyperbilirubinemia. This specific pattern of brain injury helps distinguish bilirubin-induced neurologic dysfunction from injury following hypoxic–ischemia, in which the caudate, putamen, posterior limb of the internal capsule, and cortex are more vulnerable. One theory for the selective vulnerability of the globus pallidus and subthalamic nuclei is their relatively high resting levels of neuronal activity observed in the neonatal brain, compared to nearby regions. Johnston et al. 18) observed that regions with higher neuronal activity are more vulnerable to oxidative stressors or toxins such as bilirubin. Lesions in these regions are observed in infants with classic athetotic cerebral palsy. One hypothesis for the connection between globus pallidus injury and athetosis is that reduced activity of the globus pallidus results in decreased inhibitory input to the thalamus, resulting in its “dys-inhibition” and increased motor activity, consistent with athetosis 19).

How to predict hyperbilirubinemia?

Therefore, the best available method for predicting severe hyperbilirubinemia appears to be the use of a timed total serum bilirubin measurement analyzed in the context of the infant’s gestational age . Infants of less than 38 weeks’ gestation whose total serum bilirubin concentration is greater than the 75th percentile have a greater than 10% risk of developing severe hyperbilirubinemia; similarly, infants of 39 to 40 weeks’ gestation whose total serum bilirubin concentration is above the 95th percentile have a greater than 10% risk 74).

What is the condition where babies have uncontrollable movements in the arms, legs, face and other body parts?

Athetoid cerebral palsy. Babies with this condition have uncontrollable movements in the arms, legs, face and other body parts.

What are the warning signs of kernicterus?

They should be educated to seek medical attention when any warning signs like decreased activity, altered mental status, and poor suckling reflex are present. Pearls and Other Issues. Traditionally, kernicterus is defined by neurologic damage due to excessive unconjugated bilirubin deposition in the brain tissue.

What is kernicterus in infants?

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. This activity reviews the evaluation and management of kernicterus and highlights the role of the interprofessional team in evaluating and improving care for patients with this condition.

Can a CT scan diagnose kernicterus?

Head ultrasound, CT, and MRI - not routinely used to diagnose kernicterus. They are indicated only when lab results are inadequate or inconclusive. They may aid in ruling out other causes of encephalopathy.

Is kernicterus more common in males or females?

The condition occurs primarily in children, mostly African Americans and South Asians, and is slightly more common in males than females. The incidence of kernicterus is not exactly known in the United States. Asian, Hispanic, Native American, and Eskimo infants have a higher production of bilirubin than white infants.

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How to tell if you have kernicterus?

Kernicterus symptoms depend on the levels of bilirubin build-up in the blood. Parents should look out for signs of severe jaundice so that treatment can begin immediately. These signs include poor feeding, unusual sleepiness, poor reflexes and yellowing skin.

What happens if you don't stop kernicterus?

If kernicterus is not stopped quickly, children may suffer from permanent brain damage, hearing loss and even death. Kernicterus can cause athetoid cerebral palsy and dystonic cerebral palsy. When a doctor fails to recognize signs of jaundice or kernicterus, it can be considered medical negligence.

What Causes Kernicterus?

Kernicterus starts with jaundice. Newborn jaundice occurs when there is an increase of a substance in the blood called bilirubin. Bilirubin is produced when red blood cells break down naturally. It is a toxic, yellowish substance that is removed from the blood by the liver and excreted from the body.

What is the term for the yellowing of the skin on the skin of a newborn?

Kernicterus is brain damage caused by severe newborn jaundice, a yellowing of the skin often seen in newborns. Although most cases of jaundice clear up on their own, severe cases can lead to brain damage if left untreated. Kernicterus means there are high levels of bilirubin (a yellow pigment from the liver) in the blood and toxic levels in ...

Can kernicterus occur even with low bilirubin?

Kernicterus has been known to occur even when bilirubin levels are lower than 25 milligrams. Doctors will perform a physical examination of your baby for the signs of kernicterus and whether brain damage has begun. Fortunately, prompt treatment can stop brain damage from progressing.

Can kernicterus be treated with multiple lights?

When kernicterus is involved, doctors will likely use multiple lights to speed up treatment. Doctors will not stop treatment until bilirubin levels fall to a safe level. Babies will be fed through a tube or intravenously.

Can kernicterus cause hearing loss?

This affects several critical areas of the brain involved in movement and hearing, potentially leading to cerebral palsy, hearing loss, other neurological issues and even death. Since kernicterus is preventable with proper treatment, the condition can be qualified as a birth injury.

How to diagnose kernicterus?

To diagnose kernicterus, your doctor will carefully assess your baby and their symptoms. General guidelines for postnatal care include monitoring and checking the baby’s bilirubin level via a blood test. Babi es with higher than 25 milligrams of total serum bilirubin in their blood will be treated, often in the hospital, until bilirubin levels decrease. Kernicterus can occur in babies with a bilirubin level below 25 mg, so it is important that doctors carefully examine and monitor the baby. Bilirubin levels alone are generally not enough to make a complete diagnosis. Your doctor must consider blood tests, symptoms and the child’s overall health. Immediate diagnosis and treatment can prevent brain damage from occurring.

How Does Kernicterus Affect Children?

Along with the aforementioned symptoms, Kernicterus can lead to a number of neurological disorders, some slight such as minor learning disabilities, and some as serious as athetoid cerebral palsy. Other risks and complications include complete hearing loss, and even death.

What is the brainstem affected by kernicterus?

Brain damage caused by kernicterus often affects parts of the brain responsible for hearing, movement and other motor functions. Primarily, this includes the brainstem and the basal ganglia. Kernicterus is linked to hearing loss, vision problems, athetoid cerebral palsy and intellectual disabilities.

What is kernicterus in babies?

Kernicterus, also known as Hyperbilirubinemia or bilirubin encephalopathy, is a rare neurological disorder that occurs when hyperbilirubinemia damages the brain. Whereas with jaundice treatment reduces bilirubin levels and the baby recovers, kernicterus means that bilirubin levels are toxic. Excess levels of bilirubin in the baby’s blood can enter brain tissue and cause damage. This occurs in around 5% of babies who have jaundice – or around one in every 44,000 newborns. Generally, jaundice is treated before bilirubin levels get too high and there is a risk of brain damage. Unfortunately, not all babies are diagnosed and treated quick enough to prevent kernicterus.

Why is kernicterus more common in babies?

Babies with a darker skin color are more likely to develop kernicterus because jaundice is more difficult to identify. That means that jaundice may go undiagnosed and untreated, which is a leading reason why the condition progresses into kernicterus.

How long does it take for a baby to show signs of kernicterus?

Symptoms of Kernicterus. Kernicterus symptoms will greatly depend upon if it’s in an early or later stage of the disorder. Generally, symptoms begin two to five days after the baby is born. The most notable symptom is, of course, the yellowing of the baby’s skin, whites of the eyes and mucous membranes.

Can Kernicterus be Prevented?

Since 60-80% of newborns have some level of jaundice, the best way to prevent kernicterus is to recognize the risk and take measures to diagnose and treat jaundice quickly. Although in many cases, there is no known way to discern which infant will develop jaundice severe enough to cause Kernicterus, there are some risk factors that increase the risk of a baby developing kernicterus. These include:

What does jaundice look like?

The whites of the eyes can also look yellow. Jaundice can be harder to see in babies with darker skin color. The baby’s doctor or nurse can test how much bilirubin is in the baby’s blood.

How to tell if a baby is crying?

Get emergency medical help if your baby: 1 Is crying inconsolably or with a high pitch. 2 Is arched like a bow (the head or neck and heels are bent backward and the body forward). 3 Has a stiff, limp, or floppy body. 4 Has strange eye movements.

Can kernicterus cause hearing loss?

It can cause athetoid cerebral palsy and hearing loss. Kernicterus also causes problems with vision and teeth and sometimes can cause intellectual disabilities. Early detection and management of jaundice can prevent kernicterus.