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Brain damage due to sustained elevations of bilirubin...no exact number on it....combination of factors...trauma due to birth (pre-hepatic) and immature liver (hepatic)
Curtis Strite
Wiki User
∙ 13y agoKernicterus is a rare neurological condition that occurs in some newborns with severe jaundice.
See also: Newborn jaundice
Alternative NamesBilirubin encephalopathy
Causes, incidence, and risk factorsKernicterus is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created in the body during the normal recycling of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow (which is called jaundice).
In some cases when there are extremely high levels of bilirubin in the body or the baby is extremely ill, the substance will move out of the blood and collect in the brain tissue. This can lead to serious neurological complications, including brain damage.
Kernicterus usually develops in the first week of life, but may be seen up until the third week. Newborns with Rh hemolytic disease that leads to hydrops fetalis are at high risk for severe jaundice that leads to this condition. However, kernicterus has been seen in apparently healthy babies.
SymptomsThe symptoms depend on the stage of kernicterus.
Early stage:
- Extreme jaundice
- Absent startle reflex
- Poor breast-feeding or sucking
- Extreme sleepiness (lethargy)
Mid stage:
- High-pitched cry
- Arched back with neck hyperextended backwards
- Bulging fontanel (soft spot)
- Seizures
Late stage (full neurological syndrome):
- High-frequency hearing loss
- Mental retardation
- Muscle rigidity
- Speech difficulties
- Seizures
- Movement disorder
A blood test will show a high bilirubin level (greater than 20-25 mg/dL).
Note: Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.
Treatment- Phototherapy
- Exchange transfusion (may require multiple exchanges)
Treatment depends on how old the baby is (in hours) and whether the baby has any risk factors (such as prematurity).
Expectations (prognosis)Kernicterus represents an extreme condition caused by hemolysis. The outcome is guarded. Many infants with late stage neurological syndrome die.
Complications- Permanent brain damage
- Death
Seek immediate medical help if your baby has signs of this condition.
PreventionEarly diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent this complication. The American Academy of Pediatrics recommends that infants with the first signs of jaundice have their bilirubin level measured within 24 hours. If the level is high, the infant should be screened for diseases that involve the destruction of red blood cells (hemolysis).
The association also recommends that all newborns have a follow-up appointment within 2 to 3 days after leaving the hospital. This is particularly important for premature or near-term babies.
ReferencesAmerican Academy of Pediatrics, Subcommittee on Neonatal Hyperbilirubinemia: Neonatal jaundice and kernicterus. Pediatrics. 2001;108:763.
American Academy of Pediatrics, Subcommittee on Hyperbilirubinemia Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114: 297-316.
In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 102.
Wiki User
∙ 12y agoKernicterus is a rare neurological condition that occurs in some newborns with severe jaundice.
See also: Newborn jaundice
Alternative NamesBilirubin encephalopathy
Causes, incidence, and risk factorsKernicterus is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created in the body during the normal recycling of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow (which is called jaundice).
In some cases when there are extremely high levels of bilirubin in the body or the baby is extremely ill, the substance will move out of the blood and collect in the brain tissue. This can lead to serious neurological complications, including brain damage and hearing loss.
Kernicterus usually develops in the first week of life, but may be seen up until the third week. Newborns with Rh hemolytic disease that may lead to hydrops fetalis are at high risk for severe jaundice that leads to this condition. However, kernicterus has been seen in apparently healthy babies.
SymptomsThe symptoms depend on the stage of kernicterus.
Early stage:
- Extreme jaundice
- Absent startle reflex
- Poor feeding or sucking
- Extreme sleepiness (lethargy)
Mid stage:
- High-pitched cry
- Arched back with neck hyperextended backwards
- Bulging fontanel (soft spot)
- Seizures
Late stage (full neurological syndrome):
- High-frequency hearing loss
- Mental retardation
- Muscle rigidity
- Speech difficulties
- Seizures
- Movement disorder
A blood test will show a high bilirubin level (greater than 20-25 mg/dL).
Note: Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.
TreatmentTreatment depends on how old the baby is (in hours) and whether the baby has any risk factors (such as prematurity). It may include:
- Light therapy (phototherapy)
- Exchange transfusions
Kernicterus is a serious condition. The outcome is guarded. Many infants with late stage nervous system complications die.
Complications- Permanent brain damage
- Hearing loss
- Death
Seek immediate medical help if your baby has signs of this condition.
PreventionEarly diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent this complication. The American Academy of Pediatrics recommends that infants with the first signs of jaundice have their bilirubin level measured within 24 hours. If the level is high, the infant should be screened for diseases that involve the destruction of red blood cells (hemolysis).
The association also recommends that all newborns have a follow-up appointment within 2 to 3 days after leaving the hospital. This is particularly important for premature or near-term babies.
ReferencesAmerican Academy of Pediatrics. Committee on Fetus and Newborn. Hospital stay for healthy term newborns. Pedatrics. 2010; 125: 405.
American Academy of Pediatrics, Subcommittee on Hyperbilirubinemia Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114: 297-316.
Reviewed ByReview Date: 05/09/2011
Kimberly G Lee, MD, MSc, IBCLC, Associate Professor of Pediatrics, Division of Neonatology, Medical University of South Carolina, Charleston, SC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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What is kernicterus?
Kernicterus-- A potentially lethal disease of newborns caused by excessive accumulation of the bile pigment bilirubin.
What complications may result from high levels of bilirubin in infants?
kernicterus, a form of brain damage. Signs of severe hyperbilirubinemia include listlessness, high-pitched crying, apnea (periods of not breathing), arching of the back, and seizures
Will sulfamethoxazole be harmful if you get pregnant while on it?
There is a possibility to cause oaf for those who takes sulfamethoxazole within the early 16 pregnant weeks, and kernicterus if the medicine is used several weeks before the pregnant is braught to bed. Therefore, it should only be used for the middle pregnant period.
What is the prognosis for hemolytic anemia?
In about 15% of cases, the baby is severely affected and dies before birth. Babies who survive pregnancy may develop kernicterus, which can lead to deafness, speech problems, cerebral palsy, or mental retardation.
What are the symptoms of bilirubin encephalopathy?
Kernicterus or bilirubin encephalopathy is a rare neurological disorder characterized by excessive levels of bilirubin in the blood (hyperbilirubinemia) during infancy. Bilirubin is an orange-yellow bile pigment that is a byproduct of the natural breakdown of hemoglobin in red blood cells (hemolysis). Toxic levels of bilirubin may accumulate in the brain, potentially resulting in a variety of symptoms and physical findings.These symptoms may include lack of energy (lethargy), poor feeding habits, fever, and vomiting. Affected infants may also experience the absence of certain reflexes (e.g., Moro reflex, etc.); mild to severe muscle spasms including those in which the head and heels are bent backward and the body bows forward (opisthotonus); and/or uncontrolled involuntary muscle movements (spasticity). In some cases, infants with kernicterus may develop life-threatening complications.
Can jaundice be prevented?
the activity of the enzyme UDP-glucuronyl transferase is low in the newborns.so UDP- glucuronic acid for conjugation is limited. this leads to high level of unconjugated bilirubin (beyond 25mg/dl) .it may cross the blood brain barrier resulting in hyperbilirubinemic toxic encephalopathy or kernicterus . prevention aspects: the drug phenobarbital is used, as it induce bilirubin metabolising enzymes in liver. in some neonates blood transfusion may be necessaryto prevent brain damage. phototherapy is continuously carried out till the serum bilirubin becomes normal. phototheraphy deals with the exposure of the jaundiced neonates to blue light as bilirubin absorbs the blue light and get converted to non toxic lumirubin and get excreted.
Can neonatal jaundice be prevented?
the activity of the enzyme UDP-glucuronyl transferase is low in the newborns.so UDP- glucuronic acid for conjugation is limited. this leads to high level of unconjugated bilirubin (beyond 25mg/dl) .it may cross the blood brain barrier resulting in hyperbilirubinemic toxic encephalopathy or kernicterus . prevention aspects: the drug phenobarbital is used, as it induce bilirubin metabolising enzymes in liver. in some neonates blood transfusion may be necessaryto prevent brain damage. phototherapy is continuously carried out till the serum bilirubin becomes normal. phototheraphy deals with the exposure of the jaundiced neonates to blue light as bilirubin absorbs the blue light and get converted to non toxic lumirubin and get excreted.
Is breastfeeding and taking sulfamethoxazoleTMP safe for your newborn?
This is for your physician to decide. While more dangerous in near term, there are reasons why this (alone or in synergy) may be the drug of choice. While there is a warning about early pregnancy, it does not rule out the usage of this med. ---- I respectfully disagree with the author of the answer above for the following reason: Sulfonamide antibiotics are not given to women who are breastfeeding, especially newborns, since these medications are excreted in breastmilk and are known to increase the bilirubin in newborns by displacing it from proteins in the blood, increasing the risk of kernicterus, a debilitating and permanent condition in which bilirubin stains certain areas of the brain, leading to brain damage and mental and physical retardation. If you are breastfeeding, you MUST tell your physician so s/he can take that into account when prescribing any medication, as most will pass through the breastmilk and potentially be absorbed by your child. ------------- The respectful disagreement is right on target. I'll leave the first up there, as -- while I was incomplete, what little I did say had some points that weren't totally off. Of the two answers choose the second.
What is a sickness starting with k?
Kabuki Make-Up Syndrome (not on MeSH) Kallmann Syndrome Kanner's Syndrome Kaposi Disease Kartagener Syndrome Kartagener Triad Kawasaki Disease Kearns Syndrome Kearns-Sayer Syndrome Keloid Kennedy Syndrome Keratitis Keratitis, Ulcerative Keratoconus Keratosis Follicularis Keratosis, Actinic (not on MeSH) Keratosis, Seborrheic Kernicterus Ketoacidosis, Diabetic Ketosis, Diabetic Kidney Calculi Kidney Diseases Kidney Diseases, Cystic Kidney Failure, Acute Kidney Failure, Chronic Kidney Stones Kidney Tubular Necrosis, Acute Kienbock Disease Kimura Disease Kinky Hair Syndrome Kissing Disease Klebsiella Infections Kleine-Levin Syndrome Klein-Waardenburg Syndrome Kleptomania Klinefelter Syndrome Klippel-Feil Syndrome Klippel-Trenaunay Disease Klippel-Trenaunay-Weber Syndrome Klumpke Paralysis Kniest Dysplasia Koehler Disease Konzo Krabbe Disease Krukenberg Tumor Kufs Disease Kugelberg-Welander Disease Kuru Kuru Encephalopathy Kussmaul Aphasia Kwashiorkor
What is the medication therapy for patent ductus arteriosis?
Of all the congenital heart diseases, patent ductus arteriosis (PDA) is the only disease that can be treated.The PDA treatment algorithm includes the following:Fluid restrictionDiureticsNon-steroidal anti-inflammatory drugs (NSAIDs) [See elaboration below.] Indomethacin, ORIbuprofenSurgical ligation When NSAIDs fail to treat the PDA.NSAIDs Comparison:Indomethacin Doses: Initial dose: 0.2 mg/Kg Intravenously (IV)Subsequent doses varies Post natal age - increase dose with increased ageInfant renal function - increase dosing interval for poor renal function.Ibuprofen Doses: Initial dose: 10 mg/Kg IVSubsequent dose: 5 mg/Kg IV daily for 2 days.Note: Calculate dosing for both drugs using the infant's birth weight.COX-1 inhibition (COX-1 is responsible for inflammation, so higher inhibition of COX-1 is preferred.) Indomethacin > IbuprofenStability Indomethacin (12 days) > Ibuprofen (30 minutes)Degree of protein binding (May cause accumulation of bilirubin in the brain, otherwise known as "kernicterus," which is associated with cerebral palsy.) Occurs in both NSAIDsMore incidents reported on ibuprofen.Efficacy in treating PDA Indomethacin = IbuprofenIndomethacin is generally the preferred medication for PDA treatment.Adverse Effects from Both NSAIDs Oliguria, renal failureGastrointestinal bleedingBowel perforationNecrotizing enterocolitisIntraventricular hemorrhageThrombocytopeniaContraindication to use NSAIDs (Do not use NSAIDs, if the following occurs.) Necrotizing enterocolitisIntraventricular hemorrhageActive bleedingThrombocytopeniaImpaired renal functions
Transient familial hyperbilirubinemia?
DefinitionTransient familial hyperbilirubinemia is a metabolic disorder that is passed down through families. Babies with this disorder are born with severe jaundice.Alternative NamesLucey-Driscol syndromeCauses, incidence, and risk factorsTransient familial hyperbilirubinemia is an inherited disorder. It occurs when the body does not properly break down (metabolize) a certain form of bilirubin. Bilirubin levels rapidly build up in the body. The high levels are poisonous to the brain and can cause death.SymptomsThe newborn may have:Yellow skin (jaundice)Yellow eyes (icterus)LethargyIf untreated, seizures and neurologic problems (kernicterus) may develop.Signs and testsBlood tests for bilirubin levels can identify the severity of the jaundice.TreatmentPhototherapywith blue light is used to treat the high level of bilirubin. An exchange transfusion is sometimes necessary.Expectations (prognosis)Babies who are treated can have a good outcome. If the condition is not treated, severe complications develop. This disorder tends to improve with time.ComplicationsDeath or severe brain and nervous system (neurological) problems can occur if the condition is not treated.Calling your health care providerAlthough this problem is usually found immediately after delivery, call your doctor if you notice your baby's skin turning yellow. There are other causes for jaundice in the newborn that are easily treated.Genetic counseling can help families understand the condition, its risks of recurring, and how to care for the patient.PreventionPhototherapy can help prevent the potentially serious complications of this disorder.
Crigler-Najjar syndrome?
DefinitionCrigler-Najjar syndrome is a very rare inherited disorder in which bilirubin (a substance made by the liver) cannot be broken down.Alternative NamesGlucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar)Causes, incidence, and risk factorsCrigler-Najjar syndrome is caused by an abnormal gene. The gene fails to make the enzyme that normally converts bilirubininto a form that can easily be removed from the body. Without this enzyme, bilirubin can build up in the body and lead to jaundice (yellow discoloration of skin and eyes) and damage to the brain, muscles, and nerves.Crigler-Najjar (type 1) is the early-onset form of the disease. Arias syndrome (type 2) is a later-onset condition.The syndrome runs in families (inherited). A child must get the defective gene from both parents to develop the severe form of the condition. Parents who are carriers (with just one defective gene) have about half the enzyme activity of a normal adult.SymptomsConfusion and changes in thinkingYellow skin (jaundice) and yellow in the whites of the eyes (icterus), which begin a few days after birth and get worse over timeSigns and testsTests used to evaluate liver function include:Conjugated (bound) bilirubinLiver biopsy, enzyme assayTotal bilirubin levelUnconjugated (unbound) bilirubin in bloodTreatmentLight treatment (phototherapy) is needed on a regular basis throughout life. In infants this is done using bilirubin lights (bili or 'blue' lights). Phototherapy becomes less successful after age 4, because thickened skin blocks the light.Liver transplantation has been used successfully in some people with type 1 disease.Blood transfusions may help control the amount of bilirubin in blood plasma. Calcium compounds are sometimes used to bind with and remove bilirubin in the gut.The drug phenobarbitol is sometimes used to treat Arias syndrome (type 2).Expectations (prognosis)Milder forms of the disease (type 2) do not cause severe toxicity, liver damage, or changes in thinking during childhood. People affected still have jaundice, but they have fewer symptoms and less organ damage.Infants with the severe form of the disease (type 1) may continue to have jaundice into adulthood, and may need daily treatment. If left untreated, this severe form of the disease will lead to death in childhood.People with this condition who reach adulthood will develop brain damage due to jaundice (kernicterus), even with regular treatment. The life expectancy for type 1 disease is 30 years.ComplicationsPossible complications include:A form of brain damage caused by jaundice (kernicterus)Chronic yellow skin/eyesCalling your health care providerSeek genetic counseling if you are planning to have children and have a family history of Crigler-Najjar.Call your health care provider if you or your newborn infant has jaundice that does not go away.PreventionGenetic counseling is recommended for prospective parents with a family history of Crigler-Najjar syndrome. Blood testing can identify people who carry the gene.ReferencesCarey RG, Balistreri WF. Metabolic Diseases of the Liver. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 354.
