kernicterus


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Related to kernicterus: hyperbilirubinemia

kernicterus

 [ker-nik´ter-us]
a condition in the newborn marked by severe neural symptoms, associated with high levels of bilirubin in the blood; it is commonly a sequela of icterus gravis neonatorum.

ker·nic·ter·us

(ker-nik'tĕr-ŭs),
Jaundice associated with high levels of unconjugated bilirubin, or in small premature infants with more modest degrees of bilirubinemia; yellow staining and degenerative lesions are found chiefly in basal ganglia including in the lenticular nucleus, subthalamus, Ammon horn, and other areas; may occur with hemolytic disorder such as Rh or ABO erythroblastosis or G6PD deficiency as well as with neonatal sepsis or Crigler-Najjar syndrome; characterized early clinically by opisthotonos, high-pitched cry, lethargy, and poor sucking, as well as abnormal or absent Moro reflex, and loss of upward gaze; later consequences include deafness, cerebral palsy, other sensorineural deficits, and mental retardation.
[Ger. Kern, kernel (nucleus), + Ikterus, jaundice]

kernicterus

Bilirubin encephalopathy Neonatology The staining of parts of the infant brain, especially the basal ganglia and hippocampus by BR that has penetrated the blood-brain barrier which, in older children, is more impervious to bilirubin; kernicterus is classically linked to Rh HDN, when the immune system of a mother who does not have the RhD–less commonly C, c, E, e, or other antigen on her RBCs, comes in contact with the infant's RBCs and forms antibodies to them; this causes a brisk hemolysis and ↑ BR; serum levels of ≥ 20 mg/dL of BR pose
a high risk for kernicterus, and represent a medical emergency; severe kernicterus is often fatal, and characterized by lethargy, poor feeding, hypertonicity, seizures and apnea; survivors have sequelae in the form of dental dysplasia, cerebral palsy, hearing loss Clinical, full term infants Severe jaundice, lethargy, poor feeding, choreoathetoid cerebral palsy, mental retardation, sensorineural hearing loss, gaze paresis. See Hemolytic disease of the newborn, Jaundice.

ker·nic·ter·us

(kĕr-nik'tĕr-ŭs)
Yellow staining and degenerative lesions in basal ganglia associated with high levels of unconjugated bilirubin in infants; may occur with hemolytic disorder such as Rh or ABO erythroblastosis or G6PD deficiency as well as with neonatal sepsis or Crigler-Najjar syndrome; characterized by opisthotonos, high-pitched cry, lethargy, and poor suckling, as well as abnormal or absent Moro reflex, and loss of upward gaze; later consequences include deafness, cerebral palsy, other sensorineural deficits, and mental retardation.
Synonym(s): bilirubin encephalopathy, nuclear jaundice.
[Ger. Kern, kernel (nucleus), + ikterus, jaundice]

kernicterus

Jaundice of the brain resulting from RHESUS FACTOR disease in babies in which excessive red cell breakdown results in the release of large quantities of BILIRUBIN. Death is common before, or within a week or two after, birth. Surviving infants feed poorly, suffer varying degrees of paralysis, epilepsy, spasticity of the muscles, mental retardation, deafness and blindness. Kernicterus is preventable by prenatal diagnosis and treatment.

Kernicterus

A potentially lethal disease of newborns caused by excessive accumulation of the bile pigment bilirubin.

ker·nic·ter·us

(kĕr-nik'tĕr-ŭs)
Yellow staining and degenerative lesions in basal ganglia associated with high levels of unconjugated bilirubin in infants; characterized by opisthotonos, high-pitched cry, lethargy and poor sucking, and loss of upward gaze; later consequences include deafness, cerebral palsy, other sensorineural deficits, and mental retardation.
Synonym(s): bilirubin encephalopathy, nuclear jaundice.
[Ger. Kern, kernel (nucleus), + ikterus, jaundice]
References in periodicals archive ?
Aetiology of cerebral palsy and associated problems (N=187) Aetiology, n (%) Birth asphyxia 107 (57.2) CNS infection 32 (17.1) Kernicterus 22 (11.8) Neonatal seizure 3 (1.6) Premature birth 14 (7.5) Trauma 3 (1.6) Unknown 6 (3.2) Associated problems, n (%) Speech impairment 119 (63.6) Bladder control problems 22 (11.8) OMI 25 (13.4) Intellectual disability 9 (4.8) Learning disability 10 (5.3) Seizure disorders 40 (21.4) Emotional and behavioural disorder 4 (2.1) Visual impairment 11 (5.9) Hearing impairment 4 (2.1) None 44 (23.5) CNS = central nervous system (meningitis, cerebral malaria and encephalitis); OMI = oral motor impairment (including problems with feeding, swallowing and drooling).
Exchange transfusion with D negative blood greatly increased the survival rate and almost removed the risk of kernicterus. [36] Harvey Klein and David Anstee pointed out that in 80% of cases postnatal exchange transfusion was not required when the foetus had been transfused prenatally for severe disease.
Why is kernicterus still a major cause of death and disability in low-income and middle-income countries?
Kernicterus is a preventable condition; the unconjugated bilirubin levels can be decreased to safe levels by the use of phototherapy or exchange transfusion.
Kernicterus can arise when bilirubin is displaced from albumin by drugs such as ibuprofen, ceftriaxone and co-trimoxazole.
prematurity has short and long-term complications which include respiratory distress syndrome, dysplasia, anemia, fatigue, kernicterus, intraventricular hemorrhage, bleeding into or around the abdominal, bacterial or fungal sepsis, retinopathy, necrotizing enterocolitis, learning and behavioral and cognitive problems (low IQ), mental retardation, blindness, hearing loss, and developmental problems (8-10).
The severe hyperbilirubinemia can lead to kernicterus and neurodevelopmental abnormalities such as hearing loss, athetosis, and, rarely, intellectual deficits [8].
Missing a case of severe hyperbilirubinemia with resultant kernicterus is totally unacceptable.
They also discuss the pathobiology of bilirubin-induced neurotoxicity, the clinical diagnosis and outcome of kernicterus, the contributions of hemolytic disease and glucose-6-phosphate dehydrogenase deficiency to hyperbilirubinemia, and risk assessment and treatment with phototherapy and other modalities.
However, their newborns suffered no organic abnormalities such as kernicterus or neurodevelopmental disorders.
Dysmorphic babies, neonates with kernicterus, grade III birth asphyxia, neonates on antibiotics or those cases where mothers have received antibiotics before delivery were excluded from the study.