Disease Information for Kernicterus: Definition

Ads
  • A term used pathologically to describe BILIRUBIN staining of the BASAL GANGLIA; BRAIN STEM; and CEREBELLUM and clinically to describe a syndrome associated with HYPERBILIRUBINEMIA; Clinical features include athetosis, MUSCLE SPASTICITY or hypotonia, impaired vertical gaze, and DEAFNESS; Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the BLOOD-BRAIN BARRIER (eg, SEPSIS); This condition occurs primarily in neonates (INFANT, NEWBORN), (Menkes, Textbook of Child Neurology, 5th ed, p613)

    ----------------------------Kernicterus; Bilirubin Encephalopathy; Kernicterus 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 by-product 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 (eg, 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); infants with kernicterus may develop life-threatening complications-------(NORD 2005)-----------

    (Edit)

Ads