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What disorder leads to brain damage cause by high levels of bilirubin in the blood of the newborn? 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 blood disorder causes high bilirubin? Dubin-Johnson syndrome is a rare genetic liver disorder characterized by elevated levels of bilirubin in blood (hyperbilirubinemia). Persistent yellowing of the skin, mucous membranes and whites of the eyes (jaundice) is usually the only symptom in most cases. Dubin-Johnson syndrome is usually diagnosed after puberty.
How does bilirubin cause brain damage? HOW DO JAUNDICE & HYPERBILIRUBINEMIA CAUSE BRAIN DAMAGE, KERNICTERUS, ENCEPHALOPATHY, CEREBRAL PALSY & SEIZURES? Bilirubin is a brain toxin. When bilirubin levels get too high, bilirubin can pass through the blood brain barrier and enter brain tissue.
What happens if a baby’s bilirubin is too high? High levels of bilirubin can travel to your baby’s brain. This can cause seizures and brain damage. This is called kernicterus.
What is kernicterus? Kernicterus is a very rare type of brain damage that occurs in a newborn with severe jaundice. It happens when a substance in the blood, called bilirubin, builds up to very high levels and spreads into the brain tissues. This causes permanent brain damage.
An abnormal gene you inherit from your parents causes Gilbert’s syndrome. The gene normally controls an enzyme that helps break down bilirubin in your liver. When you have an ineffective gene, your blood contains excess amounts of bilirubin because your body doesn’t produce enough of the enzyme.
Causes of Gilbert’s syndrome
Gilbert’s syndrome is a genetic condition that runs in families. People with the syndrome have a faulty gene, which causes the liver to have problems removing bilirubin from the blood.
high bilirubin levels (hyperbilirubinemia) can cause a form of brain damage called kernicterus. Also, many disorders that cause jaundice, particularly severe liver disease, cause other symptoms or serious problems.
When the level of unconjugated bilirubin gets very high, it can move out of the blood and into the brain tissue. Unconjugated bilirubin can lead to kernicterus if something causes it to build up. Conjugated bilirubin does not cross from the blood into the brain and can usually be removed from your body.
In industrialized countries, only 0.4 to 2.7 of every 100, 000 children develop kernicterus or a condition called acute bilirubin encephalopathy. This is where bilirubin levels are so high they spread to the brain and damage the central nervous system tissues.
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.
Jaundice is considered pathologic if it presents within the first 24 hours after birth, the total serum bilirubin level rises by more than 5 mg per dL (86 mol per L) per day or is higher than 17 mg per dL (290 mol per L), or an infant has signs and symptoms suggestive of serious illness.
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.
Bilirubin encephalopathy is a rare neurological condition that occurs in some newborns with severe jaundice. Kernicterus is a condition where very high bilirubin levels in the blood are deposited in the brain tissue causing irreversible damage to the brain.
Classical kernicterus or chronic bilirubin encephalopathy is a well-described clinical syndrome which includes a tetrad of (i) abnormal motor control, movements and muscle tone, (ii) an auditory processing disturbance with or without hearing loss, (iii) oculomotor impairments, especially impairment of upward vertical
People with Gilbert’s syndrome inherit a mutated gene that affects the liver’s ability to process bilirubin, a waste product that forms during the breakdown of old red blood cells. Jaundice (yellowish skin and eyes) occurs when too much bilirubin builds up in blood.
If you have Gilbert’s syndrome, you might notice these symptoms more if you do things that can further increase your bilirubin levels, such as: experiencing emotional or physical stress. exercising vigorously. not eating for a long period of time.
Thus 40 articles and 2 case reports were thoroughly studied. It was concluded that Gilbert’s syndrome has immense clinical importance because the mild hyperbilirubinemia can be mistaken for a sign of occult, chronic, or progressive liver disease.
An elevation greater than 20 mg/dL suggests severe liver disease. In patients with hepatitis-induced acute liver failure, a serum total bilirubin level > 17.5 mg/dL (300 mmol/L) is a criterion for predicting death and the need for liver transplantation.
A Benign Condition
The reference range for total bilirubin in the bloodstream for a healthy individual is between 1-17 µmol/L , whereas individuals with Gilbert’s syndrome usually have a slightly elevated level, between the ranges of 20-50 µmol/L .
Chronic bilirubin encephalopathy (kernicterus) can be diagnosed using semi-objective criteria based on history, physical and neurological examination and laboratory findings including auditory brainstem responses and magnetic resonance imaging.
Acute bilirubin encephalopathy encompasses the acute illness caused by severe hyperbilirubinemia. Presenting signs and symptoms include decreased feeding, lethargy, abnormal tone (hypotonia and/or hypertonia), high-pitched cry, retrocollis and opisthotonus, setting-sun sign, fever, seizures, and possibly death [6,7].
How is kernicterus diagnosed? Your doctor diagnoses kernicterus through a physical exam and knowledge of your child’s history of symptoms. Blood tests to measure your baby’s bilirubin levels are also done. Once a baby has kernicterus, brain damage has already occurred.
Kernicterus in an adult is very rare. In this case, extracorporeal charcoal-column perfusion treatment led repeatedly to severe depletion of fibrinogen, with extensive hemorrhages.
Regions most commonly affected include the basal ganglia; hippocampus; geniculate bodies; and cranial nerve nuclei, such as the oculomotor, vestibular, and cochlear. The cerebellum can also be affected.