Kernicterus
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdulkerim Yassin, M.B.B.S[2]
Synonyms and keywords: Chronic bilirubin encephalopathy
Overview
Kernicterus is irreversible brain damage due to chronic high levels of unconjugated bilirubin in the baby`s blood which is not treated early. Hyperbilirubinemia frequently occurs in majority of newborn infants but mostly it is benign and in severe cases can progress to kernicterus and developmental abnormalities. The risk of bilirubin induced neurologic damage and kernicterus are more in preterm than term neonates and the former suffer adverse effects at lower total bilirubin levels with worse long-term outcomes. Liver metabolizes and excretes bilirubin. During pregnancy, the mother`s liver does it for the baby. After birth, some of the baby`s liver enzyme not well developed specially in preterm, bilirubin raises in the baby`s blood and accumulates in the skin and sclera of eyes and cause jaundice. When the jaundice gets severe, The tissues protecting the brain (the blood-brain barrier) are immature in newborns. Bilirubin penetrates the brain and is deposited in the basal ganglia,hippocampus, geniculate bodies and cranial nerve nuclei causing irreversible damage. Depending on the level of exposure, the effects range from unnoticeable to severe brain damage. When the jaundice occurs within (24 hours) of life is always pathological, whereas it happens after 24 hours of life, it can be physiological. Several underlying pathologic processes responsible for hyperbilirubinemia are G6PD deficiency, Crigler-Najjar syndrome, Gilbert syndrome, hemolytic disorders, and a decreased ability to conjugate bilirubin in neonates and infants. Newborn babies are often polycythemic, meaning they have too many red blood cells. When they break down the cells, one of the byproducts is bilirubin, which circulates in the blood and causes jaundice. When hyperbilirubinemia occurs in adult and older children, it is frequently due to liver abnormalities. Some medications, such as the antibiotic co-trimoxazole (a combination of trimethoprim/sulfamethoxazole) may induce this disorder in the baby, either when taken by the mother or given directly to the baby, due to displacement of bilirubin from binding sites on serum albumin. The bilirubin is then free to pass into the Central Nervous System, because the baby's blood-brain barrier is not fully developed. In the (first 48 hrs of life), A baby should be checked for jaundice and if it is discharged before 72 hrs, the baby should be seen after 2 days. The treatment is phototherapy and exchange transfusion.
Historical Perspective
- Kernicterus was first discovered by Christrian Georg Schmorl, a German Pathologist, in 1904 [1].
- The association between hyperbilirubinemia and kernicterus was made in 1875[1].
In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].
Classification
- Based on the duration of symptoms, bilirubin encephalopathy may be classified as either acute, subtle or chronic.
- Acute bilirubin encephalopathy: comprises the cute illness caused by severe hyperbilirubinemia. The sign and symptom includes decreased feeding, lethargy, hypotonia and/or hypertonia, high-pitched cry, retrocollis and opisthotonus, setting-sun sign, fever, seizures, and may be death.
- Subtle bilirubin encephalopathy/Bilirubin induced neurologic dysfunction: defined by the presence of insidious developmental disorders without the classical findings of kernicterus. This may presents with neurodevelopmental disorders such as sensory and sensorimotor integration disorders, hypotonia, ataxia or clumsiness, aphasia, and auditory neuropathy which is impaired auditory brainstem reflexes with normal otoacoustic emissions or cochlear microphonic responses.
- Chronic bilirubin encephalopathy: the long-term outcome of acute bilirubin encephalopathy and composed of a tetrad of clinical characteristics that are typically appear after one year of age:
- Abnormal motor control, movements, and muscle tone;
- An auditory processing disturbance with or without hearing loss;
- Oculomotor impairments, especially impairment of the upward vertical gaze; and
- An auditory processing disturbance with or without hearing loss;
- Abnormal motor control, movements, and muscle tone;
Pathophysiology
The neonatal hyperbilirubinemia occurs due to increased production or limited excretion of indirect or unconjugated bilirubin. Newborns baby, especially preterm neonates, have higher rates of bilirubin production than adults, because an increased red cells turnover and a shorter life span. In newborn neonates, unconjugated bilirubin is not easily excreted, and there is limited bilirubin congugation which lead to physiologic jaundice. Excessive physiologic jaundice occurs at values above 7 to 17 mg/dl [104 to 291 μmol/l]). Serum bilirubin concentrations greater than 17 m/dl in full-term infants are no longer considered physiologic and pathologic causes should be identified, of which the most common are Crigler-Najjar syndrome, Gilbert syndrome, hemolytic disorders, and a reduced ability to conjugate bilirubin in newborn babies[3].
Causes
Bilirubin is a yellow pigment produced by the breakdown of hemoglobin in old or hemolyzed red blood cells. High levels of bilirubin is due to either increased production, decreased hepatic uptake, impaired conjugation, or increased enterohepatic circulation. Kernicterus is caused by very high levels of indirect or unconjugated bilirubin in the blood which crosses blood brain barrier and yellow staining of brain tissues that leads to brain damage and hearing loss[3].
Differentiating ((Page name)) from other Diseases
[Disease name] must be differentiated from other diseases that cause [clinical feature 1]
Epidemiology and Demographics
Kernicterus happens in all parts of the world. Geographic areas where glucose-6-phosphate dehydrogenase-deficiency is common, the risk of kernicterus is higher. The incidence rate of kernicterus in Sweden is 1.3 per 100 000 births which is slightly higher than that in other population-based studies in high-resource settings. The incidence rate of kernicterus in Canada, California, and Denmark, has been reported to be 0.5 to 1 per 100 000 births, whereas in Norway, the incidence rate has been estimated to be less than 0.5 per 100 000 births[4]. The risk of kernicterus is higher in male newborns than female. However, the mechanism is unknown[5].
Risk Factors
Screening
There is insufficient evidence to recommend routine screening for kernicterus[7].
Natural History, Complications, Prognosis
Kernicterus is a very rare type of brain damage that occurs in a newborn with severe jaundice.The term kernicterus, which refers to yellow staining of the brainstem nuclei (Greek for ‘jaundice of the nuclei’).
References
- ↑ 1.0 1.1 [+https://doi.org/10.1542/neo.4-2-e33 "Kernicterus: Past, Present, and Future | American Academy of Pediatrics"] Check
|url=value (help). - ↑ Das S, van Landeghem FKH (2019). "Clinicopathological Spectrum of Bilirubin Encephalopathy/Kernicterus". Diagnostics (Basel). 9 (1). doi:10.3390/diagnostics9010024. PMC 6468386. PMID 30823396.
- ↑ 3.0 3.1 Dennery PA, Seidman DS, Stevenson DK (2001). "Neonatal hyperbilirubinemia". N Engl J Med. 344 (8): 581–90. doi:10.1056/NEJM200102223440807. PMID 11207355.
- ↑ Alkén J, Håkansson S, Ekéus C, Gustafson P, Norman M (2019). "Rates of Extreme Neonatal Hyperbilirubinemia and Kernicterus in Children and Adherence to National Guidelines for Screening, Diagnosis, and Treatment in Sweden". JAMA Netw Open. 2 (3): e190858. doi:10.1001/jamanetworkopen.2019.0858. PMC 6583272 Check
|pmc=value (help). PMID 30901042. - ↑ Freudenberger DC, Shah RD (2021). "A narrative review of the health disparities associated with malignant pleural mesothelioma". J Thorac Dis. 13 (6): 3809–3815. doi:10.21037/jtd-20-3516. PMC 8264689 Check
|pmc=value (help). PMID 34277071 Check|pmid=value (help). - ↑ "What are Jaundice and Kernicterus? | CDC".
- ↑ Trikalinos TA, Chung M, Lau J, Ip S (2009). "Systematic review of screening for bilirubin encephalopathy in neonates". Pediatrics. 124 (4): 1162–71. doi:10.1542/peds.2008-3545. PMID 19786450.