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Acute bilirubin encephalopathy

MedGen UID:
1808922
Concept ID:
C5671282
Disease or Syndrome
Synonyms: ABE; acute bilirubin encephalopathy; Acute kernicterus; Acute kernicterus of newborn
SNOMED CT: Acute bilirubin encephalopathy (1197757006); Acute kernicterus of newborn (1197757006)
 
Monarch Initiative: MONDO:0035344
Orphanet: ORPHA529799

Definition

A rare neurologic disease characterised by lethargy, hypotonia, poor feeding, opisthotonus and a typical high-pitched cry due to bilirubin accumulation in the globus pallidus, sub-thalamic nuclei, and other brain regions, resulting from severe neonatal unconjugated hyperbilirubinaemia. Onset of symptoms is typically within the first three to five days of life. Additional features include fever, apnoea, seizures and coma. Respiratory failure or refractory seizures may lead to a fatal outcome. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute bilirubin encephalopathy

Professional guidelines

PubMed

Horn D, Ehret D, Gautham KS, Soll R
Cochrane Database Syst Rev 2021 Jul 6;7(7):CD013277. doi: 10.1002/14651858.CD013277.pub2. PMID: 34228352Free PMC Article
Muchowski KE
Am Fam Physician 2014 Jun 1;89(11):873-8. PMID: 25077393
American Academy of Pediatrics Subcommittee on Hyperbilirubinemia
Pediatrics 2004 Jul;114(1):297-316. doi: 10.1542/peds.114.1.297. PMID: 15231951

Curated

UK NICE Clinical Guideline (CG98), Jaundice in newborn babies under 28 days, 2023

Recent clinical studies

Etiology

Horn D, Ehret D, Gautham KS, Soll R
Cochrane Database Syst Rev 2021 Jul 6;7(7):CD013277. doi: 10.1002/14651858.CD013277.pub2. PMID: 34228352Free PMC Article
Olusanya BO, Kaplan M, Hansen TWR
Lancet Child Adolesc Health 2018 Aug;2(8):610-620. Epub 2018 Jun 28 doi: 10.1016/S2352-4642(18)30139-1. PMID: 30119720
Muchowski KE
Am Fam Physician 2014 Jun 1;89(11):873-8. PMID: 25077393
Lauer BJ, Spector ND
Pediatr Rev 2011 Aug;32(8):341-9. doi: 10.1542/pir.32-8-341. PMID: 21807875
American Academy of Pediatrics Subcommittee on Hyperbilirubinemia
Pediatrics 2004 Jul;114(1):297-316. doi: 10.1542/peds.114.1.297. PMID: 15231951

Diagnosis

Qian S, Kumar P, Testai FD
Curr Neurol Neurosci Rep 2022 Jul;22(7):343-353. Epub 2022 May 19 doi: 10.1007/s11910-022-01204-8. PMID: 35588044
Iskander I, Gamaleldin R
Semin Perinatol 2021 Feb;45(1):151353. Epub 2020 Dec 1 doi: 10.1016/j.semperi.2020.151353. PMID: 33323291
Olusanya BO, Kaplan M, Hansen TWR
Lancet Child Adolesc Health 2018 Aug;2(8):610-620. Epub 2018 Jun 28 doi: 10.1016/S2352-4642(18)30139-1. PMID: 30119720
Muchowski KE
Am Fam Physician 2014 Jun 1;89(11):873-8. PMID: 25077393
Lauer BJ, Spector ND
Pediatr Rev 2011 Aug;32(8):341-9. doi: 10.1542/pir.32-8-341. PMID: 21807875

Therapy

Qian S, Kumar P, Testai FD
Curr Neurol Neurosci Rep 2022 Jul;22(7):343-353. Epub 2022 May 19 doi: 10.1007/s11910-022-01204-8. PMID: 35588044
Horn D, Ehret D, Gautham KS, Soll R
Cochrane Database Syst Rev 2021 Jul 6;7(7):CD013277. doi: 10.1002/14651858.CD013277.pub2. PMID: 34228352Free PMC Article
Diala UM, Wennberg RP, Abdulkadir I, Farouk ZL, Zabetta CDC, Omoyibo E, Emokpae A, Aravkin A, Toma B, Oguche S, Slusher T; *On behalf of the Stop Kernicterus In Nigeria (SKIN) study group
J Perinatol 2018 Jul;38(7):873-880. Epub 2018 Mar 28 doi: 10.1038/s41372-018-0094-y. PMID: 29593357
Wennberg RP, Watchko JF, Shapiro SM
Curr Pediatr Rev 2017;13(3):210-219. doi: 10.2174/1573396313666170828112038. PMID: 28847288
Muchowski KE
Am Fam Physician 2014 Jun 1;89(11):873-8. PMID: 25077393

Prognosis

Wennberg RP, Imam ZO, Shwe DD, Hassan L, Farouk ZL, Turner LE, Brearley AM, Slusher TM, Oguche S
Pediatr Res 2024 Apr;95(5):1301-1307. Epub 2023 Dec 2 doi: 10.1038/s41390-023-02887-6. PMID: 38042946Free PMC Article
Horn D, Ehret D, Gautham KS, Soll R
Cochrane Database Syst Rev 2021 Jul 6;7(7):CD013277. doi: 10.1002/14651858.CD013277.pub2. PMID: 34228352Free PMC Article
Okolie F, South-Paul JE, Watchko JF
JAMA Pediatr 2020 Dec 1;174(12):1199-1205. doi: 10.1001/jamapediatrics.2020.1767. PMID: 32628268
Kang W, Yuan X, Zhang Y, Song J, Xu F, Liu D, Li R, Xu B, Li W, Cheng Y, Zhu C
Ann Clin Transl Neurol 2020 Jul;7(7):1141-1147. Epub 2020 Jun 4 doi: 10.1002/acn3.51077. PMID: 32495505Free PMC Article
Wennberg RP, Oguche S, Imam Z, Farouk ZL, Abdulkadir I, Sampson PD, Slusher TM, Bode-Thomas F, Toma BO, Yilgwan CS, Shwe D, Ofakunrin AO, Diala UM, Isichei C, Pam V, Hassan Z, Abdullahi SU, Usman F, Jibir BW, Mohammed IY, Usman HA, Abdusalam M, Kuliya-Gwarzo A, Tsiga-Ahmad FI, Umar L, Ogala WN, Abdullahi F, Hassan L, Purdue S, Lund T, Coda-Zabetta CD
J Pediatr 2020 Jun;221:47-54.e4. Epub 2020 Mar 4 doi: 10.1016/j.jpeds.2020.01.050. PMID: 32145967

Clinical prediction guides

Qian S, Kumar P, Testai FD
Curr Neurol Neurosci Rep 2022 Jul;22(7):343-353. Epub 2022 May 19 doi: 10.1007/s11910-022-01204-8. PMID: 35588044
Iskander I, Gamaleldin R
Semin Perinatol 2021 Feb;45(1):151353. Epub 2020 Dec 1 doi: 10.1016/j.semperi.2020.151353. PMID: 33323291
Kang W, Yuan X, Zhang Y, Song J, Xu F, Liu D, Li R, Xu B, Li W, Cheng Y, Zhu C
Ann Clin Transl Neurol 2020 Jul;7(7):1141-1147. Epub 2020 Jun 4 doi: 10.1002/acn3.51077. PMID: 32495505Free PMC Article
Zhang F, Chen L, Shang S, Jiang K
Medicine (Baltimore) 2020 Feb;99(9):e19364. doi: 10.1097/MD.0000000000019364. PMID: 32118780Free PMC Article
Muchowski KE
Am Fam Physician 2014 Jun 1;89(11):873-8. PMID: 25077393

Recent systematic reviews

Belay HG, Debebe GA, Ayele AD, Kassa BG, Mihretie GN, Worke MD
World J Pediatr 2022 Nov;18(11):725-733. Epub 2022 Sep 16 doi: 10.1007/s12519-022-00597-3. PMID: 36114364
Horn D, Ehret D, Gautham KS, Soll R
Cochrane Database Syst Rev 2021 Jul 6;7(7):CD013277. doi: 10.1002/14651858.CD013277.pub2. PMID: 34228352Free PMC Article
Armanian AM, Jahanfar S, Feizi A, Salehimehr N, Molaeinezhad M, Sadeghi E
Cochrane Database Syst Rev 2019 Aug 13;8(8):CD012731. doi: 10.1002/14651858.CD012731.pub2. PMID: 31425619Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2023
      UK NICE Clinical Guideline (CG98), Jaundice in newborn babies under 28 days, 2023

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