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The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs).
Curr Pediatr Rev. 2017; 13(3):199-209.CP

Abstract

BACKGROUND

Despite its lengthy history, the study of jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. For example, the term Bilirubin-induced Neurological Dysfunction (BIND) has been used to refer to all neurological sequelae caused by exposure to high levels of bilirubin, to only mild neurological sequelae, or to scoring systems that quantitate the progressive stages of Acute Bilirubin Encephalopathy (ABE).

OBJECTIVE

We seek to clarify and simplify terminology by introducing, defining, and proposing new terms and diagnostic criteria for kernicterus.

METHODS

We propose a systematic nomenclature based on pathophysiological and clinical criteria, presenting a logical argument for each term. Acknowledging observations that kernicterus is symptomatically broad and diverse, we propose the use of the overarching term Kernicterus Spectrum Disorders (KSDs) to encompass all the neurological sequelae of bilirubin neurotoxicity including Acute Bilirubin Neurotoxicity (ABE). We further suggest subclassification of KSDs based on the principal disabling features of kernicterus (motor, auditory). Finally, we suggest the term subtle KSD to designate a child with a history of significant bilirubin neurotoxicity with mild or subtle developmental delays.

RESULTS AND CONCLUSION

We conclude with a brief description of the limited treatments currently available for KSD, thereby underscoring the importance of further research. We believe that adopting a systematic nomenclature for the spectrum of clinical consequences of hyperbilirubinemia will help unify the field and promote more effective research in both prevention and treatment of KSDs.

Authors+Show Affiliations

Division of Child Neurology, Department of Pediatrics, Children's Mercy Hospital, Kansas City, MO. United States.Department of Neurology, University of Kansas Medical Center, Kansas City, KS. United States.Division of Newborn Medicine, Department of Pediatrics, University of Pittsburgh School of MedicineDivision of Newborn Medicine, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA. United States.Department of Neurology, Faculty of Children's Mercy Hospital, 2401 Gillham Road, Kanas City, MO 64108. United States.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

28814249

Citation

Le Pichon, Jean-Baptiste, et al. "The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs)." Current Pediatric Reviews, vol. 13, no. 3, 2017, pp. 199-209.
Le Pichon JB, Riordan SM, Watchko J, et al. The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs). Curr Pediatr Rev. 2017;13(3):199-209.
Le Pichon, J. B., Riordan, S. M., Watchko, J., & Shapiro, S. M. (2017). The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs). Current Pediatric Reviews, 13(3), 199-209. https://doi.org/10.2174/1573396313666170815100214
Le Pichon JB, et al. The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs). Curr Pediatr Rev. 2017;13(3):199-209. PubMed PMID: 28814249.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The Neurological Sequelae of Neonatal Hyperbilirubinemia: Definitions, Diagnosis and Treatment of the Kernicterus Spectrum Disorders (KSDs). AU - Le Pichon,Jean-Baptiste, AU - Riordan,Sean M, AU - Watchko,Jon, AU - Shapiro,Steven M, PY - 2017/06/29/received PY - 2017/07/25/revised PY - 2017/07/27/accepted PY - 2017/8/18/pubmed PY - 2018/2/22/medline PY - 2017/8/18/entrez KW - Kernicterus KW - auditory neuropathy KW - bilirubin encephalopathy KW - bilirubin neurotoxicity KW - dystonia KW - hyperbilirubinemia KW - newborn jaundice SP - 199 EP - 209 JF - Current pediatric reviews JO - Curr Pediatr Rev VL - 13 IS - 3 N2 - BACKGROUND: Despite its lengthy history, the study of jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. For example, the term Bilirubin-induced Neurological Dysfunction (BIND) has been used to refer to all neurological sequelae caused by exposure to high levels of bilirubin, to only mild neurological sequelae, or to scoring systems that quantitate the progressive stages of Acute Bilirubin Encephalopathy (ABE). OBJECTIVE: We seek to clarify and simplify terminology by introducing, defining, and proposing new terms and diagnostic criteria for kernicterus. METHODS: We propose a systematic nomenclature based on pathophysiological and clinical criteria, presenting a logical argument for each term. Acknowledging observations that kernicterus is symptomatically broad and diverse, we propose the use of the overarching term Kernicterus Spectrum Disorders (KSDs) to encompass all the neurological sequelae of bilirubin neurotoxicity including Acute Bilirubin Neurotoxicity (ABE). We further suggest subclassification of KSDs based on the principal disabling features of kernicterus (motor, auditory). Finally, we suggest the term subtle KSD to designate a child with a history of significant bilirubin neurotoxicity with mild or subtle developmental delays. RESULTS AND CONCLUSION: We conclude with a brief description of the limited treatments currently available for KSD, thereby underscoring the importance of further research. We believe that adopting a systematic nomenclature for the spectrum of clinical consequences of hyperbilirubinemia will help unify the field and promote more effective research in both prevention and treatment of KSDs. SN - 1875-6336 UR - https://wwww.unboundmedicine.com/medline/citation/28814249/The_Neurological_Sequelae_of_Neonatal_Hyperbilirubinemia:_Definitions_Diagnosis_and_Treatment_of_the_Kernicterus_Spectrum_Disorders__KSDs__ L2 - http://www.eurekaselect.com/154919/article DB - PRIME DP - Unbound Medicine ER -