347 - A Modified Kernicterus Spectrum Disorder Toolkit Is Useful In Diagnosing Kernicterus Spectrum Disorders In Resource-Limited Settings

Poster Number: 347
Publication Number: 347.123

Rose N. Gelineau-Morel, Children's Mercy Hospitals and Clinics, Kansas City, MO, United States; Fatima Usman, Bayero University Kano, Oxford, England, United Kingdom; Saadatu Shehu, Aminu kano teaching hospital, Kano, Kano, Nigeria; Mohammed Abdulsalam, Bayero University Kano/Aminu Kano Teaching Hospital, Kano., Kano, Kano, Nigeria; Steven Shapiro, University of Kansas School of Medicine, Kansas City, MO, United States; Tina M. Slusher, University of Minnesota, Roseville, MN, United States; Jean-Baptiste Le Pichon, Children's Mercy Hospitals and Clinics, Kansas City, MO, United States; Zubaida L. Farouk, Aminu Kano Teaching Hospital/Bayero University Kano, Kano, Kano, Nigeria

Background: Neonatal hyperbilirubinemia (NH) and associated acute bilirubin encephalopathy may result in Kernicterus Spectrum Disorders (KSDs), leading to severe motor and/or auditory dysfunction.  In the neonatal period, the Bilirubin-Induced Neurologic Dysfunction (BIND) score has good specificity and sensitivity for predicting KSDs.  Recently, we proposed a KSD diagnostic toolkit (KSD-TK) for older children, using clinical information and results from Magnetic Resonance Imaging (MRI) and Auditory Brainstem Response (ABR) testing, which demonstrated excellent specificity and sensitivity.  However, KSDs are more common in resource-limited settings, where diagnostic tools such as MRI and ABR are limited.

Objective: In this study, we evaluated infants with NH using a modified KSD-TK (KSD-TKm) without MRI or ABR, and compared the sensitivity and specificity of the KSD-TKm for diagnosing KSDs with the BIND score for predicting KSDs. 

Design/Methods: Twenty-five Nigerian term infants with NH (maximum total bilirubin >15, range 17.3-39.7 mg/dl) were enrolled and their BIND score calculated.  Information from hospitalization and follow-ups at 3, 6, 9, and 12 months was used to complete the KSD-TKm including: highest total bilirubin level, newborn risk factors, newborn neonatal exam, most recent follow-up exam, and the presence of enamel dysplasia.  Five patients died prior to the follow-up visit and were excluded.  The remainder were clinically diagnosed with the presence or absence of KSD by experienced clinicians during follow-up visits.  A separate examiner blinded to clinical diagnosis completed the KSD-TKm for each patient.  The total score cut-off for KSD diagnosis was adjusted from the original KSD-TK to account for the absence of MRI and ABR, with a score of < 5 indicating no KSD and a score of ≥5 indicating KSD (score range 0-10).  The sensitivity and specificity of the KSD-TKm for diagnosing KSDs and the BIND score for predicting KSDs was calculated. 

Results: A KSD-TKm score of ≥5 demonstrated equal sensitivity to a BIND score of ≥6 in diagnosing/predicting KSDs (86%) and a higher sensitivity (100% with KSD-TKm and 92% with BIND score).Conclusion(s): The KSD-TKm is a useful tool for diagnosing KSDs in older patients, with comparable sensitivity and specificity to the BIND score, and can be used in resource-limited settings without the need for MRI or ABR testing.