Clinical characteristics of neonatal cholestasis in a tertiary hospital and the development of a novel prediction model for mortality
Ho Jung Choi,
Inki Kim,
Hye-Jin Lee,
Hyun Ju Oh,
Mi Kyoung Ahn,
Woo Im Baek,
Yeong Eun Kim,
Seak Hee Oh,
Byong Sop Lee,
Jung-Man Namgoong,
Dae Yeon Kim,
Eun Joo Lee,
Jung Ok Shim,
Jae Sung Ko,
Kyung Mo Kim
Affiliations
Ho Jung Choi
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Inki Kim
Department of Convergence Medicine, Asan Medical Center, Asan Institutes for Life Sciences, University of Ulsan College of Medicine, Seoul, South Korea
Hye-Jin Lee
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Hyun Ju Oh
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Mi Kyoung Ahn
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Woo Im Baek
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Yeong Eun Kim
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Seak Hee Oh
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea; Corresponding author.
Byong Sop Lee
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Jung-Man Namgoong
Division of Pediatric Surgery, Department of Surgery, Asan Medical Center, University Ulsan College of Medicine, Seoul, South Korea
Dae Yeon Kim
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, South Korea
Eun Joo Lee
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, South Korea
Jung Ok Shim
Department of Pediatrics, Korea University College of Medicine, Seoul, South Korea
Jae Sung Ko
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, South Korea
Kyung Mo Kim
Department of Pediatrics, Asan Medical Center Children's Hospital, University Ulsan College of Medicine, 88, Olympic-ro 43-gil Songpa-Gu, Seoul 05505, South Korea
Summary: Background: Few studies have described the aetiologies of neonatal cholestasis, and the overall neonatal cholestasis-related mortality (NCM) rate is unclear. We investigated the aetiology and outcome of neonatal cholestasis in a tertiary hospital and developed an NCM prediction model for these patients. Methods: Patients aged 1.0 mg/dL were retrospectively screened. Diagnostic and laboratory data during the 8-week follow-up period after enrolment between 2005 and 2020 were extracted digitally, and medical charts were reviewed manually by clinicians. Logistic regression was used to derive a prediction model for the 1-year mortality outcome of neonatal cholestasis, and performance evaluation and external validation were conducted for the NCM prediction model. Findings: We enrolled 4028 neonates with DB of >1.0 mg/dL at least once. Prematurity and birth injury (35.4%), complex heart anomalies (18.6%), liver diseases (11.4%), and gastrointestinal anomalies (9.2%) were the most common aetiologies; 398 (9.9%) patients died before one year of age. The peak value of DB was positively correlated to the 1-year mortality rate. In the multivariate analysis, simple laboratory indices, including platelet, prothrombin time, aspartate aminotransferase, albumin, direct bilirubin, creatinine, and C-reactive protein, were independent predictors of 1-year mortality outcome of complete-case subjects. Using these laboratory indices, a logistic regression-based NCM prediction model was constructed. It showed acceptable performances on discrimination (area under the curve, 0.916), calibration (slope, 1.04) and Brier scoring (0.072). The external validation of the sample (n = 920) from two other centres also revealed similar performance profiles of the NCM model. Interpretation: Various aetiologies of neonatal cholestasis were identified in a tertiary hospital, resulting in unfavourable outcomes of a large proportion. The NCM prediction model may have the potential to help clinicians to be aware of high-risk neonatal cholestasis.
