Clinical Course among Cases of Acute Liver Failure of Indeterminate Diagnosis - 24/03/16

Doi : 10.1016/j.jpeds.2015.12.065

Ruosha Li, PhD ¹, Steven H. Belle, PhD, MScHyg ², Simon Horslen, MB, ChB ³, Ling-wan Chen, MS ⁴, Song Zhang, MS ⁵, Robert H. Squires, MD ^6,^⁎
on behalf of the
Pediatric Acute Liver Failure Study Group^{^∗}
List of additional members of the Pediatric Acute Liver Failure Study Group is available at www.jpeds.com (Appendix 1).
Kathryn Bukauskas, RN, CCRC, Michael R. Narkewicz, MD, Michelle Hite, MA, CCRC, Kathleen M. Loomes, MD, Elizabeth B. Rand, MD, David Piccoli, MD, Deborah Kawchak, MS, RD, Rene Romero, MD, Saul Karpen, MD, PhD, Liezl de la Cruz-Tracy, CCRC, Vicky Ng, MD, Kelsey Hunt : Clinical Research Coordinator, Girish C. Subbarao, MD, Ann Klipsch, RN, Estella M. Alonso, MD, Lisa Sorenson, PhD, Susan Kelly, RN, BSN, Dhey Delute, RN, CCRC, Katie Neighbors, MPH, CCRC, Philip J. Rosenthal, MD, Shannon Fleck : Clinical Research Coordinator, Mike A. Leonis, MD, PhD, John Bucuvalas, MD, Tracie Horning : Clinical Research Coordinator, Norberto Rodriguez Baez, MD, Shirley Montanye, RN : Clinical Research Coordinator, Margaret Cowie : Clinical Research Coordinator, Karen Murray, MD, Melissa Young : Clinical Research Coordinator, Heather Vendettuoli : Clinical Research Coordinator, David A. Rudnick, MD, PhD, Ross W. Shepherd, MD, Kathy Harris : Clinical Research Coordinator, Saul J. Karpen, MD, PhD, Alejandro De La Torre : Clinical Research Coordinator, Dominic Dell Olio, MD, Deirdre Kelly, MD, Carla Lloyd : Clinical Research Coordinator, Steven J. Lobritto, MD, Sumerah Bakhsh, MPH : Clinical Research Coordinator, Maureen Jonas, MD, Scott A. Elifoson, MD, Roshan Raza, MBBS, Kathleen B. Schwarz, MD, Wikrom W. Karnsakul, MD, Mary Kay Alford, RN, MSN, CPNP, Anil Dhawan, MD, Emer Fitzpatrick, MD, Nanda N. Kerkar, MD, Brandy Haydel, CCRC, Sreevidya Narayanappa : Clinical Research Coordinator, M. James Lopez, MD, PhD, Victoria Shieck, RN, BSN

¹ Department of Biostatistics, University of Texas School of Public Health, Houston, TX
² Department of Epidemiology, University of Pittsburgh, Pittsburgh, PA
³ Division of Gastroenterology, Seattle Children's Hospital, Seattle, WA
⁴ Department of Statistics, University of Pittsburgh, Pittsburgh, PA
⁵ Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA
⁶ Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, PA

^∗Reprint requests: Robert H. Squires, MD, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Pittsburgh of UPMC, 4401 Penn Ave, Faculty Pavilion Room 6116, Pittsburgh, PA 15224.Division of Pediatric Gastroenterology, Hepatology, and NutritionChildren's Hospital of Pittsburgh of UPMC4401 Penn AveFaculty Pavilion Room 6116PittsburghPA15224

Abstract

Objective

To investigate the heterogeneity in clinical course among those with pediatric acute liver failure (PALF) of indeterminate disease etiology.

Study design

We studied participants enrolled in the PALF registry study with indeterminate final diagnosis. Growth mixture modeling was used to analyze participants' international normalized ratio, total bilirubin, and hepatic encephalopathy trajectories in the first 7 days following enrollment. Participants with at least 3 values for 1 or more of the measurements were included. We examined the association between the resulting latent subgroup classification with participants' characteristics and disease outcomes. Data from participants with PALF of specified etiologies were used to investigate the potential diagnostic value of the latent subgroups.

Results

In this sample of 380 participants with indeterminate final diagnosis, 115 (30%) experienced mild and quickly improving disease trajectories and another 48 (13%) started with severe disease but improved by day 7. The majority of participants (216, 57%) had disease trajectories that worsened over time. The identified patterns of disease trajectories are predictive of outcome (P < .001). The trajectory patterns are associated with the underlying disease etiology (P < .001) for the 488 participants with PALF of specified etiologies.

Conclusions

The clinical courses of participants with PALF of indeterminate disease etiology exhibit distinct trajectory patterns, which have important prognostic and potentially diagnostic value.

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Keyword : APAP, BIC, BLRT, GMM, HE, IND, INR, KCHC, LTx, MAR, non-IND, PALF

Plan

Supported by the National Institutes of Health (2U01DK072146, UL1 RR024153, and UL1TR000005). The authors declare no conflicts of interest.

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Clinical Course among Cases of Acute Liver Failure of Indeterminate Diagnosis - 24/03/16

Abstract

Objective

Study design

Results

Conclusions

Plan

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