A Learning Collaborative Approach Increases Specificity of Diagnosis of Acute Liver Failure in Pediatric Patients

Pediatric Acute Liver Failure Study Group

Research output: Contribution to journalArticle

Abstract

Background & Aims: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)—they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation. We investigated whether performing recommended age-specific diagnostic tests (AS-DTs) at the time of hospital admission reduces the percentage PALFs with an indeterminate diagnosis. Methods: We performed a multinational observational cohort study of 658 PALF participants in the United States and Canada, enrolled at 10 medical centers, during 3 study phases from December 1999 through December 2014. A learning collaborative approach was used to implement AS-DT using an electronic medical record admission order set at hospital admission in phase 3 of the study. Data from 10 study sites participating in all 3 phases were compared before (phases 1 and 2) and after (phase 3) diagnostic test recommendations were inserted into electronic medical record order sets. Results: The percentage of subjects with an indeterminate diagnosis decreased significantly between phases 1–2 (48.0%) and phase 3 (to 30.8%) (P =.0003). The 21-day cumulative incidence rates for liver transplantation were significantly different among phase 1 (34.6%), phase 2 (31.9%), and phase 3 (20.2%) (P =.030). The 21-day cumulative incidence rates for death did not differ significantly among phase 1 (17.9%), phase 2 (11.9%), and phase 3 (11.3%) (P =.20). Conclusions: In a multinational study of children with acute liver failure, we found that incorporating diagnostic test recommendations into electronic medical record order sets accessed at time of admission reduced the percentage with an indeterminate diagnosis that may have reduced liver transplants without increasing mortality. Widespread use of this approach could significantly enhance care of acute liver failure in children.

Original languageEnglish (US)
Pages (from-to)1801-1810.e3
JournalClinical Gastroenterology and Hepatology
Volume16
Issue number11
DOIs
StatePublished - Nov 1 2018

Fingerprint

Acute Liver Failure
Routine Diagnostic Tests
Learning
Pediatrics
Electronic Health Records
Liver Transplantation
Hepatolenticular Degeneration
Mortality
Incidence
Virus Diseases
Simplexvirus
Canada
Observational Studies
Liver Diseases
Cohort Studies
Fatty Acids
Transplants
Liver

Keywords

  • Early Detection
  • Genetic Disorder
  • Hepatic
  • Management

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

Cite this

A Learning Collaborative Approach Increases Specificity of Diagnosis of Acute Liver Failure in Pediatric Patients. / Pediatric Acute Liver Failure Study Group.

In: Clinical Gastroenterology and Hepatology, Vol. 16, No. 11, 01.11.2018, p. 1801-1810.e3.

Research output: Contribution to journalArticle

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abstract = "Background & Aims: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)—they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation. We investigated whether performing recommended age-specific diagnostic tests (AS-DTs) at the time of hospital admission reduces the percentage PALFs with an indeterminate diagnosis. Methods: We performed a multinational observational cohort study of 658 PALF participants in the United States and Canada, enrolled at 10 medical centers, during 3 study phases from December 1999 through December 2014. A learning collaborative approach was used to implement AS-DT using an electronic medical record admission order set at hospital admission in phase 3 of the study. Data from 10 study sites participating in all 3 phases were compared before (phases 1 and 2) and after (phase 3) diagnostic test recommendations were inserted into electronic medical record order sets. Results: The percentage of subjects with an indeterminate diagnosis decreased significantly between phases 1–2 (48.0{\%}) and phase 3 (to 30.8{\%}) (P =.0003). The 21-day cumulative incidence rates for liver transplantation were significantly different among phase 1 (34.6{\%}), phase 2 (31.9{\%}), and phase 3 (20.2{\%}) (P =.030). The 21-day cumulative incidence rates for death did not differ significantly among phase 1 (17.9{\%}), phase 2 (11.9{\%}), and phase 3 (11.3{\%}) (P =.20). Conclusions: In a multinational study of children with acute liver failure, we found that incorporating diagnostic test recommendations into electronic medical record order sets accessed at time of admission reduced the percentage with an indeterminate diagnosis that may have reduced liver transplants without increasing mortality. Widespread use of this approach could significantly enhance care of acute liver failure in children.",
keywords = "Early Detection, Genetic Disorder, Hepatic, Management",
author = "{Pediatric Acute Liver Failure Study Group} and Narkewicz, {Michael R.} and Simon Horslen and Hardison, {Regina M.} and Shneider, {Benjamin L.} and Norberto Rodriguez-Baez and Alonso, {Estella M.} and Ng, {Vicky L.} and Leonis, {Mike A.} and Loomes, {Kathleen M.} and Rudnick, {David A.} and Philip Rosenthal and Rene Romero and Subbarao, {Girish C.} and Ruosha Li and Belle, {Steven H.} and Squires, {Robert H.} and Squires, {Robert H.} and Kathryn Bukauskas and Madeline Schulte and Narkewicz, {Michael R.} and Michelle Hite and Loomes, {Kathleen M.} and Rand, {Elizabeth B.} and David Piccoli and Deborah Kawchak and Christa Seidman and Rene Romero and Saul Karpen and {de la Cruz-Tracy}, Liezl and Vicky Ng and Kelsey Hunt and Subbarao, {Girish C.} and Ann Klipsch and Sarah Munson and Alonso, {Estella M.} and Lisa Sorenson and Susan Kelly and Katie Neighbors and Philip Rosenthal and Shannon Fleck and Leonis, {Mike A.} and John Bucuvalas and Tracie Horning and {Rodriguez Baez}, Norberto and Shirley Montanye and Margaret Cowie and Horslen, {Simon P.} and Karen Murray and Kathleen Schwarz and Karnsakul, {Wikrom W}",
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T1 - A Learning Collaborative Approach Increases Specificity of Diagnosis of Acute Liver Failure in Pediatric Patients

AU - Pediatric Acute Liver Failure Study Group

AU - Narkewicz, Michael R.

AU - Horslen, Simon

AU - Hardison, Regina M.

AU - Shneider, Benjamin L.

AU - Rodriguez-Baez, Norberto

AU - Alonso, Estella M.

AU - Ng, Vicky L.

AU - Leonis, Mike A.

AU - Loomes, Kathleen M.

AU - Rudnick, David A.

AU - Rosenthal, Philip

AU - Romero, Rene

AU - Subbarao, Girish C.

AU - Li, Ruosha

AU - Belle, Steven H.

AU - Squires, Robert H.

AU - Squires, Robert H.

AU - Bukauskas, Kathryn

AU - Schulte, Madeline

AU - Narkewicz, Michael R.

AU - Hite, Michelle

AU - Loomes, Kathleen M.

AU - Rand, Elizabeth B.

AU - Piccoli, David

AU - Kawchak, Deborah

AU - Seidman, Christa

AU - Romero, Rene

AU - Karpen, Saul

AU - de la Cruz-Tracy, Liezl

AU - Ng, Vicky

AU - Hunt, Kelsey

AU - Subbarao, Girish C.

AU - Klipsch, Ann

AU - Munson, Sarah

AU - Alonso, Estella M.

AU - Sorenson, Lisa

AU - Kelly, Susan

AU - Neighbors, Katie

AU - Rosenthal, Philip

AU - Fleck, Shannon

AU - Leonis, Mike A.

AU - Bucuvalas, John

AU - Horning, Tracie

AU - Rodriguez Baez, Norberto

AU - Montanye, Shirley

AU - Cowie, Margaret

AU - Horslen, Simon P.

AU - Murray, Karen

AU - Schwarz, Kathleen

AU - Karnsakul, Wikrom W

PY - 2018/11/1

Y1 - 2018/11/1

N2 - Background & Aims: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)—they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation. We investigated whether performing recommended age-specific diagnostic tests (AS-DTs) at the time of hospital admission reduces the percentage PALFs with an indeterminate diagnosis. Methods: We performed a multinational observational cohort study of 658 PALF participants in the United States and Canada, enrolled at 10 medical centers, during 3 study phases from December 1999 through December 2014. A learning collaborative approach was used to implement AS-DT using an electronic medical record admission order set at hospital admission in phase 3 of the study. Data from 10 study sites participating in all 3 phases were compared before (phases 1 and 2) and after (phase 3) diagnostic test recommendations were inserted into electronic medical record order sets. Results: The percentage of subjects with an indeterminate diagnosis decreased significantly between phases 1–2 (48.0%) and phase 3 (to 30.8%) (P =.0003). The 21-day cumulative incidence rates for liver transplantation were significantly different among phase 1 (34.6%), phase 2 (31.9%), and phase 3 (20.2%) (P =.030). The 21-day cumulative incidence rates for death did not differ significantly among phase 1 (17.9%), phase 2 (11.9%), and phase 3 (11.3%) (P =.20). Conclusions: In a multinational study of children with acute liver failure, we found that incorporating diagnostic test recommendations into electronic medical record order sets accessed at time of admission reduced the percentage with an indeterminate diagnosis that may have reduced liver transplants without increasing mortality. Widespread use of this approach could significantly enhance care of acute liver failure in children.

AB - Background & Aims: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)—they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation. We investigated whether performing recommended age-specific diagnostic tests (AS-DTs) at the time of hospital admission reduces the percentage PALFs with an indeterminate diagnosis. Methods: We performed a multinational observational cohort study of 658 PALF participants in the United States and Canada, enrolled at 10 medical centers, during 3 study phases from December 1999 through December 2014. A learning collaborative approach was used to implement AS-DT using an electronic medical record admission order set at hospital admission in phase 3 of the study. Data from 10 study sites participating in all 3 phases were compared before (phases 1 and 2) and after (phase 3) diagnostic test recommendations were inserted into electronic medical record order sets. Results: The percentage of subjects with an indeterminate diagnosis decreased significantly between phases 1–2 (48.0%) and phase 3 (to 30.8%) (P =.0003). The 21-day cumulative incidence rates for liver transplantation were significantly different among phase 1 (34.6%), phase 2 (31.9%), and phase 3 (20.2%) (P =.030). The 21-day cumulative incidence rates for death did not differ significantly among phase 1 (17.9%), phase 2 (11.9%), and phase 3 (11.3%) (P =.20). Conclusions: In a multinational study of children with acute liver failure, we found that incorporating diagnostic test recommendations into electronic medical record order sets accessed at time of admission reduced the percentage with an indeterminate diagnosis that may have reduced liver transplants without increasing mortality. Widespread use of this approach could significantly enhance care of acute liver failure in children.

KW - Early Detection

KW - Genetic Disorder

KW - Hepatic

KW - Management

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