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Clinically Evident Portal Hypertension

An Operational Research Definition for Future Investigations in the Pediatric Population

Bass, Lee M.*; Shneider, Benjamin L.; Henn, Lisa; Goodrich, Nathan P.; Magee, John C.§ on behalf of the Childhood Liver Disease Research Network (ChiLDReN)

Journal of Pediatric Gastroenterology and Nutrition: June 2019 - Volume 68 - Issue 6 - p 763–767
doi: 10.1097/MPG.0000000000002333
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ABSTRACT Portal hypertension (PHT) is a major cause of morbidity and mortality in pediatric liver diseases. Thus, research into causes and disease modifiers in PHT in these conditions is vitally important. PHT is rarely directly or indirectly measured in the assessment of children with chronic liver disease. A straightforward, reproducible definition of PHT could be invaluable for consistently identifying patients with PHT and for grouping these patients according to their risk of complications from their disease. We propose the term Clinically Evident Portal Hypertension (CEPH) to denote clinical findings that demonstrate evidence of elevated portal pressure. When CEPH criteria are met, PHT is highly likely to be present, although it is likely that PHT exists for variable periods of time before meeting CEPH criteria. Use of this research definition of CEPH will allow for consistent identification of these patients by clinicians in nearly any clinical setting and serve as a clinical milepost that may dictate future prognosis in pediatric patients with cirrhosis.

*Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL

Department of Pediatrics-Gastroenterology, Baylor College of Medicine, Houston, TX

Arbor Research Collaborative for Health

§Department of Surgery, University of Michigan, Ann Arbor, MI.

Address correspondence and reprint requests to Lee M. Bass, MD, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Ave, Box 65, Chicago, IL 60611 (e-mail: lbass@luriechildrens.org).

Received 28 September, 2018

Accepted 4 February, 2019

This work was supported by U01 grants from the National Institute of Diabetes, Digestive and Kidney Diseases (DK 62445 [Mount Sinai School of Medicine], DK 62497 [Cincinnati Children's Hospital Medical Center], DK 62470 [Children's Healthcare of Atlanta], DK 62481 [The Children's Hospital of Philadelphia], DK 62456 [The University of Michigan], DK 84536 [Riley Hospital for Children], DK 84575 [Seattle Children's Hospital], DK 62500 [UCSF Children's Hospital], DK 62503 [Johns Hopkins School of Medicine], DK 62466 [Children's Hospital of Pittsburgh of UPMC], DK 62453 [Children's Hospital Colorado], DK 62452 [Washington University School of Medicine], DK 84538 [Children's Hospital Los Angeles], DK 62436 [Ann & Robert H Lurie Children's Hospital of Chicago], DK103149 [Texas Children's Hospital], DK103135 [The Hospital for Sick Children], DK103140 [University of Utah]).

L.M.B. is on the speaker's bureau for Mead Johnson Nutrition. The remaining authors report no conflicts of interest.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,