Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil-Reference-Cited by-同舟云学术

Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil

Published:2020-08-19 Issue:6 Volume:23 Page:467-471
ISSN:1093-5266
Container-title:Pediatric and Developmental Pathology
language:en
Short-container-title:Pediatr Dev Pathol

Author:

Slowik Voytek¹^ORCID,Hildreth Amber²,Pacheco M Cristina³,Finn Laura S³,King Jeremy⁴,Shivaram Giridhar⁵,Files Matthew⁶,Hsu Evelyn K⁷,Horslen Simon⁷

Affiliation:

1. Division of Gastroenterology Hepatology and Nutrition, Children’s Mercy Kansas City, Kansas City, Missouri

2. Division of Gastroenterology Hepatology and Nutrition, Presbyterian/St. Luke’s Transplant Center, Rocky Mountain Hospital for Children, Denver, Colorado

3. Department of Laboratories, Seattle Children’s Hospital, Seattle, Washington

4. Department of Pediatrics, Kapi’olani Medical Center for Women and Children, Honolulu, Hawaii

5. Department of Radiology, Seattle Children’s Hospital, Seattle, Washington

6. Division of Pediatric Cardiology, Seattle Children’s Hospital, Seattle, Washington

7. Division of Gastroenterology Hepatology and Nutrition, Seattle Children’s Hospital, Seattle, Washington

Abstract

We report a patient without known preexisting liver disease who presented with hepatopulmonary syndrome (HPS) due to aberrant intrahepatic portal venous development leading to portosystemic shunting. Liver transplantation resulted in resolution of portal hypertension and HPS and sildenafil was safely tolerated in the treatment of persistent fatigue and hypoxemia. Twelve months later, patient has normal allograft function and has returned to normal activity.

Publisher

SAGE Publications

Subject

General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology and Child Health

Link

http://journals.sagepub.com/doi/pdf/10.1177/1093526620945951

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