Children's Hospital is part of the UPMC family.
Be safe anytime, anywhere.
To find a pediatrician or pediatric specialist, please call 412-692-7337 or search our directory.
A resource for our network of referring physicians.
For more information about research, please call our main office at 412-692-6438.
Ranked #8 Nationally by U.S. News & World Report.
Mitochondrial β-oxidation of long chain fatty acyl-CoAs is well recognized as a primary metabolic pathway for maintenance of energy homeostasis and body temperature. However, it also recycles carbons from many long chain fatty acids for lipid synthesis. Little is known about the mechanistic role of the latter in the pathogenesis of symptoms in genetic defects of β-oxidation, and its derangement may in part explain the features of disorders such as neurological dysfunction or acute respiratory distress syndrome that respond poorly to treatment with alternative energy sources.
Very long chain acyl-CoA dehydrogenase (VLCAD) is the dominant long chain acyl-CoA dehydrogenase (ACAD) in energy generation in human muscle and the heart. In contrast, this study provides evidence that ACAD9 and long chain acyl-CoA dehydrogenase (LCAD) more likely function in lipid recycling and synthesis in human brain and lung, respectively, supported by their unique substrate utilization and tissue distribution pattern. Furthermore, we have identified a new genetic deficiency of ACAD9 presenting with episodic liver failure and cardiomyopathy during otherwise mild illnesses, along with chronic neurologic dysfunction. This disorder represents the first in a b-oxidation enzyme primarily involved in lipid recycling or synthesis, revealing a new mechanism of pathogenesis in human disease.
National Institutes of Health
Gerard Vockley, MD, PhD
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
UPMC Children’s Hospital of Pittsburgh
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.
With myCHP, you can request appointments, review test results, and more.
For questions about a hospital bill call:
To pay your bill online, please visit UPMC's online bill payment system.
Interested in giving to Children's Hospital? Support the hospital by making a donation online, joining our Heroes in Healing monthly donor program, or visiting our site to learn about the other ways you can give back.