Hackensack Meridian Childrens Health Nephrologists Are Principal Investigators in Clinical Trial
What you need to know
Hackensack Meridian Children’s Health is the only pediatric health care network in New Jersey undertaking a new study that examines the effectiveness of a drug called sparsentan to treat rare kidney diseases in children.
Chief pediatric nephrologists and Hackensack Meridian School of Medicine professors, Ken Lieberman, M.D. and Guillermo Hidalgo, M.D., F.A.S.N, are the study’s principal investigators.
“Study of Sparsentan Treatment in Pediatrics with Proteinuric Glomerular Diseases (EPPIK)” is open to pediatric nephrology patients throughout New Jersey at both Hackensack Meridian Children’s Hospitals - Joseph M. Sanzari Children’s Hospital at Hackensack University Medical Center and K. Hovnanian Children’s Hospital at Jersey Shore University Medical Center.
This EPPIK study is investigating the use of the medication to treat three specific proteinuric diseases — steroid-resistant nephrotic syndrome*, Alport syndrome**, and IgA nephropathy***. These diseases typically develop in children or young adults, cause progressive loss of kidney function, and result in end-stage kidney disease.
Proteinuric kidney diseases are rare and result in proteinuria. Until now the three conditions being studied have been treated with other classes of medications, including immunosuppressive drugs. At times for extended periods of time.
*About Steroid-Resistant Nephrotic Syndrome (SRNS)
- Can be caused by genetic abnormalities or immune system dysfunction
- Rare and seen predominantly in males.
- Holds a significant risk for speedy progression from chronic kidney disease to end-stage renal disease.
- Leading cause of end-stage kidney disease
- Nephrotic syndrome (NS) is a clinical syndrome characterized by massive proteinuria, hypoalbuminemia, peripheral edema, and hyperlipidemia
**About Alport Syndrome
- Stems from the mutations in COL4A3, COL4A4, COL4A5, which play a major role in structures called glomeruli.
- Glomeruli are clusters of special blood vessels that remove waste products and water from the blood and create urine.
- Syndrome attacks the glomeruli by damaging the tiny blood vessels in kidneys.
- Leads to loss of this organ’s functions which in turn leads to eye problems and hearing loss.
***About IgA nephropathy also known as Berger’s Disease (Immunoglobin A)
- Caused by buildup of an antibody called immunoglobin A
- Inflammation damages the kidney which later on affects the processes that filter waste from the body’s blood.
- Disease is more common in men than it is in women
About the Pediatric Nephrology Program
As a network, Hackensack Meridian Children’s Health is a leader in research breakthroughs and exceptional outcomes for children with kidney disease and hypertension. The pediatric nephrology program at Hackensack Meridian Joseph M. Sanzari Children’s Hospital at Hackensack University Medical Center and K. Hovnanian Children’s Hospital at Jersey Shore University Medical Center, plays a critical role in understanding and improving outcomes for children with renal disease. Both children’s hospitals serve as clinical coordinating centers for the world’s largest registry for crescentic glomerulonephritis, and an expanded acute kidney injury program at K. Hovnanian includes new interdisciplinary care that provides vital dialysis services.
The comprehensive program also offers genetic testing and counseling. In 2021, Sanzari, which is home to the network’s pediatric transplant program, performed a record number of kidney transplants. Namrata G. Jain, M.D., previously the medical director of the Pediatric Transplant at New York-Presbyterian Hospital/Columbia, in 2021 became the new medical director of pediatric kidney transplant.