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Original Research from Goryeb Children’s Hospital Pediatric Gastroenterology Team Presented at Prestigious North American Society for Pediatric Gastroenterology, Hepatology and Nutrition Meeting

October 21, 2019

Atlantic Health System Case Study Named “Poster of Distinction”

Morristown, NJ – Original research from the pediatric gastroenterology team at Goryeb Children's Hospital/Atlantic Health Children’s Health was presented at the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, a premiere medical meeting for the gastroenterology community.

In addition, a poster from Atlantic Health System’s pediatric nurse practitioner Stephanie Schuckalo was recognized as a “Poster of Distinction” for her case study “High Index of Suspicion for Bile Acid Synthesis Defect in an Atypical Presentation of Liver Disease.”

Goryeb team presents research at NASPGHAN

Benefits of Home Infusion Therapy for Biologic Therapies in Children

In her poster “Patient and Caregiver Satisfaction with Home Infusion Therapy in Pediatric Inflammatory Bowel Disease,” Nancy Salmeri, BSN, RN, Nurse Navigator for the Goryeb Pediatric IBD Center, used a mailed questionnaire to gauge patient and family satisfaction of in-home infusion services (as opposed to hospital-based infusion center appointments) to deliver biologic treatment for patients with Pediatric Inflammatory Bowel Disease (IBD).

Of 62 responses received, 98 percent of participants would recommend home infusion services to another family, and 68 percent reported an overall satisfaction level of 10 (most satisfied).

“Biologics, delivered by infusion therapy, are the mainstay of IBD treatment for pediatric and adult patients, but the frequency and time required to infuse the medication has an impact on children’s schooling and caregiver work schedules,” Salmeri said. “When we offer families options for their child to receive treatment in the comfort of their home, it allows them to meet their school and work requirements and maintain their regular schedule, which is critical for long-term treatment success.”

Gabriele Hunter, DO, Pediatric Resident Annette Langseder, BSN, Pediatric Research Coordinator, Mary C. Kennedy, MSN, APN-C, Pediatric Research Coordinator, Maria Perez, DO, and Joel R. Rosh, MD, Director, Pediatric Gastroenterology and Vice Chairman, Clinical Development and Research Affairs for Goryeb Children's Hospital and Atlantic Health System, co-authored the poster.

Goryeb team presents research at NASPGHAN

Non-Invasive Alternative to Liver Biopsy

Stephanie Schuckalo, Pediatric Nurse Practitioner, used FibroScan®, an ultrasound-based, non-invasive imaging modality, to help identify predictors of severe liver stiffness in 87 pediatric patients with suspected non-alcoholic fatty liver disease (NAFLD), the most common cause of chronic liver disease in children and prevalent in 34 percent of children with obesity, in her poster “Predictors of Severe Liver Stiffness and Significant Steatosis in Patients with NAFLD.”

Before FibroScan, also known as transient elastography, pediatric and adult patients with NAFLD had to undergo painful and invasive biopsies where a thin needle was inserted into the liver to obtain a tissue sample. FibroScan uses a slight vibration to generate an elastic shear wave within the liver and help clinicians measure fibrosis (scarring) and steatosis (fatty change).

Goryeb Children’s Hospital is the only pediatric center in New Jersey and one of only a handful of children’s hospitals in the U.S to offer FibroScan.

In addition to NAFLD, children with other diseases and conditions that impact the liver benefit from this technology: rheumatological illnesses (such as lupus), inflammatory bowel disease, neonates on parenteral (IV) nutrition, congenital heart disease, sickle cell disease or oncological illness, cystic fibrosis, and metabolic or genetic disorders.

The FibroScan was fully funded by generous donors to the Foundation for Morristown Medical Center.

“Being a parent to a child with a chronic illness, or being a child with a chronic illness is incredibly difficult and often requires many rounds of invasive and potentially painful testing,” Joel R. Rosh, MD, Director, Pediatric Gastroenterology and Vice Chairman, Clinical Development and Research Affairs for Goryeb Children's Hospital and Atlantic Health System. “We are incredibly grateful to the donors who helped fund our FibroScan, which enables hundreds of children each year to avoid a needle biopsy and helps making managing their disease a bit easier.”

Schuckalo’s study found that nearly 52 percent of patients (45/87) who received FibroScan imaging had significant liver stiffness and 20.7 percent (18/87) had severe liver stiffness; 71.3 percent (62/87) had significant steatosis.

“Using new, non-invasive technology to identify children with significant liver stiffness and steatosis earlier in their disease helps clinical teams determine which patients are most vulnerable to substantial liver injury and begin more aggressive treatment,” Schuckalo said. “Because we can substitute a non-invasive test for a liver biopsy, children may be more open to regular testing to track changes in the liver.”

Nadia Ovchinsky, MD, MBA, Director, Pediatric Hepatology, and Stephanie Chiu, Manager, Grants & Research, Atlantic Center for Research, co-authored the study.

Need for Education around 3β-HSD Deficiency

In “High Index of Suspicion for Bile Acid Synthesis Defect in an Atypical Presentation of Liver Disease,” Schuckalo presents a case study of an 11-year-old girl referred for treatment of Hepatitis C (HCV). The patient had tetany (spasms) caused by high calcium and seizures in utero, was diagnosed with medullary nephrocalcinosis (deposits of calcium salts in the medulla of the kidney) and growth failure with delayed bone growth the age of 2 years. In addition to her previous HCV diagnosis, a liver biopsy demonstrated chronic moderately active hepatitis, large and small fat deposits on the liver, and liver fibrosis with benign lesions.

After a 12-week course of treatment, the hepatitis C virus was not detected in the blood, but the patient continued to have elevated liver enzymes and significant fat-soluble vitamin deficiencies, despite supplementation. Additional testing, including whole-exome sequencing (genetic testing) revealed her HSD3B7 gene had two identical alleles, which is abnormal and caused by her parents being first cousins. A mass spectroscopy test of her urine metabolites revealed the typical pattern seen in patients with 3β-HSD with her genetic mutation.

The patient was started on treatment with cholic acid (CA). After one year on therapy, she gained weight and grew, her liver parameters normalized, calcium salt deposits in the kidney resolved, and she had significant improvement in her fat-soluble vitamin levels.

“There is a strong need for education around 3β-HSD deficiency for clinicians, and need for additional molecular testing when patients don’t present with ‘normal’ liver function and fat-soluble vitamin deficiency,” Schuckalo said. “In this case, genetic testing of isolated molecules helped us to understand what was truly happening to the patient and allowed us to initiate proper treatment as soon as possible to prevent further liver disease.”

About the Foundation for Morristown Medical Center

Established in 1995, the Foundation for Morristown Medical Center’s mission is to inspire community philanthropy to advance exceptional health care for patients at Morristown Medical Center and Goryeb Children’s Hospital. Over the past 10 years, donors have contributed more than $175 million to support both Morristown Medical Center and Goryeb Children’s Hospital.

About Atlantic Health System

Atlantic Health System is at the forefront of medicine, setting standards for quality health care in New Jersey, Pennsylvania and the New York metropolitan area. Powered by a workforce of 17,000 team members and 4,800 affiliated physicians dedicated to building healthier communities, Atlantic Health System serves more than half of the state of New Jersey including 11 counties and 4.9 million people. The system offers 400 sites of care, including six hospitals: Morristown Medical Center in Morristown, NJ, Overlook Medical Center in Summit, NJ, Newton Medical Center in Newton NJ, Chilton Medical Center in Pompton Plains, NJ, Hackettstown Medical Center in Hackettstown, NJ and Goryeb Children’s Hospital in Morristown, NJ.

In addition to the employed workforce, Atlantic Alliance, a Clinically Integrated Network represents more than 2,500 health care providers throughout northern and central NJ. This network includes 1,000 physicians and providers comprising one of the largest multi-specialty practices in New Jersey - Atlantic Medical Group, as well as members of the Atlantic Accountable Care Organization and Optimus Healthcare Partners, which work to enhance patient care delivery.

Atlantic Health System provides care for the full continuum of health needs, including 12 urgent care centersAtlantic Rehabilitation and Atlantic Home Care and Hospice. Facilitating the connection between these services on both land and air is the transportation fleet of Atlantic Mobile Health.

Atlantic Health System leads the Healthcare Transformation Consortium, a partnership of seven regional hospitals and health systems dedicated to improving access and affordability, and is a founding member of the PIER Consortium – Partners in Innovation, Education, and Research – a streamlined clinical trial system expanding access to groundbreaking research across seven health systems in New Jersey.

Atlantic Health System has a medical school affiliation with Thomas Jefferson University and is home to the regional campus of the Sidney Kimmel Medical College at Morristown and Overlook Medical Centers and is the official health care partner of the New York Jets.