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Archives for Pediatrics

Grant Awarded for National Biorepository for GUDMAP Biobank

Sunder Sims-Lucas, PhD, and Jacqueline Ho, MD, are co-investigators on a $600,000 per year grant for 5 years from the National Institutes of Health to be the national biorepository for the GenitoUrinary Development Molecular Anatomy Project (GUDMAP).

GUDMAP is an international consortium working to generate gene expression data and transgenic mice as tools to study genitourinary development. This curated, high-resolution dataset serves as a powerful resource for biologists, clinicians and bioinformaticians interested in the developing urogenital system.

GUDMAP data is accessible at www.gudmap.org.

Hospitalized Patients at Risk if Sodium Levels are Low

Michael Moritz, MD, explains how hospitalized patients could be at risk of weak bones, and increased infections if physicians ignore low-sodium in the body, known as hyponatremia.

Dr. Moritz is clinical director, Division of Pediatric Nephrology at Children’s Hospital of UPMC and as Professor of Pediatrics at the University of Pittsburgh School of Medicine. Dr. Moritz is an authority on sodium and water metabolism in children.

Hospitalized patients at risk if sodium levels are low – Frontiers Science Hero from Frontiers on Vimeo.

Growing Kidneys in Lymph Nodes

Carl Bates, MD, and Sunder Sims-Lucas, PhD, of the Division of Pediatric Nephrology at Children’s Hospital of Pittsburgh of UPMC, in collaboration with Eric Lagasse, PhD, of the University of Pittsburgh McGowan Institute for Regenerative Medicine, are developing a novel approach to regenerate kidney tissues.

The group has discovered that embryonic kidney fragments and isolated nephron progenitors have the potential to form perfused kidney structures when injected into mouse lymph nodes. The Bates, Sims-Lucas, and Lagasse laboratories have received a Pediatric Device Initiative Award sponsored by the Commonwealth of Pennsylvania in support of their work.

 

Children’s Hospital of Pittsburgh Experts At the 30th Annual North American Cystic Fibrosis Conference

PrintThe 30th annual North American Cystic Fibrosis Conference was held October 27-29 in Orlando. The event brought scientists, clinicians, and other healthcare professionals from around the world to share the latest ideas and advances in cystic fibrosis research, drug development, and patient care, and included posters and presentations from field-leading experts.

Several Children’s Hospital of Pittsburgh of UPMC faculty and staff presented at the conference on a number of topics including:

 

Poster: Identification of Novel Inhibitors of Ubiquitination That Improve Rescue of F508DELCFTR by VX-809

Goeckeler-Fried, Jennifer; Estabrooks, Samuel K; Chiang, Annette; Chung, Wook Joon; Ye, Zhaofeng; Denny, Rajiah Aldrin; Weissman, Allan M; Camacho, Carlos J; Sorscher, Eric J; Brodsky, Jeffrey L

Poster: Recognition of CFTR By BCL2 Associated Athnaogene 3 (BAG3): A Pathway meditating Autophagic CFTR Degradation

Mishra, Sanjay K ; Frizzell, Raymond A.

Poster: Ubiquitin-Specific Proteases 11(USP11)and 28(USP28)Regulate F508DELCFTRStability Via SUMOConjugation

Gong, Xiaoyan; DaPaula ,AnaCarina; Ahner, Annette; Frizzell, Raymond A

Poster/Workshop Presentation: Predicted Phosphorylation Site in SLC26A9 Modulates CFTR-Dependent Activity

Wang, Xiaohui; Larsen, Mads B; Frizzell, Raymond A.; Bertrand, Carol A.

Poster: Pseudomonas aeruginosa Benefits From Respiratory Viral Infection in Cystic Fibrosis

Jeffrey Melvin

Poster: Viral-Bacterial Interactions in the Paranasal Sinuses in Cystic Fibrosis

Jeffrey Melvin

Abstract: Dynamics of Staphylococcus aureus growth on airway epithelial cells

Megan R Kiedrowski

Presenting: Thursday, Oct. 27 – W06: INF/MIC: Pathogenesis of Airway Infection (speaking 10:20-10:30a)

Poster Session I (11:15a-1:45p), Friday, Oct. 28 – JIB: Junior investigators best abstract session: Basic Science (12:15-1:35p)

Poster: Dysregulation of Nutritional Immunity during Respiratory Viral Infection Promotes Pseudomonas aeruginosa Biofilm Growth

Matthew Hendricks

Poster: SIRT1 Mediates Resveratrol Inhibition of CFTR In Primary Bronchial Epithelium

Agnieszka Swiatecka-Urban, MD, FASN

Poster: TGF-β1 Upregulates Microrna- 145 To Block ∆F508-CFTR Rescue

Agnieszka Swiatecka-Urban, MD, FASN

Poster: Anti-biofilm activity and in vivo efficacy of an engineered peptide antibiotic”

Berthony Deslouches

Workshop III: CLIN: *Emerging Issues in CF Lung Transplantation

A. Faro, J. Pilewski

Poster: Infection and depressed mucociliary clearance in pediatric and adult CF patients

Corcoran TE, Locke LW, Myerburg MM, Weiner DJ, Pilewski JM

Poster: Nitrogen Back Diffusion During Multiple Breath Washout With 100% Oxygen

Weiner DJ, Pederson K, Nielson JG.

Poster: Lung Function Perception in Cystic Fibrosis

Forno E, Weiner DJ

World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition 2016

PrintThe 5th World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition (WCPGHAN) was held October 5-8, 2016 in Montreal. This event showcased state of the art science and technology in the field of pediatric gastroenterology, hepatology, and nutrition, as well as presentations and keynote speeches from experts who are leaders in their field.

The conference encompassed nine themes including:

  • IBD
  • Celiac and other GI disorders
  • Neurogastroenterology and motility
  • Endoscopy
  • Hepatology
  • Pancreatology
  • Global health
  • Nutrition and intestinal rehabilitation
  • Transplantation

Several Children’s Hospital of Pittsburgh of UPMC faculty and staff spoke at the conference on a number topics including:

Immune Tolerance and Rejection
Pharmacology (pharmaco-genetics) and immunosuppression: Past, present and future
Patrick McKiernan, MD

Steatorrhea: What if it’s not Cystic Fibrosis
Mark Lowe, MD, PhD

Acute Liver Failure – Pathogenesis and Management
Rob Squires, MD and Anil Dhawan, MD

Pancreas
Mark Lowe, MD PhD

Pancreatitis

Inflammatory Responses/ Healing in Pancreatic Injury
Sohail Husain, MD

Poster Presentations

Sterile Cerebrosprinal Fluid Ascites: A Rare Complication After Ventriculoperitoneal Shunting
James Squires, MD, and  Kristen Critelli, MD (fellow)

Clinical Variability Following Partial External Biliary Diversion In Familial Intrahepatic Cholestasis 1 Deficiency
James Squires, MD,  Robert Squires, MD, and Amy Morris, RN, CCPC

Children’s Hospital of Pittsburgh of UPMC Part of New Pennsylvania Program to Improve Behavioral Health for Kids

PrintChildren’s Hospital of Pittsburgh of UPMC’s primary care network, Children’s Community Pediatrics (CCP), has been selected by the HealthChoices managed care organizations to take part in a new program to improve behavioral health treatment for children in the state.

Ted Dallas, secretary of the Department of Human Services in Pennsylvania, announced the selection of organizations to administer behavioral health treatment through the new Telephonic Psychiatric Consultation Service Program (TiPS). The announcement was made today at Children’s Hospital, with Dallas joining Christopher Gessner, president of Children’s Hospital, and Abigail Schlesinger, MD, medical director, Community Based Services, Behavioral Science Division of Children’s Hospital.

TiPS is a new program designed to provide real-time resources to physicians seeking consultation and advice for pediatric patients with behavioral health concerns who are served by Medicaid. A physician who uses the TiPS service will have access to a child and adolescent psychiatrist to discuss treatment options and get help linking the child to appropriate treatment if services outside of primary care are deemed necessary.

CCP, through its award-winning behavioral health program, will be manning the hotline to provide telephone consultation to any physician who is prescribing behavioral health medications to kids.

“It is critical that all children, regardless of where they live, have access to quality health care services,” said Dallas. “Access to child psychiatry has been a significant problem nationwide for years. Today’s announcement means that more of our kids will have the services they need, and not just another prescription.”

“Being able to provide behavioral health services in the primary care setting was a vision of ours and something that we’ve been doing for 8 years now,” said Dr. Schlesinger. “We’re excited to partner with the state of Pennsylvania to further enhance families’ ability to access behavioral health services in our region.”

CCP will serve the 26 counties in both the Southwest and Northwest zones. The Children’s Hospital of Philadelphia will serve the Southeast zone and Penn State Children’s Hospital will serve the Northeast and Lehigh/Capital zones.

For more information, visit www.childrenspeds.com.

Children’s Hospital of Pittsburgh of UPMC Earns National Recognition as Patient-Centered Medical Home

PrintChildren’s Hospital of Pittsburgh of UPMC’s Primary Care Center, part of the Division of General Academic Pediatrics, has received Level III Patient-Centered Medical Home (PCMH) Recognition from the National Committee for Quality Assurance (NCQA) for using evidence-based, patient-centered processes that focus on highly coordinated care and long‐term, participative relationships.

The three-year recognition is based on standards aligned with joint principles of the Patient-Centered Medical Home, a model of primary care that combines teamwork and information technology to improve care, to improve patients’ experience of care and to reduce costs.

“As the pediatric office for many of Pittsburgh’s underserved children, we are dedicated to providing the highest quality of care to the children in our region who are in need. This national recognition demonstrates our comprehensive approach and ongoing commitment to patient-centered care,” said Stacey Cook, MD, PhD, physician, General Academic Pediatrics, Children’s Hospital. “We provide increased access, implement quality improvement measures and provide continuing outreach to our families each and every day.”

Medical homes foster ongoing partnerships between patients, their families and their personal clinicians, instead of approaching care as the sum of episodic office visits. Each patient’s care is overseen by clinician-led care teams that coordinate treatment across the health care system.

“NCQA Patient-Centered Medical Home Recognition raises the bar in defining high-quality care by emphasizing access, health information technology and coordinated care focused on patients,” said NCQA President Margaret E. O’Kane. “Recognition shows that Children’s Hospital of Pittsburgh of UPMC has the tools, systems and resources to provide its patients with the right care, at the right time.”

NCQA standards are aligned with the joint principles of the Patient-Centered Medical Home established with the American College of Physicians, the American Academy of Family Physicians, the American Academy of Pediatrics and the American Osteopathic Association.

Children’s Hospital of Pittsburgh of UPMC Ranked in National Top 10 Seven Years in a Row

PrintChildren’s Hospital of Pittsburgh of UPMC, for the seventh consecutive year, has been named to the US News & World Report Honor Roll of America’s Best Children’s Hospitals.

The hospital is ranked seventh on the 2016-17 Honor Roll of America’s Best Children’s Hospitals, which was released today. The Best Children’s Hospitals rankings highlight the top 50 US pediatric hospitals in each of 10 specialties: cancer; cardiology and heart surgery; diabetes and endocrinology; gastroenterology and GI surgery; neonatology; nephrology; neurology and neurosurgery; orthopedics; pulmonology; and urology.

“We’re very proud to once again be recognized as one of the top children’s hospitals in the country,” said Christopher Gessner, president, Children’s Hospital. “Patients and families with complex medical conditions and needs increasingly are choosing our hospital because our physicians, nurses, and staff, who are among the leaders in their fields, are committed to providing the highest quality pediatric healthcare in the world every single day.”

The 2016-17 Best Children’s Hospitals rankings will be released online today and also will be published in the U.S. News “Best Hospitals 2017” guidebook, available in September.

US News introduced the Best Children’s Hospitals rankings in 2007 to help families of children with rare or life-threatening illnesses find the best medical care available. The rankings open the door to an array of detailed information about each hospital’s performance.

Children’s tied with one other hospital for seventh place. The 11 hospitals named to US News’ Honor Roll of Best Children’s Hospitals for 2016-17 are:
1. Boston Children’s Hospital
2. Children’s Hospital of Philadelphia
3. Cincinnati Children’s Hospital Medical Center
4. Texas Children’s Hospital, Houston
5. Seattle Children’s Hospital
6. Ann and Robert H. Lurie Children’s Hospital of Chicago
7. Children’s Hospital of Pittsburgh of UPMC
7. Children’s Hospital Los Angeles
9. Children’s Hospital Colorado, Aurora
10. Lucile Packard Children’s Hospital at Stanford, Palo Alto, California
10. Nationwide Children’s Hospital, Columbus, Ohio

Leading International Expert in Metabolic Liver Disease to Direct Hepatology Program at Children’s Hospital of Pittsburgh of UPMC

PrintPatrick McKiernan, MD, a leading expert in metabolic liver disease, has been appointed director of the Pediatric Hepatology Program at Children’s Hospital of Pittsburgh of UPMC, part of the Division of Pediatric Gastroenterology, Hepatology, and Nutrition. Dr. McKiernan will also join the staff of the hospital’s Center for Rare Disease Therapy.

Dr. McKiernan specializes in treating children with inherited metabolic disease and has an interest in developing less invasive therapies to help patients avoid or delay the need for liver transplantation. His research focus covers the clinical aspects of inherited metabolic liver disease, portal hypertension, novel endoscopic techniques, non-invasive markers of hepatic fibrosis, and immunosuppression following liver transplantation. He is actively involved in research on stem cell therapy for metabolic liver diseases and recently was the UK principle investigator on a stem cell study involving children with urea cycle disorders and Crigler-Najjar syndrome.

“Dr. McKiernan is among the world’s leading physician-scientists with expertise in pediatric hepatology, specifically inherited metabolic disease,” said Mark Lowe, MD, PhD, chief of the Division of Pediatric Gastroenterology, Hepatology and Nutrition at Children’s Hospital. “His appointment enhances Children’s ability to provide care for children from around the world with complex metabolic conditions in need of the highest level of care.”

Dr. McKiernan also has a special interest is tyrosinemia, an inherited disorder caused by an enzyme deficiency that can lead to life-threatening liver and kidney failure. In a study published in 2014, Dr. McKiernan and his colleagues found that children whose tyrosinemia was identified at birth through newborn screening and started on the drug nitisinone developed normally and showed no signs of liver or kidney disease.

Dr. McKiernan comes to Children’s from Birmingham Children’s Hospital in the United Kingdom, where he was a hepatologist in the liver unit since 1994. He trained in medicine and pediatrics at Queen’s University in Belfast.

Dr. McKiernan is a member of the British Medical Association, British Society of Pediatric Gastroenterology, Hepatology and Nutrition, British Association for the Study of the Liver, and American Association for the Study of Liver Diseases. He also is an associate professor at the University of Pittsburgh School of Medicine.
As an international expert in metabolic disease, Dr. McKiernan is part of the Center for Rare Disease Therapy at Children’s, an integrated team of experts who have developed innovative therapies to treat a multitude of rare diseases.

Children’s has performed more than 330 liver transplants for patients with metabolic disease, which is more than any other center, including adult facilities. In addition, Children’s is a leading center for liver transplantation as a therapeutic option for children with maple syrup urine disease (MSUD). Children’s developed the first liver transplant protocol for MSUD in 2004 and since then has successfully performed more liver transplants in patients with MSUD than any other center in the world with 100 percent patient and graft survival.

For more information on Dr. McKiernan and the Pediatric Hepatology Program at Children’s Hospital of Pittsburgh of UPMC, please visit www.chp.edu/hepatology.

New Placenta Model Could Reveal How Birth Defect-Causing Infectious Agents Cross From Mother to Baby

Researchers at the University of Pittsburgh and Magee-Womens Research Institute (MWRI) have devised a cell-based model of the human placenta that could help explain how pathogens that cause birth defects, such as Zika virus, cross from mother to unborn child. The findings were published today in Science Advances.

The placenta is a complex and poorly understood organ that anchors the developing fetus to the uterus, nourishes the baby, and provides a barrier to the spread of microorganisms from an infected mother to the fetus, explained senior investigator Carolyn Coyne, PhD, associate professor of microbiology and molecular genetics at Pitt School of Medicine and a member of the MWRI.

“The human placenta is unique and unlike that of other many other placental mammals,” she said. “With our new model in the research toolkit, we and other scientists hope to advance our knowledge of the placenta, examine its function , and learn how it can prevent most, but not all, maternal infections from causing problems for the baby.

Researchers currently can obtain and study placental cell lines, but such cells do not fuse spontaneously to form the characteristic structure of the human organ. Some scientists study cells, called primary human trophoblasts, that are isolated from placentas obtained after childbirth, but such cells do not divide, can be more difficult to obtain, and are more difficult to genetically manipulate to learn about biochemical pathways that have a role in placental function, Dr. Coyne said.

Dr. Coyne’s team took a different approach: They cultured a human placental trophoblast cell line in a microgravity bioreactor system developed by NASA. The trophoblasts along with blood vessel cells were added to small dextran beads that were then spun around in a container filled with cell culture fluid, creating shear stress and rotational forces to better mimic the environment at the maternal-fetal interface than static cell-culture systems.

As a result, the cells fused to form syncytiotrophoblasts, and thus more closely resemble the primary cells lining the outermost layer of the tree-like or villous structure of the human placental tissue. Next, the researchers tested the functional properties of their model by exposing it to a virus and to Toxoplasma gondii, a parasite found in cat feces that can lead to fetal infection, causing miscarriage, congenital disease and/or disability later in life.

“We found that the syncytiotrophoblasts formed in our system recapitulated the barrier properties of the naturally occurring cells and they resisted infection by a model virus and three genetically different strains of Toxoplasma,” said co-investigator Jon P. Boyle, PhD, associate professor of biological sciences at Pitt. “With this model, we can experiment with different biological factors to see what might allow an infectious agent to get through the placental barrier to the fetus.”

Understanding the placenta might one day lead to ways to prevent fetal damage from the so-called TORCH infections: toxoplasmosis, rubella, cytomegalovirus, herpes and HIV, he added.

The researchers are beginning to use their model to test whether Zika virus, and other pathogens associated with congenital disease, can infect placental cells and/or cross the placental barrier.

Research team members include Cameron McConkey, BS, Elizabeth Delorme-Axford, PhD, and Yoel Sadovsky, MD, all of the University of Pittsburgh; Cheryl A. Nickerson, PhD, of Arizona State University; and Kwang Sik Kim, MD, of Johns Hopkins University.

The project was funded by National Institutes of Health grants AI081759 and HD075665 and the Burroughs Wellcome Fund.

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