Pandey Appointed Director of CNI

February 1, 2023

Children's Neuroscience Institutes Welcomes Dr. Pandey!

Pitt Pediatrics is excited to announce the appointment of Dr. Udai Pandey as Director of the Children’s Neuroscience Institute (CNI) at UPMC Children’s Hospital of Pittsburgh.

Dr. Pandey is an Associate Professor of Pediatrics in the Division of Pediatric Neurology. He also holds secondary appointments in the Departments of Human Genetics and Neurology. Dr. Pandey completed his Ph.D. training at the Sanjay Gandhi Postgraduate Institute of Medical Sciences followed by postdoctoral fellowships at the University of Pennsylvania and St. Jude Children’s Research Hospital. He joined the faculty at UPMC Children’s Hospital in 2014.

Dr. Pandey’s research interests are focused on understanding the molecular basis of human neurological diseases, including motor neuron diseases, traumatic brain injury, and rare neurogenetic diseases. His research program incorporates genomic, proteomic, small-molecule, and genetic screens to identify new therapeutic targets for neurological conditions. He is a member of the training faculty in the Human Genetics, Integrated Systems Biology, and Neurobiology graduate programs and was previously CNI Associate Director.

In his role as CNI Director, Dr. Pandey will develop and foster initiatives to facilitate neuroscience research at UPMC Children’s Hospital and serve as a bridge between research and clinical faculty. He aims to bring together investigators with interdisciplinary expertise to advance mechanistic understanding of normal brain development, identifying pathogenic pathways responsible for neurological diseases and discovering personalized treatment strategies for children. Through the CNI, he will offer a collaborative environment, research resources, seed funding, visiting speakers, and training opportunities for postdoctoral fellows and junior faculty members, with an intentional focus on diversity, equity, and inclusion.

We take this opportunity to thank Dr. Hülya Bayir, the inaugural Director of the CNI, for her outstanding service to our hospital and institution and for leaving such a strong foundation on which Dr. Pandey can build.

Dr. Pandey will begin his new responsibilities on March 1, 2023.

For additional information, click here.

RRS SEMINAR PROGRAM: TIMOTHY PHOENIX, PHD

May 23, 2023

CNI-SPONSORED SPEAKER

Please join us on Tuesday, May 23, 2023, from 12:00 PM to 1:00 PM via Microsoft Team for a CNI seminar through the John G. Rangos Sr. Research Seminar Series by Dr. Timothy Phoenix, PhD. Dr. Phoenix joins us from the University of Cincinnati where he is an Assistant Professor in the James L. Winkle College of Pharmacy.

RRS SEMINAR PROGRAM: PULKIT GROVER, PHD

April 11, 2023

CNI-SPONSORED SPEAKER

Please join us on Tuesday, April 11, 2023, from 12:00 PM to 1:00 PM via Microsoft Team for a CNI seminar through the John G. Rangos Sr. Research Seminar Series by Dr. Pulkit Grover, PhD. Dr. Grover joins us from Carnegie Mellon University where he is an Angel Jordan Professor in the Department of Electrical and Computer Engineering.

RRS SEMINAR PROGRAM: NAIMA LAJUD, PHD

March 21, 2023

CNI-SPONSORED SPEAKER

Please join us on Tuesday, March 21, 2023, from 12:00 PM to 1:00 PM via Microsoft Team for a CNI seminar through the John G. Rangos Sr. Research Seminar Series by Dr. Naima Lajud, PhD. Dr. Lajud joins us from the Michoacán Biomedical Research Center where she is a Senior Researcher in the Neuroscience Division and Developmental Neurobiology Lab.

RRS SEMINAR PROGRAM: ANNA PENN, MD, PHD

December 13, 2022

CNI-SPONSORED SPEAKER

Please join us on Tuesday, December 13, 2022, from 12:00 PM to 1:00 PM via Microsoft Team for a CNI seminar through the John G. Rangos Sr. Research Seminar Series by Dr. Anna Penn, MD, PhD. Dr. Penn joins us from Columbia University where she is the Chief for the Division of Neonatology and a SFARI Investigator.

INA GRANT AWARDEES, DR. ALBERTO VAZQUEZ AND DR. MIOARA MANOLE, PUBLISH ON RESUSCITATION

August 11, 2022

Resuscitation with epinephrine worsens cerebral capillary no-reflow after experimental pediatric cardiac arrest: An in vivo multiphoton microscopy evaluation

Epinephrine is the principal resuscitation therapy for pediatric cardiac arrest (CA). Clinical data suggest that although epinephrine increases the rate of resuscitation, it fails to improve neurological outcome, possibly secondary to reductions in microvascular flow. We characterized the effect of epinephrine vs. placebo administered at resuscitation from pediatric asphyxial CA on microvascular and macrovascular cortical perfusion assessed using in vivo multiphoton microscopy and laser speckle flowmetry, respectively, and on brain tissue oxygenation (PbO2), behavioral outcomes, and neuropathology in 16-18-day-old rats. Epinephrine-treated rats had a more rapid return of spontaneous circulation and brisk immediate cortical reperfusion during 1-3 min post-CA vs. placebo.

INA GRANT AWARDEES, DR. DEEPA RAJAN AND DR. UDAI PANDEY, PUBLISH ON GEMIN4

August 11, 2022

Further delineation of GEMIN4 related neurodevelopmental disorder with microcephaly, cataract, and renal abnormalities syndrome.

Pathogenic variants in GEMIN4 have recently been linked to an inherited autosomal recessive neurodevelopmental disorder characterized with microcephaly, cataracts, and renal abnormalities (NEDMCR syndrome). This report provides a retrospective review of 16 patients from 11 unrelated Saudi consanguineous families with GEMIN4 mutations. The cohort comprises 11 new and unpublished clinical details from five previously described patients. Only two missense, homozygous, pathogenic variants were found in all affected patients, suggesting a founder effect. All patients shared global developmental delay with variable ophthalmological, renal, and skeletal manifestations.

RESEARCH FUNDING: 2022-23 INA RECIPIENTS MIOARA MANOLE, MD, FAAP AND ALBERTO VAZQUEZ, PHD

July 6, 2022

Optogenetic Targeting of Cerebral Pericytes and Smooth Muscle Cells to Ameliorate Capillary Stasis

Dr. Mioara Manole, MD, FAAP from the Department of Pediatrics and Pharmacy and Therapeutics and her co-investigators (Alberto Vazquez, PhD from the Department of Radiology and Dennis Simon, MD from the Department of Critical Care Medicine) are studying abnormal contractions of vascular contractile cells, pericytes and smooth muscle cells after successful resuscitation from cardiac arrest. These cells limit blood flow to the recovering brain and may worsen neurological outcome. They have now proposed to improve blood flow and metabolism in the brain after cardiac arrest using a specific strategy that induces relaxation of vascular cells in specific areas of the brain.

The team will generate mice that express light-sensitive channels in the pericytes and smooth muscle cells. Upon stimulation with light, relaxation is induced in these vascular contractile cells. If relaxing the perivascular cells proves to be successful in restoring normal brain perfusion metabolism, microvascular-specific therapies can be developed to improve neurological function after brain injury.

For additional information, click here.

RESEARCH FUNDING: 2022-23 INA RECIPIENTS ANTONY MICHEALRAJ, PHD AND IAN F. POLLACK, MD, FACS, FAAP

July 6, 2022

Elucidating Genetic and Therapeutic Vulnerabilities of Lethal Pediatric Ependymomas

Dr. Antony Michealraj, PhD and Ian F. Pollack, MD, FACS, FAAP from the Department of Neurological Surgery are examining supratentorial ependymoma as one of the lethal pediatric cancers which does not have an effective therapeutic option. Up to 40% of children with this Ependymoma succumb to their disease, and survivors are often left disabled because of toxicity from the tumor and treatment. They have made reasonable progress on identifying the abnormal gene elements that could potentially drive this lethal tumor. However, they are still far behind in understanding the molecular network which makes children vulnerable to this tumor. Unraveling this network is very important for novel therapeutic interventions.

For additional information, click here.

RESEARCH FUNDING: 2022-23 INA RECIPIENTS EDUARDO VIEIRA NETO, MD, PHD AND JERRY VOCKLEY, MD, PHD, FACMG

July 6, 2022

Mitochondrial Trifunctional Protein Deficiency: Mitochondrial Bioenergetics, Cardiolipin, and Novel Therapeutic Targets

Dr. Eduardo Vieira Neto, MD, PhD from the Department of Pediatrics, Jerry Vockley, MD, PhD, FACMG from the Department of Pediatrics and their co-investigators (Hülya Bayır, MD from the Department of Pediatrics, Department of Critical Care Medicine, and Department of Environmental and Occupational Health; and Valerian Kağan, PhD, DSc from the Department of Environmental and Occupational Health as well as the Department of Pharmacology and Chemical Biology, the Department of Radiation Oncology, and the Department of Chemistry) are exploring the mitochondrial trifunctional protein (TFP) deficiency as an inherited disorder caused by a block in fatty acid oxidation (FAO); the process by which stored fats are utilized for energy production during times of physiologic stress such as fasting, exercise, and intercurrent illnesses. Affected children can have a devastating disease including cardiomyopathy, hypoglycemia, and hyperammonemia with early mortality, or a chronic course with recurrent rhabdomyolysis, peripheral neuropathy, retinal degeneration as well as continued risk for cardiomyopathy. In addition to its role in FAO, TFP catalyzes a step in the synthesis of cardiolipin (CL), a lipid unique and critical to the inner mitochondrial membrane structure.

For additional information, click here.

RESEARCH FUNDING: 2022-23 INA RECIPIENTS STEPHEN P. EMERY, MD AND STEPHANIE GREENE, MD

July 6, 2022

Ventriculoamniotic Shunting for Fetal Aqueductal Stenosis (VASFAS)

Dr. Stephen P. Emery, MD from the Department of Obstetrics, Gynecology, and Reproductive Sciences and his co-investigators (Stephanie Greene, MD from the Department of Neurological Surgery and Youngjae Chun, PhD from the Department of Industrial Engineering) are investigating fetal aqueductal stenosis (FAS) as it produces noncommunicating hydrocephalus (increased cerebrospinal fluid, or CSF) and increased intracranial pressure on the developing fetal brain resulting in permanent neurologic injury in survivors. Current management is neonatal shunting and management of neurologic sequelae. They hypothesize that in-utero shunting of excess CSF will prevent neurologic damage, resulting in normal fetal brain development, thereby preventing lifelong adverse neurologic outcomes. They have developed a novel ventriculo-amniotic shunt and are testing it in a large animal model (fetal sheep), where they have demonstrated proof-of- concept. Funding from CNI will enable them to prepare a Humanitarian Use Device application for the FDA.