2022 Research

Autosomal Recessive Cerebellar Atrophy and Spastic Ataxia in Patients With Pathogenic Biallelic Variants in GEMIN5. Rajan DS, Kour S, Fortuna TR, Cousin MA, Barnett SS, Niu Z, Babovic-Vuksanovic D, Klee EW, Kirmse B, Innes M, Rydning SL, Selmer KK, Vigeland MD, Erichsen AK, Nemeth AH, Millan F, DeVile C, Fawcett K, Legendre A, Sims D, Schnekenberg RP, Burglen L, Mercier S, Bakhtiari S, Martinez-Salas E, Wigby K, Lenberg J, Friedman JR, Kruer MC, Pandey UB.Front Cell Dev Biol. 2022 Feb 28;10:783762. doi: 10.3389/fcell.2022.783762. eCollection 2022.PMID: 35295849 

Matrin-3 dysfunction in myopathy and motor neuron degeneration. Ward C, Pandey UB.Neural Regen Res. 2022 Mar;17(3):575-576. doi: 10.4103/1673-5374.320986.PMID: 34380896 

Quantitative Electroencephalography (EEG) Predicting Acute Neurologic Deterioration in the Pediatric Intensive Care Unit: A Case Series. Munjal NK, Bergman I, Scheuer ML, Genovese CR, Simon DW, Patterson CM. J Child Neurol. 2022 Jan;37(1):73-79. doi: 10.1177/08830738211053908. Epub 2021 Nov 24. PMID: 34816755; PMCID: PMC8691173. 

Corrigendum to: LTBK-01. Updates On The Phase Ii And Re-treatment Study Of AZD6244 (Selumetinib) For Children With Recurrent Or Refractory Pediatric Low Grade Glioma: A Pediatric Brain Tumor Consortium (PBTC) Study. Fangusaro JR, Onar-Thomas A, Poussaint TY, Wu S, Ligon AH, Lindeman NI, Banerjee A, Packer R, Kilburn LB, Pollack IF, Qaddoumi IA, Fisher PG, Dhall G, Baxter PA, Kreissman SG, Doyle LA, Smith MA, Fouladi M, Dunkel IJ.Neuro Oncol. 2022 Mar 21:noac029. doi: 10.1093/neuonc/noac029. Online ahead of print.PMID: 35307742 

The Hydrocephalus Clinical Research Network quality improvement initiative: the role of antibiotic-impregnated catheters and vancomycin wound irrigation. Chu J, Jensen H, Holubkov R, Krieger MD, Kulkarni AV, Riva-Cambrin J, Rozzelle CJ, Limbrick DD, Wellons JC, Browd SR, Whitehead WE, Pollack IF, Simon TD, Tamber MS, Hauptman JS, Pindrik J, Naftel RP, McDonald PJ, Hankinson TC, Jackson EM, Rocque BG, Reeder R, Drake JM, Kestle JRW; Hydrocephalus Clinical Research Network; Hydrocephalus Clinical Research Network Members.J Neurosurg Pediatr. 2022 Mar 18:1-8. doi: 10.3171/2022.2.PEDS2214. Online ahead of print.PMID: 35303708 

Reversing tozasertib resistance in glioma through inhibition of pyruvate dehydrogenase kinases. Jane EP, Premkumar DR, Rajasundaram D, Thambireddy S, Reslink MC, Agnihotri S, Pollack IF.Mol Oncol. 2022 Jan;16(1):219-249. doi: 10.1002/1878-0261.13025. Epub 2021 Jun 23.PMID: 34058053 

he current landscape of immunotherapy for pediatric brain tumors. Hwang EI, Sayour EJ, Flores CT, Grant G, Wechsler-Reya R, Hoang-Minh LB, Kieran MW, Salcido J, Prins RM, Figg JW, Platten M, Candelario KM, Hale PG, Blatt JE, Governale LS, Okada H, Mitchell DA, Pollack IF.Nat Cancer. 2022 Jan;3(1):11-24. doi: 10.1038/s43018-021-00319-0. Epub 2022 Jan 20.PMID: 35121998 

Serum levels of the cold stress hormones FGF21 and GDF-15 after cardiac arrest in infants and children enrolled in single center therapeutic hypothermia clinical trials. Herrmann JR, Fink EL, Fabio A, Au AK, Berger RP, Janesko-Feldman K, Clark RSB, Kochanek PM, Jackson TC.Resuscitation. 2022 Mar;172:173-180. doi: 10.1016/j.resuscitation.2021.11.016. Epub 2021 Nov 22.PMID: 34822938 

Association between pediatric TBI mortality and median family income in the United States: A retrospective cohort study. Pelletier JH, Rakkar J, Simon D, Au AK, Fuhrman DY, Clark RSB, Kochanek PM, Horvat CM.Lancet Reg Health Am. 2022 Jan;5:100164. doi: 10.1016/j.lana.2021.100164. Epub 2022 Jan 5.PMID: 35252952 

P. aeruginosa augments irradiation injury via 15-lipoxygenase-catalyzed generation of 15-HpETE-PE and induction of theft-ferroptosis. Dar HH, Epperly MW, Tyurin VA, Amoscato AA, Anthonymuthu TS, Souryavong AB, Kapralov AA, Shurin GV, Samovich SN, St Croix CM, Watkins SC, Wenzel SE, Mallampalli RK, Greenberger JS, Bayır H, Kagan VE, Tyurina YY.JCI Insight. 2022 Feb 22;7(4):e156013. doi: 10.1172/jci.insight.156013.PMID: 35041620 

Integrated -omics approach reveals persistent DNA damage rewires lipid metabolism and histone hyperacetylation via MYS-1/Tip60. Hamsanathan S, Anthonymuthu T, Han S, Shinglot H, Siefken E, Sims A, Sen P, Pepper HL, Snyder NW, Bayir H, Kagan V, Gurkar AU.Sci Adv. 2022 Feb 18;8(7):eabl6083. doi: 10.1126/sciadv.abl6083. Epub 2022 Feb 16.PMID: 35171671 

C-ferroptosis is an iron-dependent form of regulated cell death in cyanobacteria. Aguilera A, Berdun F, Bartoli C, Steelheart C, Alegre M, Bayir H, Tyurina YY, Kagan VE, Salerno G, Pagnussat G, Martin MV.J Cell Biol. 2022 Feb 7;221(2):e201911005. doi: 10.1083/jcb.201911005. Epub 2021 Nov 24.PMID: 34817556 

Inactivation of RIP3 kinase sensitizes to 15LOX/PEBP1-mediated ferroptotic death. Lamade AM, Wu L, Dar HH, Mentrup HL, Shrivastava IH, Epperly MW, St Croix CM, Tyurina YY, Anthonymuthu TS, Yang Q, Kapralov AA, Huang Z, Mao G, Amoscato AA, Hier ZE, Artyukhova MA, Shurin G, Rosenbaum JC, Gough PJ, Bertin J, VanDemark AP, Watkins SC, Mollen KP, Bahar I, Greenberger JS, Kagan VE, Whalen MJ, Bayır H.Redox Biol. 2022 Apr;50:102232. doi: 10.1016/j.redox.2022.102232. Epub 2022 Jan 10.PMID: 35101798 

Redox Pioneer: Professor Valerian Kagan. Bayır H, Maguire JJ, Cadenas E.Antioxid Redox Signal. 2022 Jan 24. doi: 10.1089/ars.2021.0079. Online ahead of print.PMID: 35072541 

Ascorbate deficiency confers resistance to hippocampal neurodegeneration after asphyxial cardiac arrest in juvenile rats. Wolf MS, Manole MD, New LA, Chen Y, Soysal E, Kochanek PM, Bayır H, Clark RSB.Pediatr Res. 2022 Mar;91(4):820-827. doi: 10.1038/s41390-021-01515-5. Epub 2021 Apr 12.PMID: 33846553 Free PMC article. 

Tandem Therapeutic Plasma Exchange Reduces Continuous Renal Replacement Therapy Downtime. Foglia MJ, Pelletier JH, Bayir H, Fleck A, Konyk L, McSteen C, Fisher D, Fuhrman DY.Blood Purif. 2022;51(6):523-530. doi: 10.1159/000518348. Epub 2021 Sep 1.PMID: 34515068 

The current landscape of immunotherapy for pediatric brain tumors. Hwang EI, Sayour EJ, Flores CT, Grant G, Wechsler-Reya R, Hoang-Minh LB, Kieran MW, Salcido J, Prins RM, Figg JW, Platten M, Candelario KM, Hale PG, Blatt JE, Governale LS, Okada H, Mitchell DA, Pollack IF.Nat Cancer. 2022 Jan;3(1):11-24. doi: 10.1038/s43018-021-00319-0. Epub 2022 Jan 20.PMID: 35121998 Review. 

Quantitative Sodium (23Na) MRI in Pediatric Gliomas: Initial Experience. Bhatia A, Lee VK, Qian Y, Paldino MJ, Ceschin R, Hect J, Mountz JM, Sun D, Kohanbash G, Pollack IF, Jakacki RI, Boada F, Panigrahy A.Diagnostics (Basel). 2022 May 13;12(5):1223. doi: 10.3390/diagnostics12051223.PMID: 35626378 Free PMC article. 

Endoscopic third ventriculostomy revision after failure of initial endoscopic third ventriculostomy and choroid plexus cauterization. Arynchyna-Smith A, Rozzelle CJ, Jensen H, Reeder RW, Kulkarni AV, Pollack IF, Wellons JC, Naftel RP, Jackson EM, Whitehead WE, Pindrik JA, Limbrick DD, McDonald PJ, Tamber MS, O'Neill BR, Hauptman JS, Krieger MD, Chu J, Simon TD, Riva-Cambrin J, Kestle JRW, Rocque BG; Hydrocephalus Clinical Research Network.J Neurosurg Pediatr. 2022 Apr 22:1-10. doi: 10.3171/2022.3.PEDS224. Online ahead of print.PMID: 35453104 

Loss of MAT2A compromises methionine metabolism and represents a vulnerability in H3K27M mutant glioma by modulating the epigenome. Golbourn BJ, Halbert ME, Halligan K, Varadharajan S, Krug B, Mbah NE, Kabir N, Stanton AJ, Locke AL, Casillo SM, Zhao Y, Sanders LM, Cheney A, Mullett SJ, Chen A, Wassell M, Andren A, Perez J, Jane EP, Premkumar DRD, Koncar RF, Mirhadi S, McCarl LH, Chang YF, Wu YL, Gatesman TA, Cruz AF, Zapotocky M, Hu B, Kohanbash G, Wang X, Vartanian A, Moran MF, Lieberman F, Amankulor NM, Wendell SG, Vaske OM, Panigrahy A, Felker J, Bertrand KC, Kleinman CL, Rich JN, Friedlander RM, Broniscer A, Lyssiotis C, Jabado N, Pollack IF, Mack SC, Agnihotri S.Nat Cancer. 2022 May;3(5):629-648. doi: 10.1038/s43018-022-00348-3. Epub 2022 Apr 14.PMID: 35422502 

The Eyebrow Approach for the Management of Pediatric Frontal Epidural Abscesses Secondary to Diffuse Sinusitis. Hallak H, Abou-Al-Shaar H, Mallela AN, McDowell MM, Jabbour N, Padia R, Greene S, Pollack IF.Pediatr Neurosurg. 2022 Apr 8. doi: 10.1159/000524537. Online ahead of print.PMID: 35398851 

Functional and structural deficiencies of Gemin5 variants associated with neurological disorders. Francisco-Velilla R, Embarc-Buh A, Del Caño-Ochoa F, Abellan S, Vilar M, Alvarez S, Fernandez-Jaen A, Kour S, Rajan DS, Pandey UB, Ramón-Maiques S, Martinez-Salas E.Life Sci Alliance. 2022 Apr 7;5(7):e202201403. doi: 10.26508/lsa.202201403. Print 2022 Jul.PMID: 35393353 Free PMC article. 

Pathogenic variants of Valosin-containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells. Ferrari V, Cristofani R, Cicardi ME, Tedesco B, Crippa V, Chierichetti M, Casarotto E, Cozzi M, Mina F, Galbiati M, Piccolella M, Carra S, Vaccari T, Nalbandian A, Kimonis V, Fortuna TR, Pandey UB, Gagliani MC, Cortese K, Rusmini P, Poletti A.Neuropathol Appl Neurobiol. 2022 May 2:e12818. doi: 10.1111/nan.12818. Online ahead of print.PMID: 35501124

Guidelines for measuring reactive oxygen species and oxidative damage in cells and in vivo. Murphy MP, Bayir H, Belousov V, Chang CJ, Davies KJA, Davies MJ, Dick TP, Finkel T, Forman HJ, Janssen-Heininger Y, Gems D, Kagan VE, Kalyanaraman B, Larsson NG, Milne GL, Nyström T, Poulsen HE, Radi R, Van Remmen H, Schumacker PT, Thornalley PJ, Toyokuni S, Winterbourn CC, Yin H, Halliwell B.Nat Metab. 2022 Jun;4(6):651-662. doi: 10.1038/s42255-022-00591-z. Epub 2022 Jun 27.PMID: 35760871  

The authors reply. Soysal E, Horvat CM, Simon DW, Wolf MS, Tyler-Kabara E, Gaines BA, Clark RSB, Kochanek PM, Bayir H.Pediatr Crit Care Med. 2022 Jun 1;23(6):e301-e302. doi: 10.1097/PCC.0000000000002938. Epub 2022 May 27.PMID: 35703782  

Use of Magnetic Resonance Imaging in Neuroprognostication After Pediatric Cardiac Arrest: Survey of Current Practices. PiantinoA, Ruzas CM, Press CA, Subramanian S, Balakrishnan B, Panigrahy A, Pettersson D, Maloney JA, Vossough A, Topjian A, Kirschen MP, Doughty L, Chung MG, Maloney D, Haller T, Fabio A, Fink EL; POCCA Investigators. Pediatr Neurol. 2022 Sep;134:45-51. doi: 10.1016/j.pediatrneurol.2022.06.011. Epub 2022 Jun 20. PMID: 35835025 

Resuscitation with epinephrine worsens cerebral capillary no-reflow after experimental pediatric cardiac arrest: An in vivo multiphoton microscopy evaluation. Oghifobibi OA, Toader AE, Nicholas MA, Nelson BP, Alindogan NG, Wolf MS, Kline AE, Nouraie SM, Bondi CO, Iordanova B, Clark RS, Bayır H, Loughran PA, Watkins SC, St Croix CM, Kochanek PM, Vazquez AL, Manole MD.J Cereb Blood Flow Metab. 2022 Dec;42(12):2255-2269. doi: 10.1177/0271678X221113022. Epub 2022 Jul 19.PMID: 35854408 

Association of Blood-Based Brain Injury Biomarker Concentrations With Outcomes After Pediatric Cardiac Arrest. Fink EL, Kochanek PM, Panigrahy A, Beers SR, Berger RP, Bayir H, Pineda J, Newth C, Topjian AA, Press CA, Maddux AB, Willyerd F, Hunt EA, Siems A, Chung MG, Smith L, Wenger J, Doughty L, Diddle JW, Patregnani J, Piantino J, Walson KH, Balakrishnan B, Meyer MT, Friess S, Maloney D, Rubin P, Haller TL, Treble-Barna A, Wang C, Clark RRSB, Fabio A; Personalizing Outcomes After Child Cardiac Arrest (POCCA) Investigators.JAMA Netw Open. 2022 Sep 1;5(9):e2230518. doi: 10.1001/jamanetworkopen.2022.30518.PMID: 36074465  

Endogenous Interleukin-17a Contributes to Normal Spatial Memory Retention but Does Not Affect Early Behavioral or Neuropathological Outcomes after Experimental Traumatic Brain Injury. Simon DW, Raphael I, Johnson KM, Dixon CE, Vagni V, Janesko-Feldman K, Kochanek PM, Bayir H, Clark RSB, McGeachy MJ.Neurotrauma Rep. 2022 Sep 1;3(1):340-351. doi: 10.1089/neur.2022.0017. eCollection 2022.PMID: 36204388 

Healthcare Use in the Year Following Bronchiolitis Hospitalization. Pelletier JH, Au AK, Fuhrman DY, Marroquin OC, Suresh S, Clark RSB, Kochanek PM, Horvat CM.Hosp Pediatr. 2022 Nov 1;12(11):937-949. doi: 10.1542/hpeds.2022-006657.PMID: 36281706 

Clinical translation of noninvasive intracranial pressure sensing with diffuse correlation spectroscopy. Tabassum S, Ruesch A, Acharya D, Yang J, Relander FAJ, Scammon B, Wolf MS, Rakkar J, Clark RSB, McDowell MM, Kainerstorfer JM.J Neurosurg. 2022 Nov 11:1-10. doi: 10.3171/2022.9.JNS221203. Online ahead of print.PMID: 36683191 

Association of EEG and Blood-Based Brain Injury Biomarker Accuracy to Prognosticate Mortality After Pediatric Cardiac Arrest: An Exploratory Study. Anetakis KM, Gedela S, Kochanek PM, Clark RSB, Berger RP, Fabio A, Angus DC, Watson RS, Callaway CW, Bell MJ, Sogawa Y, Fink EL. Pediatr Neurol. 2022 Sep;134:25-30. doi: 10.1016/j.pediatrneurol.2022.06.005. Epub 2022 Jun 14. PMID: 35785591 

Temporal Patterns in Brain Tissue and Systemic Oxygenation Associated with Mortality After Severe Traumatic Brain Injury in Children. Rakkar J, Azar J, Pelletier JH, Au AK, Bell MJ, Simon DW, Kochanek PM, Clark RSB, Horvat CM. Neurocrit Care. 2022 Sep 28. doi: 10.1007/s12028-022-01602-3. Online ahead of print. PMID: 36171518 

Endogenous Interleukin-17a Contributes to Normal Spatial Memory Retention but Does Not Affect Early Behavioral or Neuropathological Outcomes after Experimental Traumatic Brain Injury. Simon DW, Raphael I, Johnson KM, Dixon CE, Vagni V, Janesko-Feldman K, Kochanek PM, Bayir H, Clark RSB, McGeachy MJ.Neurotrauma Rep. 2022 Sep 1;3(1):340-351. doi: 10.1089/neur.2022.0017. eCollection 2022.PMID: 36204388 

Limbic pathway vulnerability associates with neurologic outcome in children after cardiac arrest. Jarvis JM, Roy J, Schmithorst V, Lee V, Devine D, Meyers B, Munjal N, Clark RSB, Kochanek PM, Panigrahy A, Ceschin R, Fink EL.Resuscitation. 2023 Jan;182:109634. doi: 10.1016/j.resuscitation.2022.10.026. Epub 2022 Nov 3.PMID: 36336196