Kim E. Nichols, MD
Kim E. Nichols, MD

Kim E. Nichols, MD

Member, St. Jude Faculty

  • Director, Cancer Predisposition Division

Departments

Divisions

Education

BA – Dartmouth College
MD – Duke University School of Medicine

Honors & Awards

  • 2014 Alex’s Lemonade Stand Foundation Infrastructure Award, Center for Childhood Cancer Research, The Children’s Hospital of Philadelphia
  • 2013 William B Lawrence & Blanche Hughes Award
  • 2013 Nezelof Prize for Basic Research, The Histiocyte Society
  • 2013 Alex’s Lemonade Stand Foundation Innovation Award, Center for Childhood Cancer Research, The Children’s Hospital of Philadelphia
  • 2012 Alex’s Lemonade Stand Foundation Innovation Award, Center for Childhood Cancer Research, The Children’s Hospital of Philadelphia
  • 2011 Member, Society for Pediatric Research
  • 2011 Center for Integration of Genetic Healthcare Technologies Award, University of Pennsylvania Perelman School of Medicine, Philadelphia
  • 2010 Foerderer Award, The Children’s Hospital of Philadelphia
  • 2008 XLP Research Trust Award, London, England
  • 2002 American Society of Hematology Junior Faculty Scholar Award

Research Interests

I am a pediatric oncologist with research interests in the molecular mechanisms that provide protection against viral infections and cancer. My overarching goals are to understand how defects in these mechanisms contribute to disease and use this information to develop more effective treatments for children with increased genetic risk for infection and/or malignancy.

Towards this end, my research focuses on:

  • Identifying novel genetic causes of childhood cancer
  • Optimizing methods of tumor surveillance for children with increased cancer risk
  • Evaluating the factors that influence parent and adolescent decision making and communication around genetic testing for heritable cancer risk
  • Understanding the behavioral and psychological impacts of cancer genetic testing on children and their families
  • Increasing understanding and developing new therapies for malignant and non-malignant hematologic disorders, including B-cell leukemias and lymphomas, hemophagocytic lymphohistiocytosis and Epstein-Barr virus-associated lymphoproliferations

Selected Publications

Kesserwan C, Friedman Ross L, Bradbury AR, Nichols KE. The Advantages and Challenges of Testing Children for Heritable Predisposition to Cancer. Am Soc Clin Oncol Educ Book 35:251-69, 2016. doi: 10.14694/EDBK_160621.

Hyung-Joon Kwon H-J, Choi G-E, Ryu S, Kwon SJ, Kim SC, Booth C, Nichols KE, Kim HS. Stepwise phosphorylation of p65 promotes NF-κB activation and NK cell responses during target cell recognition. Nature Communications 7:11686, 2016. doi:10.1038/ncomms11686

Guan P, Bassiri H, Patel NP, Nichols KE, Das R. Invariant natural killer T cells in hematopoietic stem cell transplantation: killer choice for natural suppression. Bone Marrow Transplant May;51(5):629-37, 2016. doi: 10.1038/bmt.2015.335. Epub 2016 Feb 15.

Alexander TB, McGee RB, Kaye EC, McCarville MB, Choi JK, Cavender CP, Nichols KE, Sandlund JT. Metachronous T-Lymphoblastic Lymphoma and Burkitt Lymphoma in a Child With Constitutional Mismatch Repair Deficiency Syndrome. Pediatr Blood Cancer Apr 1, 2016. doi:10.1002/pbc.25989. [Epub ahead of print]

Urtishak KA, Robinson BW, Rappaport EF, Sarezky MD, Biegel JA, Nichols KE, Stern JW, Felix CA.Unique Familial MLL-Rearranged Precursor B Cell Infant Acute Lymphoblastic Leukemia (ALL) in Non-twin Siblings. Pediatr Blood Cancer 2016 (in press).

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovic L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ; Paediatric Rheumatology International Trials Organisation, the Childhood Arthritis and Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group, and the Histiocyte Society. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis  Mar;75(3):481-9, 2016. doi: 10.1136/annrheumdis-2015-208982. PMID: 26865703

Das R, Guan P, Sprague L, Verbist K, Tedrick P, Qi A, Cheng C, Kurachi M, Levine RL, Wherry J, Canna S, Behrens EM, Nichols K. Janus kinase inhibition lessens inflammation and ameliorates disease in murine models of hemophagocytic lymphohistiocytosis. Blood Mar 31;127(13):1666-75, 2016. doi: 10.1182/blood-2015-12-684399. Epub 2016 Jan 29.

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovic A, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. RMD Open Jan 19;2(1):e000161, 2016. doi: 10.1136/rmdopen-2015-000161. eCollection 2016.

Ruffo E, Malacarne V, Larsen SE, Das R, Patrussi L, Wülfing C, Biskup C, Kapnick SM, Verbist K, Tedrick P, Schwartzberg PL, Baldari CT, Rubio I, Nichols KE, Snow AL, Baldanzi G, Graziani A. Inhibition of diacylglycerol kinase α restores restimulation-induced cell death and reduces immunopathology in XLP-1. Sci Transl Med Jan 13;8(321):321ra7, 2016. doi: 10.1126/scitranslmed.aad1565

Guan P, Bassiri H, Patel NP, Nichols KE, Das R. Invariant natural killer T cells in hematopoietic stem cell transplantation: killer choice for natural suppression. Bone Marrow Transplantation 2016 Feb 15. doi: 10.1038/bmt.2015.335. [Epub ahead of print] Review. PMID: 26878658

Zhang J*, Walsh MF*, Wu G*, Edmonson MN, Gruber TA, Easton J, Hedges D, Ma X, Zhou X, Yergeau DA, Wilkinson MR, Vadodaria B, Chen X, McGee RB, Hines-Dowell S, Nuccio R, Quinn E, Shurtleff SA, Rusch M, Patel A, Becksfort JB, Wang S, Weaver MS, Ding L, Mardis ER, Wilson RK, Gajjar A, Ellison DW, Pappo AS, Pui CH, Nichols KE*, Downing JR. Germline Mutations in Predisposition Genes in Pediatric Cancer. N Engl J Med Nov 18, 2015. Epub ahead of print. doi:10.1056/NEJMoa1508054. *Equal contribution

Moriyama T, Metzger ML, Wu G, Nishii R, Qian M, DevidasM, Yang W, Cheng C, Cao X, Quinn E, Raimondi S, Gastier-Foster J, Raetz E, Larsen E, Martin PL, Bowman WP, Winick N, Komada Y, Wang S, Edmonson M, Xu H,  Mardis E, Fulton R, Pui C-H, Mullighan C, Evans WE, Zhang J, Hunger SP, Relling MV, Nichols KE, Loh ML, Yang JJ. Germline Genetic Variation in ETV6 and Risk of Childhood Acute Lymphoblastic Leukemia: a Systematic Genetic Study. Lancet Oncol Oct. 27 2015. Epub ahead of print. doi:10.1016/S1470-2045(15)00369-1.

Broniscer A, Nichols KE. Predisposition to central nervous system cancers. In: Brain Tumors in Children. Gajjar A, Reaman GH, Racadio JM, Smith F, eds. New York, NY: Springer Press; 2015 (in press).

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovich L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnick-Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ, on behalf of the Pediatric Rheumatology International Trials Organization, the Childhood Arthritis & Rheumatology Research Alliance, the Pediatric Rheumatology Collaborative Study Group and the Histiocyte Society. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. RMD Open 1:e000161, 2015. doi:10.1136/ rmdopen-2015-000161

Lehmberg K, Sprekels B, Nichols KE, Woessmann W, Müller I, Suttorp M, Bernig T, Beutel K, Bode SFN, Kentouche K, Kolb R, Längler A, Minkov M, Schilling FH, Schmid I, Vieth S, Ehl S, zur Stadt U, Janka GE. Malignancy-associated Haemophagocytic Lymphohistiocytosis in Children and Adolescents. Haematologica 100(8):997-1004, 2015. doi: 10.3324/haematol.2015.123562.

Lehmberg K, Sprekels B, Nichols KE, Woessmann W, Müller I, Suttorp M, Beutel K, Bode S, Bielack S, Kentouche K, Kolb R, Körholz D, Längler A, Minkov M, Schmid I, Vieth S, Ehl S, zur Stadt U, Janka G. Hemophagotycic Lymphohistiocytosis in the context of malignancy in children and adults. Br J Haematology 170(4):539-49, 2015. doi: 10.1111/bjh.13462.

Anupindi SA, Bedoya MA, Lindell RB, Rambhatla SJ, Zelley K, Nichols KE, Chauvin NA. Diagnostic performance of whole body MRI as a tool for cancer screening in children with genetic cancer predisposing conditions. Am J Roentgenol 205(2):400-8, 2015. doi: 10.2214/AJR.14.13663

Quinn E, McGee R, Nuccio R, Pappo AS, Nichols KE. Genetic Predisposition to Neonatal Tumors. Curr Pediatr Rev 11(3):164-78, 2015.

Nichols KE, Malkin D. Genotype versus phenotype: the yin and yang of germline TP53 mutations in Li-Fraumeni Syndrome. J Clin Oncol 33(21):2331-3, 2015. doi: 10.1200/JCO.2015.61.5757.

Topka S, Vijai J, Walsh MF, Jacobs L, Maria A Villano D, Gaddam P, Wu G, McGee RB, Quinn E, Inaba H, Hartford C, Pui CH, Pappo AS, Edmonson M, Zhang M, Stepensky P, Steinherz P, Schrader K, Lincoln A, Bussel J, Lipkin S, Goldgur Y, Harit M, Stadler ZK, Mullighan C, Weintraub M, Shimamura A, Zhang J, Downing JR, Nichols KE*, Offit K*. Germline ETV6 mutations confer susceptibility to acute lymphoblastic leukemia and thrombocytopenia. PLOS Genetics Jun;11(6):e1005262, 2015. doi: 10.1371/journal.pgen.1005262. eCollection 2015 Jun.*Co-corresponding author.

Reichert SC, Zelley K, Nichols KE, Eberhard M, Zackai E, Martinez-Poyer J. Diagnosis of 9q22 microdeletion syndrome in utero following prenatal identification of craniosynostosis, overgrowth, and skeletal anomalies. Am J Med Genet, Part A 167(4):862-5, 2015.

Spessott WA, Sanmillan ML, McCormick M, Patel N, Villanueva J, Zhang K, Nichols KE*, Giraudo CG. Hemophagocytic lymphohistiocytosis caused by dominant negative mutations in STXBP2 that inhibit SNARE-mediated membrane fusion. Blood 125(10):1566-77, 2015.*Co-corresponding author.

Hedayat M, Al-Herz W, Aghamohammadi A, Nichols KE, Rezaei N. Primary Immunodeficiencies and cancers. In Cancer Immunology: Translational Medicine from Bench to Bedside, Nima Rezaei (Ed). Springer Press, 2014.

Nichols KE, Marsh RM. The X-linked lymphoproliferative syndromes. In Immunologic Disorders in Infants and Children. 6th Edition. Stiehm ER, Ochs HD, Winkelstein JA, eds. Elsevier Saunders Press, 2014.

Zelley K, Lindell RB, Schiffman JD, Nichols KE. Genetic predisposition to cancer. Nathan and Oski’s Hematology and Oncology of Childhood. 8th Edition. Orkin SH, Nathan DG, Ginsburg D, Look AT, Lux SE, eds. Elsevier Saunders Press, 2014.

Alderfer MA, Zelley K, Lindell RB, Novokmet A, Mai PL, Garber J, Nathan D, Scollon S, Chun N, Patenaude A, Ford J, Plon SE, Schiffman JD, Diller LR, Savage S, Malkin D, Ford CA, Nichols KE. Parent Decision Making around Genetic Testing of Children for Germline TP53 Mutations. Cancer 121(2):286-93. doi: 10.1002/cncr.29027.

Malkin D, Nichols KE, Zelley K, Schiffman JD. Predisposition to Pediatric and Hematologic Cancers: A Moving Target. In American Society of Clinical Oncology 2014 Educational Book. Dizon DS, Pennell N, Burke L, eds. American Society of Clinical Oncology, 2014. doi: 10.14694/EdBook_AM.2014.34.e44.

Menard L, Chamberlain N, Cantaert T, Tangye SG, Riminton S, Church JA, Klion A, Cunninham-Rundles C, Nichols KE, Meffre E. Signaling Lymphocytic Activation Moledule (SLAM)-associated protein (SAP) regulates human B cell tolerance. J Allergy Clin Immunol 133(4):1149-61, 2014.

Bassiri H, Das R, Guan P, Barrett DM, Brennan PJ, Banerjee PP, WienerSJ, Orange JS, Brenner MB, Grupp SA, Nichols KE. iNKT cell cytotoxic responses control T-lymphoma growth in vitro and in vivo. Cancer Immunol Res 2(1):59-69, 2014.

Chaigne-Delalande B, Li F-Y, O’Connor GM, Lukacs MJ, Jiang P, Zheng L, Biancalana M, Pittaluga S, Matthews HF, Jancel T, Hetherington M, Bleesing JJ, Marsh A, Kuijpers TW, Nichols KE, Lucas CL, Nagpal S, Mehmet H, Su HC, Cohen JI, Uzel G, Lenardo MJ. Mg2+ regulates cytotoxic functions of NK and CD8 T cells in chronic EBV infection. Science 341(6142):186-191, 2013.

Kalish JM, Conlin LK, Bhatti TR, Dubbs HA, Harris MC, Izumi K, Mostoufi-Moab S, Mulchandani S, Saitta S, States LJ, Swarr DT, WilkensAB, Zackai EH, Zelley K, Bartolomei MS, Nichols KE, Palladino AA, Spinner NB, Deardorff MA. Clinical features of three girls with mosaic genome wide paternal uniparental isodisomy. Am J Hum Genet 161(8):1929-1939, 2013.

Teachey DT, Rheingold SR, Maude SL, Barrett DM, Seif AE, Nichols KE, Suppa EK, Kalos M, Berg RA, Fitzgerald JC, Aplenc R, Gore L, Grupp SA. Cytokine release syndrome after blinatumomab treatment related to abnormal macrophage activation and ameliorated with cytokine directed therapy. Blood 121(26):5154-5157, 2013.

Chellapandian D, Das R, Zelley K, Wiener S, Zhao H, Teachey DT, Nichols KE. Treatment of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis with rituximab-containing immune-chemotherapeutic regimens. Br J Hematol 162(3):376-382, 2013.

May RM, Okumura M, Hsu C-J, Bassiri H, Rak G, Mace E, Baumgart T, Orange JS, Nichols KE, Kambayashi T. Two distinct signaling pathways contribute to the function of NK cells stimulated through ITAM-bearing activating receptors. Blood 121(16):3135-3146, 2013.

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Das R, Bassiri H, Guan P, Wiener S, Banerjee P, Zhong M-C, Veillette A, Orange JS, Nichols KE. The adaptor molecule SAP plays essential roles during invariant NKT cell cytotoxicity and lytic synapse formation. Blood 121(17):3386-3395, 2013.

Kalish JM, Conlin LK, Mostoufi-Moab S, Wilkens AB, Mulchandani S, Zelley K, Kowalski M, Bhatti TR, Russo P, Mattei P, Mackenzie WG, LiVolsi V, Nichols KE, Biegel JA, Spinner NB, Deardorff MA. Bilateral Pheochromocytomas, Hemihyperplasia, and Subtle Somatic Mosaicism: The Importance of Detecting Low-level Uniparental Disomy. Am J Med Genet 161(5):993-1001, 2013.

Hassan A, Booth C, Brightwell A, Allwood Z, Veys P, Rao K, Honig M, Friedrich W, Gennery A, Slatter M, Bredius R, Finocchi A, Cancrini C, Aiuti A, Porta F, Ridella M, Steward C, Filipovich A, Marsh R, Bordon V, Al-Muhsen S, Al-Mousa H, Alsum Z, Al-Dhekri H, Al Ghonaium A, Speckmann C, Fischer F, Mahlaoui N, Nichols KE, Grunebaum E, Al Zaharani D, Roifman CM, Boelens J, Davies EJ, Cavazzana-Calvo M, Notarangelo L, Gaspar HB. A study of the Inborn Errors Working Party of the Eur. Group for Blood and Marrow Transplantation and Eur. Soc. for Immunodeficiency. Outcome of Haematopoietic Stem Cell Transplantation for Adenosine Deaminase Deficient Severe Combined Immunodeficiency. Blood 120(7):3615-3624, 2012.

Palendira U, Low C, Bell AI, Ma CS, Abbott RJM, Phan TG, D Riminton S, Choo S, Smart JM, Lougaris V, Giliani S, Buckley RH, Grimbacher B, Alvaro F, Klion AD, Nichols KE, Adelstein S, Rickinson AB, Tangye SG. Expansion of somatically reverted memory CD8+ T cells in X-linked lymphoproliferative disease is due to selective pressure from Epstein Barr virus. J Exp Med 209(5):913-924, 2012.

Baldanzi G, Pighini A, Bettio V, Rainero E, Traini S, Chianale F, Porporato P, Filigheddu N, Mesturini R, Song S, Schweighoffer T, Patrussi L, Baldari CT, Zhong XP, van Blitterswijk WJ, Sinigaglia F, Nichols KE, Rubio I, Parolini O, Graziani A. SAP-mediated inhibition of diacylglycerol kinase alpha regulates TCR-induced diacylglycerol signalling. J Immunol 187(11):5941-5951, 2011.

Rothman JA, Das R, Teachey DT, Paessler ME, Nichols KE. Rapamycin Does Not Control Hemophagocytic Lymphohistiocytosis in LCMV-Infected Perforin-Deficient Mice. Pediatric Blood Cancer 57(7):1239-43, 2011.

Jyonouchi S, Abraham V, Orange JS, Spergel JM, Gober L, Dudek E, Saltzman R, Nichols KE*, Cianferoni A. Invariant natural killer T cells from food allergic versus non-allergic children exhibit differential responsiveness to milk derived sphingomyelin. J Allergy Clin Immunol 128(1):102-109, 2011. *Co-corresponding author.

Nallasamy S, Eagle RC, Rorke-Adams, Nichols KE, Brucker AH. Eye findings in X-linked lymphoproliferative disease. Retina 31(4):790-7, 2011.

Bunin GR, Felice MA, Davidson W, Friedman DL, Shields CL, Maidment A, O’Shea M, Nichols KE, Leahey A, Dunkel IJ, Jubran R, Rodriguez-Galindo C, Schmidt ML, Weinstein JL, Goldman S, Abramson DH, Wilson MW, Gallie BL, Chan HSL, Shapiro M, Cnaan A, Ganguly A, Meadows. Medical radiation exposure and risk of retinoblastoma resulting from new germline RB1 mutation. Int J Cancer 128(10):2393-404, 2011.

Booth C, Gilmour KC, Gennery AR, Slatter MA, Chapel H, Heath P, Steward C, Smith O, O’Meara A, Kerrigan H, Mahlaoui N, Cavazzana-Calvo M, Pachlopnick-Schmid J, Albert M, Notheis G, Reiber N, Strahm B, Ritterbusch H, Lankester A, Hartwig NG, Meyls I, Plebani A, Soresina A, Finocchi A, Pignata C, Broccoletti T, Bonanomi S, Peters C, Kalwak K, Pasic C, Sedlacek P, Jazbec J, Kanegane H, Terui K, Nichols KE, Hanson IC, Kapoor N, Haddad E, Cowan M, Choo S, Ip W, Arkwright P, Gaspar HB. X-linked Lymphoproliferative Disease due to SAP/SH2D1A deficiency: A Multicentre Study on the manifestations, management and outcome of the disease. Blood 117(1):53-62, 2011.

Shlien A,Baskin B, Isabel M, Achatz W, Dimitrios Stavropoulos J, Nichols KE, Hudgins L, Morel CF, Adam MP, Zhukova N, Rotin L, Novokmet A, Druker H, Shago M, Ray PN, Hainaut P, Malkin D. A common molecular mechanism underlies two phenotypically distinct 17p13.1 microdeletion syndromes. American Journal of Human Genetics 87(5):631-42, 2010.

Seif A, Biegel J, Lange BJ, Nichols KE. Inherited predisposition to childhood leukemia. In Childhood Leukemias. 3rd Edition. Pui CH, ed. Cambridge University Press, Cambridge England, 2010.

Kovalovsky D, Alonzo ES, Uhe OU, Eidson M, Nichols KE, Sant’Angelo DB. PLZF induces the spontaneious acquisition of memory/effector functions in T cells independently of Natural Killer T cell related signals. J Immunol 184(12):6746-6755, 2010.

Chen J-H, Fleming MD, Pinkus GS, Pinkus JL, Nichols KE, Jun MQ, Perez-Atayde AR. Histopathology of the liver in familial hemophagocytic lymphohistiocytosis. Am J Surg Pathol 34(6):852-867, 2010.

Alonzo E, Gottschalk R, Das J, Egawa T, Hobbs R, Pandolfi P, Pereira P, Nichols KE, Koretzky G, Jordan M, Sant'Angelo D. Development of Promyelocytic Zinc Finger and ThPOK-Expressing Innate γδ Innate T cells is controlled by strength of TCR signaling and Id3. Journal of Immunology184(3):1268-1279, 2010.

Lenox LE, Kambayashi T, Okamura M, Prieto C, Sauer K, Bunte R, Jordan MS, Koretzky GA, Nichols KE. Mutation of tyrosine 145 of lymphocyte cytosolic protein (Lcp; SLP-76) protects mice from anaphylaxis and arthritis. J Allergy Clin Immunol 124(5):1088-1098, 2009.

Snow AL, Marsh R, Krummey SM, Roehrs P, Young LR, Kejian Zhang, Jack van Hoff, Dhar D, Nichols KE, Filipovich AH, Su HC, Bleesing JJ, Lenardo MJ. SAP deficiency impairs NTB-A-dependent, restimulation-induced apoptosis of T cells from X-linked lymphoproliferative disease patients, J Clin Invest 119(10):2976-2984, 2009.

Talaat KR, Rothman JA, Cohen JI, Santi M, Rorke-Adams LB, Choi JK, Guzman M, Zimmerman R, Nallasamy S, Brucker A, Quezado M, Pittalugia S, Patronas NJ, Klion AD, Nichols KE. Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. Pediatr Blood Cancer 53(6):1120-1123, 2009.

Last update: June 2016