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Kim E. Nichols, MD

Attending Physician

Director, Pediatric Hereditary Cancer Predisposition Program

Associate professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania

Awards

2010, Foerderer Award, Children's Hospital of Philadelphia
2002, American Society of Hematology Junior Faculty Scholar Award 
2001, McCabe Foundation Award, University of Pennsylvania School of Medicine
2001, MAPS Award, Children's Hospital of Philadelphia
1999, ACS IRG, University of Pennsylvania School of Medicine
1999, Artemis Fellowship Award, Nikolas Symposium on the Histiocytosis
1998, Lee Fellowship Award, Dana-Farber Cancer Institute
1995, ACS Clinical Oncology Career Development Award (declined)
1995, Physician Scientist Award, National Institutes of Health
1989, Stead Research Fellowship, Duke University School of Medicine
1989, Alpha Omega Alpha, Duke University School of Medicine
1984, Summa Cum Laude, Dartmouth College
1984, Phi Beta Kappa, Dartmouth College
1980-84, Daniel Webster Scholar, Dartmouth College

Books

Chapters

2010

Seif A, Biegel J, Lange BJ, Nichols KE. Inherited predisposition to childhood leukemia. In: Childhood Leukemias. 3rd ed. New York: Cambridge University; in press.

2006

Nichols KE. Retinoblastoma. In: Leahey A, Meadows AT, eds. Requisites in Pediatrics: Hematology and Oncology. Philadelphia: Elsevier Health Sciences; 2006.

Goldsmith K. Hogarty M, Nichols KE. The childhood histiocytoses: Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis. In: Brodeur GM, Manno CS, editors. Requisites in Pediatrics: Hematology and Oncology. Philadelphia: Elsevier Health Sciences; 2006.

2001

Nichols KE, Tangye S. The X-linked lymphoproliferative disease gene, SH2D1A. In: Encyclopedia of Molecular Medicine. New York: John Wiley & Sons, Inc.; 2001.

Tangye SG, Nichols, KE. Function of the T-cell co-stimulatory molecule, SLAM. In: Principles of Molecular Medicine, New York:John Wiley & Sons, Inc.; 2001.

1998

Nichols KE, Haber DA. Molecular genetics of renal neoplasms: Wilms' tumor and renal cell carcinoma. In: Jameson JL. Principles of Molecular Medicine. Humana Press; 1998. 

Editorial and Academic Positions

Editorial positions

Associate Editor, Frontiers in Primary Immunodeficiency
Member, editorial board, Clinical Immunology

Ad hoc reviewer

Blood
British Journal of Hematology
Clinical Cancer Research
Experimental Immunology
EMBO
European Journal of Hematology
European Journal of Immunology
Haematologica
Human Mutation
International Journal of Cancer
Journal of Clinical Investigation
Journal of Clinical Oncology
Journal of Experimental Medicine
Journal of Immunology
Journal of Leukocyte Biology
Journal of Medical Genetics
Journal of Orthopedic Research
Journal of Hematology/Oncology
Journal of Pediatric Ophthalmology & Strabismus
Journal of Pediatrics
Medical and Pediatric Oncology
Molecular and Cellular Biology
Molecular Medicine
Nature
Nature Reviews Immunology
New England Journal of Medicine
Pediatric Blood and Cancer
Pediatric Infectious Disease Journal
Proceedings of the National Academy of Science

Academic positions

2011 Member, Search Committee for investigator in stem cell  transplantation
2009, Participant and Member, MD, PhD Admission Committee
2009, Member, Search Committee for Assistant Professor in Neurooncology

Leadership

Memberships in professional organizations

2011, American Society of Hematology
2009-present, Histiocyte Society, Macrophage Activation Subcommittee
2005-present, Histiocytosis Association of America
2004-present, Children's Oncology Group; Retinoblastoma Committee
1996-present, Histiocyte Society

Original Papers

2011

Rothman JA, Das R, Teachey DT, Paessler ME, Nichols KE. Rapamycin does not control hemophagocytic lymphohistiocytosis in LCMV-infected perforin-deficient mice. Pediatr Blood Cancer. Epub 2011 Jun 16. doi: 10.1002/pbc.23226. Cited in PubMed: PMID 21681935. Read the abstract

Rezaei N, Hedayat M, Aghamohammadi A, Nichols KE. Primary immunodeficiency diseases associated with increased susceptibility to viral infections and malignancies. J Allergy Clin Immunol. 2011 Jun;127(6):1329-41.e2;quiz 1342-3. Epub 2011 Apr 22. Cited in PubMed: PMID 21514636. Read the abstract

Jyonouchi S, Araham V, Orange JS, Spergel JM, Gober L, Dudek E, et al. Invariant natural killer T cells from children with versus without food allergy exhibit differential responsiveness to milk-derived sphingomyelin. J Allergy Clin Immunol. 2011 Jul;128(1):102-9.e.13. Epub 2011 Apr 1. Cited in PubMed: PMID 21458849. Read the abstract

Nallasamy S, Eagle RC Jr, Rorke-Adams L, Nichols KE, Brucker AJ. Eye findings in X-linked lymphoproliferative disorder. Retina. Epub 2011 Mar 9. Cited in PubMed: PMID 21394060. Read the abstract

Teplick A, Kowalski M, Biegel JA, Nichols KE. Screening in cancer predisposition syndromes: guidelines for the general pediatrician. Eur J Pediatr. Epub 2011 Jan 6. Cited in PubMed: PMID 21210147. Read the article

2010

Shlien A, Baskin B, Achatz MI, Stavropoulos DJ, Nichols KE, Hudgins L, et al.  A common molecular mechanism underlies two phenotypically distinct 17p13.1 microdeletion syndromes. Am J Hum Genet. 2010 Nov 12;87(5):631-42. Cited in PubMed: PMID 21056402. Read the abstract.

Das R, Sant'Angelo DB, Nichols KE. A common molecular mechanism underlies two phenotypically distinct 17p13.1 microdeletion syndromes. J Immunol Rev. 2010 Nov;238(1):195-215. Cited in PubMed: PMID 20969594. Read the article

Booth C, Gilmour KC, Veys P, Gennery AR,Slatter MA, Chapel H, et al. X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management, and outcome of the disease. Blood. 2010 Jan 6;117(1):53-62. Epub 2010 Oct 6. doi: 10.1182/blood-2010-06-284935. Cited in PubMed: PMID 20926771. Read the abstract

Bunin GR, Felice MA, Davidson W, Friedman DL, Shields CL, et al. Medical radiation exposure and risk of retinoblastoma resulting from new germline RB1 mutation. Int J Cancer. Epub 2010 Jul 20. Cited in PubMed: PMID 20648557. Read the abstract

Chen JH, Fleming MD, Pinkus GS, Pinkus JL, Nichols KE, Mo JQ, et al. Pathology of the liver in familial hemophagocytic lymphohistiocytosis. Am J Surg Pathol. 2010 Jun;34(6):852-67. Cited in PubMed: PMID 20442642. Read the abstract

Kovalovsky D, Alonzo ES, Uche OU, Eidson M, Nichols KE, Sant'Angelo DB. PLZF induces the spontaneous acquisition of memory/effector functions in T cells independently of NKT cell-related signals. J Immunol. 2010 Jun 15;184(12):6746-55. Epub 2010 May 21. Cited in PubMed: PMID 20495068. Read the article

Zekavat G, Mozaffari R, Arias VJ, Rostami SY, Badkerhanian A, Tenner AJ, et al. A novel CD93 polymorphism in non-obese diabetic (NOD) and NZB/W F1 mice is linked to a CD4+ iNKT cell deficient state. Immunogenetics. 2010 Jun;62(6):397-407. Epub 2010 Apr 13. Cited in PubMed: PMID 20387063. Read the abstract

Alonzo ES, Gottschalk RA, Das J, Egawa T, Hobbs RM, Pandolfi PP, et al. Development of promyelocytic zinc finger and ThPOK-expressing innate gamma delta T cells is controlled by strength of TCR signaling and Id3.  J Immunol. 2010 Feb 1;184(3):1268-79. Epub 2009 Dec 28. Cited in PubMed: PMID 20038637. Read the article

2009

Griffith LM, Cowan MJ, Notarangelo LD, Puck JM, Buckley RH, Candotti F, et al. Improving cellular therapy for primary immune deficiency diseases: recognition, diagnosis, and management. Journal of Allergy and Clinical Immunology, 2009; 124(6):1152-60. Cited in PubMed: PMID 20004776.  Read the article

Lenox LE, Kambayashi T, Okamura M, Prieto C, Sauer K, Bunte R, et al. Mutation of tyrosine 145 of lymphocyte cytosolic protein (Lcp; SLP-76) protects mice from anaphylaxis and arthritis. Journal of Allergy and Clinical Immunology, 2009; 124(5):1088-98. Cited in PubMed: PMID 19895996. Read the article

Snow AL, Marsh R, Krummey SM, Roehrs P, Young LR, Kejian Zhang, et al. SAP deficiency impairs NTB-A-dependent, restimulation-induced apoptosis of T cells from X-linked lymphoproliferative disease patients. Journal of Clinical Investigation, 2009; 119(10):2976-84. Cited in PubMed: PMID 19759517. Read the article

Talaat KR, Rothman JA, Cohen JI, Santi M, Rorke-Adams LB, Choi JK, et al. Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. Pediatric Blood & Cancer, 2009; 53(6):1120-23. Cited in PubMed: PMID 19621458. Read the abstract

Chou ST, Khandros E, Bailey C, Nichols KE, Vacok C, Yao Y, et al. Graded repression of PU.1/Sfpi1 gene function by GATA factors regulates hematopoietic cell fate. Blood, 2009 Jul 30;114(5):983-94. Cited in PubMed: PMID 19491391. Read the article

Ganguly A, Nichols KE, Grant G, Rappaport E, Shields C. Molecular karyotype of sporadic unilateral retinoblastoma tumors. Retina, 2009; 29(7):1002-12. Cited in PubMed: PMID  19491728. Read the article

Cen O, Guzman L, Jain J, Bassiri H, Nichols KE, Stain PL. The adaptor molecule SAP regulates IFN-gamma and IL-4 production in V alpha14 transgenic NK cells via effects on GATA-3 and T-bet expression. Journal of Immunology, 2009; 182(3):1370-78. Cited in PubMed: PMID 19155483.  Read the article

Posters and Presentations

2010

Nichols KE. Conditional elimination of signaling lymphocytic activation molecule-associated protein (SAP) reveals dichotomous roles in natural killer T cell functions [oral presentation]. Histiocyte Society, 26th Annual Meeting, Scientific Session 1, 2010 Oct. 18; Boston, MA.

Nichols KE. Genetic predisposition to cancer: management strategies [oral presentation]. Optum Healthcare Solutions, 2010 April 28; Scottsdale, AZ.

Nichols KE. X-linked lymphoproliferative disease: a model of impaired anti-viral and anti-tumor immunity [oral presentation]. The Children's Hospital of Philadelphia, 2010 July 28; Philadelphia, PA.

Nichols KE. Clinical case conference: Advances in the diagnosis and management of hemophagocytic lymphohistiocytosis [Grand Rounds]. The Children’s Hospital of Philadelphia, 2010 May 19; Philadelphia PA.

Nichols KE. HLH/XLP phenotypes: treatment & transplant [oral presentation]. First Clinical Immunology Society North American Primary Immunodeficiency Disease National Conference,  2010 May 23; Philadelphia, PA.

Nichols, KE. Regulation of NKT cell development and activation by SAP, the protein defective in XLP [oral presentation].  X-linked Lymphoproliferative Disease Symposium, Institute of Child Health, 2010 Feb 15; London, UK.

2009

Nichols KE. New insights into the pathogenesis and treatment of HLH [oral presentation]. Tumor Board, The Children’s Hospital of Philadelphia, 2009 Oct 15;
Philadelphia PA.

Nichols KE. Genetic predisposition to cancer: management strategies [oral presentation].  CHOP Spotlight Conference, Children’s Hospital of Philadelphia, 2009 Oct 9; Philadelphia PA .

Nichols KE. Pediatric cancer predisposition syndromes: recognition and management [oral presentation]. Pediatric Oncology Tumor Board, The Children’s Hospital of Philadelphia; 2009 Apr 30; Philadelphia, PA.

Nichols KE. Tumor surveillance in pediatric cancer predisposition syndromes [oral presentation]. 22nd Annual meeting of the American Society of Pediatric Hematology Oncology, Workshop on Pediatric Cancer Genetics for the General Oncologist; 2009 Apr 25; San Diego, CA.

Nichols KE. SAP, at the root of NKT cell development and activation [oral presentation]. Stokes Research Retreat; The Children’s Hospital of Philadelphia; 2009 Apr 17; Philadelphia, PA.

Nichols KE. N561: Case studies in the genetics of retinoblastoma [oral presentation]. University of Pennsylvania School of Nursing; 2009 Apr 6; Philadelphia, PA.

Nichols KE. N561: Molecular genetics of cancer [oral presentation]. University of Pennsylvania School of Nursing; 2009 Mar 30; Philadelphia, PA.

Nichols KE. Role of SAP in mature NKT cell functions [poster presentation]. 5th International NKT cell Workshop; 2009 Mar 24; Kamakura, Japan.

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