Judith L. Ross, MD
Philadelphia, PA 19107
(215) 860-0408 fax
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BA with High Honors, Wellesley College - 1972
MD, University of Chicago, Pritzker School of Medicine - 1977
Children's Hospital of Philadelphia
National Institute of Health
Pediatric Endocrinology - 1983
American Academy of Pediatrics - 1982
Thomas Jefferson University Hospital
New Jersey and Pennsylvania
Estrogen and androgen appear to influence brain function in females at puberty. Environmental and cultural factors interact with the biological effects of estrogen and androgen on he brain an consequently on cognition and behavior. Women with Turner syndrome have dysgenetic ovaries that do not produce estrogen or androgen, before or at puberty. Therefore, Turner syndrome represents a unique, sex hormone-deficient, model in which to study the biological effects of androgen on cognition and behavior. The overall goal of this project is to study design we will study 1 two Turner syndrome groups: A androgen and B no androgen and 2 an age-matched, normal female control group. The specific aims of this project are to: 1 examine the effects of androgen on cognitive and social function in adolescent 11-13 years, growth hormone-treated girls with Turner syndrome, and 2 documented the differences and similarities in cognitive and behavioral function between adolescent Turner syndrome girls treated or not treated with androgen and age-andVIQ-girls treated with androgen, or not androgen-treated: 1 Turner syndrome girls treated with androgen versus no androgen will perform better on the tests of visual-spatial ability and visual-motor ability, 2 Turner syndrome girls treated with androgen versus no androgen will not perform differently on tests of attention, executive function, social function, and affective competence, 3 Turner girls treated with androgen for 2 years will demonstrate the greatest treatment effects, compared to girls treated for 1 year, and 4 androgen treatment will significantly reduce the differences between Turner syndrome and normal controls girls on tests executive function, social function, and affective competence. This investigation of adolescent cognitive and social development is an important step in understanding normal brain development. In addition, these data will determine how to optimize cognitive function in Turner syndrome, and will extend knowledge of the underlying mechanisms of sexual dimorphism.
Estrogen influences brain development in females at puberty. Environmental and cultural factors interact with the biological effects of estrogen on the brain and consequently on cognition and behavior. Women with Turner syndrome lack endogenous estrogen as a result of dysgenetic ovaries. Turner syndrome, therefore, represents a unique, estrogen- deficient model in which to study the biological effects of estrogen on cognition and behavior. The specific aims of this project are to: 1 to examine the differential effects of continuous estrogen replacement in early childhood on cognitive and social function in an ongoing, unique, randomized, double-blind, placebo-controlled, treatment trial. 2 document further, the cognitive differences between girls with Turner syndrome at ages 8 and 12 years versus age-matched, normal girls. Specifically, we hypothesize that estrogen replacement in early childhood will reduce the cognitive deficits of girls with Turner syndrome. In addition, we hypothesize that earlier age 5-7 years and longer estrogen replacement will result in less impairment of visual-spacial ability, visual-motorability, social function, and affective competence compared to later 9 to12 years estrogen replacement in girls with Turner syndrome. Finally, we hypothesize that the degree of social function in these girls will correlate with visual-spatial ability and facial recognition ability. The data generated from this carefully controlled biological investigation of cognitive and social development is an important stage in understanding normal brain development. In addition, these data will help determine how to optimize cognitive function in Turner syndrome, and will extend knowledge of the underlying mechanisms of sexual dimorphism.
OTHER APPOINTMENTS (1997-present)
1983-present Adjunct Scientist, National Institute of Child Health and Human Development.
Ad hoc reviewer for NIH study section: Human Development and Aging, Subcommittee 3;
Journal of Clinical Endocrinology and Metabolism, Clinical Pediatrics,Pediatric Research,Pediatrics,and Diabetes.
POSITIONS IN PROFESSIONAL SOCIETIES (1997-present)
1995 - present Board of Directors, Phila. Endocrine Society;
1996 - present Council, Eastern Society for Pediatric Research.
Ross, J.L. Symposium: Cognitive function in Turner syndrome: hormonal, genetic and learning disability perspectives, presented at The 25th Annual Meeting of the International Neuropsychological Society, Orlando, FL, February, 1997;
Ross, J.L. Symposium: Gonadal steroids and the brain, Estrogen effects on motor and neurocognitive development, presented at The Endocrine Society Meeting, Minneapolis, MN, June 14, 1997;
Roeltgen D., Ross J.L. Faster nonverbal processing speed in adolescent girls with Turner syndrome (TS) treated with estrogen, presented at the American Academy of Neurology Meeting, Minneapolis, MN, April 27, 1998;
Ross, J.L., Feuillan, P., Kowal, K., Guthrie, L., Bernstein, L., Troendle, J., and Cutler, G.B., Jr. Combined estrogen and growth hormone treatment increases bone density of the spine in girls with Turner syndrome, presented at The Endocrine Society Meeting, New Orleans, LA, June 27, 1998;
Ross, J.L., Roeltgen, D., Zinn, A. The hallmark cognitive deficit associated with Turner syndrome maps to the short arm of the X chromosome, presented at the 51st Annual Meeting of the American Academy of Neurology, Toronto, Canada, April 21, 1999;
Mazzocco, M.M.M., Abrams, M.T., Whitley, J.A., Ross, J.L., Kaufmann, W.E., Denckla, M.B. Specificity of the neurodevelopmental effects of Turner syndrome, Archives of Clinical Neuropsychology, National Academy of Neuropsychology, San Antonio, TX, 1999;
Ross, J.L., Kushner, H., Zinn, A. The Turner syndrome-associated neurocognitive phenotype maps to distal Xp, presented at the American Society of Human Genetics Meeting, San Francisco, CA, October 19-23, 1999;
Zinn, A.R., Prueitt, R.L., Papenhausen, P.R., Roberts, V.L., and Ross, J.L. Short stature and premature ovarian failure loci in proximal Xp, presented at the American Society of Human Genetics Meeting, San Francisco, CA, October 19-23, 1999;
Prueitt, R.L., Ross, J.L., and Zinn, A.R. Identification of a premature ovarian failure candidate gene, presented at the American Society of Human Genetics Meeting, San Francisco, CA, October 19-23, 1999;
Zinn, A.R., Osterman, A.L., Scott, C.I., Jr., Nicholson, L., Ross, J.L. Relationship of Madelung's deformity to Leri-Weill syndrome and ShOX mutations, presented at the American Society of Human Genetics Meeting, Philadelphia, PA, October 3-7, 2000;
Wei, F., Chheng, S., Elder, F.F.B., Scott, C.I., Jr., Nicholson, L., Ross, J.L., Zinn, A.R. A man who inherited SRY and Leri-Weill dyschondrosteosis from his mother and neurofibromatosis from his father, presented at the American Society of Human Genetics Meeting, Philadelphia, PA, October 3-7, 2000;
Ross, J.L., Russell, H.F., Power, T.J., Mazzocco, M.M., Zinn, A., Muenke, M. Evidence against imprinting effects on cognition in Turner syndrome, presented at the American Society of Human Genetics Meeting, Philadelphia, PA, October 3-7, 2000;
Roeltgen, D., Ross, J.L. Role of estrogen deficiency in cognitive function of women with premature ovarian failure, presented at the 53rd American Academy of Neurology Annual Meeting, Philadelphia, PA, May 5-11, 2001.
SPEAKING ENGAGEMENTS (1997-present)
Congenital adrenal hyperplasia and prococious puberty, Visiting Professor, Susquehanna Health System, Williamsport, PA, Jan 24, 1997;
Neonatal hyperthyroidism, Perinatal Conference, Lankanau Hospital, March 5, 1997;
Aspects of Turner syndrome, Conference of Philadelphia Turner syndrome Society, Lankanau Hospital, April 27, 1997;
Hypothyroidism: congenital and juvenile. Pediatric Grand Rounds, Bryn Mawr Hospital, May 2
Adolescence; Androgen; Attention; Child Psychology; Clinical Trial; Cognition; Cognition Disorder; Cognitive Behavior Therapy; Estrogen; Female; Hormone Therapy; Human Puberty; Human Subject; Human Therapy Evaluation; Intelligence Test; Interview; Longitudinal Human Study; Memory; Middle Childhood; Nervous System; Neurological Disorders; Neuropsychological Test; Oxandrolone; Placebo; Questionnaire; Self Concept; Social Behavior; Somatotropin; Space Perception; Stroke; Turner'S Syndrome; Verbal Learning; Visual Perception