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Maria New

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Maria Iandolo New (1928-2024) was a professor of Pediatrics, Genomics and Genetics at Icahn School of Medicine at Mount Sinai in New York City.[1] She is an expert in congenital adrenal hyperplasia (CAH), a genetic condition affecting the adrenal gland that can affect sexual development.[2]

Medical education

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New received her undergraduate degree from Cornell University in Ithaca, New York, in 1950, and her M. D. from the Perelman School of Medicine at the University of Pennsylvania in Philadelphia, in 1954. She completed an internship in medicine at Bellevue Hospital in New York, followed by a residency in pediatrics at the New York Hospital. From 1957 to 1958 she studied renal functioning under a fellowship from the National Institutes of Health (NIH). She was a research pediatrician to the Diabetic Study Group of the Comprehensive Care Teaching Program at the New York Hospital-Cornell Medical Center from 1958 to 1961, and had a second NIH fellowship under Ralph E. Peterson from 1961 to 1964, to study specific steroid hormone production during infancy, childhood and adolescence.[citation needed]

Career

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In 1964, New was appointed Chief of Pediatric Endocrinology at Cornell University Medical College, a position she held for 40 years. In 1978, she was named Harold and Percy Uris Professor of Pediatric Endocrinology and Metabolism. In 1980, New was appointed Chairman of the Department of Pediatrics at Cornell University Medical College and Pediatrician-in-Chief of the Department of Pediatrics at New York-Presbyterian Hospital. She was one of the few women in the country to serve as Chair of a major division of a medical college, and her tenure lasted for 22 years. While Chairman, New founded and directed the 8-bed Children's Clinical Research Center, a clinical research center in pediatrics with groundbreaking research in pediatric endocrinology, hematology, and immunology, during the emergence of AIDS. In 2004, New was recruited to the Mount Sinai School of Medicine as Professor of Pediatrics and Human Genetics and Director of the Adrenal Steroid Disorders Program.[3] She is also currently Adjunct Professor of Genetics at Columbia College of Physicians and Surgeons, and Associate Dean for Research at the Herbert Wertheim College of Medicine at Florida International University.

New is recognized as one of the world's leading pediatric endocrinologists.[3] Her career links clinical and basic science. She has continued her scientific research, including the use of molecular genetic diagnosis, prenatal diagnosis and treatment. Although steroid physiology was well understood when New began her scientific career, little of the knowledge had been applied to the understanding of steroid disorders in children. New's research on the mechanism and genetics of steroid disorders has established standards for pre- and post-natal care for patients with congenital adrenal hyperplasia and apparent mineralocorticoid excess.[citation needed]

During a 43-year period, New held the longest continuously funded National Institutes of Health grant, "Androgen Metabolism in Childhood", which supported research characterizing the diverse clinical spectra of patients with rare steroidogenic enzyme defects, such as congenital adrenal hyperplasia, and their metabolic consequences.[4]

Her current primary research emphasis is on genetic steroid disorders. New continues to study three monogenic disorders: 21-hydroxylase deficiency, 11β-hydroxylase deficiency, and apparent mineralocorticoid excess, emphasizing genotype/phenotype correlation and prenatal diagnosis and treatment. She has published more than 640 academic articles in a wide range of prestigious journals and published a genetics book entitled Genetic Steroid Disorders in 2014. She has also received numerous awards recognizing her work treating mothers and children affected with the disorder.[5]

Principal discoveries

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In 1977, New first described apparent mineralocorticoid excess (AME) in a Zuni girl.[6] Her team was the first to publish mutations on the 11β-hydroxysteroid dehydrogenase type 2 enzyme (encoded by the HSD11B2 gene) causing this potentially fatal form of low renin hypertension. New opened a new field of receptor biology by demonstrating the action of the 11β-HSD2 enzyme at the mineralocorticoid receptor of the distal renal tubule to metabolize cortisol to cortisone and thus protect the receptor. This was the first demonstration of the metabolism of a ligand to down-regulate its action on receptor activation.

In 1979, New described a form of mild steroid 21-hydroxylase deficiency called nonclassical 21-hydroxylase deficiency, which is characterized by diverse hyperandrogenic symptoms appearing postnatally in males and females.[7] The remarkable prevalence of 1 in 27 Ashkenazi Jews of a mild form of CAH was documented by New in 1985 and the genetic frequency of the mutation is 1 in 3 in the Ashkenazi Jewish population.[8] These studies established nonclassical 21-hydroxylase deficiency as the most frequent disorder of all autosomal recessive diseases in humans. While a spectrum of severity of congenital adrenal hyperplasia had always been observed, New was first to identify the mild form with specific molecular mutations.

Prenatal Dexamethasone

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Ethical issues have been raised about New's research. Namely, it has been questioned whether pregnant women undergoing treatment for the possible effects of congenital adrenal hyperplasia on their unborn babies were properly informed concerning the treatments that were administrated to them.[2] In September 2010 the FDA found nothing worth pursuing. In a 2012 article Alice Dreger followed up on the issues involved.[9] Further research and long term studies are needed to establish the correct usage guideline.

Awards and honors

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Elected Member of the US National Academy of Sciences, National Academy of Medicine, and the American Academy of Arts and Sciences

References

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  1. ^ "Maria I. New, Icahn School of Medicine at Mount Sinai".
  2. ^ a b "Medical Ethics: Prenatal Dexamethasone Use Questioned". Time. 18 June 2010.
  3. ^ a b Slate "If Your Baby Girl Might Be Born With a Small Penis" August 15, 2015 http://www.slate.com/articles/double_x/doublex/2012/08/intersex_babies_should_you_treat_their_condition_with_prenatal_drugs_.html
  4. ^ "The most comprehensive innovation search platform — Wellspring". www.collectiveip.com. Retrieved April 28, 2015.
  5. ^ "Keynote Speaker, 2008 Eastern-Atlantic Student Research Forum, University of Miami Miller School of Medicine". Archived from the original on 2014-03-10.
  6. ^ New, MI; Levine, LS; Biglieri, EG; Pareira, J; Ulick, S (May 1977). "Evidence for an unidentified steroid in a child with apparent mineralocorticoid hypertension". The Journal of Clinical Endocrinology and Metabolism. 44 (5): 924–33. doi:10.1210/jcem-44-5-924. PMID 870517.
  7. ^ New, MI; Lorenzen, F; Pang, S; Gunczler, P; Dupont, B; Levine, LS (Feb 1979). ""Acquired" adrenal hyperplasia with 21-hydroxylase deficiency is not the same genetic disorders as congenital adrenal hyperplasia". The Journal of Clinical Endocrinology and Metabolism. 48 (2): 356–9. doi:10.1210/jcem-48-2-356. PMID 218988.
  8. ^ Speiser, PW; Dupont, B; Rubinstein, P; Piazza, A; Kastelan, A; New, MI (Jul 1985). "High frequency of nonclassical steroid 21-hydroxylase deficiency". American Journal of Human Genetics. 37 (4): 650–67. PMC 1684620. PMID 9556656.
  9. ^ Dreger, A; Feder, E.K.; Tamar-Mattis, A (Jul 2012). "Prenatal Dexamethasone for Congenital Adrenal Hyperplasia: An Ethics Canary in the Modern Medical Mine". Journal of Bioethical Inquiry. 9 (3): 277–294. doi:10.1007/s11673-012-9384-9. PMC 3416978. PMID 22904609.
  10. ^ "Judson J. Van Wyk Prize".
  11. ^ "Fred Conrad Koch Award". Archived from the original on 2013-08-28.
  12. ^ "NICHD's First Grantees".
  13. ^ "Citation for the 1995 Rhône-Poulenc-Rorer Clinical Investigator Award to Maria I. New". Endocr Rev. 16 (4): 551–2. Aug 1995. PMID 8521796.
  14. ^ "Society for Endocrinology medallists" (PDF). Archived from the original (PDF) on 16 May 2013. Retrieved 27 August 2013.
  15. ^ "Robert H. Williams Distinguished Leadership Award". Archived from the original on 2013-08-28.
  16. ^ "Distinguished Graduate Award, Perelman School of Medicine, University of Pennsylvania". Archived from the original on 2015-03-02. Retrieved 2013-08-28.
  17. ^ "St. Geme Lectureship, 2010" (PDF). Archived from the original (PDF) on 4 March 2016. Retrieved 27 August 2013.
  18. ^ "Past presidents, Lawson Wilkins Pediatric Endocrine Society". Archived from the original on 2013-08-28. Retrieved 2013-08-28.
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