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Maria Iandolo New (December 11, 1928 - July 26, 2024 ) was a professor of Pediatrics, Genomics and Genetics at
Icahn School of Medicine at Mount Sinai The Icahn School of Medicine at Mount Sinai (ISMMS or Mount Sinai), formerly the Mount Sinai School of Medicine, is a private medical school in New York City, New York, United States. The school is the academic teaching arm of the Mount Sina ...
in
New York City New York, often called New York City (NYC), is the most populous city in the United States, located at the southern tip of New York State on one of the world's largest natural harbors. The city comprises five boroughs, each coextensive w ...
. She was an expert in
congenital adrenal hyperplasia Congenital adrenal hyperplasia (CAH) is a group of Genetic disorder#Autosomal recessive, autosomal recessive disorders characterized by impaired cortisol synthesis. It results from the deficiency of one of the five enzymes required for the Biosy ...
(CAH), a genetic condition affecting the
adrenal gland The adrenal glands (also known as suprarenal glands) are endocrine glands that produce a variety of hormones including adrenaline and the steroids aldosterone and cortisol. They are found above the kidneys. Each gland has an outer adrenal corte ...
that can affect sexual development.


Medical education

New received her undergraduate degree in chemistry with a minor in Latin from
Cornell University Cornell University is a Private university, private Ivy League research university based in Ithaca, New York, United States. The university was co-founded by American philanthropist Ezra Cornell and historian and educator Andrew Dickson W ...
in
Ithaca, New York Ithaca () is a city in and the county seat of Tompkins County, New York, United States. Situated on the southern shore of Cayuga Lake in the Finger Lakes region of New York (state), New York, Ithaca is the largest community in the Ithaca metrop ...
, in 1950, and her M. D. from the
Perelman School of Medicine at the University of Pennsylvania The Perelman School of Medicine (commonly known as Penn Med) is the medical school of the University of Pennsylvania, a Private university, private, Ivy League research university located in Philadelphia. Founded in 1765, the Perelman School of M ...
in Philadelphia, in 1954. She completed an internship in medicine at
Bellevue Hospital Bellevue Hospital (officially NYC Health + Hospitals/Bellevue and formerly known as Bellevue Hospital Center) is a hospital in New York City and the oldest public hospital in the United States. One of the largest hospitals in the United States ...
in New York, followed by a residency in pediatrics at the
New York Hospital Weill Cornell Medical Center (; previously known as New York Hospital, Old New York Hospital, and City Hospital) is a research hospital in New York City. It is the teaching hospital for Cornell University's medical school and is part of NewYork-P ...
. From 1957 to 1958 she studied renal functioning under a fellowship from the
National Institutes of Health The National Institutes of Health (NIH) is the primary agency of the United States government responsible for biomedical and public health research. It was founded in 1887 and is part of the United States Department of Health and Human Service ...
(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 The NewYork-Presbyterian Hospital (abbreviated as NYP) is a nonprofit academic medical center in New York City. It is the primary teaching hospital for Weill Cornell Medicine and Columbia University College of Physicians and Surgeons. The hospit ...
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.


Career

In 1964, New was appointed Chief of Pediatric Endocrinology at
Cornell University Medical College Weill Cornell Medicine (; officially Joan and Sanford I. Weill Medical College of Cornell University), originally Cornell University Medical College, is the medical school of Cornell University, located on the Upper East Side of Manhattan in Ne ...
, 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 The NewYork-Presbyterian Hospital (abbreviated as NYP) is a nonprofit academic medical center in New York City. It is the primary teaching hospital for Weill Cornell Medicine and Columbia University College of Physicians and Surgeons. The hospit ...
. 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 Hematology (American and British English spelling differences#ae and oe, spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to bloo ...
, and
immunology Immunology is a branch of biology and medicine that covers the study of Immune system, immune systems in all Organism, organisms. Immunology charts, measures, and contextualizes the Physiology, physiological functioning of the immune system in ...
, during the emergence of
AIDS The HIV, human immunodeficiency virus (HIV) is a retrovirus that attacks the immune system. Without treatment, it can lead to a spectrum of conditions including acquired immunodeficiency syndrome (AIDS). It is a Preventive healthcare, pr ...
. 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.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 She was also 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 Florida International University (FIU) is a public research university with its main campus in Westchester, Florida, United States. Founded in 1965 by the Florida Legislature, the school opened to students in 1972. FIU is the third-largest univ ...
. New is recognized as one of the world's leading pediatric endocrinologists. 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 Congenital adrenal hyperplasia (CAH) is a group of Genetic disorder#Autosomal recessive, autosomal recessive disorders characterized by impaired cortisol synthesis. It results from the deficiency of one of the five enzymes required for the Biosy ...
and apparent mineralocorticoid excess. During a 43-year period, New held the longest continuously funded
National Institutes of Health The National Institutes of Health (NIH) is the primary agency of the United States government responsible for biomedical and public health research. It was founded in 1887 and is part of the United States Department of Health and Human Service ...
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 Congenital adrenal hyperplasia (CAH) is a group of Genetic disorder#Autosomal recessive, autosomal recessive disorders characterized by impaired cortisol synthesis. It results from the deficiency of one of the five enzymes required for the Biosy ...
, and their metabolic consequences. Her later primary research emphasis was on genetic steroid disorders. New continued 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.


Principal discoveries

In 1977, New first described apparent mineralocorticoid excess (AME) in a Zuni girl. 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 The mineralocorticoid receptor (or MR, MLR, MCR), also known as the aldosterone receptor or nuclear receptor subfamily 3, group C, member 2, (NR3C2) is a protein that in humans is encoded by the ''NR3C2'' gene that is located on chromosome 4q31 ...
of the distal renal tubule to metabolize
cortisol Cortisol is a steroid hormone in the glucocorticoid class of hormones and a stress hormone. When used as medication, it is known as hydrocortisone. Cortisol is produced in many animals, mainly by the ''zona fasciculata'' of the adrenal corte ...
to
cortisone Cortisone is a pregnene (21-carbon) steroid hormone. It is a naturally-occurring corticosteroid metabolite that is also used as a pharmaceutical prodrug. Cortisol is converted by the action of the enzyme corticosteroid 11-beta-dehydrogenase ...
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. The remarkable prevalence of 1 in 27
Ashkenazi Ashkenazi Jews ( ; also known as Ashkenazic Jews or Ashkenazim) form a distinct subgroup of the Jewish diaspora, that Ethnogenesis, emerged in the Holy Roman Empire around the end of the first millennium Common era, CE. They traditionally spe ...
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. 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

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 Congenital adrenal hyperplasia (CAH) is a group of Genetic disorder#Autosomal recessive, autosomal recessive disorders characterized by impaired cortisol synthesis. It results from the deficiency of one of the five enzymes required for the Biosy ...
on their unborn babies were properly informed concerning the treatments that were administered to them. In September 2010 the FDA found nothing worth pursuing. In a 2012 article Alice Dreger followed up on the issues involved. Further research and long term studies are needed to establish the correct usage guideline.


Awards and honors

* Judson J. Van Wyk Prize, Lawson Wilkins Pediatric Endocrine Society (2010) * Fred Conrad Koch Award,
The Endocrine Society The Endocrine Society is a professional, international medical organization in the field of endocrinology and metabolism, founded in 1916 as The Association for the Study of Internal Secretions. The official name of the organization was changed t ...
(2003), the highest prize from the American Endocrine Society * MERIT Award,
National Institute of Child Health and Human Development The ''Eunice Kennedy Shriver'' National Institute of Child Health and Human Development (NICHD) is one of the National Institutes of Health (NIH) in the United States Department of Health and Human Services. It supports and conducts research ai ...
(1998) * Rhône-Poulenc Rorer Clinical Investigator Award,
The Endocrine Society The Endocrine Society is a professional, international medical organization in the field of endocrinology and metabolism, founded in 1916 as The Association for the Study of Internal Secretions. The official name of the organization was changed t ...
(1995) * Dale Medal, Society for Endocrinology (1995) * Robert H. Williams Distinguished Leadership Award,
The Endocrine Society The Endocrine Society is a professional, international medical organization in the field of endocrinology and metabolism, founded in 1916 as The Association for the Study of Internal Secretions. The official name of the organization was changed t ...
(1988), the highest award from the British Endocrine Society * University of Pennsylvania Distinguished Graduate Award * St. Geme Lectureship,
University of Colorado School of Medicine The University of Colorado School of Medicine is the medical school of the University of Colorado system. It is located at the Anschutz Medical Campus in Aurora, Colorado, Aurora, Colorado, one of the four University of Colorado campuses, six mil ...
(2010) * President, Lawson Wilkins Pediatric Endocrine Society (1985) Elected Member of the US
National Academy of Sciences The National Academy of Sciences (NAS) is a United States nonprofit, NGO, non-governmental organization. NAS is part of the National Academies of Sciences, Engineering, and Medicine, along with the National Academy of Engineering (NAE) and the ...
,
National Academy of Medicine The National Academy of Medicine (NAM), known as the Institute of Medicine (IoM) until 2015, is an American nonprofit, non-governmental organization. The National Academy of Medicine is a part of the National Academies of Sciences, Engineerin ...
, and the
American Academy of Arts and Sciences The American Academy of Arts and Sciences (The Academy) is one of the oldest learned societies in the United States. It was founded in 1780 during the American Revolution by John Adams, John Hancock, James Bowdoin, Andrew Oliver, and other ...


References

{{DEFAULTSORT:New, Maria American geneticists Cornell University alumni Cornell University faculty Perelman School of Medicine at the University of Pennsylvania alumni Intersex healthcare American pediatric endocrinologists American women geneticists American women endocrinologists 20th-century American scientists 20th-century American women scientists 21st-century American scientists 21st-century American women scientists Florida International University faculty American women academics Members of the National Academy of Medicine 2024 deaths 1928 births