638
PRESENTATION OF THE ACADEMY MEDAL TO MARIA I. NEW, M.D.* JULES HIRSCH, M.D. The Rockefeller University New York, New York
ON SEPTEMBER 12, 1990 ADMIRERS and former colleagues of William B. Castle met at the Memorial Church in Cambridge to celebrate his remarkable life. Charles Davidson, the William Bosworth Castle Professor of Medicine Emeritus, began with choice anecdotes recreating the special physicianly glow and scientific enthusiasm that were characteristic of William Castle. Davidson recalled the Boston City Hospital lunch room where there was interminable debate among house staff and fellows on the roles of clinical investigation, basic science, and even molecular science in medical education -the very same debate that still tears at the fabric of medical scholarship. Davidson quoted Castle's view of the matter as follows. "We should regard the study of the patient, including all aspects of his disease and of its relation to his physical and cultural environment, as the basic investigation appropriate for a physician." The study of disease by any tools that can bring understanding to the nature of the disease and finally to its treatment and prevention was Castle's view of the science of medicine. Few physicians are medical scientists as defined by Castle, but this evening it is my special privilege to introduce you to one of them. Dr. Maria I. New, this year's recipient of the Medal of the New York Academy of Medicine, is a sterling example of such a medical scientist. Dr. New received her undergraduate education at Cornell University in Ithaca and then attended the Medical School of the University of Pennsylvania. Her postgraduate years were spent in New York City at Bellevue and New York Hospitals, with interludes of laboratory investigations performed at the National Institutes of Health. She is now the chairman of the Department of Pediatrics at Cornell University Medical College and Pediatrician-inChief of The New York Hospital. She has made many contributions to pediatrics and to endocrinology, and her work has brought her international reputation as a leader in pediatric endocrinology. But it is for her work on congenital adrenal hyperplasia that *Presented at the Stated Meeting of the New York Academy of Medicine held May 30, 1991 Address for reprint requests: Rockefeller University, Receiving Department E 29273, 64th Street and 132 York Avenue, New York, NY 10021-6399
Bull. N.Y. Acad. Med.
MEDAL639 639 PRESENTATION OF ACADEMY MEDAL
PRESENTATION OF ACADEMY
MARIA I. NEW, M.D.
we wish to honor her this evening. Her work on this disorder spans the spectrum of medical scholarship from molecular genetics to epidemiology and sociology. Yet it always finds focus and meaningfulness in its application to human disease. Congenital adrenal hyperplasia is not an esoteric or unusual affliction; in fact, it may be the most common autosomal genetic defect in man. A major function of the adrenal gland is the conversion of cholesterol, the evil agent in atherogenesis, into sex steroids as well as mineralacorticoids and glucocorticoids. Like some highly dedicated and ingenious team of organic chemists, enzymes in the adrenal modify cholesterol progressively, in a series of highly specific, selective, chemical alterations. The end products of this chemical factory are of central importance in the differentiation of sexual organs as well as in the control of mineral metabolism, carbohydrate metabolVol. 67, No. 6, November-December 1991
640
J. HIRSCH
ism and growth and development. When any of the end products of the adrenal factory is in low supply, the endocrine apparatus calls for greater production. The blueprint for this entire operation and the structure of each of the protein enzymes is of course held in the genetic code. As we know, this blueprint can become blurred or misread, creating a genetic abnormality. The genetic disturbance Dr. New has studied is that form of congenital adrenal hyperplasia in which one step, 21-hydroxylase is deficient. When this occurs, the adrenal can no longer produce adequate amounts of glucocorticoids, hence other end products of the adrenal factory pile up as the endocrine apparatus calls for more production. This leads to a failure of sexual development with virilization, a failure of growth and development and inevitably a ruined life. Early recognition of this disease and simple treatment with exogenous glucocorticoids leads to a reversal of the process. Dr. New has been committed to the study of this unfortunate disease. Over the course of many years she has made careful observations of the development of the disease in children. She assembled a team of investigators who worked with her to demonstrate that the genetic defect occurred in a specific region of chromosome 6 and then showed that non-classical or lesser forms of this deficiency occur with remarkable frequency. She has therefore been responsible for unravelling the full genotypic, biochemical, and phenotypic manifestations of an important, prevalent disorder. She has thereby enriched medical science while maintaining her career as an administrator, teacher, and clinician. She is also a caring mother, a warm colleague, a concerned citizen, but above all a living proof that there is still such a thing as medical science. She is a distinguished practitioner of that science at the bedside and at the laboratory bench. These remarkable achievements make her a most worthy recipient of the Medal of the New York Academy of Medicine for 1991.
Bull. N.Y. Acad. Med.
PRESENTATION OF THE ACADEMY MEDAL TO MARIA I. NEW, M.D.* JULES HIRSCH, M.D. The Rockefeller University New York, New York
ON SEPTEMBER 12, 1990 ADMIRERS and former colleagues of William B. Castle met at the Memorial Church in Cambridge to celebrate his remarkable life. Charles Davidson, the William Bosworth Castle Professor of Medicine Emeritus, began with choice anecdotes recreating the special physicianly glow and scientific enthusiasm that were characteristic of William Castle. Davidson recalled the Boston City Hospital lunch room where there was interminable debate among house staff and fellows on the roles of clinical investigation, basic science, and even molecular science in medical education -the very same debate that still tears at the fabric of medical scholarship. Davidson quoted Castle's view of the matter as follows. "We should regard the study of the patient, including all aspects of his disease and of its relation to his physical and cultural environment, as the basic investigation appropriate for a physician." The study of disease by any tools that can bring understanding to the nature of the disease and finally to its treatment and prevention was Castle's view of the science of medicine. Few physicians are medical scientists as defined by Castle, but this evening it is my special privilege to introduce you to one of them. Dr. Maria I. New, this year's recipient of the Medal of the New York Academy of Medicine, is a sterling example of such a medical scientist. Dr. New received her undergraduate education at Cornell University in Ithaca and then attended the Medical School of the University of Pennsylvania. Her postgraduate years were spent in New York City at Bellevue and New York Hospitals, with interludes of laboratory investigations performed at the National Institutes of Health. She is now the chairman of the Department of Pediatrics at Cornell University Medical College and Pediatrician-inChief of The New York Hospital. She has made many contributions to pediatrics and to endocrinology, and her work has brought her international reputation as a leader in pediatric endocrinology. But it is for her work on congenital adrenal hyperplasia that *Presented at the Stated Meeting of the New York Academy of Medicine held May 30, 1991 Address for reprint requests: Rockefeller University, Receiving Department E 29273, 64th Street and 132 York Avenue, New York, NY 10021-6399
Bull. N.Y. Acad. Med.
MEDAL639 639 PRESENTATION OF ACADEMY MEDAL
PRESENTATION OF ACADEMY
MARIA I. NEW, M.D.
we wish to honor her this evening. Her work on this disorder spans the spectrum of medical scholarship from molecular genetics to epidemiology and sociology. Yet it always finds focus and meaningfulness in its application to human disease. Congenital adrenal hyperplasia is not an esoteric or unusual affliction; in fact, it may be the most common autosomal genetic defect in man. A major function of the adrenal gland is the conversion of cholesterol, the evil agent in atherogenesis, into sex steroids as well as mineralacorticoids and glucocorticoids. Like some highly dedicated and ingenious team of organic chemists, enzymes in the adrenal modify cholesterol progressively, in a series of highly specific, selective, chemical alterations. The end products of this chemical factory are of central importance in the differentiation of sexual organs as well as in the control of mineral metabolism, carbohydrate metabolVol. 67, No. 6, November-December 1991
640
J. HIRSCH
ism and growth and development. When any of the end products of the adrenal factory is in low supply, the endocrine apparatus calls for greater production. The blueprint for this entire operation and the structure of each of the protein enzymes is of course held in the genetic code. As we know, this blueprint can become blurred or misread, creating a genetic abnormality. The genetic disturbance Dr. New has studied is that form of congenital adrenal hyperplasia in which one step, 21-hydroxylase is deficient. When this occurs, the adrenal can no longer produce adequate amounts of glucocorticoids, hence other end products of the adrenal factory pile up as the endocrine apparatus calls for more production. This leads to a failure of sexual development with virilization, a failure of growth and development and inevitably a ruined life. Early recognition of this disease and simple treatment with exogenous glucocorticoids leads to a reversal of the process. Dr. New has been committed to the study of this unfortunate disease. Over the course of many years she has made careful observations of the development of the disease in children. She assembled a team of investigators who worked with her to demonstrate that the genetic defect occurred in a specific region of chromosome 6 and then showed that non-classical or lesser forms of this deficiency occur with remarkable frequency. She has therefore been responsible for unravelling the full genotypic, biochemical, and phenotypic manifestations of an important, prevalent disorder. She has thereby enriched medical science while maintaining her career as an administrator, teacher, and clinician. She is also a caring mother, a warm colleague, a concerned citizen, but above all a living proof that there is still such a thing as medical science. She is a distinguished practitioner of that science at the bedside and at the laboratory bench. These remarkable achievements make her a most worthy recipient of the Medal of the New York Academy of Medicine for 1991.
Bull. N.Y. Acad. Med.
