Andrea Prader

Andrea Prader
Born	December 23, 1919 Samedan, Grisons
Died	June 3, 2001 (aged 81) Zurich
Nationality	Swiss
Alma mater	University of Zurich
Occupation	pediatric endocrinologist
Years active	1944–1986
Employer	Children's Hospital Zurich (Kinderspital, Zürich)
Known for	Prader-Willi syndrome; Andrea Prader Prize of European Society of Pediatric Endocrinologists
Home town	Zurich

Andrea Prader(December 23, 1919 - June 3, 2001) was a Swiss scientist, physician, and pediatric endocrinologist. He co-discovered the Prader-Willi syndrome and created two physiological sex development scales, the Prader scale and the Orchidometer.

Biography

Andrea Prader was born in Samedan, Grisons, Switzerland but lived in Zurich most of his life. He attended medical school at the University of Zurich. During his residency between 1944 and 1946, he worked under G. Toendury in the Department of Anatomy in Zurich, and then in 1947 in the Department of outpatient Medicine in Lausanne under A. Vannotti. In 1947 he became assistant physician at the Children's Hospital in Zürich. In 1950 was the beginning of his career as pediatric endocrinologist. His advanced research brought him to the U.S. in the early 1950s; Prader specialized at the Bellevue Hospital, New York City under the guidance of L.E. Holt, Jr, son of Luther Emmett Holt. His lasting interests in endocrinology was attributed to his contacts with Lawson Wilkins.^[1] Prader received his doctorate in 1957. In 1962 he became professor at the Medical faculty at the Zurich University. In 1965 the long term department chief Guido Fanconi retired and Prader succeeded him as professor and chairman of the Department of Pediatrics at the University of Zürich. He also took over the post of Director of the Children’s hospital (Kinderspital) and remained in these posts until 1986.^[2]

Studies

Andrea Prader's fundamental studies were in pediatric endocrinology, however in his earlier works there are other topics in the field of pediatrics. Here are some of his works and collaborations listed in chronological order:

• pediatric cardiology, with Ettore Rossi (1948)^[3]
• endocrine and metabolic disorders
• medical genetics (1951)
• pathophysiology of steroid hormones – including intersex-conditions and defects of the steroid synthesis (1953). He also devised the eponymous Prader scale, used to describe genital virilization.
• Prader-Gurtner syndrome, with HP Gurtner (1955)^[4]
• Prader-Labhart-Willi syndrome (1956)

Prader has been involved in the discovery or description of:

• lipoid congenital adrenal hyperplasia, with RE Siebenmann (1957)
• hereditary fructose intolerance, with R. Froesh, A. Labhart et al. (1957)
• pseudo-vitamin D deficiency with R. Illig et al. (1961)
• Adrenoleukodystrophy, (ALD). In 1963, Andrea Prader participated in a research effort of a collective of scientists ^[5] headed by Guido Fanconi that studied and described a condition related to chronic adrenal insufficiency combined with demyelinating disease of the brain and peripheral nervous system. They came up with diagnostic criteria, pathology and clinical picture of this heritable syndrome, described first by Siemerling and Creutzfeldt in a publication in 1923 of a clinical case they have encountered^[6] and the team named it originally after Thomas Addison ^[7] and Paul Ferdinand Schilder (Addison-Schilder's disease).^[8][9] Today the eponymous title recognizes the physicians who first described it (Siemerling and Creutzfeld). The public became familiar with one case of the disease in the movie "Lorenzo's oil." This is a primarily pediatric progressive metabolic disorder, demonstrating most commonly in boys between the ages of 5 and 15 and combining the characteristics of Addison’s disease and diffuse myelinoclastic sclerosis (Schilder's disease). Females are not affected by the condition, however due to linkage of heredity with the X chromosome, they are thought to be genetic carriers.^[10]

International acclaim

Between 1972 and 1974 he was member of honor and president of the Swiss Pediatric Society (Schweizerische Gesellschaft für Pädiatrie); he also held honorary memberships in the German Academy of Sciences Leopoldina (1968).^[11] He was Fellow of the Royal College of Physicians, London, received the Medal of the University of Helsinki, the Medal of the University of Turku, the Otto Naegeli Award, the Berthold medal of the German Society of Endocrinology, and was Doctor Honoris Causa of the Tokushima University, Japan. In 1962 and 1971 he was president of the European Society for Paediatric Endocrinology.

The Andrea Prader Prize

The Andrea Prader Prize is an annual Leadership Award, established in 1987 and given to a member of the European Society for Pediatric Endocrinology in recognition of their achievements in the field of pediatric endocrinology. At the establishment of the prize funds were provided by the Pharmacia & Upjohn, Stockholm. Currently the award is funded by Pfizer, USA.^[12]

External links

• "Who named it" - page dedicated to Andrea Prader
• The Andrea Prader Prize

References

1. Beighton, Peter; Beighton, Gretha (1986). The Man Behind the Syndrome. Berlin: Springer Verlag. ISBN 978-0-387-16218-8.
2. Wiedemann, HR (1984). "Andrea Prader: on the occasion of his 65th birthday". European Journal of Pediatrics. 148 (2): 80–1. doi:10.1007/BF00445789.
3. Rossi, Ettore; A. Prader (1948). "Die Angiokardiographie bei angeborenen Herzfehlern.". Schweizerische medizinische Wochenschrift. 78: 1054–64.
4. A. Prader, H. P. Gurtner:Das Syndrom des Pseudohermaphroditismus masculinus bei kongenitaler Nebennierenrindenhyperplasie ohne Androgenüberproduktion (adrenaler Pseudohermaphroditismus musculinus). Helvetica paediatrica acta, Basel, 1955, 10: 397–412.}
5. Guido Fanconi(pediatrician), Andrea Prader(pediatric endocrinologist), Werner Isler (pediatrician), Fritz Lüthy (neurologist), and Rudolf Siebenmann (pathologist).
6. Siemerling, E; H. G. Creutzfeldt (1923). "Bronzekrankheit und sklerosierende Encephalomyelitis (diffuse Sklerose)". Archiv für Psychiatrie. 68: 217–44.
7. Addison, T (1849). "Anaemia. Disease of the supra-renal capsules.". London Hospital Gazette. 43: 517–8.
8. Schilder, PF (1912). "Zur Kenntnis der sogenannten diffusen Sklerose (über Encephalitis periaxialis diffusa)". Zeitschrift für Neurologie, Berlin. 10 (1).
9. Scholz, W. (1925). "Klinische, pathologisch-anatomische und erbbiologische Untersuchungen bei familiärer, diffuser Hirnsklerose im Kindesalter (Ein Beitrag zur Lehre von den Heredodegenerationen).". Zeitschrift für Neurologie, Berlin. 99: 651–717.
10. Fanconi, G; A. Prader; W. Isler; F. Lüthy; R. Siebenmann (1963). "Morbus Addison mit Hirnsklerosse im Kindesalter. Ein hereditäres Syndrom mit X-chromosomaler Vererbung?". Helvetica paediatrica acta. 18: 480–501.
11. List of all members of Leopoldina, since the foundation of the Academy in 1652; Andrea Prader's name was found on page 105 of the pdf file. site visited on 12 May 2011