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John Menkes

John Hans Menkes
Born December 20, 1928
Vienna
Died November 22, 2008 (2008-11-23) (aged 79)
Los Angeles
Education University of Southern California (B.S., M.S. organic chemistry)
Johns Hopkins (M.D.)
Known for Identifying maple syrup urine disease, Menkes disease
Medical career
Profession Physician
Institutions Boston Children’s Hospital (intern)
Johns Hopkins
UCLA
Specialism Pediatric neurology
Research Genetic disorders
Notable prizes Drama-Logue Award, Hower Award (Child Neurology Society)

John Hans Menkes (December 20, 1928 – November 22, 2008) was a pediatric neurologist and author of fictional novels and plays. He identified two inherited diseases: maple syrup urine disease which is a defect in amino acid metabolism, and a defect in copper transport which bears his name. In addition to a career in academic medicine, he pursued a career in writing, publishing novels and plays.

Menkes was born in 1928 and his family fled Austria for Ireland days in advance of the start of World War II, then moved to California. Although he was interested in journalism, his father, a fourth-generation physician, convinced him to go into medicine. He earned a Bachelor's and master's degrees in organic chemistry from the University of Southern California.

After receiving his M.D. from Johns Hopkins in 1952, he was an intern at Boston Children’s Hospital. There, he encountered an unusual case. A child was showing signs of decline. The mother had two previous sons who had undergone the same changes and she had noted that their urine had a smell reminiscent of maple syrup, unlike their healthy sister. The child died after one week of life. Samples of urine were examined, but no specific compound had been identified before the samples were exhausted. Among techniques, the odor was compared, by smell, to an inventory of organic chemicals. Menkes, Peter Hurst (a resident), and John Craig (pathologist) published the account in a paper which would later be listed in the Science Citation Index "Citation Classics".

He went to Johns Hopkins for residency in psychology. The head of child neurology, Frank Ford, was aware of his paper and suggested that he should study neurology instead. He was drafted and served as a pediatrician at the Pepperrell Air Force Base in Newfoundland for the Northeast Air Command during the Korean War. Following his military service, he trained in pediatric neurology at Bellevue Hospital in New York City


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