John Menkes

John Hans Menkes (December 20, 1928 – November 22, 2008) was an Austrian-American 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.

John Hans Menkes
BornDecember 20, 1928
Vienna
DiedNovember 22, 2008 (2008-11-23) (aged 79)
Los Angeles
EducationUniversity of Southern California (B.S., M.S. organic chemistry)
Johns Hopkins (M.D.)
Known forIdentifying maple syrup urine disease, Menkes disease
Medical career
ProfessionPhysician
InstitutionsBoston Children's Hospital (intern)
Johns Hopkins
UCLA
Sub-specialtiesPediatric neurology
ResearchGenetic disorders
AwardsDrama-Logue Award, Hower Award (Child Neurology Society)

Early life

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 degree and a master's degree in organic chemistry from the University of Southern California.[1]

Medical training

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 that was later listed in the Science Citation Index "Citation Classics".[2]

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 [3]

In 1957, as a pediatric neurology fellow (with a research budget of $35), he encountered another case of maple syrup urine disease and was able to identify branched chain keto acids in the urine samples.[2] During his fellowship, he also encountered a boy who was born healthy, but developed progressive muscle weakness (hypotonia) and seizures. The child had the characteristic finding of brittle hair. He published the accounts of five cases,[4] and the metabolic defect was later identified as an X-linked recessive error in copper transport.[5]

Medical practice and writing

Menkes returned to Johns Hopkins in 1960, and became chief of pediatric Neurology in 1964. In 1966, he went to UCLA where he established their division of child neurology. In 1974, the first edition of Textbook of Child Neurology was published and Menkes served as editor for six additional editions. That year went into private practice. He also dedicated time to writing, describing Anton Chekhov as his "idol and guiding light" for his ability to maintain his medical and writing careers.[6]

In his 1986 play, The Last Inquisitor about the last Gestapo head, Ernst Kaltenbrunner, he dealt with his own relationship to The Holocaust. The Los Angeles Times found it interesting, especially in the way he took down the fourth wall and had a character play who becomes an actor who is consumed by the role of Adolf Eichmann, but found that it was more of a cerebral than emotional experience.[7] He revisited the theme, asking how things might have been had he been born Christian instead of Jewish in Vienna, in his 2003 novel, After the Tempest.[6][8]

He became a plaintiffs' expert witness in injury cases related to the pertussis vaccine[1] and was appointed to the National Institute of Medicine's Forum for Vaccine Safety.[5] He wrote about his experiences in the product liability debate in the form of a fictional novel, The Angry Puppet Syndrome (1999).[6][9]

His 2001 comedy, Lady Macbeth Gets a Divorce, received similarly mixed reviews from Variety which called it "more schematic than compelling".[10]

Death

Menkes died from complications of cancer, November 22, 2008 at Cedars-Sinai Medical Center.[11]

Awards and honors
  • Hower Award, Child Neurology Society, 1980
  • Drama-Logue Award, "The Last Inquisitor", 1986
gollark: It's at the view limit.
gollark: Which one?
gollark: Sure!
gollark: TJ's are magically sickness-proofed.
gollark: Hmm... I wonder if the cave supports IPv6, and counts each IPv6 address viewing it as a separate unique view.

References
  1. Snodgrass, S. Robert (June 2009), "John Menkes (1928-2008)" (PDF), World Neurology, 30 (3), p. 14
  2. Menkes J H, Hurst P L & Craig J M. (May 14, 1979) [1954]. "This Week's Citation Classic: A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance" (PDF). Pediatrics (Science Citation Index). 14 (5): 462–6. PMID 13214961.CS1 maint: uses authors parameter (link)
  3. "Dr. Menkes was expert on inherited metabolic diseases". AAP News. 30 (2): 25. February 1, 2009. doi:10.1542/aapnews.2009302-25 (inactive 2020-01-25). Retrieved 2019-08-20.
  4. J. H. Menkes; M. Alter; G. K. Steigleder; D. R. Weakley; J. H. Sung (May 1962). "A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration". Pediatrics. 29: 764–779. PMID 14472668.
  5. Stump, Elizabeth (2009). "Distinguished Pediatric Neurologist John H. Menkes, MD, Dies at 79". Neurology Today. 9 (1): 3–4. doi:10.1097/01.NT.0000345151.52907.f4. ISSN 1533-7006.
  6. Dawn, Antoline (December 2003), "Up close and personal with John H. Menkes, MD: pediatric neurologist, novelist, and playwright", Neurology Today, 3 (12): 34–5, doi:10.1097/00132985-200312000-00011
  7. Loynd, Ray (November 7, 1986). "'Inquisitor': Intellectual Exercise". Los Angeles Times. Retrieved 2015-08-09.
  8. Menkes, John H. (2003). After the Tempest: A Novel. Fithian Press. ISBN 978-1-56474-420-3.
  9. Menkes, John H. (1999). The Angry Puppet Syndrome. Demos Medical Publishing, LLC. ISBN 978-1-888799-27-9.
  10. Linden, Sheri (March 31, 2001), "Review: 'Lady Macbeth Gets a Divorce'", Variety, retrieved 2015-08-09
  11. Maugh II, Thomas H. (November 29, 2008). "John H. Menkes dies at 79; doctor established UCLA's pediatric neurology program". Los Angeles Times. Retrieved 2015-08-09.
  • Portrait in "Images from the history of medicine", National Library of Medicine
  • Interview with John Menkes, Connie Martinson Talks Books (October 23, 2003) from the Claremont Colleges Digital Library.
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