XYY syndrome: Difference between revisions - Wikipedia

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| caption = [[Karyotype]] from a male with 47,XYY

| field = [[Medical genetics]]

| symptoms = ~~Being~~None, being taller than ~~average~~the parents, Mildly unusual physical features, severe [[acne]], [[Developmental coordination disorder|poor coordination]], [[Hypotonia|weak muscle tone]], [[learning disability|learning]] and [[Speech disorder|speech problems]]<ref name=GHR2009/><ref name=NORD2012/>

| complications = ~~[[Autism spectrum disorder]], [[ADHD]]<ref name=GARD2017/>~~

| onset = At conception<ref>{{cite web | url=https://www.childrensdayton.org/kidshealth/a/xyy-syndrome | title=XYY Syndrome }}</ref>

~~| onset =~~

| duration = Lifelong

| types =

| causes = ~~[[Genetics]]~~Two Y chromosomes in males<ref name=NORD2012/>

| risks =

| diagnosis = [[~~Chromosomal~~Genetic ~~analysis~~testing]]<ref name=NORD2012/>

| differential = [[Klinefelter syndrome]], [[Marfan syndrome]], [[Sotos syndrome]]<ref name=NORD2012/>

| prevention = None<ref name=GARD2017/>

| treatment = [[Speech therapy]], [[tutoring]]<ref name=NORD2012/>

| medication =

| prognosis = ~~Good~~Normal life expectancy<ref name=NORD2012/>

| frequency = ~1 in 1,000 males<ref name=GHR2009/>

| deaths =

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Treatment may include [[speech therapy]] or extra help with schoolwork, however outcomes are generally positive.<ref name=NORD2012/> The condition occurs in about 1 in 1,000 male births.<ref name=GHR2009>{{Cite web|url=https://ghr.nlm.nih.gov/condition/47xyy-syndrome|title=47,XYY syndrome|website=Genetics Home Reference|language=en|access-date=2017-03-19|date=January 2009}}</ref> Many people with the condition are unaware that they have it.<ref name=GARD2017>{{cite web|title=47, XYY syndrome|url=https://rarediseases.info.nih.gov/diseases/5674/47-xyy-syndrome|website=Genetic and Rare Diseases Information Center (GARD)|access-date=11 November 2017|language=en|date=2017|archive-date=11 November 2017|archive-url=https://web.archive.org/web/20171111205247/https://rarediseases.info.nih.gov/diseases/5674/47-xyy-syndrome|url-status=dead}}</ref> The condition was first described in 1961.<ref>{{cite book|last1=Bostwick|first1=David G.|last2=Cheng|first2=Liang|title=Urologic Surgical Pathology E-Book|date=2014|publisher=Elsevier Health Sciences|isbn=~~9780323086196~~978-0-323-08619-6|page=682|url=https://books.google.com/books?id=wrHQAgAAQBAJ&pg=PA682|language=en}}</ref>

==Signs and symptoms==

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==Diagnosis==

47,XYY syndrome is not usually diagnosed until learning issues are present. The syndrome is diagnosed in an increasing number of children prenatally by [[amniocentesis]] and [[chorionic villus sampling]]<ref>{{Cite book|url=https://books.google.com/books?id=99YPDvFWBB0C&pg=PA91|title=NORD Guide to Rare Disorders|author=National Organization for Rare Disorders|date=2003|publisher=Lippincott Williams & Wilkins|isbn=~~9780781730631~~978-0-7817-3063-1|pages=91|language=en}}</ref> in order to obtain a chromosome karyotype, where the abnormality can be observed.

It is estimated that only 15–20% of children with 47,XYY syndrome are ever diagnosed. Of these, approximately 30% are diagnosed prenatally. For the rest of those diagnosed after birth, around half are diagnosed during childhood or adolescence after developmental delays are observed. The rest are diagnosed after any of a variety of symptoms, including fertility problems (5%)<ref>{{Cite book|url=https://books.google.com/books?id=rDAJ4G_8EIkC&pg=PA586|title=Psychopathology of Childhood and Adolescence: A Neuropsychological Approach|last=Davis|first=Andrew S. |date=2012-12-20|publisher=Springer Publishing Company|isbn=~~9780826109286~~978-0-8261-0928-6|pages=586|language=en}}</ref> have been seen.

==Epidemiology==

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In January 1968 and March 1968, ''[[The Lancet]]'' and ''[[Science (journal)|Science]]'' published the first U.S. reports of tall, institutionalized XYY males by Mary Telfer, a [[biochemist]], and colleagues at the [[Elwyn Inc.|Elwyn Institute]].<ref name="Telfer 1968a">{{cite journal |author1=Telfer, Mary A. |author2=Baker, David |author3=Longtin, Lucien |date=January 13, 1968 |title=YY syndrome in an American Negro |journal=[[The Lancet|Lancet]] |volume=291 |issue=7533 |page=95 |doi=10.1016/S0140-6736(68)90107-4 |pmid=4169701}}

* {{cite journal |author1=Telfer, Mary A. |author2=Baker, David |author3=Clark, Gerald R. |author4=Richardson, Claude E. |date=March 15, 1968 |title=Incidence of gross chromosomal errors among tall criminal American males |journal=[[Science (journal)|Science]] |volume=159 |issue=3820 |pages=1249–50 |doi=10.1126/science.159.3820.1249 |jstor=1723887 |pmid=5715587|bibcode=1968Sci...159.1249T |s2cid=27416349 ~~|url=https://semanticscholar.org/paper/03f33ad6030227c7d21cf574e0deab82e8b14327~~ }}</ref> Telfer found five tall, developmentally disabled XYY boys and men in hospitals and penal institutions in [[Pennsylvania]], and since four of the five had at least moderate facial [[acne vulgaris|acne]], reached the erroneous conclusion that acne was a defining characteristic of XYY males.<ref name="Telfer 1968a"/> After learning that convicted mass murderer [[Richard Speck]] had been karyotyped, Telfer not only incorrectly assumed the acne-scarred Speck was XYY, but reached the false conclusion that Speck was the [[archetype|archetypical]] XYY male—or "supermale" as Telfer referred to XYY males outside of peer-reviewed scientific journals.<ref>{{cite journal |author=Telfer, Mary A. |date=November–December 1968 |title=Are some criminals born that way? |journal=Think |volume=34 |issue=6 |pages=24–8 |issn=0040-6112}}{{blockquote|Why do men commit crimes of violence? For some, the urge to violence may be inborn—traced to something called the Y chromosome... Once in every 500 male births, for example, the sex chromosome complement is XXY rather than XY, thus erring in the direction of femaleness. The resulting individual, called a Klinefelter male, is usually retarded, unusually tall and sterile. Erring in the other direction, however, is the XYY complement resulting in the "supermale." He is also unusually tall and somewhat retarded, but appears to be highly, perhaps too highly, sexually motivated... We were intrigued by Dr. Jacobs' contention that an extra Y chromosome results in tall stature, mild mental retardation, and severely disordered personality characterized by violent, aggressive behavior. We therefore planned to confirm and extend her studies. ''Syndrome Status for the XYY'' The XXY male has long been thought to display a constellation of symptoms that makes him diagnosable; that is, he has achieved syndrome status. It would seem that the XYY male is fast achieving similar status. His symptoms, as we and other laboratories tend to think of them, are: extremely tall stature, long limbs and strikingly long arm span, facial acne, mild mental retardation, severe mental illness (including psychosis) and aggressive, antisocial behavior with a long history of arrests, frequently beginning at an early age. On reading newspaper accounts of Richard Speck, who murdered eight Chicago student nurses in 1966, we noted all these traits and therefore concluded that Speck was a likely candidate for the XYY disorder. Independently, a cytogenetic laboratory in Chicago confirmed this hunch, reinforcing our inclination to believe that the XYY syndrome is really coming of age. It seems quite possible that in the XYY male, exemplified by Speck, biologists are describing in genetic terms a certain type of defective criminal who has long been explicitly recognized by the forensic psychiatrist.}}</ref>

In April 1968, ''[[The New York Times]]''—using Telfer as a main source—introduced the XYY genetic condition to the general public in a three-part series on consecutive days that began with a Sunday front-page story about the planned use of the condition as a mitigating factor in two murder trials in [[Paris]]<ref name="Daniel Hugon">Daniel Hugon, Paris, France

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* {{cite news |date=October 10, 1968 |title=Extra chromosome brings acquittal on murder charge |newspaper=[[The New York Times]] |page=94 |url=https://www.nytimes.com/1968/10/10/archives/extra-chromosome-brings-an-acquittal-on-murder-charge.html }}

* {{cite news |author=Auerbach, Stuart |date=October 10, 1968 |title=Genetic abnormality is basis for acquittal |newspaper=[[The Washington Post]] |page=A1 |url=https://pqasb.pqarchiver.com/washingtonpost_historical/access/185091562.html?FMT=ABS&FMTS=ABS:AI |access-date=July 7, 2017 |archive-date=October 26, 2012 |archive-url=https://web.archive.org/web/20121026113916/http://pqasb.pqarchiver.com/washingtonpost_historical/access/185091562.html?FMT=ABS&FMTS=ABS:AI |url-status=dead }}

* {{cite news |author=Getze, George |date=February 3, 1969 |title=Australia precedent for XYY syndrome case held dubious |newspaper=[[Los Angeles Times]] |page=C1 |url=https://pqasb.pqarchiver.com/latimes/access/673351452.html?FMT=ABS&FMTS=ABS:AI ~~}}{{Dead~~|access-date=July 7, 2017 ~~link~~|archive-date=~~May~~January ~~2023~~31, 2013 |~~bot~~archive-url=https://archive.today/20130131173103/http://pqasb.pqarchiver.com/latimes/access/673351452.html?FMT=ABS&FMTS=~~InternetArchiveBot~~ABS:AI |~~fix~~url-~~attempted~~status=~~yes~~dead }} An Australian murder case that was reported to have been decided on the basis of the so-called XYY syndrome actually was not concerned with chromosome counts at all.</ref>—and falsely reported that Richard Speck was an XYY male and that the condition would be used in an appeal of his murder conviction.<ref name="Green 1985"/><ref name="Lyons 1968">{{cite news |author=Lyons, Richard D. |date=April 21, 1968 |title=Genetic abnormality is linked to crime; Genetics linked to violent crimes |newspaper=[[The New York Times]] |page=1 |url=https://www.nytimes.com/1968/04/21/archives/genetic-abnormality-is-linked-to-crime-genetics-linked-to-violent.html }}

* {{cite news |author=Lyons, Richard D. |date=April 22, 1968 |title=Ultimate Speck appeal may cite a genetic defect |newspaper=[[The New York Times]] |page=43 |url=https://www.nytimes.com/1968/04/22/archives/ultimate-speck-appeal-may-cite-a-genetic-defect.html }}

* {{cite news |author=Lyons, Richard D. |date=April 23, 1968 |title=Chromosome test for flaws costly; 2 scientists cite scarcity of skilled aides for analyses |newspaper=[[The New York Times]] |page=27 |url=https://www.nytimes.com/1968/04/23/archives/chromosome-test-for-flaws-costly-2-scientists-cite-scarcity-of.html }}

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* {{cite magazine |date=May 6, 1968 |title=Born bad? |magazine=[[Newsweek]] |volume=76 |issue=19 |pages=87}}</ref> and six months later in ''[[The New York Times Magazine]]''.<ref>{{cite news |author=Stock, Robert W. |date=October 20, 1968 |title=The XYY and the criminal |newspaper=[[The New York Times Magazine]] |page=SM30 |url=https://www.nytimes.com/1968/10/20/archives/the-xyy-and-the-criminal-the-xyy-and-the-criminal.html}}</ref>

In December 1968, the ''[[Journal of Medical Genetics]]'' published the first XYY review article—by Willam Michael Court Brown (~~1918 - 1969~~1918–1969),<ref>The first XYY review article, by W. Michael Court Brown, a physician and world authority on [[radiation biology]] and cytogenetics, was published in December 1968. Court Brown had established the MRC Clinical Effects of Radiation Unit at [[Western General Hospital]] in [[Edinburgh]] in 1956, which was renamed the MRC Clinical and Population Cytogenetics Unit in 1967 (and subsequently renamed the [[MRC Human Genetics Unit]] in 1988). In 1962, Court Brown had been the first to suggest—based on findings of antisocial behavior in some institutionalized [[Klinefelter syndrome]] (47,XXY) patients and psychosis in some institutionalized [[Triple X syndrome]] (47,XXX) patients—that individuals with sex chromosome abnormalities could be held in law to suffer from diminished responsibility.

* {{cite journal |last=Court Brown |first=W. Michael |date=September 8, 1962 |title=Sex chromosomes and the law |journal=[[The Lancet|Lancet]] |volume=280 |issue=7254 |pages=508–509 |doi=10.1016/S0140-6736(62)90378-1}}

* {{cite journal |date=January 4, 1969 |title=William Michael Court Brown |journal=[[The Lancet|Lancet]] |volume=293 |issue=7584 |pages=57–58 |doi=10.1016/S0140-6736(69)91029-0 |pmid=4178806}}

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* {{cite book |author=Borgaonkar, Diagamber S. |year=1978 |chapter=Cytogenic screening of community-dwelling males |pages=[https://archive.org/details/geneticissuesinp00cohe/page/215 215–234] |editor1=Cohen, Bernice H. |editor2=Lilienfield, Abraham M. |editor3=Huang, P. C. |title=Genetic issues in public health and medicine |location=Springfield, Ill. |publisher=Charles C Thomas |isbn=978-0-398-03659-1 |chapter-url=https://archive.org/details/geneticissuesinp00cohe/page/215 }}

* {{cite book |author=Washington, Harriet A. |year=2004 |chapter=Born for evil? Stereotyping the karyotype: A case history in the genetics of aggressiveness |editor1=Roelcke, Volker |editor2=Maio, Giovanni |title=Twentieth century ethics of human subjects research : historical perspectives on values, practices, and regulations |pages=319–334 |location=Stuttgart |publisher=[[Franz Steiner Verlag]] |isbn=978-3-515-08455-0}}

* {{cite book |author=Washington, Harriet A. |year=2006 |chapter=The children's crusade: research targets young African Americans |title=Medical apartheid : the dark history of experimentation on black Americans from colonial times to the present |pages=[https://archive.org/details/medicalapartheid00wash/page/279 279–283] |location=New York |publisher=[[Doubleday (publisher)|Doubleday]] |isbn=978-0-385-50993-0 |chapter-url=https://archive.org/details/medicalapartheid00wash/page/279 }}</ref> Concurrently, through 1974, psychologist [[John Money]] at Johns Hopkins Hospital experimented on thirteen XYY boys and men (ages 15 to 37) in an unsuccessful attempt to treat their history of behavior problems by [[chemical castration]] using high-dose [[Depo-Provera]]—with side-effects of weight gain (avg. 26 lbs.) and suicide.<ref name="Pyeritz 1977"/><ref>{{cite journal|last1=Blumer|first1=Dietrich|last2=Migeon|first2=Claude|date=February 1975|title=Hormone and hormonal agents in the treatment of aggression|journal=[[Journal of Nervous and Mental Disease|J Nerv Ment Dis]]|volume=160|issue=2|pages=127–137|pmid=123269|doi=10.1097/00005053-197502000-00007|s2cid=25125941}}

* {{cite journal |author1=Money, John |author-link1=John Money |author2=Wiedeking, Claus |author3=Walker, Paul |author4=Migeon, Claude |author5=Meyer, Walter |author6=Borgaonkar, Digamber |year=1975 |title=47,XYY and 46,XY males with antisocial and/or sex-offending behavior: antiandrogen therapy plus counseling |journal=Psychoneuroendocrinology |volume=1 |issue=2 |pages=165–176 |pmid=1234655 |doi=10.1016/0306-4530(75)90008-6|s2cid=41068306 }}—two other XYY boys (age 10) were deemed too young for chemical castration.

* {{cite journal |author1=Wiedeking, Claus |author2=Money, John |author3=Walker, Paul |date=May 1979 |title=Follow-up of 11 XYY males with impulsive and/or sex-offending behaviour |journal=[[Psychological Medicine|Psychol Med]] |volume=9 |issue=2 |pages=287–292 |pmid=472074 |doi=10.1017/S0033291700030786|s2cid=25174397 }}</ref>

In the late 1960s and early 1970s, screening of consecutive newborns for sex chromosome abnormalities was undertaken at seven centers worldwide: in [[Denver]] (Jan 1964–1974), [[Edinburgh]] (Apr 1967–Jun 1979), [[New Haven]] (Oct 1967–Sep 1968), [[Toronto]] (Oct 1967–Sep 1971), [[Aarhus]] (Oct 1969–Jan 1974, Oct 1980–Jan 1989), [[Winnipeg]] (Feb 1970–Sep 1973), and [[Boston]] (Apr 1970–Nov 1974).<ref name="March of Dimes">{{cite book |editor1=Robinson, Arthur |editor2=Lubs, Herbert A. |editor3=Bergsma, Daniel |year=1979 |title=Sex chromosome aneuploidy: prospective studies on children|series=Birth defects original article series '''15''' (1) |location=New York |publisher=[[John Wiley & Sons|Alan R. Liss]] |isbn=978-0-8451-1024-9}}

* {{cite book |editor=Stewart, Donald A. |year=1982 |title=Children with sex chromosome aneuploidy: follow-up studies |series=Birth defects original article series '''18''' (4) |location=New York |publisher=[[John Wiley & Sons|Alan R. Liss]] |isbn=978-0-8451-1052-2}}

* {{cite book |editor1=Ratcliffe, Shirley G. |editor2=Paul, Natalie |year=1986 |title=Prospective studies on children with sex chromosome aneuploidy |series=Birth defects original article series '''22''' (3) |location=New York |publisher=[[John Wiley & Sons|Alan R. Liss]] |isbn=978-0-8451-1062-1}}

* {{cite book |editor1=Evans, Jane A. |editor2=Hamerton, John L. |editor3=Robinson, Arthur |year=1991 |title=Children and young adults with sex chromosome aneuploidy: follow-up, clinical and molecular studies |series=Birth defects original article series '''26''' (4) |location=New York |publisher=[[John Wiley & Sons|Wiley-Liss]] |isbn=978-0-471-56846-9}}</ref> The Boston study, led by [[Harvard Medical School]] child psychiatrist Stanley Walzer at [[Children's Hospital Boston|Children's Hospital]], was unique among the seven newborn screening studies in that it only screened newborn ''boys'' (non-private-ward newborn boys at the [[Brigham and Women's Hospital|Boston Hospital for Women]]) and was funded in part by grants from the NIMH Center for Studies of Crime and Delinquency.<ref name="Boston">{{cite journal |author1=Beckwith, Jon |author2=King, Jonathan |date=November 1974 |title=The XYY syndrome: a dangerous myth |journal=[[New Scientist]] |volume=64 |issue=923 |pages=474–476 |pmid=11664346 |url=https://books.google.com/books?id=XAU4aZVrYv4C&pg=PA474 |access-date=2015-07-02 |archive-date=2023-01-11 |archive-url=https://web.archive.org/web/20230111211926/https://books.google.com/books?id=XAU4aZVrYv4C&pg=PA474 |url-status=dead }}

* {{cite news |author=Brody, Jane E. |date=November 15, 1974 |title=Scientists' group terms Boston study of children with extra sex chromosome unethical and harmful |newspaper=[[The New York Times]] |page=93 |url=https://www.nytimes.com/1974/11/15/archives/scientists-group-terms-boston-study-of-children-with-extra-sex.html }}

* {{cite news |author=Knox, Richard |date=November 16, 1974 |title=Scientists oppose survey of chromosome disorder |newspaper=[[The Boston Globe]] |page=3 |url=https://pqasb.pqarchiver.com/boston/access/1975367112.html?FMT=ABS&FMTS=ABS:AI |access-date=July 7, 2017 |archive-date=January 31, 2013 |archive-url=https://archive.today/20130131135410/http://pqasb.pqarchiver.com/boston/access/1975367112.html?FMT=ABS&FMTS=ABS:AI |url-status=dead }}

* {{cite journal |author=Culliton, Barbara J. |date=November 22, 1974 |title=Patients' rights: Harvard is site of battle over X and Y chromosomes |journal=[[Science (journal)|Science]] |volume=186 |issue=4165 |pages=715–717 |doi=10.1126/science.186.4165.715 |jstor=1739961 |pmid= 11643569|bibcode=1974Sci...186..715C }}

* {{cite news |author=Brody, Jane E. |date=December 14, 1974 |title=Harvard backs genetic study; research involving young with XYY chromosome had been criticized |newspaper=[[The New York Times]] |page=20 |url=https://www.nytimes.com/1974/12/14/archives/harvard-backs-genetic-study-research-involving-young-with-xyy.html }}

* {{cite news |author=Bruzelius, Nils |date=December 15, 1974 |title=Harvard Medical School panel rejects plea to end gene study |newspaper=[[The Boston Globe]] |page=8 |url=https://pqasb.pqarchiver.com/boston/access/2056634312.html?FMT=ABS&FMTS=ABS:AI ~~}}{{Dead~~|access-date=July 7, 2017 ~~link~~|archive-date=~~May~~January ~~2023~~31, 2013 |~~bot~~archive-url=https://archive.today/20130131215841/http://pqasb.pqarchiver.com/boston/access/2056634312.html?FMT=ABS&FMTS=~~InternetArchiveBot~~ABS:AI |~~fix~~url-~~attempted~~status=~~yes~~dead }}

* {{cite journal |author=Culliton, Barbara J. |date=December 27, 1974 |title=Briefing: Harvard faculty says XYY study should continue |journal=[[Science (journal)|Science]] |volume=186 |issue=4170 |pages=1188–1189 |doi=10.1126/science.186.4170.1189-b |jstor=1739250| pmid=17833922}}

* {{cite journal |author1=Beckwith, Jon |author2=Elseviers, Dirk |author3=Gorni, Luigi |author4=Mandansky, Chuck |author5=Csonka, Leslie |author6=King, Jonathan |date=January 31, 1975 |title=Harvard XYY study |journal=[[Science (journal)|Science]] |volume=187 |issue=4174 |doi=10.1126/science.11643259 |pmid=11643259 |pages=298–9|bibcode=1975Sci...187..298B }}

* {{cite news|date=March 15, 1975|title=Harvard vote backs child behavior study|newspaper=[[The Boston Globe]]|page=7|url=https://pqasb.pqarchiver.com/boston/access/1974822802.html?FMT=CITE&FMTS=CITE:AI|quote=The Harvard Medical School faculty voted last night 199–35, to allow continuation of studies at Children's Hospital Medical Center and at Boston Hospital for Women on children with an extra sex chromosome.~~}}{{Dead link~~|access-date=~~May~~July ~~2023~~7, 2017|~~bot~~archive-date=~~InternetArchiveBot~~January 31, 2013|~~fix~~archive-~~attempted~~url=~~yes~~ https://archive.today/20130131155148/http://pqasb.pqarchiver.com/boston/access/1974822802.html?FMT=CITE&FMTS=CITE:AI|url-status=dead}}

* {{cite news |author=Brody, Jane E. |date=June 20, 1975 |title=Babies' screening is ended in Boston; extra chromosome study stirred controversy on its ethics and value |newspaper=[[The New York Times]] |page=6 |url=https://www.nytimes.com/1975/06/20/archives/babies-screening-is-ended-in-boston-extra-chromosome-study-stirred.html |quote=Dr. Walzer said that when he stopped screening on the study's fifth anniversary in April,... }}

* {{cite journal |author=Culliton, Barbara J. |date=June 27, 1975 |title=XYY: Harvard researcher under fire stops newborn screening |journal=[[Science (journal)|Science]] |volume=188 |issue=4195 |pages=1284–1285 |doi=10.1126/science.11643276 |jstor=1741001 |pmid=11643276|bibcode=1975Sci...188.1284C }}

* {{cite news|author=Knox, Richard|date=July 6, 1975|title=XYY battle is over, research ethic war isn't|newspaper=[[The Boston Globe]]|page=A4|url=https://pqasb.pqarchiver.com/boston/access/2024063982.html?FMT=ABS&FMTS=ABS:AI|quote=In May...Walzer nevertheless decided to cut short his XYY screening by about eight months.|access-date=July 7, 2017|archive-date=January 31, 2013|archive-url=https://archive.today/20130131165145/http://pqasb.pqarchiver.com/boston/access/2024063982.html?FMT=ABS&FMTS=ABS:AI|url-status=dead}}

* {{cite news |author=Weiss, Philip |date=September 15, 1975 |title=Ending the test for extra chromosomes |newspaper=[[The Harvard Crimson]] |url=http://www.thecrimson.com/article/1975/9/15/ending-the-test-for-extra-chromosomes/ }}

* {{cite journal |last1=Bauer |first1=Diane |last2=Bayer |first2=Ronald |last3=Beckwith |first3=Jonathan |last4=Bermant |first4=Gordon |last5=Borgaonkar |first5=Digamber S. |last6=Callahan |first6=Daniel |last7=Caplan |first7=Arthur |last8=Conrad |first8=John |last9=Culver |first9=Charles M. |last10=Dworkin |first10=Gerald |last11=Edgar |first11=Harold |last12=Gaylin |first12=Willard |last13=Gerald |first13=Park |last14=Harris |first14=Clarence |last15=King |first15=Johnathan |last16=Macklin |first16=Ruth |last17=Mazur |first17=Allan |last18=Michels |first18=Robert |last19=Mone |first19=Carola |last20=Petchesky |first20=Rosalind |author20-link=Rosalind Petchesky |last21=Powledge |first21=Tabitha M. |last22=Pyeritz |first22=Reed E. |last23=Robinson |first23=Arthur |last24=Scanlon |first24=Thomas |last25=Shah |first25=Saleem A. |last26=Shannon |first26=Thomas A. |last27=Steinfels |first27=Margaret |last28=Swazey |first28=Judith P. |last29=Wachtel |first29=Paul |last30=Walzer |first30=Stanley |date=August 1980 |title=The XYY controversy: researching violence and genetics |journal=[[Hastings Center Report|Hastings Cent Rep]] |volume=10 |issue=4 |pages=Suppl 1–32 |pmid=7399889 |jstor=3560454 |quote=Stanley Walzer: On the issue of informed consent prior to newborn chromosome screening, Science for the People proved correct. They had predicted that the amount of information that had to be shared prior to screening was so extensive and complex that true informed consent was impossible to obtain ''at that point''. Arthur Robinson: When we stopped in 1974, we had done 40,000 analyses. From an epidemiological point of view, I would have preferred to have done 80,000... Why did we stop? Well, in the first place there was the informed consent problem. When we started in 1964 feelings about informed consent were very different. Try to imagine how one would go about getting truly informed consent from 40,000 people. With the implications of what we would find, in a sex chromosome variation, it would be an almost impossible task. That's one reason why we stopped.|doi=10.2307/3560454 }}</ref> The Edinburgh study was led by [[Shirley Ratcliffe]] who focused her career on it and published the results in 1999.<ref name=BMJobit>{{cite journal|last1=Sapietis|first1=Una|last2=Butler|first2=Gary|title=Obituary: Shirley Ratcliffe|journal=BMJ|date=21 July 2014|volume=349|page=g4716|doi=10.1136/bmj.g4716|s2cid=220108224}}</ref><ref name=WellcomeArchives>{{cite web|title=Archive record: Dr Shirley Ratcliffe and the Edinburgh MRC Clinical and Population Cytogenetics Unit Study of Long Term Outcomes for Children Born with Sex Chromosome Abnormalities|url=http://archives.wellcome.ac.uk/DServe/dserve.exe?dsqIni=Dserve.ini&dsqApp=Archive&dsqCmd=Show.tcl&dsqDb=Catalog&dsqSearch=(AltRefNo=%27pp/sra%27)&dsqPos=0|publisher=Wellcome Library Western Manuscripts and Archives catalogue|access-date=27 February 2018|language=en}}</ref>

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===1970s===

In December 1969, Lore Zech at the [[Karolinska Institutet|Karolinska Institute]] in [[Stockholm]] first reported intense [[fluorescence]] of the [[Adenine|A]] [[Thymine|T]]-rich distal half of the long arm of the Y chromosome in the nuclei of [[metaphase]] cells treated with [[quinacrine]] mustard.<ref name="Zech 1969">{{cite journal |author=Zech, Lore |date=December 1969 |title=Investigation of metaphase chromosomes with DNA-binding flurochromes |journal=[[Experimental Cell Research|Exp Cell Res]] |volume=58 |issue=2–3 |page=463 |doi = 10.1016/0014-4827(69)90531-X }}</ref> In April 1970, Peter Pearson and [[Martin Bobrow]] at the [[Medical Research Council (UK)|MRC]] Population Genetics Unit in [[Oxford]] and Canino Vosa at the [[University of Oxford]] reported fluorescent "male" sex chromatin bodies in the nuclei of interphase cells in buccal smears treated with quinacrine dihydrochloride, which could be used to screen for Y chromosome aneuploidies like 47,XYY.<ref name="Pearson 1970">{{cite journal |author1=Pearson, Peter L. |author2=Bobrow, Martin |author3=Vosa, Canio G. |date=April 4, 1970 |title=Technique for identifying ''Y'' chromosomes in human interphase nuclei |journal=[[Nature (journal)|Nature]] |volume=226 |issue=5240 |pages=78–80 |pmid=4190810 |doi=10.1038/226078a0|bibcode=1970Natur.226...78P |s2cid=4013867 }}

* {{cite news|author=Knox, Richard|date=August 14, 1970|title=Test may lead to choice in baby's sex|newspaper=[[The Boston Globe]]|page=26|url=https://pqasb.pqarchiver.com/boston/access/1946618622.html?FMT=ABS&FMTS=ABS:AI|access-date=July 7, 2017|archive-date=February 1, 2013|archive-url=https://archive.today/20130201075818/http://pqasb.pqarchiver.com/boston/access/1946618622.html?FMT=ABS&FMTS=ABS:AI|url-status=dead}}</ref>

In December 1970, at the annual meeting of the [[American Association for the Advancement of Science]] (AAAS), its retiring president, geneticist [[H. Bentley Glass]], cheered by the legalization of abortion in [[New York (state)|New York]],<ref>

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* {{cite journal |author=Glass, Bentley |date=April 9, 1971 |title=Reply to: Less than golden future |journal=[[Science (journal)|Science]] |volume=172 |issue=3979 |pages=111–112 |doi=10.1126/science.172.3979.111-c |jstor=1730891 |pmid=17735208}}

* {{cite journal |author=Glass, Bentley |date=July 9, 1971 |title=Reply to: What price the perfect baby? |journal=[[Science (journal)|Science]] |volume=173 |issue=3992 |pages=103–104 |jstor=1732191 |pmid=5104145 |doi=10.1126/science.173.3992.103-c }}</ref> Mischaracterization of the XYY genetic condition was quickly incorporated into high school biology textbooks<ref name="Pyeritz 1977"/><ref name="Modern Biology 1973">{{cite book |author1=Otto, James Howard |author2=Towle, Albert |year=1973 |title=Modern biology |location=New York |publisher=Holt, Rinehart and Winston |isbn=978-0-03-091337-2 |page=[https://archive.org/details/modernbiology00otto/page/185 185] |quote=Another abnormal condition results when a normal X-bearing egg is fertilized by a YY sperm, formed by non-disjunction during spermatogenesis. This produces an XYY male who is usually over six feet in height and very aggressive. |url=https://archive.org/details/modernbiology00otto/page/185 }}</ref> and medical school psychiatry textbooks,<ref name="Pyeritz 1977"/><ref name="Sadock Synopsis">{{cite book |author1=Freedman, Alfred M. |author2=Kaplan, Harold I. |author3=Sadock, Benjamin J. |year=1972 |title=Modern Synopsis of Comprehensive Textbook of Psychiatry |edition=1st |location=Baltimore |publisher=Williams & Wilkins |oclc=1232929 |page=711 |quote=Figure 43.2}}

* {{cite book |author1=Sadock, Benjamin James |author2=Sadock, Virginia Alcott |year=2007 |title=Kaplan and Sadock's Synopsis of Psychiatry : Behavioral Sciences/Clinical Psychiatry |edition=10th |location=Philadelphia |publisher=Lippincott Williams & Wilkins |isbn=978-0-7817-7327-0 |page=154 |quote=A famous case of an "XYY" insanity defense is illustrated in Figure 4.4–1. ~~[https://books.google.com/books?id=voJTE9z1ZXEC&pg=PA154 Figure 4.4–1]~~ Richard Speck. He was convicted in 1966 of slaying eight nurses in Chicago by stabbing and strangulation. His legal defense was based on his genetic makeup, which was "XYY". Individuals with these genes have been reported to be tall, mentally retarded, have acne, and show aggressive behavior... (Courtesy of Wide World Photos.)}}</ref> where misinformation still persists decades later.<ref name="Beckwith 2002"/>

In 1973, child psychiatrist Herbert Schreier at Children's Hospital told Harvard Medical School microbiologist [[Jon Beckwith]] of [[Science for the People]] that he thought Walzer's Boston XYY study was unethical; Science for the People investigated the study and, regarding the study, filed an ethics complaint with Harvard Medical School in March 1974.<ref name="Beckwith 2002"/> In November 1974, Science for the People went public with their objections to the Boston XYY study in a press conference and a ''[[New Scientist]]'' article alleging inadequate informed consent, a lack of benefit (since no specific treatment was available) but substantial risk (by stigmatization with a false stereotype) to the subjects, and that the unblinded experimental design could not produce meaningful results regarding the subjects' behavior.<ref name="Boston"/> In December 1974, the Harvard Standing Committee on Medical Research issued a report supporting the Boston XYY study, and in March 1975, the faculty voted 199–35 to allow continuation of the study.<ref name="Boston"/> After April 1975, screening of newborns was discontinued—changes to informed consent procedures and pressure from additional advocacy groups, including the [[Children's Defense Fund]], having led to the discontinuation of the last active U.S. newborn screening programs for sex chromosome abnormalities in Boston and Denver.<ref name="Boston"/>

In a paper published in ''[[New Scientist]]'' on November 14, 1974 entitled "XYY syndrome: a dangerous myth" found no link was found between having an extra Y chromosome and violent behavior. According to the paper, adolescents found to have an extra Y chromosome in a [[Maryland]] institution were [[chemical sterilization|chemically sterilized]] to attempt to maintain "normal behavior," despite this the paper found no major behavioral differences between XY and XYY individuals.<ref>{{Cite web|url=https://www.ojp.gov/ncjrs/virtual-library/abstracts/xyy-syndrome-dangerous-myth|title=XYY SYNDROME - A DANGEROUS MYTH | Office of Justice Programs|website=www.ojp.gov}}</ref>

In August 1976, ''Science'' published a [[retrospective cohort study]] by [[Educational Testing Service]] psychologist [[Herman Witkin]] and colleagues that screened the tallest 16% of men (over 184 cm (6'0") in height) born in [[Copenhagen]] from 1944 to 1947 for XXY and XYY karyotypes, and found an increased rate of minor criminal convictions for property crimes among sixteen XXY and twelve XYY men may be related to the lower intelligence of those with criminal convictions, but found no evidence that XXY or XYY men were inclined to be aggressive or violent.<ref name="Witkin 1976">{{cite journal |last1=Witkin |first1=Herman A. |last2=Mednick |first2=Sarnoff A. |last3=Schulsinger |first3=Fini |last4=Bakkestrøm |first4=Eskild |last5=Christiansen |first5=Karl O. |last6=Goodenough |first6=Donald R. |last7=Hirschhorn |first7=Kurt |last8=Lundsteen |first8=Claes |last9=Owen |first9=David R. |last10=Philip |first10=John |last11=Rubin |first11=Donald B. |last12=Stocking |first12=Martha |date=August 13, 1976 |title=Criminality in XYY and XXY men |journal=[[Science (journal)|Science]] |volume=193 |issue=4253 |pages=547–555 |doi=10.1126/science.959813 |jstor=1742747 |pmid=959813|bibcode=1976Sci...193..547W }}

* {{cite news |author=Knox, Richard |date=August 11, 1976 |title=Males with extra chromosomes not violence-prone, Danish study shows |newspaper=[[The Boston Globe]] |page=5 |url=https://pqasb.pqarchiver.com/boston/access/1976214812.html?FMT=ABS&FMTS=ABS:AI |access-date=July 7, 2017 |archive-date=January 31, 2013 |archive-url=https://archive.today/20130131200106/http://pqasb.pqarchiver.com/boston/access/1976214812.html?FMT=ABS&FMTS=ABS:AI |url-status=dead }}

* {{cite magazine |date=August 14, 1976 |title=XYY: No link to aggressive crime |magazine=[[Science News|Sci News]] |volume=110 |issue=7 |page=103 |jstor=3961261}}

* {{cite magazine |date=August 30, 1976 |title=Medicine: Capsules |magazine=[[Time (magazine)|Time]] |volume=108 |issue=9 |page=58 |url=http://www.time.com/time/magazine/article/0,9171,918265,00.html |access-date=February 8, 2012 |archive-date=August 14, 2013 |archive-url=https://web.archive.org/web/20130814015954/http://www.time.com/time/magazine/article/0,9171,918265,00.html |url-status=dead }}

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In other fictional television works, a January 1971 episode "By the Pricking of My Thumbs ..." of the British science fiction TV series ''[[Doomwatch]]'' featured an XYY boy expelled from school because his genetic condition led him to be falsely accused of nearly blinding another boy.<ref name="By the Pricking of My Thumbs ...">{{cite book |author1=Fulton, Roger |author2=Betancourt, John |year=1998 |chapter=Doomwatch |title=The Sci-Fi Channel encyclopedia of TV science fiction |location=New York |publisher=[[Hachette Book Group USA|Aspect]] |isbn=978-0-446-67478-2|page=170}} By the Pricking of My Thumbs ... written by Robin Chapman. Sixteen-year-old Stephen Franklin is expelled from school because, his father says, he has an obscure genetic defect—an extra "Y" chromosome.</ref> A November 1993 episode of the American police procedural TV series ''[[Law & Order]]'', "Born Bad", portrays a 14-year-old XYY sociopathic murderer.<ref name="Born Bad">{{cite news |author=Roush, Matt |date=November 17, 1993 |title=Critic's corner |newspaper=[[USA Today]] |page=12D |url=https://pqasb.pqarchiver.com/USAToday/access/55220486.html?FMT=ABS&FMTS=ABS:FT |access-date=July 7, 2017 |archive-date=October 26, 2012 |archive-url=https://web.archive.org/web/20121026113939/http://pqasb.pqarchiver.com/USAToday/access/55220486.html?FMT=ABS&FMTS=ABS:FT |url-status=dead }} One of TV's most consistently rewarding series takes a grim and unforgettable detour into the bleak mindset of a teen-age sociopathic murderer. Is society to blame, or as his lawyer argues, is he genetically predisposed to violence, with an extra "Y" chromosome? … the boy's hopeless future seems all too evident.</ref> The May 2007 season finale episode, "Born To Kill", of the American police procedural TV series ''[[CSI: Miami]]'' depicts a 34-year-old XYY [[serial killer]].<ref name="Born To Kill">{{cite journal |author=Hochman, David |date=May 7–13, 2007 |title=Horatio hunts a natural-born killer |journal=[[TV Guide]] |volume=55 |issue=19 |pages=34–36}} There's nothing funny about the season finale. That episode is about a serial killer with "criminal" genes. "It's a real-life natural-born killer situation", executive producer Ann Donahue says. "Usually girls have XX chromosomes and boys have XY, but this killer is XYY, which means too much testosterone." Among other niceties, the killer who has ties to Boston… brands his female victims with the letter Y.</ref>

The false stereotype of XYY boys and men as violent criminals has also been used as a [[plot device]] in the [[horror film]]s ''Il gatto a nove code'' in February 1971 (dubbed into English as ''[[The Cat o' Nine Tails]]'' in May 1971) and ''[[Alien 3]]'' in May 1992.<ref name="Green 1985"/><ref name="Beckwith 2002"/> The main character of the 2005 film ''[[Neo Ned]]'' is a [[neo-nazi]] who has an extra Y chromosome.<ref>{{cite AV media |people=dir. Van Fischer |date=2005 |title=[[Neo Ned]] |trans-title= |type= film|language=en |url= |access~~-date= |archive-url= |archive~~-date= |format= |time=10m00s|location= |publisher= |id= |isbn= |oclc= |quote= }}

</ref>

==See also==

* [[Sex chromosome anomalies]]

* [[Klinefelter syndrome]]

* [[XXYY syndrome]]

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[[Category:Sex chromosome aneuploidies]]

[[Category:Y chromosome]]

[[Category:Wikipedia medicine articles ready to translate]]

[[Category:Rare syndromes]]