Diprosopus: Difference between revisions
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{{Short description|Medical disorder}} |
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[[File:A stuffed chick with two beaks and three eyes.jpg|thumb|A chick with two beaks and three eyes]] |
[[File:A stuffed chick with two beaks and three eyes.jpg|thumb|A chick with two beaks and three eyes]] |
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'''Diprosopus''' ( |
'''Diprosopus''' ({{langx|el|διπρόσωπος}}, "two-faced", from {{lang|el|δι-}}, {{transl|el|di-}}, "two" and {{lang|el|πρόσωπον}}, {{transl|el|prósopon}} [neuter], "face", "person"; with [[Latin]] ending), also known as '''craniofacial duplication''' (cranio- from Greek {{lang|el|κρανίον}}, "skull", the other parts Latin), is an extremely rare [[congenital disorder]] whereby parts (accessories) or all of the face are duplicated on the head.<ref name=MedicineNet>[http://www.medterms.com/script/main/art.asp?articlekey=14546 Definition of diprosopus] {{Webarchive|url=https://web.archive.org/web/20131022201714/http://www.medterms.com/script/main/art.asp?articlekey=14546 |date=2013-10-22 }} at MedicineNet. Accessed 8 January 2006.</ref><ref name=BBC>[http://news.bbc.co.uk/1/hi/world/south_asia/7340091.stm 'Miracle baby' is feted in India] at [http://news.bbc.co.uk/ BBC News]. Accessed 10 April 2008.</ref><ref>{{cite journal |vauthors=al Muti Zaitoun A, Chang J, Booker M |title=Diprosopus (partially duplicated head) associated with anencephaly: a case report.|journal= Pathol Res Pract |volume=195 |issue=1 |pages=45–50 |year=1999 |pmid= 10048094 |doi=10.1016/s0344-0338(99)80094-6}}</ref><ref>{{cite journal |vauthors=Borzabadi-Farahani A, Yen SL, Yamashita DD, Sanchez-Lara PA |title=Bilateral maxillary duplication: case report and literature review.|journal= Oral Surg Oral Med Oral Pathol Oral Radiol|volume=113 |issue=5 |pages=e29–32 |year=2012 |pmid= 22676989 |doi=10.1016/j.tripleo.2011.06.017}}</ref><ref>{{cite journal |vauthors=Borzabadi-Farahani A, Gross J, Sanchez-Lara PA, Yen SL |title=An unusual accessory mandible and a submucosal cleft palate-a case report and review of the literature.|journal= Cleft Palate Craniofac J|volume=50 |issue=3 |pages=369–75 |year=2013 |pmid= 22404912 |doi=10.1597/11-032|s2cid=34449056}}</ref><ref>{{cite journal |vauthors=Borzabadi-Farahani A, Yen SL, Francis C, Lara-Sanchez PA, Hammoudeh J |title= A rare case of accessory maxilla and bilateral Tessier no. 7 clefts, a 10-year follow-up.|journal= J Craniomaxillofac Surg|volume=41 |issue=6 |pages=527–31 |year=2013 |pmid= 23333494 |doi=10.1016/j.jcms.2012.11.019}}</ref> |
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== Development == |
== Development == |
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Although classically considered conjoined twinning (which it resembles), diprosopus is not normally due to the fusion or incomplete separation of two embryos. It is the result of abnormal activity by the protein [[Sonic hedgehog (protein)|SHH (sonic hedgehog)]].<ref name=Hu>{{cite journal |vauthors=Hu D, Helms JA |title=The role of sonic hedgehog in normal and abnormal craniofacial morphogenesis |journal=Development |volume=126 |issue=21 |pages=4873–84 |date=1 November 1999|pmid=10518503 |url=http://dev.biologists.org/cgi/pmidlookup?view=long&pmid=10518503 }}</ref><ref>{{cite book |last=Ruiz i Altaba |first=Ariel |year=2006 |title=Hedgehog-Gli Signaling in Human Disease |url=https://www.springer.com/biomed/cancer/book/978-0-387-25784-6 |location=New York, NY |publisher=Springer Science+Business Media, Inc. |isbn=978-0-387-33777-7 |accessdate=March 12, 2014 }}</ref><ref>{{cite book |last1=Brugmann |first1=S.A. |last2=Helms |first2=J.A. |editor-first=Sally A. |editor-last=Moody |year=2007 |title=Principles of Developmental Genetics |chapter-url=http://store.elsevier.com/Principles-of-Developmental-Genetics/isbn-9780123695482/ |location=Burlington, MA |publisher=Academic Press, an imprint of Elsevier Inc. |chapter=Chapter 30: Craniofacial formation and congenital defects |isbn=9780123695482 |accessdate=March 12, 2014 }}</ref> (The name of this protein was inspired by the [[Sonic the Hedgehog (character)|Sonic the Hedgehog]] video game character and is part of an idiosyncratic naming tradition in molecular biology research.)<ref>{{cite journal |author=Donahue JK |title=Gene therapy, angiogenesis, Sonic Hedgehog: Sonic the Hedgehog to the rescue? |journal=Gene Ther. |volume=13 |issue=13 |pages=998–9 |date=July 2006 |pmid=17262905 |doi=10.1038/sj.gt.3302748 }}</ref><ref name="Cameron">{{cite news|last=Cameron|first=David|title=Express Yourself (Space Savers issue). Our chromosomes bear creative, even outlandish, names. Who knew?|work=Harvard Medical School News|volume=85|issue=2|year=2012|url=http://hms.harvard.edu/content/express-yourself|access-date=2012-10-22|archive-url=https://web.archive.org/web/20121031032639/http://hms.harvard.edu/content/express-yourself|archive-date=2012-10-31|url-status=dead}}</ref> |
Although classically considered conjoined twinning (which it resembles), diprosopus is not normally due to the fusion or incomplete separation of two embryos. It is the result of abnormal activity by the protein [[Sonic hedgehog (protein)|SHH (sonic hedgehog)]].<ref name=Hu>{{cite journal |vauthors=Hu D, Helms JA |title=The role of sonic hedgehog in normal and abnormal craniofacial morphogenesis |journal=Development |volume=126 |issue=21 |pages=4873–84 |date=1 November 1999|doi=10.1242/dev.126.21.4873 |pmid=10518503 |url=http://dev.biologists.org/cgi/pmidlookup?view=long&pmid=10518503 }}</ref><ref>{{cite book |last=Ruiz i Altaba |first=Ariel |year=2006 |title=Hedgehog-Gli Signaling in Human Disease |url=https://www.springer.com/biomed/cancer/book/978-0-387-25784-6 |location=New York, NY |publisher=Springer Science+Business Media, Inc. |isbn=978-0-387-33777-7 |accessdate=March 12, 2014 }}</ref><ref>{{cite book |last1=Brugmann |first1=S.A. |last2=Helms |first2=J.A. |editor-first=Sally A. |editor-last=Moody |year=2007 |title=Principles of Developmental Genetics |chapter-url=http://store.elsevier.com/Principles-of-Developmental-Genetics/isbn-9780123695482/ |location=Burlington, MA |publisher=Academic Press, an imprint of Elsevier Inc. |chapter=Chapter 30: Craniofacial formation and congenital defects |isbn=9780123695482 |accessdate=March 12, 2014 }}</ref> (The name of this protein was inspired by the [[Sonic the Hedgehog (character)|Sonic the Hedgehog]] video game character and is part of an idiosyncratic naming tradition in molecular biology research.)<ref>{{cite journal |author=Donahue JK |title=Gene therapy, angiogenesis, Sonic Hedgehog: Sonic the Hedgehog to the rescue? |journal=Gene Ther. |volume=13 |issue=13 |pages=998–9 |date=July 2006 |pmid=17262905 |doi=10.1038/sj.gt.3302748 |doi-access=free }}</ref><ref name="Cameron">{{cite news|last=Cameron|first=David|title=Express Yourself (Space Savers issue). Our chromosomes bear creative, even outlandish, names. Who knew?|work=Harvard Medical School News|volume=85|issue=2|year=2012|url=http://hms.harvard.edu/content/express-yourself|access-date=2012-10-22|archive-url=https://web.archive.org/web/20121031032639/http://hms.harvard.edu/content/express-yourself|archive-date=2012-10-31|url-status=dead}}</ref> |
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SHH and its corresponding |
SHH and its corresponding genes have been found to play an important role in signaling craniofacial patterning during embryonic development. Among other things, SHH governs the width of facial features. In excess it leads to widening of facial features and to duplication of facial structures.<ref name="Hu"/> The greater the widening, the more structures are duplicated, often in a mirror image form. This has been demonstrated in the laboratory by introducing pellets of the SHH protein into chicken embryos, resulting in chickens with duplicate beaks. Inadequate amounts of that protein lead to opposite conditions such as [[cyclopia]] where facial features are insufficiently developed.<ref>{{cite journal |vauthors=Young DL, Schneider RA, Hu D, Helms JA |title=Genetic and teratogenic approaches to craniofacial development |journal=Crit. Rev. Oral Biol. Med. |volume=11 |issue=3 |pages=304–17 |year=2000 |pmid=11021632 |url=http://cro.sagepub.com/cgi/pmidlookup?view=long&pmid=11021632 |doi=10.1177/10454411000110030201|citeseerx=10.1.1.542.1906 }}</ref> |
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Healthy brain development is also dependent on the signaling function of SHH.<ref name="Sciencedaily">[https://www.sciencedaily.com/releases/2001/03/010312072303.htm Sonic Hedgehog shapes the brain]. [https://www.sciencedaily.com/ ScienceDaily], 13 March 2001. |
Healthy brain development is also dependent on the signaling function of SHH.<ref name="Sciencedaily">[https://www.sciencedaily.com/releases/2001/03/010312072303.htm Sonic Hedgehog shapes the brain]. [https://www.sciencedaily.com/ ScienceDaily], 13 March 2001. |
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===Humans=== |
===Humans=== |
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Most human infants with diprosopus are stillborn. Known instances of humans with diprosopus surviving for longer than minutes to hours past birth are very rare; only a few are recorded. In 2002 and 2003, two living male infants with partial diprosopus were described in the medical literature in separate case reports.<ref>{{cite journal |vauthors=Hähnel S, Schramm P, Hassfeld S, Steiner HH, Seitz A |title=Craniofacial duplication (diprosopus): CT, MR imaging, and MR angiography findings case report |journal=Radiology |volume=226 |issue=1 |pages=210–3 |date=January 2003 |pmid=12511692 |url=http://radiology.rsnajnls.org/cgi/content/full/226/1/210 |doi=10.1148/radiol.2261011754}}</ref><ref name="Wu">{{cite journal |vauthors=Wu J, Staffenberg DA, Mulliken JB, Shanske AL |title=Diprosopus: a unique case and review of the literature |journal=Teratology |volume=66 |issue=6 |pages=282–7 |date=December 2002 |pmid=12486761 |doi=10.1002/tera.10102 |url=http://www.newyorkcraniofacialsurgery.com/Resources/diprosopus.pdf}}</ref> One infant was born with duplication of the nose and the cerebral frontal lobes, two widely spaced eyes, a small, underdeveloped central eye socket, and a large, asymmetric mouth. The other infant was born with duplication of the upper and lower jaw, two tongues arising from the same base, [[Cleft lip and palate|cleft palate]], a slightly divided tip of the nose, and two widely spaced eyes, as well as absence of the [[corpus callosum]], duplication of the pituitary gland and stalk, and abnormalities in the midbrain. Because they were born with a milder, partial form of diprosopus, both infants were considered candidates for surgical correction of their abnormal facial features. |
Most human infants with diprosopus are stillborn. Known instances of humans with diprosopus surviving for longer than minutes to hours past birth are very rare; only a few are recorded. In 2002 and 2003, two living male infants with partial diprosopus were described in the medical literature in separate case reports.<ref>{{cite journal |vauthors=Hähnel S, Schramm P, Hassfeld S, Steiner HH, Seitz A |title=Craniofacial duplication (diprosopus): CT, MR imaging, and MR angiography findings case report |journal=Radiology |volume=226 |issue=1 |pages=210–3 |date=January 2003 |pmid=12511692 |url=http://radiology.rsnajnls.org/cgi/content/full/226/1/210 |doi=10.1148/radiol.2261011754}}</ref><ref name="Wu">{{cite journal |vauthors=Wu J, Staffenberg DA, Mulliken JB, Shanske AL |title=Diprosopus: a unique case and review of the literature |journal=Teratology |volume=66 |issue=6 |pages=282–7 |date=December 2002 |pmid=12486761 |doi=10.1002/tera.10102 |url=http://www.newyorkcraniofacialsurgery.com/Resources/diprosopus.pdf |access-date=2008-04-21 |archive-date=2008-07-23 |archive-url=https://web.archive.org/web/20080723211820/http://www.newyorkcraniofacialsurgery.com/Resources/diprosopus.pdf |url-status=dead }}</ref> One infant was born with duplication of the nose and the cerebral frontal lobes, two widely spaced eyes, a small, underdeveloped central eye socket, and a large, asymmetric mouth. The other infant was born with duplication of the upper and lower jaw, two tongues arising from the same base, [[Cleft lip and palate|cleft palate]], a slightly divided tip of the nose, and two widely spaced eyes, as well as absence of the [[corpus callosum]], duplication of the pituitary gland and stalk, and abnormalities in the midbrain. Because they were born with a milder, partial form of diprosopus, both infants were considered candidates for surgical correction of their abnormal facial features. |
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==== Spanish case ==== |
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[[File:Les écarts de la nature page37.jpg|thumb|Spanish case of diprosopus, born 1775]] |
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In January 1775, a [[Spaniards|Spanish]] family gave birth to a girl with diprosopus. She had 3 eyes (one a combination of 2), 2 noses, 2 mouths, and 3 chins. The child was taken on tour from village to village, and gained much attention in Spain and neighboring countries. Her case is mentioned in Nicolas-François and Geneviève Regnault's ''Les écarts de la nature'', published that year.<ref>Regnault, Geneviève and Nicolas-François.''Les écarts de la nature''. 1775. https://gallica.bnf.fr/ark:/12148/btv1b8453981x/f1.planchecontact</ref> Her full lifespan is unknown, but is mentioned to still be alive in French publications as late as 1777. |
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==== Tres Johnson ==== |
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Tres Johnson was born on September 29, 2004, in Missouri, to Brandy and Joshua Johnson. He has a large cleft, two separate nostrils, and a head with an unusual shape. He also has cognitive delays and suffers from seizures. He was not expected to live more than a few hours, but he defied the odds and celebrated his 18th birthday in 2022. |
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Tres has undergone several surgeries to repair his cleft and reduce his seizures. He takes medication to control his epilepsy and attends a special school. He enjoys playing video games, listening to music, and spending time with his family and friends. He has a Facebook page<ref>{{Cite web | url=https://www.facebook.com/bnjtntjaj/ | title=Tres A. Johnson' Journey with Epilepsy and Diprosopus| date=2023-12-15|work=[[Facebook]]}}</ref> and an Instagram account where he shares his journey with diprosopus and epilepsy. |
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==== Lali Singh ==== |
==== Lali Singh ==== |
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Lali Singh |
Lali Singh (10 March – 10 May 2008)<ref name=BBC/> was born to Sushma and Vinod Singh in [[Saini, Sunpura Sohanpur]] village, near [[Delhi]]; the birth was delayed by [[dystocia]] caused by her large head, and her birth in a hospital was facilitated by her mother receiving an [[episiotomy]]. She was one of the very few infants with diprosopus to survive well past birth, and may have been the only known living individual with complete facial duplication. Her facial features included two pairs of eyes, two noses, and two mouths (but only one pair of ears). She was seen as the reincarnation of the goddess [[Durga]], who is sometimes depicted with many limbs and eyes. |
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Sushma and Vinod Singh declined an offer from local doctors to evaluate their daughter through [[CAT scan|CT]] or [[Magnetic Resonance Imaging|MRI]] scanning. Without diagnostic imaging, it was not possible to know the full extent to which the child's condition might have affected her brain and other vital structures in her head and neck. Thus, any estimation of her ability to thrive or even survive could be only speculative, though Lali's family described her as functioning normally. It is also unknown whether neurosurgeons or craniofacial surgeons, if consulted, would have had feasible solutions to offer with respect to corrective surgery. A local doctor told reporters that the baby should be considered a healthy child who currently was living a normal life, a previously unknown occurrence among sufferers of the disorder.<ref name=ABC> |
Sushma and Vinod Singh declined an offer from local doctors to evaluate their daughter through [[CAT scan|CT]] or [[Magnetic Resonance Imaging|MRI]] scanning. Without diagnostic imaging, it was not possible to know the full extent to which the child's condition might have affected her brain and other vital structures in her head and neck. Thus, any estimation of her ability to thrive or even survive could be only speculative, though Lali's family described her as functioning normally. It is also unknown whether neurosurgeons or craniofacial surgeons, if consulted, would have had feasible solutions to offer with respect to corrective surgery. A local doctor told reporters that the baby should be considered a healthy child who currently was living a normal life, a previously unknown occurrence among sufferers of the disorder.<ref name=ABC>{{cite web|url=http://www.abcnews.go.com/Health/Story?id=4549608&page=1|title=Two-Faced Baby Triggers Shock and Awe|work=[[American Broadcasting Company|ABC]]|access-date=21 April 2008|archive-date=21 January 2018|archive-url=https://web.archive.org/web/20180121000024/http://www.abcnews.go.com/Health/Story?id=4549608&page=1|url-status=dead}}</ref> |
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Lali's two middle eyes suffered from [[corneal opacity]] due to abnormal anatomy of the facial muscles, which prevented her from properly closing those eyes |
Lali's two middle eyes suffered from [[corneal opacity]] due to abnormal anatomy of the facial muscles, which prevented her from properly closing those eyes; initially, this was wrongly blamed on [[camera flash]]es. |
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A |
A cleft palate caused difficulty in her feeding under village conditions. A poor diet of bottle-fed sugar solution and diluted milk, allowed to drip down her throat, as she could not suck properly due to her cleft palate, weakened her condition, and vomiting and infection ensued. Admission to hospital was delayed by discussion (including taking her back home from hospital) among her [[extended family]] and her village's [[Village head|headman]]. Finally, her parents, alarmed at her illness and [[dehydration]], defied her other relatives and took her back to hospital, where under proper medical treatment including [[antibiotic]] and a [[saline drip]] she started to improve, stopped vomiting, started drinking milk and defecating normally. However, six hours later, at two months old to the day, she died of a [[heart attack]].<ref>{{Cite AV media|publisher=[[Channel 4]] UK television program|title=[[Body Shock]]|date=16 September 2008}}</ref> She was buried in her village, as is usual in Hinduism with children who die very young. Later a [[Hindu temple|temple]] was built at the village in her memory.{{fact|date=July 2020}} |
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==== Faith and Hope Howie ==== |
==== Faith and Hope Howie ==== |
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⚫ | Faith Daisy and Hope Alice Howie (8 May – 27 May 2014) were born in [[Sydney]], [[Australia]], to parents Simon Howie and Renee Young. Faith and Hope shared one body and skull, but had complete duplication of the facial features, as well as duplication of the brain; both brains joined to one brain stem. Young and Howie had learned at nineteen weeks gestation of their children's condition, but opted not to terminate the pregnancy. The children were born six weeks prematurely and appeared to be doing well, able to breathe unaided several days after their birth, and they were observed to sleep and cry at different times.<ref>{{Cite web | url=http://www.smh.com.au/nsw/conjoined-twins-hope-and-faith-have-distinct-personalities-say-parents-20140519-38ix2.html | title=Conjoined twins Hope and Faith have distinct personalities, say parents| date=2014-05-19|work=[[The Sydney Morning Herald]]}}</ref> They died nineteen days following their birth due to unknown causes, although some sources{{which|date=April 2021}} indicated that the girls died following an operation for unknown reasons.{{cn|date=April 2021}} |
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===Other animals=== |
===Other animals=== |
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[[File:Les écarts de la nature page32.jpg|thumb|An example of a "Janus cat"]] |
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Janus, a goat with two faces on one body, survived from April 5 to May 5, 2020 in [[Wittenberg, Wisconsin]].<ref name= nueskeFarms > |
Janus, a goat with two faces on one body, survived from April 5 to May 5, 2020 in [[Wittenberg, Wisconsin]].<ref name= nueskeFarms >{{Cite news|url=https://www.wausaudailyherald.com/story/news/2020/05/05/he-surely-missed-two-headed-goat-born-wittenberg-farm-dies/3083992001/|first=Melissa|last=Siegler|work=Wausau Daily Herald|date=May 5, 2020|title='He will surely be missed': Two-headed goat born on Wittenberg farm dies}}</ref> |
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Few two-faced animals have survived due to associated [[internal organ]] abnormalities and brain abnormalities. One of the most famous was [[Ditto (pig)|Ditto]], a pig.<ref>{{cite book |author=Armand Marie Leroi |title=Mutants: on the form, varieties and errors of the human body |publisher=Harper Perennial |location=New York, N.Y |year=2005 |isbn=978-0-00-653164-7 }}</ref> Ditto was raised to adulthood, but died of [[pneumonia]] caused by food inhalation when breathing through one muzzle while eating with the other. |
Few two-faced animals have survived due to associated [[internal organ]] abnormalities and brain abnormalities. One of the most famous was [[Ditto (pig)|Ditto]], a pig.<ref>{{cite book |author=Armand Marie Leroi |title=Mutants: on the form, varieties and errors of the human body |publisher=Harper Perennial |location=New York, N.Y |year=2005 |isbn=978-0-00-653164-7 }}</ref> Ditto was raised to adulthood, but died of [[pneumonia]] caused by food inhalation when breathing through one muzzle while eating with the other. |
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Cats with the condition are known as '[[Janus]] cats', after the Roman god.<ref>{{cite news| url=https://www.theguardian.com/world/2011/sep/30/two-faced-cat-guinness-world-record | location=London | work=The Guardian | first=Cherry | last=Wilson | title=Two-faced cat is a record breaker | date=30 September 2011}}</ref> In July 2006, a six-year-old male Janus cat called "[[Frank and Louie]]" from [[Millbury, Massachusetts]], USA, received publicity. In their case, only one [[esophagus]] (and possibly only one [[Vertebrate trachea|trachea]]) were functional; this aided survival. In September 2011, when Frank and Louie were twelve years old, it was announced that they would appear in the 2012 Guinness Book of World Records as the longest-surviving Janus cat on record.<ref>{{cite news|url=https://www.thestar.com/news/world/article/1061731--meet-frank-and-louie-the-longest-surviving-two-faced-cat?bn=1|title=Meet Frank and Louie, the longest surviving two-faced cat|last=Ngowi|first=Roderique|date=Sep 29, 2011|work=thestar.com|publisher=Toronto Star|accessdate=16 October 2011}}</ref> In 2014, Frank and Louie died at the age of fifteen.<ref name="WPXI">{{cite news|url=http://www.wpxi.com/news/lifestyles/pets/frank-and-louie-worlds-oldest-two-faced-cat-dies-1/njMLY/?icmp=cmgcontent_internallink_relatedcontent_2014_partners1|title=Frank and Louie, the |
Cats with the condition are known as '[[Janus]] cats', after the Roman god.<ref>{{cite news| url=https://www.theguardian.com/world/2011/sep/30/two-faced-cat-guinness-world-record | location=London | work=The Guardian | first=Cherry | last=Wilson | title=Two-faced cat is a record breaker | date=30 September 2011}}</ref> In July 2006, a six-year-old male Janus cat called "[[Frank and Louie]]" from [[Millbury, Massachusetts]], USA, received publicity. In their case, only one [[esophagus]] (and possibly only one [[Vertebrate trachea|trachea]]) were functional; this aided survival. In September 2011, when Frank and Louie were twelve years old, it was announced that they would appear in the 2012 Guinness Book of World Records as the longest-surviving Janus cat on record.<ref>{{cite news|url=https://www.thestar.com/news/world/article/1061731--meet-frank-and-louie-the-longest-surviving-two-faced-cat?bn=1|title=Meet Frank and Louie, the longest surviving two-faced cat|last=Ngowi|first=Roderique|date=Sep 29, 2011|work=thestar.com|publisher=Toronto Star|accessdate=16 October 2011}}</ref> In 2014, Frank and Louie died at the age of fifteen.<ref name="WPXI">{{cite news|url=http://www.wpxi.com/news/lifestyles/pets/frank-and-louie-worlds-oldest-two-faced-cat-dies-1/njMLY/?icmp=cmgcontent_internallink_relatedcontent_2014_partners1|title=Frank and Louie, the world's oldest two-faced cat, dies at 15|publisher=[[Cox Media Group]]|date=December 5, 2014|accessdate=December 5, 2014|url-status=dead|archiveurl=https://web.archive.org/web/20141209152135/http://www.wpxi.com/news/lifestyles/pets/frank-and-louie-worlds-oldest-two-faced-cat-dies-1/njMLY/?icmp=cmgcontent_internallink_relatedcontent_2014_partners1|archivedate=December 9, 2014}}</ref> |
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== See also == |
== See also == |
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*[[Durga]], a three-eyed Hindu goddess |
*[[Durga]], a three-eyed Hindu goddess |
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*[[Edward Mordake]], a disputed story of a 19th-century man with a face on the back of his head |
*[[Edward Mordake]], a disputed story of a 19th-century man with a face on the back of his head |
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*[[Futakuchi-onna]], a female Japanese |
*[[Futakuchi-onna]], a female Japanese ''[[yōkai]]'' with mouth on back of her head/hair |
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*[[Janus]], a Roman god with two faces |
*[[Janus]], a Roman god with two faces |
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*''[[Kara Mia]]'', a Philippine TV series that tells the story of a young woman with two faces divided in one body. |
*''[[Kara Mia]]'', a Philippine TV series that tells the story of a young woman with two faces divided in one body. |
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==External links== |
==External links== |
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{{Commonscat}} |
{{Commonscat}} |
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*{{cite book |last=Armand Marie |first=Leroi |year=2005 |title=Mutants: On Genetic Variety and the Human Body |url=http://www.us.penguingroup.com/nf/Book/BookDisplay/0,,9780142004821,00.html |location=New York, NY |publisher=Penguin Books |isbn=9780142004821 |accessdate=March 13, 2014 }} |
*{{cite book |last=Armand Marie |first=Leroi |year=2005 |title=Mutants: On Genetic Variety and the Human Body |url=http://www.us.penguingroup.com/nf/Book/BookDisplay/0,,9780142004821,00.html |location=New York, NY |publisher=Penguin Books |isbn=9780142004821 |accessdate=March 13, 2014 |archive-date=March 14, 2014 |archive-url=https://web.archive.org/web/20140314053129/http://www.us.penguingroup.com/nf/Book/BookDisplay/0,,9780142004821,00.html |url-status=dead }} |
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*{{cite book |editor1-last=Howie |editor1-first=Sarah |editor2-last=Fisher |editor2-first=Carolyn Elaine |year=2010 |title=Shh and Gli Signalling in Development |url=https://www.springer.com/life+sciences/evolutionary+%26+developmental+biology/book/978-0-387-39956-0 |location=New York, NY |publisher=Springer Science+Business Media, Inc. |isbn=978-0-387-39957-7 |accessdate=March 13, 2014 }} |
*{{cite book |editor1-last=Howie |editor1-first=Sarah |editor2-last=Fisher |editor2-first=Carolyn Elaine |year=2010 |title=Shh and Gli Signalling in Development |series=Molecular Biology Intelligence Unit |url=https://www.springer.com/life+sciences/evolutionary+%26+developmental+biology/book/978-0-387-39956-0 |location=New York, NY |publisher=Springer Science+Business Media, Inc. |doi=10.1007/978-0-387-39957-7 |isbn=978-0-387-39957-7 |accessdate=March 13, 2014 }} |
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*{{cite journal |vauthors=Tapadia MD, Cordero DR, Helms JA |title=It's all in your head: new insights into craniofacial development and deformation |journal=J. Anat. |volume=207 |issue=5 |pages=461–77 |date=November 2005 |pmid=16313388 |pmc=1571563 |doi=10.1111/j.1469-7580.2005.00484.x }} |
*{{cite journal |vauthors=Tapadia MD, Cordero DR, Helms JA |title=It's all in your head: new insights into craniofacial development and deformation |journal=J. Anat. |volume=207 |issue=5 |pages=461–77 |date=November 2005 |pmid=16313388 |pmc=1571563 |doi=10.1111/j.1469-7580.2005.00484.x }} |
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*[https://www.nytimes.com/2006/11/12/weekinreview/12schwartz.html ‘Sonic Hedgehog’ sounded funny, at first]. New York Times, 12 November 2006. |
*[https://www.nytimes.com/2006/11/12/weekinreview/12schwartz.html ‘Sonic Hedgehog’ sounded funny, at first]. New York Times, 12 November 2006. |
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*[http://www.learner.org/channel/courses/biology/units/gendev/experts/incardona.html Rediscovering biology: Unit 7, Genetics of development. Expert interview transcripts, interview with John Incardona, PhD]. explanation of the discovery and naming of the sonic hedgehog gene |
*[http://www.learner.org/channel/courses/biology/units/gendev/experts/incardona.html Rediscovering biology: Unit 7, Genetics of development. Expert interview transcripts, interview with John Incardona, PhD]. explanation of the discovery and naming of the sonic hedgehog gene |
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*[https://www.genenames.org/data/hgnc_data.php?hgnc_id=10848 Page for sonic hedgehog homolog (SHH)] at [http://www.hugo-international.org/ The Human Genome Organisation (HUGO)] |
*[https://www.genenames.org/data/hgnc_data.php?hgnc_id=10848 Page for sonic hedgehog homolog (SHH)] {{Webarchive|url=https://web.archive.org/web/20130918133833/http://www.genenames.org/data/hgnc_data.php?hgnc_id=10848 |date=2013-09-18 }} at [http://www.hugo-international.org/ The Human Genome Organisation (HUGO)] |
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*[http://www.skewsme.com/twofacedkitten.html Two-faced kitten has image problem] at Channel4000 |
*[http://www.skewsme.com/twofacedkitten.html Two-faced kitten has image problem] at Channel4000 |
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*[https://web.archive.org/web/20070109123547/http://www.oregonnews.com/article/20050616/NEWS/50616015 Two-faced kitten shocks owner, veterinarian] at OregonNews.com |
*[https://web.archive.org/web/20070109123547/http://www.oregonnews.com/article/20050616/NEWS/50616015 Two-faced kitten shocks owner, veterinarian] at OregonNews.com |
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*[https://www.reuters.com/article |
*[https://www.reuters.com/article/us-cat-twofaced-idUSTRE78Q6FV20110927 Two-faced cat sets record as oldest living "Janus" cat] at Reuters |
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*[http://metro.co.uk/2008/11/21/two-headed-kitten-has-twice-the-cuteness-of-regular-kittens-169916/ diprosopus kitten] |
*[http://metro.co.uk/2008/11/21/two-headed-kitten-has-twice-the-cuteness-of-regular-kittens-169916/ diprosopus kitten] |
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*[http://metro.co.uk/2007/03/07/oh-dear-its-another-two-headed-pig-164626/ diprosopus piglet] |
*[http://metro.co.uk/2007/03/07/oh-dear-its-another-two-headed-pig-164626/ diprosopus piglet] |
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<!--*[http://ca.news.yahoo.com/s/capress/080408/koddities/india_two_faced_baby Baby born with 2 faces in north India, villagers worship her as a goddess] 404 --> |
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*[http://www.nbcnews.com/id/24012024 Baby with 2 faces born in north India] MSNBC |
*[https://web.archive.org/web/20130610070459/http://www.nbcnews.com/id/24012024 Baby with 2 faces born in north India] MSNBC |
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*[http://cache.boston.com/resize/bonzai-fba/Reuters_Photo/2008/04/10/1207842162_9109/539w.jpg Image:Diprosopus] |
*[http://cache.boston.com/resize/bonzai-fba/Reuters_Photo/2008/04/10/1207842162_9109/539w.jpg Image:Diprosopus] |
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Latest revision as of 03:32, 4 December 2024
Diprosopus (Greek: διπρόσωπος, "two-faced", from δι-, di-, "two" and πρόσωπον, prósopon [neuter], "face", "person"; with Latin ending), also known as craniofacial duplication (cranio- from Greek κρανίον, "skull", the other parts Latin), is an extremely rare congenital disorder whereby parts (accessories) or all of the face are duplicated on the head.[1][2][3][4][5][6]
Development
[edit]Although classically considered conjoined twinning (which it resembles), diprosopus is not normally due to the fusion or incomplete separation of two embryos. It is the result of abnormal activity by the protein SHH (sonic hedgehog).[7][8][9] (The name of this protein was inspired by the Sonic the Hedgehog video game character and is part of an idiosyncratic naming tradition in molecular biology research.)[10][11]
SHH and its corresponding genes have been found to play an important role in signaling craniofacial patterning during embryonic development. Among other things, SHH governs the width of facial features. In excess it leads to widening of facial features and to duplication of facial structures.[7] The greater the widening, the more structures are duplicated, often in a mirror image form. This has been demonstrated in the laboratory by introducing pellets of the SHH protein into chicken embryos, resulting in chickens with duplicate beaks. Inadequate amounts of that protein lead to opposite conditions such as cyclopia where facial features are insufficiently developed.[12]
Healthy brain development is also dependent on the signaling function of SHH.[13] During embryonic development, SHH directs embryonic cells to organize in specific areas that later become specialized neural tissues, thus controlling the size and shape of brain structures.
Occurrences
[edit]Diprosopus often occurs in combination with other congenital disorders, particularly anencephaly, neural tube defect and cardiac malformations.[14] When present, the brain may show abnormalities ranging from partial to complete duplication of brain structures, and/or underdevelopment of brain tissues.[15][16]
Humans
[edit]Most human infants with diprosopus are stillborn. Known instances of humans with diprosopus surviving for longer than minutes to hours past birth are very rare; only a few are recorded. In 2002 and 2003, two living male infants with partial diprosopus were described in the medical literature in separate case reports.[17][18] One infant was born with duplication of the nose and the cerebral frontal lobes, two widely spaced eyes, a small, underdeveloped central eye socket, and a large, asymmetric mouth. The other infant was born with duplication of the upper and lower jaw, two tongues arising from the same base, cleft palate, a slightly divided tip of the nose, and two widely spaced eyes, as well as absence of the corpus callosum, duplication of the pituitary gland and stalk, and abnormalities in the midbrain. Because they were born with a milder, partial form of diprosopus, both infants were considered candidates for surgical correction of their abnormal facial features.
Spanish case
[edit]In January 1775, a Spanish family gave birth to a girl with diprosopus. She had 3 eyes (one a combination of 2), 2 noses, 2 mouths, and 3 chins. The child was taken on tour from village to village, and gained much attention in Spain and neighboring countries. Her case is mentioned in Nicolas-François and Geneviève Regnault's Les écarts de la nature, published that year.[19] Her full lifespan is unknown, but is mentioned to still be alive in French publications as late as 1777.
Tres Johnson
[edit]Tres Johnson was born on September 29, 2004, in Missouri, to Brandy and Joshua Johnson. He has a large cleft, two separate nostrils, and a head with an unusual shape. He also has cognitive delays and suffers from seizures. He was not expected to live more than a few hours, but he defied the odds and celebrated his 18th birthday in 2022.
Tres has undergone several surgeries to repair his cleft and reduce his seizures. He takes medication to control his epilepsy and attends a special school. He enjoys playing video games, listening to music, and spending time with his family and friends. He has a Facebook page[20] and an Instagram account where he shares his journey with diprosopus and epilepsy.
Lali Singh
[edit]Lali Singh (10 March – 10 May 2008)[2] was born to Sushma and Vinod Singh in Saini, Sunpura Sohanpur village, near Delhi; the birth was delayed by dystocia caused by her large head, and her birth in a hospital was facilitated by her mother receiving an episiotomy. She was one of the very few infants with diprosopus to survive well past birth, and may have been the only known living individual with complete facial duplication. Her facial features included two pairs of eyes, two noses, and two mouths (but only one pair of ears). She was seen as the reincarnation of the goddess Durga, who is sometimes depicted with many limbs and eyes.
Sushma and Vinod Singh declined an offer from local doctors to evaluate their daughter through CT or MRI scanning. Without diagnostic imaging, it was not possible to know the full extent to which the child's condition might have affected her brain and other vital structures in her head and neck. Thus, any estimation of her ability to thrive or even survive could be only speculative, though Lali's family described her as functioning normally. It is also unknown whether neurosurgeons or craniofacial surgeons, if consulted, would have had feasible solutions to offer with respect to corrective surgery. A local doctor told reporters that the baby should be considered a healthy child who currently was living a normal life, a previously unknown occurrence among sufferers of the disorder.[21]
Lali's two middle eyes suffered from corneal opacity due to abnormal anatomy of the facial muscles, which prevented her from properly closing those eyes; initially, this was wrongly blamed on camera flashes.
A cleft palate caused difficulty in her feeding under village conditions. A poor diet of bottle-fed sugar solution and diluted milk, allowed to drip down her throat, as she could not suck properly due to her cleft palate, weakened her condition, and vomiting and infection ensued. Admission to hospital was delayed by discussion (including taking her back home from hospital) among her extended family and her village's headman. Finally, her parents, alarmed at her illness and dehydration, defied her other relatives and took her back to hospital, where under proper medical treatment including antibiotic and a saline drip she started to improve, stopped vomiting, started drinking milk and defecating normally. However, six hours later, at two months old to the day, she died of a heart attack.[22] She was buried in her village, as is usual in Hinduism with children who die very young. Later a temple was built at the village in her memory.[citation needed]
Faith and Hope Howie
[edit]Faith Daisy and Hope Alice Howie (8 May – 27 May 2014) were born in Sydney, Australia, to parents Simon Howie and Renee Young. Faith and Hope shared one body and skull, but had complete duplication of the facial features, as well as duplication of the brain; both brains joined to one brain stem. Young and Howie had learned at nineteen weeks gestation of their children's condition, but opted not to terminate the pregnancy. The children were born six weeks prematurely and appeared to be doing well, able to breathe unaided several days after their birth, and they were observed to sleep and cry at different times.[23] They died nineteen days following their birth due to unknown causes, although some sources[which?] indicated that the girls died following an operation for unknown reasons.[citation needed]
Other animals
[edit]Janus, a goat with two faces on one body, survived from April 5 to May 5, 2020 in Wittenberg, Wisconsin.[24] Few two-faced animals have survived due to associated internal organ abnormalities and brain abnormalities. One of the most famous was Ditto, a pig.[25] Ditto was raised to adulthood, but died of pneumonia caused by food inhalation when breathing through one muzzle while eating with the other.
Cats with the condition are known as 'Janus cats', after the Roman god.[26] In July 2006, a six-year-old male Janus cat called "Frank and Louie" from Millbury, Massachusetts, USA, received publicity. In their case, only one esophagus (and possibly only one trachea) were functional; this aided survival. In September 2011, when Frank and Louie were twelve years old, it was announced that they would appear in the 2012 Guinness Book of World Records as the longest-surviving Janus cat on record.[27] In 2014, Frank and Louie died at the age of fifteen.[28]
See also
[edit]- Conjoined twins
- Craniopagus parasiticus
- Cyclopia
- Durga, a three-eyed Hindu goddess
- Edward Mordake, a disputed story of a 19th-century man with a face on the back of his head
- Futakuchi-onna, a female Japanese yōkai with mouth on back of her head/hair
- Janus, a Roman god with two faces
- Kara Mia, a Philippine TV series that tells the story of a young woman with two faces divided in one body.
- Polycephaly
References
[edit]- ^ Definition of diprosopus Archived 2013-10-22 at the Wayback Machine at MedicineNet. Accessed 8 January 2006.
- ^ a b 'Miracle baby' is feted in India at BBC News. Accessed 10 April 2008.
- ^ al Muti Zaitoun A, Chang J, Booker M (1999). "Diprosopus (partially duplicated head) associated with anencephaly: a case report". Pathol Res Pract. 195 (1): 45–50. doi:10.1016/s0344-0338(99)80094-6. PMID 10048094.
- ^ Borzabadi-Farahani A, Yen SL, Yamashita DD, Sanchez-Lara PA (2012). "Bilateral maxillary duplication: case report and literature review". Oral Surg Oral Med Oral Pathol Oral Radiol. 113 (5): e29–32. doi:10.1016/j.tripleo.2011.06.017. PMID 22676989.
- ^ Borzabadi-Farahani A, Gross J, Sanchez-Lara PA, Yen SL (2013). "An unusual accessory mandible and a submucosal cleft palate-a case report and review of the literature". Cleft Palate Craniofac J. 50 (3): 369–75. doi:10.1597/11-032. PMID 22404912. S2CID 34449056.
- ^ Borzabadi-Farahani A, Yen SL, Francis C, Lara-Sanchez PA, Hammoudeh J (2013). "A rare case of accessory maxilla and bilateral Tessier no. 7 clefts, a 10-year follow-up". J Craniomaxillofac Surg. 41 (6): 527–31. doi:10.1016/j.jcms.2012.11.019. PMID 23333494.
- ^ a b Hu D, Helms JA (1 November 1999). "The role of sonic hedgehog in normal and abnormal craniofacial morphogenesis". Development. 126 (21): 4873–84. doi:10.1242/dev.126.21.4873. PMID 10518503.
- ^ Ruiz i Altaba, Ariel (2006). Hedgehog-Gli Signaling in Human Disease. New York, NY: Springer Science+Business Media, Inc. ISBN 978-0-387-33777-7. Retrieved March 12, 2014.
- ^ Brugmann, S.A.; Helms, J.A. (2007). "Chapter 30: Craniofacial formation and congenital defects". In Moody, Sally A. (ed.). Principles of Developmental Genetics. Burlington, MA: Academic Press, an imprint of Elsevier Inc. ISBN 9780123695482. Retrieved March 12, 2014.
- ^ Donahue JK (July 2006). "Gene therapy, angiogenesis, Sonic Hedgehog: Sonic the Hedgehog to the rescue?". Gene Ther. 13 (13): 998–9. doi:10.1038/sj.gt.3302748. PMID 17262905.
- ^ Cameron, David (2012). "Express Yourself (Space Savers issue). Our chromosomes bear creative, even outlandish, names. Who knew?". Harvard Medical School News. Vol. 85, no. 2. Archived from the original on 2012-10-31. Retrieved 2012-10-22.
- ^ Young DL, Schneider RA, Hu D, Helms JA (2000). "Genetic and teratogenic approaches to craniofacial development". Crit. Rev. Oral Biol. Med. 11 (3): 304–17. CiteSeerX 10.1.1.542.1906. doi:10.1177/10454411000110030201. PMID 11021632.
- ^ Sonic Hedgehog shapes the brain. ScienceDaily, 13 March 2001.
- ^ al Muti Zaitoun A, Chang J, Booker M (1999). "Diprosopus (partially duplicated head) associated with anencephaly: a case report". Pathol. Res. Pract. 195 (1): 45–50, discussion 51–2. doi:10.1016/s0344-0338(99)80094-6. PMID 10048094.
- ^ Koseoglu K, Gok C, Dayanir Y, Karaman C (1 March 2003). "CT and MR imaging findings of a rare craniofacial malformation: diprosopus". AJR Am J Roentgenol. 180 (3): 863–4. doi:10.2214/ajr.180.3.1800863. PMID 12591714.
- ^ Angtuaco TL, Angtuaco EJ, Quirk JG (1 January 1999). "US case of the day. Complete brain duplication with fusion at the posterior fossa (diprosopus tetraophthalmos)". Radiographics. 19 (1): 260–3. doi:10.1148/radiographics.19.1.g99ja15260. PMID 9925407.
- ^ Hähnel S, Schramm P, Hassfeld S, Steiner HH, Seitz A (January 2003). "Craniofacial duplication (diprosopus): CT, MR imaging, and MR angiography findings case report". Radiology. 226 (1): 210–3. doi:10.1148/radiol.2261011754. PMID 12511692.
- ^ Wu J, Staffenberg DA, Mulliken JB, Shanske AL (December 2002). "Diprosopus: a unique case and review of the literature" (PDF). Teratology. 66 (6): 282–7. doi:10.1002/tera.10102. PMID 12486761. Archived from the original (PDF) on 2008-07-23. Retrieved 2008-04-21.
- ^ Regnault, Geneviève and Nicolas-François.Les écarts de la nature. 1775. https://gallica.bnf.fr/ark:/12148/btv1b8453981x/f1.planchecontact
- ^ "Tres A. Johnson' Journey with Epilepsy and Diprosopus". Facebook. 2023-12-15.
- ^ "Two-Faced Baby Triggers Shock and Awe". ABC. Archived from the original on 21 January 2018. Retrieved 21 April 2008.
- ^ Body Shock. Channel 4 UK television program. 16 September 2008.
- ^ "Conjoined twins Hope and Faith have distinct personalities, say parents". The Sydney Morning Herald. 2014-05-19.
- ^ Siegler, Melissa (May 5, 2020). "'He will surely be missed': Two-headed goat born on Wittenberg farm dies". Wausau Daily Herald.
- ^ Armand Marie Leroi (2005). Mutants: on the form, varieties and errors of the human body. New York, N.Y: Harper Perennial. ISBN 978-0-00-653164-7.
- ^ Wilson, Cherry (30 September 2011). "Two-faced cat is a record breaker". The Guardian. London.
- ^ Ngowi, Roderique (Sep 29, 2011). "Meet Frank and Louie, the longest surviving two-faced cat". thestar.com. Toronto Star. Retrieved 16 October 2011.
- ^ "Frank and Louie, the world's oldest two-faced cat, dies at 15". Cox Media Group. December 5, 2014. Archived from the original on December 9, 2014. Retrieved December 5, 2014.
External links
[edit]- Armand Marie, Leroi (2005). Mutants: On Genetic Variety and the Human Body. New York, NY: Penguin Books. ISBN 9780142004821. Archived from the original on March 14, 2014. Retrieved March 13, 2014.
- Howie, Sarah; Fisher, Carolyn Elaine, eds. (2010). Shh and Gli Signalling in Development. Molecular Biology Intelligence Unit. New York, NY: Springer Science+Business Media, Inc. doi:10.1007/978-0-387-39957-7. ISBN 978-0-387-39957-7. Retrieved March 13, 2014.
- Tapadia MD, Cordero DR, Helms JA (November 2005). "It's all in your head: new insights into craniofacial development and deformation". J. Anat. 207 (5): 461–77. doi:10.1111/j.1469-7580.2005.00484.x. PMC 1571563. PMID 16313388.
- ‘Sonic Hedgehog’ sounded funny, at first. New York Times, 12 November 2006.
- Rediscovering biology: Unit 7, Genetics of development. Expert interview transcripts, interview with John Incardona, PhD. explanation of the discovery and naming of the sonic hedgehog gene
- Page for sonic hedgehog homolog (SHH) Archived 2013-09-18 at the Wayback Machine at The Human Genome Organisation (HUGO)
- Two-faced kitten has image problem at Channel4000
- Two-faced kitten shocks owner, veterinarian at OregonNews.com
- Two-faced cat sets record as oldest living "Janus" cat at Reuters
- diprosopus kitten
- diprosopus piglet
- diprosopus calf
- Rescue Kitten Was Born With Two Face Who Rejected By Her Cat Mom diprosopus kitten
- Baby with 2 faces born in north India MSNBC
- Image:Diprosopus