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Cockayne syndrome (also called Weber-Cockayne syndrome, or Neill-Dingwall syndrome) is a rare autosomal recessive,^[1] congenital disorder characterized by growth failure, impaired development of the nervous system, abnormal sensitivity to sunlight (photosensitivity), and premature aging.^[2] Hearing loss and eye abnormalities (pigmentary retinopathy) are other common features, but problems with any or all of the internal organs are possible. It is associated with a group of disorders called leukodystrophies. The underlying disorder is a defect in a DNA repair mechanism.^[3]

It is named after English physician Edward Alfred Cockayne (1880–1956). Neill-Dingwall syndrome was named after Mary M. Dingwall and Catherine A. Neill.^[4]

Forms of Cockayne syndrome (CS)[link]

• CS Type I, the classic form, is characterized by normal fetal growth with the onset of abnormalities in the first two years of life. Impairment of vision, hearing, and the central and peripheral nervous systems progressively degenerate until death in the first or second decade of life.
• CS Type II, otherwise known as connatal CS, involves very little neurological development after birth. Death usually occurs by age seven. This specific type has also been designated as cerebro-oculo-facio-skeletal (COFS) syndrome.^[5][6] COFS syndrome can be further subdivided into several conditions (COFS types 1, 2, 3 (associated with xeroderma pigmentosum) and 4).^[7]
• CS Type III, characterized by late onset, is rare and milder than Types I and II.
• Xeroderma pigmentosum-Cockayne syndrome (XP-CS) occurs when an individual also suffers from xeroderma pigmentosum, another DNA repair disease. Some symptoms of each disease are expressed.

Genetics[link]

Cockayne syndrome has an autosomal recessive pattern of inheritance.

Cockayne syndrome is classified genetically as follows:

Mutations in the ERCC6 and ERCC8 genes are the cause of Cockayne syndrome. The proteins made by these genes are involved in repairing damaged DNA via the transcription-coupled repair mechanism, particularly the DNA in active genes. If either the ERCC6 or the ERCC8 gene is altered, DNA damage is not repaired. As this damage accumulates, it can lead to malfunctioning cells or cell death.

Mutations in the ERCC6 gene mutation makes up ~70% of cases.

Physical appearance[link]

Persons with this syndrome have smaller than normal head sizes, are of short stature, their eyes appear sunken, and they have an "aged" look.

See also[link]

• Accelerated aging disease
• Biogerontology
• Degenerative disease
• Genetic disorder
• CAMFAK syndrome — thought to be a form (or subset) of Cockayne syndrome^[8]

External links[link]

• Share and Care Cockayne Syndrome Network
• cockayne at NIH/UW GeneTests
• This article incorporates some public domain text from The U.S. National Library of Medicine
• 'Amy and Friends' — a friendly charity based in the UK supporting children with Cockayne Syndome

References[link]

Jeremy Kyle
Jeremy Kyle at Radio Festival 2010
Born	(1965-07-07) 7 July 1965 (age 46) Reading, England, United Kingdom
Nationality	British
Other names	Jezza
Education	University of Surrey
Occupation	Broadcaster
Years active	1996–present
Employer	ITV
Known for	The Jeremy Kyle Show
Spouse	Kirsty Rowley (m. 1989–1991) «start: (1989)–end+1: (1992)»"Marriage: Kirsty Rowley to Jeremy Kyle" Location: (linkback:http://en-wiki.pop.wn.com/index.php/Jeremy_Kyle) (divorced) Carla Germaine (m. 2003) «start: (2003)»"Marriage: Carla Germaine to Jeremy Kyle" Location: (linkback:http://en-wiki.pop.wn.com/index.php/Jeremy_Kyle)
Children	3 daughters, 1 son

Jeremy Kyle (born 7 July 1965)^[1] is an English radio and television presenter, best known for his British daytime television chat show on ITV, The Jeremy Kyle Show. Kyle is also the host of an American talk show of the same name which premiered on 19 September 2011.

Radio career[link]

From 1986-95, Kyle worked as a salesman for life insurance, recruitment, and radio advertising.^[2] He then became a radio presenter and after a brief stint at Orchard FM in Taunton and Leicester Sound in Leicester, he was signed by Kent's Invicta FM in 1996. In 1997, he joined BRMB in Birmingham, presenting the shows Late & Live and Jezza's Jukebox.^[3][4]

In 2000, Kyle moved to the Century FM network, taking this format with him. The show was called Jezza's Confessions. It was broadcast between 9pm and 1am. He won a Sony Award for Late & Live in 2001.^[2] On 1 July 2002, he made his first broadcast on Virgin Radio, presenting Jezza's Virgin Confessions every weekday from 8pm to midnight. In mid-2003, he broadcast the show from 9pm to 1am every weekday, and in January 2004 the show went out from 10pm to 1am, Sunday to Thursday. He left Virgin Radio in June 2004. From 5 September 2004, Kyle presented the Confessions show on London's Capital FM. The new programme aired Sunday to Thursday from 10pm to 1am with live calls on relationship issues of all kinds. Capital Confessions came to an end on 22 December 2005 to make way for The Jeremy Kyle Show, a similar show which ran from January 2006 to December 2006.

In late 2007, Kyle began a new show (The Jeremy Kyle Show), broadcasting across Gcap Media's One Network, of which Orchard FM, Invicta FM and BRMB, his previous employers, are a part. The programme differed from his previous shows in that he interviewed celebrities. Kyle also began broadcasting a new programme, on Essex FM, in November 2007.

Kyle joined Talksport on 21 September 2008 to present a lunchtime sports show every Sunday called The Jeremy Kyle Sunday Sports Show.

As a result of Talksport's Premiership coverage on a Sunday, Kyle's show was cancelled, and he left the station.^[5]

TV career[link]

In 2005 Kyle moved his format to ITV, with a programme also entitled The Jeremy Kyle Show. Here Kyle has reached his widest audience to date. His often aggressive manner with guests has been the source of both popularity and criticism. He is seemingly unafraid of reprisal from his guests, believing that speaking his mind is better than holding his peace. Guests sometimes take offence at Kyle's comments, one guest even attempting to throw a chair at him, whilst another threw an envelope at the back of his head, but he often justifies his criticism by claiming that he only wants to help them. Kyle recently claimed on air that his show was watched by 1.8 million viewers, a very high figure for a daytime chat show.

In September 2007, Judge Alan Berg described The Jeremy Kyle Show as trash which existed to "titillate bored members of the public with nothing better to do". He went on to say "It seems to me that the purpose of this show is to affect a morbid and depressing display of dysfunctional people whose lives are in turmoil" and added that it was "human bear-baiting".^[6] The judge so characterised it "after [a] husband was provoked into headbutting [his] wife's lover in front of [Kyle's] studio audience".^[7]

In February 2008, The Jeremy Kyle Show was again criticised in court after a man who found out during the recording of a show that he was not the father of his wife's baby later pointed an air rifle at her.^[8]

Other shows Kyle is involved with include Kyle's Academy, a ten-part series for ITV1 daytime which first aired on 18 June 2007.^[8] A team of experts (so-called life coaches and psychotherapists), headed by Kyle take five people and work with them over an intensive fortnight to help them on the road to a happier more fulfilled life. Kyle has also presented Half Ton Hospital, a show about morbidly obese people in the United States. In December 2009 he played himself in ITV1's comedy-drama The Fattest Man in Britain.

On 19 April 2011, Kyle began presenting a new documentary series called Military Driving School, where he visited the Defence School Of Transport in Yorkshire, following a group of new recruits as they undergo training as front line military drivers. He is the presenter of the ITV1 show High Stakes, which began on 11 October 2011. Billed as a game of "knowledge, risk, and tension," the show involves participants answering questions and stepping on the correct six squares on a grid in order to avoid trap numbers.^[9]

Writing career[link]

Kyle writes a column for Pick Me Up, a women's weekly magazine published by IPC.^[10] In his column, titled Jeremy Kyle Says..., Kyle adopts a frank style in responding to readers' problems that closely resembles the approach he takes on The Jeremy Kyle Show.

In 2009 Kyle wrote his first book, 'I'm Only Being Honest', about Britain's social problems and his views on how to solve them including recounts of his past and personal life.^[11]

Personal life[link]

Kyle was born in Reading, Berkshire.^[1] His father was an accountant and personal secretary to the Queen Mother.^[12] He attended the independent Reading Blue Coat School in Sonning, Berkshire^[2] and studied history and sociology at the University of Surrey.^[13]

He met his first wife, Kirsty Rowley, in 1988, when he was a recruitment consultant in an agency in Bristol.^[14] They became a couple within a fortnight, and were engaged within two months. They married in Almondsbury near Bristol seven months later, in July 1989. Their daughter, Harriet, was born in June 1990. The marriage ended just five months after the birth, in November 1990. His ex-wife has since claimed that Kyle had carefully concealed a destructive and expensive gambling habit from her over the course of their marriage.^[14]

He met former model Carla Germaine in 1999, while he was presenting on a BRMB radio show, and Germaine entered the controversial Two Strangers and a Wedding contest hosted by the station.^[15]

As the winner of the bride part of the contest, her prize was to marry the selected groom, Greg Cordell. Their marriage lasted only three months, after claims that Greg had an affair just days after their honeymoon, and she subsequently married Kyle in 2002.^[15][16][17] They have two daughters and a son together.

Kyle is a supporter of West Ham United.^[18] He suffers from obsessive–compulsive disorder and has stated that he "licks his mobile phone to make sure it's clean", as stated in his book "I'm Only Being Honest".^[19]

Kyle has described his opinion on "Broken Britain":

I think it starts with the breakdown of the family unit. Society should invest more in our kids. There should be community centres and youth clubs. And our benefits system – it's the greatest in the modern world. But there are loopholes and people taking advantage.^[12]

In October 2010, Kyle appeared at the Conservative Party Conference in Birmingham. He chaired "Getting Britain Back To Work" alongside George Osborne.^[20]

References[link]

External links[link]

• The Jeremy Kyle Show at itv.com