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09 June 2011

Die Antwoord's Botha: what is progeria?

Leon Botha, member of the South African musical phenomenon Die Antwoord, died of progeria and is the oldest person to die from this condition. Learn more about progeria.

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Leon Botha, artist, DJ and member of the South African musical phenomenon Die Antwoord, died of complications of progeria on Sunday, one day after his 26th birthday.

Botha was diagnosed with progeria at the age of four and is the oldest person who has died of this condition. People rarely live beyond their early teens.

Progeria, also known as Hutchinson-Gilford syndrome and dubbed by some as “the old man’s syndrome”, is a rare disease that produces rapid ageing, beginning in childhood.

Its symptoms strongly resemble normal ageing and children typically appear significantly older.

Symptoms include:

  • Growth failure during the first year of life
  • Narrow, shrunken or wrinkled face
  • Baldness
  • Loss of eyebrows and eyelashes
  • Short stature
  • Large head for size of face (macrocephaly)
  • Open soft spot (fontanelle)
  • Small jaw
  • Dry, scaly, thin skin
  • Limited range of motion
  • Delayed or absent formation of teeth

Botha talks about the disease in this video:

Life-threatening

In most cases, affected individuals experience premature, widespread thickening and loss of elasticity of artery walls (atheriosclerosis), potentially resulting in life-threatening complications. The cause of death in progeria is usually related to the heart or a stroke. Louw underwent heart bypass surgery in 2005 to prevent a heart attack as a result of atheriosclerosis and suffered a stroke in November last year.

Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs without cause. It is only very rarely seen in more than one child in a family.

Treatment and prevention

There is presently no treatment for progeria. The condition cannot be prevented. Parents are encouraged to see their health care provider if their child does not appear to be growing or developing normally.

Botha accomplished a great deal in his short life. Apart from his work as DJ, he was also an artist and photographer. See him painting in this video.

Sources: MedlinePlus and YouTube

(Ilse Pauw and Birgit Ottermann, Health24, June 2011)

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