Progeria: Wiedemann-Rautenstrauch Syndrome

Progeria is a type of genetic disorder where aging is rapid and premature. There are different genetic disorders with this condition. All of them reflect rapid premature aging in victims. Collectively, they are called progeroid syndromes. But they are treated and considered as clinically unique from one another.

Not much is written about this condition. But Hutchinson-Gilford progeria syndrome (HGPS) is often misdiagnosed as Wiedemann-Rautenstrauch Syndrome (WRS). So, many symptoms of HGPS are also symptoms displayed from children and infants with this condition.

Clinical diagnosis of these infants shows that they appear old. There is no known treatment or prevention for this condition. However, for treatment of their other diseases like respiratory infection, they are treated the same way people with these problems are treated.

Wiedemann-Rautenstrauch Syndrome

Wiedemann-Rautenstrauch syndrome is very similar with Hutchinson-Gilford progeria syndrome (HGPS). However, children and infants with this condition have varying degrees of mental retardation. It is known as neonatal progeroid syndrome. Individuals with this syndrome live only up to 6 years of age. Like many other progeroid syndromes but unlike HGPS, this condition is hereditary.

WRS was first described by Thomas Rautenstrauch in 1977 as progeria. In 1979, Hans-Rudolf Wiedemann had differentiated it from the other types of progeroid syndromes.

Symptoms include

  1. Aged appearance at birth
  2. Growth delays in height and in weight
  3. Absence of subcutaneous fats (fats under the skin)
  4. Abnormal deposit of fats around the buttocks, hips, and belly
  5. Abnormally thin arms and legs
  6. Abnormally large hands and feet for their size
  7. Progressive and/or varying degrees of mental retardation
  8. Severe delays in psychomotor development
  9. Limitations in range of motion
  10. Large head, small face, chin, and jaw
  11. Small body and short in stature
  12. High pitched voice

Usual cause of death is repeated respiratory infection. Even before birth, doctors can already diagnose the fetus’ growth delays.

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Comments 6 comments

quicksand profile image

quicksand 7 years ago

I guess regular practice of Surya Namaskar combined with Pranayama (yogic breathing) could be a preventive. :)

BeatsMe profile image

BeatsMe 7 years ago Author

Hi Quicksand, for normal people, the practices you've mentioned will help but for people with progeria, they won't.

Thanks for taking the time to read this hub. I know that this hub series can be quite boring but they are here for variety. :)

quicksand profile image

quicksand 7 years ago

It's not boring at all, it's only frightening! The variety could help if you participate in the 30-day hub challenge which starts of on 24th July. I hope you will take part. Good luck 2 U. :)

BeatsMe profile image

BeatsMe 7 years ago Author

Hi Quicksand, thanks for the encouragement. :)

As for the hub challenge, I'm afraid I'm not much of a participant. Sorry 'bout that. Besides, my free time can sometimes be unpredictable.

If you're taking part, good luck. :)

cgull8m profile image

cgull8m 7 years ago from North Carolina

It must be tough for children to be inflicted with this disease. I hope God gives them another chance.

BeatsMe profile image

BeatsMe 7 years ago Author

Hi Cgull, I know. But once inflicted, they just have to deal with it. I know it's not fair but it's life. :(

Thanks for coming by. :)

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