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Dr. Andrew Rynne
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Dr. Andrew Rynne

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Article Home Children's Health Gilford Syndrome

Gilford Syndrome

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It is also known as Hutchinson-Gilford progeria, Hutchinson-Gilford syndrome, Hutchinson-Gilford disease,Souques-Charcot syndrome (a variant of this syndrome)

It is a rare disorder charecterized by premature senility in children. It can occur in both sexes.

It is caused due to mutation in the Lamina A gene. It casues death mainly due to development of coronary artery disease. The death can occur as early as 16 years.

Features of Gilford's syndrome

  • Wizened old man facial appearance with small face,small jaw and pinched nose.
  • Faiure to thrive.
  • Alopecia.
  • Premature body aginf (progeria).
  • Dwarfism.
  • Large skull.
  • Bird like features.
  • Atrophy of muscles and skin.
  • Loss of subcutaneous fat.
  • High serum lipid levels.
  • Early atherosclerotic changes in vessels.


The child is usually normal at birth. Intelligence is normal.

Treatment

There is no effective treatment.

Complications