Hutchinson-Gilford syndrome  
 
  

Diseasereference.net - Comprehensive articles covering over 1,700 topics. The articles are organized by the disease, condition overview, symptoms, treatment, and prevention.


Terms search, click the first letter of a term name:
A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z

  

 

Disease Reference

Click on the first letter in the disease name:

| 4 | 5 | A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z



     

Hutchinson-Gilford syndrome

Definition

Progeria is a disease that produces rapid aging, beginning in childhood.

Alternative Names

Hutchinson-Gilford syndrome

Causes

Progeria is a rare condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children.

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 not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have the condition.

Symptoms

  • 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 (micrognathia)
  • Dry, scaly, thin skin
  • Limited range of motion
  • Teeth - delayed or absent formation

Exams and Tests

The signs include:

  • Skin changes similar to that seen in scleroderma (the connective tissue becomes tough and hardened)
  • Insulin-resistant diabetes (diabetes that does not respond readily to insulin injections)

Cardiac stress testing may reveal signs of early atherosclerosis of blood vessels.

Genetic testing can detect mutations in lamin A that cause progeria.

Treatment

There is presently no treatment for progeria.

Support Groups

Progeria Research Foundation, Inc. -- www.progeriaresearch.org

Outlook (Prognosis)

Progeria is associated with a short lifespan. The average patient survives to the early teens. However, some patients can live up to 30 years. The cause of death is usually related to the heart or a stroke as a result of the progressive atherosclerosis.

Possible Complications

When to Contact a Medical Professional

Call for an appointment with your health care provider if you child does not appear to be growing or developing normally.

Prevention

There is no known prevention.




   Hutchinson-Gilford syndrome
Abscess - intra-abdominal
Hypervitaminosis D
Acute interstitial (allergic) nephritis
Familial combined hyperlipidemia
Anthrax - inhalation
Anal atresia
Anemia - idiopathic autoimmune hemolytic
Bladder cancer
Adult hypothyroidism


 
  
© Copyright by Diseasereference.net 2006-2022. All rights reserved