Progeria?
- Literature - Hutchinson-Gilford Syndrome
Hutchinson-Gilford Syndrome
Biol. 2402 - Anatomy & Physiology
Richland College, Spring 2001 Class Project
Web Project Members:
Deanna McCall, Debi Blow, Rosemary Kavishe, Nicole Rainer
Hutchinson-Gilford syndrome is characterized by the accelerated aging of children. The normal aging process begins at birth with the child averaging about 20 inches in length. This rapid growth will continue for about 12 months with the average growth being about 10 inches. The child’s growth rate will slow during the second year of life to about ½ the speed with the average growth being about 5 inches. From the ages of 2 to 12 an even slower growth occurs. Most children grow on an average of 2 to 2 ½ inches a year until the start of puberty. Puberty (16 to 18 years of age) begins another acceleration in the growth cycle. The total amount of growth that will occur is determined by genetics, the age of the child at the onset of puberty and the length of time and speed of the pubertal growth spurt (Patterns of Growth, p. 1). The child afflicted with Hutchinson-Gilford syndrome will experience all of the same changes due to the aging process at about seven times the normal rate. The adult onset form of progeria is called Werner’s syndrome. Information on Werner’s syndrome can be found at Werner’s website. Progeria is a rare genetic syndrome that is being researched at this time. For information on how to make a donation to this research please contact The Progeria Research Foundation.
Growth:
- Short in stature, slowly reach the size
of a 2 or 3 year old child during the first 10 years
- Dwarfism or stunted growth
Endocrine:
The fat under the skin is nonexistent
Skeletal:
Bones are brittle and break very easily.
These breaks (fractures) seldom heal correctly.
Cardiac:
- Premature arteriosclerosis: hardening
and thickening of the arteries causing them to lose their elastic ability
- Premature coronary artery disease: the
amount of blood that flows through the arteries and supplies blood flow to the
outside of the heart is reduced
- Angina pectoris: brief attacks of chest
pain due to deficient oxygen supply to the heart muscle
- Myocardial infarction: death of a muscle to
the heart
- Congestive heart failure: the usual amount
of blood flowing into and out of the heart can no longer be maintained and the
heart fails
Liam, a South Jersey Sunshine Foundation chapter
volunteer, sharing his toys with Devin and Sammy
(Property of the Sunshine Foundation)
Face:
- The face is below a normal size
- Micrognathia is when the mandible is
abnormally small on one or both sides
- The person with this syndrome may also
appear older than they really are and may have a wizened (shriveled or dried up)
facial expression
Visual:
- Cloudy corneas (transparent covering of
the iris and pupil that allows the light to enter the eye appears cloudy)
- Possible blue sclera (white portion of the
eyeball is blue)
Organs:
- Enlargement of the spleen
- Infantile sex organs - the genital area of
both male and female does not age like the rest of the organs, but remains that
of a child’s
- Umbilical/inguinal hernia: there are two
common types of hernias (a rupture of a part of an organ through the tissue or
cavity wall that is usually enclosed): umbilical - abdomen area; inguinal -
groin area
Appearance:
Danny England
In Summary:
- Patient may have a larger than normal
head as compared to their face.
- The patient may have a beak-like (pointed)
nose.
- The patient could have a receding (moved or
sloped backwards) chin.
- The person with progeria may have very thin
skin, some may describe as “paper-like”.
- The progeria patient may appear with large
prominent (noticeable, stand out) eyes.
- Also due to alopecia (loss of hair) the
patient may not have eyebrows or eyelashes.
- The person with this syndrome may also have
irregular crowded teeth.
- The progeria patient may also have a narrow
(not wide) chest.
- In addition, the progeria patient may have
a protruding (project forward) abdomen.
- Another feature is the veins on the scalp
are very prominent or easily seen.
- The person with progeria may also appear to
have very pale or light skin.
- The person with this syndrome may also
appear older than they really are and may have a wizened (shriveled or dried up)
facial expression.
Copyright © The Progeria Project Foundation