BIOL. 2402 - ANATOMY & PHYSIOLOGY
RICHLAND COLLEGE
SPRING 2001
Class Project
Web project members:
-
Deanna McCall
-
Debi Blow
-
Rosemary Kavishe
-
Nicole Rainier
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 childs growth rate will slow during the
second year of life to about 1/2 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 1/2 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 loose 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 of 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 manible 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
(Property of the Sunshine Foundation)
For
additional information regarding the physical appearance of children with
progeria go to:Sunshine
Foundation
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 do 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.
GENETIC
INHERITANCE OF THESE DISEASES
THERAPY
Works Cited
Goldstein,
S. "The biology of aging: Looking to defuse the genetic time bomb." Geriatrics
1993; 9:76.
"Hutchinson-Gilford
Progeria Syndrome." December 1998. World Wide Web. 22 February 2001
<http://www.ibionet.com/rarediseases/hutchinsongilfordprogeriasyndrome.html>.
"Patterns
of Growth." 27 March 2001. World Wide Web. 8 January 2000
<http://www.hgfound.org/patterns.html>.
"176670
Progeria." 29 December 1999. World Wide Web. 28 January 2001 <http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?176670>.
"The
Progeria Research Foundation, Inc." World Wide Web. 22 February 2001
<http://www.progeriaresearch.org>.
"The
Sunshine Foundation." January 2001. World Wide Web. 28 January 2001
<http://www.sunshinefoundation.org>.
For feedback:
Jackie
Reynolds (Professor of Biology, Richland College)
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Reynold's HOMEPAGE
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