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$Unique_ID{BRK03791}
$Pretitle{}
$Title{Growth Delay, Constitutional}
$Subject{Growth Delay Constitutional Constitutional Delay in Growth and
Puberty CDGP Constitutional Short Stature Idiopathic Growth Delay Idiopathic
Short Stature Physiologic Delayed Puberty Sporadic Short Stature Short Stature
Growth Hormone Deficiency Hypochondroplasia Vitamin-D Deficiency Rickets
Turner Syndrome}
$Volume{}
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Copyright (C) 1991 National Organization for Rare Disorders, Inc.

865:
Growth Delay, Constitutional

** IMPORTANT **
It is possible that the main title of the article (Constitutional Growth
Delay) is not the name you expected.  Please check the SYNONYMS listing to
find the alternate name and disorder subdivisions covered by this article.

Synonyms

     Constitutional Delay in Growth and Puberty (CDGP)
     Constitutional Short Stature
     Idiopathic Growth Delay
     Idiopathic Short Stature
     Physiologic Delayed Puberty
     Sporadic Short Stature
     Short Stature

Information on the following diseases can be found in the Related
Disorders section of this report:

     Growth Hormone Deficiency
     Hypochondroplasia
     Vitamin-D Deficiency Rickets
     Turner Syndrome

General Discussion

** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

Constitutional Growth Delay is a term describing a temporary delay in the
skeletal growth and height of a child with no other physical abnormalities
causing the delay.  Short stature may be the result of a growth pattern
inherited from a parent (familial) or occur for no apparent reason
(sporadic).  Typically there is a period during childhood in which growth
slows down, eventually resuming at a normal rate.

Symptoms

Children with Constitutional Growth Delay have a period in which skeletal
growth and height are delayed.  During this period the child's height on a
growth chart is usually below the 5th percentile.  Typically, the child who
eventually has a delay in growth is born at a normal height and weight and
may grow at a normal rate for as much as two years.  When testing is done,
there is no physical impairment causing the delay.

There is often a history of this delay in growth occuring in other
members of the family.  If a parent was small as a child, with a late onset
of growth and/or puberty (eventually falling within the normal range), the
child may follow the same pattern.  This form of Constitutional Growth Delay
is referred to as familial.

Some children may experience a period of delayed growth for no known
reason (sporadic).

Normally puberty begins when the bone age reaches 10 1/2 years in females
and 11 1/2 years in males.  The chronologic age of puberty in children with
Constitutional Growth Delay is usually later.

The rounded head of the long bones in children is separated from the
shaft of the bone by the growth plate.  The growth plate is the area where
much of the growth takes place.  When the bone stops growing, the growth
plate becomes solid.  This process is known as fusion of the epiphyses.  The
normal age for the epiphyses to fuse is age 15 in females and age 18 in
males.  Female adolescents with Constitutional Growth Delay typically
continue growing until they are 17 or 18, while males may continue to grow
into their early 20's.  Puberty is normal in adolescents with CGD except for
the delay in age.  Adult height is within the normal range.

In some patients the skeletal age is not delayed as much as the height
age.

Causes

The exact cause of Constitutional Growth Delay is not known.  In some cases
the pattern of growth delay follows that of another family member.  If a
parent was small as a child and has a late onset of growth and puberty the
child may follow the same pattern.  A deficiency of growth hormone can be
ruled out through laboratory tests in cases where other physical and genetic
factors are not apparent.

Affected Population

Constitutional Growth Delay is a prevalent condition affecting males and
females.  Many more boys seek help than girls.  Approximately 35% of all
children with short stature have CGD.

Related Disorders

Symptoms of the following disorders can be similar to those of Constitutional
Short Stature.  Comparisons may be useful for a differential diagnosis:

Growth Hormone Deficiency (GHD) causes an absence or delay of lengthening
and widening of the skeletal bones inappropriate to the chronological age of
the child.  A sufficient quantity of growth hormone is required during
childhood to maintain growth and normalize sexual maturity.  In some cases
the onset of the disorder occurs prenatally, in others the condition occurs
months or years later.  Laboratory testing is necessary before a diagnosis of
Growth Hormone Deficiency can be made.  (For more information on this
disorder choose "Growth Hormone Deficiency" as your search term in the Rare
Disease Database).

Hypochondroplasia is a rare inherited skeletal disorder.  The major
symptom of this disorder is dwarfism that does not become evident until mid-
childhood.  Hypochondroplasia is characterized by a normal sized head and
small but normally shaped hands and feet.  Children with this disorder appear
normal at birth but the limbs fail to develop properly.  (For more
information on this disorder, choose "Hypochondroplasia " as your search term
in the Rare Disease Database).

Vitamin-D Deficiency Rickets is a rare disorder that appears during
infancy and childhood.  It is caused by insufficient amounts of vitamin D in
the body.  This deficiency can be caused by poor nutrition, a lack of
exposure to the sun, or malabsorption syndromes in which the intestines do
not adequately absorb nutrients from food.  Symptoms of this disorder may be
restlessness, lack of sleep, slow growth, a delay in crawling, sitting or
walking, thinness of the top and back of the skull, swelling of the skull and
a delay in the closing of the skull bone.  (For more information on this
disorder choose "Rickets" as your search term in the Rare Disease Database).

Turner Syndrome is a rare inherited disorder affecting females.  The main
symptoms of this disorder are a lack of sexual development, small stature,
possible mental retardation, a webbed neck, heart defects and various other
congenital anomalies.  Individuals with this disorder have an XO Kerotype,
i.e., they have neither the second X chromosome that characterizes females
nor the Y chromosome of males.  Individuals with Turner Syndrome have female
characteristics, but they do not develop secondary sexual characteristics.
(For more information on this disorder choose "Turner" as your search term in
the Rare Disease Database).

Disorders that are often associated with short stature such as Juvenile
Diabetes, Cystic Fibrosis, kidney diseases, asthmatics that have inhaled
steroids, etc., must all be ruled out in order to be certain that the short
stature has no known cause.

Therapies:  Standard

Treatment of Constitutional Growth Delay is not recommended since affected
children will eventually grow to a height within the normal range.  Typically
a child will get through this lull in growth with no problems.  Occasionally
there may be psychological problems associated with short stature and
counseling as well as family and peer support may be necessary.

When severe psychological symptoms are apparent, drugs may be prescribed
in conjunction with counseling.  These may include male hormones including
anabolic steroids.  This treatment should be done with caution under careful
medical supervision and frequent x-rays should be taken to make sure that the
skeletal age is not being advanced too much.  When bone development is
increased faster than height it can reduce the growing period and ultimately
reduce the patient's adult height.  Steroids can have severe physical and
behavioral side effects so this treatment is rarely recommended.

Therapies:  Investigational

Treatment with recombinant human growth hormone (hGH) is recommended only for
children with documented growth hormone deficiency as confirmed through
laboratory tests.  The use of hGH on healthy short children is controversial
since long-term consequences are unknown.

The Orphan Drug Testosterone (Sublingual) is currently being tested for
treatment of Constitutional Delay of Growth and Puberty in boys.

The Orphan Drug Oxandrolone is currently being tested for use in the
treatment of Constitutional Delay of Growth and Puberty.  Both drugs are
sponsored by:

     Gynex, Inc.
     1175 Corporate Woods Parkway
     Deerfield, IL  60015

This disease entry is based upon medical information available through
October 1991.  Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.

Resources

For more information on Constitutional Growth Delay, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     The Magic Foundation
     1327 N. Harlem Ave.
     Oak Park, IL  60302
     [phone]

     Human Growth Foundation (HGF)
     7777 Leesburg Pike
     P.O. Box 3090
     Falls Church, VA  22043
     [phone]
     [phone]

     Short Stature Foundation
     17200 Jamboree Rd., Suite J
     Irvine, CA  92714-5828
     [phone]
     800-24 DWARF

     NIH/National Institute of Child Health and Human Development
     9000 Rockville Pike
     Bethesda, MD  20892
     301-496-5751

References

INTERNAL MEDICINE, 2nd Ed.:  Jay H. Stein, Editor-In-Chief; Little, Brown and
Co., 1987.  Pp. 1986-88.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 1418.

CLINICAL PEDIATRIC ENDOCRINOLOGY, Solomon A. Kaplan M.D., W.B. Saunders
Company, 1990.  Pp. 10, 49-53.

GROWTH HORMONE THERAPY:  L. Shulman, et al.; Am Family Physician (May,
1990, issue 41(5)).  Pp. 1541-6.

TESTERONE TREATMENT IN ADOLESCENT BOYS WITH CONSTITUTIONAL DELAY IN
GROWTH AND DEVELOPMENT:  R.A. Richman, et al.; N Engl J Med (December 15,
1988, issue 319(24)).  Pp. 1563-7.

DOUBLE BLIND PLACEBO CONTROLLED TRIAL OF LOW DOSE OXANDROLONE IN THE
TREATMENT OF BOYS WITH CONSTITUTIONAL DELAY OF GROWTH AND PUBERTY:  R.
Stanhope, et al.; Arch Dis Child (May, 1988, issue 63(5)).  Pp. 501-5.

TREATMENT OF SHORT STATURE AND DELAYED ADOLESCENCE:  D.M. Wilson, et al.;
Pediatr Clin North Am (August, 1987, issue 34(4)).  Pp. 865-79.