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: Clinical Presentation of Celiac Disease in Children :
: --------------------------------------------------- :
: by Ivor D. Hill, MB, ChB, MD summarized by Jim Lyles :
:..........................................................:
Dr. Hill is Professor of Pediatrics and clinical director of Pediatric
Gastroenterology & Nutrition at the University of Maryland School of
Medicine.
Historically, CD was seen more frequently in children than in adults,
and was characterized by malabsorption, diarrhea, and failure to
thrive starting at an early age. This has changed quite a bit over
the last two decades, for reasons that are not altogether clear. The
symptoms being presented vary quite a bit, and are often not
gastrointestinal in nature. Also, the age at which symptoms begin
appears to have increased significantly. The severity of the disease
seems to vary quite a bit as well.
Today we have to accept that CD can affect many different organ
systems in the body. Unfortunately, this message is not filtering
down to the students in our medical schools. Students are being
taught the classical symptoms of CD, and are not taught to look for
all the unusual ways in which an undiagnosed celiac patient might
present symptoms.
Presentation of CD in children falls into three categories, each of
which will be discussed further.
"Classical" Early Presentation
------------------------------
This was previously the most common form, but is becoming less common
today except in Sweden and southern Italy where 80% of the diagnoses
are made under the age of 2. In this category, children usually begin
showing symptoms between six and eighteen months of age, coinciding
roughly with the introduction of gluten into the diet. Up to 25% of
children showed symptoms within a month of introducing gluten to the
diet.
In most cases symptoms come on gradually. Stools become progressively
loose and more frequent, and are often pale and bulky with an
offensive odor. Diarrhea is accompanied by a failure to gain weight
satisfactorily or even a loss of weight. The child becomes
progressively malnourished and develops abdominal distention with
subcutaneous fat loss and muscle wasting. Although weight is affected
first, eventually height is affected as well. There is progressive
misery and irritability. There may be regression of development such
as losing the ability to walk.
Occasionally CD comes on very suddenly with severe diarrhea and
vomiting, leading to dehydration and what is called a "celiac crisis".
Conversely, there a some cases where the child has no diarrhea and in
some cases constipation becomes a problem.
Late Gastrointestinal Presentation
----------------------------------
This is becoming more of the norm. Almost half the newly diagnosed
cases are six years of age or older. The symptoms are still
gastrointestinal in nature, but are much milder. Diarrhea is still
the most frequent symptom, though it may come and go. Nausea and
vomiting occur in up to 30% of the cases, usually during bouts of
diarrhea. These symptoms tend to be less severe than in very young
patients. A smaller number of patients experience frequent abdominal
pain.
Most patients in this group describe vague abdominal discomfort and
feelings of bloating if specifically asked. Loss of appetite is a
common complaint, but it some cases there is an excessive need for
food, perhaps to compensate for the malabsorption of nutrients that
occurs as a result of the intestinal mucosal damage found in CD. Most
patients eventually experience unsatisfactory weight gain or even
weight loss. Conversely, those that experience an increase in
appetite may actually have an excessive weight gain for a short period
of time.
Gastrointestinal symptoms are often accompanied by feelings of
lethargy and poor health. Moodiness and irritability are also
characteristic of the disease.
Non-Gastrointestinal Presentation
---------------------------------
CD can affect virtually any organ system:
* The musculoskeletal system: Short stature, tooth enamel defects,
osteoporosis (loss of bone mass) and osteomalacia (softening of
the bones), arthritis, and arthralgia are all areas that can be
affected by active CD.
In some cases, short stature is the ONLY symptom initially.
Linear growth is affected. In these cases, the child may not
have an appearance of wasting; his weight may be appropriate for
his height In various studies it has been estimated that 5-24% of
children attending a clinic for short stature for which no reason
has been found had CD as the cause of short stature. In some
cases these children have a depressed level of growth hormones.
However, when CD is the cause of the short stature, the child
does not respond to growth hormone treatments.
The response to a GF diet is pretty good prior to puberty; in
most cases they can "catch up" in their growth. If the diagnosis
is missed until after puberty and after they have completed their
growth spurt, they will forever be short. That is one reason why
early diagnosis is important.
Tooth enamel defects are another interesting manifestation of CD.
It affects the permanent teeth, and the effect is distributed
symmetrically in the mouth. It is such a characteristic finding
that some dentists, if they are aware of it, can even refer
patients depending on what they find while looking at teeth. The
onset of this condition occurs before the age of seven, when the
permanent teeth are being formed. Early detection of CD is
important in these cases, as this effect is permanent and can
eventually lead to the destruction of the permanent teeth.
* Skin and mucous membranes: This system can be affected in many
ways. Dermatitis herpetiformis (DH) is one of the well-known
effects of CD in some patients. There may be an association
between atopic (widespread or nonspecific) dermatitis and CD as
well. Aphthous dermatitis (stomatitis) and hives can also occur.
DH is interesting because a patient with DH often has no
gastrointestinal symptoms, yet when a duodenal biopsy is
performed villi damage is usually found. DH is normally found in
celiacs between the ages of 15-40, but it is occasionally found
in even very young children.
* The hematological (blood and circulatory) system: CD can affect
this system in many ways: anemia, a decrease in the white cell
count, a decrease in platelets, and bleeding problems due to a
decrease in clotting factors. Anemia is the most common
hematological symptom, and can be caused by an iron, B-12, or
folate deficiency. B- 12 deficiency is very unusual in children,
the other two forms of anemia occur more frequently.
Vitamin K deficiency can also occur, because vitamin K is a fat
soluble and celiacs often have trouble absorbing fats. Vitamin K
is important in producing clotting factors.
* The central nervous system: These symptoms can mostly be
categorized as behavioral changes or epilepsy. Kids with
CD-related behavioral changes can be extremely irritable. They
seem cranky non-stop all day long and even when they are
sleeping. They have temper tantrums and show marked separation
anxiety. They can also show marked emotional withdrawal. Some
have even shown symptoms consistent with autism, though this is a
controversial subject which Dr. Hill did not go into. He said
that there might be a link between CD and autism, but was careful
to point out that not every case of autism would be improved by a
GF diet.
Why does CD affect behavior in some children? It has been shown
that a specific peptide chain in the gliadin molecule is what
actually causes the toxic reaction in celiacs. It turns out that
this peptide sequence is very similar to that found in certain
endorphins. Endorphins are produced by the body and affect brain
chemistry. Therefore, it could be that gliadin affects the brain
chemistry, which would certainly lead to behavioral changes.
There is a strong correlation between epilepsy and CD, especially
when there has been calcification of the brain. There have been
reports of a GF diet reversing the calcification and reducing or
eliminating the seizures associated with epilepsy, though this
may not occur in the majority of the cases.
* The reproductive system: This system is not generally affected
until puberty. Sometimes there is a delay in the beginning of
puberty in both males and females. This can be associated with
delayed growth and short stature, but it also occurs sometimes as
an isolated feature. In later life there can be a problem with
fertility and recurrent spontaneous abortions. When there is a
delay in puberty, the body usually responds dramatically to a GF
diet.
In summary, Dr. Hill believes that pediatricians need to start
getting the message out that CD is a highly variable disease. We
cannot afford to sit back and be blase' about this condition; if we
don't actively look for it in children we will often miss the
diagnosis.
At this point Dr. Hill began taking questions from the floor:
Q: A celiac asks: I have three children with no symptoms of CD.
Should I have them get the blood test? When should they be
retested?
A: The blood test is a good start in screening for CD. If it comes
back positive, then you can go on and look for a firm diagnosis.
If it comes back negative, they probably don't need to be retested
unless they develop one or more symptoms. (Watch for unusual
symptoms!)
Q: A woman states: My mother said I was sick from the time I was
born, though I wasn't diagnosed until a few years later. Is it
possible to have CD symptoms immediately after birth?
A: This is not very probable. The generally accepted belief is that
you must first have gluten introduced to the diet before you can
develop CD. There is a slight possibility, and this is highly
controversial, that some of the toxic peptides can come through in
breast milk.
Q: With the potential effect of gliadin on the brain chemistry you
described, should celiacs avoid drugs such as morphine which also
affect brain chemistry?
A: No, not at all. Morphine can be very effective when used
properly. The discussion of gliadin and brain chemistry was
merely meant to help explain why gliadin might cause behavioral
changes.
Q: My celiac child has responded well to a GF diet, but doesn't seem
to have as much of an appetite as non-celiac children. Should I
be concerned?
A: I would not be concerned about a child's appetite if he or she
continues to grow satisfactorily. Your appetite can go up when
you are malabsorbing to try to compensate for the failure to
absorb sufficient nutrients. When you correct the underlying
problem that is causing the malabsorption, often the appetite does
fall off as there is no longer a need to "overeat" to compensate
for poor absorption.
Q: My son was diagnosed at age 7, and has "caught up" to the growth
curve. Will his adult height be affected by his time as an
undiagnosed celiac?
A: Everyone has a certain growth potential. If celiac children
follow a strict GF diet, they should return to their own
individual growth curves and eventually reach their full growth
potential.
Q: My child was at the 90th percentile at six months of age. His
growth then fell off dramatically and eventually his short stature
led to a diagnosis of CD. He is now back at the 50th percentile
and holding steady there. Should I be concerned that he has not
gone back up to the 90th percentile?
A: I wouldn't get too hung up about the 90th percentile at six months
of life. That growth phase is not necessarily related to what the
growth potential is going to be. If he was at that level at four
or five years of age then you might make a case for that being his
normal level.
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