How Often is Short Stature Predictive of Celiac Disease?
by Ron Hoggan
Following some debate on sci.med, I decided to explore the issue of short stature as a predictor of celiac disease. A Medline search revealed 54 articles listing both short stature and celiac disease. Given the relatively small body of literature on this disease, that number alone has some significance.
Of course, not all these articles could be found in the local Medical library, so I will have to await the arrival of the others to render a full accounting. Nonetheless, some significant information can be gleaned from those I was able to access immediately.
Cacciari et al reported, in 1983, an 8.3% incidence of total villous atrophy and response to gluten-free diet among a random population of children with short stature (1). Because of the date of their study, they could not assert a diagnosis of celiac disease, as the diagnostic criteria of the time required three biopsies punctuated by a gluten challenge. By today's standards they would not need to qualify their assertion of celiac disease with the term "probable" as they did then. A positive response to the diet, following discovery of villous atrophy is sufficient. Unfortunately, Cacciari excluded all but the children with total villous atrophy. Those with partial atrophy were not included. Information from a later report by some of the same investigators suggests that the incidence might have approached 20% had they included the children with partial atrophy.
Short stature was the *only* symptom which identified the subjects, and there was no other criteria on which they were pre-selected. They conclude that "....asymptomatic celiac disease represents a cause of short stature that cannot be ignored, and that only by intestinal biopsy can such patients be identified."
Neither is this group alone. Groll et. al. state: "Short stature by itself, in the complete absence of gastrointestinal symptoms, is an indication for jejunal biopsy, particularly if bone age is delayed by more than 4 years and/or there are associated haematological abnormalities"(2). The short children they investigated demonstrated a 21% incidence of celiac disease in a population where other endocrine disorders had been ruled out. So this is a selected population of children with short stature.
In another selected population of short children, Rosenbach et. al. report celiac disease in 48% of those studied (3). They recommend: "Children with short stature of unknown cause and bone age retardation should be evaluated for the presence of celiac disease."
Yet another group states: "In conclusion, we found that in a large series of CCD [childhood celiac disease] patients growth retardation was one of the most consistent symptoms. We therefore recommend searching for CCD [childhood celiac disease] in all otherwise undiagnosed short children" (4).
Stenhammar et. al. report only a 5% incidence of celiac disease among a group of 87 children with short stature which was otherwise unselected (5). They conclude with the statement that there is an over-representation of celiac disease among populations with short stature.
Cacciari et. al. reported another study of 88 children with short stature in 1985 (6). They still held to the requirement of total villous atrophy, and again revealed an incidence of celiac disease. Fortunately, this time they reported 7 patients with partial villous atrophy. By today's ESPGAN standards, this group would be included. That would suggest a total of 16 of 88 short children with probable celiac disease.
It is quite significant that based on the conservative number (9 of 88) they state: "Symptomless celiac disease is therefore a commoner cause of short stature than is hypopituitarism..."
Auricchio et. al. state the following:
"Five to 20 per cent of unexplained short children who have been biopsied
have been shown to have celiac disease. It is indeed expected by the great
difference in the incidence rate of the two diseases that short children
with GSE [gluten sensitive enteropathy] are more common than growth
hormone-deficient children" (7).
I do not pretend that I have done an exhaustive study of the literature. There appears, however, to be good cause to investigate children of short stature for the possibility of celiac disease. One may quibble about numbers, of course. Each study reflects limitations on the populations studied, and the groups studied are relatively small. It should not be surprising that the numbers have varied substantially. Nonetheless, the incidence of celiac disease usually seems to fall between 5% and 20% of short children. Whatever the specific incidence in a given study, those reporting on the connection appear to see celiac disease as a more frequent cause of short stature than hypopituitarism or growth hormone-deficiency.
Given the changes in the ESPGAN criteria for diagnosis of celiac disease, and the recent recognition of Michael N. Marsh's taxonomy of morphological changes of mucosal architecture in celiac disease, the suggestion that approximately 20% of children with short stature seems a very probable figure.
I hope this is helpful.
best wishes,
Ron Hoggan
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