We conducted this study to identify whether the
clinical presentation, mean anti-TTG level and severity of duodenal
lession are different in children with typical and atypical celiac
diseases, by a retrospective review of clinical charts of patients with
celiac disease, diagnosed between 2006 to 2010. Quantitative detection
of human IgA class anti tTG antibodies were measured with an
enzyme-linked immunosorbent assay kit (Orgentec, GmbH, Mainz-Germany).
Biopsies from the distal duodenum (minimum of four forceps biopsies)
were obtained by upper gastrointestinal endoscopy. Three pathologist
experienced with diagnosis of celiac disease reviewed the
histopathological specimens. Confirmation of a Marsh 2 or 3 lesion was
required for a diagnosis of celiac disease [1].
The clinical spectrum was categorized as (1)
typical or classical, suggested by clinical malabsorption,
chronic diarrhea or failure to thrive; and (2) atypical or
oligosymptomatic, suggested by abdominal pain, iron deficiency
anemia, chronic hypertransaminasemia, growth failure or screening of
risk groups, or familial study.
A total of 103 patients fulfilled the diagnostic
criteria for celiac disease (mean age: 6.6 years; range: 0-18 years;
median : 5 years). Clinical profile, biopsy findings, and TTG values
between the two groups are provided in
Table I.
TABLE I Comparison of Clinical, Biopsy and Serology Profile in Typical and Atypical Celiac Disease (N=103)
|
|
Typical CD |
Atypical CD |
Clinical features# |
|
malabsorption 25 (37)
|
anemia 10 (27) |
|
|
chronic diarrhea 21 (33) |
first degree relatives 9 (24.3) |
|
|
failure to thrive 12 (18) |
diabetes mellitus 6 (8.1) |
|
|
other gastrointestinal symptoms 8(12) |
nanosomia 4 (16.2) |
|
|
|
anorexia 3(10.8), urticaria 2 (54%) |
|
|
|
others 3 (8.1%) |
TTG* |
Marsh 3b and 3c |
140.5 (88.77) |
140 (73.53)
|
|
Marsh 3a |
129 (73.76) |
113.1 (89.43)
|
|
Marsh 2
|
96 (12,17) |
89 (14.85) |
Marsh degree# |
Marsh 3b and 3c
|
51 (77) |
25 (67.5)
|
|
Marsh 3a |
7 (10.6) |
4 (10.8)
|
|
Marsh 2
|
8 (12.4) |
8 (21.7)
|
#Number (%),
*Mean (SD). |
There are several studies that describe the
correlation between anti-TTG antibody level and Marsh type [2,3];
however, none of them deals with the difference between typical and
atypical group. In our study, the difference in the mean TTG level in
both groups was not significant. There was no difference in the clinical
presentation and the severity of duodenal lession, indicating that
factors other than the degree of villous atrophy must account for
typical simptoms in celiac disease.The diagnosis of atypical celiac
disease is often made only at an advanced stage because of the lack of
gastrointestinal symptoms, and it could be a possible reasons for
similar biopsy changes in children with and without typical features.As
opposed to our study, Dinler, et al. [4] found that
total/subtotal villous atrophy was significantly higher in the typical
than in atypical group . The potential drawback of our work is a
retrospective data and bias due to hospital setting.
References
1. Husby S, Koletzko S, Korponay-Szabó IR, Mearin
ML, Phillips A, Shamir R, et al. Guidelines for the Diagnosis
of Coeliac Disease in Children:Recomendation of the European Society
for Pediatric Gastroenterology, Hepatology and Nutrition. JPGN.
2012;54:136-60.
2. Hansson T, Dahlbom I, Rogberg S, Dannaeus A,
Hopfl P, Gut H, et al. Recombinant human tissue
transglutaminase for diagnosis and follow up of childhood celiac
disease. Pediatr Res. 2002;51:700-5.
3. Fabiani E, Catassi C; International Working
Group on Eu-TG. The serum IgA class anti-tissue transglutaminase
antibodies in the diagnosis and follow up of celiac disease: results
of an international multi-centre study. Eur J Gastroenterol Hepatol.
2001;13:659-65.
4. Dinler G, Atalay E, Kalayci G. Celiac disease in 87 children with
typical and atypical symptoms in Black sea region of Turkey.World J
Pediatr. 2009;5: 282-6.