RESUMEN
BACKGROUND: A few studies on pediatric Celiac Disease (CD) are available from Central Asia. Recent immunogenetic research has highlighted that the HLA-DQ2/8 genetic predisposition to CD as well as the dietary intake of gluten in this geographical area, are comparable to other regions of the world where CD prevalence is known to be 1% or higher. METHODS: This is a prospective and cross-sectional study investigating the prevalence and clinical characteristics of CD in symptomatic children referred to the pediatric gastroenterology department of a tertiary hospital in Uzbekistan from 1 September 2021, until 31 July 2022. In addition to collecting the relevant information related to clinical manifestations and laboratory analyses from the clinical files, a specific survey was also administered to patients' guardians. Serological, histopathological, and immunogenetic parameters specific to CD, fecal zonulin, and pancreatic elastases were assessed in CD patients. RESULTS: The study population consisted of 206 children. Overall, almost all of them (n = 192; 93.2%) were referred because of gastrointestinal manifestations, which were associated with extra-gastrointestinal manifestations in most cases (n = 153; 74.3%); a minority (n = 14; 6.8%) was mainly referred due short stature and/or growth failure only. Among all of these study participants, CD was diagnosed in 11 children (5.3%). Notably, although diarrhea was similarly reported in CD and non-CD patients, watery diarrhea (type 7 according to the Bristol stool scale) was much more frequently and significantly observed in the former group. All of these CD patients showed anti-tTG IgA 10 times higher than the upper normal limit, except one child with lower serum levels of total IgA; however, all of them received a diagnostic confirmation by histopathological analysis due to the lack of EMA testing in the country. Notably, most CD children (82%) showed a Marsh III histological grading. Around half patients (54.5%) showed zonulin values above the reference range, whereas none showed insufficient levels of pancreatic elastase. However, no correlation or association between zonulin and clinical, laboratory, histopathological, and immunogenetic parameters was found. CONCLUSIONS: This study may further suggest a relevant prevalence of CD in Uzbek children, based on this partial picture emerging from symptomatic patients only. Additionally, we highlighted the prevalence of typical CD forms with watery diarrhea, which should strongly support a full diagnostic work-up for CD in the local clinical setting. The high levels of anti-tTG IgA and high Marsh grade might also lead us to speculate a significant diagnostic delay despite the classical clinical expression of CD.
RESUMEN
Background: Celiac disease (CD) is an immune-mediated disorder of the gut in which innate and adaptive responses are involved. Antimicrobial peptides (AMPs) constitute an arsenal of innate immunity regulators of paramount importance in the gut. However, the role of AMPs in CD is unclear. Aims: To evaluate the levels of fecal ß-defensin-2, fecal calprotectin (FC), and antibodies against bactericidal/permeability-increasing protein (BPI) in the serum of children with active CD and to compare them with those of healthy controls (HCs). Methods: We examined 76 children with recently diagnosed CD between the age of 2-10 years (average age: 6.1 ± 1.2 years) and 32 HC (average age: 6.2 ± 3.8 years) in this study. We evaluated the level of fecal ß-defensin-2 and FC levels in coprofiltrates, and the level of anti-BPI antibodies in blood serum. Correlation relationships between the parameters were assessed according to Pearson correlation coefficient. Results: Fecal ß-defensin-2 concentration was greater in the CD group than in HC group, amounting to 99.6 ± 15.5 ng/mL and 64.0 ± 2.4 ng/mL, respectively (p < 0.02). The level of FC in the CD children was 35.4 ± 8.1 µg/g, while that in the control group was 19.1 ± 1.1 µg/g, (p < 0.05), representing a slightly increase. The concentration of anti-BPI antibodies in the CD and HC groups was 35.9 ± 10.1 U/mL and 5.2 ± 3.2 U/mL, respectively (p < 0.002). There was a strong and direct correlation between fecal ß-defensin-2 and FC (r = 0.69), as well as a direct but weak relationship between fecal ß-defensin-2 and anti-BPI antibodies (r = 0.35). Conclusions: Our data reinforce that fecal ß-defensin-2 and anti-BPI antibodies are greatly increased in patients with active CD. These biomarkers may be components of epithelial innate immunity in the intestine, with each having a distinct functional role in intestinal6 mucosal defense.