en POLSKI
eISSN: 2300-8660
ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
Current issue Archive Manuscripts accepted About the journal Editorial board Abstracting and indexing Contact Instructions for authors Ethical standards and procedures
Editorial System
Submit your Manuscript
SCImago Journal & Country Rank
2/2021
vol. 96
 
Share:
Share:
abstract:
Original paper

The prevalence of celiac disease, related HLA-genotypes, and comorbidities among Egyptian girls with Turner syndrome: a single-centre study

Nanees Abdel-Badie Salem
1
,
Shimaa R. Hendawy
2

  1. Pediatric Endocrinology and Diabetes Unit, Department of Pediatrics, Faculty of Medicine, Mansoura University, Egypt
  2. Department of Clinical Pathology, Mansoura University, Egypt
Pediatr Pol 2021; 96 (2): 101–108
Online publish date: 2021/07/01
View full text Get citation
 
PlumX metrics:
Introduction
An association between celiac disease (CD) and Turner syndrome (TS) has gained widespread recognition with wide-ranging prevalence rates. Insufficient data are available concerning this association among Egyptian TS girls. Therefore, we aimed to examine the prevalence of CD; to define the predisposing HLA-genotypes, clinical characteristics and associated comorbidities in a cohort of Egyptian TS girls; and to assess the impact of karyotypes and oestrogen exposure on the overall prevalence of autoimmunity.

Material and methods
Fifty-five TS girls were initially screened by total IgA and anti-tissue transglutaminase (anti-tTG) IgA. Anti-tTG-IgG assay is a second step in IgA-deficient girls. CD-seropositive TS girls were subjected to HLA-typing and endoscopic duodenal biopsies, and were evaluated for associated comorbidities.

Results
Seroprevalence (anti-tTG-IgA > 10 U/ml) and biopsy-confirmed prevalence of CD were 5.5% (3/55) and 3.6% (2 girls only underwent endoscopic biopsies and displayed Marsh-IIIb), respectively. Absolute and partial IgA-deficiency were observed in 1 and 5 girls, respectively; they were all negative for anti-tTG-IgG. HLA-typing of CD-seropositive girls showed that case 1: DQA1+/DRB1–; case 2: DQA1+/DRB1+; and case 3: DQB1+/DRB1–. Iron-deficiency anaemia, vitamin-D deficiency, and low bone mineral density were detected among celiac girls. No significant associations were observed between different karyotypes or oestrogen exposure and autoimmunity prevalence.

Conclusions
Seroprevalence and biopsy-confirmed prevalence of CD in Egyptian TS girls were 5.5% and 3.6%, respectively, which supports the association between these disorders and reinforces the importance of screening for CD in TS patients as a high-risk population. All CD-seropositive girls displayed the predisposing CD HLA-DQ2 and/or DR4 alleles. Careful surveillance for comorbidities is essential to improve overall health outcomes.

keywords:

celiac disease, immunoglobulin A, Turner syndrome, autoimmune disorders