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Celiac disease and Turner syndrome
M Bonamico1, G Bottaro, A M Pasquino
1Department of Pediatrics, University La Sapienza, Rome, Italy.
Journal of Pediatric Gastroenterology and Nutrition
|May 20, 1998
Summary
Short stature is common in Turner syndrome (TS). Screening for celiac disease (CD) in TS patients is recommended, as CD may hinder growth hormone therapy effectiveness.
Area of Science:
- Endocrinology
- Gastroenterology
- Genetics
Background:
- Short stature is a hallmark of Turner syndrome (TS).
- Celiac disease (CD) can present as monosymptomatic short stature.
- The association between TS and CD requires further investigation.
Observation:
- Two antiendomysium antibody-positive TS girls unresponsive to growth hormone (GH) treatment prompted screening.
- 35 TS patients were screened for IgA/IgG antigliadin and antiendomysium antibodies.
- Intestinal biopsy confirmed subtotal villous atrophy in one patient.
Findings:
- The prevalence of CD in TS patients was high (8.1-10.8%).
- CD presented atypically, typically, or silently in affected TS patients.
- GH therapy was ineffective in two older TS patients with villous atrophy, but effective in a younger patient detected via screening.
Implications:
- Celiac disease may be associated with Turner syndrome.
- CD can cause growth hormone therapy failure in TS.
- Screening for CD with antiendomysium antibodies, followed by biopsy, is advisable for TS patients before GH treatment.