Introduction
With a prevalence of 1-2%, celiac disease (CD) is one of childhood’s most common chronic diseases. Clinical follow-up of CD aims to ensure adherence to a strict gluten-free diet, monitor symptoms and prevent disease-related complications. However, few studies have examined health outcomes of children who lack CD follow-up. Related to this knowledge gap, there have until recently been no evidence-based pediatric guidelines on CD follow-up.
Aims & Methods
To examine the association between the lack of follow-up for celiac disease (CD) during childhood and dietary adherence, disease remission status, and health-related quality of life (HRQoL).
In a cohort of 162 children diagnosed with CD in 2013-2018 in Gothenburg, Sweden we retrieved information on clinical follow-up and current wellbeing using data from medical and laboratory records, as well as validated questionnaires on symptoms of CD, dietary adherence, and HRQoL. We analyzed tissue-transglutaminase antibodies (tTGA) as a measure of disease remission. We defined lack of follow-up as no CD-related physician/dietician-led visit or measurement of tTGA over the past 24 months of study enrollment.
Results
The mean age at study assessment was 12.7 (range 7.8-18.2) years. Out of 162 children with an average disease duration of 5.3 (range 2.3-8.8) years, 23 (14%) lacked follow-up. The tTGA had normalized (<7/ml) in 94% (95% confidence interval [CI]=71-100) of children without follow-up versus 91% (95% CI=85-95%) of children with continued follow-up. Of children without follow-up, 65% (95% CI=38-86%) reported a dietary adherence score indicating very good adherence, versus 72% (95% CI=63-80%) of those with continued follow-up. Mean dietary adherence scores were similar between groups (p=0.93). Also, lack of follow-up was not significantly associated with growth, symptom scores, or HRQoL.
Variable n(%)
| Total
(n=162)
| Continued follow-up
(n=139)
| Lack of follow-up
(n=23)
| p-value
|
|---|
| Age, years at study enrollment Mean (SD), range | 12.7 (2.8) 7.8-18.2
| 12.7 (2.6) 8.0-18.2
| 13.2 (3.6) 7.8-18.2
| 0.68
|
Years since CD diagnosis
Mean (SD), range
| 5.3 (1.7) 2.3-8.8
| 5.2 (1.8) 2.3-8.8
| 5.8 (1.6) 3.1-8.1
| 0.11 |
| Sex (Girls) | 94 (58%)
| 85 (61%)
| 9 (39%)
| 0.08
|
Biopsy-verified CD diagnosis
| 65/162 (40%)
| 60/139 (43%)
| 5/23 (22%)
| 0.08
|
Dietary adherence (CDAT) score, mean (SD)
|
| 11 (3.4)
| 11 (3.4)
| 0.93
|
Gastrointestinal Rating Scale (GSRS)Total score, mean (SD)
|
| 1.7 (0.7)
| 1.5 (0.6)
| 0.13
|
KIDSCREEN-27 Raw score; median (min; max)
|
| 110 (69; 134)
| 115 (87; 134)
| 0.55
|
CDDUX total score, mean (SD)
|
| 2.4 (0.8)
| 2.4 (0.86)
| 0.91
|
| tTGA* negative <7 U/mL n(%; 95% CI) |
| 91 (91%; 84-96%)
| 16 (94%; 71-100%)
| 0.58**
|
Teble legend: Lack of follow-up was defined as no registered visit for CD or tTGA over the past 24 months of study enrollment. *
Including ten children with a tTGA measurement recorded within 30 days of study enrolment. **Refers to the distribution of negative <7, positive 7-100 and positive >100 U/ml between the two groups.
The Swedish version of CDAT was used to assess gluten-free dietary adherence.
1, 2 A score of <13 has been considered indicative of a very good adherence (corresponding to standardized dietician evaluation: consumes gluten <3 times/year, reads labels well, cross-contamination potential eliminated in the kitchen).
2 Scores >17 are indicative of poor adherence (corresponding to standardized dietician evaluation: gluten consumption 1-2/week, does not check medications/supplements, cheats regularly and intentionally [1x/week]).
2 Scores of 13-17 are inconclusive.
2KIDSCREEN-27 sub-scores have been transformed into T-scores. A mean of 50 (standard deviation, SD) +/-10 defines normality for European children aged 8-18 years. A higher score indicates a better HRQoL. A between-group-difference of 0.5 SD or a difference of 0.5 SD from European/National norms, is considered noticeable.
3CDDUX ranges from 1 to 5. Higher scores indicate better CD-related HRQoL.
4Conclusion
In this regional cohort study of mostly oder children and adolescents, lack of follow-up for CD was not significantly linked to dietary adherence, disease remission status, or HRQoL. How these results hold in larger samples with longer follow-ups, including the transition to adult care, warrants further study.
References
1. Johansson K, Norström F, Nordyke K, et al. Celiac Dietary Adherence Test simplifies Determining Adherence to a Gluten-free Diet in Swedish Adolescents. J Pediatr Gastroenterol Nutr 2019;69:575-580.
2. Leffler DA, Dennis M, Edwards George JB, et al. A simple validated gluten-free diet adherence survey for adults with celiac disease. Clin Gastroenterol Hepatol 2009;7:530-6, 536.e1-2.
3. Ravens-Sieberer U, Europe KG. The Kidscreen questionnaires: quality of life questionnaires for children and adolescents; handbook: Pabst Science Publ., 2006.
4. van Doorn RK, Winkler LM, Zwinderman KH, et al. CDDUX: a disease-specific health-related quality-of-life questionnaire for children with celiac disease. J Pediatr Gastroenterol Nutr 2008;47:147-52.