Estimation of Incidence and Prevalence of Pediatric Channelopathies in a Mediterranean Population Based on a Single-Center, Retrospective Analysis
Highlights
- Long QT syndrome was the predominant channelopathy;
- Affected children represented the first diagnoses within families in half of the cases;
- Genetic testing was positive in 77% when performed;
- The epidemiology of pediatric channelopathies on the Mediterranean island of Crete seems similar to that reported in the literature, with regional clusters of increased prevalence.
- Screening children for channelopathies could potentially improve the detection of affected families;
- Knowledge of the epidemiology in selected populations can be helpful for the best design of large-scale screening programs in the pediatric population.
Abstract
1. Introduction
2. Materials and Methods
2.1. Study Population
2.2. Data Sources
2.3. Epidemiologic Data
2.4. Statistical Analysis
3. Results
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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| Diagnostic Criteria | |
|---|---|
| Long QT Syndrome |
|
| Brugada Syndrome |
|
| Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) |
|
| Short QT Syndrome |
|
| Cases (N) | Incidence/ 100,000 Children < 18 Y/Year (95% C.I.) | Prevalence/ 100,000 Children < 18 Y (95% C.I.) | |
|---|---|---|---|
| Total | 43 | 1.43 (1.03–1.92) | 31.1 (22.1–42.5) |
| LQTS | 38 | 1.26 (0.9–1.7) | 30.3 (21.4–41.6) |
| BrS | 3 | 0.09 (0.02–0.3) | 2.4 (0.5–6.9) |
| CPVT | 2 | 0.06 (0.01–0.2) | 0.8 (0.02–4.4) |
| Long QT Syndrome | Schwartz [2] Circulation 2009 | Nosetti [31] Clinics and Practice 2024 | Yoshinaga [32] Circ Arrhythm Electroph 2013 | Simma [33] Neonatology 2020 | Hayashi [34] Clinical Science 2009 | Yoshinaga [11] European Heart Journal 2016 | |
|---|---|---|---|---|---|---|---|
| Retrospective/prospective | Prospective | Retrospective | Prospective | Prospective | Prospective | Prospective | |
| Duration of study | 2001–2006 | 2001–2017 | 2010–2011 | 2015–2018 | 2004–2005 | 2008–2013 | |
| Methodology | Neonatal ECG screening | Neonatal ECG screening | Neonatal ECG screening | Neonatal ECG screening | Population-based ECG screening | Population-based ECG screening | |
| Country | Italy | Italy | Japan | Germany Regensburg | Japan Kanazawa | Japan Kagoshima | |
| Age of children | 15–25 days | 20–40 days | 4 weeks | 27 days | 6–12 years | 6 years | 12 years |
| Number of children screened | 44,596 infants | 42,200 neonates/young infants | 4285 young infants | 2251 neonates | 7961 school-aged children | 32,982 1st-grade students | 34,572 7th-grade students |
| LQTS cases (positive diagnostic criteria) | 17 | 27 | 1 | 2 | 3 | 10 | 32 |
| Prevalence (cases per 100,000) | 38 | 42 | 23.3 | 88 | 37.6 | 30 | 93 |
| Sex distribution (boys/girls) (%) | 51/49 | 40/60 | 60/40 | 51/49 | 51/49 | 50/50 | 47/53 |
| Crete Total Cases/ Average Incidence (95% C.I.) | Western Region Cases/ Average Incidence (95% C.I.) | Middle-West Cases/ Average Incidence (95% C.I.) | Central Region Cases/ Average Incidence (95% C.I.) | Eastern Region Cases/ Average Incidence (95% C.I.) | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Total | N = 43 | 1.43 (1.03–1.92) | N = 6 | 0.81 (0.3–1.7) | N = 11 | 2.46 * (1.2–4.4) p = 0.02 | N = 25 | 1.66 (1.1–2.4) | N = 1 | 0.3 (0.008–1.8) |
| LQTS | N = 38 | 1.26 (0.9–1.7) | N = 6 | 0.81 (0.3–1.7) | N = 10 | 2.24 * (1.1–4.1) p = 0.04 | N = 21 | 1.4 (0.9–2.1) | N = 1 | 0.3 (0.008–1.8) |
| BrS | N = 3 | 0.09 (0.02–0.3) | N = 0 | N = 0 | N = 3 | 0.2 (0.04–0.6) | N = 0 | |||
| CPVT | N = 2 | 0.07 (0.01–0.2) | N = 0 | N = 1 | 0.22 (0.005–1.2) | N = 1 | 0.06 (0.001–0.3) | N = 0 | ||
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Bagkaki, A.; Papagiannis, J.; Anastasakis, A.; Parthenakis, F.; Chlouverakis, G.; Galanakis, E.; Germanakis, I. Estimation of Incidence and Prevalence of Pediatric Channelopathies in a Mediterranean Population Based on a Single-Center, Retrospective Analysis. Children 2026, 13, 659. https://doi.org/10.3390/children13050659
Bagkaki A, Papagiannis J, Anastasakis A, Parthenakis F, Chlouverakis G, Galanakis E, Germanakis I. Estimation of Incidence and Prevalence of Pediatric Channelopathies in a Mediterranean Population Based on a Single-Center, Retrospective Analysis. Children. 2026; 13(5):659. https://doi.org/10.3390/children13050659
Chicago/Turabian StyleBagkaki, Alena, John Papagiannis, Aris Anastasakis, Fragiskos Parthenakis, Gregory Chlouverakis, Emmanuil Galanakis, and Ioannis Germanakis. 2026. "Estimation of Incidence and Prevalence of Pediatric Channelopathies in a Mediterranean Population Based on a Single-Center, Retrospective Analysis" Children 13, no. 5: 659. https://doi.org/10.3390/children13050659
APA StyleBagkaki, A., Papagiannis, J., Anastasakis, A., Parthenakis, F., Chlouverakis, G., Galanakis, E., & Germanakis, I. (2026). Estimation of Incidence and Prevalence of Pediatric Channelopathies in a Mediterranean Population Based on a Single-Center, Retrospective Analysis. Children, 13(5), 659. https://doi.org/10.3390/children13050659

