Periodontal Ehlers–Danlos syndrome in early childhood: a case report

| Posted in: Science

Journal name: Journal of Indian Society of Periodontology
Original article title: Periodontal Ehlers–Danlos syndrome in early childhood: A case report of loss of deciduous teeth
The Journal of Indian Society of Periodontology (JISP) publishes original scientific articles on periodontology (the study of supporting structures of teeth) and oral implantology. It is a bimonthly open-access journal with special issues for specific occasions.
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Original source:

This page is merely a summary which is automatically generated hence you should visit the source to read the original article which includes the author, publication date, notes and references.

Author:

Ricardo Souza Martins, Francisco Wilker Mustafa Gomes Muniz, Juliana Oliveira Gondim, Luísa De Souza Maurique, Clélia Maria Nolasco-Lopes, Bibiana Mello De Oliveira, Rosimary De Sousa Carvalho


Journal of Indian Society of Periodontology:

(A bimonthly open-access journal)

Full text available for: Periodontal Ehlers–Danlos syndrome in early childhood: A case report of loss of deciduous teeth

Year: 2023 | Doi: 10.4103/jisp.jisp_266

Copyright (license): CC BY-NC-SA


Summary of article contents:

Introduction

This study presents a case of periodontal Ehlers–Danlos syndrome (pEDS) in a 3-year-old child who experienced severe tooth mobility and gingival bleeding leading to the early loss of deciduous teeth. Despite the rarity of periodontitis in children, especially as a manifestation of systemic disorders like EDS, this case underscores the complexities involved in diagnosing and managing dental issues associated with genetic conditions. The treatment implemented included strict biofilm control and periodontal maintenance, leading to further dental extractions and the introduction of scaling and root planing for the remaining teeth.

Impact of Ehlers–Danlos Syndrome on Periodontal Health

Ehlers–Danlos syndrome (EDS) is characterized by connective tissue fragility, primarily due to collagen metabolism disorders. The periodontal subtype (pEDS) of EDS manifests significant periodontal tissue destruction, leading to early tooth loss due to severe periodontitis. This case illustrates that pediatric patients with pEDS can exhibit profound periodontal challenges, requiring careful clinical management. The presentation of gingival recession and generalized severe periodontitis in the patient revealed the profound impact of EDS on dental health, as many affected individuals in her family experienced similar difficulties.

Conclusion

This report emphasizes the importance of a multidisciplinary approach in the management of pediatric patients with periodontal EDS, highlighting the need for strict oral hygiene protocols and regular follow-up to prevent disease recurrence. Given that EDS can significantly impact oral health, it is crucial to increase awareness among healthcare providers to ensure early diagnosis and tailored management strategies. Future research is warranted to further understand the implications of pEDS on dental health and improve treatment outcomes for affected individuals, thereby enhancing their quality of life.

FAQ section (important questions/answers):

What is periodontal Ehlers-Danlos syndrome (pEDS) in children?

Periodontal Ehlers-Danlos syndrome (pEDS) is a rare condition affecting connective tissue, primarily manifesting as severe periodontitis in children. Symptoms include tooth mobility, gingival bleeding, and early loss of teeth, usually associated with a family history of similar conditions.

How was the 3-year-old patient diagnosed with pEDS?

The diagnosis of pEDS was established based on the child's clinical symptoms, dental history, and family genetic predisposition to periodontal disease, as well as the observation of early tooth loss and other connective tissue-related symptoms.

What treatment was provided to the child with pEDS?

The treatment included the removal of non-salvageable teeth, strict supragingival biofilm control, and the implementation of a periodontal maintenance program. The child was also referred to a medical geneticist for further investigation of systemic conditions.

What role does genetic predisposition play in pEDS?

Genetic predisposition is significant in pEDS, as it is often inherited in an autosomal dominant pattern. Many family members may exhibit similar symptoms, indicating a hereditary link that affects connective tissue and periodontal health.

What are the expected outcomes for children with pEDS?

While pEDS is not curable, proper management through multidisciplinary care can control symptoms. Regular periodontal maintenance and oral hygiene are essential to prevent further periodontal issues and improve the child's overall quality of life.

Glossary definitions and references:

Scientific and Ayurvedic Glossary list for “Periodontal Ehlers–Danlos syndrome in early childhood: a case report”. This list explains important keywords that occur in this article and links it to the glossary for a better understanding of that concept in the context of Ayurveda and other topics.

1) Table:
A table is a structured arrangement of data, typically consisting of rows and columns. In the context of scientific articles, tables condense complex information, such as diagnostic criteria or results from medical tests, making it easier for readers to understand and reference vital data efficiently.

2) Disease:
Disease refers to an impairment of bodily functions or systems, often characterized by specific symptoms and signs. Understanding diseases, particularly those with systemic implications like periodontal Ehlers-Danlos syndrome, is crucial for diagnosis, treatment planning, and improving patient outcomes in health care.

3) Family:
Family units play a vital role in health and medical history as genetic disorders like Ehlers-Danlos syndrome can exhibit familial patterns. The study highlighted the importance of family histories in diagnosing conditions and understanding the inheritance of related health issues, thus facilitating targeted interventions.

4) Hygiene (Hygienic):
Hygiene encompasses practices that maintain health and prevent disease, particularly in oral health. In the context of periodontal disease, strict oral hygiene protocols are essential for managing symptoms and preventing exacerbation of conditions, ensuring better long-term dental health and quality of life for patients.

5) Study (Studying):
A study is a systematic exploration aimed at answering specific questions or hypotheses about particular phenomena. In this context, the study presented a case of periodontal Ehlers-Danlos syndrome in a child, contributing to the understanding and awareness of rare diseases impacting dental health.

6) Bleeding:
Bleeding, particularly in the context of gum health, indicates an underlying issue such as periodontal disease. It is a key symptom that practitioners assess to gauge oral health status, inform diagnosis, and tailor appropriate treatment, making it critical for understanding systemic and oral health interplay.

7) Blood:
Blood is critical for diagnosing systemic and genetic conditions, as laboratory analyses can reveal various health indicators. In the context of the study, blood tests were conducted to rule out other systemic issues associated with periodontal Ehlers-Danlos syndrome, emphasizing the role of blood in health diagnostics.

8) Accumulation (Accumulating, Accumulate):
Accumulation refers to the build-up of substances, such as biofilm or plaque on teeth, which can lead to periodontal diseases. Understanding the factors that contribute to biofilm accumulation is crucial in managing oral health and preventing the progression of periodontal conditions, particularly in vulnerable populations.

9) Inflammation:
Inflammation is the body's response to injury or infection and plays a pivotal role in the progression of periodontal diseases. In the context of the study, understanding inflammation can guide treatment approaches, highlighting the need for effective therapies to manage inflammatory responses in affected patients.

10) Mutation:
Mutation refers to alterations in genetic material, which can predispose individuals to conditions like Ehlers-Danlos syndrome. Identifying specific mutations aids in understanding the genetic basis of diseases, improving diagnosis accuracy, and informing treatment options tailored to the patient's unique genetic profile.

11) Relative:
In genetics, a relative is a family member whose health history may provide insight into hereditary conditions. The study demonstrated how assessing relatives' health can identify patterns of diseases, aiding in diagnosing and understanding Ehlers-Danlos syndrome-related conditions among multiple affected family members.

12) Quality:
Quality refers to the standard of health and well-being experienced by individuals. The study emphasizes how periodontal conditions can impair quality of life, reinforcing the importance of effective treatment strategies that enhance overall health outcomes and improve life quality for children with systemic disorders.

13) Rules:
Rules refer to guidelines that govern practice or behavior, such as dental hygiene protocols. In clinical contexts, adherence to established rules regarding treatment and care is significant for preventing complications in diseases like periodontal Ehlers-Danlos syndrome, ensuring patients receive the best evidence-based care.

14) Pur:
Poor indicates substandard conditions, which in this context relates to health outcomes or hygiene practices. The study highlights how poor oral hygiene can exacerbate periodontal disease symptoms, underlining the need for educational interventions to improve cleanliness and overall health in affected individuals.

15) Life:
Life in this context relates to the overall well-being and health quality of individuals affected by diseases such as Ehlers-Danlos syndrome. The impact that oral health has on life quality underscores the importance of comprehensive and ongoing management plans for holistic health improvement.

16) Discussion:
Discussion is a critical component of research reporting that evaluates findings within a broader context. In this study, the discussion synthesizes case observations, implications for treatment practices, and the relevance of recognizing systemic diseases in dental health to inform future research and clinical approaches.

17) Meeting:
Meeting refers to a formal gathering for discussion and decision-making. Collaborations among health professionals during meetings can lead to better patient management strategies for complex conditions like periodontal health, ensuring a multidisciplinary approach that enhances outcomes through shared knowledge and expertise.

18) Nature:
Nature refers to the inherent characteristics or qualities of something, such as genetic predispositions related to diseases. Understanding the nature of syndromes like periodontal Ehlers-Danlos aids health professionals in diagnosing, treating, and predicting health trajectories for affected individuals based on observed patterns in nature.

19) Visit:
A visit, particularly in healthcare, refers to an appointment between a patient and healthcare provider. The study highlights the significance of regular visits for monitoring treatment progress, emphasizing that frequency and quality of these interactions are crucial to managing conditions effectively, especially in pediatric cases.

20) Birth:
Birth signifies the beginning of life and can be significant in understanding hereditary conditions. In the context of Ehlers-Danlos syndrome, assessing birth histories, including premature births, among family members provides valuable diagnostic clues, enabling better understanding of genetic conditions and their prevalence.

21) Male:
Male refers to one of the two primary biological sexes, which may influence disease prevalence and presentation. The study indicates that Ehlers-Danlos syndrome affects all ethnic groups similarly, suggesting that both male and female patients require equal attention in research, diagnosis, and treatment considerations.

Other Science Concepts:

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Discover the significance of concepts within the article: ‘Periodontal Ehlers–Danlos syndrome in early childhood: a case report’. Further sources in the context of Science might help you critically compare this page with similair documents:

Early childhood, Multidisciplinary approach, Periodontitis, Tooth mobility, Gingival Recession, Deciduous Teeth, Dental extraction, Gingival bleeding, Clinical attachment loss, Gingivitis prevalence, Collagen metabolism, Periodontal maintenance program, Oral manifestation.

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