Hypophosphatasia in Children

Hypophosphatasia is a rare genetic disorder characterized by defective mineralization of bones and teeth due to a deficiency in alkaline phosphatase, an enzyme crucial for bone mineralization. This condition can lead to various dental and skeletal abnormalities, particularly in children.

Clinical Findings

1. Premature Exfoliation of Primary Teeth:

   • One of the hallmark clinical findings in children with hypophosphatasia is the premature loss of anterior primary teeth.
   • This loss is associated with deficient cementum, which is the tissue that helps anchor teeth to the alveolar bone.
   • Teeth may be lost spontaneously or as a result of minor trauma, highlighting the fragility of the dental structures in affected children.

2. Absence of Severe Gingival Inflammation:

   • Unlike other dental conditions that may cause tooth mobility or loss, severe gingival inflammation is typically absent in hypophosphatasia.
   • This absence can help differentiate hypophosphatasia from other periodontal diseases that may present with similar symptoms.

3. Limited Alveolar Bone Loss:

   • The loss of alveolar bone associated with hypophosphatasia may be localized, often limited to the anterior region where the primary teeth are affected.

Pathophysiology

• Deficient Alkaline Phosphatase Activity:

  • The disease is characterized by improper mineralization of bone and teeth due to deficient alkaline phosphatase activity in various tissues, including serum, liver, bone, and kidney (tissue nonspecific).
  • This deficiency leads to inadequate mineralization, resulting in the clinical manifestations observed in affected individuals.

• Increased Urinary Phosphoethanolamine:

  • Patients with hypophosphatasia often exhibit elevated levels of urinary phosphoethanolamine, which can serve as a biochemical marker for the condition.

Autism in Pedodontics

Autism Spectrum Disorder (ASD) is a complex developmental disorder that affects communication, behavior, and social interaction. In the context of pediatric dentistry (pedodontics), understanding the characteristics and challenges associated with autism is crucial for providing effective dental care. Here’s an overview of autism in pedodontics:

Characteristics of Autism

1. Developmental Disability:

   • Autism is classified as a lifelong developmental disability that typically manifests during the first three years of life. It is characterized by disturbances in mental and emotional development, leading to challenges in learning and communication.

2. Diagnosis:

   • Diagnosing autism can be difficult due to the variability in symptoms and behaviors. Early intervention is essential, but many children may not receive a diagnosis until later in childhood.

3. Symptoms:

   • Poor Muscle Tone: Children with autism may exhibit low muscle tone, which can affect their physical coordination and ability to perform tasks.
   • Poor Coordination: Motor skills may be underdeveloped, leading to difficulties in activities that require fine or gross motor skills.
   • Drooling: Some children may have difficulty with oral motor control, leading to drooling.
   • Hyperactive Knee Jerk: This may indicate neurological differences that can affect overall motor function.
   • Strabismus: This condition, characterized by misalignment of the eyes, can affect visual perception and coordination.

4. Feeding Behaviors:

   • Children with autism may exhibit atypical feeding behaviors, such as pouching food (holding food in the cheeks without swallowing) and a strong preference for sweetened foods. These behaviors can lead to dietary imbalances and increase the risk of dental caries (cavities).

Dental Considerations for Children with Autism

1. Communication Challenges:

   • Many children with autism have difficulty with verbal communication, which can make it challenging for dental professionals to obtain a medical history, understand the child’s needs, or explain procedures. Using visual aids, simple language, and non-verbal communication techniques can be helpful.

2. Behavioral Management:

   • Children with autism may exhibit anxiety or fear in unfamiliar environments, such as a dental office. Strategies such as desensitization, social stories, and positive reinforcement can help reduce anxiety and improve cooperation during dental visits.

3. Oral Health Risks:

   • Due to dietary preferences for sweetened foods and potential difficulties with oral hygiene, children with autism are at a higher risk for dental caries. Dental professionals should emphasize the importance of oral hygiene and may need to provide additional support and education to caregivers.

4. Special Accommodations:

   • Dental offices may need to make accommodations for children with autism, such as providing a quiet environment, allowing extra time for appointments, and using calming techniques to help the child feel more comfortable.

CARIDEX and CARISOLV

CARIDEX and CARISOLV are both dental products designed for the chemomechanical removal of carious dentin. Here’s a detailed breakdown of their components and mechanisms:

CARIDEX

• Components:

  • Solution I: Contains sodium hypochlorite (NaOCl) and is used for its antimicrobial properties and ability to dissolve organic tissue.
  • Solution II: Contains glycine and aminobutyric acid (ABA). When mixed with sodium hypochlorite, it produces N-mono chloro DL-2-amino butyric acid, which aids in the removal of demineralized dentin.

• Application:

  • CARIDEX is particularly useful for deep cavities, allowing for the selective removal of carious dentin while preserving healthy tooth structure.

CARISOLV

• Components:

  • Syringe 1: Contains sodium hypochlorite at a concentration of 0.5% w/v (which is equivalent to 0.51%).
  • Syringe 2: Contains a mixture of amino acids (such as lysine, leucine, and glutamic acid) and erythrosine dye, which helps in visualizing the removal of carious dentin.

• pH Level:

  • The pH of the CARISOLV solution is approximately 11, which helps in the dissolution of carious dentin.

• Mechanism of Action:

  • The sodium hypochlorite in CARISOLV softens and dissolves carious dentin, while the amino acids and dye provide a visual cue for the clinician. The procedure can be stopped when discoloration is no longer observed, indicating that all carious dentin has been removed.

Conditioning and Behavioral Responses

This section outlines key concepts related to conditioning and behavioral responses, particularly in the context of learning and emotional responses in children.

1. Acquisition

• Acquisition refers to the process of learning a new response to a stimulus through conditioning. This is the initial stage where an association is formed between a conditioned stimulus (CS) and an unconditioned stimulus (US).
• Example: A child learns to associate the sound of a bell (CS) with receiving a treat (US), leading to a conditioned response (CR) of excitement when the bell rings.

2. Generalization

• Generalization occurs when the conditioned response is evoked by stimuli that are similar to the original conditioned stimulus. This means that the learned response can be triggered by a range of similar stimuli.
• Example: If a child has a painful experience with a doctor in a white coat, they may generalize this fear to all doctors in white coats, regardless of the specific individual or setting. Thus, any doctor wearing a white coat may elicit a fear response.

3. Extinction

• Extinction is the process by which the conditioned behavior diminishes or disappears when the association between the conditioned stimulus and the unconditioned stimulus is no longer reinforced.
• Example: In the previous example, if the child visits the doctor multiple times without any unpleasant experiences, the fear associated with the doctor in a white coat may gradually extinguish. The lack of reinforcement (pain) leads to a decrease in the conditioned response (fear).

4. Discrimination

• Discrimination is the ability to differentiate between similar stimuli and respond only to the specific conditioned stimulus. It is the opposite of generalization.
• Example: If the child is exposed to clinic settings that are different from those associated with painful experiences, they learn to discriminate between the two environments. For instance, if the child visits a friendly clinic with a different atmosphere, they may no longer associate all clinic visits with fear, leading to the extinction of the generalized fear response.

Devitalisation Pulpotomy (Two-Stage Procedure)

The two-stage devitalisation pulpotomy is a dental procedure aimed at treating exposed primary pulp tissue. This technique involves the use of paraformaldehyde to fix both coronal and radicular pulp tissues, ensuring effective devitalization. The medicaments employed in this procedure possess devitalizing, mummifying, and bactericidal properties, which are crucial for the success of the treatment.

Key Features of the Procedure:

• Two-Stage Approach: The procedure is divided into two stages, allowing for thorough treatment of the pulp tissue.
• Use of Paraformaldehyde: Paraformaldehyde is a key component in the medicaments, providing effective fixation and devitalization of the pulp.
• Medicaments: The following formulations are commonly used in the procedure:

Medicament Formulations:

1. Gysi Triopaste:

   • Tricresol: 10 ml
   • Cresol: 20 ml
   • Glycerin: 4 ml
   • Paraformaldehyde: 20 ml
   • Zinc Oxide: 60 g

   Gysi Triopaste is known for its strong devitalizing and bactericidal effects, making it effective for pulp treatment.

2. Easlick’s Paraformaldehyde Paste:

   • Paraformaldehyde: 1 g
   • Procaine Base: 0.03 g
   • Powdered Asbestos: 0.05 g
   • Petroleum Jelly: 125 g
   • Carmine (for coloring)

   This paste combines paraformaldehyde with a local anesthetic (Procaine) to enhance patient comfort during the procedure.

3. Paraform Devitalizing Paste:

   • Paraformaldehyde: 1 g
   • Lignocaine: 0.06 g
   • Propylene Glycol: 0.50 ml
   • Carbowax 1500: 1.30 g
   • Carmine (for coloring)

   This formulation also includes Lignocaine for local anesthesia, providing additional comfort during treatment.

Esthetic Preformed Crowns in Pediatric Dentistry

Esthetic preformed crowns are an important option in pediatric dentistry, providing a functional and aesthetic solution for restoring primary teeth. Here’s a detailed overview of various types of esthetic crowns used in children:

i) Polycarbonate Crowns

• Advantages:
  • Save time during the procedure.
  • Easy to trim and adjust with pliers.
• Usage: Often used for anterior teeth due to their aesthetic appearance.

ii) Strip Crowns

• Description: These are crown forms that are filled with composite material and bonded to the tooth. After polymerization, the crown form is removed.
• Advantages:
  • Most commonly used crowns in pediatric dental practice.
  • Easy to repair if damaged.
• Usage: Ideal for anterior teeth restoration.

iii) Pedo Jacket Crowns

• Material: Made of tooth-colored copolyester material filled with resin.
• Characteristics:
  • Left on the tooth after polymerization instead of being removed.
  • Available in only one shade.
  • Cannot be trimmed easily.
• Usage: Suitable for anterior teeth where aesthetics are a priority.

iv) Fuks Crowns

• Description: These crowns consist of a stainless steel shell sized to cover a portion of the tooth, with a polymeric coating made from a polyester/epoxy hybrid composition.
• Advantages: Provide a durable and aesthetic option for restoration.

v) New Millennium Crowns

• Material: Made from laboratory-enhanced composite resin material.
• Characteristics:
  • Bonded to the tooth and can be trimmed easily.
  • Very brittle and more expensive compared to other options.
• Usage: Suitable for anterior teeth requiring esthetic restoration.

vi) Nusmile Crowns

• Indication: Indicated when full coverage restoration is needed.
• Characteristics: Provide a durable and aesthetic solution for primary teeth.

vii) Cheng Crowns

• Description: Crowns with a pure resin facing that makes them stain-resistant.
• Advantages:
  • Less time-consuming and typically requires a single patient visit.
• Usage: Suitable for anterior teeth restoration.

viii) Dura Crowns

• Description: Pre-veneered crowns that can be placed even with poor moisture or hemorrhage control.
• Challenges: Not easy to fit and require a longer learning curve for proper placement.

ix) Pedo Pearls

• Material: Aluminum crown forms coated with a tooth-colored epoxy paint.
• Characteristics:
  • Relatively soft, which may affect long-term durability.
• Usage: Used for primary teeth restoration where aesthetics are important.