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Pedodontics

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.

Photostimulable Phosphors (PSPs) in Digital Imaging

  • Photostimulable phosphors (PSPs), also known as storage phosphors, are materials used in digital imaging for the acquisition of radiographic images. They serve as an alternative to traditional film-based radiography.

Characteristics of PSPs

  • Storage Mechanism: Unlike conventional screen materials used in panoramic or cephalometric imaging, PSPs do not fluoresce immediately upon exposure to x-ray photons. Instead, they capture and store the incoming x-ray photon information as a latent image.

  • Latent Image: The latent image is similar to that found in traditional film radiography, where the image is not visible until processed.

Image Acquisition Process

  1. Exposure:

    • The PSP plate is exposed to x-rays, which causes the phosphor material to absorb and store the energy from the x-ray photons.
  2. Scanning:

    • After exposure, the PSP plate is scanned by a laser beam in a drum scanner. This process is crucial for retrieving the stored image information.
  3. Energy Release:

    • The laser scanning excites the phosphor, causing it to release the stored energy as an electronic signal. This signal represents the latent image captured during the x-ray exposure.
  4. Digitalization:

    • The electronic signal is then digitized, with various gray levels assigned to different points on the curve. This process creates the final image information that can be viewed and analyzed.

Advantages of PSP Systems

  • Image Quality: PSPs can produce high-quality images with a wide dynamic range, allowing for better visualization of anatomical structures.

  • Reusability: PSP plates can be reused multiple times, making them a cost-effective option for dental practices.

  • Compatibility: PSP systems can be integrated into existing digital imaging workflows, providing flexibility for dental professionals.

Available PSP Imaging Systems

  • Soredex: OpTime
  • AirTechniques: Scan X
  • Gendex: Denoptix

These systems offer various features and capabilities, allowing dental practices to choose the best option for their imaging needs.

Classification of Early Childhood Caries (ECC)

  • Type 1 ECC (Mild to Moderate)

    • Affects molars and incisors
    • Typically seen in children aged 2-5 years
  • Type 2 ECC (Moderate to Severe)

    • Characterized by labiolingual caries affecting maxillary incisors, with or without molar involvement
    • Usually observed soon after the first tooth erupts
    • Mandibular incisors remain unaffected
    • Often caused by inappropriate bottle feeding
  • Type 3 ECC (Severe)

    • Involves all primary teeth
    • Commonly seen in children aged 3-5 years 

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.

Stainless Steel Crowns

Stainless steel crowns (SSCs) are a common restorative option for primary teeth, particularly in pediatric dentistry. They are especially useful for teeth with extensive carious lesions or structural damage, providing durability and protection for the underlying tooth structure.

Indications for Stainless Steel Crowns

  • Primary Incisors or Canines:
    • SSCs are indicated for primary incisors or canines that have extensive proximal lesions, especially when the incisal portion of the tooth is involved.
    • They are particularly beneficial in cases where traditional restorative materials (like amalgam or composite) may not provide adequate strength or longevity.

Crown Selection and Preparation

  1. Crown Selection:

    • An appropriate size of stainless steel crown is selected based on the dimensions of the tooth being restored.
  2. Contouring:

    • The crown is contoured at the cervical margin to ensure a proper fit and to minimize the risk of gingival irritation.
  3. Polishing:

    • The crown is polished to enhance its surface finish, which can help reduce plaque accumulation and improve esthetics.
  4. Cementation:

    • The crown is cemented into place using a suitable dental cement, ensuring a secure fit even on teeth that have undergone significant carious structure removal.

Advantages of Stainless Steel Crowns

  • Retention:
    • SSCs provide excellent retention and can remain in place even when extensive portions of carious tooth structure have been removed.
  • Durability:
    • They are highly durable and can withstand the forces of mastication, making them ideal for primary teeth that are subject to wear and tear.

Esthetic Considerations

  • Esthetic Limitations:

    • One of the drawbacks of stainless steel crowns is their metallic appearance, which may not meet the esthetic requirements of some children and their parents.
  • Open-Face Stainless Steel Crowns:

    • To address esthetic concerns, a technique known as the open-face stainless steel crown can be employed.
    • In this technique, most of the labial metal of the crown is cut away, creating a labial "window."
    • This window is then restored with composite resin, allowing for a more natural appearance while still providing the strength and durability of the stainless steel crown.

The American Academy of Pediatric Dentistry (AAPD) Caries Risk Assessment Tool is designed to evaluate a child's risk of developing dental caries (cavities). The tool considers various factors to categorize a child's risk level as low, moderate, or high.

Low Risk:
- No carious (cavitated) teeth in the past 24 months
- No enamel white spot lesions (initial stages of tooth decay)
- No visible dental plaque
- Low incidence of gingivitis (mild gum inflammation)
- Optimal exposure to fluoride (both systemic and topical)
- Limited consumption of simple sugars (at meal times only)

Moderate Risk:
- Carious teeth in the past 12 to 24 months
- One area of white spot lesion
- Gingivitis present
- Suboptimal systemic fluoride exposure (e.g., not receiving fluoride supplements or living in a non-fluoridated water area)
- One or two between-meal exposures to simple sugars

High Risk:
- Carious teeth in the past 12 months
- More than one area of white spot lesion
- Visible dental plaque
- Suboptimal topical fluoride exposure (not using fluoridated toothpaste or receiving professional fluoride applications)
- Presence of enamel hypoplasia (developmental defect of enamel)
- Wearing orthodontic or dental appliances that may increase caries risk
- Active caries in the mother, which can increase the child's risk due to oral bacteria transmission
- Three or more between-meal exposures to simple sugars

Digit Sucking and Infantile Swallow

Introduction to Digit Sucking

Digit sucking is a common behavior observed in infants and young children. It can be categorized into two main types based on the underlying reasons for the behavior:

  1. Nutritive Sucking

    • Definition: This type of sucking occurs during feeding and is essential for nourishment.
    • Timing: Nutritive sucking typically begins in the first few weeks of life.
    • Causes: It is primarily associated with feeding problems, where the infant may suck on fingers or digits as a substitute for breastfeeding or bottle-feeding.
  2. Non-Nutritive Sucking

    • Definition: This type of sucking is not related to feeding and serves other psychological or emotional needs.
    • Causes: Non-nutritive sucking can arise from various psychological factors, including:
      • Hunger
      • Satisfying the innate sucking instinct
      • Feelings of insecurity
      • Desire for attention
    • Examples: Common forms of non-nutritive sucking habits include:
      • Thumb or finger sucking
      • Pacifier sucking

Non-Nutritive Sucking Habits (NMS Habits)

  • Characteristics: Non-nutritive sucking habits are often comforting for children and can serve as a coping mechanism in stressful situations.
  • Implications: While these habits are generally normal in early childhood, prolonged non-nutritive sucking can lead to dental issues, such as malocclusion or changes in the oral cavity.

Infantile Swallow

  • Definition: The infantile swallow is a specific pattern of swallowing observed in infants.
  • Characteristics:
    • Active contraction of the lip musculature.
    • The tongue tip is positioned forward, making contact with the lower lip.
    • Minimal activity of the posterior tongue and pharyngeal musculature.
  • Posture: The tongue-to-lower lip contact is so prevalent in infants that it often becomes their resting posture. This can be observed when gently moving the infant's lip, causing the tongue tip to move in unison, suggesting a strong connection between the two.
  • Developmental Changes: The sucking reflex and the infantile swallow typically diminish and disappear within the first year of life as the child matures and develops more complex feeding and swallowing patterns.

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