Periodontitis as a Manifestation of Systemic Disease Flashcards

1
Q

Periodontitis can affect the ______

Likewise systemic conditions & disease can affect the _____

A

rest of body

peridontium

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2
Q

Periodontal diseases & systemic disorders have three common contributing factors including:

A
  1. environmental factors
  2. host factors
  3. microbial factors
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3
Q

What do we mean by:

“Periodontitis as a manifestation of a systemic disease”

A

Meaning systemic disease is altering the course of periodontitis

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4
Q

How do systemic disorders result in a loss of periodontal tissues?

A
  1. influence periodontal inflammation
  2. influence the pathogenesis of periodontal diseases
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5
Q

Major impact of systemic diseases on periodontal health:

A

severe/early-onset periodontitis

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6
Q

Moderate impact of systemic diseases on periodontal health:

A
  1. increase prevalence
  2. no otherwise different clinical presentations of chronic periodontitis
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7
Q

Systemic disorders that have a major impact on the loss of periodontal tissue by influencing periodontal inflammation:

A
  1. Genetic disorders
  2. Acquired immunodeficiency diseases
  3. Inflammatory diseases
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8
Q

List the categories of genetic disorders (that affect the periodontium):

A
  1. diseases associated with immunologic disorders
  2. diseases affecting the oral mucosa & gingival tissue
  3. diseases affecting the connective tissues
  4. metabolic & endocrine disorders
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9
Q

Diseases associated with immunologic disorders (that affect the periodontium):

A
  1. down syndrome
  2. leukocyte adhesion deficiency
  3. papillon lefevre syndrome
  4. chediak higashi syndrome
  5. congenital neutropenia
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10
Q

Down syndrome may also be called:

A

Trisomy 21 or mongolism

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11
Q

Down syndrome is characterized by:

A
  1. characteristic physical appearance
  2. mental deficiency
  3. growth retardation
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12
Q

List some physical characteristics associated with Down syndrome:

A
  1. small nose, mouth & ears
  2. slightly bent 5th finger (pinky)
  3. short, stocky build
  4. almond-shaped eyes with skin fold covering inner corner
  5. white spots on colored part of eyes
  6. flat, round face
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13
Q

What chromosome is affected in Down syndrome?

A

21st

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14
Q

Describe periodontitis in an individual with Down syndrome:

A

moderate to severe periodontitis with rapid progression

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15
Q

Describe the progression of periodontist in an individual with down syndrome:

A

rapid progression

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16
Q

T/F: in an individual with down syndrome, local factors alone explain the severity of periodontal destruction

A

False- local factors alone FAIL TO EXPLAIN the severity of periodontal destruction

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17
Q

Describe the affects of down syndrome on the immune system:

List some examples: (3)

A

Intrinsic immune system defects

  1. poor PMN chemotaxis
  2. poor phagocytosis
  3. poor intracellular killing
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18
Q

Caused by a mutation in beta-2 integrin (ITGB2) gene:

A

Leukocyte adhesion deficiency

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19
Q

What gene is affected in leukocyte adhesion deficiency?

A

Beta-2 integrin (ITGB2)

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20
Q

In LAD, there is a mutation in Beta-2 integrin (ITFB2), describe what this mutation causes: (2)

A
  1. lack of beta-2 integrin mRNA in leukocytes
  2. low integrin (CD18 or CD15) expression in neutrophils
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21
Q

What happens to the neutrophils in LAD? What does this result in?

A

Neutrophils are confined to blood vessels resulting in disruption of neutrophil-associated homeostasis

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22
Q

In individuals affected by this disease we often see a history of severe recurrent infection with no pus; leukocytosis is common:

A

LAD

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23
Q

In individuals with LAD, we often see a history of _____. ______ is common.

A

Severe recurrent infection but no pus; leukocytosis

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24
Q

Describe the oral manifestations of LAD:

A
  1. Severe gingival inflammation
  2. aute gingival lesions
  3. early on-set & rapidly progressive alveolar bone loss
  4. early loss of the primary & permanent teeth
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25
Q

This image shows oral findings in a 6-year old bow with _____. Note diastema, gingival retraction, and partial root exposure with cervical erosions affecting primary dentition. Permanent lower incisors are erupting.

A

LAD1

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26
Q

Caused by a mutation in the cathepsin C gene (CTSC):

A

Papillon-Lefévre Syndrome

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27
Q

What gene is mutated in Papillon-Lefévre Syndrome? What chromosome is this located on?

A

Cathepsin C gene (CTSC); Chromosome 11q14

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28
Q

In Papillon-Lefévre Syndrome, these patients expérience compromised _____ function, resulting in ____.

A

neutrophil; decreased phagocytosis

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29
Q

What characteristic lesion is seen in Papillon-Lefévre Syndrome? What are some common locations for this lesion?

A

Hyperkeratotic lesions
-palms
-soles of the feet
-elbows
-knees

30
Q

The following images are characteristic of:

A

Papillon-Lefévre Syndrome

31
Q

Describe the oral manifestations of Papillon-Lefévre Syndrome: (3)

A
  1. severe gingival inflammation
  2. early-onset and rapidly progressive alveolar bone loss
  3. early loss of the primary & permanent teeth
32
Q

Case Study:
A 10yo male child presented with what appeared to be severe generalized aggressive periodontitis. His parents presented no contributory medical history except for skin lesions that had been managed by his medical doctor since birth,

CC: Parents complaining of child’s bad breath & loose teeth

History of main complaint: 1 year. Previously attended a private dentist who had done cleanings & prescribed antibiotics

Medical history: NKDA, med history unknown
-eczema on hands and feet from an early age, been treated by general practitioner with aqueous cream since early childhood

Family history: NC

Dental history & habits: Patient brushes 1x per day, no other dental aids used

(Initial presentation seen in image)

A

Diagnosis: Papillon-Lefévre Syndrome

33
Q

Disease caused by mutations in CHS1 LYST gene:

A

Chediak-Highashi Syndrome

34
Q

Chediak-Higashi Syndrome is caused by a mutations in:

A

CHS1 LYST gene

35
Q

The mutation in Chediak-Higashi Syndrome (CHS1 LYST gene) results in what lysosomal defect?

A

Lysosomal trafficking defect

36
Q

In Chediak-Higashi Syndrome, defects in melanin granules results in:

A

partial oculocutaneous albinims

37
Q

What results in the partial oculocutaneous albinisim in Chediak-Higashi Syndrome?

A

Defects in melanin granules

38
Q

In Chediak-Higashi Syndrome the circulating leukocytes exhibit defective lysosomes causing a decrease in phagocytosis. Clinically, what does this cause?

A

Recurrent pyogenic infections

39
Q

In Chediak-Higashi Syndrome, we clinically see recurrent pyogenic infections. What is this caused by?

A

Circulating leukocytes exhibit defective lysosomes resulting in a decrease in phagocytosis

40
Q

Chediak-Higashi Syndrome is associated with varying neurologic problems including:

A
  1. intellectual deficit
  2. dementia
41
Q

Describe the oral manifestations of Chediak-Higashi Syndrome:

A
  1. severe gingival inflammation
  2. early-onset & rapidly progressive alveolar bone loss
  3. early loss of the primary & permanent teeth
  4. poor response to dental treatment
42
Q

Caused by mutations in the ELANE (50%) & HAX1 (10%) gene:

A

Congenital neutropenia

43
Q

Congenital neutropenia is caused by a mutation in the _____ gene 50% of the time and the _____ gene 10% of the time

A

ELANE; HAX1

44
Q

In Congenital neutropenia _____ of the causes remain unknown

A

1/3

45
Q

Congenital neutropenia results in a ____ number of ____

A

decrease; neutrophils

46
Q

What neutrophil value is considered “neutropenic”?

A

ANC <500 cell/uL and static

47
Q

The decreased number of neutrophils in congenital neutropenia results in: (2)

A
  1. deficiency in the immune response
  2. severe & recurrent infections
48
Q

Describe the oral manifestations of congenital neutropenia:

A
  1. severe periodontitis is common
  2. higher risk for tooth loss
  3. oral ulcers
49
Q

Epidermolysis bullosa (Kindler syndrome) is a disease affecting the _____ & ____

A

oral mucosa & gingival tissue

50
Q

Epidermolysis bullosa (Kindler syndrome) is caused by a mutation in the:

A

Fermitin family homologue 1 gene

51
Q

Disease caused by a mutation in the Fermitin family homologue 1 gene:

A

Epidermolysis bullosa (Kindler syndrome)

52
Q

Epidermolysis bullosa (Kindler syndrome) is caused by a mutation in the ferritin family homologue 1 gene that causes:

  1. A lack of ______
  2. Affects _______
  3. Can lead to molecular defects in the ______
A
  1. integrin activation
  2. keratinocyte adhesion
  3. basement membrane zone
53
Q

Epidermolysis bullosa (Kindler syndrome) is caused by a mutation in the ferritin family homologue 1 gene that causes:

  1. A lack of integrin activation
  2. Affects keratinocyte adhesion
  3. Can lead to molecular defects in the basement membrane zone

All of these contribute clinically to:

A
  1. photosensitivity
  2. recurrent blister formation
54
Q

The following images show symptoms characteristic of:

A

Epidermolysis bullosa (Kindler syndrome)

55
Q

Ehlers-Danlos syndrome is categorized as a disease affecting the:

A

connective tissue

56
Q

Caused by mutations in genes coding fibrillar collagens or enzymes involved in the biosynthesis of these proteins:

A

Ehlers-Danlos syndrome

57
Q

The mutations in genes coding fibrillar collagens or enzymes involved in the biosynthesis of these proteins in Ehlers-Danlos syndrome affects:

A

collagen production & compositions

58
Q

In Ehlers-Danlos syndrome the collagen production and compositions are affected mainly in:

A
  1. joints
  2. skin
  3. walls of blood vessels
59
Q

What is clinically seen in Ehlers-Danlos syndrome? (3)

A
  1. joint hypermobility
  2. skin extensibility
  3. tissue fragility
60
Q

The following images are characteristic of:

A

Ehlers-Danlos syndrome

61
Q

What is the MAIN oral manifestation seen in Ehlers-Danlos syndrome? What are some others seen?

A
  1. GENERALIZED LACK OF ATTACHED GINGIVA
  2. generalized, early-onset severe periodontitis
  3. gingival recession
  4. early loss of primary & permanent teeth
62
Q

Hypophosphatasia is categorized as a:

A

metabolic and endocrine disorder

63
Q

Caused by a mutation in the gene alkaline phosphatase (ALPL):

A

Hypophosphatasia

64
Q

Hypophosphatasia is caused by a mutation in:

A

Alkaline phosphatase (ALPL) gene

65
Q

There are both _____ & ____ forms of hypophosphatasia

A

mild & severe

66
Q

Describe the oral manifestations of hypophosphatasia:
(3)

A
  1. defective cementum
  2. alveolar bone loss
  3. premature loss of teeth
  4. impaired bone/tooth mineralization
  5. compromised periodontal attachment
67
Q

In hypophosphatasia, patients have a compromised _____, resulting in reduction of the alveolar bone height

A

compromised periodontal attachment

68
Q

In hypophosphatasia, the teeth are not adequately:

A

anchored to the alveolar bone via the PDL

69
Q

Oral signs of HPP (common dental signs): (6)

A
  1. exfoliation of the primary dentition before the age of three
  2. exfoliation of the primary teeth with roots intact
  3. alveolar bone loss
  4. loss of permanent teeth with no signs of periodontal inflammation
  5. enlarged pulp chambers and root canals
  6. thin dentinal walls
70
Q

Disorder of glucose metabolism:

A

diabetes mellitus

71
Q

AGEs:

A

advanced glycylation end products

-where there is excess amount of glucose in the circulation, everything gets glycated (once glycated its no longer a simple molecule)

72
Q
A