High Yield: Periodontitis as a Manifestation of Systemic Disease Flashcards
T/F: Diseases and conditions can affect the periodontal tissues by affecting the periodontal supporting tissues INDEPENDENTLY of dental-plaque biofilm-induced inflammation
True
Systemic disorders that have a major impact on the loss of periodontal tissue by influencing periodontal inflammation include: (3)
- genetic disorders
- AIDs
- Inflammatory diseases
-trisomy 21 or mongolism
-characteristic physical appearance
-mental deficiency & growth retardation
Down syndrome
List some of the physical characteristics of down syndrome:
- small nose, mouth & ears
- slightly bent fifth finger (pinky)
- short, stocky build
- almond-shaped eyes with skin fold covering inner corner
- white spots on colored part of eyes
- flat, round face
Give another name for down syndrome:
- mongolism
- trisomy 21
Describe how periodontal disease is categorized in a patient with down syndrome:
moderate to sever periodontitis with rapid progression
T/F: In down syndrome, local factors alone explain the severity of periodontal destruction
False- local factors alone FAIL to explain the severity of periodontal destruction
What aspects of down syndrome might contribute to the moderate to severe periodontal destruction with rapid progression:
INTRINSIC IMMUNE SYSTEM DEFECTS
-poor PMN chemotaxis
-poor phagocytosis
-intracellular killing
List the three intrinsic immune system defects seen in down syndrome:
- Poor PMN chemotaxis
- Poor phagocytosis
- Intracellular killing
Leukocyte adhesion deficiency (LAD) is due to a mutation in:
Beta-2 integrin gene (ITGB2)
Lack of beta-2 integrin gene in LAD results in:
- lack beta-2 integrin mRNA in leukocytes
- low integrin (CD18 or CD15) expression on neutrophils
- lack beta-2 integrin mRNA in leukocytes
- low integrin (CD18 or CD15) expression on neutrophils
This is seen in ____ due to ____
LAD; due to mutation in beta-2 integrin gene (ITGB2)
Describe the neutrophil insufficiency in LAD:
Neutrophils are confined to blood vessels resulting in disruption of neutrophil associated-homeostasis
In what disease are neutrophils confined to blood vessels? What does this result in?
LAD; Disruption of neutrophil-associated homeostasis
Describe what is seen in the peridontium of LAD individuals:
History of severe recurrent infection but no pus; leukocytosis is common
What is a common manifestation that comes along with the history of severe recurrent infection without pus, in LAD patients:
Leukocytosis
Papillon-Lefevre syndrome is caused by a mutation in:
Cathepsin C gene (CTSC)
Papillon-Lefevre syndrome is caused by a mutation in cathepsin C gene (CTSC) that is located on chromosome:
11q14
The mutation in Papillon-Lefevre syndrome results in:
- Compromised neutrophil function
- Decreased phagocytosis
- hyperkeratotic lesions
What type of lesions result from the mutation in the Cathepsin C gene in Papillon-Lefevre syndrome?
Hyperkeratotic lesions
-palms
-soles of feet
-elbows
-knees
Chediak-Higashi syndrome is due to a mutation in:
CHS1 gene
LYST gene
The mutations in CHS1 & LYST genes seen in Chediak-Higashi syndrome result in:
lysosomal tracking defect
Partial oculocutaneous albinism is seen in:
Chediak-Higashi syndrome
Why do we see partial oculocutaneous albinism is seen in Chediak-Higashi syndrome- why?
Defects in melanin granules
Patients with Chediak-Higashi syndrome have recurrent pyogenic infections due to:
- circulating leukocytes exhibit DEFECTIVE LYSOSOMES
- decrease in phagocytosis
The varying neurologic problems seen in Chediak-Higashi syndrome include:
- intellectual deficit
- dementia
- Mutation is CHS1
- Mutation in LYST
- Lysosomal tracking defect
- Partial oculocutaneous albinism (defective melanin granules)
- Recurrent pyogenic infections (defective lysosomes & circulating leukocytes & decrease in phagocytosis)
- Varying neurologic problems (intellectual deficit & dementia)
Chedaik- Highashi Syndrome
Congenital neutropenia results from a mutation in:
ELANE (50%)
HAX1 (10%)
Unknown cause (33%)
Congenital neutropenia results in a _____ number of ____
decreased; neutrophils
What is the ANC level in an individual with congenital neutropenia:
Less than 500 cells per microliter and static