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Disease that results from the lack of an enzyme necessary to process GAGs
Mucopolysaccharidosis
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Oral manifestations of Mucopolysaccharidosis (3)
- Macroglossia
- Gingival hyperplasia (drying due to mouth breathing or medications)
- Impacted teeth with large follicular spaces and thin enamel
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what are the oral manifestations of Hunter Syndrome, a type of Mucopolysaccharidosis (2)
- Macroglossia
- Wide-spaced teeth
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Disease that presents in patients that lack enzymes that process certain lipids, allowing cellular accumulation of those lipids
Lipid Reticuloendothelioses
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Most common Lipid Reticuloendothelioses
Gaucher disease
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adults with ____ disease have an increased incidence of lymphoma, multiple myeloma
Gaucher's
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A patient presents with a mixed, ill-defined RL/RO in mandible that is bilateral and diffuse. They also present with gingivitis, bone pain, and anemia and thrombocytopenia. What do they have?
Gaucher Disease
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Diffuse yellowing discoloration of skin and mucosa (most notable in sclera)
jaundice
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Bilirubin has a preference for structures with elastic tissue. So where would we see it in the oral cavity?
Soft palate
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How can u tell between hypercarotenemia, which causes the skin to be yellow-orange and jaundice
Hypercarotenemia doesn't cause the sclare to change color
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What three other considerations must be taken into account when juandice is present
- Bleeding tendency (Vit K dependent clotting factors)
- Immune deficiency
- Impaired drug metabolism
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Amyloidosis can be secondary to what?
Multiple myeloma
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Extracellular deposition of proteinaceous material results in what disease?
Amyloidosis
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Which form of amyloidosis presents with oral changes?
Systemic form
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Disease that presents with macroglossia and waxy nodular lesions noted periocularly
Amyloidosis
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What shows confirmation (80% positive) of amyloidosis
Rectal mucosal and labial salivary gland biopsy
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Patient presents with the following symptoms:
poor wound healing, perio disease, capillary fragility, scorbutic gingivitis (gingival edema, erythema, ulceration, spontaneous bleeding) and petechiae on the skin. what is the probable cause?
Vitamin C deficiency
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Vitamin C is responsible for what?
Collagen synthesis
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a patient presents with defective skeletal development, loss of lamina dura, and fibrous dysplasia-like appearance in radiographs. What is the probable cause
Vitamin D deficiency
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What is the most common anemia?
Iron deficiency anemia
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A patient presents with angular chalets, atrophic glossitis (papillae are blunted), mucosal atrophy, fatigue and palpitations. What is the likely diagnosis?
Iron deficiency anemia
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disease that presents wit iron deficiency, glossitis, dysphagia (esophageal webs), spoon shaped, brittle nails
Plummer-Vinson Syndrome
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People with Plummer-Vinson Syndrome have increased incidence of what?
oral and esophageal squamous cell CA
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What are the oral manifestion for cretinism (3)(hypothyroidism in children)
- Delayed eruption of fully formed teeth
- Thickened lips
- Diffuse tongue enlargement
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Hypoparathyroidism causes what?
Serum calcium levels to drop
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What are the oral manifestations of hypoparathyroidism (2)
- Delayed tooth eruption
- See diffuse brown spots on the teeth due to hypocalcification during tooth development
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What is the oral manifestation of hyperparathyroidism
Diffuse palatal enlargement is common in the secondary to renal disease form
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Stones, bones and abdominal groans refers to what disease?
Hyperparathyroidism
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What is the meaning of
Stones:
Bones:
Abdominal Groans:
- Stones: renal caluli
- Bones: "ground glass" appearance of trabeculae, loss of lamina dura of surrounding teeth
- Abdominal Groans: duodenal ulcers
also psychic moans-- weakness, confusion and dementia develop
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Histologically central giant cell granuloma and hyper PTH look the same. What is the way to differentiate ? (3)
- Blood work (evaluated PTH)
- Age (CGCG is in kids, hyper PTH in older people)
- HyperPTH has stones bones and abdominal groans findings
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