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Summer 2017-Oral Medicine Final > Metabolic Disorders > Flashcards

Metabolic Disorders Flashcards

1
Q

Inborn Errors of Metabolism

A
  1. inherited enzyme defects
  2. ethnic predilections
  3. usually autosomal recessive
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2
Q

Gaucher Disease

A
  1. most common lysosomal storage disease
  2. common in Ashkenazic Jews**
  3. glucocerebrosidase deficiency**
  4. hepatosplenomegaly
  5. anemia**
  6. thrombocytopenia
  7. honey-combed mandibular radiolucencies**
GGAA
G: Gaucher
G: glucocerebrosidase
A: Ashkenazic
A: anemia
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3
Q

Glycogen Storage Disease

A
  1. unable to convert glycogen–>glucose
  2. hepatomegaly
  3. hypoglycemia
  4. macroglossia
  5. periodontal disease
  6. masticatory muscle pain
  7. bleeding tendency
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4
Q

Defects in Cholesterol and Lipoprotein Metabolism

A
  1. chylomicrons: carry triglycerides to the liver
  2. VLDL: carry triglycerides and cholesterol to tissues
  3. LDL: promotes atherosclerosis
  4. HDL:
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5
Q

Cutaneous xanthomas

A
  1. associated with problems with cholesterol metabolism

2. lesions in the skin

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6
Q

Xanthelasma

A
  1. associated with problems with cholesterol metabolism

2. white/yellow plaques around the eyelids

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7
Q

Hyperhomocysteinemia

A
  1. error in cysteine metabolism

2. causes carotid stenosis and increased stroke risk

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8
Q

Homocystinuria

A
  1. autosomal recessive inheritance
  2. increased risk of heart attacks and strokes
  3. error in amino acid metabolism
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9
Q

Porphyrias

A
  1. abnormal hemoglobin metabolism
  2. most variants are clinically insignificant
  3. clinically SIGNIFICANT DRUG INDUCED
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10
Q

Clinical Features of Porphyrias

A
  1. tachycardia
  2. hypertension–>postural hypotension
  3. respiratory difficulty
  4. convulsions
  5. neuropsychiatric disturbances
  6. autonomic dysfunction
  7. SIADH–>hyponatremia
  8. red urine (due to altered heme metabolism)
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11
Q

General Management of Porphyrias

A
  1. discontinue triggering drug (if there is one)
  2. decrease 5-aminolevulinic acid
  3. discontinue alcohol (trigger)
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12
Q

What is contraindicated for porphyrias?

A
  1. anesthetics
  2. analgesics
  3. antimicrobials
  4. anxiolytics
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13
Q

Red, fluorescent teeth are associated with __

A

porphyrias

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14
Q

Hemochromatosis

A
  1. hemosiderin deposition
  2. multiple organ damage, especially liver
  3. causes bleeding and clotting problems, hyperpigmentation
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15
Q

Xeroderm pigmentosum

A
  1. disorder of defective DNA repair
  2. autosomal recessive
  3. increased skin cancer
  4. increased oral cancer**
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16
Q

Dyskeratosis congenital

A
  1. disorder of defective DNA repair
  2. abnormal skin pigmentation
  3. bone marrow failure
  4. increased skin, oral, GI cancer**
  5. autosomal recessive**
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17
Q

Glucsoe-6-phosphatase dehydrogenase deficiency

A
  1. most common enzyme deficiency in humans
  2. hemolysis
  3. methemaglobinemia
  4. multiple drug triggers
  5. multiple food triggers
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18
Q

Malignant Hyperthermia

A
  1. triggered by drugs or general anesthesia
  2. Tachypnea, tachycardia, rapid hypertension, hyperthermia, arrhythmia, DIC
  3. 80% mortality if left untreated
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19
Q

Dental Aspects - Malignant Hyperthermia

A
  1. AVOID epinephrine
  2. AVOID stress/anxiety/pain
  3. AVOID anxiolytics
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20
Q

Methemaglobinemia

A
  1. cannot carry oxygen
  2. multiple triggers: inorganic agents, organic nitrites/nitrates, various other agents
  3. treated with methylene blue and ascorbic acid
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21
Q

Amyloidosis

A
  1. acquired metabolic disorder
  2. eosinophilic, hyaline protein

AMyloiDosis: acquired metabolic disorder

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22
Q

Primary Amyloidosis

A
  1. immunoglobulin light chain over-production

2. myeloma-associated

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23
Q

Secondary Amyloidosis

A
  1. most common type

2. excessive stimulation of RE system or inflammatory diseases

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24
Q

Dental Aspects - Amyloidosis

A
  1. macroglossia
  2. gingival enlargement
  3. mucosal petechiae
  4. biopsy: stain with Congo red, view with polarized light

AMY has a big mouth

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25
Metabolic Syndrome
1. risk factors for ischemic heart disease, diabetes, stroke * *hypertension * *insulin resistance * *obesity * *dyslipidema 2. 2X ischemic heart disease risk 3. 5X diabetes risk
26
Inborn errors of metabolism are usually __ (inheritance)
Autosomal recessive
27
most common lysosomal storage disease
Gaucher disease
28
Ashkenazic Jews
Gaucher disease
29
Glucocerebrosidase deficiency
Gaucher disease
30
hepatosplenomegaly
Gaucher disease
31
anemia
Gaucher disease
32
thrombocytopenia
Gaucher disease
33
honey-combed mandibular radiolucencies
Gaucher disease
34
unable to convert glycogen to glucose
glycogen storage disease
35
hepatomegaly
glycogen storage disease
36
hypoglycemia
glycogen storage disease
37
macroglossia
glycogen storage disease
38
periodontal disease
glycogen storage disease
39
masticatory muscle pain
glycogen storage disease
40
bleeding tendency
glycogen storage disease
41
lesions in the skin, problems with cholesterol metabolism
Cutaneous xanthomas
42
white/yellow plaques around eyelids, problems with cholesterol metabolism
Xanthelasma
43
Abnormal hemoglobin metabolism
Porphyrias
44
Drug induced
Porphyrias
45
Tachycardia
Porphyrias
46
Hypertension-->postural hypotension
Porphyrias
47
Respiratory difficulty
Porphyrias
48
Convulsions
Porphyrias
49
Neuropsychiatric disturbances
Porphyrias
50
Autonomic dysfunction
Porphyrias
51
SIADH-->hyponatremia
Porphyrias
52
Red urine
Porphyrias
53
Hemosiderin deposition
Hemochromatosis
54
Multiple organ damage, especially liver
Hemochromatosis
55
Bleeding/clotting problems/hyperpigmentation
Hemochromatosis
56
Disorder of defective DNA repair
Xeroderm pigmentosum
57
Autosomal recessive
Xeroderm pigmentosum, Dyskeratosis congenital
58
Bone marrow failure
Xeroderm pigmentosum
59
Increased skin/oral/GI cancer
Dysteratosis congenital
60
most common enzyme deficiency in humans
Glucose-6-Phosphate Dehydrogenase deficiency
61
Hemolysis
Glucose-6-Phosphate Dehydrogenase deficiency
62
Metemagloinemia
Glucose-6-Phosphate Dehydrogenase deficiency
63
Multiple food and drug triggers
Glucose-6-Phosphate Dehydrogenase deficiency
64
Triggered by drugs or general anesthesia
Malignant hyperthermia
65
Tachypnea, tachycardia, rapid hypertension, hyperthermia, arrythmia, DIC
Malignant hyperthermia
66
Cannot carry oxygen
Methemaglobinemia
67
Tx with methylene blue and ascrobic acid
Methemaglobinemia
68
Macroglossia
Amyloidosis
69
Gingival enlargement
Amyloidosis
70
Mucosal petechia
Amyloidosis
71
risk factors for ischemic heart disease, diabetes, stroke (hypertension, insulin resistance, obesity)
Metabolic Syndrome

Summer 2017-Oral Medicine Final (17 decks)

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