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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
Q

Metabolic Syndrome

A
  1. risk factors for ischemic heart disease, diabetes, stroke
    * *hypertension
    * *insulin resistance
    * *obesity
    * *dyslipidema
  2. 2X ischemic heart disease risk
  3. 5X diabetes risk
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26
Q

Inborn errors of metabolism are usually __ (inheritance)

A

Autosomal recessive

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27
Q

most common lysosomal storage disease

A

Gaucher disease

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28
Q

Ashkenazic Jews

A

Gaucher disease

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29
Q

Glucocerebrosidase deficiency

A

Gaucher disease

30
Q

hepatosplenomegaly

A

Gaucher disease

31
Q

anemia

A

Gaucher disease

32
Q

thrombocytopenia

A

Gaucher disease

33
Q

honey-combed mandibular radiolucencies

A

Gaucher disease

34
Q

unable to convert glycogen to glucose

A

glycogen storage disease

35
Q

hepatomegaly

A

glycogen storage disease

36
Q

hypoglycemia

A

glycogen storage disease

37
Q

macroglossia

A

glycogen storage disease

38
Q

periodontal disease

A

glycogen storage disease

39
Q

masticatory muscle pain

A

glycogen storage disease

40
Q

bleeding tendency

A

glycogen storage disease

41
Q

lesions in the skin, problems with cholesterol metabolism

A

Cutaneous xanthomas

42
Q

white/yellow plaques around eyelids, problems with cholesterol metabolism

A

Xanthelasma

43
Q

Abnormal hemoglobin metabolism

A

Porphyrias

44
Q

Drug induced

A

Porphyrias

45
Q

Tachycardia

A

Porphyrias

46
Q

Hypertension–>postural hypotension

A

Porphyrias

47
Q

Respiratory difficulty

A

Porphyrias

48
Q

Convulsions

A

Porphyrias

49
Q

Neuropsychiatric disturbances

A

Porphyrias

50
Q

Autonomic dysfunction

A

Porphyrias

51
Q

SIADH–>hyponatremia

A

Porphyrias

52
Q

Red urine

A

Porphyrias

53
Q

Hemosiderin deposition

A

Hemochromatosis

54
Q

Multiple organ damage, especially liver

A

Hemochromatosis

55
Q

Bleeding/clotting problems/hyperpigmentation

A

Hemochromatosis

56
Q

Disorder of defective DNA repair

A

Xeroderm pigmentosum

57
Q

Autosomal recessive

A

Xeroderm pigmentosum, Dyskeratosis congenital

58
Q

Bone marrow failure

A

Xeroderm pigmentosum

59
Q

Increased skin/oral/GI cancer

A

Dysteratosis congenital

60
Q

most common enzyme deficiency in humans

A

Glucose-6-Phosphate Dehydrogenase deficiency

61
Q

Hemolysis

A

Glucose-6-Phosphate Dehydrogenase deficiency

62
Q

Metemagloinemia

A

Glucose-6-Phosphate Dehydrogenase deficiency

63
Q

Multiple food and drug triggers

A

Glucose-6-Phosphate Dehydrogenase deficiency

64
Q

Triggered by drugs or general anesthesia

A

Malignant hyperthermia

65
Q

Tachypnea, tachycardia, rapid hypertension, hyperthermia, arrythmia, DIC

A

Malignant hyperthermia

66
Q

Cannot carry oxygen

A

Methemaglobinemia

67
Q

Tx with methylene blue and ascrobic acid

A

Methemaglobinemia

68
Q

Macroglossia

A

Amyloidosis

69
Q

Gingival enlargement

A

Amyloidosis

70
Q

Mucosal petechia

A

Amyloidosis

71
Q

risk factors for ischemic heart disease, diabetes, stroke (hypertension, insulin resistance, obesity)

A

Metabolic Syndrome

Summer 2017-Oral Medicine Final (17 decks)

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