Ch 17 Systemic Diseases 3 Flashcards

1
Q

What are the 2 alternate names for Plummer-Vinson Syndrome?

A
  1. Paterson-Kelly Syndrome

2. Sideropenic Dysphagia

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2
Q

What are the 3 components of Plummer-Vinson Syndrome?

A
  1. iron-deficiency anemia
  2. glossitis
  3. dysphagia
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3
Q

What are the age, gender, and ethnicity demographics for Plummer-Vinson patients?

A

Females, 30-50 years old, of Scandinavian or Northern European background

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4
Q

What are often found on endoscopy or esophageal barium contrast radiographic studies of Plummer-Vinson patients?

A

esophageal webs (“abnormal bands” of tissue)

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5
Q

What is the term for the “spoon-shaped” configuration of the nails in some patients with Plummer-Vinson?

A

koilonychia

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6
Q

What is the diagnostic readout of the blood test for Plummer-Vinson patients?

A

hypochromic microcytic anemia, consistent with iron-deficiency anemia
(CBC= low MCV, low iron, low ferritin, increased iron binding potential)

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7
Q

Aren’t the numbers SO helpful? What is the % range for the prevalence of upper aerodigestive tract malignancy for Plummer-Vinson patients?

A

5-50%, a nice, broad range

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8
Q

What age group and ethnicity are affected most often by pernicious anemia?

A

older patients of Northern European heritage

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9
Q

What is the blood lab diagnosis for pernicious anemia?

A

megaloblastic anemia: increased MCV, decreased Hg, decreased B12, possibly decreased platelets and WBCs

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10
Q

What vitamin deficiency causes pernicious anemia? What is produced by the parietal cells of the stomach that is needed to absorb this vitamin?

A

Vit B12, cobalamin (extrinsic factor)…parietal cells make intrinsic factor to absorb B12

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11
Q

WHY is intrinsic factor not being made in pernicious anemia patients? What antibodies are found in these patients?

A

autoimmune destruction of parietal cells in the stomach, antibodies against intrinsic factor

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12
Q

Besides an autoimmune cause for pernicious anemia, what are 2 other common etiologies that need to be considered?

A
  1. s/p gastric bypass surgery 2. vegetarians/vegans
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13
Q

Since cobalamin is essential for nucleic acid synthesis, what two cell types are most often affected in pernicious anemia?

A
  1. hematopoietic cells 2. GI lining epithelial cells
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14
Q

What causes the nervous system symptoms (parestheia, numbness, tingling of the extremeties) in pernicous anemia patients?

A

cobalamin maintains myelin

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15
Q

What are the 2 oral manifestations of pernicious anemia?

A
  1. burning sensation (tongue, lips, buccal mucosa)

2. focal patchy oral erythemia/atrophy (tongue affected in 50-60% of patients)

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16
Q

What can Pernicious Anemia reseble histologically?

A

dysplasia (although the nuclei in pernicious anemia typically are pale staining and show peripheral chromatin clumping)

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17
Q

What are the 3 blood lab markers for pernicious anemia?

A
  1. macrocytic anemia
  2. reduced serum cobalamin
  3. serum antibodies against intrinsic factor (“quite specific”)
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18
Q

What is the now obsolete test comparing absorption and excretion rates of cobalamin for pernicious anemia?

A

the Schilling test

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19
Q

How quickly can the oral lesions of pernicious anemia dissipate following intramuscular injections of cyanocobalamin?

A

5 days

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20
Q

What type of cancer are pernicious anemia patients susceptible to?

A

gastric carcinoma (7x more likely)

21
Q

What two deficiencies BOTH cause a megaloblastic anemia?

A
  1. Vit B12

2. Vit B9 Folate

22
Q

What happens if pernicious anemia (Vit B12/cobalamin def) is treated with folate (Vit B9)?

A

resolution of the anemia and oral lesions, but the reduced myelin production continues resulting in CNS damage

23
Q

What hormone is pituitary dwarfism related to? What are the two processing centers involved with this hormone?

A

Growth hormone - hypothalamus releases growth hormone releasing hormone and then GH is released from the Anterior Pituitary

24
Q

What might be the reason for dwarfism in a patient that has normal or elevated growth hormone?

A

An AR trait resulting in abnormal/reduced GH receptors in the target cells (there is no treatment, unlike being able to supplement with GHRH or GH)

25
Q

What are the 5 oral manifestations of pituitary dwarfism?

A
  1. smaller maxilla/mandible
  2. delayed eruption (1-3 years delay for primary teeth, and 3-10 year delay for permanent teeth)
  3. lack of 3rd molar development
  4. smaller size of teeth (reduced in proportion to other anatomic structures)
  5. severe periodontal disease
26
Q

What does a radioimmunoassay for HGH show in patients with pituitary dwarfism?

A

HGH levels that are markedly below normal

27
Q

What is the usual cause of gigantism?

A

over production of growth hormone due to a functional pituitary adenoma

28
Q

What does a skull radiograph typically show in gigantism?

A

an enlarged sella (due to a pituitary adenoma)

29
Q

What two hormonal deficiencies can occur in a pituitary adenoma (gigantism)?

A

hypothyroidism, hypoadrincocorticoism (the adenoma is inhibiting normal growth)

30
Q

What syndrome accounts for 20% of gigantism cases?

A

McCune-Albright syndrome (polyostotic FD, cafe au lait, endocrine disturbances)

31
Q

What are two oral manifestations of gigantism?

A
  1. enlarged mandible

2. true generalized macrodontia

32
Q

What is the average age at diagnosis for acromegaly? What is the gender predilection? How long does it usually take from the onset of symptoms to the diagnosis?

A

42 years old, no sex predilection…6-10 years from symptoms to dx

33
Q

What are the 3 oral manifestations of acromegaly?

A
  1. mandibular prognathism (resulting in apertognathia - aka anterior open bite)
  2. diastema formation
  3. macroglossia
34
Q

What are the two alternate names for hypothyroidism? What is the difference between them?

A
  1. Cretinism (decrease occurs during infancy)

2. Myxedema (occurs in adulthood)

35
Q

Primary hypothyroidism = problem with _______

Secondary hypothyroidism = problem with _______

Which type is more common?

A

In primary hypothyroidism, the thyroid gland
itself is in some way abnormal, in secondary hypothyroidism, the pituitary gland does not produce an adequate amount of thyroid-stimulating hormone (TSH)

Primary is more common

36
Q

What is the ‘usual’ cause of primary hypothyroidism?

A

hypoplasia or agenesis of the thyroid gland at birth

37
Q

What dietary deficiency leads to hypothyroidism?

A

iodine

38
Q

What autoimmune process leads to hypothyroidism?

A

Hashimoto Thyroiditis

39
Q

What are 3 oral findings in hypothyroid patients?

A
  1. thickened lips due to GAG deposition (myxedema)
  2. Macroglossia (again GAG deposition)
  3. teeth fail to erupt
40
Q

What lab value is used to diagnose hypothyroidism?

A

free thyroxine (T4)

41
Q

If T4 levels are low, what test is done next and why?

A

TSH levels measured to determine if it is primary or secondary hypothyroidism

Primary = elevated TSH (compensatory by ant pituitary)

Secondary = TSH normal or borderline

42
Q

What are the two alternate names for hyperthyroidism?

A
  1. Thyrotoxicosis

2. Graves Diseases

43
Q

What causes 60-90% of hyperthyroidism? What is the mechanism?

A

Graves disease: stimulation of thyroid hormone production by autoantibodies binding to TSH receptors

44
Q

Graves disease: gender, age, prevelence

A

female (5-10x more than males), 3rd-4th decades, 2% of the adult female population

45
Q

What are the two terms for protrusion of the eyes seen in Graves disease? What is the mechanism?

A

exopthalmos or proptosis: accumulation of GAGs in the retro-orbital tissue

46
Q

What lab values are used to diagnose hyperthyroidism?

A

T4 (free thyroxine): elevated

TSH: depressed

47
Q

What is the most common tx for patients with Graves in the US? Which tx is favored in Europe?

A

radioactive iodine (131I) in US….Europe uses PTU and methimazole (these block the normal use of iodine in the thyroid gland, but PTU has been linked to liver toxicity)

48
Q

What are 4 clinical signs/symptoms of a thyroid storm in hyperthyroidism? What is the mortality rate of this situation?

A
  1. delirium 2.convulsions 3.elevated temperature (up to 106degF) 4. Tachycardia (140bpm)… 20%