Ch 17 Systemic Diseases 3 Flashcards
What are the 2 alternate names for Plummer-Vinson Syndrome?
- Paterson-Kelly Syndrome
2. Sideropenic Dysphagia
What are the 3 components of Plummer-Vinson Syndrome?
- iron-deficiency anemia
- glossitis
- dysphagia
What are the age, gender, and ethnicity demographics for Plummer-Vinson patients?
Females, 30-50 years old, of Scandinavian or Northern European background
What are often found on endoscopy or esophageal barium contrast radiographic studies of Plummer-Vinson patients?
esophageal webs (“abnormal bands” of tissue)
What is the term for the “spoon-shaped” configuration of the nails in some patients with Plummer-Vinson?
koilonychia
What is the diagnostic readout of the blood test for Plummer-Vinson patients?
hypochromic microcytic anemia, consistent with iron-deficiency anemia
(CBC= low MCV, low iron, low ferritin, increased iron binding potential)
Aren’t the numbers SO helpful? What is the % range for the prevalence of upper aerodigestive tract malignancy for Plummer-Vinson patients?
5-50%, a nice, broad range
What age group and ethnicity are affected most often by pernicious anemia?
older patients of Northern European heritage
What is the blood lab diagnosis for pernicious anemia?
megaloblastic anemia: increased MCV, decreased Hg, decreased B12, possibly decreased platelets and WBCs
What vitamin deficiency causes pernicious anemia? What is produced by the parietal cells of the stomach that is needed to absorb this vitamin?
Vit B12, cobalamin (extrinsic factor)…parietal cells make intrinsic factor to absorb B12
WHY is intrinsic factor not being made in pernicious anemia patients? What antibodies are found in these patients?
autoimmune destruction of parietal cells in the stomach, antibodies against intrinsic factor
Besides an autoimmune cause for pernicious anemia, what are 2 other common etiologies that need to be considered?
- s/p gastric bypass surgery 2. vegetarians/vegans
Since cobalamin is essential for nucleic acid synthesis, what two cell types are most often affected in pernicious anemia?
- hematopoietic cells 2. GI lining epithelial cells
What causes the nervous system symptoms (parestheia, numbness, tingling of the extremeties) in pernicous anemia patients?
cobalamin maintains myelin
What are the 2 oral manifestations of pernicious anemia?
- burning sensation (tongue, lips, buccal mucosa)
2. focal patchy oral erythemia/atrophy (tongue affected in 50-60% of patients)
What can Pernicious Anemia reseble histologically?
dysplasia (although the nuclei in pernicious anemia typically are pale staining and show peripheral chromatin clumping)
What are the 3 blood lab markers for pernicious anemia?
- macrocytic anemia
- reduced serum cobalamin
- serum antibodies against intrinsic factor (“quite specific”)
What is the now obsolete test comparing absorption and excretion rates of cobalamin for pernicious anemia?
the Schilling test
How quickly can the oral lesions of pernicious anemia dissipate following intramuscular injections of cyanocobalamin?
5 days
What type of cancer are pernicious anemia patients susceptible to?
gastric carcinoma (7x more likely)
What two deficiencies BOTH cause a megaloblastic anemia?
- Vit B12
2. Vit B9 Folate
What happens if pernicious anemia (Vit B12/cobalamin def) is treated with folate (Vit B9)?
resolution of the anemia and oral lesions, but the reduced myelin production continues resulting in CNS damage
What hormone is pituitary dwarfism related to? What are the two processing centers involved with this hormone?
Growth hormone - hypothalamus releases growth hormone releasing hormone and then GH is released from the Anterior Pituitary
What might be the reason for dwarfism in a patient that has normal or elevated growth hormone?
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)
What are the 5 oral manifestations of pituitary dwarfism?
- smaller maxilla/mandible
- delayed eruption (1-3 years delay for primary teeth, and 3-10 year delay for permanent teeth)
- lack of 3rd molar development
- smaller size of teeth (reduced in proportion to other anatomic structures)
- severe periodontal disease
What does a radioimmunoassay for HGH show in patients with pituitary dwarfism?
HGH levels that are markedly below normal
What is the usual cause of gigantism?
over production of growth hormone due to a functional pituitary adenoma
What does a skull radiograph typically show in gigantism?
an enlarged sella (due to a pituitary adenoma)
What two hormonal deficiencies can occur in a pituitary adenoma (gigantism)?
hypothyroidism, hypoadrincocorticoism (the adenoma is inhibiting normal growth)
What syndrome accounts for 20% of gigantism cases?
McCune-Albright syndrome (polyostotic FD, cafe au lait, endocrine disturbances)
What are two oral manifestations of gigantism?
- enlarged mandible
2. true generalized macrodontia
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?
42 years old, no sex predilection…6-10 years from symptoms to dx
What are the 3 oral manifestations of acromegaly?
- mandibular prognathism (resulting in apertognathia - aka anterior open bite)
- diastema formation
- macroglossia
What are the two alternate names for hypothyroidism? What is the difference between them?
- Cretinism (decrease occurs during infancy)
2. Myxedema (occurs in adulthood)
Primary hypothyroidism = problem with _______
Secondary hypothyroidism = problem with _______
Which type is more common?
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
What is the ‘usual’ cause of primary hypothyroidism?
hypoplasia or agenesis of the thyroid gland at birth
What dietary deficiency leads to hypothyroidism?
iodine
What autoimmune process leads to hypothyroidism?
Hashimoto Thyroiditis
What are 3 oral findings in hypothyroid patients?
- thickened lips due to GAG deposition (myxedema)
- Macroglossia (again GAG deposition)
- teeth fail to erupt
What lab value is used to diagnose hypothyroidism?
free thyroxine (T4)
If T4 levels are low, what test is done next and why?
TSH levels measured to determine if it is primary or secondary hypothyroidism
Primary = elevated TSH (compensatory by ant pituitary)
Secondary = TSH normal or borderline
What are the two alternate names for hyperthyroidism?
- Thyrotoxicosis
2. Graves Diseases
What causes 60-90% of hyperthyroidism? What is the mechanism?
Graves disease: stimulation of thyroid hormone production by autoantibodies binding to TSH receptors
Graves disease: gender, age, prevelence
female (5-10x more than males), 3rd-4th decades, 2% of the adult female population
What are the two terms for protrusion of the eyes seen in Graves disease? What is the mechanism?
exopthalmos or proptosis: accumulation of GAGs in the retro-orbital tissue
What lab values are used to diagnose hyperthyroidism?
T4 (free thyroxine): elevated
TSH: depressed
What is the most common tx for patients with Graves in the US? Which tx is favored in Europe?
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)
What are 4 clinical signs/symptoms of a thyroid storm in hyperthyroidism? What is the mortality rate of this situation?
- delirium 2.convulsions 3.elevated temperature (up to 106degF) 4. Tachycardia (140bpm)… 20%
