Chapter 17: Oral manifestations of systemic diseases Flashcards

Question

Hyperparathyroidism

Answer 1

-‐ Primary (90% gland adenoma) or secondary (chronic low calcium from chronic renal disease-‐ vitamin D not activated thus calcium not absorbed) -‐ Parathyroid hormone: mobilization of calcium from bone; increases renal tubular reabsorption of calcium; promotes renal production of 1,25-‐dihydroxyvitamin D; and increases serum calcium -‐ Can be induced by MEN1 or MEN2A and hyperparathyroidism-‐jaw tumors syndrome -‐ Triad: kidney stones (from  Ca), bones (various) and abdominal groans (duodenal ulcers) -‐ Osteitis fibrosa cystica: central degeneration and fibrosis of long standing brown tumors -‐ Renal osteodystrophy: striking enlargement of jaws, when hyperPARATHY is secondary -‐ Brown tumor, subperiosteal bone resorption (esp. phalanges), generalized osteopenia and loss of lamina dura and blurring of trabeculae (ground glass) -‐ Histo: brown tumor = CGCG. RO = trabeculae of woven bone + giant cells in fibrous background

Answer 2

-‐ Adrenal glands: produce corticoids. Stimulated by ACTH (produced by pituitary) -‐ If exogenous corticoids (drugs): Cushing syndrome. -‐ If endogenous (adrenal/pituitary adenoma): Cushing disease. Rare. Most in young females. -‐ Signs: Central weight gain, buffalo hump, moon facies, HT, diabetes, osteoporosis -‐ Cushing disease: high cortisol, high ACTH following dexamethasone test (in normal pts, both decrease. Howver, tumors do not respond to feedback loop) -‐ Addisonian crisis: acute episode of hypoadrenocorticism, seen after withdrawal of corticodrugs (drugs suprress pituitary to an extent where it cannot produce ACTH, even upon stress) -‐ NAME: nevi, atrial myxoma, myxoid NF, ephelides + hypercortisolism. Subset of Carney complex.

Answer 3

-‐ Addison’s disease: low corticoids due to adrenal dysfunction -‐ Causes: AI and infection (TB, deep fungal-‐histo and PCM). Also mets, sarcoid, amyloid -‐ Secondary adrenocorticism: low corticoids due to low ACTH from pituitary dysfunction -‐ Features only appear when 90% of gland has been destroyed -‐ Bronzing of skin (increased beta-‐lipotropin and ACTH stimulate melanocytes) -‐ Addison + hypoparaTHY + mucocutaneous candida: consider APECED -‐ Oral: diffuse pigmentation of oral mucosa (hx sudden onset to diff from physiologic pigmtation) -‐ Dx: low serum cortisol < 20 ug/dL. High ACTH (primary) or low ACTH (secondary) -‐ Sheehan’s syndrome: acute adrenal cortical insufficiency caused by meningococci

Answer 4

-‐ Type 1-‐insulin dependent (lack insulin due to AI destruction of pancreas islet cells); type 2-‐non insulin dependent (insulin resistance; genetic+environmental) -‐ Type 1: polyruia, polyphagia and polydipsia, ketoacidosis (because there’s no glucose, fat and protein are used for energy, releasing ketones as by-‐product) -‐ Microangiopathy (occlusion of small blood vessels due to uptake of glucose by EC) leads to peripheral vascular disease and consequently ischemia, which in turn can cause renal failure, gangrene of limbs, neuropathy, inability to fight infections, infarct, stroke and blindness. -‐ Kimmelstiel-‐Wilson disease: diabetic nephropathy -‐ Oral: periodontal disease, delayed healing, sialadenosis, gingival enlargement, fungal infections (candida, mucor) and xerostomia -‐ Metformin: increases glucose use and reduces insulin resistance -‐ Insulin shock: if type 1 pt does not ingest carbohydrate after insulin injection, glucose levels may fall dramatically (below 40 mg/dL)

Answer 5

-‐ Deficiency of alkaline phosphatase (enzyme that removes phosphate groups from molecules) -‐ Clinically: 1) reduced alkaline phosphatase in bone, liver and kidney; 2) increased blood and urinary phosphoethanolamine; and 3) bone abnormalities similar to rickets -‐ Forms: perinatal (death few hours doe to respiratory failure) -‐ Infantile: short, bowed limbs (from 6 mths of age on). Similar to rickets. Premature shedding of deciduous teeth (teeth lack cementum). -‐ Childhood: Loss of primary teeth w/o infl (incisors most). Bowed legs, short stature. Beaten copper skull (uniformly spaced, poorly defined RL). Due to thinning of inner cortical plate. -‐ Adult: mild disease. History of loss of primary or permanent dentition -‐ Dx: clinical +  serum alkaline phosphatase +  phosphoethanolamine

Answer 6

-‐ Clinical features of rickets but did not respond to vit D tx. -‐ PHEX mutation. X-‐linked (females less severely affected due to lyonization) -‐ Rachitic changes + hypophosphatemia (low phosphate) and decreased reabsorption of phosphate in kidney. Pts have short stature and bowed lower limbs. -‐ Enlarged pulps, with elongated pulp horns extending into the incisal edge just below the dentinoenamel junction. Also, large calcospherites of globular dentin -‐ Small exposures cause necrosis in healthy teeth -‐> multiple abcesses in 1ary dentition. -‐ TX: calcitrol + phosphate -‐ Vitamin D-‐dependent rickets: AD, lacks 1α-‐hydroxylase (activates vit D). Similar features to vit D-‐ resistant, but shows hypocalcification of teeth. No response to vit D

Answer 7

-‐ Affects distal portion of small bowel and proximal colon (skip lesions). -‐ Can affect any region from mouth to anus. Also extra-‐GI such as eyes, skin -‐ Oral: First sign of disease in 30% of cases. Swelling of oral tissues, cobblestone appearance and deep granulomatous ulcers (linear and in the buccal vestibule). Also, IFH-‐like or aphthous-‐like. -‐ Mucogingivitis: patchy ertyhematous macules and plaques involving gingival -‐ Drug of choice: sulfasalazine

Answer 8

-‐ Oral manifestation of inflammatory bowel disease (Crohn’s and ulcerative colitis). -‐ Yellowish, linear, serpentine pustules over an erythematous mucosa (“snail track ulcerations”) -‐ Most on buccal/labial mucosa, soft palate and ventral tongue. -‐ Histo: Acantholytic appearance of epithelium. Intra or subbepithelial eosinophilic abscesses

Answer 9

-‐ Increased urea levels in acute or chronic renal failure. -‐ Urease degrades urea in saliva and releases ammonia, which damages the oral mucosa. -‐ Painful white plaques in cheek, tongue and FOM. -‐ May mimic oral hairy leukoplakia -‐ Ammonia/urine odor. -‐ Tx: clears with dialysis in up to 3 wks. Diluted H2O2 as rinse also helpful.