Oral Path: Oral Manifestations Of Systemic Disease

Front 1

Mucopolysaccharidoses

Back 1

AR, lack enzyme necessary to process glycosaminoglycans (Heparan Sulfate, Dermatan S., Keratan S., Chondroitin S.)

Front 2

Lipid Reticuloendothelioses (Lipid storage Diseases)

Back 2

Rare, AR (heterozygotes asymptomatic),

Lack enzymes to process certain lipids so they build up in cells

Front 3

Gaucher Disease (LR)

Back 3

Most common LR - lack of glucocerebrosidase. Glucosylceramide accumulates in lysosomes of macrophages rendering them non-functional. They accumulate in bone marrow.

Front 4

Niemann-Pick Disease (LR)

Back 4

mutations causing deficiency of acid sphingomyelinase -> accumulation of sphingomyelin or defective cholesterol processing. Neuronopathic and visceral subtypes.

Front 5

Tay-Sachs Disease (LR)

Front 6

Jaundice

Back 6

accumulation of bilirubin in tissue. Product of hemoglobin breakdown - taken up by hepatocytes and cunjugated with glucuronic acid so it can be excreted in bile.

Caused by: (two general causes - excessive blood breakdown or liver problem) alcoholic cirrhosis, hepatitis B, malignancy, hepatotoxic drugs & chemicals, autoimmune hemolytic anemia, sickle cell anemia.

Also consider: bleeding tendency (Vit K clotting factors II, VII, IX, X), immune deficiency, impaired drug metabolism.

Front 7

Amyloidosis

Back 7

Extracellular deposition of amyloid (b-pleated sheet). Many causes: hereditary, chronic inflammatory disease, hemodialysis associated, malignancy associated. Organ-limited and systemic forms. Systemic form most often presents with oral changes.

Front 8

Vitamin Deficiencies

Back 8

Rare in US but can occur due to fad diets, alcoholism, and malabsorption states. Vitamins are often co-enzymes or co-factors in metabolic processes

Front 9

Vitamin A (Retinol)

Back 9

Maintenance of vision, growth & tissue differentiation

Front 10

Thiamin (Vit. B1)

Back 10

maintains neuronal function

Front 11

Riboflavin (Vit. B2)

Back 11

extracellular deposition of amyloid (b-pleated sheet). Many causes: hereditary, chronic inflammatory disease, hemodialysis associated, malignancy associated. Organ-limited and systemic forms. Systemic form most often presents with oral changes.

Front 12

Niacin (Vit. B3)

Back 12

Cellular redox rxns.

Front 13

Vitamin C (ascorbic acid)

Back 13

responsible for collagen synth.

Front 14

Vitamin D (now considered a hormone)

Back 14

Role in calcium metabolism

Front 15

Vitamin K

Back 15

necessary for coagulation factor synthesis

Front 16

Vitamin E (a-tocopherol)

Back 16

fat soluble vitamin widely stored throughout body. Antioxidant.

Front 17

Vitamin B6 (pyridoxine)

Back 17

Co-factor associated with enzymes that participate in amino acid synth.

Front 18

Iron Deficiency Anemia

Back 18

Most common anemia. Diagnosis: CBC with RBC line indices, hypochromic, microcytic, decreased RBCs.

Front 19

Plummer-Vinson Syndrome (Paterson-Kelly Syndrome)

Back 19

Iron deficiency.

Front 20

Pernicious Anemia

Back 20

Rare, megaloblastic anemia due to malabsorbtion of Vit B12 (extrinsic factor). Parietal cells either destroyed or lack intrinsic factor.

Front 21

Pituitary Dwarfism

Back 21

Diminished function of anterior pituitary or inability of peripheral tissues to respond to growth hormone.

Front 22

Acromegaly

Back 22

Excess growth hormone secretion (post epihyseal plate closure). Most are due to secretory adenoma of pituitary.

Front 23

Hypothyroidism

Back 23

decreased thyroid hormone. Free thyroxine levels are low (T4)

Front 24

Hyperthyroidism (Grave's disease)

Back 24

Excess thyroid hormone is produced.

Front 25

Hypoparathyroidism (DiGeorge Syndrome, endocrine-candidiasis syndrome)

Back 25

reduced PTH - serum calcium level drops. Calcium necessary for muscle function, buffering

Front 26

Hyperparathyroidism

Back 26

excess PTH release. 1º due to parathyroid adenoma, 2º due to renal disease, chronic low serum calcium. Stones, bones, abdominal groans, (and psychic moans)

Front 27

Cushing's Syndrome

Back 27

adrenal hypercorticolism, chronically increased glucocorticoid levels.

Front 28

Addison's Disease

Back 28

adrenal hypocorticolism. Autoimmune destruction or granulomatous inflammation, but also metastatic tumors, hemochromatosis, amyloidosis.

Front 29

Diabetes Mellitus

Back 29

impaired glucose metabolism due to relative lack of insulin. Most manifestations associated with insulin dependent diabetes mellitus.

Front 30

Hypophosphatasia

Back 30

rare, metabolic bone disease. Reduced alkaline phosphatase, increased urinary phosphoethanolamine.

Front 31

Vitamin D-resistant Rickets

Back 31

inability to convert Vitamin D to active form in kidney.

Front 32

Crohn's Disease

Back 32

Chronic granulomatous inflammatory disease all along the GI tract, "mouth to anus".

Front 33

Pyostomatitis Vegetans

Back 33

Unusual expression of inflammatory bowel disease, Crohn's disease or ulcerative colitis - more commonly

Front 34

Uremic Stomatitis

Back 34

complication of renal failure, increased urea in blood, saliva.

Front 35

Lipid Proteinosis (Hyalinosis cutis et mucosae; Urbach-wiethe syndrome)

Back 35

Rare, AR. Deposition of waxy material in dermis and submucosal tissue of affected patients

Front 36

Gigantism

Back 36

Increased GH, usually due to functional pituitary adenoma (before closure of epiphyseal plate)