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OPT Systemic Disease
| Term | Definition |
|---|---|
| mucopolysaccharidoses | group of inherited disturbances in metabolism of {glycosaminoglycans}; facial and spinal abnormalities, mental retardation -enlarged tongue, gingival hyperplasia, broad/short mandible |
| lipid reticuloendothelioses | disorders of lipid metabolism so lipids accumulate in cells; includes Gaucher disease, Niemann-Pick disease and Tay-Sachs disease |
| Gaucher disease | caused by genetic deficiency of {glucocerebrosidase} resulting in abnormal deposition of {glucosylceramide} in macrophage lysosomes |
| glucocerebrosidase | Gaucher disease caused by deficiency of this enzyme |
| glucosylceramide | deposited in lysosomes of macrophages in Gaucher disease |
| Type 1 (nonneuronopathic) Gaucher disease | accumulation of lipid-filled macrophages in bone marrow, causing anemia, thrombocytopenia and bone pain -{Erlenmeyer flask deformities}- seen radiographically in long bones |
| Erlenmeyer flask deformities | seen radiographically in long bones in Type 1 Gaucher disease |
| Types 2 and 3 Gaucher disease (neuronopathic) | more severe Gaucher disease involving the CNS; don't respond to enzyme replacement or transplant |
| Niemann-Pick disease | due to lack of {sphingomyelinase} resulting in deposition of {sphingomyelin} in lysosomes of macrophages giving them sea blue cytoplasm |
| spingomyelinase | deficiency of this enzyme causes Niemann-Pick disease |
| Tay Sachs disease | due to lack of {beta-hexosaminidase A} causing accumulation of {ganglioside} in the lysosomes of neurons; blindness, seizures, death by 5 |
| beta-hexosaminidase A | deficiency in this enzyme causes Tay Sachs disease, causing build up of ganglioside |
| ganglioside | builds up in Tay Sachs disease |
| lipoid proteinosis | deposition of waxy material in dermis and submucosa, causes hoarseness during infancy and childhood and waxy papules -tongue can be bound to the floor of the mouth |
| jaundice | condition due to excess bilirubin in the blood normally taken up by hepatocytes, can be caused by number of diseases ie {autoimmune hemolytic anemia} and {sickle cell anemia} |
| unconjugated bilirubin | shown in lab tests for jaundice in infants |
| conjugated bilirubin | elevated in conditions caused by reduced excretion of bilirubin into the bile ducts ie liver disease and cancer |
| amyloidosis | group of conditions with deposition of extracellular protein material called "amyloid", a starch-like material -organ-limited form and systemic forms |
| organ-limited amyloidosis | amyloidosis in variety of organs but rarely oral tissues; not associated with any systemic condition |
| systemic amyloidosis | amyloidosis further classified into primary, myeloma-associated, secondary, hemodialysis-associated, and heredofamilial types |
| primary and myeloma associated forms of amyloidosis | form of amyloidosis occurring in older patients caused by deposition of Ig light chains |
| secondary form of amyloidosis | form of amyloidosis resulting form long-standing chronic inflammatory diseases ie TB |
| hemodialysis-associated form of amyloidosis | form of amyloidosis seen in patients on longterm dialysis due to accumulation of globins not removed by dialysis |
| heredofamilial form of amyloidosis | rare form of inherited amyloidosis, causing heart and kidney problems aka {famililial Mediterranean fever} |
| Xanthelasma | yellowish plaques on periocular skin, indicating elevated serum lipids and risk of atherosclerosis |
| Vitamin A | necessary for vision and tissue growth; deficiency causes night blindness, keratomalacia, xerostomia, enamel hypoplasia |
| Vitamin D | can be made by sunlight, promotes calcium absorption and regulate phosphorus; deficiency causes {rickets} in kids and {osteomalacia} in adults |
| Vitamin E | functions as antioxidant, deficiency is rare but occurs in liver diseases |
| Vitamin K | necessary for protein synthesis and clotting factors; deficiency leads to impaired clotting and gingival bleeding |
| thiamin (vitamin B1) | coenzyme for metabolic reactions and maintains neurons; deficiency causes {beriberi} and {Wernicke-Korsakoff syndrome} in alcoholics |
| riboflavin (vitamin B2) | necessary for cellular ox-red reductions; deficiency causes atrophic glossitis, angular chelitis, sore throat and swelling of oral mucosa |
| niacin | coenzyme for ox-red reactions; deficiency causes {pellagra} with 3 D's: {dermatitis, dementia, and diarrhea} |
| pellagra | caused by deficiency of niacin; dermatitis, dementia, diarrhea |
| pyridoxine | cofactor for amino acid synthesis; deficiency is very similar to riboflavin |
| vitamin C | needed for collagen synthesis; deficiency causes {scurvy} and {scorbutic gingivitis} |
| scorbutic gingivitis | red, swollen and boggy gingiva caused by deficiency in vitamin C |
| iron-deficiency anemia | most common type of anemia caused by 1. excessive blood loss, 2. increased demand for RBC, 3. decreased iron intake, 4. decreased iron absorption -oral manifestations: angular cheilitis, atrophic glossitis mucosal atrophy -microcytic hypochromic anemia |
| Plummer-Vinson syndrome | iron deficiency anemia with glossitis and dysphagia; spoon-shaped nails and esophageal webbing |
| pernicious anemia | deficiency of vitamin B12 due to inability to absorb it from lack of intrinsic factor ; necessary for normal nucleic acid synthesis, disrupts tissue that turns over rapidly -pts who have had {gastric bypass surgery}, {vegans}, and {H. pylori} |
| oral signs pernicious anemia | atrophic glossitis, focal patchy areas of mucosal atrophy and erythema -macrocytic anemia, confirmed by Schilling test |
| pituitary dwarfism | caused by diminished production of GH or reduced capacity of tissues to respond |
| gigantism | overproduction of GH before epiphyseal plates have closed, causing patients to grow very tall; most caused by functional adenoma of pituitary |
| acromegaly | overproduction of GH after epiphyseal plates have closed, usually due to functional adenoma |
| hypothyroidism | decreased levels of thyroid hormone; {cretinism} in infancy, {myxedema} in adulthood |
| Hashimoto's thyroiditis | autoimmune destruction of the thyroid gland, major cause of primary hypothyroidism in adults |
| hyperthyroidism | condition caused by excessive thyroid hormones, mostly due to {Graves disease}- binding of antibodies to TSH receptors, stimulating excess thyroid hormone -goiter, high metabolic rate, heat intolerance, exophthalmos |
| thyroid storm | uncontrolled hyperthyroidism, can be triggered by infections, physical trauma and stress; delirium, convulsions, tachycardia |
| hypoparathyroidism | reduction in serum PTH; calcium levels drop, hyperventilation, tetany, pitted enamel -Chvosstek's sign- twitching of lip by tapping on facial nerve to exhibit tetany |
| pseudohypoparathyroidism | adequate PTH levels but pathways don't respond; mental retardation, obesity, round face |
| hyperparathyroidism | excessive production of PTH |
| primary hyperparathyroidism | caused by solitary parathyroid adenoma; "stones, bones and abdominal groans"- kidney stones, osteoporosis, and duodenal ulcers |
| secondary hyperparathyroidism | occurs in response to chronic hypocalcemia, usually associated with chronic renal failure |
| brown tumor of hyperparathyroidism | lytic bone lesions occurring with chronic hyperparathyroidism; dark red-brown color -can lead to osteitis fibrosa cystica, identical to central giant cell granuloma in jaws |
| osteitis fibrosa cystica | expanding brown tumors that become fibrotic; due to hyperparathyroidism |
| hypercortisolism (Cushing syndrome) | caused by sustained increase in glucocorticoid levels, most secondary to medications -called {Cushing disease} if caused by pituitary adenoma that secretes ACTH -moon face, buffalo hump, weigh gain, depression |
| primary Addison disease | insufficient production of adrenal corticosteroid hormones caused by destruction of adrenal cortex; can be autoimmune, due to TB and infections -hyperpigmentation, fatigue, |
| secondary Addison disease | caused by decreased ACTH production in pituitary gland; symptoms don't appear until 90% of gland has been destroyed |
| type I diabetes mellitus | caused by lack of insulin production due to autoimmune destruction of beta pancreatic cells -childhood onset, ketoacidosis, glycosuria, requires insulin |
| type II diabetes mellitus | caused by reduction in insulin receptors or prevention of normal glucose uptake; leads to {insulin resistance} despite normal insulin levels -familial component, |
| hypophosphatasia | rare metabolic bone disease caused by deficiency of alkaline phosphatase; bone abnormalities resemble rickets |
| perinatal lethal form of hypophosphatasia | form of hypophosphatasia in which newborns have severe hypo calcification of the skeleton and die within a few hours of birth |
| odontohypophosphatasia | form of hypophosphatasia, only clinical manifestation is premature loss of incisors due to reduction in cementum on root surface |
| vitamin-D resistant rickets | inherited X-linked condition in which patients with rickets do not respond to treatment with vitamin D; hypophosphatasia and decreased ability to absorb phosphate -pointed pulp horns that extend to DEJ so frequent abscesses |
| vitamin-D dependent rickets | deficiency in vitamin D due to lack of 1alpha-hydroxylase; responds to replacement therapy of vitamin D |
| Crohn disease | inflammatory disease of GIT; affects any portion but mostly distal small intestine and proximal large intestine, can have skip lesions -oral manifestations often the first sign of disease: nodules, cobblestone mucosa, and ulcers |
| pyostomatitis vegetans | oral expression of inflammatory bowel disease, usually ulcerative colitis and Crohn disease; yellow, elevated, serpentine pustules on erythematous mucosa, "snail track ulcerations" |
| uremic stomatitis | finding in pts with chronic renal failure probably due to elevated urea and free ammonia; painful white plaques on buccal mucosa, tongue and floor of mouth |
Created by:
aharnold
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