Oral Pathology and Systemic Conditions

Meiosis

2-step cellular division of primitive germ cells (46, diploid)  mature germ cells (23, haploid)

Lyon Hypothesis

genetic activity of 1 of the X chromo in each cell of a female embryo; when X chromo in female is inactivated = Barr body forms; X chrome dispersed into nucleus; gross abnormalities

Trisomy 21

Down Syndrome; 3 identical chromo at the same allele; most frequent; fissured tongue; macroglossia, slanted eyes, low IQ, hypodontia

Trisomy 13

poor prognosis; 3 chrom on 13; bilateral cleft lip/palate, microphthalmia/anopthalmia

Karyotype

a photographic representation of of a person’s chromosomal make-up; gross abnormalities

Turner Syndrome

lack X chromosome from father; no Barr bodies on smear; webbed neck, short statue

Klinefelter

extra X chrom in males; hyper plastic max

Cat Cry (Cri Du Chat)

severe mental retardation

Autosomal-Dominant Inheritance

males/females equally affected; 50% of passing on condition

Autosomal-Recessive Inheritance

both parents of the affected child must be carriers of the trait

Papillon-Lefevre Syndrome

hyperkeratosis of the palms of the hands & soles of the feet; edema, bleeding, alveolar bone resorption, mobility of teeth; both sets of teeth are lost premature 

Gingival Fibromatosis

gingival hyperplasia, excessive hair, multiple fibromas/very firm, granulated, corrugated gingival surface

Laband Syndrome

ADI; gingival fibromatosis (absent nails, ear & nose tissues), gingival hyperplasia, short fingers

Cherubism

bilateral enlargement of face, multiple radioluc of giant cell tumors/soap bubble appearance

Congenital Epulis

xtra kiss on max ridge/present @ birth

Cleidocranial Dysplasia

supernumerary teeth (crowded in jaws & don’t erupt), multiple cysts of md; uni/bilateral hypoplasia of clavicles

Garner Syndrome

supernal tth, multiple odontomas, polyps a concern

Treacher-Collins Syndrome (Md Facial Dysostosis)

fish shaped mouth/high arched palate, crowded tth, poor facial features, deafness due to poorly formed structures, hypo plastic md

Osteogenesis Imperfecta

abnormally formed bones that fracture easily; blue sclera

Dentinogenesis Imperfecta

grey -> opalescent dentin; no pulp chambers/rt canals seen; roots are short, tth fracture easily b/c poor dentin formation

Cleft Lip/Palate

multi factorial in nature; affects 1 in 800 births

Peutz-Jehger Syndrome

must melanotic macules of the skin, eyes, oral mucosal tissue, intestinal polyps

White Sponge Nevus

thick layer of keratin that desquamates & leaves raw mucosal surface; free gingiva not affected

Ectodermal Dysplasia

partial anodontia (hypodontia); decreased hair; hearing loss (hypotrichosis); can’t sweat (hypohidrosis); hearing loss

Hypophosphatasia

decrease in serum alkaline phosphatase levels; no cementum & exfoliate prematurely esp md incisors

Hypophosmatemic Vitamin D – Resistant Rickets

dentin has cracks = pulpal infections

Taurodontism

bulls tth, large pulp chambers, found in Klinefelter

Amelogenesis Imperfecta

Type I: enamel does not develop to a normal thickness; Type II: hypocalcified AI; Type III: hypomaturation AI: softer enamel, snow-capped

Marfan Syndrome

slender build with long extremities, fish-like max, crowded tth, high max arch

Elhlers-Danlos Syndrome

CT disorder, flexible joints, healing concerns due to collagen, affects skin, blood vessels, joints

Pleomorphic

varies size

Hyperchromatic

dark nuclei, increase nuclei to cytoplasm ratio

Types of epic tumors

squamous, salivary, odontogenic

Papilloma

epi growth or sessile

HPV (papilloma)

verruca vulgarism (common wart): strain 3, 4; Condyloma acuminatum: sex transmit strains 6, 11; Orapharngeal found/malignancy: strains 16, 18

Erythroplakia

undiagnosed red patch, high potential for dysplasia

Leukoplakia

undiagnosed white patch; most common site = dental of tongue, floor of mouth, uvula/soft palate

Verrucas carcinoma

squamous cell carcinoma, buccal mucosa 

Basal Cell Carcinoma

high cure rate

Pleomorphic adenoma

: salivary gland material, palate Ameloblastoma: slow growing, invasive & aggressive, has epic & ameloblast cells, posterior md; primordial cysts can turn into ameloblastomas Calcifying odontogenic cyst: cyst lined w/ ghost cells, uni/multiocular Cementoblastoma: occurs most in md, hypercementosis, found on rt, well defined lesion on rt apices Odontoma (Odontogenic tumor): common areas ant mx, posterior md, children & young adults; made of dentin, cementum, enamel structures; Compound: ant max, resembles teeth; Complex: post md; failure of tooth eruption Lipoma: fat cells, buck mucosa, vestibular area Neurofibroma/Schwannoma: prolix Schwann cells, tongue Peripheral ossifying fibroma: derived from cells of PDL, contains fibrous CT, found on gingival tissue Fibroma (granula cell tumor): fibers tissue, found on tongue & buccal mucosa, painless Hemangioma: vascular lesion, blue in color, capillary prolif, common = tongue then lips, bucc mucosa Karposi Sarcoma: malig, HIV pt, common site = hard palate, caused by herpes virus Melanoma: blue/black mass, aggressive, common = palate & max ridge Melanotic nevi: well defined colored lesion Osteoma: ben bone mass, common in Gardner Syn Osteosarcoma: malign bone mass Chondrosarcoma: cartilage malig Multiple myeloma: Bence Jones Proteins (immunoglobulins) found in urine Rhabdomyosarcoma: soft tissue tumor of head/neck in children, most common, aggressive, poor prognosis Dysplasia: abnormal & disordered production of cementum & bone; abnormal cellular replication Periapical Cemento-Osseous Dysplasia: common disease of unknown cause that affects PA bone; anterior md 30+; circumscribed & radiolucent, overtime they become calcified; teeth affected are vital; hist exam shows fibro-fibr-osseous lesion composed of fibrous tissue & calcifications; early lesions consist of mainly fibrous tissue, where older lesions contain numerous calcifications; no treatment Fibrous Dysplasia: developmental, replacement of bone w/ abnormal fibrous CT; painless; progresive unilateral enlargement of md or max; malocclusion due to expanding bone; radiopacities resembles "ground glass”; lesions primarily radiolucent contain much fibrous CT; cafe au lait spots Paget Disease of Bone: chronic metabolic bone disease; resorption, osteoblastic repair, & remineralization of involved bone; maybe due to a virus; men 50+; max > md; enlargement of bone; pain; cotton wool; hypercementosis, loss of lamina dura, & obliteration of PDL can occur; serum alkaline phosphatase level is elevated in active disease; treatment = bisphosphonate Central Giant Cell Granuloma: vasculated fibrous CT w/ giant cells; surgical removal; occurs in ant portion of max/md (more common in md) Aneurysmal Bone Cyst: pseudocyst (no epic cells); blood-filled spaces w/ multinucleate giant cells & fib CT; X-rays = honeycomb/soap bubbles Osteomalacia: result of Vitamin D deficiency & induced by certain tumors; rickets Hyperpituitarism: excess hormone production by the anterior pituitary gland; caused by ben tum, a pituitary adenoma, that produces growth hormone; gigantism occurs before the closure of long bones; acromegaly occurs when hyper secretion occurs during adult life; enlargement of hands & feet; facial changes; macroglossia & thick lips; diagnosis = measurement of growth hormone; untreated: diabetes, CVD, respiratory disease, colon cancer Hyperthyroidism: excess production of thyroid hormone; Graves disease is an autoimmune disorder in which antibodies, thyroid-stimulating; excessive sweating, bulging eyes (exophthalmos), fine hair; premature exfoliation of deciduous teeth in children & premature eruption of perm teeth, osteoporosis may affect alveolar bone, burning tongue; treatment = medications to suppress thyroid activity, administration of radioactive iodine Hypothyroidism: decreased output of thyroid hormone, causes = developmental disturbances, autoimmune destruction of thyroid: Hashimoto thyroiditis, iodine deficiency, drugs, treatment for hyperthyroidism; enlarged tongue Hyperparathyroidism: results from excessive secretion of parathyroid hormone (PTH) from parathyroid glands; PTH plays a role in calcium & phosphorus metabolism; hypercalcemia: elevated blood levels of calcium; hypophosphatemia: low levels of blood phosphorus; middle-aged adults; PTH increased the uptake of dietary calcium from the GI tract & is able to move calcium from bone to circulating blood when necessary; bone changes: loosening of teeth; well-defined uniocular or multi ocular radiolucencies; “ground glass” appearance, loss of lamina dura; lesions appear to be central giant cell granulomas (CGCGs); measurement of PTH blood levels, to include serum calcium & phosphorus measurements Diabetes Mellitus: abnormally high blood glucose levels; Hyperglycemia: high blood glucose levels; results from a lack of insulin, defective insulin that does not work to lower blood glucose levels, or increased insulin resistance caused by obesity; glucose signals beta cells of the pancreas to make insulin; insulin secretes into blood stream to make the uptake of glucose into fat & skeletal muscle; fat & skeletal muscle cells can use glucose as an energy source; absence of glucose = cells starved of energy; (ketoacidosis: production of ketone acid/ketones that lowers the blood pH; most common endocrine disease in the US; breakdown of fatty tissue leads to production of ketone acid/ketones (large cells); ketone acid lowers the blood pH; acute condition can lead to coma & death; complications with organ systems = blindness, end-stage kidney failure, parathesia or numbness; Atherosclerosis of large & medium-size blood vessels: microvascular disease; diagnostic = fasting blood glucose > or equal to 126 mg/dL Prediabetes: fasting blood glucose 100-125 mg/dL Type 1: Insulin-Dependent Juvenile Diabetes: autoimmune disease; associated with Addison disease, Graves disease, pernicious anemia; insulin-producing cells of the pancreas are destroyed; pts need insulin forever; hypoglycemia: low blood sugar; severe hypoglycemia: insulin shock; treatment = oral hypoglycemic medications, insulin pump, drugs available today = (Metformin, Glibizide, Glyberide, Victoza, Trulicity), injectible insulin = (humalog, novolog, apidar, glucagon, reverses blood sugar level if too low as a result of insulin tx) Type 2: Non-Insulin Dependent & Non-insulin dependent Diabetes Mellitus: insulin resistance; gradual onset; 35-40+; weight gain; obesity decreases the # of receptor for insulin binding in fat & muscle Gestational Diabetes: occurs during pregnancy; disappears after pregnancy; increased birth weight of child = macrosomia; mother & child have higher risk of developing type 2; vascular system adversely affected; decreased resistance to infection; atherosclerosis causes impaired oxygenation & nutrition in tissue; diabetic retinopathy in the eye can lead to blindness; NS can be affected (neuraopathy); can have decreased resistance to infection; oral candidiasis, fungal infections in general (sugar = yeast), xerostomia, periodontal disease, accentuated response to plaque, slow wound healing, increased susceptibility to infection Addison Disease (Primary Hypoadrenocorticism): insufficient production of adrenal steroids; causes = malignant tumor, tuberculosis, deep fungal infections, HIV infection, AI disease, unknown; to compensate, the pituitary gland increases production of adrenocorticotropic hormone (ACTH); melanotic macule on oral mucosa; treatment = steroid replacement Hypercortisolism (Cushing Syndrome): caused by sustained increase in glucocorticosteroid levels; develops slowly; weight gain; hypertension, hyperglycemia, mood alterations, decreased ability to respond to stress Anemia: reduction in the oxygen-carrying capacity of blood; related to a decrease # of circulating RBC; pallor of skin & oral mucosa, angular cheilitis, erythema & atrophy of oral mucosa, loss of filliform & fungiform papillae on the dorsal of the tongue Iron Deficiency Anemia: insufficient amount of iron is supplied to bone marrow for RB development; Plummer-Vinson syndrome may result from long-standing iron deficiency anemia; lab tests show low hemoglobin content & reduced hematocrit; hematocrit: volume of RBC in blood; treatment = dietary supplements Pernicious Anemia: caused by deficiency in intrinsic factor; probably an autoimmune disorder in most situations; vitamins B12 is needed for DNA synthesis; weakness, pallor, fatigue on exertion, nausea, dizziness, diarrhea, abdominal pain, loss of appetite, weight loss; angular cheilitis, painful, atrophic, & erythematous mucosa, mucosal ulceration, loss of papillae on the dorsum of the tongue, burning & painful tongue; schilling test detects an inability to absorb oral vitamin B12; treatment = injection of vitamin B12 Folic Acid & Vitamin B12 Deficiency Anemia: from dietary deficiencies, malnutrition, increased metabolic requirements, alcoholism/pregnancy Thalassemia (Mediterranean or Cooley Anemia): inherited disorder of hemoglobin synthesis, autosomal dominant inheritance pattern; yellow skin pallor, enlarged spleen & liver, prominent cheekbones, prominent maxilla, “salt-&-pepper” pattern, thinning of lamina dura, circular radiolucencies in alveolar boneSickle Cell Anemia: inherited blood disorder; black individuals, Mediterranean, or asian origin;  RBCs develop a sickle shape when there is decreased oxygen; triggered by exercise, exertion, admin of general anesthetic, pregnancy, or even sleep; most common inherited disorder of RBC; can be present as early as 6 months of age; joint pain, heart enlargement & cardiac failure; loss of trabeculation, & large, irregular marrow spaces appear; treatment = admin of oxygen Aplastic Anemia: severe depression of bone activity causes a decrease in all circulating blood cells: pancytopenia; life-threatening blood disorder; low platelets: bruising, hematoma, ecchymosis (bruising of the skin), retinal & cerebral hemorrhages; WBC essential in defense again infection; infection, spontaneous bleeding, petechia, purpuric spots; leukopenia: decrease in WBC, thrombocytopenia: decree in platelets Polycythemia: increase in # of circulating RBC Polycythemia Vera (Primary Polycythemia): itching of skin: pruritus, vascular stasis, poor circulation, thrombocytopenia; deep red to purple oral mucosa, gingiva = edematous & bleeds easily, submucosal petechiae, ecchymosis, hematoma formation; lab testing & measurement of hemoglobin & hematocrit Disorders of WBC: 3 founds found in circulation (1) granulocytes (polymorphonuclear leukocytes (neutrophils), eosinophils, basophils) (2) lymphocytes (3) monocytes Agranulocytosis: reduction in circulating neutrophils; leukopenia: abnormally low WBC count; neutropenia: reduction in the # of circulating neutrophils; results from a problem in development of neutrophils or accelerated destruction of neutrophils Cyclic Neutropenia: rare form of angrulocytosis; severe depression of neutrophils cyclically; gingival inflammation, ulceration of tongue, ulceration of mucosal tissue, gingivalstomatitis; protect pt from opportunistic infections Leukemia: malignant neoplasms of hematopoietic (blood forming) stem cells; excessive # of abnormal WBC in circulating blood Acute Leukemias: immature cells & rapidly fatal course if not treated Acute lymphoblastic leukemia: immature lymphocytes, children & young adults; Acute myleoblastic leukemia: immature granulocytes, not good prognosis; sudden & dramatic onset, weakness/fatigue caused by anemia, enlargement of lymph nodes, thrombocytopenia: decrease in platelets; gingival enlargement caused by infiltration of leukemic cells; treatment = bone marrow transplant Normal platelet count: 200,000-400,000/mm3 Bleeding time (platelet function): 1-6 min PT (prothombin time): 11-16sec INR (internal natural ratio value): 2-3 used to monitor anticoagulant tx (coumadin) Purpura: red/blue discoloration of skin/mucosa due to tissue bleeding; submucosal bleeding if larger; petechia Thrombocytopenic purpura: bleeding disorder from low platelet Hemophilia: inherited disorder of blood coagulation; Type A and B: x-linked recessive trait, males predominantly; Type A: most common form Type B: Christmas disease, less common form Von Willebrand Disease: most common form of all inherited bleeding disorders; men & women Osteonecrosis: due to radiation of head/neck; due to decreased blood supply to bone (less oxygen) Chem TX: oral mucositis, candida, Low WBC for dental TX considerations MRONJ (Med. Related Osteonecrosis of the Jaw): biophosphonate tx; Fosamax; always begins as a blister, trauma, mandible most affected Sequestration: exfoliation of bone fragment, from tooth extraction/osteonecrosis Osteomyelitis: bone infection of the jaw, prob from PA infection Meds Affecting Gingiva: Dilantan (Phenytoin), Procardia (Nifedapine), Calcium channel blockers, beta blockers — all cause hyper plastic tissue Burning Mouth Disorder: xerostomia, diabetes, candida, mouth breather, meds, oral mucositis (NOT ALLERGIES) Dysgeusia: altered taste Trigeminal Neuralgia (Tic Douloureux): 5th cranial nerve, sharp/shooting/stabbing pain, electric shock-like pain, unilateral max trigeminal nerve is affected, pt’s have trigger points; when touched or temp change can trigger episode; pain raged by tegratol, Neurontin Bell’s Palsy: 7th cranial nerve, unilateral facial paralysis; may be triggered by a virus; unable to blink, smile, speech issues; resolve in 1-2 months or 6 months TMJ disorders: pain, restricted opening, crepitus (audible sound when opening (clicking, crackling)); diagnostic (MRI, panorex, CT scan); pt hx & clinical eval very important Trismus: unable to open mouth fully Subluxation: hyper