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Pathology
Pathology II
237
Pathology
Professional
02/20/2011

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Term
The skin has (high/low) pH to combat bacteria, and the genito-urinary tract has __________ to outcompete other bacteria.
Definition
Low......lactobacilli.
Term
"Vertical transmission"
Definition
AKA Placental fetal transmission
Term
Mechanisms of adhesion for bacteria......
Definition
Lipoteichoic acid (N. gonorrhoeae and S. pyogenes) and M protein (S. pyogenes).
Term
Mechanism of exotoxin for bacteria......
Definition
AB toxins of Anthrax; also superantigens.
Term
The etiologic agent for syphilis.
Definition
Treponema pallidum; resistant to phagocytosis (although there are no known endotoxins or exotoxins); seen in DARK FIELD MICROSCOPY; does not cause direct lysis of cells (eventually see a lymphocyte response); OCCUPATIONAL HAZARD FOR DENTISTS!
Term
Chancres are normally seen on..........
Definition
Genitalia 98% of the time (oral lesions are normally on the lip); histologically, we see periarteritis and endarteritis(cuts off blood supply).
Term
__% of syphilis patients progress to the secondary phase; 2-3 months after the chancre we see generalized papular lesions on the skin and mucous patches (HIGHLY CONTAGIOUS).
Definition
50%
Term
Papillary lesions in seconday syphilis that resemble viral papillomas.
Definition
Condylomata lata
Term
Explosive and widespread form of secondary syphilis in immunocompromised patients.
Definition
Lues maligna
Term
Immune complexes formed in secondary syphilis may progress to ____________ or ____________.
Definition
Transient arthritis or glomerulonephritis.
Term
__% of patients with secondary syphilis progress to tertiary syphilis 5-30 years later; no overt clinical signs of disease; cannot infect others except via placental transfer.
Definition
30%
Term
The biggest complication of tertiary syphilis.
Definition
Cardiovascular problems due to endarteritis obliterans causing aortic aneurysm and MI (80%); 10% have neurosyphilis (anterior spinal artery which can cause tabes dorsalis and primary optic nerve atrophy), and 10% have benign tertiary syphilis (gummas in organs).
Term
In patients with tertiary syphilis, ____________ causes general paresis.
Definition
Cerebral cortex invovlement; it is common to find spirochetes in these lesions.
Term
Intraoral gummas usually affect the ___________ and __________.
Definition
Palate (perforation) and tongue (interstitial glossitis- large, lobulated, and irregular in shape); treatment with arsenic can lead to luetic glossitis (loss of dorsal tongue papillae).
Term
Gummas are defined by......
Definition
A central area of coagulative necrosis surrounded by lymphocytes, giant cells, and fibrous tissue.
Term
Early congenital syphilis
Definition
Appears within 2 years of birth; skip chancre and go straight to secondary syphilis; progress to tertiary syphilis.
Term
Late congenital syphilis
Definition
Develops after 2 years of age; similar to tertiary syphilis.
Term
Hutchinson's Triad
Definition
1) interstitial keratits (corneal inflammation)
2) deafness
3) Hutchinson's incisors

Saddle nose is also common with congenital syphilis.
Term
To see if you've ever had syphilis, you need a _____________ test.
Definition
High-sensitivity with FTA-ABS or TPHA; is positive, need to see if it's active via a low-sensitivity test with RPR or VDRL.
Term
Mechanism of diptheria
Definition
C. diptheriae is a gram+ rod that produces AB toxin; blocks host cell protein synthesis; ADP-ribosylating causes inactivation of more than a millions EF-2 molecules; the A fragment of the toxin is responsible for catalyzing the transfer of ADP-ribose to EF-2.
Term
Hallmarks of diptheria
Definition
Grayish necrotic area on tonsils! Also, anorexia, vomiting, lymphadenopathy (bull neck); in severe cases, myocarditis and neurologic involvement (paralysis of oculomotor, pharyngeal, diaphragm, and intercostal muscles).
Term
Treatment for diptheria
Definition
Penicillin and anti-toxin; infection does not confer immunity; only cured when you have three straight negative cultures of the lesion.
Term
Gram+ cocci; many lead to suppurative infections of the skin, oropharynx, lungs, and heart valves.
Definition
Streptococcal bacteria
Term
Group A streptococci appear like this on the culture plate......
Definition
Non-motile, non-spore-forming organisms that form a 1-2mm culture on the plate surrounded by zones of complete hemolysis; some colonies produce hyaluronic acid, which appears as mucoid on the plate.
Term
Beta-hemolytic streptococci are classified on the basis of........
Definition
M-protein precipitin reactions.
Term
Group A streptococcal infections are associated with ___________ and _____________.
Definition
Acute rheumatic fever (ARF) and glomerulonephritis; also toxic shock syndrome (TSS) with group A streptococci tonsillopharyngitis.
Term
Gram+ cocci; many lead to suppurative infections of the skin, oropharynx, lungs, and heart valves.
Definition
M-protein
Term
A hemolytic toxin produced by GAS; antibodies to this are an indicator of recent infection; inactivated by oxygen; it is a single chain protein that induces a brisk antibody response.
Definition
Streptolysin O
Term
Produced by the bacteria in the presence of serum; non-antigenic; it is a polypeptide that has lytic effects for RBCs and WBCs (responsible for the hemolysis seen on culture plates).
Definition
Streptolysin S
Term
A toxin produced by GAS that destroys the hyaluronic acid in connective tissue.
Definition
Hyaluronidase
Term
Produced by GAS and are responsible for fever, scarlet fever rash, and TSS; produce cardiac and hepatic necrosis in animals; depress antibody synthesis while stimulating T-cell proliferation (superantigens).
Definition
A, B, and C exotoxins
Term
Toxins produced by GAS that assist in the liquefaction of pus and help to generate substrate for growth.
Definition
Deoxyribonuclease A, B, C, and D
Term
Two streptokinases produced by GAS serve to......
Definition
Form complexes with plasminogen activator and catalyze the conversion of plasminogen to plasmin (leading to digestion of fibrin).
Term
With streptococcal tonsillitis/pharyngitis, we usually don't see ________, _________, or ____________.
Definition
Rhinitis, laryngitis, or bronchitis - testing for strep should be discouraged if we see these; 25% of these cases are caused by Group A, beta-hemolytic streptococci.
Term
Scarlet fever starts out with _______ tongue but progresses to ______ tongue by day 4.
Definition
White strawberry tongue......strawberry tongue (prominence of fungiform papillae); Pastia's lines also seen at this point; "sunburn with goosebumps" rash that spares the palms, soles, and face; caused by an erythrogenic exotoxin; skin desquamates for up to 8 weeks after the rash clears.
Term
Etiologic agent of impetigo
Definition
S. pyogenes (different strain than the one causing tonsillitis) or S. aureus causing a superficial skin infection; usually arises over previous trauma or dermatitis.
Term
Two forms of pruritic lesions that occur with impetigo:
Definition
1) Fragile vesicles that erupt to form adherent amber crusts
2) Longer lasting bullous lesions that form honey-colored crusts (S. aureus associated)

Mistreatment with corticosteroids will reduce crusts but not clear the red raw lesions.
Term
AKA St. Anthony's Fire, Egyptian Healer, or French Hospital with Red Walls; can be confused with facial cellulitis from a dental infection; "orange peel" butterfly lesion similar to SLE; cultures are not beneficial; gangrene may be a complication.
Definition
Erysipelas
Term
Poststreptococcal glomerulonephritis is usually caused by GAS types __ and __.
Definition
12 and 49; more common in children with skin infections than pharyngitis; probably due to immune complex deposition within the glomerulus.
Term
__ and __ types of M-protein are associated with TSS.
Definition
1 and 3; associated with streptococcal pyrogenic exotoxins A, B, and C as well as streptococcal superantigen.
Term
Which two types of hepatitis are the most significant clinical diseases?
Definition
B and C; present in all body fluids except stool; Hepatitis B has infected about 2 billion people throughout the globe.
Term
Over __% of patients with acute hepatitis B do not have any readily identifiable risk factors.
Definition
30%; hepatitis B has one of the smallest genomes of all viruses.
Term
_____ is a core protein (antibodies to this do not help to clear the infection; only serve as a marker for infection) and ______ is a longer polypeptide with a precore and core region (present with clinical onset and disappears 2 weeks after; immune complexes with antibodies for this can cause glomerulonephritis, arteritis, and arthritis).
Definition
HBcAg and HBeAg
Term
A viral protein expressed in the surface of the hepatocyte; T-cell then induces apoptosis via TNF, etc with the help of Fas ligand; it is detected 2 months to 2 weeks before clinical onset, and antibodies to it provide lifelong immunity.
Definition
HBsAg
Term
A protein from a region of hepatitis B that may play a role in the causation of hepatocellular carcinoma.
Definition
HBx
Term
Of the patients with acute hepatitis, __% go on to fulminant hepatitis.
Definition
1%; the other 99% make a recovery.
Term
Of the 4% of patients that have chronic persistent hepatitis, _______% become chronic active.
Definition
10-33%; of these people, 20-50% develop cirrhosis/hepatocellular carcinoma; many chronic carriers have virions in their hepatocytes with no evidence of cell injury. 70-90% of people with chronic persistent hepatitis go on to make a recovery.
Term
4 stages of acute hepatitis
Definition
1) Incubation
2) Pre-icteric- when you become jaundiced (not common in children.....50% of adults become jaundiced).
3) Icteric (fever, malaise, loss of appetite, vomiting).
4) Convalescence
Term
A finding in hepatitis where bile plugs (canaliculi) and brown pigmentation are seen in the hepatocytes; there is also ballooning degeneration of the hepatocytes.
Definition
Cholestasis
Term
Two patterns of hepatocyte death in hepatitis:
Definition
1) Cytolysis- necrotic cells have "dropped out"
2) Apoptosis- intensely eosinophilic and fragmented nuclei
Term
Fatty change is associated with hepatitis __.
Definition
C
Term
_________ is often seen in Kupffer cells which have undergone hypertrophy and hyperplasia from hepatitis.
Definition
Lipofuscin (from phagocytosis of hepatocellular debris).
Term
Inflammation during hepatitis mainly occurs in the ________ region of the hepatocyte.
Definition
Portal region
Term
The main cause of death with fulminant hepatitis
Definition
Cerebral edema due to decreased osmolarity of blood; there is massive hepatic necrosis with collapse of the reticulin framework; liver is shrunken to as little as 500g! Surprisingly we may not see much inflammation until the cleanup process occurs days later.
Term
________ and ________ are most susceptible to chronic hepatitis.
Definition
Children (mother to infant) and men (women more likely to get autoimmune hepatitis); also, homosexual orientation and altered immune function increase chances.
Term
During chronic persistent hepatitis, _______ levels are high but there is little viral replication.
Definition
HBsAg levels are high; we see "Ground Glass Hepatocytes"; inflammation is limited to portal tract region with Kupffer cells appearing normal and serum aminotransferase levels being low.
Term
During chronic active hepatitis, we see........
Definition
Fibrotic strands linking portal tracts to central vein (cirrhosis!); "piecemeal necrosis" (inflammation around portal tracts making them look irregular) is a hallmark of progressive disease.
Term
Hepatocellular carcinoma develops in __% of patients with __________.
Definition
20% of patients with cirrhosis regardless of whether it's from HBV or not; carcinoma is far more likely to occur in individuals exposed to HBV in childhood or infancy.
Term
_________ after birth reduces hepatitis B infection by 95%
Definition
HBsAg immunoglobulin (antiserum?)
Term
An explosive form of HPV where warts cover the entire body (genetically related); 30% chance of developing malignancy (higher with trauma or sun exposure).
Definition
Epidermodysplasia verruciformis
Term
Small, non-enveloped icosahedral viruses; most common human viral infection!
Definition
Papillomaviruses
Term
HPV initially infects __________; mature virions are shed from the ____________ layer.
Definition
Basal cells in the epithelium......stratum corneum.
Term
Characteristic perinuclear vacuolization in epithelial cells due to HPV.
Definition
Koilocytosis
Term
HPV DNA is maintained as an _____________.
Definition
Episomal plasmid; however, some HPV DNA integrate into the cell's DNA (smoking, oral contraceptives, STDs) and can cause malignancy!
Term
HPV enters the carcinogenesis pathway by.........
Definition
Inactivating tumor suppressors p53 (via E6) and pRb (via E7 to prevent Rb's interaction with E2F transcription factors to allow virus entry).
Term
The majority of HPV infections are ________.
Definition
Latent with no visible lesions; acetic acid can show keratinized tissue in a wart.
Term
Genital warts; most common benign genital tumor; associated with HPV 6 and 11 (same as for oral lesions!); laryngeal papillomas are a rare condition found in both adults and children.
Definition
Condylomata acuminata
Term
HPV types most commonly associated with cervical cancer.....
Definition
16, 18, 31, and 35; 16 is the most dangerous with deletions of 3p and amplification of 3q in a chromosome; HPV can also prevent replicative senescence by upregulating telomerase via E6.
Term
Diagnosis of HPV.......
Definition
DNA hybridization; currently there are no serological markers
Term
Mechanism of Epstein Barr Virus........
Definition
The EBV envelope contains a glycoprotein that binds C3d complement on B cells; B-cells are the main reservoir in latent infections; during active infection these B cells are lysed.
Term
T cell appearance during EBV.......
Definition
Abundant cytoplasm, multiple clear vacuoles, and an oval, indented, or folded nucleus; cytoplasmic azurophilic granules; EBV is diagnosed with a peripheral blood smear.
Term
Enlarged spleen from EBV in the follicles and red pulp sinusoids can lead to ___________; in the liver, elevated lymphocyte levels can cause ____________.
Definition
Thrombocytopenia (increased bleeding time!)........parenchymal necrosis (CNS edema possible).
Term
Most EBV infections have a ___________ phase.
Definition
2-week prodromal phase; except in infants and toddlers who are asymptomatic (developing countries); these latent people have polyclonal activation/proliferation of B-cells.
Term
Only give corticosteroids to treat EBV if there is ______________.
Definition
Airway obstruction (we need the immune system to overcome the virus); only give non-aspirin anti-pyretics.
Term
AIDS patients can experience this complication from EBV.
Definition
Life-threatening polyclonal B-cell proliferation (do not give corticosteroids!)
Term
Histoplasmosis capsulatum has _____________ phase at body temperature and __________ phase in its natural environment.
Definition
Haploid unicellular phase...........mold-like phase.
Term
A self-limited pulmonary infection with symptoms similar to the flu.
Definition
Acute histoplasmosis
Term
Chronic histoplasmosis usually affects.......
Definition
Elderly, emphysematous, or immunosuppressed individuals; much less common than acute histoplasmosis; reminiscent of TB; 20% mortality if left untreated.
Term
Only occurs in 0.5% of patients with acute histoplasmosis; same type of people that get chronic histoplasmosis; oral lesions may be white or erythematous; solitary, variably painful ulcerations in the mouth; some larger oral lesions have firm, rolled margins that may look like malignancy; mortality is 90% if untreated (Amphotericin B!!!)
Definition
Disseminated histoplasmosis
Term
A Grocott-Gomori stain will make the field _________ with the fungi appearing ________.
Definition
Silver.....black; a Periodic Acid Schiff stain will also make the fungi stand out; fungi have their own nuclei and cell bodies.
Term
Treatment for histoplasmosis.....
Definition
Antifungal agents (Amphotericin B but causes kidney toxicity, ketoconazole, itraconazole).
Term
Two phases of candida albicans
Definition
Yeast and hyphal (more invasive); part of normal oral flora in 30-50% of people.
Term
AKA thrush; creamy white removable plaques; burning sensation; foul taste; often caused by antibiotic therapy and immunosuppression.
Definition
Pseudomembranous candidiasis
Term
Erythematous candidiasis can occur from...........
Definition
Denture stomatitis (not a true infection since it's only on the denture) or angular cheilitis (most often S. aureus); in acute atrophic candidiasis, we see red macules with loss of filiform papillae on the tongue ("scalded tongue"); caused by too many antibiotics.
Term
A form of erythematous candidiasis that is similar to central papillary candidiasis; "kissing lesion" between tongue and hard/soft palate; caused by immunosuppression or is idiopathic.
Definition
Chronic multifocal candidiasis
Term
Sabouraud's agar slant
Definition
A test used to determine denture stomatitis; denture will show organismal growth while mucosa will not.
Term
White plaques that are not removable; not a true leukoplakia because it cures with anti-fungals; patient is asymptomatic.
Definition
Hyperplastic candidiasis
Term
Thick white patches that do not rub off, but lesion also has many characteristics of erythematous and pseudomembranous candidiasis at the same time; associated with immunologic disorders leading to autoimmunity (a recessive genetic form has also been found); the autoimmunity usually targets endocrine glands (we see the candidiasis first though!).
Definition
Mucocutaneous candidiasis
Term
_____ shows loss of rete ridges while ____ has elongated rete ridges.
Definition
HPV.....mucocutaneous candidiasis.
Term
Actinomycosis is normally caused by these two bacteria:
Definition
1) A. israelii
2) A. viscosis

Filamentous, branching Gram+ bacteria that are part of the normal oral flora.
Term
Characteristics of actinomycosis infection.......
Definition
Painless, indurated swelling that doesn't follow normal facial spaces; SULFUR GRANULES surrounded by neutrophils form a GRANULOMA; associated with fibrosis.
Term
Actinomycosis lesions demonstrate......
Definition
Both acute and chronic inflammation at the same time; organisms surrounded by PMNs which are surrounded by chronically inflamed granulation tissue that is surrounded by fibrosis; organisms within the abscess form a rosette pattern.
Term
Treatment for actinomycosis
Definition
6 weeks of antibiotics if caught early; up to a year if late! Need to penetrate fibrosis; this disease is non-transmissible.
Term
MEN 2B
Definition
Multiple Endocrine Neoplasia 2B; mucosal neuromas on tongue and lips (pathognomic for this disease); medullary thyroid cancer, pheochromocytoma, hypotonia, loose joints, and long face.
Term
Phenylketonuria
Definition
Defect in phenylalanine hydroxylase causing production of various toxic secondary metabolites (autosomal recessive); eczema, severe mental retardation, seizures and neurologic abnormalities.
Term
APC, BRCA1, and BRCA2
Definition
Cancer susceptibility genes that are autosomal dominant.
Term
A "gain of function" autosomal dominant disease is ____________ while ___________ is a "loss of function."
Definition
Achondroplasia.........Marfan Syndrome.

Huntington's Disease is caused by a "gain of function" toxic protein.
Term
Defect in DSG1 gene; blue/brown or opalescent teeth; pulp chambers may be absent.
Definition
Dentinogenesis imperfecta type II
Term
FGF-3 defect, trident hand, prominent brow, depressed bridge of nose, and small foramen magnum; dental malocclusion with an anterior overbite!
Definition
Achondroplasia
Term
The gene mutation that causes achondroplasia is simply a..........
Definition
Single mutation of a glycine to an arginine in fibroblast growth factor 3 (FGF-3); it is the most mutable single base in the human genome.
Term
Mutation of Fibrillin 1 or 2; narrow mouth with high palate (crowded teeth); off-center lens in the eye and myopia; decreased elasticity of lung tissue, blood vessels, and heart valves (AORTIC DISSECTION!); caved-in or pushed-out breastbone; prominent stretch marks from growth.
Definition
Marfan Syndrome

Give beta blockers to decrease the blood pressure to prevent aortic rupture.
Term
A female that is heterozygous for an X-linked recessive gene will eliminate the bad gene if it's a fatal mutant as in ____________________.
Definition
Ornithine transcarbamylase mutation
Term
____________________ is an example of a mitochondrial inheritance mutation.
Definition
Leber hereditary optic neuropathy; may be ok thanks to heteroplasmy.
Term
Deletion of chromosome 15 can cause _____________ or _____________ depending on if the mutation was inherited from mother or father.
Definition
Prader-Willi (UVE ligase missing) or Angleman syndrome (ubiquitin ligase missing)
Term
_________ is when mutations is different genes at different loci cause similar/same disease.
Definition
Genocopy
Term
___________ is an example of variable expressivity when varying mutations within the same gene cause different degrees of disease; due to ALLELIC VARIATION.
Definition
Neurofibromatosis 1

Variable number of cafe au lait, skeletal malformations, and cardiac defects.
Term
________ genes may alter the impact of a genetic mutation relative to someone else with the same mutation.
Definition
Modifier genes
Term
Examples of allelic variation when mutations is the same gene can cause different diseases.
Definition
MEN2A (exon 10,11), MEN2B neuromas (exon 15,16), and familial medullary thyroid carcinoma (exon 10-15).
Term
Examples of trinucleotide repeat diseases
Definition
Huntington's disease, fragile X syndrome, and Kennedy disease.
Term
Examples of multifactorial inheritance diseases
Definition
Cleft lip/palate, neural tube defects, diabetes, schizophrenia, and coronary heart disease.

There is some degree of familial aggregation.
Term
A chromosomal microdeletion condition that is characterized by supravalvular aortic stenosis, elfin facies, short stature, dental malformation, lacey iris pattern, infantile hypercalcemia, and happy disposition.
Definition
Williams syndrome
Term
Williams syndrome has a deletion at chromosome __ and patients are........
Definition
7q....retarded but with normal IQs (strengths in muscial and verbal areas); affects elastin gene (aortic stenosis) and LIM-kinase-1 gene (poor visuospatial development).
Term
A balanced translocation can have the features of a ________________ gene disorder.
Definition
Autosomal dominant single gene disorder; you'll be ok but your offspring may get double of one chromosome and none of the other.
Term
_____________ is characterized by a specific subset of balanced translocations which involve the acrocentric chromosomes fusing at the two short ends.
Definition
Robertsonian translocation
Term
An aneuploidy condition where there are three copies of chromosome 21; short neck, flat nasal bridge, Brushfield spots on the iris, incurving of the fifth finger.
Definition
Down syndrome

One third of patients have congenital heart disease; premature senility similar to Alzheimer's; in 5% of people with the disease, there is another type of translocation other than chromosome 21.
Term
45X chromosomal make-up; webbed neck, low posterior hairline, growth retardation, failure to develop secondary sex characteristics, high arched palate, and aortic/kidney malformations.
Definition
Turner syndrome
Term
Acute abscess arising within a periapical granuloma.
Definition
Phoenix abscess
Term
Osteomyelitis is more common in (men/women) and (mandible/maxilla); hypovascularity of the area may be caused by ____________ or ______________.
Definition
Men.....mandible; Paget's disease or florid cemento-osseus dysplasia.
Term
Bone production at the apex of a tooth; usually lower first molar.
Definition
Focal sclerosing osteomyelitis (Condensing Osteitis); mild pain but otherwise asymptomatic; bone is vital, but tooth is dead :(
Term
In order for caries crystals to form, _______________ must be viable.
Definition
Odontoblastic processes; caries crystals is different than dentinal sclerosis.
Term
Chronic reparative dentin is more (organized/irregular).
Definition
Organized; the more irregular the dentin is, the more impermeable it will be.
Term
A condition when pulp gets completely covered by dentin; the tooth is non-vital and looks yellow/brown.
Definition
Calcific metamorphosis
Term
Pulp is RAPIDLY invaded by VIRULET bacteria.
Definition
Acute pulpitis
Term
Cellulitis can occur instead of an abscess when the infection is above or below this muscle.
Definition
Buccinator
Term
Abscess in the gingiva.
Definition
Parulis
Term
Chronic pulpitis will lead to a _______________.
Definition
Periapical granuloma (not a true granuloma); we see collagen, fibroblasts, and mast cells.
Term
Tooth decay where most of the pulp is exposed to the environment; chronic hyperplastic pulpitis as a protective mechanism.
Definition
Pulp polyp; tooth is still VITAL; give CaOH to relieve the hyperplasia.
Term
Bone appearance during acute osteomyelitis.......
Definition
Bone has no osteocytes; necrotic bone becomes sequestered; we should do minimal debridement unless necessary; do NOT surgically remove infected bone.
Term
During chronic osteomyelitis, we see.........
Definition
Scar tissue replacing bone; this lesion WILL STILL SPREAD; still see jagged resorption around bone; surgical intervention is mandatory and hyperbaric oxygen may be necessary.
Term
Teeth appear fused but tooth count is normal.
Definition
Gemination
Term
Three conditions which cause the formation of pathologic tertiary dentin:
Definition
1) attrition (bruxism)
2) abrasion (can also be caused by sucking on orange peels)
3) Erosion
Term
Long-necked "bull teeth"
Definition
Taurodontism
Term
Hypercementosis is common with ____________.
Definition
Paget's disease
Term
Hypodontia can be caused by ___________ or ___________.
Definition
Ectodermal dysplasia (most common variant is X-linked) or hereditary oligodontia (everything else is normal but the teeth; can be caused by several genetic mutations).
Term
Hyperdontia can be caused by _____________ or ______________.
Definition
Cleidocranial dysplasia (patient also missing clavicles) or familial adenomatous polyposis (patients predisposed to cancer, etc.)
Term
AMELX, ENAM, MMP20, KLK4, WDR72, and FAM83H
Definition
Genes that can cause amelogenesis imperfecta; can be autosomal dominant, recessive, or X-linked; enamel is hypoplastic, hypomatured, and hypocalcified.
Term
Type I dentinogenesis imperfecta is caused by _______ or _______ genes while Type II dentinogensis imperfecta is caused by the ______ gene.
Definition
COL1A1 or COL1A2.........DSPP (only teeth affected).
Term
Radiographic findings for dentinogenesis imperfecta.
Definition
Pulpal obliteration in most cases (or else "ghost teeth"), cervical constriction, and canals either enlarged OR obliterated.
Term
Defect in radicular dentin; "rootless" or "stumped" teeth; pulps obliterated, prone to infection; severely affects deciduous teeth.
Definition
Type I dentin dysplasia
Term
Defect in coronal dentin; DSPP mutations.
Definition
Type II dentin dysplasia

Identical to dentinogenesis imperfecta (just a specific genotype).
Term
A metabolic bone disease that causes a deficiency in alkaline phosphatase; increased urinary phosphoethanolamine; absence or decrease in the amount of cementum creating enlarged pulps and spontaneous tooth exfoliation.
Definition
Hypophosphatasia
Term
Defects in these two genes can cause hypophosphatasia.
Definition
ALPL or DLX3
Term
Porphyria (inability to metabolize heme) can cause this in the oral cavity.
Definition
Abnormal tooth color; the dentin is also soft.
Term
An oral condition that causes "ghost teeth", thin enamel (no contrast between enamel and dentin), enlarged pulps, usually affects contiguous teeth, and would not exclusively occur in permanent teeth.
Definition
Regional odontodysplasia
Term
Which immunoglobulin has no memory?
Definition
IgM
Term
___________ and ___________ play a role in both the innate and adaptive immune systems.
Definition
Neutrophils and macrophages
Term
The order for most common immunoglobulins in serum is......
Definition
IgG > IgA > IgM
Term
Ig__ is primarily involved in agglutination.
Definition
IgA; IgG, IgD, and IgG can be the receptor on B-cells.
Term
Classical pathway to make a C3 convertase...........
Definition
Activated antibody (mannose binding lectin) that changes its Fc region and binds C1, C2, and C4.
Term
Alternative pathway for C3 convertase......
Definition
Bacteria activates residual C3b which binds Factors P, B, and D.
Term
Which two complement proteins are anaphylatoxins?
Definition
C3a and C5a
Term
Which two complement proteins are chemotactic factors?
Definition
C5a and C567
Term
Which complement proteins are involved in virus neutralization?
Definition
C1-C4 are bound by antibody-coated virus.
Term
Donnan effect
Definition
Water enters a cell with the hydrophilic transmembrane channels created by complement-mediated cell lysis; this causes the cell to swell and burst.
Term
Which two interleukins downregulate lymphatic activity?
Definition
IL-10 and TGF.
Term
A CD4 T-cell can either differentiate into a _______ or _______.
Definition
Memory T-cell or CD8 cytotoxic cell via cytokines.
Term
Two of the cytokines produced by cytotoxic T-cells are.......
Definition
Perforin and TNF
Term
Class I MHC binds........
Definition
Peptides derived from proteins synthesized in cells (i.e. viral protein).
Term
Class II MHC binds.......
Definition
Peptides from exogenous proteins that are first internalized and processed.
Term
Natural killer cells do not have _____________ or express ___________.
Definition
No antigen receptor nor do they express cell surface CD3; has an inhibitory self Class I MHC; can induce APOPTOSIS via direct contact or ADCC (tumor resistance); also called Large Granular Lymphocytes (LGLs); part of the INNATE IMMUNE SYSTEM!
Term
__________ is a substance that enhances the immunogenicity of an antigen; polyclonal cell activators (PCA) are an example of these on bacteria or plants that NONSPECIFICALLY activate the immune system (both T and B cells).
Definition
Adjuvants

Mixed with vaccines to make antigens last longer; calcium phosphate, etc.
Term
Atopy
Definition
Umbrella term for diseases associated with the production of IgE; can be due to both genetic and environmental factors.
Term
Reaginic antibody
Definition
Term applied to those antibodies responsible for allergic responses; only IgE causes anaphylaxis.
Term
CD__ is the part of the Fc receptor on IgE (and sometimes IgG) that allows it to attach to mast cells.
Definition
CD23; the antibody is now "homocytotropic"
Term
When IgE is crosslinked by an antigen, these mediators play a role.....
Definition
Histamine (also stimulated by platelet activating factor), eosinophil chemotactic factor (ECF-A), neutral proteases (cleave complement), and heparin (a proteoglycan that serves to package other secondary mediators).
Term
________ is the most common form of allergic disease.
Definition
Allergic rhinitis (can cause conjunctivitis as well); chronic rhinitis may cause serous otitis media as well.
Term
________ is a transient eruption of erythematous and/or edematous swelling of the dermis in response to Type I hypersensitivity.
Definition
Urticaria

Hereditary angioedema (affects face and lips) is NOT Type I hypersensitivity (C1 complement inhibition).
Term
A manifestation of allergic gastroenteritis is.......
Definition
Perioral erythema along with hives and vomiting.
Term
Anaphylactic shock can be avoided by giving the patient __________.
Definition
Cromolyn sodium (inhibits mast cell degranulation).
Term
In Type II hypersensitivity, complement activation can cause __________ and ___________.
Definition
Neutropenia and lymphopenia; C3b if complement is involved or ADCC by NK cells if complement activation is absent.
Term
Haptens usually involve this type of cell.
Definition
Blood cells
Term
The warm type of hemolytic anemia is.......
Definition
Extravascular hemolysis in the spleen mediated by IgG.
Term
________ is a passive anti-serum that attacks Rh antigen before mom can.
Definition
Rhogam; jaundice in the mother is a sign of Erythroblastosis fetalis (Type I Hypersensitivity along with hyperacute allograft rejection)
Term
Type III hypersensitivity reactions can cause necrosis either by.......
Definition
PMN release of lysozymes (via complement activation) or ischemia (via platelet and Hageman factor activation).

There can also be just vasodilatino and edema via kinin and complement activation.
Term
Hepatitis B can cause a Type III hypersensitivity reaction called ______________.
Definition
Polyarteritis nodosa (vasculitis of medium-sized vessels).
Term
The best type of antigen to cause a Type III hypersensitivity reaction is......
Definition
A multivalent antigen that is in slight excess of antibodies and forms intermediate-sized complexed that can fix complement.
Term
Ig__ and Ig__ are involved in Type III hypersensitivity because they can.......
Definition
IgG or IgM because they can activate complement.
Term
Most immune complexes require CD4 T-cells to induce class switching on B-cells; however, this is an exception......
Definition
ABO antigens on RBCs can induce the release of IgM by themselves!
Term
_________ necrosis is seen in Type III hypersensitivity reactions.
Definition
Fibrinoid necrosis; not true necrosis though; sign of an advanced stage of the disease.
Term
The Arthus Reaction
Definition
Local injection of antigen to which IgG antibodies have been made will result in vasculitis and possibly vessel occlusion.
Term
______________ is an example of a Type III hypersensitivity reaction.
Definition
Hypersensitivity pneumonitis (caused by Micropolyspora faeni resulting in Farmer's lung, etc.).
Term
Example of a microbial antigen causing a Type III hypersensitivity reaction.
Definition
Syphilis resulting in subacute bacterial endocarditis; periodontal disease is also thought to be able to form immune complexes from plaque bacteria.
Term
DTH reactions are directly dependent upon........
Definition
The development of an altered state of macrophage activity whose ultimate goal is the accumulation of nonspecific cytotoxic macrophage effector cells (increased size and enzymes of the Embden-Meyerhoff pathway and HMP shunt).
Term
In order for macrophages to become activated in DTH reactions, they need these three lymphokines....
Definition
1) IFN-gamma
2) IL-4
3) GM-CSF
Term
In contact dermatits, we see........
Definition
Intercellular edema (spongiosis) and vascular cuffing (vessel not destroyed though).
Term
The main types of T-cells present in DTH reactions are......
Definition
CD4Th1 and CD8; usually no need for antibody production via CD4Th2 cells.
Term
Bacillic Calmetta Geurin (BCG)
Definition
An attenuated strain of TB for vaccination.
Term
Ghon complex
Definition
An early TB lesion draining to the hilar lymph nodes (this is primary TB unlike miliary TB where there are granulomas all over the body).
Term
"Activation-induced death"
Definition
Self antigen is presented to a CD4 cell in the context of MHC Class I; CD4 cell then undergoes apoptosis, thus killing off that clone.
Term
In ___________, intrinsic factor is the antigen which causes megaloblastic anemia and reduced hematocrit.
Definition
Pernicious anemia

Other hematopoietic problems arise from large megakaryocytes.
Term
In ___________, the antigen is a membrane glycoprotein IIb/IIIa complex.
Definition
Autoimmune thrombocytopenia

IgG and IgM cause classic low platelet manifestations.
Term
The destructive agent in Type I diabetes.
Definition
T-cells (auto-antibodies are not destructive); there is a genetic susceptibility associated with specific alleles of Class II MHC.
Term
Some of the clinical manifestations in diabetics include.......
Definition
Bacterial and fungal infections causing pyelonephritis from chronic renal failure; ketoacidosis potentiates the growth of Mucor in the nasopharnygeal region; periodontal disease is more aggressive due to the vascular pathology.
Term
Three mechanisms that account for the loss of ACh receptors in myasthenia gravis:
Definition
1) Crosslinking of receptors with their removal by internalization
2) Destruction of receptors by the action of complement
3) Blocking of the receptors

Can cause ptosis
Term
In _____________, the antigen is thyroglobulin (thyroid peroxidase) causing high TSH levels and weight gain; also fatique and myxedema.
Definition
Hashhimoto's Thyroiditis

Several types of antibodies including antimicrosomal and antithyroglobulin antibodies.
Term
Patients with this HLA gene have a predisposition to Hashhimoto's Thyroiditis, and patient's with HLA-___ are susceptible to Graves disease.
Definition
HLA-DR5..........HLA-DR3
Term
In ___________, the antigen is the TSH receptor which activates adenylate cyclase pathways; causes weight loss and low TSH levels.
Definition
Graves disease

The antibodies are called thyroid growth-stimulating immunoglobulins (TGIs)
Term
Clinical manifestations of Graves disease.....
Definition
Exophthalmos and infiltrative dermopathy on the dorsum of the legs or feet (accumulation of glycosaminoglycans with inflammatory cells; no inflammatory cells in the thyroid (unlike Hashhitmoto)....just too many follicular cells with too little colloid.

Orally, premature exfoliation of deciduous teeth and osteoporosis of the jaws.
Term
In ____________, the antigen is a cross reacting antibody (antigenic mimicry) that occurred from fighting beta-hemolytic streptococci; we see carditis due to Type II response and cardiac dilation with possible insufficiency of the mitral valve.
Definition
Rheumatic fever
Term
In rheumatic fever, this is seen histologically and is pathognomonic.
Definition
Aschoff bodies; foci of collagen necrosis with firbin deposition and surrounded by lymphocytes, macrophages, and Aschoff cells; sometimes the Aschoff cells form multinucleated giant cells.

Aschoff bodies mainly affect heart valves and are replaced by a fibrous scar.

Anitschkow cells are also seen (they make up the Aschoff body).
Term
In __________, the antigen is a cytoskeletal protein (alpha-fodrin); due to B-cell dysfunction, hypergammaglobulinemia, rheumatoid factor in the serum, antinuclear antibodies, keratoconjunctivitis, and xerostomia.
Definition
Sjogren syndrome

A lip biopsy is REQUIRED to show T-CELL infiltrate in the minor salivary glands.
Term
Primary Sjogren Syndrome vs. Secondary Sjogren Syndrome
Definition
Primary is when the sicca complex (dry eyes and dry mouth) occurs in conjunction with a connective tissue disease, and Secondary is when the sicca complex occurs by itself.
Term
In _____________, the antigen is alpha-3 chain of Type IV collagen in the basement membrane; mainly affects lung and kidney (hemorrhagic).
Definition
Goodpasture disease

In connective tissue disease, the antigen is ALWAYS soluble!
Term
In multiple sclerosis, how do the "plaques" vary with age?
Definition
Initially, they are slight pink and swollen; later, they become gray, sunken, and opalescent.

Most of the damage is done by T-cells and macrophages; earliest loss of myelin is seen around small veins and venules.
Term
In SLE, antibodies to __________ and __________ are virtually diagnostic of the disease.
Definition
Double-stranded DNA and the Sm(Smith) antigen.

Antigens are both soluble and tissue-bound (antibodies against platelets and blood cells)!
Term
LE bodies
Definition
Nuclei of damaged cells react with antinuclear antibodies, lose their chromatin pattern, and become homogenous.

LE cells are only seen in vitro.
Term
This clinical manifestions is considered highly specific for SLE.
Definition
Granular deposits in uninvolved skin.
Term
Systems affected by SLE:
Definition
1) Kidney- glomerulonephritis
2) Serosal membranes- pericarditis and pleuritis; foci of fibrinoid necrosis
3) Cardiovascular system- Libman-Sacks endocarditis, tachycardia, and ECG abnormalities.
4) Oral lesions with similar histology to skin lesions
Term
In ___________, the antigen is IgG that is abnormally glycosylated; may resemble SLE and involve other organs.
Definition
Rheumatoid arthritis
Term
This finding is pathognomonic for rheumatoid arthritis.
Definition
Rheumatoid subcutaneous nodule; partially moveable, nontender oval or rounded nodules.

They appear on the extensor surfaces of joints; central focus of fibrinoid necrosis surrounded by macrophages which in turn is rimmed by granulation tissue.
Term
Skin manifestations of lichen planus.....
Definition
Purple, pruritic, and polygonal papules that are limited to the flexor surfaces of the extremities.

Not an autoimmune disease.....Type IV hypersensitivity.
Term
In lichen planus, the basal cell layer exhibits __________ and the epithelial cells form ___________.
Definition
Hydropic degeneration.......colloid bodies.
Term
______________ is a condition which looks similar to lichen planus, but the lymphocyte infiltrate is not as dense; can be caused by medications; we also see PERIVASCULITIS.
Definition
Lichenoid mucositis
Term
________________ is an autoimmune disease that looks like lichen planus but does not respond well to topical corticosteroids.
Definition
Chronic ulcerative stomatitis
Term
BPAg1, BPAg2, and epiligrin are known antigens for ____________.
Definition
BMMP; diagnosis often made by histology alone; ophthalmology consult is mandatory!
Term
Bullous pemphigoid differs from BMMP in that.......
Definition
Skin lesions are almost always present, heals without scarring, has different epitopes on antigens BPAg1 and BPAg2, and both DIF and IIF can be used.
Term
In ___________, the basal cells look like tombstones lined up in a row.
Definition
Pemphigus vulgaris; mortality is usually due to complications from treatment.
Term
The lymphocyte responsible for recurrent aphthous stomatitis is..........
Definition
CD8 T-cells; there is a decreased percentage of CD4 T-cells.
Term
Which form of aphthous stomatitis is the most severe?
Definition
Major form (Sutton's disease); ulcerations tend to heal with scarring.

Topical corticosteroids may help.
Term
The diagnosis for Behcet's syndrome......
Definition
6 or more aphthae that have recurred at least 3 times in 1 year AND at least two of the following:

1) ocular lesions
2) skin lesions
3) positive pathergy test

Aphthous ulcers usually precedes other sites of disease involvement.
Term
Secondary herpes (like herpes labialis) differs from primary herpes in the fact that.............
Definition
Systemic signs and symptoms are usually not seen.
Term
The pathognomonic lesion for erythema multiforme minor.......
Definition
Target-like or iris lesion (not always present though); lips are often covered with blood-filled blisters that may have a fibrinous pseudomembrane.
Term
Erythema multiforme major (Steven-Johnson syndrome) differs from EM minor in the fact that.......
Definition
It is usually triggered by a drug, and ocular and genital mucosa are often involved; severe involvement of the eye may lead to scarring.
Term
Toxic epidermal necrolysis (Lyell's disease) differs from the other forms of EM in the fact that.............
Definition
It occurs in older people and affects WOMEN MORE THAN MEN; potentially life-threatening diffuse sloughing of the skin and mucous membranes.
Term
Differential diagnosis for desquamative gingivitis (painful loss of epithelium covering gingiva).
Definition
1) Erosive lichen planus/lichenoid mucositis
2) Cicatricial pemphigoid
3) Pemphigus vulgaris
Term
Complement deficiencies often lead to infections by __________ and __________.
Definition
N. meningitidis and N. gonorrhea
Term
Failure of pre-B cells to differentiate into mature B-cells; mutation in tyrosine kinase; rearrangement of immunoglobulin heavy chain genes; does not become apparent until 6 months of age.
Definition
X-linked agammaglobulinemia; patient is susceptible to some viruses such as echovirus and enterovirus infections as well as Pneumocytis carinii and Giardia lambdia.
Term
Inability of B-cells to differentiate into plasma cells; may be due to T-cell, B-cell, or suppressor T-cell deficit; presents clinically as a respiratory tract infection.
Definition
Common variable immune deficiency (acquired or late onset agammaglobulinemia)
Term
T-cell deficiency resulting from an intrauterine accident; patient is also susceptible to intracellular bacteria due to lack of cytokines required by macrophages.
Definition
Di-Georges syndrome
Term
Clinical manifestations of Di-Georges syndrome
Definition
Aortic arch defects, low calcium due to parathyroid hypoplasia (hypocalcemic tetany), and midline developmental abnormalities.
Term
Failure in heavy chain class switching; eyes are far apart.
Definition
Hyper IgM Syndrome
Term
X-linked deficiency in cytokine receptors causing deficiencies in both T-cell and B-cells (B-cells sometimes present but not functional); thymus is embryonic (pathognomonic for this disease).
Definition
Swiss type SCID; three types:

1) SCID with adenosine deaminase deficiency
2) SCID with purine nucleoside phosphorylase deficiency
3) SCID with MHC Class II deficiency

*most patients have deficiencies in enzymes linked to purine metabolism.
Term
The neoplastic disease most often present in AIDS patients is ____________.
Definition
Kaposi's sarcoma (tumor of the vascular epithelium - HSV-8)
Term
HLA-D Class II gene differs from HLA-A, B, and C Class I in the fact that......
Definition
It is lymphocyte defined (instead of serologically defined) and its receptor has no beta-microglobulin.

The serologically defined antigens have antibody-mediated cytotoxicity and agglutination while lymphocyte defined antigens have a mixed lymphocyte reaction.
Term
This type of transplant rejection mainly involves Class I MHC and attacks endothelial cells and forms a fibrous thrombus.
Definition
Hyperacute/accelerated rejection
Term
This type of transplant rejection involves both Class I and Class II MHC; CD8 T-cells attack endothelium causing hemorrhage; controlled by immunotherapy.
Definition
Acute rejection
Term
Graft versus host reactions involve which two types of cells and target which four organs?
Definition
T-cells and NK cells; target:

1) Lung- interstitial pneumonia
2) Skin- loss of pigmentation in blacks
3) GI tract- bloody diarrhea
4) Liver- destruction of bile ducts leading to jaundice

GvH reactions can also result in oral ulcers.
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