| Term 
 
        | Dental Problems in Special Pt Population |  | Definition 
 
        | 
perio disease (most important)poor OH# of unrestored/extracted teethmalocclusioncaries (same as other populations) |  | 
        |  | 
        
        | Term 
 
        | Why do special patients have difficulty maintaining proper OH? |  | Definition 
 
        | - lack of coordination - lack of understanding |  | 
        |  | 
        
        | Term 
 
        | What are 3 systemic health problems related to poor oral health? |  | Definition 
 
        | 
heart diseasestrokepremature birth |  | 
        |  | 
        
        | Term 
 
        | What are 6 barriers to access for care for special pts? |  | Definition 
 
        | 
finding a dentist (that will treat them)architectural (e.g., lack of wheelchair ramp)transportationalfinancialattitudinalpsychological (fear) |  | 
        |  | 
        
        | Term 
 
        | What are 3 sources of funding for special pt care? |  | Definition 
 
        | 
medicaid (ends at 21)Dinar Grottosprivate funds (church, family, foundations) |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - non-progressive neuromuscular disorder caused by brain damage due to anoxia at or near birth   |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | 
spastic - most common; e.g., hands compressed against chestathetoid - involuntary movementhypotonic - lack of muscle toneataxic - walking dysfunctionrigidmixed (usually see mixed) |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - spastic tongue thrusting - speech problems - mouth breathing   |  | 
        |  | 
        
        | Term 
 
        | Oral/Dental Problems for CP Pts |  | Definition 
 
        | 
poor OHperio diseaseanterior open bite |  | 
        |  | 
        
        | Term 
 
        | Mouth breathing in CP pts can cause __, which can be treated with __ products. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | CP pts may take the medication __, which can cause __. |  | Definition 
 
        | - phenytoin (brand name dilantin) - gingival hyperplasia |  | 
        |  | 
        
        | Term 
 
        | What percentage of children with CP have seizure disorders? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What precautions are taken with a (CP) pt who is prone to seizures? |  | Definition 
 
        | - if pt can tell they're about to have seizure, call 911 - don't put anything in the mouth that isn't ligated - use a bite block |  | 
        |  | 
        
        | Term 
 
        | After a pt has a seizure, what steps should be taken? |  | Definition 
 
        | - discontinue Tx - don't let them go home alone - notify doctor - document details in chart |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - prolonged seizure - life-threatening |  | 
        |  | 
        
        | Term 
 
        | Valproic Acid (Depakene) Use |  | Definition 
 
        | - for CP pts that have seizures |  | 
        |  | 
        
        | Term 
 
        | Valproic Acid (Depakene) Side Effect |  | Definition 
 
        | - thrombocytopenia (increased bleeding)   *consult with physician before surgery   |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - seizure control - anxiety control |  | 
        |  | 
        
        | Term 
 
        | Phenobarbital Side Effects |  | Definition 
 
        | - none major, but possible behavior problems in kids |  | 
        |  | 
        
        | Term 
 
        | What can be done to help manage CP pts in the office? |  | Definition 
 
        | - give muscle relaxants like benzodiazepine (e.g., valium) - nitrous - restraints (e.g., hand-holding, papoose boards) |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - complex developmental disorder that affects communication and social interaction - symptom onset usually before age 3 |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - higher functioning form of autism |  | 
        |  | 
        
        | Term 
 
        | Asperger's Characteristics |  | Definition 
 
        | - dysfunctional social interaction - clumsiness (delayed motor milestones) - behavior repetition - focused area of unusual intelligence |  | 
        |  | 
        
        | Term 
 
        | Autism Spectrum Disorder Characteristics |  | Definition 
 
        | - overly sensitive to physical stimuli - distress from change in routine - repeated body movements - unusual attachment to objects |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - expectation of unpredictable behavior - discuss behavior with caregiver - anxiolytic meds - restraints - general anesthesia |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - not as many trisomy 21 cells - higher functioning |  | 
        |  | 
        
        | Term 
 
        | Trisomy 21 Characteristics |  | Definition 
 
        | 
low-set earsflat nasal bridgeorbital hypertelorismmid-facial hypoplasia (makes jaw look big, like class III)short statureoverweightexaggerated epicampal foldsextra skin on back of neck |  | 
        |  | 
        
        | Term 
 
        | Oral Manifestations of Down's Syndrome |  | Definition 
 
        | 
low carieshigh perio disease - early tooth losscongenital absence of teeth (max laterals most common)delayed eruption of 1˚ teethbruxismtaurodont molars   |  | 
        |  | 
        
        | Term 
 
        | In pts with Trisomy 21, where do the first signs of perio disease appear? |  | Definition 
 
        | - lower anterior permanents |  | 
        |  | 
        
        | Term 
 
        | What are 2 reasons for the increased risk of perio disease in trisomy 21 pts? |  | Definition 
 
        | - immune cell dysfunction - increased risk of leukemia |  | 
        |  | 
        
        | Term 
 
        | How is bruxism handled for CP and Down's pts with primary dentition? |  | Definition 
 
        | - do nothing (stainless crowns are expensive and will wear thru) |  | 
        |  | 
        
        | Term 
 
        | What are the major systemic complications associated with Down's Syndrome? |  | Definition 
 
        | - leukemia - congenital heart defects - intestinal problems - URT infections with high fevers |  | 
        |  | 
        
        | Term 
 
        | Which subset of Down's Syndrome pts require prophylactic antiobiotics? Why? |  | Definition 
 
        | - those with congenital heart defects - they are susceptible to subacute bacterial endocarditis (SBE) |  | 
        |  | 
        
        | Term 
 
        | What is one of the most significant complications for paralytics? What can be done to manage it? |  | Definition 
 
        | - bedsores - have cushions available to minimize shearing forces |  | 
        |  | 
        
        | Term 
 
        | What is the major complication of spina bifida? What causes it? |  | Definition 
 
        | - hydrocephaly - stenosis of aqueduct of sylvius (cannot maintain fluid equilibrium b/t lateral ventricles in brain, leading to expansion of one ventricle) |  | 
        |  | 
        
        | Term 
 
        | Should you provide antibiotic coverage for ventriculo-atrial shunts? For ventriculo-peritoneal shunts? |  | Definition 
 
        | - yes, for procedures that cause bleeding - no (b/c shunt does not go to heart) |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - water soluble proteins from latex used in various dental materials |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - milky sap derived from rubber tree Hevea brasiliensis |  | 
        |  | 
        
        | Term 
 
        | Management of Pts with Latex Allergies |  | Definition 
 
        | 
nitrile gloves, damcover contact areassilk tapeelastomeric threadmolt mouth propmetal based prohpy brushJP drains |  | 
        |  | 
        
        | Term 
 
        | Signs/Symptoms of Allergic Reactions |  | Definition 
 
        | 
rashhivesitchingtearingsneezing, coughingwheezing, difficulty breathingchest pains/tightnessthroat tightnesshypotension |  | 
        |  | 
        
        | Term 
 
        | How do you manage a limited allergic reaction? |  | Definition 
 
        | Step 1: 1 mg/kg diphenhydramine every 4-6 hrs (max dose 300mg/24hrs) OR .01mL/kg 1:1000 SC epinephrine (max dose 0.5mL)   Step 2: 1 mg/kg diphenhydramine every 4-6 hrs for minimum of 48 hrs AND consult physician |  | 
        |  | 
        
        | Term 
 
        | What are the signs/symptoms of a myocardial infarction? |  | Definition 
 
        | - chest pain - pain in left shoulder/arm - pain in lower jaw/neck - pain in upper back - nausea, dizziness, vomiting   |  | 
        |  | 
        
        | Term 
 
        | For how many months after a myocardial infarction is a pt at increased risk for a second episode? |  | Definition 
 
        | - 3 months (so wait at least 3 months before dental treatment) |  | 
        |  | 
        
        | Term 
 
        | How do you manage a pt with chest pain? |  | Definition 
 
        | - if pt has been to cardiologist and is managing disease, OK to treat - if severe, may refer to oral surgeon - make sure pt has nitroglycerin - if angina occurs during Tx, stop and let pt take nitroglycerin, then wait for symptoms to resolve - nitrous OK |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - congestive heart failure - enlarged heart from having to pump harder |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - shortness of breath - swelling in extremities (clubbing of fingers = sign of hypoxia) - tortuous neck veins |  | 
        |  | 
        
        | Term 
 
        | At what BP measurement should Tx be postponed? |  | Definition 
 
        | - a diastolic of over 100 |  | 
        |  | 
        
        | Term 
 
        | Is it OK to use nitrous and oral sedation meds with CHF pts? |  | Definition 
 
        | - yes (maybe even increase oral sedation dosage for day of Tx) |  | 
        |  | 
        
        | Term 
 
        | How should LA administration be altered for CHF pts? |  | Definition 
 
        | - don't use anything over 1:100,000 epi - don't give more than 3 carpules (1.5 for kids) - aspirate |  | 
        |  | 
        
        | Term 
 
        | What are 6 types of congenital heart defects? |  | Definition 
 
        | 
ventricular septal defectatrial septal defectpatent ductus arteriosusaortic stenosiscoarctation of ortatetralogy of fallot |  | 
        |  | 
        
        | Term 
 
        | For which heart conditions is antibiotic prophylaxis required? |  | Definition 
 
        | 
prosthetic valve or part of valveprevious infective endocarditisunrepaired cyanotic CHD, including shuntsrepaired CHD (with prosthetic material) w/in 6 months of repair (until endothelialization)repaired CHD with residual effects (that prevent endothelialization)cardiac transplant pts with valvulopathy |  | 
        |  | 
        
        | Term 
 
        | For which dental procedures do pts with heart disorders require prophylactic antibiotics? |  | Definition 
 
        | 
all that involve manipulation of gingiva or periapical regionall that involve perforation of oral mucosaE.g., S/RP, oral surgery, prophy (for pts with inflamed gingiva) |  | 
        |  | 
        
        | Term 
 
        | Which dental procedures do not require antibiotic prophlyaxis? |  | Definition 
 
        | 
routine LA (even thru infection)x-raysplacement of removable/ortho appliances (including brackets)adjustment of ortho appliancesshedding of deciduous teethbleeding from trauma to oral mucosa |  | 
        |  | 
        
        | Term 
 
        | When should antibiotic coverage be given? |  | Definition 
 
        | 30-60 min before procedure |  | 
        |  | 
        
        | Term 
 
        | What are the dosages for prophylactic antiobiotics? |  | Definition 
 
        | Amoxicillin - 2g for adults, 50mg/kg for kids Clindamycin - 600mg for adults, 20mg/kg for kids |  | 
        |  | 
        
        | Term 
 
        | What is more important than prophylactic antibiotics for prevention of IE? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | T/F: IE is more likely to result from daily activities like brushing, flossing, and using a toothpick than from dental procedures. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | Is antibiotic prophylaxis indicated for pts with nonvalvular indwelling devices? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | Which type of shunts require antibiotic coverage? Which do not? |  | Definition 
 
        | coverage - ventriculoatrial and ventriculovenous no coverage - ventriculoperitoneal |  | 
        |  | 
        
        | Term 
 
        | Is antibiotic prophylaxis indicated for pts with prosthetic joints? |  | Definition 
 
        | - no (but they may be considered during the first 2 years after implantation) |  | 
        |  | 
        
        | Term 
 
        | What if prophylaxis is not administered before the procedure? |  | Definition 
 
        | - can take up to 2 hours afterwards |  | 
        |  | 
        
        | Term 
 
        | What if a pt that needs prophylactic antibiotics is already on long-term antibiotic treatment? |  | Definition 
 
        | - use a different kind for prophylaxis |  | 
        |  | 
        
        | Term 
 
        | Is antibioitic coverage indicated for CABG or coronary artery stent pts? |  | Definition 
 
        | - no (outside heart, so blood flow less turbulent, so bacteria less likely to colonize) |  | 
        |  | 
        
        | Term 
 
        | Why should pts that need antibiotic coverage be scheduled as the first pt in the morning or afternoon? |  | Definition 
 
        | - so that you won't get behind and miss their window of coverage |  | 
        |  | 
        
        | Term 
 
        | How much time between dental appts are necessary for pts with previous Hx of IE? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | In addition to antibiotic coverage, what topical med can be administered to reduce risk of IE? |  | Definition 
 
        | - 0.12% chlorhexidine rinse (peridex) |  | 
        |  | 
        
        | Term 
 
        | What are fasting blood sugar levels for normal pts, pre-diabetics, and diabetics? |  | Definition 
 
        | normal = < 100mg/dL pre-diabetic = 100-125mg/dL diabetic = > 125 mg/dL |  | 
        |  | 
        
        | Term 
 
        | Besides high blood sugar, what are the signs/symptoms of diabetes? |  | Definition 
 
        | 
increased thirstincreased hunger w/o weight gainfrequent urinationweight loss blurred vision |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - measures amt of glycated hemoglobin (amt of sugar sticking to RBCs) - tells how well diabetic pt is doing over time (2-3 months) |  | 
        |  | 
        
        | Term 
 
        | What is the incidence of type I diabetes? |  | Definition 
 
        | - 1 in 500 (from 5-18 yrs) |  | 
        |  | 
        
        | Term 
 
        | Causes of Type I Diabetes? |  | Definition 
 
        | - viral or toxic damage to pancreatic islet cells in kids who are genetically predisposed - autoimmune damage to ß cells |  | 
        |  | 
        
        | Term 
 
        | What are the medical complications of diabetes? |  | Definition 
 
        | - kidney failure - MI - blindness |  | 
        |  | 
        
        | Term 
 
        | What blood sugar level is indicative of hypoglycemia? |  | Definition 
 | 
        |  | 
        
        | Term 
 | Definition 
 
        | - incoherence - bizarre behavior - dizziness - seizure |  | 
        |  | 
        
        | Term 
 
        | How do you manage a pt with diabetes? |  | Definition 
 
        | - make sure they've eaten - have source of glucose on hand - stress OH (b/c increased risk of perio disease) - morning appts (when blood sugar best controlled) - consult physician before invasive treatment   |  | 
        |  | 
        
        | Term 
 
        | Dental Complications of Diabetes |  | Definition 
 
        | - perio disease - xerostomia - delayed healing |  | 
        |  | 
        
        | Term 
 
        | Is antibiotic coverage needed for diabetics? |  | Definition 
 
        | - only if poorly controlled for oral surgery OR if infection present |  | 
        |  | 
        
        | Term 
 
        | For HIV and chemo pts, at what PMN count should treatement be postponed/abandoned? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What are 3 major causes of immunosuppression? |  | Definition 
 
        | - HIV - chemo - bone marrow transplants |  | 
        |  | 
        
        | Term 
 
        | Signs/Symptoms of Immunosuppression |  | Definition 
 
        | - fever - sore throat - muscle/joint pain - swollen glands |  | 
        |  | 
        
        | Term 
 
        | Oral Manifestations of AIDS in Kids |  | Definition 
 
        | - candidiasis - atypical (fiery red) gingivitis - parotid swelling |  | 
        |  | 
        
        | Term 
 
        | Oral Manifestations of AIDS in Adults |  | Definition 
 
        | - Karposi's sarcoma - herpes - hairy leukoplakia |  | 
        |  | 
        
        | Term 
 
        | What cellular entity is the key to defense against infection? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | At what platelet count does excessive bleeding occur? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What can be used to treat mucositis in immunocompromised pts? |  | Definition 
 
        | - magic mouthwash (careful w/ kids b/c of lidocaine - toxic) - tylenol - chlorhexidine - lasers |  | 
        |  | 
        
        | Term 
 
        | What is the most common bleeding disorder? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What are the activity levels of blood factors that cause: severe hemophilia and moderate hemophilia? |  | Definition 
 
        | severe - less than 1% moderate - 2-5% |  | 
        |  | 
        
        | Term 
 
        | At what level of blood factor activity is it safe to undergo surgery? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What clotting factor deficiency is responsible for type A hemophilia? What percentage of hemophiliacs have this type? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | A deficiency in which clotting factor is responsible for type B hemophilia? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | T/F: Type A and B hemophilias are X-linked recessive disorders, which means that female carriers never show symptoms. |  | Definition 
 
        | F - it is X-linked recessive, but female carriers may still show symptoms |  | 
        |  | 
        
        | Term 
 
        | Management of Hemophiliacs |  | Definition 
 
        | 
if pt has had replacement therapy, OK to treatdo as much as possible in 1 visit to limit need for replacement therapynormal replacement therapy contraindicated for hemophiliacs w/ inhibitorstell hemotologist before procedure (especially if giving an IAN)use 27 gauge to facilitate aspirationif aspirate positive, then move needle and continue injection, but keep pt for a few hrs after procudureprotect soft tissue (careful with film)never prescribe aspirin-containing meds   |  | 
        |  | 
        
        | Term 
 
        | T/F: Hemophiliacs bleed faster than other patients. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What is the only suitable test for hemophilia? Why? |  | Definition 
 
        | - partial thromboplastin time (PTT) - prothrombin time, bleeding time, platelet function and count are w/in normal limits, but PTT is prolonged in mod-severe cases (may be normal in mild cases)   |  | 
        |  | 
        
        | Term 
 
        | What percentage of type A hemophiliacs have no family Hx of disease? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | In type A hemophila, factor VIII levels are __, but factor VIII coagulation activity is __. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | Type B hemophilia makes up what percentage of hemophiliacs? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | Hemophilia C, a factor __ deficiency, is a(n) __ recessive disorder. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | Clinical Manifestations of Hemophilia |  | Definition 
 
        | - joint and muscle bleeding (most common) - post-op bleeding - easy bruising - social, psychological, vocational problems - lack of excessive hemorrhage from cuts b/c platelets are normal - mucosal bleeding infrequent |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | 
fresh frozen plasmacryoprecipitaterecombitant DNAamicar (slows clot breakdown)concentrates (most common) - take blood from donors and concentrate into powder, pts administer themselves |  | 
        |  | 
        
        | Term 
 
        | What are factor VIII inhibitors? |  | Definition 
 
        | - antibodies to factor VIII (seen in 10-15% of hemophiliacs) |  | 
        |  | 
        
        | Term 
 
        | What is done to bypass factor VIII inhibitors? |  | Definition 
 
        | - give such a high dose of factor VIII that body can't make enough antibodies - give factor IX, hoping that body will make antibodies to it instead |  | 
        |  | 
        
        | Term 
 
        | What are some local measures taken to reduce bleeding in hemophiliacs? |  | Definition 
 
        | - adrenaline - topical thrombin - absorbable collagen - pressure   |  | 
        |  | 
        
        | Term 
 
        | What 2 blood components are affected in Von Willebrand disease? |  | Definition 
 
        | - factor VIII (deficiency) - platelets (adhesion disorder) |  | 
        |  | 
        
        | Term 
 
        | T/F: Von Willebrand disease is X-linked recessive. |  | Definition 
 
        | F - it is autosomal dominant |  | 
        |  | 
        
        | Term 
 
        | T/F: Platelet count in Von Willebrand disease is normal. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | Signs of Von Willebrand Disease |  | Definition 
 
        | - severe, frequent nosebleeds - bruising easily - prolonged menstruation |  | 
        |  | 
        
        | Term 
 
        | What 2 complications are especially prevalent in chemo patients? |  | Definition 
 
        | - bleeding (decreased platelets) - infection (decreased lymphocytes) |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - when blood leaves brain |  | 
        |  | 
        
        | Term 
 
        | What cardio symptoms are associated with syncope? |  | Definition 
 
        | - bradycardia - hypotension |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - if not sure what to do, call 911 - trendelenburg - ammonium carpules - rag on face or back of neck - refer pt to physician to make sure not due to underlying condition |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - when ANS pathways disrupted (e.g., by spinal cord injury) - afferents sense that bladder is full, but cannot relay message to relax sphincter and empty bladder - BP increases until bladder empty, so will increase indefinitely unless catheterized - make sure bladder bag not full before Tx |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - get consult from physician for sedation (especially w/ respiratory depressors like hydrocodone) - get chest x-ray to determine lung capacity - ask pt how they can best be seated |  | 
        |  | 
        
        | Term 
 
        | Duchenne Muscular Dystrophy |  | Definition 
 
        | - x-linked recessive defect in dystrophin gene (for muscular protein) - causes progressive muscular degeneration - poor prognosis |  | 
        |  | 
        
        | Term 
 
        | What are the 2 main causes of death in Duchenne muscular dystrophy? |  | Definition 
 
        | - inability to breathe (due to muscle degeneration) - CHF (heart tries to pump hard to compensate for respiratory difficulties) |  | 
        |  | 
        
        | Term 
 
        | Symptoms of Duchenne Muscular Dystrophy |  | Definition 
 
        | - delayed muscle development - drooling - ptosis - ataxia / frequent falls - difficulty breathing |  | 
        |  | 
        
        | Term 
 
        | Duchenne muscular dystrophy pts are prone to what kind of condition affecting body temp? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | How is malignant hyperthermia managed? |  | Definition 
 
        | - dantrolene Na - manages fulminant hypermetabolism of skeletal muscle |  | 
        |  | 
        
        | Term 
 
        | Characteristics of Hypohydrotic Ectodermal Dysplasia |  | Definition 
 
        | - hypodontia - hypotrichosis - hypohydrosis (can lead to malignant hypothermia) - angular chelitis - cone-shaped incisors - saddle-shaped nose - face like inverted triangle |  | 
        |  | 
        
        | Term 
 
        | What type of genetic disorder is hypohydrotic ectodermal dysplasia? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What component of skin is missing in epidermolysis bullosa? |  | Definition 
 
        | - desmosomes b/t dermis and epidermis |  | 
        |  | 
        
        | Term 
 
        | T/F: Epidermolysis bullosa may diminish with age. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What are the 4 types of epidermolysis bullosa? |  | Definition 
 
        | - autosomal dominant simplex (no scarring) - autosomal dominant dystrophic (scarring) - autosomal recessive dystrophic (scarring) - autosomal recessive lethalis (early death) |  | 
        |  | 
        
        | Term 
 
        | What medication can be used to diminish sores from epidermolysis bullosa? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What is the dental prognosis of hypohydrotic ectodermal dysplasia? |  | Definition 
 
        | - very good - dentures when young (adjusted as they grow) - implants when older |  | 
        |  | 
        
        | Term 
 
        | What are the dental ramifications of epidermolysis bullosa? |  | Definition 
 
        | - enamel hypoplasia (increased decay) - taurodont molars |  | 
        |  | 
        
        | Term 
 
        | How do you manage a pt with epidermolysis bullosa during a procedure? |  | Definition 
 
        | - everything that touches skin must have (corticosteroid) cream on it to reduce friction |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - autoimmune reaction to antibiotics (e.g., sulfur, penicillin) - can have erythema multiforme (target lesions) - can have permanent systemic sequelae - can have uneruption of permanent teeth |  | 
        |  | 
        
        | Term 
 
        | What type of genetic disorder is neurofibromatosis? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | T/F: Neurofibromatosis has one of the lowest mutation rates of any genetic disorder. |  | Definition 
 
        | F - it has one of the highest (can pop up w/ no family Hx) |  | 
        |  | 
        
        | Term 
 
        | Osteogenesis Imperfecta Characteristics |  | Definition 
 
        | - fragile bones (fractures) - blue sclera - dentinogenesis imperfecta - deafness - joint laxility - skin fragility - hernias - cardiac valvular insufficiency |  | 
        |  | 
        
        | Term 
 
        | Neurofibromatosis Type I Dx Criteria |  | Definition 
 
        | 
6+ cafe au lait spots2+ neurofibromas of any kind OR 1 plexiform neurofibromafreckling of axilla or groin2+ lisch nodules (benign iris hamartomas)hearing loss |  | 
        |  | 
        
        | Term 
 | Definition 
 
        | - forms in deep tissue - has fingerlike projections that cannot be removed |  | 
        |  | 
        
        | Term 
 
        | T/F: Neurofibromatosis can be highly disfiguring. |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | What are 2 diseases that involve taurodont molars? |  | Definition 
 
        | - Down's syndrome - epidermolysis bullosa |  | 
        |  | 
        
        | Term 
 
        | What is meant by the multifactorial etiology of cleft lip/palate? |  | Definition 
 
        | - it involves multiple (poly)genes and environmental factors |  | 
        |  | 
        
        | Term 
 
        | What is the most infrequent presentation of cleft deformities: cleft lip alone, cleft palate alone, or both together? |  | Definition 
 | 
        |  | 
        
        | Term 
 
        | T/F: Isolated cleft palate occurs more frequently in males. |  | Definition 
 
        | F - more frequently in females |  | 
        |  | 
        
        | Term 
 
        | T/F: Isolated cleft palate is purely an autosomal dominant disease. |  | Definition 
 
        | F - can be autosomal dominant, autosomal recessive, or x-linked |  | 
        |  | 
        
        | Term 
 
        | What hereditary conditions are characterized by supernumerary teeth? |  | Definition 
 
        | - cleidocranial dysplasia - gardner syndrome - cleft lip/palate |  | 
        |  | 
        
        | Term 
 
        | What hereditary conditions are characterized by enamel hypoplasia? |  | Definition 
 
        | 
ectodermal dysplasiasepidermolysis bullosamucopolysaccharidosistuberous sclerosiscleft lip/palate |  | 
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