Special needs patients part 2

Question 1

Short acting bronchodilators: what are their names? what is their method of action?

Answer 1

Albuterol: beta2 agonist, bronchodilator-relaxes smooth muscle

Ipatroprium (Atroven): anticholinergic, used w/b2 agonists, relaxes smooth muscle by blocking acetylcholine receptors in smooth muscle

Question 2

Long term control medications: what kinds of medicines are they? what are their effects? What are their methods of ingesting and examples? risks?

Answer 2

1. Corticosteroids : anti inflammatory and immunosuppressive
2. Inhaled: fluticason (flovent) and budesamide (pulmicort) or systemic : prednisolone
3. Risks: Low risk in general, suppressed adrenal fxn, decreased bone density, increased risk for cataracts, growth retardation

Question 3

Other classes of medications for asthma treatment

Answer 3

1. Long acting Beta2 agonists: 12 hr activity
   - used when inhaled corticosteroids cannot control asthma alone. Combined with inhaled corticosteroids ex: advair, symbicort
2. Leukotriene receptor antagonist: blocks chain rxn that causes inflammation, tablet form (ex singulair accolate)
3. Anti IgE (xolair) used for severe persistent asthma. Injections 1-2 times/month

Question 4

Oral findings in asthma patients?

Dentofacial findings?

Answer 4

Decreased salivary flow,
increased caries, dental erosion, calculus, gingivits
- Evidence of steroid use
- High palate, increased anterior face heigh, increased overjet, and greater incidence of posterior xbite

Question 5

Dental treatment can trigger asthma…why?

Answer 5

Dental tx can result in 15% decreased lung fxn

• Prolonged supine position
• position of dental instruments
• tooth dust
• sealant materials
• aerosols

Question 6

Caution with sedation for asthma? what’s okay ?

Answer 6

Nitrous oxide OK for mild-moderate, severe asthma it will irritate their airway.
- Caustion with narcotics and barbituates for asthma. Antihistamines like vistaril are possible.

Question 7

Congenital heart disease: incidence? percent of congenital anomalies?

Answer 7

• 1/8000 live birts

- approx 30% of congenital anomalies (pretty common)

Question 8

Heart Murmors: prevalence? normal/abnormal?

Answer 8

Related to increased blood flow velocity across a NORMAL valve

• common, present in 80% of all children
• Vast majority (90%) are normal
• systolic murmurs may be innocent
• diastolic murmurs and continuous murmurs are abnormal

Question 9

Cardiac Defects:

- Increased Pulmonary blood flow– examples of this? clinical presentation? what occurs over time?

Answer 9

L to R shunts: ASD, VSD, PDA, and AV

• increase in pulmonary blood flow at the expense of systemic circulation
• enlarged heart
• Appears as CHF over time

Question 10

Cardiac Defects:
Decreased pulmonary blood flow :
examples of? Clinical presentation? Risks?

Answer 10

R to L shunt: tetralogy of fallot, tricuspid atresia, ASD or VSD + obstructive defect + overarching aorta

• Pt appears cyanotic due to hypoxemia
• Highest risk of endocarditis

Question 11

Tetralogy of Fallot : whats the tetra?

Answer 11

1. Pulmonary stenosis
2. Right ventricular hypertrophy (right ventricle is trying to beat against the narrow pulmonary artery)
3. Overarching aorta
4. VSD

Question 12

Cardiac defects:

Obstructive defects: types? clinical appearance? what occurs over time?

Answer 12

• Anatomic narrowing impededs flow : physical defect ie Coarction of the aorta, aortic stenosis, pulmonic stenosis
• Appear as congestive heart failure over time

Question 13

Cardiac Defects:

Primary pump failure

Answer 13

• Dilated cardiomyopathy: abnormality of myocardium which limites ability of heart to contract. Etio: disease/meds
• Hypertrophic cardiomyopathy: dysrhymias, can result in syncope, sudden death Etio: the heart gets thicker/stronger and eventually impedes/blocks the aorta, most often effects the ventricular septum

Question 14

Cardiac Defects:

- Congestive Heart failure: etiology? manage how/meds?

Answer 14

Heart unable to pump adequate amount of blood to systemic circulation at normal pressure to meet metabolic demands.
- Managed medically, surgically, or combo
–Remove accumulated fluid,
–Improve cardiac fxn
–Improve tissue oxygenation
–Reduce demands on heart
Meds: digoxin (increases contractility of heart) and diuretics (decreases fluids)

Question 15

Cardiac Defects: long QT syndrome

- What is it? Prevalence? What happens? tx?

Answer 15

• Delayed repolarization of the heart following heartbeat
• May lead to palpitations, v-fib, sudden death
• Dx difficult: 2.5% of normal patients have a prolonged Q-T wave use LQTS scoring system to categorize
• Tx: meds, severe cases, placement of implantable cardioverter-defibrillator (ICD)

Question 16

Prevalence of hypertension in children?

Answer 16

2% of children 4-15yrs

Question 17

Benefits of bioprosthetic valvue? vs a mechanical valve?

Answer 17

Bioprosthetic valve: porcine, human, bovine–benefits good hemodynamics and no need for anticoagulants
- Mechanical valves: limited life span (10yrs), doesn’t grow w/the patient, readily available, replacements available, need anticoagulants and limited to the left side of the heart

Question 18

Oral findings for children with cardiac defects?

Answer 18

• Increased caries
• poorer oral health than siblings, increase in untreated caries in cases of severe CHD
• gingival bleeding related to plaque (fear of inciting bleeding by tooth brushing?)

Question 19

Infective Endocarditis : incidence in population/children? What is it? symptom?

Answer 19

5 cases per 100,000 person years (very low incidence in general population and less freq in children)

• Microbial infection : affects valves, muscle, defects
• Symptoms: may appear as other infection (fatigue, fever, rash) 7-14 day typical incubation period

Question 20

Risks of transient bacteremia: most to least?

Answer 20

Dental procedures (done infrequently) : extractions 10-100%; perio sx (40-80%); Sc/rp (10-80%), teeth cleaning (40%) and RD placement (10-30%)

Routine daily procedure (done frequently):
-Toothbrushing/flossing (20-70%); Toothpicks (20-40%); Chewing food (10-50%)

Question 21

Cardiac patients who will receive SBE? Other patients at high risk?

Answer 21

1. Prosthetic heart valve
2. Previous infective endocarditis
3. Congenital heart disease : (unrepaired cyanotic CHD, including palliative shunts/conduits; completely repaired congenital heart defect w/prosthetic material or device needed 6 months after procedure; repaired CHD w/residual defects)
4. Cardiac transplant receipients who develop cadiac valvulopathy
5. Also patients with compromised immunity (HIV, SCID, neutropenia, cancer tx, stem cell/organ transplant)
6. Pts w/shunts and central lines (at time of placement, VA, VC, VV shunts at greatest risk)
7. Pts w/prosthetic joints for high risk procedures w/in 2 years of implant sugery or w/hx of joint infection)

Question 22

Hemostasis Process:

1. Primary : what is this step called? what occurs first? what are the factors involved? blood vessel reaction?
2. Secondary: same ?s as above, and describe in/extrinsic pathway activation/production

Answer 22

1. Primary: platelet aggregation
   - Injury causes platelets to aggregate
   - Release of vWF and collagen fibers from endothelium
   - Platelets adhere to subendothelial matrix-vWF-collagen
   - Vasoconstriction occurs
2. Secondary: coagulation cascade
   - Extrinsic pathway activated when injuy exposes blood to Tissue factor
   - Intrinsic pathway produces factor X
   - Common pathway generates thrombin

Question 23

Using the cell based model of coaguation–describe the 1. initiation and 2. amplification phases of the pathway

Answer 23

1. Initiation:
   - Tissue factor binds to FVII (this activates the extrinsic pathway via factors 9 and 10, eventually leading to thrombin production)
2. Amplification: mainly occurs in platelets
   - Thrombin then activates platelets, factors 5, 8 and 9 to release vWF. Overall the amplification phase ends with 5a and 8a being bound to activated platelets

Question 24

3/3 Propagation phase of the cell based model of coagulation:

Answer 24

3. Propagation: results in prothrombin production
   - activated factors 8 and 9 form a complex on the platelet membrane which leads to activating factor 10
   - 10a binds with 5a&raquo_space;prothrombinase
   - prothrombinase complex converts prothrombin to thrombin

Question 25

How does fibrinolysis occur?

Answer 25

- Plasmin dissolves fibrin clot- | - Plasmin is regulated by anti-plasmin and plasminogen activator inhibitors

Question 26

Anticoagulant medications: name them, what do they do?

Answer 26

Aspirin NSAIDS - Warfarin (coumadin) : inhibits production of vitamin K dependent factors (2, 7, 9 and 10) - Pradaxa: direct thrombin (production) inhibitor

Question 27

``` Screening- what are these testing for? PT? PTT? Platelet count? PFA-100 ? Bleeding time? What is better at detecting vWF disease? ```

Answer 27

- PT: tests for factors 1, 2, 5, 7, 10, thrombin and fibrinogen (extrinsic pathway) - PTT: Tests for factors 8, 9, 10, 11, 5, 1, and 2, thrombin and fibrinogen (intrinsic pathway) - Platelet count: platelet phase - PFA-100 : platelet function - Bleeding time: platelet phase vascular --inaccurate in young children - -PFA-100 is better than bleeding time for vWF

Question 28

Low, intermediate and high risk dental procedures?

Answer 28

1. Low: supragingival restorations or prophylaxis, infiltration anesthesia 2. Intermediate: subgingival restorations, single EXT, endo, nerve blocks 3. High: multiple EXTs, perio sx, gingival curettage

Question 29

Clinical manifestations of a PRIMARY process defect: What is the primary process? Examples of the syndromes? and clinical manifestations

Answer 29

``` Primary process: platelet aggregation - Defects in platelet #/fxn - Von Willebrand Disease Clinical Manifestations: - longer bleeding time, - bleeding from superficial and deep cuts, - - - petechiae, small/multiple ecchymoses, - spontaneous bleeding : epistaxis, CNS, pulmonary ```

Question 30

Clinical manifestations of a SECONDARY process defect: What is the secondary process? Examples of the syndromes? and clinical manifestations

Answer 30

Secondary process: coagulation cascade - Defect in coagulation pathway - hemophilia A and B Clinical manifestations: -- no significant bleeding after superficial cuts, instead, significant bleeding after DEEP cuts, -- -- no petechiae-instead LARGE widespread ecchymoses, -- hematoma, hemarthrosis (bleeding/bruise in joints), -- spontaneous bleeding--musculoskeletal and CNS

Question 31

von Willebrand disease: a defect of which process (1/2ndardy?), what does vWF do? Prevalence? inheritance? when is it detected/how?

Answer 31

Primary process is affected - vWF plasma protein in endothelium - 1% of population is affected, most common inherited coagulation disorder - Defecienct or defective vWF - Often detected after prolonged bleeding episode - low levels of vWF, low levels of factor 8, may have a longer PTT - Prolonged bleeding time, abnormal platelet function test

Question 32

Where is vWF made? What does it do?

Answer 32

- Produced in endothelial cells of vessels - Functions as a bridge between platelets and injury site in vessel, helps in platelet plug formation - Protects factor 8 from quick degredation

Question 33

Management of vWF disease: what are patients likely to respond to it? What is its method of action? how long does it take and how is it administered

Answer 33

1. Desmopressin (DDAVP) - a synthetic peptide for mild hemophilia A or vWD - Causes rapid release of factor 8 and vWF - 30-45 min to take effect - IV or subq; also stimate a nasal spray 2. Amicar

Question 34

Amicar: what is it? how does it work? how long does it take? hows it administered? dosage?

Answer 34

e-aminocaproic acid it is effective in stablizing clots - Anti-fibrinolytic inhibits activation of plasminogen to plasmin - 2 hours to peak effect - give IV or PO : 50 mg/kg q6h until healed (usually 7 days) - may be given alone or w/desmopressin

Question 35

Thrombocytopenia: a defect of which process? due to which causes and why? lab results?

Answer 35

1. Primary process defect - An insufficient # of platelets - May be due to LOW production of platelets : aplastic anemia, cancer in bone marrow - May be due to INCREASE BREAKDOWN of platelets: idiopathic thrombocytopenic purpura (ITP) and drug-induced immune thrombocytopenia Labs show low platelets on CBC - PT/PTT normal - Clinical manifestations: bruising, epistaxis, gingival beleding, petechiae (like other primary process defects)

Question 36

Hemophilia: which process is defective? What are inheritance patterns? break down % of each type of hemophilia, which are relatively more common, what about severity?

Answer 36

X linked recessive inheritance 1/3 of cases are new mutations - Hemophilia A (Factor 8): 85% - Hemophilia B (Factor 9): 15% --Of those w/hemophilia A it is mostly like (70%) that they will have a SEVERE form --Of those w/Hemophilia B 50% will have a severe form and 30% moderate Severe is less than 1% of factor activity; moderate 1-5%

Question 37

Bleeding w/hemophilia: what occurs in the pathways? Clinically how is this manifested: to the body? the mouth?

Answer 37

- normal platelet plug form (primary) - secondary cascade does not do well therefore delayed and malformation of fibrin clot - bleeding is not faster, but is prolonged - Have deep bleeding into joints or muscles - increased bleeding from open wounds - bleeding will appear to stop, then restart. - exfoliating primary teeth can pose a problem (sharp edges, etc)

Question 38

What are two important complication of hemophilia treatment?

Answer 38

1. Antibodies that block activity of clotting factors occurs in 15% of severe hemophilia A patients and 2.5% of hemophilia Bs - -occurs after a number of factor exposures - -Tx: high dose of clotting factor, bypassing agents, efforts to induce immune tolerance 2. Arthropathy: can occur w/o injury, chronic/acute pain, joint degenerates, can exhibit ROM, 3. bleeding in to CNS/airway 4. HIV/hepatitis infections (blood transfusions)

Question 39

Treatment for Hemophilia: medical mgmt? and what about playing sports?

Answer 39

General prophylaxis: use clotting factors 2-3x week - prevent bleeds - goals, decrease join disease/hospitalizations/time lost from school or work * *they can participate in sports! decreases obesity and does not increase risk of joint hemorrhage, puts less stress on joints

Question 40

Mgmt of hemorrhage? what are the goals? The amount/dose needed?

Answer 40

Need to have factor level to achieve and maintain clot - goal 30-40% of normal - 1 unit factor 8/kg = a 2% increase in factor 8 level - 1 unit of factor9/kg = 1% increase in factor 9

Question 41

Thrombotic disorders: acquired examples and inherited (w/incidence?)

Answer 41

1. Acquired thrombotic disorders: short duration, pregnancy, surgery, immobilization 2. Inherited: a) related to impaired function of protein C - anticoagulation system b) Factor V Leiden: incidence 5% in north america, predominant in whites, results in increase in prothrombin

Question 42

Management of thrombotic disorders: acute and long term?

Answer 42

Acute: heparin for several days, followed by warfarin for 3-6 months Long term: Anti-coagulant therapy