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Describe the 4 components of Tetralogy of Fallot.
Very Rude Overbearing Parents
1. VSD
2. RVH
cyanosis, high risk for endocarditis.
A patient needs AP but is currently taking Amoxicillin for acute otitis media, what do you do?
1. If possible, delay dental procedures 10 days to allow normal flora to return.
2. Use another class of antibiotic such as clindamycin (20mg/kg 600mg max) or Azithromycin (15mg/kg 500mg max)

Recall: Amoxicillin dosage 50mg/kg (max 2g)
Vitamin K dependent factors are inhibited by which drugs?
Warfarin (Coumadin) -oral
Heparin - parenteral
PT (prothrombin time) tests the Extrinsic pathway. What are the factors?
thrombin and fibrinogen
PTT (partial prothrombin time) tests the intrinsic pathway. What are the factors?
thrombin and fibrinogen
What are the Vitamin K dependent factors?
Why are barbiturates and narcotics contraindicated in asthmatics?
They stimulate histamine release leading to bronchospasm.
Proventil and Ventolin are examples of which class of drugs? What do they treat?
B2 agonists, bronchodilators that treat asthma.
Rescue inhalers.
LTRA-leukotriene receptor antagonists are used in the long term treatment of asthma. Name an example.
Montelukast (Singulair)
What are 3 common steroids for the long term management of asthma?
1. Flovent (fluticasone)
2. Pulmicort (Budesamide)
3. Q'VAR (beclemethasone)
What is the most common inherited coagulation disorder?
von Willebrand disease

- primary defect; autosomal dominant
- deficient or defective vWF
- low levels of factor VIII
- prolonged bleeding time
- abnormal platelet function test
What is the role of vWF?
1. Protects factor VIII from quick degradation.
2. Helps in platelet plug formation at injury site.
What are drugs used in the management of von Willebrand disease?
1. Desmopressin (DDAVP)
- Nasal spray is known as Stimate.
- also TX hemophilia A
- causes rapid release of factor VIII and vWF, doubling their levels
- 30-45 minute onset

2. Amicar (oral)
- antifibrinolytic, stabilizes clot until healed.
- may be used with DDAVP

3. Oral contraceptives to relieve menorrhagia
What may cause Thrombocytopenia (low platelets)?
1. Decreased production (aplastic anemia, bone marrow cancer)
2. Increased destruction (ITP, drug induced)
Hemophilia A results from a deficiency of which factor?
It is the most common hemophilia.
X-linked recessive
Which pathway could test for hemophilia? Which test would you use?
Intrinsic path; PTT test
Factors V, VIII, IX, X, XI
Hemophilia B is a deficiency in...
Factor IX
X-linked recessive
Management of hemorrhage in hemophiliacs.
1 unit Factor VIII/kg = 2% rise
1 unit factor IX/kg = 1% rise.

DDAVP, amicar, local measures.
Name 5 oral findings of SCA
1. Pale mucosa
2. Enamel Hypoplasia
3. Delayed eruption
4. Step ladder trabeculae
5. Dental/jaw pain from infarcts
6. Pulp calcifications
7. Thin mandibular inferior border (panorex)
Normal healthy
Mild systemic disease
What is the highest ASA classification considered for sedation?
Severe systemic disease
(ex. SCA)
Severe systemic disease that is a constant threat to life.
Moribound patient not expected to survive with or without the operation
Brain dead patient; organ donor
What is the ASA class of an asthmatic and SCA patient respectively?
Seizure management
Position patient to prevent injury
Post-ictal airway support: suction airway, towel under shoulders, oxygen as needed.

Seizure >5 minutes, activate EMS
Ritalin, Concerta, Adderall, Strattera and Clonidine are all common ADHD medications.
Which ones interact with sedation medications?
Adderall: caution with meperidine.

Clonidine: pontentiates CNS depressants.
True or False:
Males are 3-4 times more likely to be affected by Autism.
Females are more likely to have mental retardation when affected.
What are the key differences between Autism and Asperger's?
Autism: social, language and cognitive impairment.

Asperger: social impairment only
Name some common medications for ASD.
Abilify, Prozac, Ritalin, Concerta, Risperdal, Tegretol

Note: Risperdal (anti psychotic, anti aggression) & prozac potentiates CNS depressants. Caution with sedations.
Name an example of a syndrome caused by chromosomal anomaly.
Down syndrome

Trisomy 21-sporadic mutation resulting in extra copy of chromosome.

Risk increases with maternal age.
True/false: those with the mosaic variant of trisomy 21 are higher functioning.
True. Up to 3% may have this variant where not all cells are equally affected.
Describe Celiac disease.
Damage to small intestine caused by gluten.

Decreased absorption of nutrients.

Increased incidence in:
DS, autoimmune disorders, Addison\'s disease, diabetes, thyroid disease.
What are the varying sedation NPO times?
Clear liquids: 2 hrs

Breast milk: 4 hrs

Formula, non-human milk, light meal (toast & clear liquid): 6 hrs

Fried or fatty foods, meat: 8hrs
Describe the characteristic features of Down Syndrome.
Eyes: epicanthal folds, upward slanting papebral features.

Flat nasal bridge, midface hypoplasia, class III malocclusion.

Atlantoaxial instability (caution with shoulder rolls), hypotonia.

Mental deficiency, cardiac anomalies.

Macroglossia, protruding, fissured tongue.

Single palmar crease.

PMN defects leading to higher incidences of perio disease.
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Oblitered, pulp chambers and canals.

Seen in DI type 2:
Autosomal dominant
Primary and permanent
Blue grey-yellow brown
Susceptible to rapid wear, loss of enamel.
What are the vitamin K dependent factors?
Seizures that originate from a localized area of the brain are known as:
Partial Seizures.
May be simple (no LOC) or complex (LOC).
Seizures that involve the entire brain are known as:
Generalized seizures.
May be convulsive or non convulsive.
What percentage of CP patients have developmental delay?
more common with bilaterally affected patients.
Name a common medication used to treat spacticy in CP patients.
Baclofen: muscle relaxant.
Botox: paralytic
TX of DI
Primary dentition: SSC\'s, extractions.

Permanent dentition: bleaching, veneers, crowns, RCT, overdentures.
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Dentin Dysplasia (Sheilds Type 1)
AKA radicular Dentin dysplasia
Short blunted roots/rootless in both dentitions
obliterated pulp Chambers
Severe mobility and malalignment
normal color of crowns

Describe the characteristics of Dentin Dyplasia Type II.
AKA Coronal dentin dysplasia
Primary teeth mainly affected
Coronal and root dentin involved
Amber colored primary teeth 
Permanent teeth look OK but radiographically demonstrate thistle tube shaped pulps.
Cystic Fibrosis (CF) is characterized by 3 main things:
1. Chronic pulmonary disease
2. Pancreatic insufficiency
3. High sweat electolyte concentrations (Na, Cl, K)
Autosmal recessive (AR)
List some symptoms of CF:
- recurrent wheezing, cough

- recurrent pnemonia

- salty skin

- Pancreatic insufficiency→malabsorption of fat and protein→failure to gain weight→GI discomfort

- Clubbed digits
Describe the MOA of CF
Abnormal CFTR protein

Abnormal ion transport in sweat glands (↓ Cl; ↑ Na)

Exocrine glands produce thick, sticky secretions leading to mucous formation.

Lungs, intestine & reproductive epithelium affected.
Sparse hair, dry skin, decreased ability to sweat and conical teeth are all physical characteristics of which X-linked recessive disorder?
Ectodermal Dysplasia

Other Dental/Craniofacial features include:

- midface hypoplasia w/ frontal bossing
- periorbital pigmentation
- hypodontia or anodontia of both dentitions
- flat alveolar ridges
- hypoplastic salivary glands→xerostomia→ ↑caries risk 
- dysphagia, photobia
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