Special needs pts (sickles and seizures) part 3

Question 1

Sickle Cell disease: inheritance? what type of defect? prevalence among blood disorders?

Answer 1

1. AR
2. A molecular defect: substitution of valine for glutamic acid at 6th amino acid in beta-globin gene: this allows HbS to polymerize when deoxygenated
   - It is the most common genetic blood disorder

Question 2

Sickle Cell Trait

Answer 2

Not a SC disease
1/12 Afr Americans
- Considered benign, patients are healthy
- Protective from malarial infection: RBC’s infected by P falciparum sickle and are destroyed

Question 3

Sickle Cell anemia: genetics? Prevalence in which populations? how is it dx?

Answer 3

Homozygote HbSS

• Dx by neonatal screening : RBCs exposed to deoxygenating agent, sickling of cells occurs if trait or disease is present, if disease sickling occurs rapidly
• 1/600 African ams; Also hispanic, mediterranean and middle eastern

Question 4

Sickle cell anemia : RBC survival, other issues?

Answer 4

Chronic anemia : Hbg 6-9 g/dL

• Anemia: due to decreased survival of sickled RBCs (normal 120, SC-12 days)
• Delayed growth/puberty
• Susceptible to sepsis
• Bone pain (RBCs trapped in sinusoids)
• Hand/foot syndrome - when small vessels blocked
• Chest syndrome: severe pain, cough/fever dyspnea, sickled RBC block alveoli circulation
• Abdominal pain (liver, spleen, kidney damage)
• Aplastic crisis
• Thrombotic crisis : microvasculature obstruction/stasis

Question 5

SCA : morbidity/mortality?

Answer 5

Pneumococcal infections: early tx w/antibiotics decreases incidence (may be on long term antibiotics)

• CNS infarction
• Acute chest syndrome
• splenic sequestration crisis
• Life expectancy : late 40’s

Question 6

Medical management?

Answer 6

Early dx

• Avoid sickling inducing condition conditions: dehydration, acidosis, cold exposure
• blood transfusions regularly
• hydroxyurea : utilized more in adults

Question 7

Oral findings: soft tissue, bone, teeth, ceph, occlusion

Answer 7

1. pale mucosa,
2. enamel hypoplasia, dental/jaw pain, delayed eruption, pulp calcifications, decreased caries when taking antibiotics
3. Increased incidence of osteomyelitis
4. Lateral skull films: “hair on end” appearnce
   - Mandible-decreased trabeculae, thin inferior border, distinct radiopacities
   - Class 2 : protrusive maxilla

Question 8

Dental managment of patients w/sickle cell anemia

Answer 8

ASA III- want to avoid elective surgery

• No contraindications to local anesthesia w/vasoconstrictor
• Nitrous oxide: if used minimum of 50% oxygen to avoid hypoxia
• acetaminophen for pain

Question 9

Define epilepsy

• Prevalence?

- Cause?

Answer 9

Epilepsy is 3 or more recurrent seizures. It involves spontaneous uncontrollable excessive discharge of cerebral neurons.

• Affects 1% of general population
• w/no identifiable etiology

Question 10

Seizure classification - percent of each?

Answer 10

Partial (40%) can be simple or complex

• Generalized: convulsive or nonconvulsive (40%)
• unclassified
• status epilepticus: seizure lasting >30 minutes

Question 11

Describe Simple partial seizure: where? what happens to patient? Exs of?

Answer 11

• Originate from localized area of brain
• Pt remains conscious
• Motor autonomic, sensory or psychic symptoms
  ie localized muscle twitching, numbness or tingling, chewing/smacking lips, flashes of light, feeling of dissociation from body
• 3rd most common form of seizure (15%)

Question 12

Partial seizures: complex

where? What happens to patient?

Answer 12

Originate from a localized area of brain

• may be preceded by an aura (strange smell, sense of deja vu)
• 1-2 minute loss of consciousness
• Impairment of consciousness may be only symptom
• Motor/autonomic/sensory/psychic symptoms: localized motor activity, paresthesia, overwhelming sense of fear, visual disturbances, distorted perceptions, confusion continues 1-2 mins postictal
• Most common form of seizure (35%)

Question 13

Generalized seizures: classifications? involvement?

Answer 13

Involve the entire brain and loss of consciousness
- Classified by presentation:
absence, myoclonic, tonic-clonic, atonic, cloni tonic

Question 14

Absence seizure:

- type of seizure? what occurs (brain, body, looks like)? how long?

Answer 14

Abence is a generalized seizure

• 10-30 second LOC
• brief eye or muscle fluttering
• sudden stop of activity
• Onsent generally 4-10 per year
• 50% w/this will go on to develop tonic-clonic seizures at puberty
• Often misdx as behavior or learning problem

Question 15

Tonic-Clonic seizures:

- type of seizure? what occurs (brain, body, looks like)? how long?

Answer 15

Tonic clonic is a generalized seizure

• Aura or prodromal mood change-hours to days before seizure
• LOC leads to falling
• Tonic: 10-20 seconds muscle rigidity, 2-5 minutes clonic contractions of muscles of extremities, head, and trunk
• Urinary/fecal incontinence
• Postictal period 10-30 minutes or more, leads to deep sleep, headache, muscle soreness, mental confusion
• Full recovery - 3 hours
• 2nd most common form of seizure (25%)

Question 16

Other types of generalized seizures: atonic, clonic, and tonic- describe

Answer 16

• Atonic: brief loss of muscle tone, may/may not LoC, many injuries from falls
• Clonic: Alternating muscle contraction and relaxations
• Tonic: persistent firm/violent muscle contractions

Question 17

Carbamazepine (Tegretol)

Answer 17

Used to tx: simple partial, complex partial, generalized tonic-clonic

• Common side fx: lethargy, ataxia, vision disturbances
• Side fx: liver dysxn, aplastic anemia, leukopenia
• dental: xerostomia, erythromycin elevates blood levels, drug interactions

Question 18

Ethosuximide

Answer 18

Generalized absence tx

• lethargy, GI distress, hiccoughs
• Can cause rash, leukopenia
• Dental cautions: potential for drug interactions

Question 19

Gabapentin

Answer 19

Simple partial/Complex partial

- Can cause dry mouth

Question 20

Phenytoin (Dilantin)

Answer 20

Generalized tonic-clonic

• Ataxia hirsutism
• Can cause gingival hyperplasia

Question 21

Valproic Acid (Depakene, depakote)

Answer 21

Generalized, all types; simple partial and complex partial

• may cause anemia, thrombocytopenia, pancreatitis
• Gingival bleeding

Question 22

Common side fx and dental effects of seizure meds (all together now! )

Answer 22

Common side fx: lethargy, dizzy, ataxia, potential for drug interactions

• Dental effects:
  1. Xerostomia (Tegretol, nuerotonin)
  2. Gingival bleeding (depakene/depakote)
  3. Gingival hyperplasia (dilantin)

Question 23

Dilantin gingival ovegrowth: pathophysiology? prevalence?

Answer 23

Prevalence: 50% of patients on dliantin
- overgrowth is firboepithelial in nature
- w/inflammation gingiva serves as reservoir for dilantin
- Increase in plaque and inflammation related to increase in dilantin induced gingival overgrowth
(hygiene can help but its not everything)

Question 24

ADHD: Prefontal brain functions what do they allow us to do as it relates to attn?

-incidence of ADHD in school age children? gender?

Answer 24

Prefrontal:

• Maintain attn
• self regulate impulsivity
• Delay gratification
• -Incidence: 3-5% of school age children
• Most commonly dx behavioral disorder in childhood
• More common in boys

Question 25

Dx of ADHD: what is required? What is ADHD considered? what is it influenced by?

Answer 25

- Behaviors or inattention, impulsivity, hyperactivity: 1. Occur in more than one setting (home/school) 2. Be more severe than in other children same age 3. Start before age 7, even if recognized later 4. Continue for >6 months 5. Make it difficult to fxn in various settings * *ADHD is NOT considered a developmental disorder * *ADHD is influenced by child's social environment and school environment as well as child characteristics

Question 26

Possible etiology of ADHD? inheritance? physical 'evidence'? other environmental or history possibilities?

Answer 26

Genetic predisposition likelY: 40% hav ea parent w/ADHD, 35% have a sibling w/ADHD - Genetic evidence of ADHD : increased rate of large copy number variants (chromosomal deletions and duplicates) in individuals w/ADHD - Brain size 3 to 4 % smaller in ADHD - systemic: neurotransmitter deficiencies - also possibly: environmental toxisn, severe head injury, hx of childhood cancer

Question 27

Types of ADHD : Inattentive only (formerly ADD), symptoms? gender? tx?

Answer 27

- Not overly active - withdrawn from peers - symptoms may go unnoticed - late detection - this form is most common in girls w/ADHD - Responds to low dose stimulants

Question 28

Hyperactive/impulsive?

Answer 28

Child can pay attention, child is hyperactive/impulsive

Question 29

Combined inattentive/hyperactive/impulsive: %, what occurs

Answer 29

the MOST COMMON form of ADHD | - conduct problems, aggression, usually detected early, linear response to stimulants

Question 30

What are some important co-existing conditions of ADHD and the % of their occurrence? What behaviors do these patients display?

Answer 30

1. Oppositional defiant disorder/conduct disorder (35%): break rules, lose temper easily, defiant w/authority figures, destroy property, common w/combined and hyperactive/impulsive subtypes 2. Depression (18%): increased risk for suicide in adolescence, inattentive/combined subtypes 3. Anxiety disorders (25%): extreme fears/worry/panic. May display physical symptoms. 4. Learning disabilities

Question 31

Behavioral therapy for ADHD?

Answer 31

1. Positive reinforcement 2. Time out: remove access to activity due to unwanted behavior 3. Response-cost: withdraw rewards due to unwanted behavior 4. Token economy: combines reward and consequence --Additional behavioral strategies: keep childo n a schedule, cut down on overstimulating distractions, provide an organized environment, reward positive behavior, set small attainable goals, limit choices, use calm discipline

Question 32

ADHD Medications and dental side effects: 1. Drugs which interact w/ local anesthetics 2. name drugs which cause xerostomia * *precaution in what situation?

Answer 32

1. Adderall (dextro-amphetamine) interacts w/Meperidine (demerol) - - Amoxetine (strattera) interacts with Levonordefrin 2. Ritalin (concerta), Dextro-ampethamine (adderall), amoxetine (strattera), clonidine and guanfacine (intuniv) all cause xerostomia. aka all ADHD meds * *SEDATION CAUTION: will end up in a deeper state of sedation than intended***

Question 33

Methyphenidate Ritalin (Concerta): Mechanism of axn, dental efects, systemic side effects

Answer 33

1. Non-ampetamine CNS stimulant 2. Xerostomia 3. Tachycardia, anorexia, insomnia - -potentiate tCAs

Question 34

Dextro-amphetamine (adderall): Mechanism of axn, dental efects, systemic side effects

Answer 34

1. Amphetamine CNS stimulant 2. Xerostomia, altered taste, BRUXism 3. Caution w/Meperidine, hypertension, insomnia, anorexia

Question 35

Amoxetine (strattera): Mechanism of axn, dental efects, systemic side effects, **special precautions**

Answer 35

1. Selective norepinephrine reuptake inhibitor 2. Xerostomia 3. Anorexia, fatigue, elevated BP, avoid levonordefrin * *Caution with vasoconstrictors--add to increased BP caused by straterra

Question 36

Clonidine Catapres: Mechanism of axn, dental efects, systemic side effects

Answer 36

1. Antihypertensive 2. Xerostomia, dyspagia, sialadenitis (salivary gland bacterial infection w/hyposalivation) 3. Potentiates CNS depressants, cardiac arrhythmias

Question 37

Guanfacine (Intuniv): Mechanism of axn, dental efects, systemic side effects **Special precautions**

Answer 37

1. Anti-Hypertensive 2. Xerostomia, dysphagia 3. Hypotension, bradycardia, constipation, dizziness, syncope * *hypotension and bradycardia, may slow down even more w/sedation

Question 38

Which ADHD drugs are classified as anti-hypertensives? | - Which ADHD drug has systemic side effects unlike the rest?

Answer 38

Clonidine Catpres Guanfacine (intuniv) - Guanfacine(intuniv) causes hypotension, bradycardia, constipation, dizzyness--generally slowing down while all others are more stimulating tachycardia/nervous/anorexia/high BP