SHCN Flashcards
(73 cards)
1
Q
Definition of SHCN
A
Any physical, developmental, mental, sensory, behavioral, cognitive or emotional impairment or limiting condition requiring medical management, health care intervention and/or use of specialized services
2
Q
AAPD Policy on Transitioning Care
A
Specific transitioning planning should begin 14-16 years
Dentistry is most common category of unmet health needs
3
Q
Guideline on management of patients with SCHN
A
Main recommendation is preventive strategies
Education of parents, sealants, fluoride, etc.
4
Q
Lesch-Nyhan Syndrome
A
X-linked trait
Affects purine metabolism = high uric acid
Intellectual disability, speech articulation
Self-mutilative behavior
5
Q
Hurler, Hunter Syndromes
A
Mucopolysaccharidosis type I (Hurler) and II (Hunter)
Build up of glycosaminoglycans
Claw hand, mental decline, heart valve problems, abnormal bones, coarse features
Macrocephaly, large tongue, wide spacing of teeth
6
Q
Sturge-Weber Syndrome
A
Port-wine stain Seizures Bleeding issue with treatment Early eruption of teeth due to increased vascularity Hemorrhage from angiomas Some intellectual disabilities
7
Q
Neurofibromatosis
A
Tumors of nervous system Wide inferior alveolar canal Enlarged fungiform papilla Oral neurofibromas Malpositioned teeth Small cafe au lait spots
8
Q
Rubenstein-Taybi Syndrome
A
Heart defects Broad toes, thumbs Excess hair Intellectual disability Seizures Thin upper lip Talon cusps Crowding High caries
9
Q
Treacher Collins Syndrome
A
Small mandible, short ramus/absence of ramus, glenoid fossa, TMJ Difficult oral intubation Normal intelligence Facial nerve may be affected Malocclusion Tooth agenesis Ectopic eruption of first molars
10
Q
Pierre Robin Sequence
A
Small lower jaw
Cleft palate
Difficulties breathing and feeding
Glossoptosis (associated with airway obstruction)
Not candidates for oral sedation in office
11
Q
Rett Syndrome
A
Disorder of brain and nervous system Normal development for 6-18 months Floppy arms and legs, apraxia Excessive saliva/drooling Loss of social engagement Intellectual disability Seizures Tongue thrusting, mouth breathing, open bite, gingivitis
12
Q
Beckwith-Wiedemann Syndrome
A
Congenital growth disorder causing large body size/large organs
Ridge in forehead (premature closure of sutures)
Macroglossia,
increased risk for certain cancers especially Wilm’s tumor (kidney)
13
Q
Crouzon Syndrome (craniofacial dysostosis)
A
Premature fusion of skull bones Hypertelorism PDA and aortic coarctation Normal intelligence Hypodontia, crowding, high palate, clefts, underbite Maxillary hypoplasia
14
Q
Ellis-Van Creveld (chondroectodermal dysplasia)
A
Affects bone growth Polydactyly Congenital heart defects Cleft lip, palate Microdontia, abnormally shaped teeth Multiple frenula and abnormal attachments Natal teeth Congenitally missing teeth Microdontia
15
Q
Fragile X
A
Elongated face
Large ears
High palate
Higher occurrence of malocclusion
16
Q
Marfan Syndrome
A
Hypermobile joints High arched palate, narrow jaw Crowding of teeth Congenital heart problems Small lower jaw
17
Q
Prader-Willi
A
Obesity is main feature Soft tooth (enamel hypoplasia) Poor oral hygiene Bruxism Thick saliva
18
Q
Tuberous Sclerosis
A
Hypomelanotic macules Fibromas Seizures Enamel hypoplasia/pitted enamel Gingival hyperplasia (secondary to seizure meds)
19
Q
Sotos Syndrome (cerebral gigantism)
A
Facial features Learning disability Overgrowth Hypotonia Premature eruption of teeth Second premolar hypodontia
20
Q
Hemihyperplasia
A
Cases with hemihypertrophy not fulfilling criteria of complicated hemihypertrophies are grouped under isolated hemihypertrophy
Non-progressive
May or may not have intellectual disability
Hemi-mandibular hypertrophy: excessive unilateral growth of mandible
Unknown etiology
21
Q
Oculo-Auriculo-Vertebral (Goldenhar, hemifacial microsomia)
A
1st and 2nd branchial arches affected Condyle, middle ear, facial nerve affected Facial asymmetry Diminished to absent parotid secretion Anomalies in tongue Clefts
22
Q
Fetal Alcohol
A
Smooth philtrum Thin upper lip Micrognathia Hypoplastic mandible Flat midface Minor ear abnormalities Cleft palate
23
Q
Cornelia de Lange
A
Low birth weight, hirsutism, microcephaly
Self-injury, compulsive repetition, autism-like
Thin eyebrows (unibrow)
Long eyelashes
Cleft/high palate
Delayed eruption of teeth
24
Q
Apert Syndrome
A
Craniosynostosis Syndactyly (mitten hands) Midface hypoplasia Tooth agenesis Supernumerary teeth Ectopic eruption of maxillary first molars Cleft palate Enamel hypoplasia
25
Turner Syndrome
```
Absent or incomplete development at puberty (sparse pubic hair and small breasts)
XO
Webbed neck
Premature eruption of permanent molars
Micrognathia
```
26
Noonan Syndrome
```
Congenital heart issues
Delayed puberty
Down slanting/wide eyes
Hearing loss, low set ears
Mild intellectual disability sometimes
Delayed eruption of teeth
Atypical eruption sequence
```
27
Lowe Syndrome (Oculo-Cerebral-Renal)
Periodontal disease with severe bone loss
Defective inositol phosphate metabolism
Taurodontism
28
Kleinfelters Syndrome
```
XXY
Only in males
Some learning problems
Taurodontism
Weak muscles, sparse body hair, enlarged breasts
```
29
Down Syndrome
```
Trisomy 21
Microdontia
Macroglossia
Small chin
Delayed eruption of teeth
Small conical roots
Oval palate
Hypoplasia of midface
Congenital heart disease
```
30
Cri-Du-Chat
Micrognathia, high palate, malocclusion
| Dental erosion provoked by GERD/bruxism
31
Achondroplasia
```
Large head-to-body size
Frontal bossing
Maxillary hypoplasia
Disturbed genesis of teeth
Disturbed eruption of teeth
Tooth shape abnormalities
```
32
ADHD
3-5% of school-age children affected (up to 7-8%)
Risk factors: age, male, chronic health problems, family dysfunction, low SES
Pre-frontal cortex and dopamine implicated
Treatment = stimulants
Prefrontal cortex and dopamine affected
33
Cerebral Palsy
Non-progressive disorder
Paralysis, weakness, motor function aberrations
1.5-3.6 cases/1000 children
No cure but can have near normal lives if neurologic problems are managed
Common = spastic-tightness CP (lack of control of muscle movements)
60% have intellectual disability
Dental: periodontal disease, bruxism, drooling, erosion, malocclusion, caries
No cure but many patients live near normal lives if neurological issues are controlled
34
Maternal Risk Factors for Congenital Heart Conditions
```
Rubella
Diabetes
Alcoholism
Irradiation
Drugs - thalidomide, phenytoin, warfarin
```
35
Incidence of congenital heart conditions
8-10 per 1000 births
36
Non-cyanotic heart conditions
Connection between systemic and pulmonary circulation
Left-to-right shunt
ASD, VSD and PDA most common
Others: coarctation of aorta, aortic stenosis, pulmonary stenosis
37
Cyanotic heart conditions
Right-to-left shunt
Tetralogy of Fallot
Transposition of great vessels
Tricuspid atresia
38
Tetralogy of Fallot
VSD
Pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy
39
AHA Prophylaxis Recommendations for SBE
```
Prosthetic cardiac valve
Previous infective endocarditis
Cardiac transplantation that develop valvulopathy
Congenital Heart Disease
-unrepaired cyanotic CHD
-completely repaired in first 6 months
-repaired with residual defects
```
40
Conditions where SBE prophylaxis is no longer recommended
```
Mitral valve prolapse with regurgitation
Rheumatic heart disease and other acquired valvular heart disease
VSD
ASD
Hypertrophic cardiomyopathy
```
41
Dental procedures which endocarditis prophylaxis is recommended
Procedures involving manipulation of gingival tissues or periapical region of teeth or perforation of oral mucosa
Does NOT include
-routine anesthetic injection through non-infected tissue
-taking radiographs
-placement or removable prosth/ortho appliances
-adjustment of ortho appliances
-shedding of deciduous teeth
-bleeding from trauma to lips or oral mucosa
42
Asthma
Inflammatory restrictive obstructive disease
43
Agents used for asthma
Corticosteroid treatments (fluticasone)
Beta-2 agonists (albuterol) - cause relaxation of lung
Leukotriene receptor inhibitors
Mast cell stabilizers
44
Oral manifestations of asthma
Increased prevalence of caries with moderate/severe asthma (secondary to medications)
Decreased salivary flow from beta-2 agonists
Candidiasis risk with corticosteroids
Optimal asthma control is desirable before treatment
Macrolide antibiotics may elevate theophylline levels (erythromycin)
45
Asthma and aspirin
Aspirin and other NSAIDs should be avoided in aspirin-sensitive persons
10-28% of children with asthma may be intolerant to asthma
46
Types of Diabetes
Type I: insulin deficiency, autoimmune disease
Type II: relative, progressive insulin deficiency, non-autoimmune
Type III: caused by other identifiable etiology (genetic defect)
Type IV: gestational
47
Insulin
Beta-cells of pancreas
Promotes entry of glucose into cells
Increases with eating
48
Glucagon
Produced by alpha-cells of pancreas
| Glucagon decreases with eating, increases at rest
49
Hemoglobin A1c
Measures blood glucose past 2-3 months
Percentage of glucose captured by hemoglobin
Diabetes >7%
-8% or below reflects good control
50
Complications of diabetes
```
Macrovascular disease
-poor healing
-accelerated atherosclerosis
Microvascular disease
-thickened capillary basement membrane
-nephropathy
-retinopathy
Neuropathy
Decreased resistance to infection
Hypoglycemia
```
51
Hypoglycemia
Most likely problem to be encountered in dental office
Low blood sugar from excess insulin, excess exercise, stress, poor diet
Acute, rapidly progressive
Do not give oral carbohydrates if unconscious
52
Hyperglycemia
Deficient insulin
Diabetic ketoacidosis
Chronic, slowly progressive
53
Dental considerations for diabetes
Morning appointments are best (avoid peak insulin)
Prevent hypoglycemia
May need antibiotic prophylaxis
Well-controlled, stable diabetic taking insulin requires little or no modification for routine dental care including surgery
Poorly controlled or uncontrolled - defer elective treatment
54
Renal osteodystrophy
Muscle weakness, bone pain, fractures with minor trauma
Treatment: vitamin D
Results from chronic kidney disease
Can result in poor calcification of bones (maxilla/mandible)
Delays eruption of permanent teeth
55
End Stage Renal Disease
```
Peritoneal dialysis is required
-Continuous or cycles
-daily, usually at night
Hemodialysis
-usually kids 5 and older
```
56
Consequences for patients on dialyssi
```
Bleeding tendencies
Impaired drug excretion
Hypertension
Shunt infections
Hep B or C
Anemia
Renal osteodystrophy
```
57
Dental management of patients on dialysis
Screen for bleeding disorder prior to surgery
Medical consultation indicated
Avoid dental treatment on day of dialysis
May need SBE
58
Oral changes in renal failure
Brown tumor of hyperparathyroidism (scattered, multinucleate giant cells)
Ground glass appearance and loss of lamina dura
Pallor of mucosa
Xerostomia
Metallic taste
Petechiae/ecchymoses
59
Management of kidney transplant patients
Susceptibility to infection is primary concern
May need frequent recalls, daily antibacterial mouth rinses
Avoid NSAIDs
Consider need for supplemental corticosteroids
60
Most frequent documented source of sepsis in immunosuppressed cancer patients
Mouth
61
Prevention for cancer patients
All patients with cancer should have an oral examination before chemotherapy
Chlorhexidine rinses recommended
Dental floss is not contraindicated (but careful)
62
Antibiotic prophylaxis for central lines
Only indicated at time of placement
63
Absolute neutrophil count
Calculated from WBC count (neutrophils are 50% of WBC)
When less than 1000/mm3, defer elective treatment
When greater than 1000/mm3, no need for antibiotic prophylaxis but may be prescribed if infection is present
64
Platelet count
>75000/mm3: no additional support needed (but be ready to treat prolonged bleeding)
40-75000/mm3: platelet transfusions may be considered prior to treatment
<40000/mm3: defer elective treatment; platelet transfusions necessary for emergencies
65
Dental Treatment and Cancer Therapy
Ideally all dental treatment occurs before cancer therapy starts
66
When should RCT be completed before cancer treatment?
1 week before
Pulp therapy in primary teeth is ok, but most pediatric dentists prefer to provide more definitive treatment of extraction
67
When should extractions be completed before cancer treatment?
7-10 days
68
Ortho and cancer therapy
Appliances should be removed if patient has poor oral hygiene and/or treatment protocol of HCT regimen carries risk for mucositis
Patients may start or resume ortho treatment after completion of all therapy and after at least 2 years disease-free survival
69
Phases of hematopoietic cell transplantation
Phase 1: pre-transplantation
-chemotherapy and/or radiation results in prolonged immunosuppression following transplant
-elective dentistry must be postponed until immunological recovery (9-12 months post HCT)
Phase 2: conditioning/neutropenic phase
-no elective treatment
-ANC is low, neutrophils almost 0
Phase 3: initial engraftment to hematopoietic reconstitution
70
Hemophilia A
```
Factor 8
Limited to males mostly
Prolonged bleeding is hallmark
Bleeding into joints is common
X-linked recessive
Long PTT time and normal PT time
```
71
Hemophilia B
Factor 9
| Other similarities with hemophilia A
72
Von-Willibrand
Autosomal dominant
Bleeding, epistaxis, easy bruising
vWf serves as carrier protein for factor 8
Treated with desmopressin or vWf replacement
73
What is the most common inherited bleeding disorder
Von-Willibrand
| Chromosome 12
