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ABPD 2021 > SHCN > Flashcards

SHCN Flashcards

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

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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

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3
Q

Guideline on management of patients with SCHN

A

Main recommendation is preventive strategies

Education of parents, sealants, fluoride, etc.

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4
Q

Lesch-Nyhan Syndrome

A

X-linked trait
Affects purine metabolism = high uric acid
Intellectual disability, speech articulation
Self-mutilative behavior

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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

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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
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7
Q

Neurofibromatosis

A 
Tumors of nervous system 
Wide inferior alveolar canal 
Enlarged fungiform papilla
Oral neurofibromas
Malpositioned teeth
Small cafe au lait spots
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8
Q

Rubenstein-Taybi Syndrome

A 
Heart defects
Broad toes, thumbs 
Excess hair
Intellectual disability
Seizures
Thin upper lip
Talon cusps 
Crowding
High caries
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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
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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

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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
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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)

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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
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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
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15
Q

Fragile X

A

Elongated face
Large ears
High palate
Higher occurrence of malocclusion

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16
Q

Marfan Syndrome

A 
Hypermobile joints
High arched palate,  narrow jaw
Crowding of teeth
Congenital heart problems
Small lower jaw
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17
Q

Prader-Willi

A 
Obesity is main feature
Soft tooth (enamel hypoplasia)
Poor oral hygiene
Bruxism
Thick saliva
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18
Q

Tuberous Sclerosis

A 
Hypomelanotic macules
Fibromas
Seizures
Enamel hypoplasia/pitted enamel
Gingival hyperplasia (secondary to seizure meds)
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19
Q

Sotos Syndrome (cerebral gigantism)

A 
Facial features
Learning disability
Overgrowth 
Hypotonia
Premature eruption of teeth 
Second premolar hypodontia
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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

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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
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22
Q

Fetal Alcohol

A 
Smooth philtrum
Thin upper lip
Micrognathia
Hypoplastic mandible
Flat midface
Minor ear abnormalities 
Cleft palate
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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

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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
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25
Q

Turner Syndrome

A 
Absent or incomplete development at puberty (sparse pubic hair and small breasts)
XO 
Webbed neck 
Premature eruption of permanent molars
Micrognathia
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26
Q

Noonan Syndrome

A 
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
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27
Q

Lowe Syndrome (Oculo-Cerebral-Renal)

A

Periodontal disease with severe bone loss
Defective inositol phosphate metabolism
Taurodontism

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28
Q

Kleinfelters Syndrome

A 
XXY
Only in males
Some learning problems 
Taurodontism 
Weak muscles, sparse body hair, enlarged breasts
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29
Q

Down Syndrome

A 
Trisomy 21
Microdontia
Macroglossia
Small chin
Delayed eruption of teeth
Small conical roots 
Oval palate
Hypoplasia of midface 
Congenital heart disease
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30
Q

Cri-Du-Chat

A

Micrognathia, high palate, malocclusion

Dental erosion provoked by GERD/bruxism

31
Q

Achondroplasia

A 
Large head-to-body size 
Frontal bossing
Maxillary hypoplasia
Disturbed genesis of teeth 
Disturbed eruption of teeth
Tooth shape abnormalities
32
Q

ADHD

A

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
Q

Cerebral Palsy

A

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
Q

Maternal Risk Factors for Congenital Heart Conditions

A 
Rubella
Diabetes
Alcoholism
Irradiation 
Drugs - thalidomide, phenytoin, warfarin
35
Q

Incidence of congenital heart conditions

A

8-10 per 1000 births

36
Q

Non-cyanotic heart conditions

A

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
Q

Cyanotic heart conditions

A

Right-to-left shunt
Tetralogy of Fallot
Transposition of great vessels
Tricuspid atresia

38
Q

Tetralogy of Fallot

A

VSD
Pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy

39
Q

AHA Prophylaxis Recommendations for SBE

A 
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
Q

Conditions where SBE prophylaxis is no longer recommended

A 
Mitral valve prolapse with regurgitation
Rheumatic heart disease and other acquired valvular heart disease 
VSD
ASD 
Hypertrophic cardiomyopathy
41
Q

Dental procedures which endocarditis prophylaxis is recommended

A

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
Q

Asthma

A

Inflammatory restrictive obstructive disease

43
Q

Agents used for asthma

A

Corticosteroid treatments (fluticasone)
Beta-2 agonists (albuterol) - cause relaxation of lung
Leukotriene receptor inhibitors
Mast cell stabilizers

44
Q

Oral manifestations of asthma

A

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
Q

Asthma and aspirin

A

Aspirin and other NSAIDs should be avoided in aspirin-sensitive persons
10-28% of children with asthma may be intolerant to asthma

46
Q

Types of Diabetes

A

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
Q

Insulin

A

Beta-cells of pancreas
Promotes entry of glucose into cells
Increases with eating

48
Q

Glucagon

A

Produced by alpha-cells of pancreas

Glucagon decreases with eating, increases at rest

49
Q

Hemoglobin A1c

A

Measures blood glucose past 2-3 months
Percentage of glucose captured by hemoglobin
Diabetes >7%
-8% or below reflects good control

50
Q

Complications of diabetes

A 
Macrovascular disease
-poor healing
-accelerated atherosclerosis
Microvascular disease
-thickened capillary basement membrane
-nephropathy
-retinopathy
Neuropathy
Decreased resistance to infection
Hypoglycemia
51
Q

Hypoglycemia

A

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
Q

Hyperglycemia

A

Deficient insulin
Diabetic ketoacidosis
Chronic, slowly progressive

53
Q

Dental considerations for diabetes

A

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
Q

Renal osteodystrophy

A

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
Q

End Stage Renal Disease

A 
Peritoneal dialysis is required 
-Continuous or cycles
-daily, usually at night
Hemodialysis
-usually kids 5 and older
56
Q

Consequences for patients on dialyssi

A 
Bleeding tendencies
Impaired drug excretion
Hypertension
Shunt infections
Hep B or C
Anemia
Renal osteodystrophy
57
Q

Dental management of patients on dialysis

A

Screen for bleeding disorder prior to surgery
Medical consultation indicated
Avoid dental treatment on day of dialysis
May need SBE

58
Q

Oral changes in renal failure

A

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
Q

Management of kidney transplant patients

A

Susceptibility to infection is primary concern
May need frequent recalls, daily antibacterial mouth rinses
Avoid NSAIDs
Consider need for supplemental corticosteroids

60
Q

Most frequent documented source of sepsis in immunosuppressed cancer patients

A

Mouth

61
Q

Prevention for cancer patients

A

All patients with cancer should have an oral examination before chemotherapy
Chlorhexidine rinses recommended
Dental floss is not contraindicated (but careful)

62
Q

Antibiotic prophylaxis for central lines

A

Only indicated at time of placement

63
Q

Absolute neutrophil count

A

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
Q

Platelet count

A

> 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
Q

Dental Treatment and Cancer Therapy

A

Ideally all dental treatment occurs before cancer therapy starts

66
Q

When should RCT be completed before cancer treatment?

A

1 week before
Pulp therapy in primary teeth is ok, but most pediatric dentists prefer to provide more definitive treatment of extraction

67
Q

When should extractions be completed before cancer treatment?

A

7-10 days

68
Q

Ortho and cancer therapy

A

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
Q

Phases of hematopoietic cell transplantation

A

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
Q

Hemophilia A

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
Q

Hemophilia B

A

Factor 9

Other similarities with hemophilia A

72
Q

Von-Willibrand

A

Autosomal dominant
Bleeding, epistaxis, easy bruising
vWf serves as carrier protein for factor 8
Treated with desmopressin or vWf replacement

73
Q

What is the most common inherited bleeding disorder

A

Von-Willibrand

Chromosome 12

ABPD 2021 (14 decks)

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