Chapter 16: Allergic and Immune Disorders Flashcards

1
Q

Definition of anaphylaxis

A

Overwhelming, immediate systemic reaction due to IgE-mediated release of mediators from tissue mast cells and peripheral blood basophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Epidemiology of Anaphylaxis

A

Responsible for 500-1000 fatalities yearly

Causes can be food, medication, insect, latex, exercise, or idiopathic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Course of disease of Anaphylaxis

A

Mild reactions may occur with only scattered hives, puritus, and/or nausea
Significant reactions include widespread hives, tongue/lip/throat swelling, wheezing, coughing, stridor, vomiting/diarrhea, anaphylactic shock

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is biphasic reaction of anaphylaxis?

A

Patients have symptoms recurring 2-8 hours up to 72 hours later

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Significance of asthma and anaphylaxis

A

Patients with asthma are at greater risk for severe reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Diagnosis of Anaphylaxis

A

Other conditions may appear similar (vasovagal reaction, flushing episode, anxiety, cardiac events)
Look for a triggering event within 2 hours of onset
If in doubt, treat for anaphylaxis to prevent serious consequences

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Dental Considerations for Anaphylaxis

A

Identify known allergies prior to treatment
Avoid known allergens and any material/drug with cross-reactivity
Prompt recognition of anaphylaxis is critical
Appropriate emergency management drugs should be immediately available

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Allergic Rhinitis Clinical Presentation

A

Seasonal or perennial in nature
Nasal congestion, nonpurulent rhinorrhea, sneezing
Puritis of eyes, nose and palate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Etiology of allergic rhinitis

A

Inflammation of nasal mucous membranes resulting from IgE-mediated allergic reaction to protein/glycoprotein of inhaled aeroallergen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Diagnosis of allergic rhinitis

A

Focused history

Physical exam with correlation of symptoms with positive skin-prick test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Management of allergic rhinitis

A

Avoid inciting allergens
Pharmacotherapy: antihistamines, intranasal corticosteroids, decongestants
Immunotherapy for patients who do note receive adequate relief from pharmacotherapy
For patients with comorbidities (asthma, chronic otitis media, sinusitis) specific treatment targeted to those medical conditions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Complications of allergic rhinitis

A

Acute/chronic sinusitis
Recurrent otitis media with eustachian tube dysfunction and hearing loss
Impaired speech development
Nasal polyps
Sleep apnea
Increased likelihood of developing asthma or aggravation of existing asthma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Dental considerations of allergic rhinitis

A

None with adequate symptom relief
In severe cases, mouth breathing may predispose to alterations of facial growth
Mouth breathing without a diagnosis should alert dentist to allergic rhinitis - refer to allergist
May consider consulting ENT if considering sedation and airway patency is a concern

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Atopic Dermatitis Clinical Presentaiton

A

Chronic dermatitis characterized by puritis and relapsing inflammation
Typical lesions begin acutely with erythema and excoriations triggered by scratching
Uncontrolled itching and rash takes chronic appearance of lichenification and hyperpigmentation without erythema
Affects infants/young children on extremities, cheeks, forehead, neck
Affects older children in flexural areas (knee and elbow)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Etiology of Atopic Dermatitis (atopic eczema)

A

Distinct causal relationships are ill-defined
Sensitization to foods or aeroallergens may contribute
1/3 of cases may be exacerbated by at least one food
Strong familial atopic association exists including those with asthma and allergic rhinitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Diagnosis of atopic dermatitis

A

Made by history and physical examination
90% of cases present before age 5, most resolve by puberty
Diagnostic criteria include puritis, pattern of skin involvement, history of atopic disease, age, elevated serum IgE
Many diseases present with similar lesions as atopic dermatitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Management of atopic dermatitis

A

Education: trigger avoidance
SKin hydration
Itch control (antihistamines), topical steroids for flares
Newer steroid sparing anti-inflammatory therapies used with caution in children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Dental considerations for atopic dermatitis

A

None except for patients on high dose corticosteroids which necessitates consultation with physician

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Clinical presentation of urticaria

A

Extremely puruitic, erythematous, raised lesions affecting the superficial dermal layers that blanch with pressure
Associated with angioedema in 40% of cases
Acute urticaria typically lasts less than 6 weeks and has a trigger, chronic is idiopathic and lasts longer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Clinical presentation of angioedema

A

Swelling is deeper and primarily affects face, extremities, genitalia with occasional tongue enlargement or laryngeal edema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Diagnosis of urticaria and angioedema

A

Depends on focused clinical history, identifying triggers and systems review
Further workup includes blood tests, biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Management of urticaria and angioedema

A

Avoid triggers
Antihistamines
In severe refractory cases, oral steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Dental considerations of urticaria and angioedema

A

Avoid treating patients in active phase

Be aware of any medication triggers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Hereditary Angioedema

A

Etiology: autosomal dominant disorder resulting from deficiency in functional C1 Esterase inhiibtor
Submucous or subcutaneous edema lasting for 2-5 days before spontaneously resolving
Triggered by trauma, medical/dental, emotional stress, menstruation, infections, medication
Non-pitting, tensely swollen, painful, non-erythematous
Most common areas are lips, eyelids, tongue, genitalia, extremities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Management of Hereditary Angioedema - Prophylaxis

A

Prophylaxis

  • daily anabolic attenuated androgens used to be only therapy
  • Purified C1-Esterase Inhibitor is approved for routine prophylaxis of attacks for patients above 16 years
  • Fresh frozen plasma occasionally used before major surgical procedures
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Management of Hereditary Angioedema - Acute Attack

A

Purified C1-Esterase inhibitor for above 16 years
Ecallantide for above 16 years
Rapid administration of anabolic steroids is no longer preferred
Tracheotomy may be potentially lifesaving
Epinephrine and antihistamines are NOT useful

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Dental considerations of hereditary angioedema

A

Routinely well-managed patient is not a contraindication for dental treatment
Some perioral swelling may occur following dental procedures; this should not discourage dentist from seeing these patients

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Food Allergy - clinical presentation

A

Cutaneous reactions include urticaria, angioedema
GI manifestations especially in children
Oral symptoms include tongue, lip and perioral edema and pruritis of palate or lips
Sneezing, rhinorrhea, nasal pruritis, bronchoconstriction are signs of generalized anaphylactic reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Etiology of Food Allergy

A

Aberrant immune response induced by exposure to particular food protein
May be IgE-mediated, cell-mediated, or both
IgE meaited 6-8% of children <3 years
Overall prevalence in general population s 2%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Common food allergens in children

A
Eggs
Peanuts*
Cow milk
Soy
Tree nuts*
Fish*
Shellfish*
Wheat

*indicated allergies that persist into adulthood; others may be grown out of

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Is there a relationship between food allergies and atopic dermatitis?

A

Yes - 35% of children with moderate to severe atopic dermatitis have confirmed food allergies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Diagnosis of food allergies

A

Thorough history
Temporal association
Reproducibility of symptoms on every exposure
Clinical features
Diet history and ingredient labels need careful review
Skin-Prick-Tests or blood test confirm IgE mediated food allergy

True food allergy is distinguished from food intolerance (lactose, gluten, food additives) which are often limited to GI symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Management of food allergies

A

Avoidance is key principle
Read labels carefully for hidden ingredients
Patients should carry epipen at all times

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Dental considerations for food allergies

A

Avoid major food allergens when making dietary recommendations
Children, especially those highly sensitized to peanuts, may react to air-borne food allergens and even to contact from someone who has recently consumed these products

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Latex Allergy Etiology and Pathogenesis

A

Reaction to certain proteins in latex rubber
Amount of latex exposure needed to produce sensitization is unknown
Latex products manufactured from milky fluid derived from rubber tree
True prevalence is unknown, with estimates of general population 5-10% and healthcare workers 0.5-17%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Clinical presentation of latex allergy

A

Irritant contact dermatitis (ICD)
Allergic contact dermatitis (ACD)
Immediate allergic reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Irritant Contact Dermatitis

A

Non-immunological mediated dermatitis characterized by dry, itchy, irritated areas of skin, usually of the hands
Causative factors: maceration and abrasion from glove wearing, repeated hand washing, use of cleaners/sanitizers, exposure to powders in gloves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Allergic Contact Dermatitis

A

Delayed hypersensitivity reaction caused by accelerators, promoters, and antioxidants added to natural rubber latex
T-cell mediated response
Rash, redness, itching 24-28 hours after contact
Rash may progress to oozing skin blisters and may spread to skin untouched by latex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

How to differentiate between ICD (irritant contact dermatitis) and ACD (allergic contact dermatitis)?

A

Allergy patch testing distinguishes Type IV hypersensitivity reaction of ACD from non-allergic reaction of ICD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Immediate allergic reaction

A

Some patients may develop ACD, then urticaria, allergic rhinitis, sneezing, scratchy throat, conjunctivitis, angioedema, wheezing, asthma, and rarely anaphylaxis
Immediate allergic reactions are all IgE-mediated: hallmark symptoms are swelling, redness and itching

41
Q

Diagnosis of latex allergy

A

Thorough clinical history

Skin puncture testing

42
Q

Populations at risk for latex allergy

A

Patients who undergo multiple surgical procedures where extensive or chronic contact of latex occurs

  • Spina bifida (with or without myelomeningocele)
  • Spinal cord trauma
  • Urogenital and GI malformations
  • Neurogenic bladder
  • Hydrocephalus with VP shunts
  • First surgery before one year of age
43
Q

Prevalence of latex allergy in spina bifida

A

18-73%

Complete latex avoidance necessary

44
Q

Other patients at risk for latex allergy

A

Patients with rhinitis, conjunctivitis, urticaria, angioedema with previous latex exposure
Atopic individuals
Occupational exposure
Persons with food allergies (especially bananas, avocados, bananas, figs, kiwis, tomatoes)
-patient with a history of fruit allergy has 11% risk of latex allergy

45
Q

Preventive strategies for latex allergies

A

Use non-latex gloves and dental products
Avoid oil-based hand creams/lotions unless known to reduce latex-related problems
Perform adequate hand hygiene after using latex gloves
Clean filters and ventilation for latex dust
Identify allergic or high-risk patients
Treat in latex-free environment

46
Q

Treatment of an acute allergic reaction to latex

A

Stop exposure to latex
Allergic contact dermatitis: apply high=potency topical corticosteroid
Allergic rhinitis: administer topical intranasal corticosteroids and antihistamine
Acute urticaria: oral H1 receptor antagonist
Asthmatic reaction: inhaler
Anaphylaxis: epipen and other management

47
Q

Dosage for diphenhydramine (Benadryl)

A

1-1.25mg/kg for children under 12

25-50mg for adults

48
Q

Latex products

A
Gloves 
Prophy polishing cups
Rubber dams 
Ortho elastics
Adhesive tape
Local anesthetic carpule
Impression materials
Gutta percha (no alternative for this, avoid over-fill)
49
Q

Definition of Asthma

A

Chronic inflammatory disorder of the airways in which many cells and cellular elements play a role, especially mast cells, eosinophils, T lymphocytes, neutrophils and epithelial cells
Recurrent episodes of wheezing, breathlessness, chest tightness an cough
Inflammation causes increase in existing bronchial hyperresponsiveness to stimuli

50
Q

Epidemiology of Asthma

A

Most common chronic medical condition of childhood
Lifetime prevalence of 12% in children under 18
Most common in African Americans (10.3%) and those below 100% of poverty threshold (11.1%)

51
Q

Cause of Asthma

A

Symptoms caused by airways narrowing secondary to airway muscle constriction
-mucosal edema, airway mucous accumulation, viral infections, inflammatory infiltrate
Triggers for this process include:
-viral infections, allergens, exercise, cold air, GERD, tobacco use, pollutants, sinusitis, stress

52
Q

Course of Asthma Disease

A

50-80% of children will have onset of symptoms before age 5
In children with early onset (<3 years) wheezing there are two patterns
-wheeze with viral infections and resolve by 5-7 years
-persistent wheezing
Children with persistent, early wheezing (>3x/year) are more likely to develop persistent wheezing with concurrent atopic dermatitis or two or more allergic rhinitis

53
Q

Comorbidities of Asthma

A
Allergic rhinitis
Chronic sinusitis
GERD
Food allergy
Atopic dermatitis
54
Q

Consequences of asthma in dental setting

A

Acute asthma attack, secondary to exposure
Adrenal suppression - does NOT develop in children with <700mcg equivalent of inhaled beclomethasone daily, but if present, must supplement for extensive surgical procedures

55
Q

Diagnosis of Asthma

A

Chronic asthma diagnosed by combination of history, physical examination and pulmonary function testing
Primary symptoms of asthma in children may be cough and dyspnea without associated wheeze

56
Q

Acute Asthma Attack Signs

A
Chest tightness
Dry cough
Wheeze on auscultation 
Dyspnea
Anxiety
57
Q

Differential diagnosis for Asthma

A
Diagnosis differential
-Congenital abnormalities of the airway 
-Cystic fibrosis 
-Immunodeficiency diseases 
-GERD
-Foreign body
-Malignancy 
Acute attack differential
-vocal cord/laryngeal dysfunction
-GERD
-mouth breathing
-anxiety attack
58
Q

Medical Treatment of Asthma

A

Controllers - daily to keep asthma in control

Relievers - used to relieve acute episode

59
Q

Controllers of Asthma

A

All patients with chronic asthma should be on controlling medication
First-line is inhaled corticosteroid
Leukotriene antagonists, theophyllines, cromologs, long-acting beta agonists can be used in addition

60
Q

Relievers of Asthma

A

Short-acting beta agonist (albuterol)
Anticholinergic (ipratropium) used occasionally in combination with beta-agonist
All patients with asthma should have a reliever available and bring it to the dental appointment!

61
Q

Management of Acute Asthma Attack - a medical emergency

A
  • Get patient into comfortable position and help to relax
  • Check HR, RR, oxygen saturation, BP (if tolerated)
  • Listen for air movement (if chest is quiet with little air movement and/or patient extremely anxious, call 911)
  • Give child’s rescue medication if available (2 puffs over 2 minutes)
  • If holding chamber and mask are used, child can take 6 deep breathes for each puff
  • If unavailable, give 2.5mg albuterol in 3cc normal saline, nebulized, repeated in 30 min if needed
  • If neither of the above are available, give 0.01mg/kg 1:1000 epinephrine IM
  • Administer oxygen and monitor vital signs
  • tremor and increased heart rate are common side effects of short-acting beta agonists
62
Q

Dental Considerations for Asthma

A
  • child is more likely to have an attack in the dental office if poor daily control
  • safe dental management depends on pulmonary status, level of asthma control
  • important to know if steroid use has occurred requiring supplementation
  • postpone treatment if asthma is poorly controlled
63
Q

Signs of poor asthma control

A

Use of rescue inhaler (albuterol) >2x/week
Nighttime awakening with symptoms > 2x/month
Concurrent upper respiratory illness causing asthma symptoms
Uncontrolled exercise-induced bronchospasm

64
Q

Sedation and Asthma

A

Recommended sedatives: hydroxyzine and benzodiazepines
Avoid barbituates and narcotics - they stimulate histamine release
Nitrous oxide is effective in mild/moderate asthmatics but avoid prolonged periods
Light sedation or general anesthesia may be the best choice
Extreme caution with IV sedation

65
Q

Oral findings of Asthma

A

Possible increased prevalence of dental caries and tooth wear
Oral candidiasis - well documented side effect of steroid inhaler use
Decreased salivary flow rates
Gingivitis from mouth breathing

66
Q

Prevention (dental) for asthma

A

Good oral hygiene
Topical fluoride
Healthy, noncariogenic diet
Avoid acidogenic drink consumption following inhaler use (especially at night)
Drink or rinse with water after inhaler use

67
Q

Anecdotal asthma triggers

A
Dentrifices
Fissure sealants
Tooth enamel dust
methyl methacrylate
Fluoride trays
Cotton rolls
Sulfites
68
Q

Local anesthetics and asthma

A

Local anesthetics with vasoconstrictors are safely used unless there is a suspected or known allergy to sodium metabisulfite
Vasoconstrictors may potentiate effect of beta-agonist inhalers with rare possibility of palpitations, increased blood pressure, and arrhythmias

69
Q

Other considerations of asthma

A

4% allergic to aspirin and other NSAIDS - use acetaminophen in these cases
Patients on theophylline medication (rarely used today) should not receive erythromycine as it raises blood levels of theophylline to toxic range

70
Q

Juvenile Arthritis Types

A

Previously called juvenile rheumatoid arthritis
Systemic onset: 10-15% of cases
-arthritis of >1 joint with fever, and at least one of rash, lymph node enlargement, hepatomegaly, splenomegaly or serositis

Oligoarticular JIA: 50% of cases
-involvement of fewer than 5 joints within 6 months of onset

Polyarthritis: 30-40% of cases
-involvement of more than 4 joints in 6 months of illness

Enthesitis-related
-arthritis plus two ore more of sacroiliac joint tenderness, inflammatory spinal pain, HLA-B27, IBS, spondyloarthropathy

Psoriatic arthritis
-arthritis and psoriasis

71
Q

Prognosis of JIA

A

Prognosis is variable within each group

  • Systemic: resolves in 40-50% of patients, 1/3 have prolonged illness with chronic disease including fever and rash
  • Oligoarticular: most cases benign, major complicaiton is uveitis and leg length discrepancy
  • Polyarthritis: guarded prognosis of early onset, complications include flexion contracture, weakness, TMJ involvement, uveitis
  • Enthesis-related: absence of HLA-B27 is mild, presence of HLA-B27 is increased risk of developing juvenile ankylosing spondylitis, IBS, psoriasis
  • Psoriatic: varies from mild to severe
72
Q

Treatment for JIA

A

NSAIDs or other COX-2 inhibitors first line
Second-line therapies are immunosuppressive (corticosteroids, methotredxate, hydroxychloroquine, gold)
Newer biologic therapies include etanercept or infliximab
Intra-articular corticosteroids used too

73
Q

Dental considerations for JIA

A

Patient may be in chronic pain
Patient may have limited movement - may need pillow
Possible TMJ involvement (small opening, decreased mandibular growth, open bite, ankylosis)
-children may refer to TMJ pain as earache
Multiple medications - careful with drug interaction
Difficulty with oral hygiene (modify toothbrush)

74
Q

Surgical considerations for JIA

A

Consult with rheumatologist for necessary workup
For children on aspirin, delay 10 days and determine PT and PTT if necessary
Patients on oral steroids may require supplemental steroids
Thorough medical evaluation with lab testing should be performed prior to GA including CBC
Immunosuppressives are held prior to some surgical procedures

75
Q

Systemic Lupus Erythematosus (SLE)

A

Chronic inflammatory disorder of unknown cause
Multi-organ systemic involvement
Rare in childhood - 5-10,000 children in US
More common in females older than 5
Prevalence and severity vary: white

76
Q

Clinical Presentation of SLE

A

Variable presentation
Most common presentation in childhood includes fever, malaise, and failure to thrive
Hematologic: anemia, leukopenia, thrombocytopenia
Mucocutaneous: malar rash, oral uclers
Musculoskeletal: arthritis, arthalgia
Nephritis
Abdominal complaints

77
Q

Diagnosis of SLE

A

Based on four or more of following criteria present simultaneously or serially

  • Malar rash: erythema, flat or raised over malar eminences
  • Discoid rash: erythematous raised patches, keratotic scaling, folliculuar plugging
  • Photosensitivity
  • Oral uclers (or nasopharyngeal), painless
  • Arthritis (2 or more peripheral joints)
  • Serositis: pleuritis or pericarditis
  • Renal disorder: proteinuria, cellular casts
  • Neurologic disorder: seizures, psychosis
  • Hematologic disorder: anemia, leukopenia, etc.
  • Immunologic disorder: positive antiphospholipid antibody, anti-DNA antibody
  • Antinuclear antibody
78
Q

Prognosis of SLE

A

With appropriate care, prognosis is good
Poor prognosis related to poor compliance with treatment, neurologic complications, intercurrent infections, renal disease
Survival rate 100% at 5 years, 83% at 10 years

79
Q

Long-term complications of SLE

A

Malignancy secondary to therapeutic regimens
Cardiovascular disease
Organ failure (especially renal)

80
Q

Treatment of SLE

A

Corticosteroids
NSAIDs
Hydroxychloroquine (mild)
Steroid sparing agents like azathioprine, methotrexate (moderate)
Cytotoxic agents to induce remission (severe)

81
Q

Dental considerations of SLE

A

Increased susceptibility to infection
Establish patient’s ability to fight infection
Assess need for SBE
Supplemental steroids to prevent adrenal suppression may be needed
Consider drug interactions
Assess kidney function, beware of drugs metabolized and excreted by kidney
Consider short appointments for patient with musculoskeletal symptoms
Sjogren syndrome is secondary complication

82
Q

Congenital Immunodeficiency

A

Occurs secondary to genetic defects and usually lead to increased susceptibility to infection at birth or early childhood

83
Q

Acquired (secondary) Immunodeficiency

A

Develop later in life usually as consequence of infection (HIV/AIDS), malnutrition, malignancy, or immunosuppressive medications

84
Q

Primary B-Cell Immunodeficiencies

A

50% of all primary immunodeficiencies generally have fewer oral complications than other immunodeficiencies
Types
-Agammaglobulinemia
-Hyper IgM syndromes
-Selected immunoglobilin isotype deficiencies

85
Q

Agammaglobulinemia

A

May be X-linked or autosomal recessive
Normal T cells, low/absent B cells, reduced serum immunoglobulins
Complications include recurrent bacterial infections, recurrent aphthous ulcers, odontogenic infections
Predisposed to septicemia
Managed by IV gammaglobulin

86
Q

Hyper IgM syndromes

A

Patients may have recurrent sinopulmonary infections, hepatitis, lymphoid hyperplasia, autoimmune disease
Oral candidiasis and ulceration are common
Managed with gamma globulin replacement

87
Q

Selected Immunoglobluin isotype deficiencies

A

Defect in B-cell differentiation with reduced/no production of selected isotype
IgA deficiency is most common
Complications range from susceptibility to bacterial infection to no concern
Prophylactic antibiotics may be prescribed

88
Q

Primary T-Cell Immunodeficiency - DiGeorge Syndrome

A

Deletion chromosome 22q11.2 leading to 3rd/4th pharyngeal pouch maldevelopment
Associated with cleft palate, cardiac malformation, dysmorphic facial features
Thymic hypoplasia/agenesis leads to deficient T-cell maturation
Mycobacteria, viral and fungal infections most common
Oral candidiasis, herpes infection common
T-cell function improves with age, often normal by 5 years

89
Q

Combined Immunodeficiencies

A
SCID
Adenosine deaminase deficiencies
Purine nucleoside deficiencies
Ataxia-Telangiectasia syndrome
Wiskott-Aldrich syndrome
90
Q

Severe Combined Immunodeficiency (SCID)

A

X-linked
Decreased T cells and NK cells, normal B cells
Usually begins at infancy
Recurrent, severe bacterial infection, diarrhea, failure to thrive
Oral candidiasis, herpes, recurrent tongue and buccal mucosa ulceration, severe necrotizing ulcerative gingival stomatitis
HLA-identical stem cell transplantation is curative

91
Q

Adenosine Daminase (ADA) or Purine nucleoside phosphorylase (PNP) Deficiencies

A

Absence of enzyme leads to buildup of toxic purine metabolites in lymphocytes
Lymphopenia; progressie decreased in T and B cells
Clinical picture similar to X-linked SCID
Absent tonsils and lymph tissue
HLA-identical stem cell transplantation is curative
PEG-ADA Enzyme replacement weekly

92
Q

Ataxia-Telangiectasia Syndrome

A

Autosomal recessive
Elevated alpha-fetoprotein in baby
Immunoglobulin deficiency: poor production of antibody to bacteria containing polysaccharides in cell wall
Characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, diabetes, malignancies, pulmonary disease
Treated with antibiotics, gamma globulin infusion

93
Q

Wiskott-Aldrich Syndrome

A

X-linked
Features range from thrombocytopenia to effect on all blood cells
Bleeding problems common
Infections common
Eczema
Autoimmune disorders
Preferred treatment with HLA-matched bone marrow transplantation

94
Q

Chronic granulomatous disease

A

Disorder of innate immunity
70% x-linked, 30% autosomal recessive
Decreased phagocytic NADPH activity
Recurrent fungal and bacterial infections
Oral candidiasis, gingivitis, oral ulcers
Treated with subcutaneous injections of interferon gamma and antibiotic/antifungal prophylaxis

95
Q

Leukocyte Adhesion Deficiency Type I (LAD-I)

A

Rare, autosomal recessive condition
Deficiency in migration/chemotaxis of leukocytes and adhesion through endothelial cells
Characterized by delayed separation of umbilical cord and recurrent bacterial infections, impaired wound healing
Oral findings: gingivitis, rapidly progressing periodontitis with premature loss of teeth, slowly healing oral ulceration with scarring
Severe phenotype often fatal; moderate survives to adulthood
Treated with bone marrow or stem cell transplantation (severe) and antibiotic therapy (mild)

96
Q

Cyclic Neutropenia

A

Caused by mutation in neutrophil elastase gene
Childhood form is autosomal dominant
Neutrophil count fluctuate normal to <500 on 21 day cycle
Neutropenia lasts 1 week
Asymptomatic or severe infections of skin/mucous membranes
Localized or generalized early onset periodontitis
Treated with subcutaneous injection of granulocyte-colony stimulating factor to increase neutrophil count

97
Q

Chediak-Higashi Syndrome

A

Defective gene associated with defective transport of bacteria to lysosome
Partial oculocutaneous albinism, neurologic abnormalities, photophobia, nystagmus, recurrent pyogenic infections, malignant lymphomas, neutropenia, anemia, thrombocytopenia
Oral features: gingival inflammation, rapidly progressing early periodontitis with early exfoliation of teeth
Treated with aggressive antibacterial therapy, splenectomy, bone marrow transplant
Evaluate hematologic status prior to any dental procedures

98
Q

Dental Considerations of Child with Immunodeficiency

A

Aggressive prevention and regular health history review critical
Liaison with patient physician
May need CBC, white cell, platelets prior to procedures
Patients may present with premature loss of primary/permanent teeth due to progressive perio disease
Consider prophylactic antibiotics other than penicillin
Consider extraction of pulpally-involved teeth to prevent septicemia
Acyclovir for recurrent herpes
Antifungals (nystatin, amphotericin B)
Chlorhexidine mouthwashes