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Pediatric ENT, Allergy, Immunology Flashcards

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1
Q

Acute epiglottitis may present with a muffled (“hot potato”) voice and inspiratory […].

A

Acute epiglottitis may present with a muffled (“hot potato”) voice and inspiratory stridor.

the presence of drooling helps differentiate epiglottitis from laryngotracheobronchitis (croup)

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2
Q

Acute otitis media often follows an […] infection.

A

Acute otitis media often follows an upper respiratory infection.

nasal congestion contributes to Eustachian tube inflammation and fluid accumulation in the middle ear space; other predisposing factors include cigarette smoke exposure, day care attendance, and formula feeds

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3
Q

Acute epiglottitis often presents with acute-onset fever, […], and dysphagia with respiratory distress.

A

Acute epiglottitis often presents with acute-onset fever, drooling, and dysphagia with respiratory distress.

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4
Q

Acute […] often presents with acute-onset fever, drooling, and dysphagia with respiratory distress.

A

Acute epiglottitis often presents with acute-onset fever, drooling, and dysphagia with respiratory distress.

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5
Q

C1 esterase inhibitor deficiency results in elevated levels of the edema-producing factors C2b and […].

A

C1 esterase inhibitor deficiency results in elevated levels of the edema-producing factors C2b and bradykinin.

results in hereditary angioedema

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6
Q

Can the rotavirus vaccine be administered to patients with a history of intussusception?

A

No (contraindicated)

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7
Q

Chronic granulomatous disease (CGD) is due to a defect in […] enzyme.

A

Chronic granulomatous disease (CGD) is due to a defect in NADPH oxidase enzyme.

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8
Q

Conditions causing […] deficiency predispose patients to chronic giardiasis.

A

Conditions causing IgA deficiency predispose patients to chronic giardiasis.

e.g. selective IgA deficiency, Bruton’s, CVID

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9
Q

Contraindications to the DTaP vaccine include history of anaphylaxis to vaccine ingredients, unstable neurologic disorders, and […] within a week of previous vaccine administration.

A

Contraindications to the DTaP vaccine include history of anaphylaxis to vaccine ingredients, unstable neurologic disorders, and encephalopathy within a week of previous vaccine administration.

patients with unstable neurologic disease or encephalopathy following vaccination should receive diphtheria and tetanus toxoids without pertussis (Td)

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10
Q

Contraindications to the DTaP vaccine include history of […] to vaccine ingredients, unstable neurologic disorders, and encephalopathy within a week of previous vaccine administration.

A

Contraindications to the DTaP vaccine include history of anaphylaxis to vaccine ingredients, unstable neurologic disorders, and encephalopathy within a week of previous vaccine administration

patients with unstable neurologic disease or encephalopathy following vaccination should receive diphtheria and tetanus toxoids without pertussis (Td)

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11
Q

In preterm infants, all vaccines should be administered according to […] age. (chronologic or gestational)

A

In preterm infants, all vaccines should be administered according to chronologic age. (chronologic or gestational)

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12
Q

Leukocyte adhesion deficiency is characterized by marked leukocytosis with […] predominance.

A

Leukocyte adhesion deficiency is characterized by marked leukocytosis with neutrophil predominance.

due to impaired adhesion of the marginated pool of leukocytes

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13
Q

Myringotomy with tympanostomy tube placement should be considered in children with > […] episodes of acute otitis media in 6 months or > […] episodes in 12 months.

A

Myringotomy with tympanostomy tube placement should be considered in children with > 3 episodes of acute otitis media in 6 months or > 4 episodes in 12 months.

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14
Q

Otitis externa is most commonly caused by infection with […].

A

Otitis externa is most commonly caused by infection with Pseudomonas aeruginosa.

Staphylococcus aureus is another common cause

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15
Q

Otitis media with effusion can be distinguished from acute otitis media by the lack of […] signs.

A

Otitis media with effusion can be distinguished from acute otitis media by the lack of acute inflammatory signs.

e.g. absence of fever and no bulging of the tympanic membrane

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16
Q

Patients with acute epiglottitis may […] the neck and maintain a tripod position, which is a sign of respiratory distress.

A

Patients with acute epiglottitis may hyperextend the neck and maintain a tripod position, which is a sign of respiratory distress.

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17
Q

SCID is characterized by absent […] cells and dysfunctional […] cells.

A

SCID is characterized by absent T cells and dysfunctional B cells.

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18
Q

The first hepatitis B vaccine should be administered when the newborn weighs > […] kg.

A

The first hepatitis B vaccine should be administered when the newborn weighs > 2 kg.

2 kg = 4 lb 6 oz; this is the only exception to the rule that preterm infants should receive vaccines according to chronologic age

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19
Q

What are the most common causative organisms (3) associated with acute otitis media?

A

Streptococcus pneumoniae, nontypeable Haemophilus influenzae, and Moraxella catarrhalis.

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20
Q

What imaging test/study may be used to confirm the diagnosis of laryngomalacia?

A

Direct laryngoscopy

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21
Q

What is the definitive treatment for severe combined immunodeficiency (SCID)?

A

Stem cell transplant

no concern for rejection; other options include antimicrobrial prophylaxis, IVIG, and sterile isolation

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22
Q

What is the first-line treatment for acute otitis media?

A

Oral amoxicillin

if infection reoccurs within one month, amoxicillin-clavulanic acid should be given to cover beta-lactamase resistant strains; children with mild symptoms (e.g. mild pain, low-grade fever, unilateral disease) may be treated with supportive care and observation

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23
Q

What is the likely diagnosis in a 8-month-old infant with low serum IgG and normal serum IgM/IgA?

A

Transient hypogammaglobulinemia of infancy

due to decreased maternally derived IgG by 3 - 6 months; usually resolves by 12 months of age

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24
Q

What is the likely diagnosis in a child that presents with ear pain with a bulging, erythematous tympanic membrane on otoscopic exam?

A

Acute otitis media

tympanic membrane bulging is the most specific sign of AOM; young children are predisposed to middle ear infections due to narrower and straighter Eustachian tubes

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25
What is the likely diagnosis in a child that presents with ear pain, erythema, and discharge after swimming in an outdoor pool?
Otitis externa ("swimmers ear") loss of cerumen due to swimming or excessive ear cleaning can increase the risk
26
What is the likely diagnosis in a child that presents with persistent ear discharge, despite antibiotic treatment, with granulation tissue and skin debris visible on otoscopic exam?
Cholesteatoma may be complicated by hearing loss, cranial nerve palsies, vertigo, and/or infection
27
What is the likely diagnosis in a child that presents with worsening purulent nasal discharge and facial pain one week after a viral URI?
Acute bacterial rhinosinusitis viral URI is the most common predisposing factor
28
What is the likely diagnosis in an infant with chronic inspiratory stridor that worsens in the supine position?
Laryngomalacia stridor typically peaks at age 4 - 8 months; occurs due to "floppy" supraglottic structures that collapse during inspiration
29
What is the most common cause of acute epiglottitis in unvaccinated children?
H. influenzae type B incidence has decreased with vaccination; bacteria that are now typically involved include Streptococcus pneumoniae, Streptococcus pyogenes, or Staphylococcus aureus
30
What is the most common primary immunodeficiency?
Selective IgA deficiency
31
What is the recommended management for infants with laryngomalacia?
Reassurance typically resolves by 18 months of age; rarely requires surgery
32
What is the recommended management for patients with chronic variable immunodeficiency (CVID)?
IV immunoglobulins
33
What is the recommended pharmacotherapy for otitis externa?
Topical antibiotics (e.g. fluoroquinolones) the antibiotic must cover for Pseudomonas
34
What is the recommended treatment for acute bacterial rhinosinusitis?
Oral amoxicillin-clavulanic acid
35
Which complement deficiency results in increased risk for recurrent Neisseria infection?
C5-C9 (MAC) deficiency
36
Which immunodeficiency is associated with albinism?
Chediak-Higashi syndrome melanocytes unable to distribute melanin to nearby keratinocytes
37
Which immunodeficiency is characterized by absent B cells in peripheral blood?
X-linked (Bruton) agammaglobulinemia due to disordered B-cell maturation; pre- and pro-B cells cannot mature
38
Which immunodeficiency is characterized by absent T-cell receptor excision circles (TRECs) in dried blood?
Severe combined immunodeficiency (SCID) TRECs are circular DNA excreted by developing T cells in the thymus; this test is part of routine newborn screening
39
Which immunodeficiency is characterized by giant granules in leukocytes and platelets?
Chediak-Higashi syndrome due to fusion of granules arising from the Golgi apparatus
40
Which immunodeficiency is characterized by no B-cell differentiation and decreased immunoglobulins of all classes?
Common variable immunodeficiency (CVID)
41
Which immunodeficiency is characterized by no B-cell maturation?
X-linked (Bruton) agammaglobulinemia versus CVID, which is caused by poor B-cell differentiation
42
Which immunodeficiency may be diagnosed with the dihydrorhodamine test?
Chronic granulomatous disease (CGD) dihydrorhodamine test is flow cytometry; CGD presents with decreased fluoresence
43
Which immunodeficiency may be diagnosed with the nitroblue tetrazolium test?
Chronic granulomatous disease (CGD) dye turns blue if NADPH oxidase is functional; stays colorless if NADPH oxidase is defective
44
Which immunodeficiency presents with defective class switching?
Hyper-IgM syndrome thus resulting in increased IgM with decreased IgA, IgG, and IgE
45
Which immunodeficiency presents with delayed separation of the umbilical cord (\> 21 days)?
Leukocyte adhesion deficiency
46
Which immunodeficiency presents with recurrent infection, failure to thrive, chronic diarrhea, and thrush?
Severe combined immunodeficiency (SCID)
47
[...] deficiency is associated with anaphylaxis during blood transfusions.
**Selective IgA** deficiency is associated with anaphylaxis during blood transfusions. other possible manifestations include recurrent sinopulmonary and GI infections and atopic disease (e.g. eczema, asthma)
48
Selective IgA deficiency is associated with [...] during blood transfusions.
Selective IgA deficiency is associated with **anaphylaxis** during blood transfusions. other possible manifestations include recurrent sinopulmonary and GI infections and atopic disease (e.g. eczema, asthma)
49
C1 inhibitor deficiency results in [...].
C1 inhibitor deficiency results in **hereditary angioedema.** characterized by rapid-onset edema of the face, genitals, limbs, larynx, and intestines without urticaria (helps distinguish hereditary angioedema from type 1 hypersensitivity reactions); treatment options include with fresh frozen plasma (contains C1 inhibitor), icatibant (bradykinin antagonist), and/or ecallantide (kallikrein inhibitor)
50
[...] deficiency results in hereditary angioedema.
**C1 inhibitor** deficiency results in hereditary angioedema. characterized by rapid-onset edema of the face, genitals, limbs, larynx, and intestines without urticaria (helps distinguish hereditary angioedema from type 1 hypersensitivity reactions); treatment options include with fresh frozen plasma (contains C1 inhibitor), icatibant (bradykinin antagonist), and/or ecallantide (kallikrein inhibitor)
51
Chronic granulomatous disease is characterized by recurrent infection and granuloma formation with [...]-positive organisms.
Chronic granulomatous disease is characterized by recurrent infection and granuloma formation with **catalase-**positive organisms. particularly Staphylococcus aurues, Pseudomonas, Serratia, Nocardia, and Aspergillus; common manifestations include pneumonia, skin abscesses, and suppurative adenitis
52
[...] disease is characterized by recurrent infection and granuloma formation with catalase-positive organisms.
**Chronic granulomatous** disease is characterized by recurrent infection and granuloma formation with catalase-positive organisms. particularly Staphylococcus aurues, Pseudomonas, Serratia, Nocardia, and Aspergillus; common manifestations include pneumonia, skin abscesses, and suppurative adenitis
53
Hyper-IgM syndrome is most commonly due to mutated [...] on Th cells.
Hyper-IgM syndrome is most commonly due to mutated **CD40L** on Th cells. CD40L normally binds to CD40 on B-cells to induce class switching
54
[...] syndrome is most commonly due to mutated CD40L on Th cells.
**Hyper-IgM** syndrome is most commonly due to mutated CD40L on Th cells. CD40L normally binds to CD40 on B-cells to induce class switching
55
Leukocyte adhesion deficiency is characterized by recurrent skin and mucosal bacterial infections with absent [...] formation.
Leukocyte adhesion deficiency is characterized by recurrent skin and mucosal bacterial infections with absent **pus** formation. patients also have impaired wound healing and severe periodontal disease
56
[...] deficiency is characterized by recurrent skin and mucosal bacterial infections with absent pus formation.
**Leukocyte adhesion** deficiency is characterized by recurrent skin and mucosal bacterial infections with absent pus formation. patients also have impaired wound healing and severe periodontal disease
57
Hyper-IgM syndrome is characterized by absent [...] on lymph node biopsy
Hyper-IgM syndrome is characterized by absent **germinal centers** on lymph node biopsy.
58
In patients with IgA deficiency and celiac disease, Ig[...] antibodies against endomysium, tTG, or gliadin are useful for diagnosis.
In patients with IgA deficiency and celiac disease, Ig**G** antibodies against endomysium, tTG, or gliadin are useful for diagnosis.
59
Leukocyte adhesion deficiency (type 1) is due to a defect in [...] protein on phagocytes.
Leukocyte adhesion deficiency (type 1) is due to a defect in **LFA-1 integrin (CD18)** protein on phagocytes .
60
SCID may be caused by an autosomal recessive defect in the [...] enzyme.
SCID may be caused by an autosomal recessive defect in the **adenosine deaminase (ADA)** enzyme.
61
SCID may be caused by an X-linked defect in the [...] receptor (most common).
SCID may be caused by an X-linked defect in the **IL-2** receptor (most common).
62
What is the mode of inheritance of hyper-IgM syndrome?
X-linked recessive
63
What is the most common mode of inheritance of chronic granulomatous disease?
X-linked recessive
64
What type of hypersensitivity reaction is serum sickness?
Type III HSR
65
When does X-linked agammagloblinemia typically present?
6 months after birth due to decreased maternal IgG; presents with recurrent bacterial, enterovirus, and Giardia infections
66
Which classes of immunoglobulin are increased in Wiskott-Aldrich syndrome?
IgA and IgE levels of IgG and IgM are normal or decreased
67
X-linked (Bruton) agammaglobulinemia is caused by a defect in the [...] gene.
X-linked (Bruton) agammaglobulinemia is caused by a defect in the **BTK** gene. encodes for a tyrosine kinase; defect results in failure for pre- and pro-B cells to mature
68
X-linked (Bruton) agammaglobulinemia is characterized by [...] lymph nodes and tonsils.
X-linked (Bruton) agammaglobulinemia is characterized by **absent/scanty** lymph nodes and tonsils.
69
B-Cell Disorders
* Bruton Agammaglobinemia * Common Variable Immunodeficiency (CVID) * IgA Deficiency * Hyper-IgM Syndrome
70
T-Cell Disorders
•Thymic Aplasia (DiGeorge syndrome)
71
Combined Disorders
* Ataxia Telangiectasia * Severe Combined Immunodeficiency * Wiskott-Aldrich syndrome
72
Phagocytic Deficiencies
* Chronic Granulomatous Disease (CGD) * Leukocyte Adhesion Deficiency * Chedial-Higashi syndrome * Job syndrome (Hyperimmunoglobulin E syndrome)
73
Complement Disorders
* C1 esterase Deficiency (hereditary angioedema) * Terminal Complement Deficiency (C5-C9)
74
Bruton Agammaglobulinemia
* X linked, boys * symptoms after 6 months when maternal IgG no longer active
75
Common Variable Immunodeficiency (CVID)
* combined B and T cell defect * all Ig levels low * normal B cell numbers, decreased plasma cells * 15-35 years old
76
IgA Deficiency
* mild * most common immunodeficiency * decreased IgA only
77
Hyper-IgM syndrome
* absence of CD40 ligand that allows class switching from IgM to others * increased IgM, all others low * normal number of lymphocytes
78
DiGeorge syndrome
* thymic aplasia * AD * CATCH 22 * presents with tetany secondary to hypocalcemia
79
Ataxia Telangiectasia
* progressive cerebellar ataxia and oculocutaneous telangiectasias * AR mutation in gene resposnible for dsDNA breaks
80
Severe Combined Immunodeficiency
* X linked recessive * severe lack of B and T Cells caused by a defect in stem cell maturation amd decreased adenosine deaminase * "bubble boy disease"
81
Wiskott-Aldrich syndrome
* X linked recessive, boys only * increased IgE/IgA, decreased IgM and thrombocytopenia * bleeding, eczema, recurrent otitis media
82
Chronic Granulomatous Disease (CGD)
* X linked or AR * deficient super oxide production by polymorphonuclear leukocytes and macrophages * anemia, hyperagammaglobulinemia
83
Leukocyte Adhesion Deficiency (LAD)
* defect in chemotaxis of leukocytes * decreased phagocytic activity
84
Chediak-Higashi syndrome
* AR * defect in neutrophil chemotaxis/microtubule polymerization * partial oculocutaneous albinism, peripheral neuropathy, neutropenia
85
Job syndrome (Hyperimmunoglobulin E syndrome)
•defect in neutrophil chemotaxis
86
C1 esterase Deficiency
* herediatry angioedema * AD
87
Terminal Complement Deficiency
•inability to form membrane attack complex (MAC)
88
Chronic Granulomatous Disease infections are catalase [].
Chronic Granulomatous Disease infections are catalase **positive**. S,aureus, E. coli, Candida, Klebsiella, Pseudomonas, Aspergillus, Norcardia
89
Bruton's Agammaglobulinopathy infections are often [] and [].
Bruton's Agammaglobulinopathy infections are often **enteroviruses** and **pneumonia.**

Pediatrics (20 decks)

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