Anatomy, physiology and pathology Flashcards

1
Q

which cytokine produced by bone marrow and thymic stromal cells is a growth factor required for the development of T and B cells?

A

IL-7

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2
Q

what process results in the apoptosis of autoreactive or nonfunctional lymphocytes?

A

Negative selection

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3
Q

what diseases are associated with congenital thymic aplasia or hypoplasia in humans? (4)

A
  • DiGeorge syndrome
  • SCID
  • CHARGE
  • infant of diabetic mother
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4
Q

what condition is associated with hypogammaglobulinemia and thymoma?

A

Good’s syndrome

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5
Q

what pathogen is frequently associated with gastric MALT lymphoma?

A

chronic H.pylori infection is associated with 72-98% of cases

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6
Q

this normal anatomic variant is characterized by a pneumatized cavity within the concha

A

concha bullosa

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7
Q

what is the name of the junction between the nasal vestibule and main nasal cavity, just anterior to the tip of the inferior turbinate?

A

nasal valve

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8
Q

physical or functional absence of a spleen is associated with increased susceptibility infections with what pathogens?

A

Encapsulated bacteria (Neisseria meningitidis, Haemophilus influenzae, streptococcus pneumoniae)

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9
Q

how is nasal airway resistance regulated?

A

swelling of inferior turbinate

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10
Q

what family of medications would be best suited to treat clear watery nasal secretions?

A

anti-cholinergic medications (ipratropium bromide nasal spray)

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11
Q

how can mucociliary clearance be clinically evaluated?

A

Saccharin test

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12
Q

what syndrome is characterized by ciliary dyskinesia, situs inversus, bronchiectasis, and chronic sinusitis?

A

Kartagener’s syndrome
(50% of PCD patients have Kartagener’s)

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13
Q

identify structures of A,B,C and D in Fig 3-2

A

(A) maxillary sinus; (B) ethmoid sinus; (C) concha bullosa; (D) inferior turbinate

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14
Q

identify structure E and F in figure 3-3

A

(E) sphenoid sinus; (F) posterior ethmoid sinus

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15
Q

which sinuses are present at birth?

A

maxillary and ethmoid sinuses

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16
Q

what is the name of the ethmoid air cells found in the infraorbital area?

A

Haller cells

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17
Q

what are displaced sphenoethmoid air cells called?

A

onodi cells

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18
Q

what is the name of the anterior ethmoid air cells found above the middle turbinate?

A

Agger nasi cells

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19
Q

what are the pathologic features of airway remodeling? (4)

A
  • airway wall thickening with increased collagen deposition
  • airway smooth muscle and globlet cell hyperplasia
  • sub epithelial fibrosis
  • angiogenesis
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20
Q

what chymase and tryptase containing cells are typically found in the skin, conjunctiva, heart and intestinal mucosa and have CD88 receptor?

A

connective tissue mast cells

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21
Q

what carbohydrate epitope helps dermal T cells home to the skin?

A

cutaneous lymphocyte antigen (CLA) -1

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22
Q

Mutations in which gene are associated with ichthyosis vulgaris and atopic eczema?

A

Filaggrin (FLG)

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23
Q

What condition is associated with flaccid bullae affecting the scalp, chest, intertriginous areas and oral mucosa?

A

Pemphigus vulgaris

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24
Q

A 75-year-old woman complains of a very pruritic rash characterized by tense blisters. Tissue immunofluorescence demonstrates linear basement membrane zone IgG and C3. What condition does she have?

A

Bullous pemphigoid

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25
Q

a 4 year old boy with a history of chronic diarrhea presents with an itchy rash characterized by small blisters on his elbows and knees. what is the name of his rash?

A

Dermatitis herpetiformis (associated with celiac disease)

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26
Q

what is a diagnostic biopsy characteristic of EoE?

A

> 15 eos/hpf while on PPI for 8 weeks.

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27
Q

What sinuses are the last to develop? what age?

A

Frontal sinus at 6 year old

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28
Q

what autoimmune condition is associated with dermatitis herpetiformis?

A

Celiac disease

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29
Q

What does CD88 receptor bind to?

A

C5a anaphylatoxin

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30
Q

What paired structures are found on the lateral walls of the nasal cavity in a curled like shape?

A

turbinates

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31
Q

cavernous sinus thrombosis is a complication of sinusitis in which sinus cavities?

A

sphenoid sinuses

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32
Q

what are the ciliated columnar cells sloughed in the bronchial mucosa and are histopathologic findings indicative of asthma?

A

Creola bodies

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33
Q

these microscopic structures are composed of eosinophil protein galectin-1, they stain purplish-red and found in asthma and parasitic pneumonia

A

Charcot-Leyden crystals

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34
Q

What is the narrowest point of the nasal anatomy?

A

nasal valve

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35
Q

what condition will demonstrate epidermal IgG and C3 cell surface staining of the suprabasal layers on immunofluorence?

A

Pemphigus vulgaris

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36
Q

describe symptoms of food protein-induced allergic proctocolitis (3)

A
  • blood streaked stool in the first few months of life
  • more common in breastfed babies
  • usually healthy otherwise
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37
Q

How does acute FPIES differ from chronic FPIES (6 each)

A

Acute: severe repetitive vomiting/diarrhea, lethargy, pallor, hypothermia, hypotension, abdominal distension within 1-3 hours of ingestions
Chronic: chronic intermittent vomiting, watery diarrhea with blood/mucus, lethargy, pallor, weight loss, failure to thrive

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38
Q

most widely used and most validated asthma prediction tool

A

Asthma Predictive Index (API)

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39
Q

how does API compare to PARS?

A
  • API has excellent negative predictive value, but poor PPV.
  • PARS has better PPV
40
Q

What is part of API? (6)

A

Any one of MAJOR criteria
- parental asthma
- physician diagnosed AD
- sensitization to aeroallergens
OR any two of MINOR criteria
- sensitization to food
- >4% eos
- wheezing apart from colds

41
Q

what is part of PARS? (6)

A
  • parental asthma
  • eczema before 3yo
  • wheezing apart from colds
  • wheezing before 3yo
  • African Am race
  • SPT > or = 2 to aeroallergen and/or food
42
Q

what score is moderate risk for PARS?

A

5-8

43
Q

what histologic finding is a hallmark of fatal asthma?

A

neutrophil accumulation

44
Q

characteristic features of asthmatic sputum (3)

A
  • Curschmann’s spirals (related to excess mucus production)
  • Creola bodies (clusters of surface airway epithelial cells)
  • charcot- Leyden crystals (colorless, needle shaped structures)
45
Q

gender risk of development of asthma

A
  • more common in males until 16yo, then more common in females
46
Q

genetic factors in asthma (5)

A
  • epigenetics
  • Chr. 5q (has IL-4 gene cluster)
  • beta2-adrenoreceptor gene (polymorphisms affect response to B2 agonists)
  • CD14 (coreceptor for TLR4; polymorphisms lead to low IgE and shift toward Th1)
  • ADAM33 metalloproteinase on Chr 20p13 (affects remodeling)
47
Q

has protective effect on dev of allergy but not asthma

A

early life exposure to dog or farm animals

48
Q

which cytokine is a hematopoietic growth factor that is required for T and B cell development?

A

IL-7

49
Q

what is the largest specialized lymphoid organ?

A

spleen

50
Q

what is the T cell zone of the spleen?

A

Periarteriolar lymphoid sheath (PALS)

51
Q

where is the B cell zone of the spleen?

A

lymphoid follicle and marginal zone

52
Q

what is the difference between T and B cell selection process?

A

T cells undergo both positive and negative selection.
- B cells only undergo negative selection

53
Q

where do different sinuses drain? (5)

A

FM AM PS SS
- frontal, maxillary and anterior ethmoid - middle meatus
- posterior ethmoid and sphenoid - sphenoethmoidal recess above superior turbinate

54
Q

where do nasolacrimal ducts drain into?

A

inferior meatus

55
Q

what are agger nasi cells

A

anterior ethmoid air cells above the middle turbinate

56
Q

what are Haller cells

A

extramural ethmoid air cells found on the floor of the orbit

57
Q

what are Onodi cells

A

sphenoethmoid air cell found in close proximity to the internal carotid artery and optic nerve

58
Q

what is concha bullosa

A

air cell in the middle turbinate

59
Q
A

Curschmann’s spirals - corkscrew shaped twists of condensed mucus

60
Q
A

Charcot Leyden crystals - colorless, needle-shaped. formed by eosinophil membrane protein

61
Q
A

Creola body - clusters of surface epithelial cells

62
Q

homing of T cells (CLA+) to skin involves which cytokines and their receptors? (4)

A
  • CCR4 (on T cells) - CCL17
  • CCR10 (on T cells) - CCL27
63
Q

name skin diseases that have linear basement membrane zone IgG and/or C3 on immunofluorescence (5)

A
  • both IgG and C3: Bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita
  • just C3: herpes gestationis
  • just IgG: bullous lupus erythematosis
64
Q

tissue immunofluorescence finding in pemphigous vulgaris

A

epidermal IgG and C3 cell surface staining of the suprabasal layer

65
Q

tissue immunofluorescence finding in pemphigous foliaceous

A

epidermal IgG and C3 cell surface staining of the granular layer

66
Q

tissue immunofluorescence finding in paraneoplastic pemphigus

A

epidermal IgG and C3 cell surface and basement membrane staining

67
Q

tissue immunofluorescence finding in IgA pemphigus

A

epidermal IgA cell surface staining

68
Q

tissue immunofluorescence finding in bullous pemphigoid

A

linear basement membrane zone IgG and C3

69
Q

tissue immunofluorescence finding in herpes gestationis

A

linear basement membrane zone C3

70
Q

tissue immunofluorescence finding in epidermolysis bullosa aquisita

A

linear basement membrane zone IgG and C3

71
Q

tissue immunofluorescence finding in Linear IgA bullous dermatosis

A

linear basement membrane zone IgA

72
Q

tissue immunofluorescence finding in dermatitis herpetiformis

A

granular basement membrane zone IgA with stippling in dermal papillae

73
Q

tissue immunofluorescence finding in bullous lupus erythematosus

A

linear basement membrane zone IgG
- may show granular IgM and C3 basement membrane zone

74
Q

serum autoAb in pemphigous vulgaris

A

IgG autoAb to desmoglein 1 and 3

75
Q

serum autoAb in pemphigous foliaceous

A

IgG autoAb to desmoglein 1

76
Q

serum autoAb in paraneoplastic pemphigus (3)

A

IgG autoAb to plakin proteins and desmoglein 1 and 3

77
Q

serum autoAb in IgA pemphigus (2)

A

IgA autoAb to desmoglein 3 and desmocollin 1

78
Q

serum autoAb in bullous pemphigoid

A

IgG autoAb to BP180 and BP230

79
Q

serum autoAb in herpes gestationis (2)

A

complement-fixing to basement membrane and autoAb to BP180

80
Q

serum autoAb in epidermolysis bullosa acquisita

A

IgG autoAb to collagen VII

81
Q

serum autoAb in linear IgA bullous dermatosis (2)

A

IgA autoAb to LABD97, LAD-1, and others

82
Q

serum autoAb in dermatitis herpetiformis

A

IgA autoAb to epidermal transglutaminase

83
Q

serum autoAb in bullous lupus erythematosus

A

IgG autoAb to collage VII

84
Q

clinical presentation in pemphigous vulgaris (4)

A
  • Flaccid>tense bullae
  • crusting
  • Nikolsky’s sign
  • affects scalp, chest, intertriginous areas and oral mucosa
85
Q

clinical presentation in pemphigous foliaceous (5)

A
  • superficial bullae
  • erosions
  • scale with crusting
  • Nikolsky’s sign
  • does NOT involve mucosa
86
Q

clinical presentation in paraneoplastic pemphigus (4)

A
  • flaccid bullae
  • lichenoid or erythema multiforme-like lesions
  • affects mucosa
  • Nikolsky’s sign
87
Q

clinical presentation in IgA pemphigus (3)

A
  • pustules
  • erythema
  • flaccid lakes of pus
88
Q

clinical presentation in bullous pemphigoid (3)

A
  • Tense > flaccid bullae
  • prominent pruritus
  • Nikolsky’s sign in some pt
89
Q

clinical presentation in herpes gestationis (3)

A
  • tense bullae
  • onset in 2nd trimester of pregnancy
  • pruritus
90
Q

clinical presentation in epidermolysis bullosa acquisita (4)

A
  • tense bullae
  • erosions
  • scarring
  • sites of trauma and oral mucosa
91
Q

clinical presentation in dermatitis herpetiformis (3)

A
  • small bullae on elbows and knees
  • markedly pruritic
  • associated with celiac dz
92
Q

clinical presentation in bullous lupus erythematosus (2)

A
  • tense bullae
  • photo distributed
93
Q

mutations in FLG (filaggrin) gene is associated with which diseases (2)

A

ichthyosis vulgaris, atopic eczema

94
Q

Tense bullae that commonly occur in areas of trauma and in the oral mucous. The proteins involved include type VII collagen and anchoring fibrils - what is the disease?

A

epidermolysis bullosa acquisita

95
Q

types of cells in the nasopharynx (1)

A

stratified squamous epithelium

96
Q
A