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Anatomy, physiology and pathology Flashcards

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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
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?
Dermatitis herpetiformis (associated with celiac disease)
26
what is a diagnostic biopsy characteristic of EoE?
>15 eos/hpf while on PPI for 8 weeks.
27
What sinuses are the last to develop? what age?
Frontal sinus at 6 year old
28
what autoimmune condition is associated with dermatitis herpetiformis?
Celiac disease
29
What does CD88 receptor bind to?
C5a anaphylatoxin
30
What paired structures are found on the lateral walls of the nasal cavity in a curled like shape?
turbinates
31
cavernous sinus thrombosis is a complication of sinusitis in which sinus cavities?
sphenoid sinuses
32
what are the ciliated columnar cells sloughed in the bronchial mucosa and are histopathologic findings indicative of asthma?
Creola bodies
33
these microscopic structures are composed of eosinophil protein galectin-1, they stain purplish-red and found in asthma and parasitic pneumonia
Charcot-Leyden crystals
34
What is the narrowest point of the nasal anatomy?
nasal valve
35
what condition will demonstrate epidermal IgG and C3 cell surface staining of the suprabasal layers on immunofluorence?
Pemphigus vulgaris
36
describe symptoms of food protein-induced allergic proctocolitis (3)
- blood streaked stool in the first few months of life - more common in breastfed babies - usually healthy otherwise
37
How does acute FPIES differ from chronic FPIES (6 each)
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
38
most widely used and most validated asthma prediction tool
Asthma Predictive Index (API)
39
how does API compare to PARS?
- API has excellent negative predictive value, but poor PPV. - PARS has better PPV
40
What is part of API? (6)
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
what is part of PARS? (6)
- parental asthma - eczema before 3yo - wheezing apart from colds - wheezing before 3yo - African Am race - SPT > or = 2 to aeroallergen and/or food
42
what score is moderate risk for PARS?
5-8
43
what histologic finding is a hallmark of fatal asthma?
neutrophil accumulation
44
characteristic features of asthmatic sputum (3)
- Curschmann's spirals (related to excess mucus production) - Creola bodies (clusters of surface airway epithelial cells) - charcot- Leyden crystals (colorless, needle shaped structures)
45
gender risk of development of asthma
- more common in males until 16yo, then more common in females
46
genetic factors in asthma (5)
- 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
has protective effect on dev of allergy but not asthma
early life exposure to dog or farm animals
48
which cytokine is a hematopoietic growth factor that is required for T and B cell development?
IL-7
49
what is the largest specialized lymphoid organ?
spleen
50
what is the T cell zone of the spleen?
Periarteriolar lymphoid sheath (PALS)
51
where is the B cell zone of the spleen?
lymphoid follicle and marginal zone
52
what is the difference between T and B cell selection process?
T cells undergo both positive and negative selection. - B cells only undergo negative selection
53
where do different sinuses drain? (5)
FM AM PS SS - frontal, maxillary and anterior ethmoid - middle meatus - posterior ethmoid and sphenoid - sphenoethmoidal recess above superior turbinate
54
where do nasolacrimal ducts drain into?
inferior meatus
55
what are agger nasi cells
anterior ethmoid air cells above the middle turbinate
56
what are Haller cells
extramural ethmoid air cells found on the floor of the orbit
57
what are Onodi cells
sphenoethmoid air cell found in close proximity to the internal carotid artery and optic nerve
58
what is concha bullosa
air cell in the middle turbinate
59
Curschmann's spirals - corkscrew shaped twists of condensed mucus
60
Charcot Leyden crystals - colorless, needle-shaped. formed by eosinophil membrane protein
61
Creola body - clusters of surface epithelial cells
62
homing of T cells (CLA+) to skin involves which cytokines and their receptors? (4)
- CCR4 (on T cells) - CCL17 - CCR10 (on T cells) - CCL27
63
name skin diseases that have linear basement membrane zone IgG and/or C3 on immunofluorescence (5)
- both IgG and C3: Bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita - just C3: herpes gestationis - just IgG: bullous lupus erythematosis
64
tissue immunofluorescence finding in pemphigous vulgaris
epidermal IgG and C3 cell surface staining of the suprabasal layer
65
tissue immunofluorescence finding in pemphigous foliaceous
epidermal IgG and C3 cell surface staining of the granular layer
66
tissue immunofluorescence finding in paraneoplastic pemphigus
epidermal IgG and C3 cell surface and basement membrane staining
67
tissue immunofluorescence finding in IgA pemphigus
epidermal IgA cell surface staining
68
tissue immunofluorescence finding in bullous pemphigoid
linear basement membrane zone IgG and C3
69
tissue immunofluorescence finding in herpes gestationis
linear basement membrane zone C3
70
tissue immunofluorescence finding in epidermolysis bullosa aquisita
linear basement membrane zone IgG and C3
71
tissue immunofluorescence finding in Linear IgA bullous dermatosis
linear basement membrane zone IgA
72
tissue immunofluorescence finding in dermatitis herpetiformis
granular basement membrane zone IgA with stippling in dermal papillae
73
tissue immunofluorescence finding in bullous lupus erythematosus
linear basement membrane zone IgG - may show granular IgM and C3 basement membrane zone
74
serum autoAb in pemphigous vulgaris
IgG autoAb to desmoglein 1 and 3
75
serum autoAb in pemphigous foliaceous
IgG autoAb to desmoglein 1
76
serum autoAb in paraneoplastic pemphigus (3)
IgG autoAb to plakin proteins and desmoglein 1 and 3
77
serum autoAb in IgA pemphigus (2)
IgA autoAb to desmoglein 3 and desmocollin 1
78
serum autoAb in bullous pemphigoid
IgG autoAb to BP180 and BP230
79
serum autoAb in herpes gestationis (2)
complement-fixing to basement membrane and autoAb to BP180
80
serum autoAb in epidermolysis bullosa acquisita
IgG autoAb to collagen VII
81
serum autoAb in linear IgA bullous dermatosis (2)
IgA autoAb to LABD97, LAD-1, and others
82
serum autoAb in dermatitis herpetiformis
IgA autoAb to epidermal transglutaminase
83
serum autoAb in bullous lupus erythematosus
IgG autoAb to collage VII
84
clinical presentation in pemphigous vulgaris (4)
- Flaccid>tense bullae - crusting - Nikolsky's sign - affects scalp, chest, intertriginous areas and oral mucosa
85
clinical presentation in pemphigous foliaceous (5)
- superficial bullae - erosions - scale with crusting - Nikolsky's sign - does NOT involve mucosa
86
clinical presentation in paraneoplastic pemphigus (4)
- flaccid bullae - lichenoid or erythema multiforme-like lesions - affects mucosa - Nikolsky's sign
87
clinical presentation in IgA pemphigus (3)
- pustules - erythema - flaccid lakes of pus
88
clinical presentation in bullous pemphigoid (3)
- Tense > flaccid bullae - prominent pruritus - Nikolsky's sign in some pt
89
clinical presentation in herpes gestationis (3)
- tense bullae - onset in 2nd trimester of pregnancy - pruritus
90
clinical presentation in epidermolysis bullosa acquisita (4)
- tense bullae - erosions - scarring - sites of trauma and oral mucosa
91
clinical presentation in dermatitis herpetiformis (3)
- small bullae on elbows and knees - markedly pruritic - associated with celiac dz
92
clinical presentation in bullous lupus erythematosus (2)
- tense bullae - photo distributed
93
mutations in FLG (filaggrin) gene is associated with which diseases (2)
ichthyosis vulgaris, atopic eczema
94
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?
epidermolysis bullosa acquisita
95
types of cells in the nasopharynx (1)
stratified squamous epithelium
96

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