Anatomy, physiology and pathology Flashcards
which cytokine produced by bone marrow and thymic stromal cells is a growth factor required for the development of T and B cells?
IL-7
what process results in the apoptosis of autoreactive or nonfunctional lymphocytes?
Negative selection
what diseases are associated with congenital thymic aplasia or hypoplasia in humans? (4)
- DiGeorge syndrome
- SCID
- CHARGE
- infant of diabetic mother
what condition is associated with hypogammaglobulinemia and thymoma?
Good’s syndrome
what pathogen is frequently associated with gastric MALT lymphoma?
chronic H.pylori infection is associated with 72-98% of cases
this normal anatomic variant is characterized by a pneumatized cavity within the concha
concha bullosa
what is the name of the junction between the nasal vestibule and main nasal cavity, just anterior to the tip of the inferior turbinate?
nasal valve
physical or functional absence of a spleen is associated with increased susceptibility infections with what pathogens?
Encapsulated bacteria (Neisseria meningitidis, Haemophilus influenzae, streptococcus pneumoniae)
how is nasal airway resistance regulated?
swelling of inferior turbinate
what family of medications would be best suited to treat clear watery nasal secretions?
anti-cholinergic medications (ipratropium bromide nasal spray)
how can mucociliary clearance be clinically evaluated?
Saccharin test
what syndrome is characterized by ciliary dyskinesia, situs inversus, bronchiectasis, and chronic sinusitis?
Kartagener’s syndrome
(50% of PCD patients have Kartagener’s)
identify structures of A,B,C and D in Fig 3-2
(A) maxillary sinus; (B) ethmoid sinus; (C) concha bullosa; (D) inferior turbinate
identify structure E and F in figure 3-3
(E) sphenoid sinus; (F) posterior ethmoid sinus
which sinuses are present at birth?
maxillary and ethmoid sinuses
what is the name of the ethmoid air cells found in the infraorbital area?
Haller cells
what are displaced sphenoethmoid air cells called?
onodi cells
what is the name of the anterior ethmoid air cells found above the middle turbinate?
Agger nasi cells
what are the pathologic features of airway remodeling? (4)
- airway wall thickening with increased collagen deposition
- airway smooth muscle and globlet cell hyperplasia
- sub epithelial fibrosis
- angiogenesis
what chymase and tryptase containing cells are typically found in the skin, conjunctiva, heart and intestinal mucosa and have CD88 receptor?
connective tissue mast cells
what carbohydrate epitope helps dermal T cells home to the skin?
cutaneous lymphocyte antigen (CLA) -1
Mutations in which gene are associated with ichthyosis vulgaris and atopic eczema?
Filaggrin (FLG)
What condition is associated with flaccid bullae affecting the scalp, chest, intertriginous areas and oral mucosa?
Pemphigus vulgaris
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?
Bullous pemphigoid
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)
what is a diagnostic biopsy characteristic of EoE?
> 15 eos/hpf while on PPI for 8 weeks.
What sinuses are the last to develop? what age?
Frontal sinus at 6 year old
what autoimmune condition is associated with dermatitis herpetiformis?
Celiac disease
What does CD88 receptor bind to?
C5a anaphylatoxin
What paired structures are found on the lateral walls of the nasal cavity in a curled like shape?
turbinates
cavernous sinus thrombosis is a complication of sinusitis in which sinus cavities?
sphenoid sinuses
what are the ciliated columnar cells sloughed in the bronchial mucosa and are histopathologic findings indicative of asthma?
Creola bodies
these microscopic structures are composed of eosinophil protein galectin-1, they stain purplish-red and found in asthma and parasitic pneumonia
Charcot-Leyden crystals
What is the narrowest point of the nasal anatomy?
nasal valve
what condition will demonstrate epidermal IgG and C3 cell surface staining of the suprabasal layers on immunofluorence?
Pemphigus vulgaris
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
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
most widely used and most validated asthma prediction tool
Asthma Predictive Index (API)
how does API compare to PARS?
- API has excellent negative predictive value, but poor PPV.
- PARS has better PPV
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
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
what score is moderate risk for PARS?
5-8
what histologic finding is a hallmark of fatal asthma?
neutrophil accumulation
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)
gender risk of development of asthma
- more common in males until 16yo, then more common in females
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)
has protective effect on dev of allergy but not asthma
early life exposure to dog or farm animals
which cytokine is a hematopoietic growth factor that is required for T and B cell development?
IL-7
what is the largest specialized lymphoid organ?
spleen
what is the T cell zone of the spleen?
Periarteriolar lymphoid sheath (PALS)
where is the B cell zone of the spleen?
lymphoid follicle and marginal zone
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
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
where do nasolacrimal ducts drain into?
inferior meatus
what are agger nasi cells
anterior ethmoid air cells above the middle turbinate
what are Haller cells
extramural ethmoid air cells found on the floor of the orbit
what are Onodi cells
sphenoethmoid air cell found in close proximity to the internal carotid artery and optic nerve
what is concha bullosa
air cell in the middle turbinate
Curschmann’s spirals - corkscrew shaped twists of condensed mucus
Charcot Leyden crystals - colorless, needle-shaped. formed by eosinophil membrane protein
Creola body - clusters of surface epithelial cells
homing of T cells (CLA+) to skin involves which cytokines and their receptors? (4)
- CCR4 (on T cells) - CCL17
- CCR10 (on T cells) - CCL27
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
tissue immunofluorescence finding in pemphigous vulgaris
epidermal IgG and C3 cell surface staining of the suprabasal layer
tissue immunofluorescence finding in pemphigous foliaceous
epidermal IgG and C3 cell surface staining of the granular layer
tissue immunofluorescence finding in paraneoplastic pemphigus
epidermal IgG and C3 cell surface and basement membrane staining
tissue immunofluorescence finding in IgA pemphigus
epidermal IgA cell surface staining
tissue immunofluorescence finding in bullous pemphigoid
linear basement membrane zone IgG and C3
tissue immunofluorescence finding in herpes gestationis
linear basement membrane zone C3
tissue immunofluorescence finding in epidermolysis bullosa aquisita
linear basement membrane zone IgG and C3
tissue immunofluorescence finding in Linear IgA bullous dermatosis
linear basement membrane zone IgA
tissue immunofluorescence finding in dermatitis herpetiformis
granular basement membrane zone IgA with stippling in dermal papillae
tissue immunofluorescence finding in bullous lupus erythematosus
linear basement membrane zone IgG
- may show granular IgM and C3 basement membrane zone
serum autoAb in pemphigous vulgaris
IgG autoAb to desmoglein 1 and 3
serum autoAb in pemphigous foliaceous
IgG autoAb to desmoglein 1
serum autoAb in paraneoplastic pemphigus (3)
IgG autoAb to plakin proteins and desmoglein 1 and 3
serum autoAb in IgA pemphigus (2)
IgA autoAb to desmoglein 3 and desmocollin 1
serum autoAb in bullous pemphigoid
IgG autoAb to BP180 and BP230
serum autoAb in herpes gestationis (2)
complement-fixing to basement membrane and autoAb to BP180
serum autoAb in epidermolysis bullosa acquisita
IgG autoAb to collagen VII
serum autoAb in linear IgA bullous dermatosis (2)
IgA autoAb to LABD97, LAD-1, and others
serum autoAb in dermatitis herpetiformis
IgA autoAb to epidermal transglutaminase
serum autoAb in bullous lupus erythematosus
IgG autoAb to collage VII
clinical presentation in pemphigous vulgaris (4)
- Flaccid>tense bullae
- crusting
- Nikolsky’s sign
- affects scalp, chest, intertriginous areas and oral mucosa
clinical presentation in pemphigous foliaceous (5)
- superficial bullae
- erosions
- scale with crusting
- Nikolsky’s sign
- does NOT involve mucosa
clinical presentation in paraneoplastic pemphigus (4)
- flaccid bullae
- lichenoid or erythema multiforme-like lesions
- affects mucosa
- Nikolsky’s sign
clinical presentation in IgA pemphigus (3)
- pustules
- erythema
- flaccid lakes of pus
clinical presentation in bullous pemphigoid (3)
- Tense > flaccid bullae
- prominent pruritus
- Nikolsky’s sign in some pt
clinical presentation in herpes gestationis (3)
- tense bullae
- onset in 2nd trimester of pregnancy
- pruritus
clinical presentation in epidermolysis bullosa acquisita (4)
- tense bullae
- erosions
- scarring
- sites of trauma and oral mucosa
clinical presentation in dermatitis herpetiformis (3)
- small bullae on elbows and knees
- markedly pruritic
- associated with celiac dz
clinical presentation in bullous lupus erythematosus (2)
- tense bullae
- photo distributed
mutations in FLG (filaggrin) gene is associated with which diseases (2)
ichthyosis vulgaris, atopic eczema
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
types of cells in the nasopharynx (1)
stratified squamous epithelium