lecture 33+34+DLA Flashcards

1
Q

area of the brain responsible for breathing rhythm

A

pre-botzinger area in the rostral ventromedial medulla

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2
Q

neurons that drive inspiration?

A

DRG (doral root ganglion) and rostral VRG (ventral respiratory group)

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3
Q

pontine nuclei

A

switch between inspiration and expiration

pons

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4
Q

The reflex pathways?

A
  1. pulmonary stretch receptors: respond to transmural pressure
  2. irritant receptors: in response to touch or certain substances; results in coughing/ gasping
  3. proprioceptors: signal breathing effort
  4. J receptors: fire in response to lung injury, results in shallow breathing, airway secretion, constriction
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5
Q

central receptors?

A

located in the medulla

responds to changes in pH and CO2 levels of CSF

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6
Q

H+ and HCO3 permeability (BBB)

A

largely impermeable

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7
Q

CSF sensitivity and breathing rate

A

sensitive to changes in CO2

affected by respiratory acid/base changes

CSF pH decreases (coming more acidic) the rate
of ventilation increases blowing away the acid (CO2)

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8
Q

peripheral chemoreceptors

A

located in carotid and aortic body

detect changes in pH, O2, and CO2

carotid: glosso-pharyngeal nerve (afferent to CNS)
aortic: vagus nerve (afferent to CNS)

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9
Q

treatment for patient with breathlessness?

A

administration of oxygen

do not give 100 percent oxygen, titrate it

maintain O2 (60-65mm) (88 to 92%)

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10
Q

which way does the flow volume loop move during obstructive and restrictive lung diseases?

A

obstructive: moves to left
restrictive: Moves to right

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11
Q

airway resistance decreases going down the respiratory tree even though radius get smaller, why?

A

The radius is smaller, but the cross sectional area is very large

thus air way resistance decreases

flows in a parallel fashion

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12
Q

which areas have the largest and smallest airway resistance?

A

highest: medium-sized bronchi
smallest: small airways

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13
Q

what has the greatest impact on air flow into lungs

A

The radius!

R = 8nL / pi (r)4

reducing radius by 1/2, increases resistance by 16!

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14
Q

what diseases have a large increase in airway resistance?

A

COPD and asthma

V = delta P / R

must compensate by breathing harder

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15
Q

dynamic airway compression

A

starts after the equal-pressure point (EPP)

increases resistance to airflow and limits flow during expiration (flow is limited by collapsed airways)

collapse due to intrapleural pressure becoming greater than the alveolar pressure (forced expiration)

compression decreases radius and increases resistance

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16
Q

alpha and beta globin genes?

where are they on the chromosome and how many copies?

A

alpha globin is found on chromosome 16 and their are 4 genes.

beta globin is found on chromosome 11 and their are two genes.

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17
Q

expression patterns of the globin genes during fetal life? adult life?

A

fetal: high gamma, zeta, and alpha expression
low beta expression

adult: high alpha and beta expression
low gamma expression

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18
Q

what are the compositions of HbA, HbF, and HbA2?

A

HbA: two beta and two alpha (most common)

HbF: two alpha and two gamma (fetal)

HbA2: two alpha and two delta

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19
Q

Hemoglobin S

A

also known as sickle cell disease

single point mutation in the Beta-globin gene on the 6th codon.
glutamic acid to valine

autosomal recessive inheritance

the presence of ‘sticky’ patches, thus can lead to sickling/polymerizing

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20
Q

Can sickle cell anemia be distinguished during fetal life?

A

No.

beta-globin protein has low expression until birth

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21
Q

what is the treatment for HbS?

A

Hydroxyurea (inhibit histone deacetylation)

this drug will increase fetal globin expression
de-repression of the gamma globin gene
More HbF formation

22
Q

Hemoglobin C

A

point mutation at the 6th codon of the B-globin gene, thus a missense mutation

glutamic acid to lysine (a + AA)

homozygotes have mild hemolysis
low solubility

23
Q

HbSC disease

A

Two different mutations on the beta globin gene

sickling is similar to sickle cell disease but more mild

24
Q

what does the electrophoresis look like when comparing globin proteins?

A

HbA moves the furthest

HbF moves second furthest

HbS (sickle cell) moves third furthest

HbC moves little

A, F, S, C (in order)

A Fat Santa Clause

25
Q

overview of beta and alpha thalassemia?

A

Alpha - reduced alpha chain synthesis

beta - reduced beta strand synthesis

26
Q

alpha thalassemia?

what are the four different kinds?

A

reduced alpha chain synthesis

  1. normal, all 4 genes are functional
  2. silent carrier, 3 of the genes are functional (no symptoms)
  3. cis and trans carrier (heterozygote)
    cis: deletion of two genes on the same chromosome
    trans: deletion of one copy from each chromosome
    may have mild anemia
4. HbH alpha thalassemia
deletion of three genes; one functional allele 
most likely one parent was cis and other was trans 
presents in infancy or childhood
mild to moderate anemia 
bone changes may be seen 
aggregation of beta tetramers 
HbA levels might be low 
  1. hemoglobin bart hydrops fetalis syndrome
    most severe form
    see Hb bart (aggregations of gamma tetramers)
    all alleles are non-functional
    no detectable HbF or HbA
    lethal occurrence
    most likely both parents had a cis mutation
27
Q

overview of beta-thalassemia

A

autosomal recessive inheritance
allelic heterogenicity and compound heterogenicity can be seen
variable severity can also be seen

two types of mutations:

  1. Beta +: reduced expression
  2. Beta 0: suppression of gene expression

excessive alpha chains can precipitate and lead to severe hemolytic anemia by binding to RBC membranes and causing damage

do not form tetramers

28
Q

what are the three forms of beta-thalassemia?

A

major:
two severe mutations
very low levels or absence of HbA
high levels of HbA2 and HbF to compensate

intermedia:
one severe mutation and one less severe or two less severe mutations
Low HbA levels
High HbA2 and HbF levels to compensate

minor:
almost normal levels of HbA
heterozygote

29
Q

what bone changes are seen in beta- thalassemia?

A

extramedullary erythropoiesis (looks like hair on the bone)

frontal bossing, malar prominence

30
Q

Hemophilia A

A

X-linked recessive
lot enough factor 8 due to gene deletion, intron inversion

replacement therapy is treatment

31
Q

hemophilia B

A

X-linked recessive
lost enough factor 9 due to point mutation and deletion

replacement therapy for RX

32
Q

closed vs open pneumothorax

A

closed: air goes from lung into pleural cavity
open: air goes from outside the body into the pleural cavity

the lung will collapse due to the transpulmonary pressure becoming zero

33
Q

what is the tidal volume (TV)?

A

the volume of air inhaled or exhaled with each

normal breath

34
Q

what is the Inspiratory Reserve Volume (IRV)?

A

the volume of air that can be inhaled at the end of a normal tidal inspiration

35
Q

Expiratory Reserve Volume (ERV)?

A

the volume of air within the lungs that can be exhaled after the end of a tidal exhalation

36
Q

Residual Volume (RV)?

A

the remaining air in the lungs that cannot be expelled

37
Q

Functional Residual Capacity (FRC)

A

the total volume of air remaining in the lungs at the end of a tidal exhalation

38
Q

Total Lung Capacity (TLC)

A

the volume of air in the lungs at the end of a maximal inspiration

39
Q

Vital Capacity (VC)

A

the volume of air exhaled from maximal inspiration to maximal exhalation; maximum expiration

40
Q

Forced Expiratory Volume in 1 second (FEV1):

A

the volume of air exhaled in the first second of a FVC test

41
Q

how to calculate total long capacity

A

(TLC = IRV+VT+ERV+RV)

42
Q

how to calculate Inspiratory Capacity

A

(IC = VT + IRV)

43
Q

how to calculate functional residual capacity

A

(FRC = ERV + RV)

44
Q

how to calculate vital capacity

A

(VC = IRV + ERV + VT)

45
Q

what cannot be calculated by spirometry

A

the residual volume

46
Q

spirometry and obstructive disorders?

A

Expiratory flow rate is significantly decreased resulting in decreased FEV1 and FVC

The FEV1/FVC ratio is low

the ratio is lower than 80%
it takes longer to get to the needed volume

47
Q

spirometry and restrictive disorders?

A

Lung inflation is decreased resulting in decreased FEV1 and FVC

The FEV1/FVC ratio is normal or increased.

the ratio is still 80%, but the graph looks different.
volume is much lower

48
Q

FEF25-75

A

has the greatest sensitivity for the detection of

early airflow obstruction

49
Q

the flow volume loop and obstructive lung disease?

what about restrictive?

A

obstructive: will have a ‘scooped out’ appearance
seen commonly in those with emphysema

restrictive: significant reduction in size

50
Q

what is COPD?

A

combination of chronic bronchitis, emphysema, and asthma

commonly caused by smoking

decreased airflow, thus reduced FVC, FEV1, and decreased FEV1/FVC ratio