Chest Wall and Respiratory Muscle disorders

Question 1

in newborns, FRC is maintained by

Answer 1

increase RR

decreased duration of inspiratory contraction

decreased time for lung deflation

glottal closure

Question 2

ventilator induced diaphragm dysfunction can occur as quickly as how many hrs

Answer 2

18 hrs on vent

Question 3

chest wall dysfuntion

Answer 3

reduced: lung volume
absolute flow rates
strength of respi muscles

RV and FEV1/FVC ratio normal
*RV can be augmented

Question 4

hypercapnia is present during wakefulness with respi muscle strength less than how many %

Answer 4

less than 25% predicted

Question 5

decrease in VC greater than expected if muscle strength is

Answer 5

less than 50% predicted

(atelectasis -decreased compliance)
joint ankylosis

Question 6

can be the 1st symptom of chronic respi pump impairment

Answer 6

General fatigue and dyspnea on exertion

Question 7

clinical signs of impending respi failure

Answer 7

Question 8

to meet muscle work requirement

Answer 8

increase respi drive and phrenic nerve output

increase recruitment of FAST twitch muscle fiber

recruitment of Accessory respi muscles of chest and neck

change in respi pattern (reduce itime)

Question 9

Upper motor neuron d/o causing chest wall dysfunction

Answer 9

CP
hemi and quadrilegia

Question 10

Critical periods at risk of vent failure

Answer 10

neonatal
respi infection
GA
last tri preg

Question 11

Frequent cause of diaphragmatic paralysis in newborn

Answer 11

stretching of c3 to c5 root during breech delivery (Erbs palsy)

Question 12

most common side affected in diaphragmatic paralysis

Answer 12

right side

(more than 2 rib spaces higher than the left)

Question 13

bilateral diaphragmatic paralysis, TLC is often less than how many percent

Answer 13

50%

Question 14

confirmatory test for diaphragmatic paralysis

Answer 14

EMG with percutaneous stimulation of phrenic nerve

Question 15

Ultrasound in spont breathing patient witg diaphragmatic paralysis

Answer 15

paradoxical INspiratory UPward motion of the PARaLYZED diaphragm

Question 16

onset or age of 3 SMA types

Answer 16

0-6 months (type 1)
after 6 months (type 2)
after 10 months (type 3)

Question 17

SMA type which has poor prognosis
and usually die by 18 months from respi failure

intact sensory
lack of motor

Answer 17

SMA type 1

Question 18

Closing parasol

good quality of life

Answer 18

SMa type II

Question 19

proximal limb weakness

walks 25 meters

frequent fall

normal lifespan

Answer 19

sma type 3

Question 20

diagnosis of sma

Answer 20

gene testing

Question 21

atrophy of type 1 and 2 muscle fiber

Answer 21

biopsy fx of sma

Question 22

chromosome involve in SMA vs DMD

Answer 22

SMA chromosome 5
DMD short arm of chromosome Xp21

Question 23

Difference between DMD and Becker Md

Answer 23

DMD absence of dystrophin
Becker Mutant dystrophin

Question 24

Difference of MG subtypes

Answer 24

Question 25

In DMd, age onset

Answer 25

2-4 yo
boys
proximal muscle weakness

Question 26

by what age does dmd px reach maximum plateau in vital capacity and progressive decrease thereafter

Answer 26

10 to 12 yo

Question 27

1st sign of chronic respi failure in dmd px

Answer 27

Nocturnal hypoventilation

Question 28

Presents in a young boy
with speech and muscle function delay

what to request if suspecting DMD

Answer 28

serum CK
50 to 100 times

genetic testing

Question 29

When to do surgery in dmd px with scoliosis

Answer 29

progressing cob angle 20 degrees
VC of atleast 40% of predicted

Question 30

pharma tx for dmd

Answer 30

pred 0.75mg/kg/day
Deflazacort 0.9 (cataract)

Question 31

chromosome 19

Answer 31

Myotonic dystrophy

Question 32

type of MD not presenting with myotonia

Answer 32

CONGENITAL

Question 33

what raises susp in px to have congenital md

Answer 33

mother has mild weakness of eyelid closure

overt grip myotonia

Question 34

Steinheart dx (autosomal dominant)

Or type 1 Myotonic dystrophy has mutation on

Answer 34

Chromosome 19

expansion of CTG trinucleotide in DMPK gene

Question 35

what can be seen in cxr of congenital myotonic dystrophy

Answer 35

thin ribs
right diaphragm elevation

Question 36

type of myotonic dystrophy presenting with aspiration pneumonia and post anesth failure

Answer 36

JUVEnile myotonic dystrophy

Question 37

system involved in Md

Answer 37

eyes
cns
endo
heart

skeletal
smooth muscles

Question 38

60% of pectus excavatum has shallow chest in ap described as

Answer 38

pectus gracilis

Question 39

it is polygenetic, common in white males, more than 90% of all congenital chest wall abnormalities

Answer 39

Pectus excavatum

Question 40

pectus excavatum is associated with what syndrome

Answer 40

Marfan

Question 41

spine and chest wall disorder impairing lung growth

Answer 41

Thoracic insufficiency syndrome

Question 42

Syndrome with small thoraces leading to secondary respi failure

Answer 42

Hypoplastic thorax syndrome

Question 43

Hypoplastic thorax syndrome occurs due to

Answer 43

reduced circumference
reduced thoracic vertebral height

Question 44

most common hypoplastic thoracic syndrome

Answer 44

Asphyxiating Thoracic Dystrophy

Question 45

Also known as Jeune Syndrome
auto recessive
ciliary chondroplasia

Answer 45

Asphyxiating TD
Thoracic pelvic phalangeal dystrophy
Shwachman syndrome