Respirology Nelson 2 Flashcards
Which of the following is not a cause of unilateral hyper lucent lung?
a) pneumonia
b) bronchiolitis obliterans
c) cystic fibrosis
d) vasculitis obliterans
c) cystic fibrosis more likely to be generalized hyper inflation if present
manifestations of unilateral hyper lucent lung:
- can follow cardiac/pulmonary disease
for pneumonias most likely adenovirus
clinical findings - can be pneumonia sx or asymptomatic, some have hemoptosys, CXR shows mediastinum shift towards abrnomal (discuss) lung, sometimes shifts away
- aka Swyer - James syndrome, no specific treatment is known
Which lobe is most commonly affected by Congenital lobar emphysema
a) RLL
b) LLL
c) LUL
d) RUL
c) LUL most commonly affected
upper and middle affected, low not so much
affected lob is essentially nonfunctional
mediastinum to the opposite side
when lots of symptoms lobectomy is the surgical treatment
in older kids, investigations should include CXR, CT, VQ scan, bronchoscopy , in <2 months, CXR/CT and clinical
Which of the following is the most common cause of subcutaneous emphysema?
a) tracheostomy
b) esophageal wounds
c) fracture of the orbit
d) pneumomediastnum or pneumothorax
d) most common is pneumomediastinum or pneumothorax, the others are causes too (pg 1780)
Usually don’t need any specific treatment, avoid activities that increase airway pressure (cough, high pressure pulmonary function testing
resolution after air resorbed (and source eliminated)
rarely the air can compress the trachea
Which of the following about alpha - 1 anti trypsin deficiency in children is false?
a) commonly presents with lung disease
b) autosomal recessive inheritance
c) usually physical examination in childhood is normal
d) serum immunoassay will show low levels of alpha1 antitrypsin
a) false- rarely causes lung disease in children, generally causes liver disease in children and lung disease in adults (30-40 year old)
when their is lung disease it is emphysema
smoking speeds up the development of lung disease
treamtnet is enzyme replacement with enzyme from pooled human plasma
tx: prevent smoking, supportive treatment for lungs
Which of the following is not commonly associated with pulmonary hypoplasia?
a) CCAM (now known as CPAM)
b) polyhydramnios
c) thoracic distrophy
d) CDH
e) pleural effusions with fetal hydrous
b) in fact the opposite, oligohydramnios (associated with for exam fetal renal insufficiency or PROM) can lead to diminished air grown
the other causes are associated (1783) usually secondary to other intrauterine disorders that produce impairment of lung development
bilateral - oligohydramnios, thoracic dystrophy (ie spine and rib cage deformities)
unilateral - CDH and CCAM can displace the mediastinum and produce hypoplasia
What is the blood supply to a pulmonary sequestration?
systemic artery supplies the blood to a pulmonary sequestration
Pulmonary sequestration -
What are causes of false positive sweat chloride
adrenal insufficiency eczema ectoderma dysplasia nephrogenic DI hypothyroidism, hypoparathyroidism, panhypopit, pseudohypoaldosteronism Dehydration Malnutrition poor technique Type i glycogen storage disease, fucosidosis, mucopolysaccharidosis PGE administration
What are causes of false negative sweat chloride
edema
poor technique/inadequate sweat collection
atypical CF (very are)
Treatment of CF
Target:
Rep - chest physio, antibiotics to control infections/monitor the bugs with sputum cultures
GI - exocrine pancreatic insufficiency - treat with pancreatic enzymes (lipase/proteases), high calorie diets, don’t withhold fat, give fat soluble vitamins (ADEK)
Meconium ileus - may need surgical intervention, sometimes enemas
DIOS - often the cause of obstruction beyond the neonatal period
CF related diabetes - insulin to improve nutrition and prevent dehydration
Rare manifestation - liver diseae - progressive dirrhoses with postal hypertension
MULTI D team
associated conditions with congenital hypoventilation syndrome
Hirschprung disease
neural crest tumours (ie neuroblastoma)
