Peds Lung Disease Flashcards
Resp Distress Syndrome
- aka
- cause
- MC in who?
- when is sufficient surfactant produced to sustain life?
aka: hyaline membrane dz
cause: insufficient surfactant along with immature lungs.
MC in premature infants, major cause of morbidity and mortality in infants born prior to 30wks gestation.
surfactant becomes functional at 28wks, viable in adequate amounts at 34wks.
What part of the lungs are being developed during the canalicular and saccular stages of development?
How many weeks gestation does surfactant become detectable in amniotic fluid?
Canalicular stage: 16-26wks, resp bronchioles are being formed.
Saccular stage: 24-38, alveolar ducts are formed.
Surfactant becomes detectable in amniotic fluid at 24weeks gestation.
Describe the vicious cycle that prematurity and insufficient surfactant preludes.
Prematurity leads to surfactant deficiency and therefore alvoelar collapse. This collapse promotes intrapulmonary shunting leading to hypoxemia and acidosis.
RDS
- onset
- CXR findings
- PE findings
- ABGS
- labs
- clinical course
Onset: presents in 1st minutes/hrs of life. severity of resp failure increases during the first 2-3days of life
- ground glass appearance, air bronchograms, elevation of diaphragm
- lungs appear solid and congested with diffuse atelectasis
PE findings:
- tachypnea
- subcostal and sternal retractions
- grunting
- cyanosis
ABGs:
- hypoexmia
- hypercarbia
- metabolic acidosis
Labs:
-hyponatremia from H20 retention
Prognosis:
-infants greater than 32 wks usually improve by 1 wk of life.
-infants less than 28-26wks course is usually prolonged and complicated volutrauma, barotrauma PDA, infection, intraventricular hemorrhage.
RDS
- tx
- prevention
Tx:
- Oxygen, CPAP, mechanical ventillation
- proper thermal, metabolic, and nutritional support
Prevention:
- prevent premature delivery
- antenatal corticosteroids
SIDS:
- what is this?
- peak incidence at how many months?
- MC occurrence before how many months old?
What: sudden unexpected death of infant less than 1 year of age
Peak incidence at 3mo
MC occurrence is before 9mo old. (95%)
What is the most important preventable risk factor for SIDS?
When is it okay to use a pacifier?
maternal smoking
Ok to use pacifier at night while sleeping until 12 mo. Okay to use pacifier throughout day until 6 mo.
SIDS:
-what sleep position puts infant at risk of hypercapnia?
-what is the recommended sleeping position for SIDS prevention?
sleeping in prone position puts infant at risk of hypercapnia
Recommended sleeping position is supine :) NO blankets or anything in the crib with the infant, EVER. Firm flat sleep surface is preferred.
-avoid overheating, bedroom temperatures should be comfortable for lightly clothed adult.
Cystic Fibrosis: -what is this? -predicted lifespan -What gene encodes for CF? -
What: syndrome of chronic sinopulmonary infections, malabsorption, and nutritional abnormalities. Deranged chloride channels, thick viscous secretions.
Lifespan of 37years
Gene CFTR (CF transmembrance conductance regulator)
Organ dysfunction in CF?
- sinuses: sinusitis, nasal polyps
- lung; endobronchitis, bronchiectasis
- pancreas: exocrine insufficiency, CF related diabetes
- intestines: meconium ileus, constipation/DIOS (Distal intestinal obstruction syndrome)
- Liver: focal sclerosis
- Vas Deferens: failure to develop
- Sweat gland: salt losing dehydration
CF related complications
- steatorrhea*
- intussusception (currant jelly stools)*
- rectal prolapse*
- male infertility*
- stunting,wasting
- airway infection
- DM
- sinusitis
CF Genetics
- inheritance?
- chromosome involved
- dx
Autosomal recessive inheritance
Chromosome 7
Dx:
- clinical
- newborn screening: sweat electrolytes, mutations, meconium ileus, delayed emptying
- CFTR dysfunction (sweat chloride)
At what chloride sweat levels is:
- CF diagnosis confirmed
- possible CF
- CF very unlikely
Dx confirmed: greater than 60mmol/L
Possible CF: 30-59mmol/L
CF very unlikely: less than 29mmol/L
Describe End Stage Lung Dz in CF
- bronchiectasis
- infection
- inflammation
- mucous plugging
- severe airflow obstruction.
*end up passing away from lung dz if dont get lung transplant.
What bacteria are most CF pts colonized with during end stage lung dz?
Pseudomonas aeruginosa
