SFP: prematurity, CF, hydrops

Question 1

What is considered preterm?

Answer 1

Prior to 37 weeks

Question 2

What are some causes of prematurity?

Answer 2

Multiple gestation, rupture of membranes, chorioamnionitis

Question 3

What is considered post term?

Answer 3

After 42 weeks

Question 4

What is considered small for gestational age?

Answer 4

Less than 2500 g or weighing less than 10th percentile

Question 5

What is considered large for gestational age?

Answer 5

More than 4000-4500g at term, otherwise larger than 90th percentile

Question 6

What tends to be true of babies with diabetic mothers?

Answer 6

They tend to be bigger

Question 7

What is respiratory distress syndrome?

Answer 7

Decreased surfactant leads to collapsed alveoli unable to properly expand

Question 8

What are factors associated with increased risk for respiratory distress syndrome?

Answer 8

Male baby, maternal diabetes, c section

Question 9

What does insulin do to surfactant production?

Answer 9

Suppresses it

Question 10

What do steroids do to surfactant production?

Answer 10

Increase it

Question 11

Describe the formation of hyaline membranes.

Answer 11

1. Low surfactant causes hypoxemia and CO2 retention due to ventilation issues.
2. High CO2 leads to respiratory acidosis.
3. Acidosis causes pulmonary vasoconstriction and hypoperfusion.
4. Endothelial and epithelial damage result, leading to plasma fluid leakage into alveoli.
5. Fibrin and necrotic cells from the plasma combine to make hyaline membranes.

Question 12

What causes ‘ground glass’ lung appearance and white out on x-ray?

Answer 12

Respiratory distress syndrome

Question 13

What is a big complication of respiratory distress syndrome?

Answer 13

Interstitial emphysema from trying to force too much air into the lungs with external ventilation

Question 14

What is bronchopulmonary dysplasia?

Answer 14

Alveolar hypoplasia; the number of mature alveoli is decreased

Question 15

What is retinopathy of prematurity?

Answer 15

Increased oxygen from external ventilation leads to lower VEGF production, leading to less blood vessel proliferation. Oxygen weans off a bit, and VEGF returns to normal. This abnormal cycle leads to proliferation of poorly formed vessels, leading to aneurysms in the eyes.

Question 16

What is germinal matrix hemorrhage?

Answer 16

A lack of ability to regulate blood pressures leads to hemorrhage in the brain

Question 17

Describe grade I germinal matrix hemorrhage.

Answer 17

Bleeding is in the subependymal region. Prognosis is good!

Question 18

Describe grade II germinal matrix hemorrhage.

Answer 18

Bleeding extends to ventricles, typically filling less than half of it. Does not dilate the ventricles. Prognosis is still good.

Question 19

Describe grade III germinal matrix hemorrhage.

Answer 19

Bleeding extends into dilated ventricles; associated with 20% mortality.

Question 20

Describe grade IV germinal matrix hemorrhage.

Answer 20

Bleeding extends to the parenchyma; associated with 90% mortality.

Question 21

What is necrotizing enterocolitis associated with?

Answer 21

Prematurity, low birth weight, tube feeding

Question 22

What is necrotizing enterocolitis?

Answer 22

Infection and inflammatory mediators from underdeveloped immune response leads to necrosis of the bowel wall

Question 23

What are symptoms of necrotizing enterocolitis?

Answer 23

Bloody stools, bloating

Question 24

What is pathology of necrotizing enterocolitis?

Answer 24

Patchy full thickness necrosis of the bowel

Question 25

What is a complication of necrotizing enterocolitis?

Answer 25

Pneumatosis intestinalis aka air in the wall of the bowel; sign of perforation

Question 26

What are signs of meconium aspiration?

Answer 26

Foul smelling discolored amniotic fluid. May cause respiratory distress after delivery

Question 27

Why is meconium aspiration an issue?

Answer 27

Meconium is sterile, but it can cause obstruction of the airways

Question 28

What is the inheritance of CF?

Answer 28

Autosomal recessive

Question 29

What is the most common lethal genetic condition in white populations?

Answer 29

CF

Question 30

What is one of the most severe genetic mutations associated with CF?

Answer 30

deltaF508

Question 31

What does CFTR do?

Answer 31

Moves chloride ions to the outside of the cell, bringing water along with it

Question 32

What happens when CFTR doesn’t work?

Answer 32

Lack of chloride transport out of the cell results in lack of water transport, causing thick sticky mucus

Question 33

Describe CFTR in the skin.

Answer 33

It brings chloride into the cells from sweat glands instead of pushing it out, resulting in movement of water and sodium into the cells. When it doesn’t work, we have chloride, sodium, and water remaining in the lumen of sweat ducts and results in salty sweat.

Question 34

What airway infections are common in CF?

Answer 34

Pseudomonas and burkholderia cepacia

Question 35

How does CF impact the pancreas?

Answer 35

Leads to fibrosis that causes malabsorption due to impacted pancreatic enzyme secretion, diarrhea, and potentially rectal prolapse and edema

Question 36

How does CF impact the liver?

Answer 36

Leads to fatty, plugged ducts and cirrhosis

Question 37

How does CF impact the small intestine?

Answer 37

Leads to meconium ileus

Question 38

How do we test for CF?

Answer 38

1. Start with immunoreactive trypsinogen screening in newborn. 2. Sweat chloride test. 3. Molecular genetic testing.

Question 39

What will sweat glands look like in CF?

Answer 39

Perfectly normal! No real skin manifestations

Question 40

What is bronchiectasis?

Answer 40

Dilated, fixed bronchioles caused from chronic infection often from CF.

Question 41

What is hydrops?

Answer 41

Accumulation of edema within a fetus

Question 42

What used to be a big cause of immune fetal hydrops?

Answer 42

Rh and ABO incompatibility

Question 43

What are causes of non-immune fetal hydrops?

Answer 43

Cardiovascular defects causing heart failure, primary anemias (parvovirus), chromosomal anomalies, infections like CMV, syphilis, toxoplasmosis

Question 44

What is erythroblastosis fetalis?

Answer 44

Immature RBCs in the fetus associated with hydrops caused by anemia

Question 45

What is extramedullary hematopoiesis?

Answer 45

Development of RBCs outside the bone marrow associated with hydrops caused by anemia

Question 46

What is kernicterus?

Answer 46

Complication of hydrops caused by hemolysis that leads to bilirubin in the blood that deposits into the basal ganglia of the brain; leads to longterm developmental delay