08b: Pediatric Pathology, Risk/Benefit

Question 1

“Prematurity” is gestational age under (X) weeks and weight under (Y) grams/lb.

Answer 1

X = 37
Y = 2500 grams (5.5 lbs)

Question 2

T/F: Prematurity is the most common cause of neonatal mortality.

Answer 2

False - congenital anomalies are most common (prematurity is second)

Question 3

Which organ systems are most affected by prematurity?

Answer 3

Neuro, GI, Resp

Question 4

T/F: Twin pregnancy (multiple gestation) is risk factor for prematurity.

Answer 4

True

Question 5

Vaginal delivery provides compression on baby’s chest. Why would this be a good thing, especially to prevent (X)?

Answer 5

X = neonatal respiratory distress syndrome

Physical stress increases glucocorticoids (to allow surfactant development)

Question 6

Maternal medical history of (X) increases risk of neonatal RDS due to (high/low) levels of fetal (Y).

Answer 6

X = diabetes
High
Y = insulin

Question 7

List treatment options for neonatal RDS.

Answer 7

1. (Before birth) Maternal steroids
2. Artificial surfactant
3. Mechanical ventilation
4. O2 supplementation

Question 8

List some complications of oxygen therapy.

Answer 8

(RIB acronym)

1. Retrolental Fibroplasia
2. Intraventricular hemorrhage
3. Bronchopulmonary Dysplasia (BPD)***

Question 9

Clinical presentation of necrotizing enterocolitis:

Answer 9

1. Abdominal distension
2. Absent bowel sounds
3. Bloody stools
4. Possible perforation (air in abdomen)

Question 10

Predisposing factors for intraventricular hemorrhage in infant.

Answer 10

1. Prematurity
2. Hypoxia
3. Breech (butt first) delivery

Question 11

Clinical symptoms of intraventricular hemorrhage in infant.

Answer 11

1. Apnea
2. Lethargy
3. Poor muscle tone
4. Seizures

Question 12

T/F: Autopsy usually provides clear cause of death in SIDS.

Answer 12

False

Question 13

T/F: CF is the most common lethal genetic disease that affects Caucasians.

Answer 13

True

Question 14

CF primary defect is abnormal function of (X) channel on (Y) chromosome. What’s the most common mutation among Caucasians?

Answer 14

X = epithelial Cl (CFTR)
Y = 7

DeltaF(508)

Question 15

Meconium Ileus is a condition common in (X) disease. Briefly describe this condition.

Answer 15

X = CF

Thick, sticky meconium blocks ileus

Question 16

CF patients likely have which vitamin/mineral deficiencies?

Answer 16

Fat-soluble vitamins (ADEK)

Question 17

Microscopically, Bronchopulmonary Dysplasia (BPD) shows (collapsed/dilated) alveoli, airway (hypo/hyper)-plasia, alveolar wall (thinning/thickening) and (presence/absence) of fibrosis.

Answer 17

Dilated;
Hyperplasia (and squamous metaplasia)
Thickening;
Presence

Question 18

The major abnormality in Bronchopulmonary Dysplasia (BPD) is:

Answer 18

Alveolar hypoplasia (decrease number of alveoli)

Question 19

Microscopically, Neonatal RDS shows (collapsed/dilated) alveoli and (X)-rich (Y) in the alveolar spaces.

Answer 19

Collapsed;
X = protein/fibrin
Y = exudate

Question 20

Among environmental factors, which are the most important modifiable risk factors for
SIDS?

Answer 20

1. Sleeping prone and on soft surfaces

2. Thermal stress

Question 21

List the two most common causes of infant sudden “unexpected” death.

Answer 21

1. Infection

2. Unsuspected congenital anomalies

Question 22

List two advantages of RCTs.

Answer 22

1. Estimate absolute and relative risk

2. Determine causality

Question 23

List two disadvantages of RCTs.

Answer 23

1. Expensive

2. Not feasible for low-incidence outcomes

Question 24

T/F: Generalizability of findings is not a limitation of RCTs.

Answer 24

False

Question 25

Relative risk equation for groups p1 v p2. And absolute risk reduction?

Answer 25

RR: p1/p2

Question 26

Absolute risk reduction equation for groups p1 v p2. And NNT (number needed to treat) equation?

Answer 26

ARR: p1-p2 NNT: 1/ARR

Question 27

T/F: In case-control studies, it isn't possible to determine absolute risk.

Answer 27

True

Question 28

Drug is given to those at higher risk of outcome, so it appears that drug causes that outcome. What kind of bias?

Answer 28

Indication bias

Question 29

Case-control study: if (X) group much larger than (Y) group, the odds ratio can be an approximation of (Z).

Answer 29

``` X = control (no effect) Y = case (effect) Z = RR ```

Question 30

Which cytokine do (X) leukocytes produce to kill multicellular parasites?

Answer 30

X = eosinophils Major Basic Protein

Question 31

Eosinophilia can result from which condition(s)?

Answer 31

(NAAACP acronym) 1. Neoplasm 2. Allergic diseases 3. Asthma 4. Addison's 5. Collagen Vascular Disease 6. Parasites

Question 32

Eosinophilic Granulomatosis with Polyangiitis: 95% of the time starts with (X) condition. The disease is essentially inflammation of (Y).

Answer 32

``` X = asthma Y = small and medium-sized arteries (vasculitis) ```

Question 33

Eosinophilic Granulomatosis with Polyangiitis: positive lab results for (X) Ab.

Answer 33

X = P-ANCA (anti-neutrophil cytoplasmic Ab)

Question 34

Eosinophilic Granulomatosis with Polyangiitis: treatment options include...

Answer 34

1. Corticosteroids (low-dose for life) | 2. Cyclophosphamide

Question 35

Cyclophosphamide is primarily a(n) (X) agent with (Y) properties that allow its use for Eosinophilic Granulomatosis with Polyangiitis.

Answer 35

``` X = anti-neoplastic Y = immunosuppressive ```

Question 36

Cyclophosphamide must be activated to (X) by (Y) enzyme.

Answer 36

``` X = 4-hydroxy-cyclophosphamide Y = liver cytochrome p450 ```

Question 37

T/F: Cyclophosphamide has wide utility (multiple cancers and for immunosuppressive effects).

Answer 37

True