Gen Path: Pediatric Pathology Sg Studies (35)

Front 1

What is the significance of premature membrane rupture during pregnancy?

Back 1

Already infected with pathogen or provides a clear pathway for infection (transcervical/ascending infection)

Front 2

What is an Apgar score?

5 parameters?

What does the one-min score predict? 5-min score?

Back 2

A clinically useful method of evaluating the physiolgic condition and responsiveness of a newborn infant, and hence her/his chances of survival.

Apperance (color)
Pulse (HR)
Grimace (response to nasal catheter)
Activity (muscle tone)
Respiratory effort

1min score - immediate action to be taken

5min score - one month mortality

Front 3

What are 4 risk factors for PPROM (preterm premature rupture of plancental membranes)?

Complications of PPROM?

Back 3

1. maternal smoking
2. prior preterm delivery
3. vaginal bleeding during pregnancy
4. poor maternal nutrition

Complication: infection

Front 4

Define the following terms:

Chorioamnionitis
Funisitis

Back 4

chorioamnionitis: inflammation of placental membranes

funisitis: inflammation of umbilical cord

Front 5

LIst 2 placental factors for fetal growth restriction (intrauterine growth retardation).

3 fetal factors?

2 maternal factors?

Back 5

placental factors - uteroplacental insufficiency, confined placental mosaicism.

Fetal factors - chromosomal disorders, congeital anomalies, congental infections (esp. TORCH group)

Maternal factors - decreased placental blood flow (most commonly associated SGA) and maternal malnutrition

Front 6

What is the most common cause of respiratory distress in the neonates?

Back 6

Hyaline membrane disease (neonatal respiratory distress syndrome)

Front 7

What is the etiology and pathogenesis of neonatal RDS (hyaline membrane disease)?

Back 7

etiology - pulmonary immaturity

pathogenesis - surfactant deficiency --> increased alveolar surface tension --> atelectasis(lack of gas exchange in the alveoli)--> uneven perfusion and hypoventilation --> --> pulmonary vasoconstriction and hypoperfusion --> endothelial and epithelial damage --> fibrin + necrotic cells (hyaline membrane formation)

Front 8

List 4 factors predipose an infant to HMD (hyaline membrane disease).

Back 8

1. Prematurity - surfactant production about 35wks gestation
2. Male sex
3. Maternal DM - high insulin antagonizes corticosteroids, which induces surfactant formation
4. Cesarean birth

Front 9

Describe the anatomic lesions of HMD.

Back 9

damage to alveolocapillary membrane --> fibrongen leakage and type II pneumocyte necrosis --> fibrin + cell debris visualized as hyaline membrane

Front 10

What are clinical signs and symptoms of HMD?

Back 10

dyspnea and cyanosis within 30 mins after birth

Progressive signs of respiratory difficulty: tachypnea, grunting, nasal flaring, intercostal and substernal retractions, and cyanosis

Front 11

How can HMD be prevented? (2)

How do you measure lung maturity? (2)

Back 11

1. delay delivery
2. maternal administration of corticosteroids --> production of surfactants

Measure lung maturity via amniotic fluid analysis of:
1. Lecithin:sphingomyelin (L:S); L:S ratio ≥2 preferred
2. Phosphatidylglycerol (PG) level; PG presence indicates lung maturity.

Front 12

What is the classic treatment for HMD? modern treatment?

Back 12

Classic treatment: supportive
1. O2 + high PRESSURE mechanical ventilation

Modern treatment
1. O2 + high-FREQUENCY mechanical ventilation (HFV)
2. aerosolized exogenous surfactant.

Front 13

What are complications of HMD and its treatment?

Describe each.

Back 13

1. Bronchopulmonary dysplasia (BPD) - alveolar hypoplasia, alveolar wall fibrosis, and arrest of pulmonary septation

2. Retrolental fibroplasia (retinopathy of prematurity) - endothelial cell apoptosis and eventaully retinal detachment and blindness

3. pulmonary air-leak syndromes

4. patent ductus arteriosus (PDA) - hypoxia --> pulmonary arterial vasoconstriction --> R to L shunting through PDA.

Front 14

Discuss the pathogenesis of retrolental fibroplasia.

Back 14

high O2 levels -->
decreased VEGF expression -->
endothelial cell apoptosis

return to room air --> increased VEGF expression --> neovascularization and fibrosis --> growth of new vessels and fibroblasts into vitreous --> fibrovascular tissue contraction --> retinal detachment --> blindness

Front 15

List 2 complications of prematurity other than HMD.

Back 15

1. necrotizing enterocolitis (NEC)
2. Intracerebral hemorrhage

Front 16

What is the pathogenesis of necrotizing enterocolitis (NEC)?

Complications of NEC?

Back 16

hypoxia --> intestinal ischemia (prerequisite) + intro of bacteria from feeding + inflammatory mediators (esp. PAF) --> transmural intesttinal inflammation with gas formation by bacteria

complications
1. high perinatal mortality
2. survivors: fibrosis --> post-NEC strictures

Front 17

Where are 2 common places of hemorrhage in neonetal intracerebral hemorrage?

Back 17

Germinal matrix hemorrange.

Periventricular hemorrhage extending to intraventricular spaces.

Front 18

What are 4 consequences of intracerebral hemorrhage?

Back 18

1. increased intracranial pressure
2. damage to brain substance
3. herniation of brain into foramen magnum
4. fatal depression of vital medullary centers

Front 19

What is the most common cause of an intra-abdominal mass in an infant?

What other differential diagnoses can be made?

Back 19

unilateral utereopelvic junction obstruction resulting in an enlarged hydronephrotic kidney

Cysts in kidneys or pancreas

Benign neoplasms - mature teratoma, lymphagioma

Malignant neoplasms
neuroblastoma
Wilms tumor (nephroblastoma)
hepatoblastoma
immature teratoma
lymphoma

Front 20

What are tumor markers are present in urine and blood in neuroblastoma patients?

Back 20

blood - catecholamines

Urine - catecholamine metabolites (VMA and HVA)

Front 21

How is ganglion neuroblastoma different from neuroblastoma?

Which one is less aggressive? why?

What is a "favorable" histological feature in ganglionneuroblastoma?

Back 21

Ganglion neuroblastoma is more differentiated.

Ganglion neuroblastoma is less aggressive and has the potential to differentiate and become benign.

Presence of schwannian stroma indicates more favorable histology

Front 22

List favorable (5) and unfavorable (5) prognostic features of neuroblastoma.

Back 22

favorable
1. low stage
2. age ≤ 18 months
3. favorable histology (more schwannian stroma)
4. N-MYC not amplified
5. hyperdiploid*

Unfavorable
1. high stage
2. age > 18 months
3. unfavorable histology
4. N-MYC amplified
5. near-diploid

Front 23

What are 3 distinct histological features of Wilms tumor (nephroblastoma) that differentiate it from neuroblastoma?

Back 23

1. blastema - embryonal cells
2. epithelium: abortive glomeruli and tubule
3. stroma - spindle cells

Front 24

What constitutes unfavorable histology in Wilms tumor?

Back 24

Anaplasia
1. large, hyperchromatic, pleomorphic nuclei
2. mitoses

Front 25

Where do malignant neoplasms rank with regard to causes of death in children?

Back 25

early childhood (1-4 yrs): 3rd

Later childhood (5-14 yrs): 2nd

Front 26

Where are 2 most frequent sites of primary malignancy in children?

Back 26

Hematopoietic system and Nervous system

Front 27

What is most common neoplasm of infancy?

Most common childhood malignancy?

Most common cause of cancer death in childhood?

3 most common solid malignancies in childhood?

Back 27

Most common neoplasm of infancy: HEMANGIOMA (benign tumor of endothelial cells)

Most common childhood malignancy: LEUKEMIA

Most common cause of cancer death in childhood: LEUKEMIA

Three most common solid malignancies in childhood
BRAIN TUMORS (collectively)
NEUROBLASTOMA
WILMS TUMOR (NEPHROBLASTOMA)

Front 28

This is CXR of an infant.

What do you see?

Most common cause of this?

Back 28

Common cause - Hyaline Membrane disease

Front 29

This is a slide of an infant lung.

ID the upper arrows and lower arrows.

diagnosis?

Back 29

Upper arrows - hyaline membranee

Lower arrows: atelectatic alveoli

Hyaline Membrane Disease

Front 30

This is a CXR of an infant with HMD that developed complications from mechanical ventilation treatment.

ID the upper and lower arrows.

Back 30

Upper arrow - residual fibrosis

Lower arrow - hyperlucency (darker) indicates alveolar hypoplasia

Front 31

This CXR indicates pneumopericardium of an infant.

What syndrome is this?
What is a possible cause of this?

Back 31

Pulmonary air-leak syndromes.

Caused by high pressure mechanical ventilation (treatment of HMD).

Front 32

This is a CT scan in the region of the left kidney.

What do the darker areas indicate in the tumor?

Its significance?

Back 32

indicates necrosis and hemorrhage.

Suggests rapidly growing and malignant.

Front 33

This is a microscopic section of an intra-abdominal mass(near the kidney region) in an infant.

What is the Dx? How do you know?

Back 33

Neuroblastoma

Must be malignancy of kidney or adrenal gland.
aplastic and No triphasic histology --> neuroblastoma

Front 34

This is a section of neuroblastoma

Is there necrosis? If so, what kind?

Back 34

Yes

Coagulative necrosis.

Front 35

The left picture is a slide of ganglioneuroblastoma and the right picture is a slide of ganglioneuroma.

ID the arrows.

Back 35

Ganglioneuroblastoma
Top - Schwannian storma
middle - ganglion cells
bottom - residual neuroblasts

Ganglioneuroma
top - schwannian stroma
bottom - ganglion cells

Front 36

ID each picture as either neuroblastoma or Wilms tumor

Back 36

Left - Neuroblastoma
Middle and Right - Wilms tumor (nephroblastoma)

Front 37

These are pictures of Wilms tumor.

Which one has favorable histology. How do you know?

Back 37

Left one.

The right one has cells with properties of anaplasia: Large, hyperchromatic, and plemorphic nuclei and mitoses.

Front 38

These are slides of Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma.

ID each picture.

Which one is most favorable? Least favorable? How do you know?

Back 38

Left - ganglioneuroblastoma

Middle - ganglioneuroma

Right - neuroblastoma

Ganglioneuroma (middle) is most favorable b/c there is no aplasia (benign) and lots of Schwannian stroma (more = favorable)

Ganglioneuroblastoma is more favorable than neuroblastoma b/c there is some Schwannian stroma.