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38 Cards in this Set

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What is the significance of premature membrane rupture during pregnancy?
Already infected with pathogen or provides a clear pathway for infection (transcervical/ascending infection)
What is an Apgar score?

5 parameters?

What does the one-min score predict? 5-min score?
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
What are 4 risk factors for PPROM (preterm premature rupture of plancental membranes)?

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

Complication: infection
Define the following terms:

Chorioamnionitis
Funisitis
chorioamnionitis: inflammation of placental membranes

funisitis: inflammation of umbilical cord
LIst 2 placental factors for fetal growth restriction (intrauterine growth retardation).

3 fetal factors?

2 maternal factors?
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
What is the most common cause of respiratory distress in the neonates?
Hyaline membrane disease (neonatal respiratory distress syndrome)
What is the etiology and pathogenesis of neonatal RDS (hyaline membrane disease)?
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)
List 4 factors predipose an infant to HMD (hyaline membrane disease).
1. Prematurity - surfactant production about 35wks gestation
2. Male sex
3. Maternal DM - high insulin antagonizes corticosteroids, which induces surfactant formation
4. Cesarean birth
Describe the anatomic lesions of HMD.
damage to alveolocapillary membrane --> fibrongen leakage and type II pneumocyte necrosis --> fibrin + cell debris visualized as hyaline membrane
What are clinical signs and symptoms of HMD?
dyspnea and cyanosis within 30 mins after birth

Progressive signs of respiratory difficulty: tachypnea, grunting, nasal flaring, intercostal and substernal retractions, and cyanosis
How can HMD be prevented? (2)

How do you measure lung maturity? (2)
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.
What is the classic treatment for HMD? modern treatment?
Classic treatment: supportive
1. O2 + high PRESSURE mechanical ventilation

Modern treatment
1. O2 + high-FREQUENCY mechanical ventilation (HFV)
2. aerosolized exogenous surfactant.
What are complications of HMD and its treatment?

Describe each.
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.
Discuss the pathogenesis of retrolental fibroplasia.
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
List 2 complications of prematurity other than HMD.
1. necrotizing enterocolitis (NEC)
2. Intracerebral hemorrhage
What is the pathogenesis of necrotizing enterocolitis (NEC)?

Complications of NEC?
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
Where are 2 common places of hemorrhage in neonetal intracerebral hemorrage?
Germinal matrix hemorrange.

Periventricular hemorrhage extending to intraventricular spaces.
What are 4 consequences of intracerebral hemorrhage?
1. increased intracranial pressure
2. damage to brain substance
3. herniation of brain into foramen magnum
4. fatal depression of vital medullary centers
What is the most common cause of an intra-abdominal mass in an infant?

What other differential diagnoses can be made?
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
What are tumor markers are present in urine and blood in neuroblastoma patients?
blood - catecholamines

Urine - catecholamine metabolites (VMA and HVA)
How is ganglion neuroblastoma different from neuroblastoma?

Which one is less aggressive? why?

What is a "favorable" histological feature in ganglionneuroblastoma?
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
List favorable (5) and unfavorable (5) prognostic features of neuroblastoma.
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
What are 3 distinct histological features of Wilms tumor (nephroblastoma) that differentiate it from neuroblastoma?
1. blastema - embryonal cells
2. epithelium: abortive glomeruli and tubule
3. stroma - spindle cells
What constitutes unfavorable histology in Wilms tumor?
Anaplasia
1. large, hyperchromatic, pleomorphic nuclei
2. mitoses
Where do malignant neoplasms rank with regard to causes of death in children?
early childhood (1-4 yrs): 3rd

Later childhood (5-14 yrs): 2nd
Where are 2 most frequent sites of primary malignancy in children?
Hematopoietic system and Nervous system
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?
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)
This is CXR of an infant.

What do you see?

Most common cause of this?
Common cause - Hyaline Membrane disease
This is a slide of an infant lung.

ID the upper arrows and lower arrows.

diagnosis?
Upper arrows - hyaline membranee

Lower arrows: atelectatic alveoli

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

ID the upper and lower arrows.
Upper arrow - residual fibrosis

Lower arrow - hyperlucency (darker) indicates alveolar hypoplasia
This CXR indicates pneumopericardium of an infant.

What syndrome is this?
What is a possible cause of this?
Pulmonary air-leak syndromes.

Caused by high pressure mechanical ventilation (treatment of HMD).
This is a CT scan in the region of the left kidney.

What do the darker areas indicate in the tumor?

Its significance?
indicates necrosis and hemorrhage.

Suggests rapidly growing and malignant.
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?
Neuroblastoma

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

Is there necrosis? If so, what kind?
Yes

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

ID the arrows.
Ganglioneuroblastoma
Top - Schwannian storma
middle - ganglion cells
bottom - residual neuroblasts

Ganglioneuroma
top - schwannian stroma
bottom - ganglion cells
ID each picture as either neuroblastoma or Wilms tumor
Left - Neuroblastoma
Middle and Right - Wilms tumor (nephroblastoma)
These are pictures of Wilms tumor.

Which one has favorable histology. How do you know?
Left one.

The right one has cells with properties of anaplasia: Large, hyperchromatic, and plemorphic nuclei and mitoses.
These are slides of Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma.

ID each picture.

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