Perinatal pathology

Question 1

causes of death, under 1 year of age

Answer 1

disorders related to short gestation and low birth weight
SIDS
respiratory distress of newborn
bacterial sepsis of newborn- gbs, g neg infxns like ecoli, intrauterine hypoxia,

Question 2

causes of death 1-4 years of age

Answer 2

accidents
congenital malformations
malignant neoplasms
homicide
disease of heart 
influenza and pneumonia

Question 3

fetal causes of prematurity and growth restriction

Answer 3

chromosomal abnormalities- XO- turner’s
congenital anomalies- potter sequence
congenital TORCH infxns

placental causes-cord abnormalites, placental infarction, abruption

maternal causes- smoking, drugs, etoh

Question 4

Neonatal resp distress syndrome

Answer 4

dec alveolar surfactant, atelectasis–> hypoxemia and CO2 retention–> acisosis–> pulmonary vasoconstriction and hypoperfusion–> endothelial and epithelial damage–> fibrin + necrotic cells (hyaline membrane) –> hypoxemia + co2 retention

Question 5

bronchopulmonary dysplasia

Answer 5

complication of hyaline membrane disease- squamous metaplasia, peri-bronchial and interstitial fibrosis, decrease in alveolar septation
relatively rare due to artificial surfactant and improvements in NICU

Question 6

necrotizing enterocolitis

Answer 6

feeding intolerance, abdominal distention and bloody stools after 8-10 days of age

morphology- pneumatosis intestinalis, portal venous gas

mortality 20-30%

7% prevalence among infants with 500-1500 g birth weight

enteral feeding and introduction of bacteria–> tissue destruction and inflammation

Question 7

SIDS

Answer 7

peaks at 2-4 months
occurs during sleep (crib death)

triple risk model: underlying vulnerability- male sex, prematurity, native american, genetic polymorphisms in serotonin transporter proteins
critical developmental period (sleep-wake transitions)
extrinsic or exogenous risk factors- exposure to smoking, sleeping on stomach, soft bedding, infxn

Question 8

TORCH infxn

Answer 8

toxoplasma, rubella, cmv, herpes

syphilis and HIV

Question 9

Perinatal sepsis

Answer 9

group b strep from vaginal flora

polymicrobial sepsis (more than 1 organism from blood culture) in a child should be soncidered possible child abuse- intentional injection of foreign material into child

Question 10

pediatric tumors vs adult tumors

Answer 10

mesenchymal vs epithelial derivation
malignant tumors have primitive histologic appearance
abnormal embryogenesis
spontaneous regression or cytodifferentiation
familial or genetic pattern

Question 11

0-4 years tumors

Answer 11

leukemia
retinoblastoma
neuroblastoma
wilms tumor 
hepatoblastoma
soft tissue sarcoma (rhabdomyosarcoma) 
teratomas
cns tumor

Question 12

5-6 years tumors

Answer 12

leukemia
retinoblastoma
neuroblastoma
hepatocellular carcinoma
soft tissue sarcoma
cns tumors
ewing sarcoma
lymphoma

Question 13

10-14 years tumors

Answer 13

hepatocellular carcinoma
soft tissue
osteogenic sarcoma
thyroid carcinoma
hodgkin's disease

Question 14

most common tumor of infancy

Answer 14

hemangioma
derived from blood vessels
skin-face and scalp
spontaneous regression
can be associated with von hippel lindau syndrome

Question 15

teratomas

Answer 15

mature, immature, malignant

from pluripotent germ cells

Question 16

risk factors of canncer

Answer 16

primitive histologic appearance
exposures of radiation and benzene for mother
genetic- inherited cancer syndromes
immunosuppression

Question 17

leukemia

Answer 17

most common childhood cancer
ALL and AML 
less than 4- ALL
teenager- AML (auer rods)
symptoms- anemia, bleeding, fever, weightloss

Question 18

cns tumors

Answer 18

second most common cancer
cell of origin- can be glial
involve cerebellum or brains tem
inc intracranial pressure, headaches, nausea,

Question 19

neuroblastoma

Answer 19

most common extracranial solid tumor of childhood
ncc origin
parasympathetic ganglia or adrenal medulla

large abdominal masses in younger children
pseudorosettes, dense core granules

Question 20

neuroblastoma prognostic factors

Answer 20

prognostic factors: stage, age, n-myc, chr 17q gain

Question 21

Wilms tumor

Answer 21

most common renal tumor

abdominal flank mass, may have pain, fever

Question 22

4 syndromes for wilm’s tumor

Answer 22

WAGR syndrome
Denys-drash syndrome
beckwith wiedemann syndrome

Question 23

wagr syndrome

Answer 23

germline deletion of 11p13

aniridia (absence of iris), genital anomalies, mental retardation

Question 24

denys-drash syndrome

Answer 24

dominant neg missense mutations of WT1

gonadal dysgenesis, nephropathy

Question 25

beckwith-wiedemann syndrome

Answer 25

loss of imprinting and overexpression of IGF-2- organomegaly, macrosomia, hemihypertrophy, omphalocele, adrenal cytomegaly

Question 26

retinoblastoma

Answer 26

malignant tumor of one or both eyes
from retinal cells
40% inherited mutation of Rb
occurs in children <4

Question 27

Hodgkin’s lymphoma

Answer 27

childrena nd adults
derived from wbcs
reed sternberg cells are pathognomonic
can have nodular sclerosis

Question 28

rhabdomyosarcoma

Answer 28

most common soft tissue sarcoma
from embryonic skeletal muscle cells
symptoms depend on site of origin

Question 29

secondary malignant neoplasms

Answer 29

bone, soft tissue, cns, thyroid, leukemias, lymphomas

high risk of secondary primary tumor (>10%) in lymphomas, soft tissue sarcomas, ewing’s sarcoma