Perinatal Pathology

Question 1

• disorders related to short gestation and low birth weight
• SIDS
• respiratory distress
• bacterial sepsis

Answer 1

causes of death under 1 yr of age

Question 2

• accidents
• congenital malformations
• malignant neoplasms
• homicide
• diseases of heart
• influenze, pneumonia

Answer 2

causes of death, 1-4 years of age

Question 3

• preterm, premature rupture of membranes prior to 37 WGA
• intrauterine infection
• structural abnormalieis of uterus, placenta
• multiple gestations

Answer 3

causes of prematurity and growth restriction

Question 4

• chromosomal abnormalities
• congenital anomalies (Potter sequence)
• TORCH infections

Answer 4

fetal causes of prematurity and growth restriction

Question 5

• cord abnormalities

- placental infarction, abruption

Answer 5

placental causes of prematurity

Question 6

• leading cause of resp distress in newborns, now decreased because of artificial surfactant
• exofurg, infasurf, survanta
• increased alveolar surface tension, atelectasis, hypoxemia and CO2 retention
• acidosis, endothelial and epithelial damage
• hyaline membranes

Answer 6

neonatal respiratory distress syndrome

Question 7

• complication of treatment of hyaline membrane disease

- squamous metaplasia, peribrochial and interstitial fibrosis, decrease in alveolar septation

Answer 7

bronchopulmonary dysplasia

Question 8

• feeding intolerance, abdominal distention and blood stools after 8-10 days of age
• pneumatosis intestinalis, portal venous gas

Answer 8

necrotizing enterocolitis

Question 9

• peaks at 2-4 months of age
• male predominance
• occurs during sleep period, Japan lowest and New Zealand highest
• rates in native americans and African americans are 2-7X higher
• history of mild upper respiratory tract infections, presumably viral

Answer 9

SIDS

Question 10

• petechial hemorrhages most consistent finding, but they are agonal events
• triple risk model: underlying vulnerability, critical development period, extrinsic risks (smoking, sleeping prone, mild infection)

Answer 10

SIDS

Question 11

-toxoplasma, rubella, CMV, herpes simplex, syphilis and HIV

Answer 11

TORCH infections

Question 12

• due to group B strep, positive cultures an indication for C section
• more than one organism consered child abuse, Muchausen by proxy

Answer 12

perinatal sepsis

Question 13

• mesencyhmal derivation
• often have primitive histo appearance
• abnormal embryogenesis common
• spontaneous regression and cytodifferentiation common
• familial or genetic pattern

Answer 13

pediatric tumors

Question 14

• most common tumor of infancy
• derived from blood vesels
• skin- face, scalp
• spontaneous regression
• associated with VHL syndrome

Answer 14

hemangiomas

Question 15

• mature, immature, malignant
• derived from pluripotent germ cells
• females > males
• sacrococcygeal the most common
• risk of malignant transformation is increased after 2 months of age

Answer 15

teratomas

Question 16

• most common childhood cancer
• derived from immature white blood cells
• rapid onset
• anemia, bleeding, fever, weight loss

Answer 16

leukemia

Question 17

-second most common cancer in children
-cell of origin variable, glial
-most involve cerebellum or brainstem
-increased intracranial pressure, headache, nausea, vomiting, blurred vision, dizzy
-

Answer 17

central nervous system cancers

Question 18

• derived from neuroectoderm
• 20% of brain tumors in children
• 17p deletion

Answer 18

medulloblastoma

Question 19

• most common extracranial solid tumor of childhood
• neural crest origin
• parasympathetic ganglia or adrenal medulla
• large abdominal mass
• Homer wright pseudorosettes
• regress or cytodifferentiate
• skin papules
• N-myc, 17q, prognostic factors

Answer 19

neuroblastoma

Question 20

• most common renal tumor in children
• derived from pluripotent embryonal renal cells
• presents w/ abdominal flank mass, pain, fever
• risk increased with WAGR and Denys drash syndrome, Beckwith Wiedemann, loss of imprinting

Answer 20

Wilms tumor

Question 21

• germline deletion of 11p13

- aniridia, genital anomalies, mental retardation

Answer 21

WAGR syndrome

Question 22

• dominant negative missense mutation of WT1

- gonadal dysgenesis, nephorpathy

Answer 22

Denys Drash syndrome

Question 23

• malignant tumor of one or both eyes, derived from retinal cells
• occurs in children under the age of 4
• inherited mutation on chromosome 13
• prognosis excellent if no spread

Answer 23

retinoblastoma

Question 24

• dervied from white blood cells
• risks: age, gender, family history, past EBV infection, immunosuppression
• Reed Sternberg cells

Answer 24

Hodgkin’s disease

Question 25

- most common soft tissue sarcoma in children - derived from embryonic skeletal muscle cells (Rhabdomyoblasts) - head and neck, groin, abdomen, pelvis, arms, legs - stain positive for muscle intermediate filament markers (desmin, myogenin)

Answer 25

rhabdomyosarcoma

Question 26

bone and soft tissue sarcomas, CNS tumors, thyroid carcinomas, leukemias, lymphomas, have risk for?

Answer 26

secondary malignant neoplasms