Perinatal pathology Flashcards
causes of death, under 1 year of age
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,
causes of death 1-4 years of age
accidents congenital malformations malignant neoplasms homicide disease of heart influenza and pneumonia
fetal causes of prematurity and growth restriction
chromosomal abnormalities- XO- turner’s
congenital anomalies- potter sequence
congenital TORCH infxns
placental causes-cord abnormalites, placental infarction, abruption
maternal causes- smoking, drugs, etoh
Neonatal resp distress syndrome
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
bronchopulmonary dysplasia
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
necrotizing enterocolitis
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
SIDS
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
TORCH infxn
toxoplasma, rubella, cmv, herpes
syphilis and HIV
Perinatal sepsis
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
pediatric tumors vs adult tumors
mesenchymal vs epithelial derivation
malignant tumors have primitive histologic appearance
abnormal embryogenesis
spontaneous regression or cytodifferentiation
familial or genetic pattern
0-4 years tumors
leukemia retinoblastoma neuroblastoma wilms tumor hepatoblastoma soft tissue sarcoma (rhabdomyosarcoma) teratomas cns tumor
5-6 years tumors
leukemia retinoblastoma neuroblastoma hepatocellular carcinoma soft tissue sarcoma cns tumors ewing sarcoma lymphoma
10-14 years tumors
hepatocellular carcinoma soft tissue osteogenic sarcoma thyroid carcinoma hodgkin's disease
most common tumor of infancy
hemangioma derived from blood vessels skin-face and scalp spontaneous regression can be associated with von hippel lindau syndrome
teratomas
mature, immature, malignant
from pluripotent germ cells
risk factors of canncer
primitive histologic appearance
exposures of radiation and benzene for mother
genetic- inherited cancer syndromes
immunosuppression
leukemia
most common childhood cancer ALL and AML less than 4- ALL teenager- AML (auer rods) symptoms- anemia, bleeding, fever, weightloss
cns tumors
second most common cancer
cell of origin- can be glial
involve cerebellum or brains tem
inc intracranial pressure, headaches, nausea,
neuroblastoma
most common extracranial solid tumor of childhood
ncc origin
parasympathetic ganglia or adrenal medulla
large abdominal masses in younger children
pseudorosettes, dense core granules
neuroblastoma prognostic factors
prognostic factors: stage, age, n-myc, chr 17q gain
Wilms tumor
most common renal tumor
abdominal flank mass, may have pain, fever
4 syndromes for wilm’s tumor
WAGR syndrome
Denys-drash syndrome
beckwith wiedemann syndrome
wagr syndrome
germline deletion of 11p13
aniridia (absence of iris), genital anomalies, mental retardation
denys-drash syndrome
dominant neg missense mutations of WT1
gonadal dysgenesis, nephropathy
beckwith-wiedemann syndrome
loss of imprinting and overexpression of IGF-2- organomegaly, macrosomia, hemihypertrophy, omphalocele, adrenal cytomegaly
retinoblastoma
malignant tumor of one or both eyes
from retinal cells
40% inherited mutation of Rb
occurs in children <4
Hodgkin’s lymphoma
childrena nd adults
derived from wbcs
reed sternberg cells are pathognomonic
can have nodular sclerosis
rhabdomyosarcoma
most common soft tissue sarcoma
from embryonic skeletal muscle cells
symptoms depend on site of origin
secondary malignant neoplasms
bone, soft tissue, cns, thyroid, leukemias, lymphomas
high risk of secondary primary tumor (>10%) in lymphomas, soft tissue sarcomas, ewing’s sarcoma
