Pa14-Pediatric Pathology I

Front 1

What is pediatric pathology?

Back 1

A pathology subspecialty focusing on diseases of the fetus, placenta, infant, & child.

Front 2

What is a malformation?

Back 2

Abnormal embryogenesis; intrinsic abnormality

Front 3

What is a deformation?

Back 3

Ext forces secondarily deform tissues; mechanical forces mold normal developing tissue. Can infer magnitude & dir based on physical features

Front 4

What are disruptions? Causes?

Back 4

Extrinsic; secondary breakdown of normal tissue. Caused by vascular occulsion/hemorrahage, ischemia, radiation, infection, early amnion rupture. Eg amniotic bands

Front 5

What is a sequence?

Back 5

Set of anomalies due to a single problem. A single early developmental change w/ multiple secondary changes later; snowball effect. Eg oligohydramnios (Potter's seq)

Front 6

What is a syndrome?

Back 6

Set of anomalies that cannot be related to a single initiating defect. Freq etiologies are viral infection or specific chrom abnormalities. Eg Beckwith-Wiedmann syndrome

Front 7

What causes oligohydramnios? Effects?

Back 7

Renal agenesis, amniotic leak, or other (maternal hypertension)-> small lungs, altered face, malpositioned limbs, & breech presentation

Front 8

Cause of congenital anomalies?

Back 8

unknown in 50% of cases, genetic, chrom, envir (infection, maternal disease, drugs, radiation), multifactorial (eg folate & spina bifida)

Front 9

Most common chrom anomalies surviving birth?

Back 9

Trisomy 21*, 18 (Edward's), 13 (Patau), Turner's (45,X), Klinefelter's (47, XXY)

Front 10

Charac of Down's syndrome?

Back 10

1/700 births. Assoc w/ advanced maternal age. Flat face, redundant nuchal skin, congenital heart disease, acute leukemia, early Alzheimer's, immunodeficiency, median death age 47.

Front 11

Charac of Patau (T13) syndrome?

Back 11

Microcephaly, cleft lip/palate, & polydacyly

Front 12

Charac of Edward's (T18) syndrome?

Back 12

Overlapping fingers & rocker bottom feet

Front 13

How do Down's, Edward's, & Patau differ?

Back 13

Edwards & Patau are >10X as rare, malformations more sever, & survival in infancy is rare

Front 14

What is the critical period of development?

Back 14

3-5 wks? (3-9 wks)

Front 15

Appropriate weight for gestational age? Small for gest age? Large for gest age? Premature? Post term?

Back 15

B/w 10th & 90th percentile. SGA<10th percentile. LGA>90 percentile. Premature<37wks. Postterm>42wks.

Front 16

Types of SGA?

Back 16

Symmetric (small head & body) & Asymmetric (small body w/ normal head).

Front 17

Charac of Symmetric SGA>

Back 17

Early onset, constitutional, reduced growth potential, organ weight ratios normal

Front 18

Charac of asymmetric SGA?

Back 18

Late onset, envir (including placental factors), late growth arrest, brain relatively large

Front 19

Factors that cause IUGR (SGA or FGR)?

Back 19

Fetal, placental, & maternal factors

Front 20

Fetal factors that result in IUGR?

Back 20

Chrom disorders, congenital anomalies, congenital infections (TORCH group)-> SYMMETRIC growth retardation

Front 21

Placental factors that result in IUGR?

Back 21

Uteroplacental insufficiency (single umbilical artery, placental abruptio, placenta previa), Confined placental mosaicism (from viable genetic mutations occuring after zygote formation)-> ASYMMETRIC

Front 22

What are the most common factors assoc w/ SGA?

Back 22

Maternal factors

Front 23

Maternal factors that result in IUGR?

Back 23

Vascular disease (preeclapsia, chronic hypertension), Drugs/EtOH (antimetabolites, phenytoin, & materanl malnutrition (hypoglycemia)

Front 24

What are the complication of a SGA infant?

Back 24

Perinatal asphyxia, meconium aspiration, hypoglycemia, necrotizing entercolitis, pulmonary hemorrhage. There is amoe overlap b/w complications of prematurity & SGA

Front 25

What is the 2nd most common cause of neonatal mortality?Causes?

Back 25

Prematurity (<37wks). Caused by preterm rupture of membranes, infection, uterin/cervical/placental abnormalities, & multiple gestation

Front 26

Complications of prematurity?

Back 26

RDS (hyaline membrane disease), necrotizing entercolitis, intraventricular hemorrhage, sepsis, developmental delay

Front 27

Charac of RDS?

Back 27

Occurs in 60% of infants <28wks. Lack of surfactant by Type II cells before 35 wk

Front 28

Composition of surfactant?

Back 28

Dipalmitoyl phosphatidylcholine (lecithin), Phosphatidyl glycerol, hydrophilic proteins SP-A & SP-D, hdrophobic proteins SP-B & SP-C

Front 29

Factors that modulate surfactant synthesis?

Back 29

Corticosteroids (induce formation), Intrauterine stress (inc corticosteriod release), Insulin (counteracts the effects of steroids), Labor (inc surfactant synthesis)

Front 30

Prevention & treatment of RDS?

Back 30

Prevent preterm labor, prophylactic corticosteriod therapy given to mom. Treat w/ exogenous surfactant, mech vent (bronchopul dysplasia as complication), or supp oxygen (retinopathy as complication)

Front 31

Charac of necrotizing entercoltis?

Back 31

Dead bowel w/ ubknown etiology. Presents w/ vomiting, abdominal distension, pneumatosis coli, skin discoloration, sepsis. Treated w/ peritoneal drainage or bowel resection

Front 32

What is germinal matrix/intravascular hemorrhage?

Back 32

Vascular cellular region underlying ventricular lining may blees into ventricles or brain-> hydrocephalus or herniation. Can occur in utero. Caused by hypoxia/ishemia

Front 33

What is hydrops fetalis?

Back 33

Fetal soft tissue edema & effusion involving @ least 1 body cavity. Caused by inc hydrostatic press or dec oncotic press-> failure of lymphatic drainage

Front 34

Causes of inc hydrostatic press in hydrops?

Back 34

Chronic intrauterine anemia (treat w/ intrauterine transfusion), cardiac malformations, arrhythmias, cardiomyopathy, high output states (AV malformation or fistulas), or Vascular obstruction (thrombus or compression)

Front 35

Causes of hydrops?

Back 35

Immune (hemolytic disease) or non-immune (CV defects, chrom anomalies, fetal anemia, tumors)

Front 36

Pathogenesis of Rh incompatibility related hydrops?

Back 36

Rh- Mom makes Ab (IgG or IgM) against Baby Rh+-> destroy baby RBC-> hemolysis, anemia, jaundice, inc RBC turnover, & brain kernicterus damage

Front 37

Pathogenesis of ABO incompatibility related hydrops?

Back 37

Most common hemolytic disease in newborn. Infant is usually type A & mother type O -> IgM Ab to A & B-> mild disease-> jaundice. Can happen in 1st pregnancy

Front 38

Charac of fetal anemia due to parvovirus B19 infection?

Back 38

Destroys young RBCs. Shows nucleated RBC in placenta

Front 39

What is the most common genetic disease affecting caucasians?

Back 39

Cystic fibrosis

Front 40

Pathogenesis of CF?

Back 40

Defective Cl channel (CFTR)-> defect in epi ion transport leading to inc viscosity of fluid. CFTR gene on 7q.

Front 41

Sites of CFTR disruptions?

Back 41

Defects in protein production, processing, regulation, or Cl conduction

Front 42

Organs affected by CF?

Back 42

Respiratory (broncholitis, bronchiectasis), pancreas (chronic pancreatitis), liver (secondary biliary cirrhosis), GI (meconium ileus), & reproductive (atrophy of ducts in males & thick cervial mucus/anovulatory cycles)

Front 43

Leading cause of death b/w 1mth & 1yo?

Back 43

SIDS. 90% during the 1st 6mths usually 2-4mths. Most occur @ home during sleep

Front 44

What is SIDS?

Back 44

The sudden death of an infant under 1 yo of unexplained cause after thorough investigation. Diagnosed via exclusion