Pathology - Pediatric Path

Front 1

Top 3 Causes of death in neonates and infants

Back 1

1. Congenital malformations, chromosomal abnormalities, deformations.
2. Low birth weight/short gestational age related
3. SIDS

Front 2

Top 3 causes of death in children 1-4 years of age

Back 2

1. Accidents
2. Congenital malformations, deformations, chromosomal abnormalities
3. Malignant Neoplasms

Front 3

Top 3 causes of death in children 5-14 years of age

Back 3

1. Accidents
2. Malignant neoplasms
3. Homicide

Front 4

Top 3 causes of death in people 15-24 years of age

Back 4

1. Accidents
2. Homicide
3. Suicide

Front 5

TOP CAUSE OF DEATH IN PEOPLE FROM 1-24 YEARS OF AGE

Back 5

ACCIDENTS

Front 6

Define malformations

Back 6

Primary errors of morphogenesis, an intrinsically abnormal process.
-usually multifactorial

Front 7

Define disruptions

Back 7

Secondary destruction of an organ or body region that was previously normal in development: arise from EXTRINSIC disturbance in morphogenesis (i.e., amniotic bands, environmental causes)

Front 8

Define deformations

Back 8

EXTRINSIC interruption of normal development caused by compression of fetus from abnormal biomechanical forces (most common form is from uterine constraint): can resutlt from oligohydramnios, multiple fetuses, small uterus, first pregnancy, leiomyomas.

Front 9

Define sequence

Back 9

A sequence is a pattern of CASCADE ANOMALIES, often explained by a single localized aberration in organogenesis (i.e., oligohydramnios (or Potter's) sequence)

Front 10

Define syndrome

Back 10

A syndrome is a constellation of congenital abnormalities, believed to be pathologically related, that can NOT be explained by a single localized defect.

Front 11

Define Agenesis

Back 11

Agenesis refers to the complete absence of an organ AND its associated anlage.

Front 12

Define aplasia

Back 12

Absence of an organ, owing to the failure of development of the anlage.

Front 13

Define Atresia

Back 13

Absense of an opening to a hollow organ (i.e., esophagus, gut)

Front 14

Define hypoplasia (context of embryogenesis/fetal development)

Back 14

the incomplete development or underdevelopment of an organ associated with a decreased number of cells.

Front 15

Define hyperplasia (context of embryogenesis/fetal development)

Back 15

the overdevelopment of an organ associated with increased number of cells.

Front 16

Causes of congenital anomalies in humans.

Back 16

Genetic
- chromosomal aberrations (10-15%)
- mendelian inheritance
(2-10%)
Environmental
- Maternal/placental (2-3%)
- Rubella
- toxoplasmosis
- syphillus
- cytomegalovirus
- HIV
- Maternal disease states (6-8%)
- diabetes
- PKU (mother)
- endocrinopathies
- drugs & chemicals (1%)
- alcohol
- folic acid antagonists
- androgens
- phenytoin
- thalidomide
- wafarin
- 13-cis retinoic acid
- others
- irradiation (1%)
Multifactorial (20-25%)
Unknown (40-60%)

Front 17

Two phases of intrauterine development (important, since the timing of the teratogenic insult has an important impact on the occurrence and type of anomaly produced)

Back 17

Embryonic period: the first 9 weeks of development
- early embryonic period (0-3 weeks) - embryo either dies or lives
- between weeks 3-9, the embryo is especially sensitive to teratogens, since this is when the organs are developing from germ layers (weeks 4-5 are particularly sensitive)

Fetal period: 9 weeks - birth
- reduced susceptibility to teratogenic insults

Front 18

Steps/factors potentially affected by teratogenic insults:

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-Proper cell migration
-Cell proliferation
-Cellular interactions
-Cell-matrix associations
-Programmed cell death (apoptosis)
-Hormonal influences and mechanical forces

Front 19

AGA

Back 19

average for gestational age (between 10th & 90th percentiles in weight for gestational age)
- at term, normal weight is at least 2500 grams (at least 5.5 lbs)

Front 20

SGA

Back 20

small for gestational age
(<10th percentile)

Front 21

LGA

Back 21

large for gestational age
(>90th percentile)

Front 22

Normal term length

Back 22

40 weeks, +/- 2 weeks

Front 23

Preterm

Back 23

birth before 37 weeks gestation

Front 24

Postterm

Back 24

birth After 42nd week of gestation

Front 25

Risk factors for prematurity
(many of these are also result in FETAL GROWTH RESTRICTION, or IUGR (intrauterine growth restriction) )

Back 25

-PPROM (preterm premature rupture of placental membranes, causes 30-40% of preterm deliveries (the single largest cause of preterm deliveries)
-Intrauterine infection (causes preterm delivery with & without rupture of membranes, present in approx 25% of preterm deliveries - correlates with chorioamnionitis and funisitis)
- uterine, placental, and cervical structural abnormalities
- multiple gestation

Front 26

Sequelae of fetal growth restriction:

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- hyaline membrane disease
- necrotizing enterocolitis
- sepsis
- intraventricular hemorrhage
- long term complications, including developmental delay

Front 27

Fetal causes of IUGR (FGR)

Back 27

##results in symmetrical growth restriction##
- chromosomal disorders (triploidy, trisomy 18, trisomy 21, trisomy 13, others)
- congenital infection (fetal infection) - the TORCH

Front 28

Placental causes of IUGR

Back 28

##results in assymetrical FGR, with relative sparing of brain##
- umbilical-placental vascular anomalies
- placenta abruptio
- placenta previa
- placental thrombosis & infarction
- placental infection
- multiple gestations
- CONFINED PLACENTAL MOSAICISM

Front 29

Maternal causes of IUGR

Back 29

##results in assymetrical FGR##
- preeclampsia (toxemia of pregnancy)
- chronic hypertension
- narcotic abuse
- alcohol use
- nicotine use
- certain drugs (teratogens, phenytoin)
- malnutrition of mother

Front 30

Stages of embryonic lung development

Back 30

- glandular stage:
- begins in month 7; imperfectly formed alveoli, cuboidal epithelium, thick inter & intra-lobular CT
- saccular stage
- between weeks 26-32; cuboidal epithelium becomes flat type 1 alveolar cell, and lamellar-body-containing type 2 alveolar cells
- alveolar stage
- reduction of interstitial tissues & increase in capillaries

##note: full development of lungs is complete by 8 years of age

Front 31

physiological jaundice

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normal jaundice, caused by break down of fetal RBC's, coupled with inadequacy of biliary excretory function of liver

Front 32

APGAR (appearance, pulse, grimace, activity, respirations)

Back 32

- taken at 1 & 5 minutes
- 5 metrics, scored 0, 1, or 2
- COLOR (appearance) - 0 = blue, pale; 1 = body pink, extremities blue; 2 = pink baby
- HEART RATE (pulse) - 0 = absent; 1 = below 100; 2 = over 100
- RESPONSE TO CATHETER IN NOSTRIL (grimace) - 0 = no response; 1 = grimace; 2 = cough or sneeze
- MUSCLE TONE (activity) - 0 = limp; 1 = some flexion of extremities; 2 = active motion
- RESPIRATORY EFFORT (respirations) - 0 = absent; 1 = slow, irregular; 2 = good, crying

Front 33

Most common birth injury

Back 33

intracranial hemorrhages:

- generally due to excessive molding of the head or sudden pressure changes as the head is subject to the pressure of forceps or sudden precipitate expulsion
- predisposed by prolonged labor, hypoxia, hemorrhagic disorders, or intracranial vascular anomalies

Front 34

perinatal infections

Back 34

infections passed from mother to child - fall into two groups: transcervical (ascending) infections & transplacental (hematologic) infections

Front 35

Transcervical infections

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- passed from mother to child either in utero or during child birth
- transferred to child when it inhales amniotic fluid shortly before birth, or when passing through the birth canal

Front 36

transplacental infections

Back 36

- parasitic, viral, and a few bacterial infections gain access to fetus transplacentally, via the chorionic villi
- may occur any time during gestation or during birth as a result of maternal-to-fetal transfusion
- example is parvovirus B19

Front 37

TORCH infections

Back 37

T - Toxoplasmosis
O - Other (treponema pallidium,
R - Rubella virus
C - cytomegalovirus
H - Herpes virus

Front 38

Why are the TORCH infections grouped together?

Back 38

because the evoke clinically similar manifestations:
fever, encephalitis, chorioretinitis, hepatosplenomegaly, pneumonitis, myocarditis, hemolytic anemia, vesicular or hemorrhagic skin lesions
- if infected early, may cause congenital, chronic sequelae, such as: growth & mental retardation, cataracts, congenital heart defects, and bone defects