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Y2 - Path - Exam 4 > 12_Peds Path 1 > Flashcards

12_Peds Path 1 Flashcards

1
Q

why do we separate pediatric diseases from adult?

A
  • Genetic origin
  • Not genetic:
    • Unique to children
    • Take distinctive forms in children
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2
Q

epidemiology of childhood disease mortality?

A

if patient survives beyond one year, likely to survive

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3
Q

annual incidence of congenital anomalies (structural defects present at birth)?

A

120,000 babies born w/ birth defect each year in US (1:33)

some (cardiac or renal) defects may not be clinically apparent until later

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4
Q

5 types of morphogenesis errors?

A
  • malformations
  • deformations
  • disruptions
  • sequence
  • malformation syndrome
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5
Q

define: malformations

A

primary errors of morphogenesis; intrinsically abnormal developmental process;

usually multifactorial

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6
Q

what are the 2 possible manifestations of malformations?

A
  • single body system (e.g. congenital heart disease)
  • multiple coexisting malformations involving many organs and tissues
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7
Q

polydactyly vs syndactyly

A
  • polydactyly: one or more extra digits
  • syndactyly: fusion of digits’

little functional consequence if occurs in isolation

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8
Q

if cleft lip and cleft palate are isolated anomaly, likely compatible w/ life.

When would cleft lip likely be INCOMPATIBLE with life?

A

If it’s a sign of an underlying malformation syndrome, e.g. trisomy 13

(related to other health effects such as cardiac defects)

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9
Q

what does a severe degree of external dysmorphogenesis of the head indicate?

A

associated w/ severe internal anomalies (e.g. maldevelopment of brain and cardiac defects);

in this case, the mid-face structures are fused or illformed;

often a lethal malformation

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10
Q

define: disruption

A

results from secondary destruction of an organ or body region that was previously normal in development;

  • potential environmental causes;
  • NOT heritable
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11
Q

how do malformations AND disruptions differ?

A

disruptions arise from extrinsic disturbance in morphogenesis;

whereas malformations are primary errors/intrinsically abnormal development

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12
Q

what type of congenital anomaly is amniotic bands?

A

type of disruption;

  • rupture of amnion –> “bands”
    • encircle, compress, and attach to parts of developing fetus
  • occurs when the inner membrane (amnion) ruptures, or tears, without injury to the outer membrane (chorion).
  • The developing fetus is still floating in fluid but is then exposed to the floating tissue (bands) from the ruptured amnion. This floating tissue can become entangled around the fetus.
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13
Q

pathogenesis of deformations

(type of extrinsic disturbance of development)

A

localized or generalized compression of the growing fetus by abnormal biomechanical forces –> structural abnormalities

*COMMON: 2% of newborn infants

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14
Q

most common cause of deformations?

pathology?

A

uterine constraint;

during weeks 35-58 of gestation –> rapid increase in fetus size outpacing growth of uterus –> relative decrease in amniotic fluid

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15
Q

what maternal conditions can cause uterine constraint, and subsequently deformation?

A
  • first preganncy
  • small uterus
  • malformed (bicornuate) uterus
  • leiomyomas
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16
Q

what fetal conditions can cause uterine constraint, and subsequently deformation?

A
  • multiple fetuses
  • oligohydramnios (less cushioning ofr baby)
  • abnormal fetal presentation
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17
Q

define: sequence;

what are the 3 initiating events?

A
  • def: multiple congenital anomalies that result from secondary effects of a single localized aberration in organogenesis; initiating events incl:
    • Malformation
    • Deformation
    • Disruption
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18
Q

oligohydramnios is decreased amniotic fluid;

what are the potential maternal, placental, and fetal causes?

A
  1. maternal: chronic leakage of amniotic fluid due to rupture of amnion
  2. placental: uteroplacental insufficiency from maternal hypertension or severe toxemia
  3. fetal: renal agenesis
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19
Q

oligohydramnios (potter) sequence:

pathology, and resulting phenotype

A

Oligohydramnios –> fetal compression –> classic phenotype:

  • flattened facies (facies is distinctive facial expression or appearance )
  • positional abnormalities of hands /feet
  • hips may dislocate
  • compromised growth of chest wall and lungs
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20
Q

malformation syndrome:

define / pathology

A

presence of several defects that can’t be explained on basis of single localizing initiating error in morphogenesis;

path: single causative agent –> simultaneously affecting several tissues (e.g. viral infection or specific chromosomal abnormality)

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21
Q

define: agenesis

A

complete absence of an organ or its anlage (rudimentary basis of a particular organ or other part)

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22
Q

define: hypoplasia

A

underdevelopment of an organ

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23
Q

difference between aplasia and atresia

A
  • aplasia: incomplete development of an organ
  • atresia: absence of an opening, usually of hollow visceral organ or duct (e.g. of intestines and bile ducts)
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24
Q

causes of congenital malformations in humans?

A

genetic

environmental

multifactorial

unknown

25
Q

pathogenesis of congenital anomalies is complex and poorly understood;

what are the 2 general principles?

A
  1. timing of the prenatal teratogenic insult
  2. interplay b/w environmental teratogens and intrinsic genetic defects
26
Q

which period of development do internal/external factors most often affect fetus?

A

**embryonic period, which is the critical phase of growth

(3-8 weeks of development)

27
Q

two types of perinatal infections?

A

transcervical (ascending) infections

transplacental infections

28
Q

define: transcervical (ascending infections)

A

spread from cervicovaginal canal;

acquired in utero or during birth

29
Q

define: transplacental** infections**

A

gains access to fetal bloodstream by crossing the placenta via the chorionic villi (hematogenous transmission)

30
Q

how does fetus acquire infection in transcervical infections?

A
  • by “inhaling” infected amniotic fluid into lungs
  • passing through infected birth canal during delivery

Fetal infection –> inflammation of placental membranes (chorioamnioitis) umbilical cord (funisitis)

31
Q

examples of conditions w/ transcervical as typical mode of spread ?

A
  • bacterial infections (e.g., α-hemolytic streptococcal infection)
  • viral infections (e.g., herpes simplex)
  • pneumonia
  • sepsis
  • meningitis
32
Q

when do transplacental infections occur?

A

may occur at any time during gestation or at the time of delivery via maternal-to-fetal transfusion (e.g. HBV & HIV);

33
Q

mnemonic for transplacental infections?

A

TORCH :

  • Toxoplasma ( T)
  • Other (O) microbes (e.g., Treponema pallidum)
  • Rubella virus (R)
  • Cytomegalovirus (C)
  • Herpesvirus (H)
34
Q

effects of early TORCH infections?

A

CHRONIC SEQUELAE

  • growth restriction
  • mental retardation
  • cataracts
  • congenital cardiac anomalies
35
Q

effects of later TORCH infections?

A

tissue injury & inflammation

  • encephalitis
  • choriorenitis
  • hepatosplenomegaly
  • pneumonia
  • myocarditis
36
Q

prematurity:

definition, epidemiology

A
  • def: gestational age less than 37 weeks
  • epi: 2nd most common cause of neonatal mortality (2nd only to congenital anomalies)
37
Q

difference b/w prematurity and fetal growth restrictions?

A
  • premature infants weigh less than normal (<2500), but appropriate for gestational age
  • fetal growth restrictions (1/3 of infants weighing <2500 gm BORN AT TERM; undergrown, rather than immature)
38
Q

what are risk factors for prematurity?

A
  • prematurity rupture of membranes
  • intrauterine infxn leading to chorioamnionitis
  • structural abnormalities of the uterus, cervix, and placenta (e.g. bicorunate nucleus)
  • multiple gestation
39
Q

what complications may result from prematurity?

A
  • respiratory distress syndrome (RDS) aka “hyaline membrane disease”
  • necrotizing enterocolitis (NEC)
  • sepsis
  • intraventricular and germinal matrix hemorrhage
  • long-term sequelae, incl developmental delay
40
Q

small-for-gestational-age (SGA) infants suffer fetal growth restriction.

what are the causes of fetal growth restrictions?

A
  • fetal
  • maternal
  • placental
  • unknown
41
Q

fetal factors causing fetal growth restrictions?

A

conditions that intrinsically reduce growth potential of fetus despite an adequate supply of nutrients from the mother

  • chromosomal disorders
    • cause 17% of fetuses evaluated for growth restriction
    • 66% of fetuses w/ documented Ultrasonographic malformations
  • congenital anomalies
  • congenital infections
42
Q

what should be considered in all growth-restricted neonates?

A

transplancental infections (TORCH);

cause is intrinsic to fetus –> growth retardation is symmetric (affecting all organs equally)

43
Q

what are the placental factors that cause fetal growth restrictions?

A

any factor that compromises the uteroplacental supply line;

  • placenta previa (low implantation of placenta)
  • placental abruption (separation of placenta from decidua by retroplacental clot)
  • placental infarction
44
Q

which factors of fetal growth restriction cause symmetric retardation?

which cause asymmetric?

A
  • SYMMETRIC growth retardation if cause is INTRINSIC to fetus (fetal factors)
    • affects all organ systems equallty
  • ASYMMETRIC (e.g. brain spared relative to visceral organs) if cause is placental or maternal causes of growth restriction
45
Q

what is most common causes of growth deficit in small-for-gestational-age (SGA) infants?

A

maternal factors

  • avoidable influences
    • narcotic abuse
    • alcohol intake
    • heavy cigarette smoking
  • maternal malnutrition (prolonged hypoglycemia)
  • drugs causing fetal growth restriction
    • teratogens (phenytoin, dilantin)
    • non-teratogenic agents
  • others
    • vascular disease (preeclampsia, “toxemia of pregnancy”
    • chronic HTN
46
Q

Respiratory distress syndrome (RDS) in infants is MOST COMMON CAUSE of respiratory insufficiency bc “membranes” form in peripheral air spaces;

what are the causes of RDS in newborn?

A
  • excessive sedation of the mother
  • fetal head injury during delivery
  • aspiration of blood or amniotic fluid
  • intrauterine hypoxia secondary to compression from coiling of umbilical cord around the neck
47
Q

incidence and mortality of Respiratory Distress Syndrome in infants in US?

A
  • Incidence: 24,000 cases reported annually in US
  • Mortality: now 900/yr, down from 5000/year due to improvements in management
48
Q

pathophysiology of Respiratory Distress Syndrome?

A
  1. premature infant
  2. reduced surfactant synthresis, storage, release (due to underdevelopment)
  3. decreased alveolar surfactant –>
  4. inc alveolar surface tension –>
  5. ATELECTASIS–> uneven perfusion & hypoventilation
  6. hypoxemia & CO2 retention
  7. acidosis
  8. pulmonary vasoconstriction
  9. pulmonary hypoperfusion –> endothelial and epithelial damage
  10. plasma leak into alveoli –>
  11. fibrin + necrotic cells –> HYALINE MEMBRANE
  12. decreased diffusion gradient –> HYPOXEMIA & CO2 RETENTION
    13.
49
Q

microscopic morphology of hyaline membrane disease?

A
  • alternating atelectasis & dilation of alveoli
  • eosinophilic thick hyaline membranes lining dilated alveoli
50
Q

clinical features of respiratory distress syndrome includes maturity, birth weight, & promptness of institution of therapy.

how is RDS prevented?

A
  • delaying labor until fetal lungs are mature
  • induce maturation of fetal lungs at risk
  • analyze amniotic fluid phospholipids (est level of surfactant)
  • prophylactic admin of exogenous surfactant at birth to extremely premature infants
51
Q

respiratory distress syndrome:

complications

A
  • uncomplicated –> recover in 3-4 days
    • affected infants require oxygen
  • complicated –> long-term use of high conc of O2 of ventilators can cause:
    • retrolental fibroplasia (retinopathy of prematurity)
    • bronchopulmonary dysplasia (BPD)
52
Q

necrotizing enterocolitis (the wall of the intestine is invaded by bacteria, which cause local infection and inflammation that can ultimately destroy the wall of the bowel);

epidemiology?

A

most commonly in premature infants;

occurs in 1 out of 10 very low birth weight infants (<1500 grams)

53
Q

what is thought to be the cause of necrotizing enterocolitis (NEC)?

A

associated w/ enteral feeding;

some postnatal insult sets cascade –> tissue destruction

(likely infectious agents involved, but no single pathogen is linked to disease)

54
Q

what are the effects of inflammatory mediators in necrotizing enterocolitis?

A

platelet activating factor (PAF) –>

  • increases mucosal permeability
  • promotes enterocyte apoptosis
  • compromises intercellular tight junctions
55
Q

which organs/segments are involved in necrotizing enterocolitis?

what is the gross anatomical morphology of affected segments?

A

*see image

  • at postmortem exam, you may see entire small bowel markedly distended w/ thin wall –> implies IMPENDING PERFORATION
56
Q

what does the congested portion of ileum correspond to?

what are the arrows pointing to?

A
  1. congested portion –> areas of hemorrhagic infarction and transmural necrosis seen on microscopy
  2. submucosal gas bubbles (pneumatosis intestinalis) – indicated by arrows
57
Q

microscopic findings of necrotizing enterocolitis?

A
58
Q

clinical course and dx of necrotizing enterocolitis?

A
  • course: bloody stools –> abdominal distention –> circulatory collapse
  • dx; abdominal radiographs showing gas w/in intestinal wall (pneumoatosis intestinalis)
59
Q

outcomes of necrotizing enterocolitis?

A
  • Early detection –> can be managed conservatively
  • 20-60% require operative intervention (incl resection of necrotic segments of bowel)
    • Assoc w/ high perinatal mortality
    • Infants who survive often develop post-NEC strictures from healing fibrosis

Y2 - Path - Exam 4 (13 decks)

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