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PBD II > Pediatric Pathology > Flashcards

Pediatric Pathology Flashcards

1
Q

transplacental (hematologic) infections:

A

TORCH (Toxoplasma, Other (Treponema pallidum), Rubella, CMV, Herpes)
Parvovirus: 5th disease

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2
Q

transcervical infections

A

Most bacterial, some viral (HSV II)
Frequent with preterm delivery
Gonnococcal and Chlamydial infections

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3
Q

post-natal maternal milk

A

CMV
HIV
HTLV I
Heat-labile E. coli enterotoxin

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4
Q

perinatal infections

A

Group B strep #1 within 1 week of delivery

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5
Q

hydrops fetalis

A

Accumulation of edema in fetus
Immune or nonimmune
Erythroblastosis fetalis if accompanied by anemia

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6
Q

immune hydrops

A

Rh- mother, Rh+ baby
first pregnancy: anti-D IgM (can’t cross placenta)
second pregnancy: anti-D IgG (can cross placenta)
concurrent ABO incompatibility helps prevent sensitization to Rh

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7
Q

Coombs test

A

tests for presence of anti-RBC Abs on RBCs

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8
Q

non-immune hydrops

A

Cardiovascular defects
Chromosomal anomalies (Turner, Trisomy 21 and 18)
Lymphatic anomalies
Twin-twin transfusion
Fetal non-immune anemia (homozygous alpha-thalassemia, parvovirus B19 induced aplastic anemia)

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9
Q

self-mutilation

A

Lesch-Nyhann syndrome

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10
Q

sweaty feet

A

isovaleric acidemia

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11
Q

mousy or musty

A

PKU

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12
Q

cataracts

A

galactosemia

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13
Q

cherry red macula

A

Tay-Sachs, GM1

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14
Q

dislocated lens

A

homocysteinuria or Marfans

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15
Q

cystic fibrosis

A

AR, 1 in 2500 live births, most common lethal genetic disease
Affects transport of fluid secretions in exocrine glands and epithelial lining of respiratory, GI, and reproductive tracts
Viscous mucous secretions, obstruct organ passages
Pancreatic insufficiency, steatorrhea, malnutrition, hepatic cirrhosis, intestinal obstruction, male infertility, recurrent pulmonary infections

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16
Q

hemangiomas

A

usually skin, most common tumor of infancy, benign

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17
Q

lymphangiomas

A

skin or deeper (neck, axilla, mediastinum, retroperitoneum), benign

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18
Q

fibrous tumors

A

t(12;15)(p13;q25) in congenital-infantile fibrosarcoma (ETV6-NTKR3 fusion transcript making tyrosine kinase stimulating RAS and PI3K/AKT oncogenic pathways)

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19
Q

most common malignancy in children

A

acute lymphoblastic leukemia

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20
Q

1 solid tumor in children

A

CNS tumors

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21
Q

2 solid tumor in children

A

neuroblastoma (#1 in infants)

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22
Q

neuroblastoma genes and findings

A

17q gain, 1p deletion
N-myc amplification
DNA hyperdiploidy, near triploidy
urinary catecholamines

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23
Q

Ewing sarcoma genes

A

t(11;22)

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24
Q

rhabdomyosarcoma genes

A

PAX3 and PAX7

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25
Q

Burkitt lymphoma gene

A

t(8;14)

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26
Q

*Wilms tumor gene

A

11p13 (WT1)

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27
Q

retinoblastoma gene

A

13q14 (RB)

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28
Q

neuroblastoma findings

A

Presents as abdominal mass, most commonly adrenal medulla
90% secrete catecholamines, VMA and HVA in urine
Small blue cell tumor
*Homer-Wright pseudorosettes

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29
Q

neuroblastoma cutaneous metastases

A

blueberry muffin baby

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30
Q

neuroblastoma stage 4s

A

localized primary tumor in infants with dissemination limited to skin, liver, and/or bone marrow. Good prognosis.

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31
Q

N-MYC amplification

A

poor neuroblastoma prognosis

Double minutes

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32
Q

retinoblastoma findings

A

Malignant eye tumor
Familial (60-70%): germline RB1 mutation
Sporadic (30-40%): somatic RB1 mutation
Flexner-Wintersteiner rosettes

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33
Q

nephroblastoma

A 
#1 primary renal tumor in children, #4 pediatric malignancy
Triphasic histology: stroma, tubules, blastemal elements
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34
Q

embryo

A

implantation to first 8 weeks

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35
Q

fetus

A

9 weeks to birth

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36
Q

neonatal

A

first 4 weeks after birth (most hazardous)

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37
Q

perinatal

A

5 months before to 1 month after birth

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38
Q

infancy

A

first year after birth

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39
Q

childhood

A

birth to puberty or legal adult age

40
Q

most common cause of death from 1-12 months

A

SIDS

41
Q

most common cause of death from 1-14 years

A

accidents

42
Q

congenital anomalies

A

Present at birth, may not be expressed until later in life
Up to 3% of newborns
The most severe anomalies cause intrauterine death

43
Q

most common birth defects

A
  1. bicuspid aortic valve

2. Down syndrome

44
Q

malformation

A

Primary failure, intrinsically abnormal development > abnormal morphogenesis
E.g. anencephaly, congenital heart defects
Usually multifactorial

45
Q

disruption

A

Secondary disruption of previously normal structure
E.g. amniotic bands, environmental cuases
Not heritable!

46
Q

deformation

A

Extrinsic disturbance from abnormal biomechanical forces > structural abnormalities
Maternal (uterine constraint) or fetal/placental (oligohydramnios) cause
2% of newborns
E.g. breech presentation

47
Q

sequence

A

Cascade of anomalies set off by one initiating aberation
May be a “complex”
E.g. Potter sequence

48
Q

malformation syndrome

A

Constellation of congenital anomalies thought to be pathologically related but cannot be explained by a single initiating event
May be a “complex”
E.g. viral disease or chromosomal alteration that affects different tissues

49
Q

Potter sequence (oligohydramnios)

A

renal agenesis > oligohydramnios > amnion nodosum pulmonary hypoplasia, fetal compression > altered facies, positioning defects, breech presentation

50
Q

Potter facies

A

Ocular hypertelorism (increased space btw eyes)
Low-set ears
Receding chin
Flattening of nose

51
Q

TORCH organism

A 
Toxoplasma
Other (T. pallidum)
Rubella
CMV
Herpes simplex

Cataracts, conjunctivitis, pneumonitis, HSM, petechiae and purpura
Early infection: heart problems

52
Q

agenesis

A

complete absence of an organ an primordium (nothing formed)

53
Q

aplasia

A

absence of an organ d/t primordium development failure

54
Q

atresia

A

block in something with a lumen

55
Q

dysplasia

A

abnormal organization of cells (malformation context only)

56
Q

omphalocele

A

abdominal musculature fails to form, covered by peritoneum, easier to repair

57
Q

gastroschisis

A

part of abdominal wall fails to form, not covered by peritoneum

58
Q

causes of congenital anomalies

A
  1. 40-60% unknown

2. 20-25% multifactorial

59
Q

most sensitive period of fetal development

A

4-5 weeks (heart forms first 6 weeks)

60
Q

most sensitive period for teratogenicity

A

3-9 weeks

61
Q

Rubella embryopathy

A 
Worst of TORCH, usually 1st trimester
Tetrad of: 
Cataracts
Deafness
Heart defects
Mental retardation
62
Q

CMV

A

Later in pregnancy (2nd trimester)
CNS involvement
Microcephaly, mental retardation, deafness, HSM
Does not affect organogenesis much, primarily affects growth of organs

63
Q

thalidomide

A

limb developmental problems

64
Q

Vit A/13-cis-retinoic acid/accutane(isotretinoin)

A

disrupts HOX genes

65
Q

valproic acid (antiepilectic)

A

disrupts HOX genes

66
Q

fetal alcohol syndrome (FAS)

A

atrial septal defects, #1 cause of mental retardation, growth retardation, microcephaly

67
Q

phthalates

A

Added to PVC and used in flexible plastics
Exposure to in lab animals causes endocrine disruption and testicular dysgenesis syndrome
Some concern for BPA effects on brain, behavior, and prostate glands in fetuses, infants, and children

68
Q

maternal diabetes

A

Diabetic embryopathy
Hyperglycemia in mother induces hyperinsulinemia in infant > decreased surfactant
Fetal macrosomia (birth weight > 4000g)
Heart problems, brain and spinal defects, oral clefts, kidney and GI defects, limb deficiencies

69
Q

caput succedaneum

A

scalp edema, extremely common (vaginal delivery > cone head)

70
Q

cephalhematoma

A

hemorrhage (more problematic), 25% will have skull fracture

71
Q

HOX 1-4

A

face

72
Q

HOX 9-13

A

extremeties

73
Q

HOXD13

A

syndactyly/polydactyly

74
Q

HOXA13

A

hand-foot-genital syndrome (distal limb and distal urinary tract malformations)

75
Q

PAX2

A

renal-coloboma syndrome (developmental defects of kidneys, ears, eyes, brain)

76
Q

PAX3

A

Waardenburg syndrome (congenital pigment abnormalities and deafness)

77
Q

PAX6

A

aniridia (congenital absence of iris)

78
Q

Robertsonian translocation

A

2 copies of gene on 1 chromosome, none on the homolog

Trisomies or monosomies in progeny

79
Q

B9

A

lowers incidence of neural tube defects

80
Q

2 requirements for congenital dislocation of hip

A
  1. shallow acetabulum (genetic)

2. breech presentation (environment)

81
Q

probability of neonate mortality (APGAR)

A

0-1: 50% mortality
4: 20%
7 or better: almost 0%

82
Q

premature (pre-term)

A

Before 37 weeks
2nd leading cause of neonatal mortality behind congenital anomalies
Risk factors: premature rupture of placental membranes, intrauterine infection, uterine, cervical, or placental structural abnormalities, multiple gestaion

83
Q

postmature (post-term)

A

after 42 weeks

84
Q

PROM

A

Premature rupture of membranes (after 37 weeks)

Okay to deliver baby

85
Q

PPROM

A

Pre-term premature rupture of membranes (before 37 weeks)

Administer anti-labor meds to delay deliver

86
Q

intrauterine infection

A

Present in 25% of preterm births
Choriamnitis (membranes)
Funisitis (cord)
U. urealyticum, M. hominis, G. vaginalis, T. vaginalis, N. gonorrheae, C. trachomatis

87
Q

fetal causes of FGR

A

Usually symmetric growth
Chromosomal disorders
Congenital anomalies
Infection by a TORCH

88
Q

placental causes of FGR

A

Usually asymmetric
Uteroplacental insufficiency
Confined placental mosaicism (trisomy 7)

89
Q

maternal causes of FGR

A 
Most frequent causes of FGR
Hypertension
Hypercoagulable states
Alcohol, narcotics, heavy smoking, drugs (dilantin)
Malnutrition
90
Q

hyaline membrane disease

A

Most are preterm
Surfactant deficiency
More common in males, maternal DM, multiple gestation, C-section

91
Q

surfactant

A 
Mature type II pneumocytes
Dipalmitoyl phosphatidylcholine (lecithin)
SP-B and SP-C (surface tension)
L/S ratio > 2 lungs are mature
L/S ratio
92
Q

retinopathy of prematurity (retrolental fibroplasia)

A

Phase I: O2 therapy > decreased VEGF > endothelial cell apoptosis
Phase II: relative hypoxia (room air) > increased VEGF > angiogenesis

93
Q

bronchopulmonary dysplasia

A

alveolar hypoplasia

94
Q

necrotizing enterocolitis (NEC)

A

intestinal ischemia

platelet activating factor elevated in stool

95
Q

germinal matrix hemorrhage

A

Subependymal (periventricular) hemorrhage with extension into ventricles in preterm infants

PBD II (12 decks)

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