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pathology > infancy and childhood > Flashcards

infancy and childhood Flashcards

1
Q

malformations are

A

primary errors of morphogenesis

intrinsically developmental process

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2
Q

disruptions result from

A

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

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3
Q

malformations are ___ but disruptions and deformations are ___

A

intrinsic; extrinsic

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4
Q

are disruptions heritable

A

no

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5
Q

deformation pathogenesis

A

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

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6
Q

most common cause of deformation

A

uterine constraint

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7
Q

maternal conditions of deformations

A

first pregnancy, small uterus, malformed uterus, leiomyomas

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8
Q

fetal conditions of deformations

A

multiple fetuses, oligohydramnios, abnormal fetal presentation

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9
Q

what is oligohydramnios

A

decreased amniotic fluid

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10
Q

sequnce can be caused by

A

maternal: chronic leakage of amniotic fluid from rupture of amnion
placental: uteroplacental insufficiency from maternal HT ir toxemia
fetal: renal agenesis

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11
Q

what is oligohydrambios sequence

A

fetal compression due to oligohydramnios
flattened faces, positional abnormalities of hands feet, hips may be dislocated, comprised growth of chest wall and lungs

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12
Q

what is malformation syndrome

A

presence of several defects that cannot be explained on basis of single localizing error in morphogenesis
often from single causative condition

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13
Q

agenesis

A

complete absence of an organ

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14
Q

aplasia

A

incomplete organ development

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15
Q

hypoplasia

A

underdevelopment of organ

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16
Q

atresia

A

absence of opening

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17
Q

types of perinatal infections

A

transcervical(ascending) and transplacental

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18
Q

how do you get transcervical infections

A

spread from cervicovaginal canal

acquired in utero or during birth

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19
Q

transplacental infections

A

gain access to fetal bloodstream by crossing placenta via chronionic villi

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20
Q

examples of transcervical infections

A

bacterial (strep) or viral (herpes) anf pneumonia, sepsis, meningitis

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21
Q

examples of transplacental infections

A

HBV HIV, parasitis (toxoplasma, malaria), viral, bacterial (listeria, treponema)
TORCH- toxoplasma, rubella, cytomegalovirus, herpes, other microbes

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22
Q

what happens with early transplacental (torch) infections

A

growth retardation, mental retardation, cataracts, congenital cardiac anomalies

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23
Q

what happens with late transplacental (torch) infections

A

tissue injury and inflammation

encephalitis, chorioretinitis, hepatospenomegaly, pneumonia, myocarditis

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24
Q

prematurity is when gestational age is

A

less than 37 weeks

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25
Q

major risk factors of prematurity

A

premature rupture of membranes, intrauterine infection leading to chorioamniotitis, structural abnormalities of uterus cervix placenta, multiple gestation

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26
Q

complication sof prematurity

A

RDS, NEC, sepsis, intraventricular and germinal matrix hemorrhage, long term sequelae

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27
Q

what are fetal factors

A

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

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28
Q

what are placental factors

A

any factor that compromises the uteroplacental supply line

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29
Q

placenta previa

A

low implantation of placenta

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30
Q

placental abruption

A

seperation of placenta from decidua by retroplacental clot

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31
Q

growth retardation is symmetric when the cause is

A

intrinsic

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32
Q

growth retardation is asymmetric with

A

placental and maternal causes of growth restriction

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33
Q

maternal factors is the most common cause of

A

growth deficit in SGA infants

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34
Q

drugs causing fetal growth restriction

A

teratogens and non teratogenic afents

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35
Q

causes of respiratory distress in newborn

A

excessive sedation of mother,
fetal head injury in deliver
aspiration of blood or amniotic fluid
intrauterine hypoxia secondary to compression from coiling of umbilical cord on neck

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36
Q

RDS is the most common cause of

A

respiratory insufficiency because formation of membranes in peripheral air spaces

37
Q

what happens in RDS

A

no surfactant

lungs not ready for exchange

38
Q

in infants affected with RDS ___ is required

A

oxygen

39
Q

necrotizing enterocolitis (NEC) is most commonanges in

A

premature infants

40
Q

inflammatory mediators of NEC

A

platelet activating factors PAF

increasing mucosal permeability, promoting enterocute apoptosis, compromising intercellular tight jxns

41
Q

NEC typically involves

A

terminal ileum, cecum, right colon

42
Q

involved segment in NEC is typically

A

distended, friable, congested, frankly ganrenous

43
Q

microscopic examination of NEC

A 
mucosal or transmural coagulative necrosis 
ulceration 
bacterial coloniztion 
submucosal gas bubbles 
reparative changes
44
Q

clinical course of NEC

A

bloody stools, abdominal distention and circulatory collapse
gas within abdominal walls

45
Q

SIDS is leading cause of death in US infants ages

A

1month to 1 year

46
Q

SIDS pathogenesis

A

multifactoral condition
triple risk
1.vulnerable infant: delayed development of arousal and cardiorespiratory
2. critical development period
3. one or more exogenous stressors
-prone sleeping, sleeping on soft surfaces, thermal stress

47
Q

SIDS morphology

A

multiple petechiae
congested lungs
vascular engorgement with or w/o pulm edema
hypoplasia of arcuate nucleus or a subtle decrease in brain stem neuronal populations

48
Q

most likely basis for SIDS is

A

delayed development in arousal reflexes and cardiorespiratory control

49
Q

what is fetal hydrops

A

accumulation of edema fluid in fetus during intrauterine growth

50
Q

immune hydrops

A

hemolytic anemia caused by Rh blood group incompatibility b/t mother and fetus

51
Q

nonimmune hydrops is due to

A

successful pregnancy prophylaxis

52
Q

fetal hydrops is usually lethal when the intrauterine fluid accumulation is

A

progressive, generalized edema of fetus

53
Q

fetal hydrops is compatible with life when the intrauterine fluid accumulation is

A

more localized and less marked edema

54
Q

immune hydrops results from

A

Ab induced hemolytic disease in newborn caused by blood group incompability between mom and fetus (ABO and Rh)

55
Q

factors associated with SIDS

A

maternal smoking during pregnancy, drug abuse, low socioeconomic group, males, African Americans, prone position while sleeping, hyperthermia, sleeping with parents in 1st 3 months of life

56
Q

most common cause of immune hemolytic disease of newborn

A

fetomaternal ABO incompatibility

57
Q

major causes of nonimmune hydrops

A

cardiovascular defects
chromosomal anomalies- 45 X karyotupe, trisomy 21, trisomy 18
fetal anemia

58
Q

what happens in fetal hydrops

A 
tissue ischemia 
secondary myocardial dysfunction 
circulatory failure 
secondary liver failure 
hypoalbuminemia 
reduced plasma osmotic pressure 
edema
59
Q

fluid accumulation in hydrops detalis is particularly prominent in

A

soft tissues in neck

60
Q

transpalacental infection by ____ is increasingly recognied as an important cause of fetal hydrops

A

parvovirus B19

61
Q

kernicterus is

A

hemolysis in Rh or ABO incompatibility –> increased circulating bilirubin from RBC breakdown
characteristic yellow hue to the parenchyma (

62
Q

____ are prone to deposition of bilirubin pigment

A

basal ganglia and brainstem

63
Q

what is heterotopia or choristoma

A

microscopically normal cells or tissue that are present in abnormal locations

64
Q

hamartoma is

A

focal overgrowth of cells and tissues native to organ in which it occurs

65
Q

hemangioma is most common

A

tumor of infancy

66
Q

hemangioma is types

A

cavernous and capillary

67
Q

hemangioma produces

A

flat to elevated, irregular, red blue masses, flat larger lesions

68
Q

lymphangioma occurs on

A

skin also in deeper regions of neck, axilla, mediastinum, retroperitoneum

69
Q

lymphangioma are histologically

A

benign

70
Q

sacrococcygeal teratoma is most common

A

germ cell tumor of childhood

71
Q

neuroblastoma are tumors of

A

sympathetic ganglia and adrenal medulla derived from primordial neural crest cells

72
Q

neuroblastoma is second most common

A

solid malignancy of childhood after brain tumors

73
Q

mutation of neuroblastoma

A

ALK

74
Q

morphology of neuroblastoma

A

minute nodules to large masses

cut surface: soft, gray tan brain like tissue

75
Q

ganglioneuroma arise from

A

spontaneous or therapy induced maturation of neuroblastomas

76
Q

most important prognosis of neuroblastoma

A

stage of tumor and age of patient

77
Q

neuroblastomas arise from

A

neural crest derived cells in sympathetic ganglia and adrenal medulla

78
Q

homer wright pseudo rosettes are characteristic of

A

neuroblastomas

79
Q

neuroblastomas secrete

A

catecholamines

80
Q

retinoblastoma is most common

A

primary intraocular malignancy of children

81
Q

familial cases of retinoblastoma are

A

multiple bilateral tumors

82
Q

sporadic cases of retinoblastoma are

A

nonheritable and unilateral and unifocal

83
Q

patients with familial retinoblastoma are at increased risk for

A

osteosarcoma

84
Q

clinical features of retinoblastoma

A

poor vision, strabismus, whitish due to pupil, pain and tenderness in eye

85
Q

most common sites of distant metastases of retinoblastoma

A

CNS skull distal bones lymp nodes

86
Q

wilms tumor is most common

A

primary tumor of kidney in children

nephroblastoma

87
Q

morphology of wilms tumor

A

blastemal component: sheets of small blue cells
epithelial differentiation: abortive tubules or glomeruli
stromal cells: fibrocytic, myxoid or skeletal muscle differentiation

88
Q

complains of wilms tumor are related to

A

tumor size

89
Q

clinical of wilms tumor

A

palpable abdominal mass and fever and abdominal pain

pathology (8 decks)

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