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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
major risk factors of prematurity
premature rupture of membranes, intrauterine infection leading to chorioamniotitis, structural abnormalities of uterus cervix placenta, multiple gestation
26
complication sof prematurity
RDS, NEC, sepsis, intraventricular and germinal matrix hemorrhage, long term sequelae
27
what are fetal factors
conditions that intrinsically reduce growth potential of fetus despite an adequate supply of nutrients from mother
28
what are placental factors
any factor that compromises the uteroplacental supply line
29
placenta previa
low implantation of placenta
30
placental abruption
seperation of placenta from decidua by retroplacental clot
31
growth retardation is symmetric when the cause is
intrinsic
32
growth retardation is asymmetric with
placental and maternal causes of growth restriction
33
maternal factors is the most common cause of
growth deficit in SGA infants
34
drugs causing fetal growth restriction
teratogens and non teratogenic afents
35
causes of respiratory distress in newborn
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
36
RDS is the most common cause of
respiratory insufficiency because formation of membranes in peripheral air spaces
37
what happens in RDS
no surfactant | lungs not ready for exchange
38
in infants affected with RDS ___ is required
oxygen
39
necrotizing enterocolitis (NEC) is most commonanges in
premature infants
40
inflammatory mediators of NEC
platelet activating factors PAF | increasing mucosal permeability, promoting enterocute apoptosis, compromising intercellular tight jxns
41
NEC typically involves
terminal ileum, cecum, right colon
42
involved segment in NEC is typically
distended, friable, congested, frankly ganrenous
43
microscopic examination of NEC
``` mucosal or transmural coagulative necrosis ulceration bacterial coloniztion submucosal gas bubbles reparative changes ```
44
clinical course of NEC
bloody stools, abdominal distention and circulatory collapse gas within abdominal walls
45
SIDS is leading cause of death in US infants ages
1month to 1 year
46
SIDS pathogenesis
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
SIDS morphology
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
most likely basis for SIDS is
delayed development in arousal reflexes and cardiorespiratory control
49
what is fetal hydrops
accumulation of edema fluid in fetus during intrauterine growth
50
immune hydrops
hemolytic anemia caused by Rh blood group incompatibility b/t mother and fetus
51
nonimmune hydrops is due to
successful pregnancy prophylaxis
52
fetal hydrops is usually lethal when the intrauterine fluid accumulation is
progressive, generalized edema of fetus
53
fetal hydrops is compatible with life when the intrauterine fluid accumulation is
more localized and less marked edema
54
immune hydrops results from
Ab induced hemolytic disease in newborn caused by blood group incompability between mom and fetus (ABO and Rh)
55
factors associated with SIDS
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
most common cause of immune hemolytic disease of newborn
fetomaternal ABO incompatibility
57
major causes of nonimmune hydrops
cardiovascular defects chromosomal anomalies- 45 X karyotupe, trisomy 21, trisomy 18 fetal anemia
58
what happens in fetal hydrops
``` tissue ischemia secondary myocardial dysfunction circulatory failure secondary liver failure hypoalbuminemia reduced plasma osmotic pressure edema ```
59
fluid accumulation in hydrops detalis is particularly prominent in
soft tissues in neck
60
transpalacental infection by ____ is increasingly recognied as an important cause of fetal hydrops
parvovirus B19
61
kernicterus is
hemolysis in Rh or ABO incompatibility --> increased circulating bilirubin from RBC breakdown characteristic yellow hue to the parenchyma (
62
____ are prone to deposition of bilirubin pigment
basal ganglia and brainstem
63
what is heterotopia or choristoma
microscopically normal cells or tissue that are present in abnormal locations
64
hamartoma is
focal overgrowth of cells and tissues native to organ in which it occurs
65
hemangioma is most common
tumor of infancy
66
hemangioma is types
cavernous and capillary
67
hemangioma produces
flat to elevated, irregular, red blue masses, flat larger lesions
68
lymphangioma occurs on
skin also in deeper regions of neck, axilla, mediastinum, retroperitoneum
69
lymphangioma are histologically
benign
70
sacrococcygeal teratoma is most common
germ cell tumor of childhood
71
neuroblastoma are tumors of
sympathetic ganglia and adrenal medulla derived from primordial neural crest cells
72
neuroblastoma is second most common
solid malignancy of childhood after brain tumors
73
mutation of neuroblastoma
ALK
74
morphology of neuroblastoma
minute nodules to large masses | cut surface: soft, gray tan brain like tissue
75
ganglioneuroma arise from
spontaneous or therapy induced maturation of neuroblastomas
76
most important prognosis of neuroblastoma
stage of tumor and age of patient
77
neuroblastomas arise from
neural crest derived cells in sympathetic ganglia and adrenal medulla
78
homer wright pseudo rosettes are characteristic of
neuroblastomas
79
neuroblastomas secrete
catecholamines
80
retinoblastoma is most common
primary intraocular malignancy of children
81
familial cases of retinoblastoma are
multiple bilateral tumors
82
sporadic cases of retinoblastoma are
nonheritable and unilateral and unifocal
83
patients with familial retinoblastoma are at increased risk for
osteosarcoma
84
clinical features of retinoblastoma
poor vision, strabismus, whitish due to pupil, pain and tenderness in eye
85
most common sites of distant metastases of retinoblastoma
CNS skull distal bones lymp nodes
86
wilms tumor is most common
primary tumor of kidney in children | nephroblastoma
87
morphology of wilms tumor
blastemal component: sheets of small blue cells epithelial differentiation: abortive tubules or glomeruli stromal cells: fibrocytic, myxoid or skeletal muscle differentiation
88
complains of wilms tumor are related to
tumor size
89
clinical of wilms tumor
palpable abdominal mass and fever and abdominal pain

pathology (8 decks)

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