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PBD II Exam 1 > Diseases of Infancy and Childhood IV > Flashcards

Diseases of Infancy and Childhood IV Flashcards

1
Q

SIDS

A

yes, cause of death
prior to 1 year of age**

unexplained death after autopsy, review of death scene, and review of clinical history

diagnosis of exclusion

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2
Q

leading cause of death between 1 month and 1 year

A

SIDS

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3
Q

most occurence of SIDS

A

between 2 - 4 months old

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4
Q

ALTE

A

apnea, change in color, choking

increased risk for SIDS

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5
Q

environmental risk factors for SIDS

A

prone or side sleeping
co-sleeping
hyperthermia

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6
Q

infant risk factors for SIDS

A 
brainstem abnormality - resp center
prematurity
male
multiple birth
prior SIDS sibling
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7
Q

maternal risk for SIDS

A 
young age
smoking
drug abuse - either parent
no prenatal care
low socioeconomic status
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8
Q

postmortem findings in cases of sudden infant death

A

not SIDS

  • viral myocarditis
  • conenital anomaly
  • child abuse
  • long QT syndrome
  • bronchopneumonia
  • aortic stenosis
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9
Q

rolling over and killing baby

A

only with intoxicated parents

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10
Q

SIDS postmortem findings

A

most common - petechiae
-thymus, parietal pleura, epicardium

  • congested lungs
  • CNS astrogliosis
  • hypoplasia of arcuate nucleus
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11
Q

triple risk model for SIDS

A

vulnerable infant
critical development period
exogenous stressor

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12
Q

serotonergic 5-HT system

A

in medulla

  • involved in arousal
  • abnormalities here may be basis of SIDS

-also - laryngeal chemoreceptors that inhibit resp centers

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13
Q

choristoma

A

normal cells in abnormal location

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14
Q

hamartoma

A

overgrowth of cells native to organ

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15
Q

most common benign tumor of infancy

A

hemangioma
-most go away over time

skin, face, scalp

aka port-wine stains

maybe present with VHL disease

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16
Q

lymphangiomas

A

hamartomas or neoplastic

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17
Q

lymphangiectasis

A

dilation of lymph channels

diffuse swelling of extremity

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18
Q

lymph tumors in children

A

often in deep neck

-typically benign

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19
Q

ETV6-NTRK3

A

fusion transcript present in congenital-infantile fibrosarcomas

useful diagnostic marker

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20
Q

mature teratoma

A

benign

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21
Q

immature teratoma

A

malignant

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22
Q

2 peaks of teratomas in children

A

first at 2 years of age
-congenital neoplasma

second in late adolescence
-slower growing

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23
Q

sacrococcygeal teratoma

A

most common teratomas in children
-more often in female (4:1)

  • majority are benign
  • but malignant are highly aggressive

benign - usually infants less than 4 months

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24
Q

fibrosarcomas

A

bad prognosis in adults
-congenital-infantile variant has better prognosis

-presence of EVT6-NTRK3 fusion transcript

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25
Q

most deaths in children under age 15

A

leukemias

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26
Q

small round blue cell tumors

A

sheets of cells with small, round nuclei, and frequently show features of organogenesis specific to site of tumor origin

“-blastoma”

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27
Q

malignant neoplasms 0-4 years

A 
leukemia
retinoblastoma
neuroblastoma
hepatmblastoma
wilms tumor
soft tissue sarcoma (rhabdo)
teratomas
CNS tumors
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28
Q

malignant tumors 5-9 years

A 
leukemia
retinoblastoma
neuroblastoma
hepatocellular carcinoma
soft tissue sarcoma
CNS tumor
ewing sarcoma
lymphoma
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29
Q

period for wilms tumor

A

0-4 years

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30
Q

eriod for teratomas

A

0-4 years

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31
Q

malignant tumors 10-14 years

A 
hepatocellular carcinoma
soft tissue carcinoma
osteogenic sarcoma
thyroid carcinoma
hodgkin disease
32
Q

neuroblastoma

A

most common extracranial solid tumor of childhood

  • normal age at Dx 18 months
  • most sporadic
  • familial - bilateral or multifocal
33
Q

genetic predisposition to neuroblastoma

A

ALK

-anaplastic lymphoma kinase

34
Q

good prognosis for neuroblastoma

A 
less than 18 months old
stage 1, 2A, 2B, 4S
presence of schwannan stroma
hyperdiploid or neartriploid
TRKA presence
no N-myc
35
Q

TRKB

A

bad prognosis for neuroblastoma

36
Q

older than 18 months

A

bad prognosis for neuroblastoma

37
Q

N-MYC amplification

A

bad prognosis for neuroblastoma

trumps out all other good prognostic factors

38
Q

near-diplid

A

bad prognosis for neuroblastoma

39
Q

stage 3, 4

A

bad prognosis for neuroblastoma

40
Q

catecholamine release

A

from neuroblastoma

-but does not cause increased blood pressure

41
Q

pheochromocytoma

A

releases catecholamines

-but DOES cause increased blood pressure and HTN

42
Q

neuropil

A

neuritic processes

-in neuroblastomas

43
Q

homer-wright pseudorosettes

A

seen in neuroblastoma

-tumor cells around central space of neuropil

44
Q

presence of schwannian stroma

A

maturation of neuroblasts to ganglion cells

prerequisite for designation of ganglioneuroblastoma or gaglioneuroma

good prognosis

45
Q

ganglioneuroblastoma

A

ganglion cells and neuroblasts

46
Q

ganglioneuroma

A

large mature ganglion cells with little to no neuroblasts

47
Q

stage 4S

A

localized primary tumor with dissemination to skin, liver, bone marrow

infants younger than 1 year

seen to regress

48
Q

stage 1

A

localized with complete resection and negative ispi nodes

49
Q

stage 2A

A

localized with incomplete gross resection with negative ipsi nodes

50
Q

stage 2B

A

localized with positive ipsi nodes

51
Q

stage 3

A

infiltrating across midline

or localized with contra nodes

52
Q

stage 4

A

primary tumor with dissemination to distant nodes, bone, bone marrow, liver, skin, etc.

53
Q

presentation of neuroblastoma

A

large abdomen mass
fever
weigt loss

proptosis (periorbital met)
ecchymosis

blueberry muffin baby - cutaneous mets

54
Q

diagnosis of neuroblastoma

A

increased in serum catecholamines

increased in urine VMA and HVA

55
Q

chromosomes with negative prognosis

A

17q gain
1p loss
11q loss

56
Q

wilms tumor

A

most common primary renal tumor of childhood
-between age 2-5 year old

majority sporadic
minority familial (syndromic)
57
Q

most common malignant

A

B cell ALL - leukemia

58
Q

familial wilms tumors

A

if bilateral

-also earlier onset than sporadic

59
Q

WAGR syndrome

A

wilms tumor, aniridia, genital anomalies, retardation

involves WT1 gene and PAX6

60
Q

aniridia

A

absence of iris

61
Q

mutation in WAGR syndrome

A

deletion of 11p13

-WT1 and PAX6 genes

62
Q

WT1

A

increased susceptibility for wilms tumor

63
Q

PAX6

A

increased susceptibility for aniridia

64
Q

denys-drash syndrome

A

gonadal dysgenesis
-similar to WAGR

plus nephropathy and gonadoblastoma

65
Q

gonadoblastoma

A

in denys-drash syndrome

66
Q

beckwith-wiedemann syndrome

A

like WAGR and denys-drash

but with asociated hemihypertrophy

increased risk for liver, pancreas, skeletal m blastomas

67
Q

hemihypertrophy

A

in beckwith-wiedemann

68
Q

genetic of denys drash

A

dominant-negative missense mutation in zinc finger region of WT1 gene
-affects DNA -binding properties

69
Q

WT1

A

tumor suppressor gene

70
Q

genetic of beckwith-wiedemann

A

impronting of band 11p15.5 - WT2

distal to WT1

71
Q

loss of imprinting in wilms

A

overexpression of IGF-2

72
Q

gain of function mutation in beta-catenin

A

sporadic wilms tumors

73
Q

nephrogenic rests

A

precursor lesions to wilms tumor

74
Q

triphasic in wilms

A

blastemal
stromal
epithelial

75
Q

anaplasia

A

cells with large, hyperchromatic, pleomorphic nuclei and abnormal mitosis

76
Q

presentation of wilms

A

large abdominal masses

hematuria

pain

intestine obstruction

HTN

most patients cured
-but secondary tumors may arise - from therapy

PBD II Exam 1 (14 decks)

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