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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
most deaths in children under age 15
leukemias
26
small round blue cell tumors
sheets of cells with small, round nuclei, and frequently show features of organogenesis specific to site of tumor origin "-blastoma"
27
malignant neoplasms 0-4 years
``` leukemia retinoblastoma neuroblastoma hepatmblastoma wilms tumor soft tissue sarcoma (rhabdo) teratomas CNS tumors ```
28
malignant tumors 5-9 years
``` leukemia retinoblastoma neuroblastoma hepatocellular carcinoma soft tissue sarcoma CNS tumor ewing sarcoma lymphoma ```
29
period for wilms tumor
0-4 years
30
eriod for teratomas
0-4 years
31
malignant tumors 10-14 years
``` hepatocellular carcinoma soft tissue carcinoma osteogenic sarcoma thyroid carcinoma hodgkin disease ```
32
neuroblastoma
most common extracranial solid tumor of childhood - normal age at Dx 18 months - most sporadic - familial - bilateral or multifocal
33
genetic predisposition to neuroblastoma
ALK | -anaplastic lymphoma kinase
34
good prognosis for neuroblastoma
``` less than 18 months old stage 1, 2A, 2B, 4S presence of schwannan stroma hyperdiploid or neartriploid TRKA presence no N-myc ```
35
TRKB
bad prognosis for neuroblastoma
36
older than 18 months
bad prognosis for neuroblastoma
37
N-MYC amplification
bad prognosis for neuroblastoma trumps out all other good prognostic factors
38
near-diplid
bad prognosis for neuroblastoma
39
stage 3, 4
bad prognosis for neuroblastoma
40
catecholamine release
from neuroblastoma | -but does not cause increased blood pressure
41
pheochromocytoma
releases catecholamines | -but DOES cause increased blood pressure and HTN
42
neuropil
neuritic processes | -in neuroblastomas
43
homer-wright pseudorosettes
seen in neuroblastoma | -tumor cells around central space of neuropil
44
presence of schwannian stroma
maturation of neuroblasts to ganglion cells prerequisite for designation of ganglioneuroblastoma or gaglioneuroma good prognosis
45
ganglioneuroblastoma
ganglion cells and neuroblasts
46
ganglioneuroma
large mature ganglion cells with little to no neuroblasts
47
stage 4S
localized primary tumor with dissemination to skin, liver, bone marrow infants younger than 1 year seen to regress
48
stage 1
localized with complete resection and negative ispi nodes
49
stage 2A
localized with incomplete gross resection with negative ipsi nodes
50
stage 2B
localized with positive ipsi nodes
51
stage 3
infiltrating across midline or localized with contra nodes
52
stage 4
primary tumor with dissemination to distant nodes, bone, bone marrow, liver, skin, etc.
53
presentation of neuroblastoma
large abdomen mass fever weigt loss proptosis (periorbital met) ecchymosis blueberry muffin baby - cutaneous mets
54
diagnosis of neuroblastoma
increased in serum catecholamines increased in urine VMA and HVA
55
chromosomes with negative prognosis
17q gain 1p loss 11q loss
56
wilms tumor
most common primary renal tumor of childhood -between age 2-5 year old ``` majority sporadic minority familial (syndromic) ```
57
most common malignant
B cell ALL - leukemia
58
familial wilms tumors
if bilateral | -also earlier onset than sporadic
59
WAGR syndrome
wilms tumor, aniridia, genital anomalies, retardation involves WT1 gene and PAX6
60
aniridia
absence of iris
61
mutation in WAGR syndrome
deletion of 11p13 | -WT1 and PAX6 genes
62
WT1
increased susceptibility for wilms tumor
63
PAX6
increased susceptibility for aniridia
64
denys-drash syndrome
gonadal dysgenesis -similar to WAGR plus nephropathy and gonadoblastoma
65
gonadoblastoma
in denys-drash syndrome
66
beckwith-wiedemann syndrome
like WAGR and denys-drash but with asociated hemihypertrophy increased risk for liver, pancreas, skeletal m blastomas
67
hemihypertrophy
in beckwith-wiedemann
68
genetic of denys drash
dominant-negative missense mutation in zinc finger region of WT1 gene -affects DNA -binding properties
69
WT1
tumor suppressor gene
70
genetic of beckwith-wiedemann
impronting of band 11p15.5 - WT2 distal to WT1
71
loss of imprinting in wilms
overexpression of IGF-2
72
gain of function mutation in beta-catenin
sporadic wilms tumors
73
nephrogenic rests
precursor lesions to wilms tumor
74
triphasic in wilms
blastemal stromal epithelial
75
anaplasia
cells with large, hyperchromatic, pleomorphic nuclei and abnormal mitosis
76
presentation of wilms
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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