Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology 2 > 3.4 Peds 2 > Flashcards

3.4 Peds 2 Flashcards

1
Q

APGAR

A 
appearance, 
pulse, 
grimace, 
activity, 
respiration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

APGAR score

A

a physiologic assessment tied to probability of survival

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

apgar scoring

A

scored at 1 and 5 minutes following birth.
5 functions evaluated scored from 0-2.
perfect score of 10.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

chart

A

on slide 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

if apgar scre is 0 to 1 at 5 min

A

50% mortality in first 28 days.

Drops to 20% with a score of 4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

score of 7 or more

A

almost 0% mortality

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

apgar score correlates with

A

survival NOT normal life thereafter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Fetal Hydrops

A

accumulation of edema in fetus during IU growth.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

hydrops fetalis

A

progressive generalized

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

cystic hygroma

A

localized

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

fetal hydrops can be classified as

A

immune (mother ab to fetal RBC) and

non immune hydrops (most common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

immune hydrops

A

hemolytic disease due to blood group incompatability btw mother/fetus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

most common cause of immune hydrops

A

Rh incompatablitty – most due to classic Rh incompatibility btw Rh (-) mother and 2nd Rh(+) child (D ag)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

C D and E ag

A

cause of incompatibility but most common is C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

response of mother

A

1st Rh+ child to Rh- mother can sensitize late in pregnancy when barrier cytotrophoblast not present.

1st child evokes 1gM Abs but they cant cross placenta so no disease.

2nd Rh+ child evokes IgG that can cross so you see the disease damaging fetal RBCs causing hemolytic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

clinical presentations of erythroblastosis fetalsis

A 
hemolytic anemia, 
congestive heart failure, 
hepatospenomegaly, 
edema, 
jaundice, 
kernicterus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

kernicterus

A

biliruben in the brain - neural damage usually with billirubin >20mg/dl

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

immune hydrops - factors that influence immune response to Rh+ fetal RBC

A

concurrent ABO incompatibility can prevent sensitization to Rh,
maternal response is dose dependent.
Needs more than 1ml of fetal cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

immune hydrops - prevention

A

administration of rhesus anti-D globulin (Rhogam) to mothers at 28w and w/in 72hrs of delivery greatly reduced rate of immune hydrops.
Also following abortions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

immune hydrops - antenatal identification of at risk fetus

A 
blood typing, 
indirect coombs. 
Ab screening, 
amniocentesis, 
chronic villi and fetal blood sampling 
(fetal genotyping),

now NIPD -non invasive prenatal diagnosis - RHD genotyping of fetuses using maternal blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

immune hydrops - treatment

A

if hemolysis occurs inutero, treat by IU transfusion.

Delivery (if mature), phenobarbital.

Phototherapy (reduce bilirubine to a way to be excreted) and

exchange transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

cystic hygroma

A

commonly occurs in neck, abdomen and associated with Turner’s syndrome (45X)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

immune hydrops -maternal fetal ABO incompatibility

A

mild, 20-25% of pregnancies,
lab evidence of hemolytic disease in 1/10,
requre treatment in 1/200 cases,

bc of low rh incompatibiliyt, abo incomatability became the main cause of hemolytic disease of the new born,

some “O” motheres have preformed IgG anti A and B, therefor first born can be affected, no effective prophylaxis against ABO rxn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

immune hydrops - why low incided of hydrops in ABO incompatibility?

A

most anti A and B are IgM. Do not corss the placenta. Fetal cells express AgA and B poorly. Most other cells also express AgA and B so absorb some transferred Abs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

immune hydrops - treatment for ABO

A

most have mild jaundice, erythroblastosis, is uncommon.

Phototherapy.
Exchange transfusion in very rare cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

nonimmune hydrops causes

A

cardiovascular defects (IU cardiac failure),

chormosomal anomalies (turner and lymphatic drainage abnormalities. Tri21 and Tre28),

fetal non-immune anemia (homozygous alpha thalassemia in Southeast asia, also Parvovirus B19)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what is the most common infectious cause of nonimmun hydrops

A

Parvovirus B19

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

SIDS

A 
sudden unexplained death of an infant 
less than 1 yr of age after a 
thorough scene investigation, 
autopsy, and 
review of clinical record

(NICHHD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

SIDS Dx

A

of exclusion
– negative death scene,
negative autopsy,
review clinical Hx.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Unclassified sudden infant death

A

not SIDS bc we couldn’t review the other factors.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

most common cause of death btw birth and 1 mont

A

chromosomal anomalies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

most common cause of death btw 1month and 1yr

A

SIDS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

SIDS and death

A

3rd leading overall behind congenital anomalies and complications of prematurity.

90% occur in 1st 6mo, btw 2-4 mo, usually occurs at home, at night, after or during sleep, rarely observed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

SIDS pathogenesis

A

Multifactorial, triple risk infant model

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

triple risk infant modle

A

vulnerable infant that during critical period of development period of homeostatic controls are exposed to exogenous stressor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

hypothesis of sids

A

a delayed development of arousal and cardiorespiratory control.

Brainstem abnormalities (medulla oblongata) which control arousal response to hypoxia/thermal stress.

Dec serotonergic and muscarinic receptor binding in brain stem.

Mice w. mutations in Krox20 homeobox gene involved in bran stem developemnt have prolonged apnea.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

SIDS risk factors - prenatal

A

maternal pregnancy – 2x the risk

38
Q

SIDS risk factors - infant

A

SIDS in a prior sibling – 5x the risk

39
Q

SIDS risk factors - environment

A

prone sleeping position – most important MODIFIABLE risk factor

40
Q

post mortem abnormalities in cases of Sudden unexpected infant death

A

infections,
unexpected congenital nomaly,
traumatic child abuse,
genetic metabolic defects

41
Q

sudden unexpected infant death - infections

A

viral myocarditis,

bronchopnemonia

42
Q

slide 22

A

details

43
Q

SIDS autopsy findings

A

subtle,

petechia over thymus, pericardium, pleura (80%),

congestion of lungs and pulmonary edema (found in other causes of death),

possibly lingering evidence of mild upper respiratory infection,

hypoplasia of aruate nucleus in brainstem or decrease in other brain stem neuron groups (morphometric sudies),

no explanation of death

44
Q

SIDS environmental stressors

A

prone (or side) sleeping (hypoxia, hypercarbia, thermal stress),

sleeping with parent during 1st 3 months, sleeping on soft surfaces

45
Q

dec in SIDS

A

“back to sleep campaign” –AAP says now the supine spleeing postions is the only safe postion to dec the risk of SIDS

46
Q

Tumors of Tumor-like lesions of infancy and childhood clinical scenarios

A

tumor as chief complaint,
incidental,
tumor related manifestations

47
Q

with tumors you need to do

A

immaging studies and biopsy (only test to tell you type of tumor and if benign or malignant) and then you can try to treat the pt

48
Q

tumors in infancy and childhood

A

2%

49
Q

malignant tumors 4-14 yo

A

9%

50
Q

much more common tumors

A

benign

51
Q

heterotopia and choristoma

A

normal cells from a tissue type in the wrong place (eg. Pancreas in the stomach wall)

52
Q

hamartoma

A

focal overgrowth of cells and tissues native to the organ where occurs (eg. Mass of cartilage in lung)

Normal cells, abnormal architecture

53
Q

benign tumor - Hemangioma

A

most common tumor of infancy.
Cavernous and capillary.
Skin, face, scalp. “port wine stains”.
Enlarge or spontaneous regression.
Cosmetic significance.
Vonhipple-lindeau syndrome (hemangio blastoma in brain, cerebellum or kidney and can be compsed of renal cell carcinoma nd tumors in other areas),
subset of of CNS cavernous hemangioms with cerebral cavernos malformation (CCM) gene mutations

54
Q

benign tumor - Lymphangioma

A

cystic and cavernos spaces.
Subcutaneous or in deeper regions (neck, axilla, mediastinum).
Occasionaly enourmous, common in Turner syndrome.
See them in lymphatic spaces and endothelial lining

55
Q

markers for lymph and hemangioma

A

CD31 and 34

56
Q

markers for only lymphangioma

A

D2-40

57
Q

fibrous tumor

A

may occur in children from spalrsely cellular (fibromatosis) to those that resemble adult fibrosarcomas (hypercelluar)

58
Q

congenital infantile fibrosarcoma

A

diagnostic chromosomal translocation
T(12;15) (p13q25)–>ETV6-NTRK3 fusion.

Rapid growth with extensive local invasion but low metastatic rate.

59
Q

Teratomas

A

may occur as benign,
well differentiated cystic (mature teratoma 75%) as
indeterminante potential (immature), or
frankly malignant tumors (12%)

60
Q

age peaks of teratomas

A

2yrs and late adolesens

61
Q

region of teratomas

A

saccrococcygeal area – 40% of childhood teratomas,

more common in girls.

10% associated with other defects.

62
Q

benign teratomas

A

younger the patient the more likely benign <4 months

63
Q

areas of teratomas

A

testes, ovary, midline (mediastainum, retroperitoneal, head and neck)

64
Q

malignant tumors

A

malignancies in childhood differ biologically and histologic from the adult counterpart.

65
Q

differences bt child and adult malignancyes

A

type, incidence, biologic behaivour, prognosis (most comm hematopogenesis, and CNS -juvenille astrocytoma, or pilocytic astrocytoma, meduloblastoma, epyndoma in th 4th ventricle but in adult in SC),
prevalence of underlying familial or genetic aberrations,
tendency to regress spontaneously or cyto differentiate

66
Q

most common inchildren

A

leukemia,
CNS (ependymoma, meduloblastoma (medulla of adrenal gland), juvenile astrocytoma),
Wilms tumor (most common renal),
hepatoblastoma,
rhabdomyosarcomma,
Ewing sarcoma (2nd most common after bone in 5-9yo)

67
Q

leukemia

A

mostly acute lymphoblastic leukemia accounts for more deaths than all other tumors comined.

Although common in children leukemia is similar to adult forms so discussed elsewhere as are soft tissue tumors like rabdomyosarcoma

68
Q

histology of tumors

A

mony childhood tumors are immature (primitive cells) rather than peomorphic-anaplastic (adult).

Tumors similar in appearance called colllectivley small round blue cell tumors.

Many desiganted as blastoma –nephroplastoma, AKA Wilm’s, hepatoblastoma, neuroblastoma

69
Q

neuroblastoma

A

n-myc amplification,

neuron specifce enolase (NSE) expression

70
Q

ewing sarcoma/PNET

A

translocation 11;22–CD99

71
Q

rhabdomyosarcoma

A

translocation 3:13 - myogenin and Myo D1

72
Q

burkitt lymphoma

A

t(8;14) –cd 19, cd20, cd10 – becell phenotype

73
Q

retinoblastoma

A

13q14 RB deletion/mutation–retinal S ag

74
Q

Medullopastoma

A

17p deletion–synpatophysin expression GFAP exp

75
Q

Neuroblastoma

A

most common neuroblastic tumors are derived from sympathetic ganglia and adrenal medulla,

themost common extracranial solid malignant tumor of childhood,

the most common diagnosed solid tumor in infancy,

age of dx 18mo,

white>black ppl,

76
Q

baby 2 or 3 yo with abdominal mass

A

it may be neuroblastoma so rule it out

77
Q

neuroblastoma size

A

minet lesions, can regress but can be >1kg

78
Q

neuroblastoma gross

A

some well circumscribed, but others more infiltrative. Necrosis, cyst, hemorrhage, calcifications

79
Q

neuroblastoma histology

A 
small round blue celsl (SBRC). 
Mitotic activity pleomorphism (=/-). 
Fibrillary material in stroma. 
Homer Right pseudorosetts. 
Neuron specifec enolase (NSE)+. 
Neuro secretory granules on EM. 
Presence of ganglion and Schwann cells has favorable prognosis.
80
Q

progression of neuroblastoma with better prognosis

A

neuroblastoma –>
ganglioneuroblastoma –>
ganglioneuroma

81
Q

neuroblastoma spread

A

local infiltrate and LN spread.

Blood stream to liver, lung, BM, bone

82
Q

neuroblastoma clinical manifestations

A

abdoinal mass (firm irregualr non tender) less than 2yo with fever.

Metastasis may be first manifest with 
bone pain,
 respiratory or 
GI sypmtoms, or 
on the skin "bluberry muffin baby.

90% secrete catecholamines but hypertension is less frequent in Pheochromocytoma.

Catecholamine metabolites vanillymandelic acid (VMA) and homovanillic acid (HVA) are increased in urine

83
Q

neurobalstoma screening

A

bc catecholamins are secreted by most, this can be used as screeing or monitoring progress.

Prognosis is variable.

Low intermediate or high risk groups based on numerous factors.

84
Q

Prognostic factors in Neuroblastoma that are unfavourable

A

stage 3 and 4,
>1yr,
DNA diploid,
N-myc at high levels

85
Q

treatment of nephrobalstoma

A 
surgery, 
chemo, 
radiation,
retinoikd, 
TRKB inhibitor
86
Q

Wilms tumor

A

most common primary renal tumor and 4th most common pediatruc maligancy in us,

most in <15yo usually 2-5,

5-10% will involve both kidneys (synchronous) or
one after other (metachronus),
bilateral present earlier in age,

minority (10%) associated with congenital malformation syndromes (syndromic tumros)

87
Q

WAGR syndrome

A

aniridia,
genital anomalies and
mental retardation,

33% chance of getting Wilms,

deletion of 11p13, 11p13 has
Wilms tumor supprssor WT1 and PAX6 gene (involved in aniridia)

88
Q

Denys-Drash syndrom

A

godandal dysgenesis,
early onset nephropaty,
dominant negative missence mutation in WT1,
90% risk of Wilms

89
Q

Bekwith-Wiedmann syndrome

A 
organomegaly, 
omphalocele, 
adrenal cytomgaly, 
Loss of 11p15.5 (WT2) distal to WT1, 
also risk of hepato/pancreatobalstoma, 
adrenocortical tumors, and 
rhabdomyosarcoma, 
beta catenin gene mutation found in 10% of sporadic WT, 
Most Wilms tumors are not symdromic
90
Q

nephrogenic rests

A

through the precursor lesions and found in 40% of unilateral and 100% of bilateral.

Inportant to document in ressected specimen bc may be a predictive marker of occurrence in C/L kidney

91
Q

Wilms tumor presentation

A 
abcominal mass, 
meaturia, 
abdominal pain, 
obsturction, 
HTN, 
pulmonary metastasis often present at Dx,

prognosis is good with surgery and chemo,
2yr survival even if spread,

presence of diffuse anaplasia has poor prognosis

92
Q

incicence of wilms tumor

A

high incidence of 2nd neoplasms,

bone, soft tissue sarc, leuk, lymph, breast ca, some germline mutations, other concequences of therapy (radation)

Pathology 2 (15 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions