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Pathology 2 > 3.4 Peds 2 > Flashcards

3.4 Peds 2 Flashcards

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1
Q

APGAR

A 
appearance, 
pulse, 
grimace, 
activity, 
respiration
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2
Q

APGAR score

A

a physiologic assessment tied to probability of survival

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3
Q

apgar scoring

A

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

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4
Q

chart

A

on slide 3

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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

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6
Q

score of 7 or more

A

almost 0% mortality

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7
Q

apgar score correlates with

A

survival NOT normal life thereafter

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8
Q

Fetal Hydrops

A

accumulation of edema in fetus during IU growth.

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9
Q

hydrops fetalis

A

progressive generalized

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10
Q

cystic hygroma

A

localized

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11
Q

fetal hydrops can be classified as

A

immune (mother ab to fetal RBC) and

non immune hydrops (most common)

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12
Q

immune hydrops

A

hemolytic disease due to blood group incompatability btw mother/fetus

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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)

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14
Q

C D and E ag

A

cause of incompatibility but most common is C

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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

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16
Q

clinical presentations of erythroblastosis fetalsis

A 
hemolytic anemia, 
congestive heart failure, 
hepatospenomegaly, 
edema, 
jaundice, 
kernicterus
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17
Q

kernicterus

A

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

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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

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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

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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

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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

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22
Q

cystic hygroma

A

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

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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

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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

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25
immune hydrops - treatment for ABO
most have mild jaundice, erythroblastosis, is uncommon. Phototherapy. Exchange transfusion in very rare cases
26
nonimmune hydrops causes
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)
27
what is the most common infectious cause of nonimmun hydrops
Parvovirus B19
28
SIDS
``` sudden unexplained death of an infant less than 1 yr of age after a thorough scene investigation, autopsy, and review of clinical record ``` (NICHHD)
29
SIDS Dx
of exclusion -- negative death scene, negative autopsy, review clinical Hx.
30
Unclassified sudden infant death
not SIDS bc we couldn't review the other factors.
31
most common cause of death btw birth and 1 mont
chromosomal anomalies
32
most common cause of death btw 1month and 1yr
SIDS
33
SIDS and death
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
34
SIDS pathogenesis
Multifactorial, triple risk infant model
35
triple risk infant modle
vulnerable infant that during critical period of development period of homeostatic controls are exposed to exogenous stressor
36
hypothesis of sids
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.
37
SIDS risk factors - prenatal
maternal pregnancy -- 2x the risk
38
SIDS risk factors - infant
SIDS in a prior sibling -- 5x the risk
39
SIDS risk factors - environment
prone sleeping position -- most important MODIFIABLE risk factor
40
post mortem abnormalities in cases of Sudden unexpected infant death
infections, unexpected congenital nomaly, traumatic child abuse, genetic metabolic defects
41
sudden unexpected infant death - infections
viral myocarditis, | bronchopnemonia
42
slide 22
details
43
SIDS autopsy findings
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
SIDS environmental stressors
prone (or side) sleeping (hypoxia, hypercarbia, thermal stress), sleeping with parent during 1st 3 months, sleeping on soft surfaces
45
dec in SIDS
"back to sleep campaign" --AAP says now the supine spleeing postions is the only safe postion to dec the risk of SIDS
46
Tumors of Tumor-like lesions of infancy and childhood clinical scenarios
tumor as chief complaint, incidental, tumor related manifestations
47
with tumors you need to do
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
tumors in infancy and childhood
2%
49
malignant tumors 4-14 yo
9%
50
much more common tumors
benign
51
heterotopia and choristoma
normal cells from a tissue type in the wrong place (eg. Pancreas in the stomach wall)
52
hamartoma
focal overgrowth of cells and tissues native to the organ where occurs (eg. Mass of cartilage in lung) Normal cells, abnormal architecture
53
benign tumor - Hemangioma
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
benign tumor - Lymphangioma
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
markers for lymph and hemangioma
CD31 and 34
56
markers for only lymphangioma
D2-40
57
fibrous tumor
may occur in children from spalrsely cellular (fibromatosis) to those that resemble adult fibrosarcomas (hypercelluar)
58
congenital infantile fibrosarcoma
diagnostic chromosomal translocation T(12;15) (p13q25)-->ETV6-NTRK3 fusion. Rapid growth with extensive local invasion but low metastatic rate.
59
Teratomas
may occur as benign, well differentiated cystic (mature teratoma 75%) as indeterminante potential (immature), or frankly malignant tumors (12%)
60
age peaks of teratomas
2yrs and late adolesens
61
region of teratomas
saccrococcygeal area -- 40% of childhood teratomas, more common in girls. 10% associated with other defects.
62
benign teratomas
younger the patient the more likely benign <4 months
63
areas of teratomas
testes, ovary, midline (mediastainum, retroperitoneal, head and neck)
64
malignant tumors
malignancies in childhood differ biologically and histologic from the adult counterpart.
65
differences bt child and adult malignancyes
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
most common inchildren
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
leukemia
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
histology of tumors
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
neuroblastoma
n-myc amplification, | neuron specifce enolase (NSE) expression
70
ewing sarcoma/PNET
translocation 11;22--CD99
71
rhabdomyosarcoma
translocation 3:13 - myogenin and Myo D1
72
burkitt lymphoma
t(8;14) --cd 19, cd20, cd10 -- becell phenotype
73
retinoblastoma
13q14 RB deletion/mutation--retinal S ag
74
Medullopastoma
17p deletion--synpatophysin expression GFAP exp
75
Neuroblastoma
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
baby 2 or 3 yo with abdominal mass
it may be neuroblastoma so rule it out
77
neuroblastoma size
minet lesions, can regress but can be >1kg
78
neuroblastoma gross
some well circumscribed, but others more infiltrative. Necrosis, cyst, hemorrhage, calcifications
79
neuroblastoma histology
``` 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
progression of neuroblastoma with better prognosis
neuroblastoma --> ganglioneuroblastoma --> ganglioneuroma
81
neuroblastoma spread
local infiltrate and LN spread. | Blood stream to liver, lung, BM, bone
82
neuroblastoma clinical manifestations
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
neurobalstoma screening
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
Prognostic factors in Neuroblastoma that are unfavourable
stage 3 and 4, >1yr, DNA diploid, N-myc at high levels
85
treatment of nephrobalstoma
``` surgery, chemo, radiation, retinoikd, TRKB inhibitor ```
86
Wilms tumor
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
WAGR syndrome
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
Denys-Drash syndrom
godandal dysgenesis, early onset nephropaty, dominant negative missence mutation in WT1, 90% risk of Wilms
89
Bekwith-Wiedmann syndrome
``` 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
nephrogenic rests
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
Wilms tumor presentation
``` 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
incicence of wilms tumor
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)

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