Skel D part II Flashcards

1
Q

what is abnormal is sickle cell anemia

A

Hb S

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2
Q

what causes the sickling phenomenon

A

substitution of valine for glutamic acid on beta globulin chain

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3
Q

what is true SCA

A

sickle cell anemia

homozygous HbSS

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4
Q

what is the initial sign of SCA? and when do they manifest

A

hand/foot syndrome:
painful swelling dt infarct or salmonella infection
6 months

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5
Q

common pathology associated with SCA

A

ACN of femoral and humeral heads

anemia–> spenic enlargement then atrophy

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6
Q

what is also known as sickle cell crisis

A

mesenteric infarcts

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7
Q

common xray features of SCA

A
honeycombing of cortex--> hair on end appearance in skull
signs of AVN
 H shaped vertebra
splenic calcifications (30%)
extra medullary hematopoiesis
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8
Q

aka for vertebral shape in SCA

A

fish vertebra
h shaped
step down sign
reynold phenomenon

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9
Q

what infection are SCA patients prone to? where? apperance

A

salmonella (100x)
femur, tibia,fibula, humerus, radius, ulna, spine
bilateral, symmetric and in diaphysis
motheaten destruction and periosteal reaction

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10
Q

thalassemia aka

A

cooley anemia

Mediterranean anemia

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11
Q

how many types of thalassemia are there

A

3
Major (die age 3-4)
Minor
Intermediate

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12
Q

what is the cause of thalassemia

A

imbalance globulin chain production (alpha/beta)

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13
Q

what type of cells are present with thalassemia

A

hypochomic and microcytic

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14
Q

s/s associated with thalassemia

A
joint pain
scoliosis
LBP
pallor, lethargy
rodent face (maxillary over growth)
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15
Q

xray findings with thalassemia

A
coarse trabeculae (honey combing)+ thin cortex
erlenmeyer flask deformity from lack of normal metaphyseal diaphysela concave constriction
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16
Q

vertebral changes in thalassemia

A

coarse trabeculae but endplate remains

17
Q

thalassemia of the skull characteristics

A
hair on end appearance
lack definition outer table
lack pneumatization  (create air cells) of sinuses EXCEPT ethmoid
18
Q

what other things can be associated with thalassemia

A

cardiomegaly

opaque liver dt hemosiderin accumulation

19
Q

most common hemophilia

A

classic= hemophilia A, Factor VIII

Christmas disease= Hemophilia B, Factor IX

20
Q

how is hemophilia manifested clinically

A

males but is passed from the mother

21
Q

what are xray features of hemophilia

A

bleeding around joints and organs

80% around organs

22
Q

what is the most common malignant childhood disease

A

leukemia

23
Q

what is the peak age for leukemia

A

2-5

24
Q

what is the most common for of leukemia

A

acute lymphocytic (death within a year of Dx)

25
Q

what are some s/s leukemia

A

generalized jt pain, weakness, lethargy, pallor, loss of appetite, easy brusing

26
Q

a key xray finding in childhood leukemia

A

radiolucent submetaphyseal bands
bilateral and symmetric
bone destruction metaphysis–>diaphysis

27
Q

most common periosteal reaction in child leukemia

A

laminated

28
Q

which type of adult leukemia has xray changes and why

A

chronic because in acute cases patients often die before sings show up

29
Q

which type of chronic adult leukeima has xray changes

A

chronic lyphatic leukemia

30
Q

what is the most consistent xray finding adult leukemia

A

diffuse osteoporosis in spine

31
Q

aka for achondroplasia

A

chondrodystrophic dwarfism

chondrodystrophia fetalis

32
Q

clinical s/s achondroplasia

A
rhizomelic dwarf (torso is normal, extremities are short)
waddling gait
lumbar hyperlordosis
large head prominent forehead
depressed nasal bridge
normal spinal length
trident hand
back pain
spinal stenosis
33
Q

xray characteristics of achondroplasia

A
proximal limb shortening with wide shafts
champagne glass pelvis
narrow thorax
posterior vertebral body scalloping 
narrowed interpedicular distance 
trident hand