Skel D part II

Question 1

what is abnormal is sickle cell anemia

Answer 1

Hb S

Question 2

what causes the sickling phenomenon

Answer 2

substitution of valine for glutamic acid on beta globulin chain

Question 3

what is true SCA

Answer 3

sickle cell anemia

homozygous HbSS

Question 4

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

Answer 4

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

Question 5

common pathology associated with SCA

Answer 5

ACN of femoral and humeral heads

anemia–> spenic enlargement then atrophy

Question 6

what is also known as sickle cell crisis

Answer 6

mesenteric infarcts

Question 7

common xray features of SCA

Answer 7

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

Question 8

aka for vertebral shape in SCA

Answer 8

fish vertebra
h shaped
step down sign
reynold phenomenon

Question 9

what infection are SCA patients prone to? where? apperance

Answer 9

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

Question 10

thalassemia aka

Answer 10

cooley anemia

Mediterranean anemia

Question 11

how many types of thalassemia are there

Answer 11

3
Major (die age 3-4)
Minor
Intermediate

Question 12

what is the cause of thalassemia

Answer 12

imbalance globulin chain production (alpha/beta)

Question 13

what type of cells are present with thalassemia

Answer 13

hypochomic and microcytic

Question 14

s/s associated with thalassemia

Answer 14

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

Question 15

xray findings with thalassemia

Answer 15

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

Question 16

vertebral changes in thalassemia

Answer 16

coarse trabeculae but endplate remains

Question 17

thalassemia of the skull characteristics

Answer 17

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

Question 18

what other things can be associated with thalassemia

Answer 18

cardiomegaly

opaque liver dt hemosiderin accumulation

Question 19

most common hemophilia

Answer 19

classic= hemophilia A, Factor VIII

Christmas disease= Hemophilia B, Factor IX

Question 20

how is hemophilia manifested clinically

Answer 20

males but is passed from the mother

Question 21

what are xray features of hemophilia

Answer 21

bleeding around joints and organs

80% around organs

Question 22

what is the most common malignant childhood disease

Answer 22

leukemia

Question 23

what is the peak age for leukemia

Answer 23

2-5

Question 24

what is the most common for of leukemia

Answer 24

acute lymphocytic (death within a year of Dx)

Question 25

what are some s/s leukemia

Answer 25

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

Question 26

a key xray finding in childhood leukemia

Answer 26

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

Question 27

most common periosteal reaction in child leukemia

Answer 27

laminated

Question 28

which type of adult leukemia has xray changes and why

Answer 28

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

Question 29

which type of chronic adult leukeima has xray changes

Answer 29

chronic lyphatic leukemia

Question 30

what is the most consistent xray finding adult leukemia

Answer 30

diffuse osteoporosis in spine

Question 31

aka for achondroplasia

Answer 31

chondrodystrophic dwarfism | chondrodystrophia fetalis

Question 32

clinical s/s achondroplasia

Answer 32

``` 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 ```

Question 33

xray characteristics of achondroplasia

Answer 33

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