Abos Day 36 B 1-3

Front 1

Fig A & B- AP/lateral XRAYS 15yo boy who injured his ankle skateboarding. What is the MoI with this type of fx? 1 sup/add; 2 Ex rotatn; 3 In rotatin; 4 pron/abd; 5 axial load

Back 1

2 external rotation injury
triplane fracture that occurs in patients during early adolescence

Front 2

1 define the Triplane Fractures; 2 what part of bone & what plane are the 3 fx in triplane fx; 3 on xray see what on AP/L? 4 what other fx has similar fx, how?

Back 2

1 complex SH 4 (IV) fx pattern in all 3 planes; 2 metaphysis fx-coronal plane; epiphysis fx-sagittal plane (same as tillaux fracture); physis separtd- axial plane; 3
AP SH III/L SH II 4 ER-> tillaux fx

Front 3

Triplane Fractures 1 indications-CRPP vs ORIF 2 techniques during surgery?

Back 3

1 > 2 mm displacement 2 epiphyseal screw placed parallel to physis & arthroscopic aided reduction can be used

Front 4

What is the genetic cause of the dwarfism characterized by a hitchhikers thumb, cauliflower swelling of the ears, and severe clubfeet?

Back 4

ans-Sulfate transport protein; Diastrophic Dysplasia- form of dwarfism; hitchhikers thumb, cauliflower swelling of the ears, and severe clubfeet. AR- mutation in DTD gene--> def sulfate transporter prtn,-->undersulfation of cartlg proteoglycan.

Front 5

Fig A is a photograph of a newborn's hand. Genetic testing reveals-mutation in the SLC26A2 gene on Chrom 5q & both the parents were carriers. What condition does this child have?

Back 5

Ans:Diastrophic dysplasia. AR Clinical manif include: disproportionate-shrt-limb dwarfism (dx'd @ birth), dysmorphic ear lobes & palate, gen limit jnt movement, clubft, "Hitchhiker" thumb -short 1st mc & radial sublxn MCPJ

Front 6

3yo child with short-limbed dwarfism clinical features Fig A&B. What pathoanatomy dz? 1 Sulfate transprt prtn gene mutn. aff cartg matrix formtn 2 Mutn gene CBFA-1 affecting Ocalcin formn 3 Defct lysosomal -strg 4 Defct lipid-strg 5 Mutn gene FGFR-3

Back 6

1 Sulfate transprt prtn gene mutn. aff cartg matrix formtn; defct in SLC26A2 gene-> sulfate transpr->under-sulfation of proteoglycans-> cartilage matrix & abn hydraulic proprts of cartilage;strctrs -trachea, ear, lig. pinnae ears-cauliflower ear.

Front 7

Diastrophic Dysplasia
1 defition of condition; 2 genetic defct; 3 zone affect
4 Sx-assoc; PE-I/P; provc; n/v E; 5 xray findings; 6 Tx

Back 7

1 short-limbed dwarfism aka twisted dwarf-> failure of formtn 2ry ossification cntr (epiphysis), progressive; 2 defct SLC26A2 gene->Sulfate transprt prtn mutn ->cartg matrix formtn;

Front 8

Diastrophic Dysplasia
3 zone affect
4 Sx-assoc; PE-I/P; provc; n/v E; 5 xray findings; 6 Tx

Back 8

3 spongiosa 4 intelligence nor; short stature ("twisted dwarf"), cleft palate, cauliflower ears , poorly devlpd UE, hitchhikers thumb,

Front 9

Diastrophic Dysplasia
5 xray findings; 6 Tx

Back 9

5 thoracolumbar scoli, sev cervical kyphosis, hip/knee contrctres, genu valgum, rigid clubft (equinocavovarus)
6 ASF / PSF, occipital-cervical fusion-AAI w/neurologic sx; osteotomies for corrctn; ST surgi relse

Front 10

3yo Afrcn-Amer child w/ irritability, fever, warm, swollen leg. xray shows-osteomyelitis, trans-cortical bx reveals multp Salmonella. This child has which condtins? 1 OI; 2 Child abuse; 3 Thalassemia; 4 Sickle cell A 5. Renal failure

Back 10

3 Sickle cell anemia; Salmonella osteomyelitis is assocd w/ SCA. MC cause osteomyelitis SCA is ->Staph A. MC spread of Salmonella from the GI tract. dactylitis (acute hand / foot swelling) SCA 2 abn hemoglobin S alleles;

Front 11

Hematogenous osteomyelitis caused by Salmonella is MC in which of the following pt popltn? 1 Neonates 2 IV drug abusers; 3 Patients with sca dz; 4 Pts w/ chronic kidney failr req HD 5 pts w/ puncture wounds through athletic shoe

Back 11

3 pts w/sca dz; Once in the blood, sickle cell dz pts have a decr ability to fight infect 2^functional asplenia & impaird complement activity; bone infarcts common-fem; biconcave "fishtale" vertebrae

Front 12

12yo boy w/ SCA c/o 24 hrs pain in R thigh. denies traumatic inj. R leg has tend w/palp mid-thigh. Pt's temp.100.2, ESR 45. xrays fem unremarkable. radionuclide bone scan- abn uptake in the mid-femur. what's best step in mangt?

Back 12

Sx care for pain w/ NWB crutches, IV hydration, & conslt w/ peds. SCA crises bone infarcts difficult diff from acute osteo. Radionuclide bone marrw & bone scan DDX osteomyelitis vs bone infarct. sickle cell trait usu asymptc