FRCS Paediatric Ortho — improved notes
FRCS Paediatric Ortho — visual inline notes
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069. Morquio – most likely survive into adulthood

visual-inline rebuild1 extracted figuresspeaker notes XML22 study lines2 Q&A

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Clean FRCS notes with inline figures

Morquio – most likely survive into adulthood

Clinical photograph
Fig 1. Clinical photographtop left
•Accumulation of keratan sulphate
•Presents at 18 - 24months
•Type A (galactosamine 6 sulphate sulphatase deficiency) – more severe
•Type B (beta galactosidase deficiency)
•AR

Px:

•Proportionate dwarfism
•Corneal clouding
•Normal IQ
•Waddling gait
•Genu valgum
•Thoracic kyphosis

XR:

•Thickened skull
•Wide ribs
•Vertebral beaking
•Coxa vara with unossified femoral head
•Bullet shaped metacarpals
•Odontoid hypoplasia leading to cervical instability
•Thoracic kyphosis with platyspondia
•Corneal clouding in Morquio and Hurler

Direct Q&A

Q1 What are the radiographic findings for Morquio – most likely survive into adulthood?

Thickened skull

•Wide ribs
•Vertebral beaking
•Coxa vara with unossified femoral head
•Bullet shaped metacarpals
•Odontoid hypoplasia leading to cervical instability
•Thoracic kyphosis with platyspondia
•Corneal clouding in Morquio and Hurler
Q2 How is Morquio – most likely survive into adulthood managed?

Morquio – most likely survive into adulthood

•Accumulation of keratan sulphate
•Presents at 18 - 24months
•Type A (galactosamine 6 sulphate sulphatase deficiency) – more severe
•Type B (beta galactosidase deficiency)
•AR

Px:

•Proportionate dwarfism
•Corneal clouding
•Normal IQ
•Waddling gait
•Genu valgum
•Thoracic kyphosis

XR:

•Thickened skull
•Wide ribs

Raw speaker-note hierarchy audit

#lvlmarLindentlogicaloriginal note line
10NoneNone0Morquio – most likely survive into adulthood
20171450-1714501Accumulation of keratan sulphate
30171450-1714501Presents at 18 - 24months
40000Type A (galactosamine 6 sulphate sulphatase deficiency) – more severe
50000Type B (beta galactosidase deficiency)
60000AR
70000Px:
80000Proportionate dwarfism
90000Corneal clouding
100000Normal IQ
110000Waddling gait
120000Genu valgum
130000Thoracic kyphosis
140000XR:
150000Thickened skull
160000Wide ribs
170000Vertebral beaking
180000Coxa vara with unossified femoral head
190000Bullet shaped metacarpals
200000Odontoid hypoplasia leading to cervical instability
210000Thoracic kyphosis with platyspondia
220000Corneal clouding in Morquio and Hurler

Improved semantic hierarchy used for the study notes

#semantic levelsection?rewritten line
10Morquio – most likely survive into adulthood
21Accumulation of keratan sulphate
31Presents at 18 - 24months
41Type A (galactosamine 6 sulphate sulphatase deficiency) – more severe
51Type B (beta galactosidase deficiency)
61AR
70yesPx:
81Proportionate dwarfism
91Corneal clouding
101Normal IQ
111Waddling gait
121Genu valgum
131Thoracic kyphosis
140yesXR:
151Thickened skull
161Wide ribs
171Vertebral beaking
181Coxa vara with unossified femoral head
191Bullet shaped metacarpals
201Odontoid hypoplasia leading to cervical instability
211Thoracic kyphosis with platyspondia
221Corneal clouding in Morquio and Hurler
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