Ординатура / Офтальмология / Английские материалы / Illustrated Tutorials in Ophthalmology Kanski, Bolton 2001
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Systemic Implications of Aniridia
AN-1 - 85%
•Autosomal dominant
•Isolated
AN-2 (Miller syndrome) - 13%
•Deletion of short arm of chromosome 11
•Wilm tumour, genitourinary anomalies and mental handicap
AN-3 (Gillespie syndrome) - 2%
•Autosomal recessive
•Mental handicap and cerebellar ataxia
Signs of aniridia
Partial absence |
Subtotal absence |
Synechial angle-closure |
Occasional cataract and lens |
glaucoma in 75% |
subluxation |
Glaucoma in Sturge-Weber syndrome
Glaucoma |
Causes |
• |
Glaucoma in 30% |
• |
Caused by raised episcleral venous |
• |
Ipsilateral to facial haemangioma |
|
pressure associated with episcleral |
|
haemangioma |
||
|
|
|
|
• |
Buphthalmos in 60% |
• |
Angle anomaly may also be responsible |
Spondylarthropathies
|
Gender |
HLA-B27 |
Acute iritis |
|
|
|
|
Ankylosing |
70% males |
95% |
30% |
|
|
|
|
spondylitis |
|
|
|
|
|
|
|
Reiter syndrome |
90% males |
60% |
20% |
|
|
|
|
Psoriatic arthritis |
equal |
30% |
10% |
|
|
|
|
Spondylarthropathies
Sacroiliitis |
Peripheral arthritis |
Bowel |
|
|
inflammation |
Ankylosing |
100% |
20% |
Common |
|
spondylitis |
||||
|
|
|
||
|
|
|
|
|
Reiter |
60% |
100% |
Uncommon |
|
syndrome |
||||
|
|
|
|
|
Psoriatic |
30% |
100% |
Occasional |
|
arthritis |
||||
|
|
|
|
Clinical features of Reiter syndrome
Conjunctivitis |
Plantar fasciitis |
Painless oral ulceration |
|
|
|
Urethritis and |
Keratoderma |
Nail dystrophy |
|
circinate balanitis |
blenorrhagica |
||
|
Juvenile idiopathic arthritis
Pauciarticular |
Polyarticular |
Systemic |
(60%) |
(20%) |
(20%) |
Joints no. |
< 5 |
> 4 |
Variable |
Onset |
< 6 years |
Variable |
Variable |
Systemic features |
Absent |
Mild or absent |
Severe |
Positive ANA |
75% |
40% |
10% |
Iridocyclitis |
20% |
5% |
Absent |
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