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46 Cards in this Set
- Front
- Back
What is anterior lenitconus caused by?
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-thinning of affected anterior capsule (fragile basement membrane)
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Is anterior lenticonous bi or unilateral
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bilateral
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Anterior lenticonus is commonly associated with
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Alport's syndrome
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What is the most common and earliest manifest of Alport Syndrome?
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Hematuria (gross of microscopic)
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T/F Microscopic hematuria is observed in all males and females.
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False
all males and 95% females |
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What is the most common ocular fidning in Alport's Syndrome? (Hint: X-lined)
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dot and fleck retinopathy
85% males |
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T/F Alport's/Anterior Lenticonus dec. VA.
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FALSE
spares the macula |
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___ and ___ systemic conditions almost always accompany Alport's.
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renal failure and hearing loss
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Posterior polymorphous corneal dystrophy
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rare ocular manifestation in pts. with AS
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What is the difference between Anterior and Posterior Lenticonus
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Posterior Lenticonus is NOT associated w/ systemic problems and is unilateral
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If you see posterior lenticonus bilaterally, what should you suspect?
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inheritable condition
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Lentiglobus
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-unilateral
-can cause polar cataracts -3-15yo -globular rather than conical protrusion |
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The most common cause of ectopia lentis is
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trauma
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What are the 1st and 2nd most common causes of hereditary ectopia lentis
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1-marfan
2-homcystinuria |
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Weil-Marchesani
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rare skeletal malformation syndrome, limited joint mobility, and microspherophakia
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Marfan syndrome:
lens dislocation (75%) is where? |
bilateral, symmetrical, and supertemporal
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Homocystinuria:
lens dislocation is where? |
bilateral, symmetrical, and inferonasal
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How does Weil pt. subluxates?
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inferiorly
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Diagnostic Triad of Lowe's
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congenital cataracts, neonatal/infantile hypotonia, renal tubular dysfunction
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When do Lowe's pts. usually die?
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20s/30s
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Lens coloboma
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segmental agenesis rather than true coloboma
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Lens coloboma is associated with (3)
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3M's
Microphthalmia Marfans Morning Glory |
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Mittendorf Dot
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lens vesicle is formed independent of closure of fetal fissure
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Mittendorf Dot may be associated with (3)
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-posterior lenticonus
-lentiglobus -microspherophakia |
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Posterior Embryotoxin
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-thickened, anteriorly displaces Schwalbe's line
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2 syndromes associated wtih Posterior Embryotoxin
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Axenfield-Rieger
Alagille's |
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Axenfield's Anomaly
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describes findings of post embryotoxon coupled with peripheral iris processes extending to or beyond prominent Schwalbe's loine
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Axenfield Syndrome is when anaomlay is present along with _____.
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associated glaucoma
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glaucoma develops in __% of pts with Axenfield anomly
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50%
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Rieger's Anomaly Triad
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Axenfield's anomaly (post embryotoxin +iris strands) + iris,
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What is the most widely held theory on the development of Axenfield/Rieger's?
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developmental arrest of specific anterior segment tissues derived from neural crest cells
(retention of a primordial endothelial cell layer on portions of iris and angle structures) |
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What is the difference between Type I and Type II Peter's Anomaly
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Type I- no lens involvement
Type II- lens involvement |
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Glaucoma occurs in ___% of Peter's Anomaly pts.
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50-70%
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T/F Peter's Anomaly is usually unilateral
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False, Bilateral
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What is a key systemic feature of Alagilles?
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neonatal jaundiceT/F
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T/F Nearly ALL Alageille pts. have cardiac murmurs.
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true
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What is the most frequent ophthalmologic finding in Alagille?
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Posterior embryotoxon
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Miller's syndrome
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aniridia and Wilm's tumor
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Gillespie's Syndrome
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mentral retardation and cerebellar ataxia
(associated with automosomal recessive with inherited cases of aniridia) |
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WAGR syndrome
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A rare genetic disorder that has
Wilms Tumor Aniridia Genitourinary malformations Retardation |
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What are the 2 types PPM?
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type I- has attachments only to the iris
type II- iridolenticular adhesions |
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Wachendorf's membrane
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part of TVL
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Microphthalmos is defined as
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total axial length at least 2 standard deviations below age-similar controls
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There are 4 types of microphthalmos
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-simple: not associated with other major ocular malformations
-complex: associated with coloboma -with cyst: associated with formation of orbital cyst -posterior:decreased TAL, normal corneal diam., high hyeropia, retinal folds |
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What are the two types of anophthalmos?
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-simple:
-with cysts: globe is replaced by cysts |
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T/F Nanophthalmos is a small eye with NORMAL structures.
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N for Normal
TRUE |