Anatomy, Biochemistry, Physiology, Embryology and Development Flashcards

1
Q

index of refraction of lens

A

1.4 centrally, 1.36 peripherally

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2
Q

average nonaccommodative power of lens

A

15-20 D

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3
Q

size of lens at birth? adult?

A

birth: 6.4mm equatorially and 3.5mm A-P
adult: 9mm equatorially and 5mm A-P

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4
Q

refractive changes in lens due to age?

A

decreasing refractive index, increased relative thickness of cortes, increasingly curved shape, and overall increased refractive power

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5
Q

what substance predominantly makes up lens capsule

A

type IV collagen

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6
Q

thinnest portion of lens capsule

A

posterior pole

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7
Q

largest lens crystalline? most abundant? smallest?

A

largest: alpha
most abundat: beta
smallest: gamma

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8
Q

increase or decrease in water insoluble lens proteins with age?

A

increase

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9
Q

rate limiting step in glucose metabolism in lens?

A

posphorlyation of glucose by hexokinase into glucose-6-phosphate (G6P)

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10
Q

% glucose metabolism by glycolysis? by TCA cycle? %ATP produced aerobically?

A

78%. 3% (low oxygen tension in lens), but due to high efficiency still produces 25% of ATP

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11
Q

percentage of G6P metabolized in lens by HMP shunt pathway? more or less than other tissues?

A

5%, which is proportionally higher than other tissues

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12
Q

metabolic pathway responsible for diabetic cataract?

A

sorbitol

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13
Q

affect of hyperbaric oxygen on lens?

A

nuclear cataract formation

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14
Q

relative proportions of sodium, potassium, water, chloride, and amino acids in lens

A

potassium and amino acids high

sodium, chloride, and water low (due to Na+/K+ pump)

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15
Q

embryologic origin of lens

A

surface ectoderm

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16
Q

name the first three steps of lens development in order

A

lens placode, lens pit, lens vesicle

17
Q

orientation of Y sutures in lens?

A

upright Y anteriorly, inverted Y posteriorly

18
Q

where do zonules originate?

A

secreted by ciliary epithelium

19
Q

Laterality, relative commonality, and associated syndromes with anterior and posterior lenticonus

A

Anterior: bilateral, less common, Alport syndrome
Posterior: unilateral, more common, Lowe syndrome

20
Q

most common location of Mittendorf dot

A

inferonasal

21
Q

when does the lens vesicle normally separate from the surface ectoderm

A

33 weeks gestation

22
Q

Microspherophakia:

  1. refractive state
    • stubby fingers, small stature, reduce joint mobility (and inheritance)
  2. feared complication
  3. medications contraindicated in (3)
  4. medical and procedural treatment of (3)
A
  1. highly myopic
  2. Weill-Marchesani, AR
  3. pupillary block glaucoma
  4. miotics (increase pupillary block)
  5. cycloplegics (decrease A-P lens diameter) + LPI
23
Q

Aniridia:

  1. laterality
  2. % sporadic
  3. associated gene
  4. associated findings
  5. workup for sporadic?
A
  1. almost always bilateral
  2. 1/3 sporadic
  3. PAX6
  4. epitheliopathy from decreased limbal stem cells, angle closure glaucoma, optic nerve and foveal hypolasia
  5. abdominal u/s to eval for Wilms tumor (WAGR = Wilms tumor, aniridia, GU anomalies, mental retardation)
24
Q

basic causes of congenital cataracts and relative frequency

A

1/3 isolated inherited trait, 1/3 associated with systemic syndromes, 1/3 unknown

25
most common type of congenital cataract?
lamellar
26
anatomy of lamellar cataract
clear center, opaque spherical shell, clear surrounding of shell
27
small bluish cortical lens opacities in newborn? management?
Cerulean cataract; observe as are usually not visually significant
28
Marfan: 1. inheritance and gene product 2. location of lens subluxation 3. management of lens subluxation
1. AD, fibrillin 2. usually superotemporal 3. refractive correction first, as surgery os complicated by zonular dehiscence, vitreous loss, and RD
29
Homocystinuria: 1. inheritance 2. diagnositc lab findings 3. location of lens subluxation 4. treatment
1. AR 2. high homocystine, high methionine 3. inferonasal 4. cysteine and B6 supplements; methionine restriction
30
inheritance of ectopia lentis et pupillae? location of lens subluxation?
AR; 180 degrees away from pupillary displacement