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Additional Slide

RPE
The retinal pigment epithelium (RPE) forms a monolayer of
highly specialized neuroectodermally derived pigmented cells
• located between the neurosensory retina and the vascular
choroid.
The RPE mediates functions essential for normal outer
• retinal physiology, including participation in the visual cycle,
• phagocytosis of shed photoreceptor outer segments (OS),
maintenance
• of the outer blood–retinal barrier
• secretion of neurotrophic, inflammatory, and
vasculotrophic growth factors, water transport out of the
subretinal space, and regulation of bidirectional ion and
metabolic transport between the retina and choroid.
Cone vs Rods
• In the fully dark adapted state, rods can reliably report the
absorption of single photons to the retinal output, and they permit
our scotopic, or night, vision.
• Cone photoreceptors are morphologically and functionally distinct
from rods and express several types of visual pigments, or opsins,
whose spectral sensitivity varies based on the cone’s subtype. In
humans, three classes of cones confer robust color vision: S, M, and
L cones
• Cones are also less sensitive than rods and generate light responses
that are temporally briefer. This allows cones to mediate our
photopic, or day, vision with improved temporal resolution. The
concerted action of these two types of photoreceptors, and the
retinal circuitry that carries their signals to the retinal output,
ultimately underlie our rich visual experience.
Rodopsin
• Rhodopsin is a member of the superfamily of seven-
helix, G-protein–coupled receptor proteins (GPCRs). In
contrast to chemosensing GPCRs, rhodopsin has its
ligand, the light-absorbing “chromophore”
retinaldehyde, prebound.
• The bound form, 11-cis retinal, acts as a powerful
antagonist, holding rhodopsin in its completely inactive
state. Absorption of a photon of light isomerizes the
chromophore to a second form, the all-trans
configuration, which acts as a powerful agonist that
rapidly triggers a conformational change in the protein,
activating rhodopsin as an enzym
Drug Toxicity
Penggunaan jangka panjang dapat
menyebabkan pseudoretinitis pigmentosa
• Thioridazine
• Chlorphromazine
• Chloroquinine
• Hydroxychloroquinine
• Quinine
Future Therapy
• Terapi gen
• Transplantasi sel
• Prostesis Elektronik (Retina Buatan)
Pemeriksaan Penglihatan Warna
• Isihara
• Fansworth Munsel
Bone Spicule Formation
• Photoreceptor cell death  RPE detach from
bruch membrane  RPE cell disintegration 
Pigmen migration into neurosensory retina 
accumulation in intersitial space 
Pigmentary cuffing and spicule shaped
deposits
RP Variant
• Leber congenital amaurosis: gejala tampak
sejak lahir.
• Bardet – Biedl syndrome: Retinopathy,
polydactili, congenital obesity, mental
retardation, hypogenitalism
• Refsum syndrome: Progressive neurologic
deficits, deafness, liver disease, skeletal
abnormality.
LOW VISION
• Pasien dengan visus < 20/40
• Atau dengan field loss central atau perifer.
• Vision Rehabilitation  pasien dengan
penyakit mata yang tidak dapat disembuhkan
dengan obat-obatan atau operasi dan
mengganggu activity daily living
• Intervensi termasuk konseling dan support
grup.