Posttest for Practical Management of Inherited Retinal Disorders

Individuals with retinitis pigmentosa are at risk for the following complication:*
- A. Early-onset cataracts
- B. Cystoid macular edema
- C. Glaucoma
- D. A and B
The following test can help in the initial screening of individuals with a family history of retinitis pigmentosa:*
- A. Widefield fundus autofluorescence
- B. Farnworth D-15 color panel
- C. Microperimetry
- D. Visual acuity
A patient with retinitis pigmentosa has cystoid macular edema in one eye. Treatment with the following topical agent may be beneficial:*
- A. Timolol
- B. Dorzolamide
- C. Latanoprost
- D. Brimonidine
Inherited retinal dystrophies:*
- A. Are not accompanied by associated systemic features
- B. Present before age 20
- C. Always get worse with age
- D. Are caused by mutations in a single gene
Genetic testing for inherited retinal dystrophies:*
- A. Currently identifies a causative gene in approximately 2/3 of individuals
- B. Can help to determine eligibility for clinical trials
- C. Requires sequencing all the genes in the body
- D. A and B
A patient with a longstanding clinical diagnosis of retinitis pigmentosa undergoes genetic testing that does not identify a causative gene. The following is advised:*
- A. Reassessing the clinical diagnosis
- B. Genetic counseling to help the patient understand the result
- C. Repeating genetic testing in several years
- D. B and C
A 75 year-old man with retinitis pigmentosa and a defined genetic cause of disease has “light perception” vision and widespread outer retinal atrophy on OCT. Which investigational therapeutic strategy is most likely to be helpful in restoring visual function?*
- A. Subretinal gene augmentation therapy
- B. Intravitreal gene augmentation therapy
- C. Retinal prosthetic device
- D. Oral neuroprotective agent
Gene-specific therapies for inherited retinal diseases include all but the following:*
- A. Introducing a non-mutated copy of the gene to the retina
- B. Editing specific sequences of chromosomal DNA
- C. Modifying the translation of mRNA into protein
- D. Replacing degenerative rods and cones
The most common type of cataract identified in patients with retinitis pigmentosa is:*
- A. Nuclear cataract
- B. Cortical cataract
- C. Posterior polar cataract
- D. Posterior subcapsular cataract
A potential complication of cataract surgery that is more common in retinitis pigmentosa than in the general population is:*
- A. Dropped lens due to zonular weakness
- B. Post-operative intraocular pressure spike
- C. Cystoid macular edema
- D. A and C

Posttest for Practical Management of Inherited Retinal Disorders

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