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achromatopsia [2025/12/01 17:00] Scott Larsonachromatopsia [2025/12/01 17:17] (current) – [Resources] Scott Larson
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 ====== Achromatopsia ====== ====== Achromatopsia ======
-FIXME+
 ====Main Features==== ====Main Features====
   * Reduced vision due to lack of functioning cones   * Reduced vision due to lack of functioning cones
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     * normal ROD function- normal dark adapted dim and bright flash waveforms     * normal ROD function- normal dark adapted dim and bright flash waveforms
 ====Differential Dx==== ====Differential Dx====
-  * Unordered List Item+  * [[congenital_stationary_night_blindness|Congenital Stationary Night Blindness]] 
 +  * [[leber_congenital_amaurosis|Leber Congenital Amaurosis]] 
 +  * [[retinitis_pigmentosa|Retinitis Pigmentosa]] 
 +  * Idiopathic Nystagmus Syndrome 
 ====Pathogenesis==== ====Pathogenesis====
 +  * 1 in 30,000 live births 
 +  * Recessively inherited mutation in a gene that codes for a part of the cone phototransduction pathway.  
 +  * 70-80% from mutations in the following genes: CNGA3 and CNGB3  
 +  * 1-2% from mutations in GNAT2, PDE6C, PDE6H, ATF6 
 ====Treatment==== ====Treatment====
 +  * Supportive care 
 +  * Sunglasses
 ====Resources==== ====Resources====
 +  - [[https://www.clinicalkey.com/#!/content/book/3-s2.0-B9780702082986000457#hl0000659|Taylor and Hoyt Textbook article]]
 +  - [[https://www.tandfonline.com/doi/full/10.1080/13816810.2017.1418389 |Hirji N. et al. Achromatopsia: clinical features, molecular genetics, animal models and theraputic options. Ophthalmic Genetics 2018;39(2):149-157]] [[https://1drv.ms/b/c/31d83ae8e55e0542/EdksFbEMfIBMkILEHEs01NcB3l10U7_oZE0oUhaTNeVNVA?e=aWa9GE|_]]
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 {{tag>inherited_retinal_disease retina}} {{tag>inherited_retinal_disease retina}}