Cataract surgery in SeniorÞenLoken syndrome is beneficial despite severe retinopathy

Sarangapani, Susan; Chang, Lydia; Gregory-Evans, Kevin

doi:10.1038/sj.eye.6700171

Cited by 4 publications

(1 citation statement)

References 12 publications

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“…However, there is currently no treatment to prevent or stop the progression of vision loss due to retinal dystrophy, but various low-vision aids may be helpful for those who have remaining vision. Coexisting cataracts should be surgically corrected despite severe retinopathy as patients benefit from improved visual acuity following surgery [ 14 ]. Families and individuals with a family history of autoimmune disorders should be genetically screened for any of the NPHP genes.…”

Section: Discussionmentioning

confidence: 99%

Successful Renal Transplantation in a Patient With Senior-Loken Syndrome and Antiphospholipid Syndrome: A Case Report

Attiq,

Elahi,

Ashraf

et al. 2023

Cureus

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Senior-Loken syndrome (SLS) is a rare autosomal recessive disorder affecting the eyes and the kidneys. It is an extremely rare disorder with an incidence of 1/1,000,000. Like most hereditary disorders, it is more commonly seen in families with consanguineous marriages. Here, we present a case of a 35-year-old male with a complicated past medical history, who presented to us in the outpatient department for kidney transplant consideration. The patient was diagnosed case of Senior-Loken syndrome with a family history of autoimmune diseases, renal disease, and multiple unexplained miscarriages. He also had multiple dialysis access-related complications requiring frequent access changes. He previously had an unrelated pre-emptive renal transplant which resulted in graft failure within 48 hours. In view of his history, a prothrombotic condition was suspected and the patient was started on warfarin. Workup was positive for lupus anticoagulant and hematology recommended lifelong anticoagulation. The patient had a related renal transplant that was successful. He is now on apixaban and has not had any thrombotic complications to date. This patient had antiphospholipid syndrome leading to multiple thrombotic events and a failed graft, but was never worked up for autoimmune disorders despite having a strong family history. His renal disease was presumed to be secondary to a rare condition - Senior-Loken syndrome and he was not investigated for a co-existing condition (e.g., antiphospholipid syndrome {APLS} in this case) which led to early graft failure. Hence when considering a patient for transplant, care should be taken to rule out autoimmune diseases and not ignore possible co-existing conditions in the presence of a renal pathology.

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Section: Discussionmentioning

confidence: 99%