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Sjogren TIN Treatment — ESENeph MCQ

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HardTubular DisordersSjogren TIN TreatmentESENeph

A child has genetically confirmed primary hyperoxaluria type 1 with recurrent calcium-oxalate nephrocalcinosis. Which disease-modifying NHS treatment is recommended by NICE for this diagnosis?

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Correct answer: A — Treat primary hyperoxaluria type 1 with lumasiran

The best answer is “Treat primary hyperoxaluria type 1 with lumasiran”. NICE HST25 recommends lumasiran for primary hyperoxaluria type 1 in people of all ages. RNA interference reduces hepatic oxalate production and is added to specialist supportive management. “Avacopan” is less appropriate because avacopan treats severe active GPA or MPA “Imlifidase” is less appropriate because imlifidase is a transplant-desensitisation treatment “Caplacizumab” is less appropriate because caplacizumab treats acute acquired TTP “Finerenone” is less appropriate because finerenone is used in selected adults with diabetic CKD, not inherited hepatic oxalate overproduction

Reference: NICE HST25: lumasiran for primary hyperoxaluria type 1: https://www.nice.org.uk/guidance/hst25/chapter/1-Recommendations