nature reviews nephrologyhttps:/doi.org/10.1038/s41581-022-00661-1Consensus statement间Check for updatesClinical practice recommendationsfor primary hyperoxaluria:an expert consensus statementfrom ERKNet and OxalEuropeAlist of authors and their affiliations appears at the end of the paperAbstractSectionsPrimary hyperoxaluria(PH)is an inherited disorder that results fromIntroductionthe overproduction of endogenous oxalate,leading to recurrent kidneyMethodsstones,nephrocalcinosis and eventually kidney failure;the subsequentManagementstorage ofoxalate can cause life-threatening systemic disease.Diagnosisrecommendationsof PHis often delayed or missed owing to its rarity,variableclinicalDiagnostic approachexpression and other diagnostic challenges.Management of patients withTherapyPHand kidney failure is also extremely challenging.However,in the pastfew years,several new developments,including new outcome data fromNew therapiespatients withinfantile oxalosis,from transplanted patients with type1PHManagement of PHinlow-resource countries(PH1)and from patients with the rarer PHtypes 2 and 3,have emerged.Inaddition,two promising therapies based on RNA interference have beenUnanswered questions andresearch agendaintroduced.These developments warrant an update of existing guidelineson PH,based on new evidence and on a broad consensus.In response toConclusionsthis need,a consensus development coregroup,comprising(paediatric)nephrologists,(paediatric)urologists,biochemists and geneticists fromOxalEurope and the European Rare Kidney Disease Reference Network(ERKNet),formulated andgraded statements relating to the managementof PH on the basis of existing evidence.Consensus was reached followingreview of the recommendations by representatives ofOxalEurope,ESPN,ERKNet and ERA,resulting in 48 practical statements relatingto the diagnosis and management of PH,including consideration ofconventional therapy(conservative therapy,dialysis and transplantation),new therapies and recommendations for patient follow-up.e-mail:j.w.groothoff@amsterdamumc.nlNature Reviews Nephrology
