2020
Stiripentol fails to lower plasma oxalate in a dialysis-dependent PH1 patient
Kempf C, Pfau A, Holle J, Müller-Schlüter K, Bufler P, Knauf F, Müller D. Stiripentol fails to lower plasma oxalate in a dialysis-dependent PH1 patient. Pediatric Nephrology 2020, 35: 1787-1789. PMID: 32418144, PMCID: PMC7385015, DOI: 10.1007/s00467-020-04585-5.Peer-Reviewed Original ResearchConceptsEnd-stage renal diseaseChronic kidney diseaseLactate dehydrogenase 5Urinary oxalateAdvanced chronic kidney diseaseAnti-convulsive drugsMultisystemic metabolic disorderLiver-kidney transplantationMajority of patientsCurrent standard treatmentTreatment of childrenHyperoxaluria type 1Anuric infantsPH patientsUox excretionRenal diseaseDravet syndromeKidney diseasePrimary hyperoxaluriaCompassionate useStandard treatmentPlasma concentrationsMetabolic disordersPromising drugEarly infancy
2016
Oxalate, inflammasome, and progression of kidney disease
Ermer T, Eckardt KU, Aronson PS, Knauf F. Oxalate, inflammasome, and progression of kidney disease. Current Opinion In Nephrology & Hypertension 2016, 25: 363-371. PMID: 27191349, PMCID: PMC4891250, DOI: 10.1097/mnh.0000000000000229.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsChronic kidney diseaseProgressive renal failureRenal inflammationRenal failurePlasma oxalateKidney diseaseInflammasome activationElevated plasma oxalate levelsNOD-like receptor familyProgressive renal damageGlomerular filtration rateMore rapid progressionWarrants clinical trialsPlasma oxalate levelsRenal damageEnteric hyperoxaluriaMacrophage infiltrationIL-1βFiltration rateClinical trialsRapid progressionInflammasome proteinsMice protectsUrinary oxalatePyrin domain