Characteristics of continuous venovenous hemodiafiltration in the acute treatment of inherited metabolic disorders

• Published in: Pediatric nephrology (Berlin, West) West), 2022-06, Vol.37 (6), p.1387-1397
• Main Authors: Eminoğlu, Fatma Tuba, Öncül, Ümmühan, Kahveci, Fevzi, Okulu, Emel, Kraja, Elvis, Köse, Engin, Kendirli, Tanıl
• Format: Article
• Language: English
• Subjects: Acidosis; Age; Aged; Ammonia; Blood pressure; Care and treatment; Child; Children; Continuous Renal Replacement Therapy; Encephalopathy; Hemodiafiltration - adverse effects; Hemodialysis; Humans; Hypocalcemia; Hypotension; Hypotension - etiology; Inborn errors of metabolism; Infant; Infant, Newborn; Intensive care units; Leucine; Maple syrup urine disease; Maple Syrup Urine Disease - complications; Maple Syrup Urine Disease - therapy; Medicine; Medicine & Public Health; Metabolic acidosis; Metabolic Diseases - complications; Metabolic disorders; Metabolism; Metabolism, Inborn Errors - complications; Metabolism, Inborn errors of; Metabolites; Neonates; Nephrology; Original; Original Article; Patients; Pediatrics; Renal Dialysis; Thrombocytopenia; Urology; Vasoactive agents
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-021-05329-9

Background Continuous kidney replacement therapies (CKRT) have been reported to be an effective approach to removing toxic metabolites in inborn errors of metabolism (IEM). The present study evaluates efficiency and complications of CKRT in children with IEM. Methods Patients diagnosed with IEM who underwent CKRT in pediatric and neonatal intensive care units were analyzed. CKRT were initiated in patients with persistently high blood ammonia levels (≥ 500 μmol/L), blood ammonia levels > 250 μmol/L in the presence of moderate encephalopathy, high blood leucine levels (≥ 1500 μmol/L), and blood leucine levels