ArticleJournal of inherited metabolic disease2013
Chronic kidney disease in adolescent and adult patients with phenylketonuria.
Article in Journal of inherited metabolic disease, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02176603 (Observational, Investigator Initiated, Monocentric Study of the Endothelial Dysfunction in Adolescent and Adult Patients With Phenylketonuria Due to Phenylalanine Hydroxylase Deficiency), which is not on this map. Cited by 29 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Observational, Investigator Initiated, Monocentric Study of the Endothelial Dysfunction in Adolescent and Adult Patients With Phenylketonuria Due to Phenylalanine Hydroxylase Deficiency
Who cites it
29 citing papers in PubMed, 58 citations in OpenAlex.
- Trial
- Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial.The American journal of clinical nutrition · 2016Trial
- Current state of the treatment landscape of phenylketonuria.Orphanet journal of rare diseases · 2025Review
- The relationship between adult phenylketonuria and the cardiovascular system - insights into mechanisms and risks.Orphanet journal of rare diseases · 2025Review
- Large Variations in Phenylalanine Concentrations Associate Adverse Cardiac Remodelling in Adult Patients With Phenylketonuria-A Long-Term CMR Study.Journal of cachexia, sarcopenia and muscle · 2025Article
- Novel Metabolites Associated with Decreased GFR in Finnish Men: A 12-Year Follow-Up of the METSIM Cohort.International journal of molecular sciences · 2024Article
- Impact of Phenylketonuria on the Serum Metabolome and Plasma Lipidome: A Study in Early-Treated Patients.Metabolites · 2024Article
- Amino Acid Profile Alterations in Phenylketonuria: Implications for Clinical Practice.Metabolites · 2024Article
- Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria.Nutrients · 2023Article
- Low bone mineralization in phenylketonuria may be due to undiagnosed metabolic acidosis.Molecular genetics and metabolism reports · 2023Article
- Phenylketonuria (PKU) Urinary Metabolomic Phenotype Is Defined by Genotype and Metabolite Imbalance: Results in 51 Early Treated Patients Using Ex VivoMolecules (Basel, Switzerland) · 2023Article
- Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein.Nutrients · 2022Review
- Phenylalanine hydroxylase mRNA rescues the phenylketonuria phenotype in mice.Frontiers in bioengineering and biotechnology · 2022Article
- Maple syrup urine disease associated with nephrotic syndrome in a Filipino child.BMJ case reports · 2021Article
- Metabolite Signature of Albuminuria Involves Amino Acid Pathways in 8661 Finnish Men Without Diabetes.The Journal of clinical endocrinology and metabolism · 2021Article
- Lower plasma cholesterol, LDL-cholesterol and LDL-lipoprotein subclasses in adult phenylketonuria (PKU) patients compared to healthy controls: results of NMR metabolomics investigation.Orphanet journal of rare diseases · 2020Article
- Minimal Change Disease and Phenylketonuria in an Adult Patient: The Two Sides of Protein Homeostasis.European journal of case reports in internal medicine · 2020Article
- Prospects for Cell-Directed Curative Therapy of Phenylketonuria (PKU).Molecular Frontiers journal · 2019Article
- Sex effects of dietary protein source and acid load on renal and bone status in the PahPhysiological reports · 2019Article
- The cardiovascular phenotype of adult patients with phenylketonuria.Orphanet journal of rare diseases · 2019Article
Corrections and comments
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Authors and funding
9 authors at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesA lifelong phenylalanine-restricted diet with supplementation of a phenylalanine-free amino acid formula is recommended in patients with phenylketonuria (PKU). The effect of a long-term PKU diet on renal function and blood pressure has not been investigated yet.
designWe analyzed renal function in 67 patients with PKU, aged 15-43 years, by measuring glomerular filtration rate (GFR) and effective renal plasma flow by isotope clearance ((51)Cr-EDTA, (123)J-Hippuran), estimated GFR, blood retention parameters, urinary protein and electrolyte excretion. Renal ultrasound and 24 h ambulatory blood pressure monitoring were performed additionally. Patients were divided into three groups according to their: 1) current diet (CD), i.e., daily protein intake: ICD <0.8 g/kg, IICD 0.8-1.04 g/kg, IIICD >1.04 g/kg; 2) life-long diet time (LDT), i.e., cumulative years of life in which daily protein intake exceeded dietary recommendations: ILDT <15 years, IILDT 15-19 years, IIILDT >19 years.
resultsGFR was decreased in 19 % of the patients. With increasing protein intake, GFR decreased significantly (ICD 111 ml/min; IICD 105 ml/min; IIICD 99 ml/min. ILDT 112 ml/min; IILDT 103 ml/min; IIILDT 99 ml/min). Proteinuria was detected in 31 %, microalbuminuria in 7 %, and hypercalciuria in 23 % of the patients. 23 % of the patients had arterial hypertension, and 41 % revealed a nocturnal non-dipping status.
conclusionsIn patients with PKU on a lifelong diet we could detect impaired renal function in 19 %, proteinuria in 31 %, and arterial hypertension in 23 %. Thus, chronic kidney disease may develop in PKU patients, and routine renal function tests should be performed during long-term follow-up.
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