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Chronic kidney disease in adolescent and adult patients with phenylketonuria.
MedLine Citation:
PMID:  23138985     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVES: A 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. DESIGN: We 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: I(CD) <0.8 g/kg, II(CD) 0.8-1.04 g/kg, III(CD) >1.04 g/kg; 2) life-long diet time (LDT), i.e., cumulative years of life in which daily protein intake exceeded dietary recommendations: I(LDT) <15 years, II(LDT) 15-19 years, III(LDT) >19 years. RESULTS: GFR was decreased in 19 % of the patients. With increasing protein intake, GFR decreased significantly (I(CD) 111 ml/min; II(CD) 105 ml/min; III(CD) 99 ml/min. I(LDT) 112 ml/min; II(LDT) 103 ml/min; III(LDT) 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. CONCLUSIONS: In 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.
Authors:
Julia B Hennermann; Sylvia Roloff; Jutta Gellermann; Ilka Vollmer; Elke Windt; Barbara Vetter; Ursula Plöckinger; Eberhard Mönch; Uwe Querfeld
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-9
Journal Detail:
Title:  Journal of inherited metabolic disease     Volume:  -     ISSN:  1573-2665     ISO Abbreviation:  J. Inherit. Metab. Dis.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7910918     Medline TA:  J Inherit Metab Dis     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Pediatric Endocrinology, Gastroenterology and Metabolic Diseases, Charité Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany, julia.hennermann@charite.de.
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