title: Effective renal plasma flow in patients with glycogen storage disease type I.
abstract: To evaluate effective renal plasma flow (ERPF) we performed renal scintigraphies with 99mTc-Mercaptoacetyl-triglycine (MAG3) in nine patients with glycogen storage disease I (GSD I) (age: 16 +/- 7 years). Two patients presented with proteinuria, none showed hyperaminoaciduria, disturbed tubular reabsorption of phosphate or hypertension. 99mTc-MAG3 clearance values were elevated in eight out of nine patients (865 +/- 233 ml/min/1.73 m2 body surface area) and exceeded the age-dependent mean values by 21%-145%. ERPF values in patients with poor metabolic control were higher than in patients with long-term good metabolic control (988 +/- 186 vs. 619 +/- 55 ml/min/1.73 m2; P < 0.05). We conclude that enhanced ERPF is a common finding in GSD I patients, which precedes clinically overt nephropathy. Renal scintigraphy with 99mTc MAG3 is a suitable method for the early detection and monitoring of kidney dysfunction in GSD I.
authors.affiliation: Department Nuclear Medicine, University of Münster, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Hahn-Ullrich
authors.forename: J
authors.initials: J
authors.lastname: Sciuk
authors.forename: P
authors.initials: P
authors.lastname: Bartenstein
authors.forename: P
authors.initials: P
authors.lastname: Kreysing
authors.forename: K
authors.initials: K
authors.lastname: Ullrich
mesh_terms: D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D007668:Kidney / Q000000981:diagnostic imaging; D008297:Male; D011877:Radionuclide Imaging; D017596:Renal Plasma Flow, Effective; D017263:Technetium Tc 99m Mertiatide
chemical_list: D017263:Technetium Tc 99m Mertiatide