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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 152 (1993), S. 674-676 
    ISSN: 1432-1076
    Keywords: Glycogen storage disease type I ; Kidney involvement ; Renal scintigraphy ; Effective renal plasma flow
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To evaluate effective renal plasma flow (ERPF) we performed renal scintigraphies with99mTc-Mercaptoacetyl-triglycine (MAG3) in ninepatients 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 agedependent 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 preceeds clinically overt nephropathy. Renal scientigraphy with99mTc MAG3 is a suitable method for the early detection and monitoring of kidney dysfunction in GSD I.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1619-7089
    Keywords: Iodine-123 metaiodobenzylguanidine single photon emission tomography ; Arrhythmogenic right ventricular disease ; Cardiac sympathetic nerve system
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Arrhythmogenic right ventricular disease (ARVD) is a disease of unknown origin that primarily affects the right ventricle and is characterized by ventricular tachyarrhythmias which may lead to syncope and even, though rarely, sudden cardiac death. In 25 patients with ARVD, sympathetic innervation of the left ventricle was assessed by iodine-123 metaiodobenzylguanidine single photon emission tomography (1231-MIBG SPET). In addition, thallium-201 SPET was performed. The diagnosis of ARVD was made by an electrophysiological study and right and left heart catheterization including right ventricular endomyocardial biopsy. Ischaemic heart disease was excluded by coronary angiography. A group of seven patients without any evidence of heart disease served as a control group. Twenty-two of the 25 patients showed reduced uptake of 123I-MIBG. The abnormal areas were located predominantly in posterior and posteroseptal segments of the heart. No focus of increased 123I-MIBG activity could be demonstrated. No patient had signs of left ventricular involvement on left ventricular angiography. In contrast to the results of the 123I-MIBG SPET, those of 201TI SPET were normal in 16 patients. The remaining nine patients showed areas of slight hypoperfusion not correlated with the reduced 123I-MIBG uptake. 123I-MIBG scintigraphy allows detection of left ventricular adrenergic dysinnervation in ARVD patients without morphological or functional abnormalities of the left ventricle.
    Type of Medium: Electronic Resource
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