title: Renal involvement in patients with congenital cyanotic heart disease.
abstract: Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.
authors.affiliation: Department of Paediatric Nephrology, Medizinische Hochschule, Hannover, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Krull
authors.forename: J H
authors.initials: JH
authors.lastname: Ehrich
authors.forename: U
authors.initials: U
authors.lastname: Wurster
authors.forename: U
authors.initials: U
authors.lastname: Toel
authors.forename: S
authors.initials: S
authors.lastname: Rothgänger
authors.forename: I
authors.initials: I
authors.lastname: Luhmer
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001830:Body Surface Area; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000652:urine; D005860:Germany, West / Q000453:epidemiology; D005919:Glomerular Filtration Rate; D006330:Heart Defects, Congenital / Q000097:blood / Q000150:complications*; D006400:Hematocrit; D006596:Hexosaminidases / Q000652:urine; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007674:Kidney Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D011507:Proteinuria / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D012189:Retrospective Studies
chemical_list: D003404:Creatinine; D006596:Hexosaminidases