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Glycosaminoglycans in urine and amniotic fluid in congenital nephrotic syndrome of the Finnish type

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Abstract

The heparan sulphate proteoglycan (HSPG) of the glomerular basement membrane (GBM) is considered to be mainly responsible for the charge selectivity of the GBM; decreased HSPG results in a decreased anionic charge of the GBM with increased heparan sulphate (HS) in the urine and is believed to be responsible for the proteinuria of the congenital nephrotic syndromes (CNS). Urinary HS and chondroitin sulphate (CS) concentrations in children with CNS of the Finnish type (CNF) and the total glycosaminoglycans (GAG) in amniotic fluid of CNF pregnancies were measured by three methods: Alcian blue, Safranine O and uronic acid assays. The total urinary GAG in CNF and other nephrotic patients was comparable to controls with all three methods. Urinary CS and HS in CNF did not differ significantly from controls. Total amniotic fluid GAG was also similar in CNF and control pregnancies. These results suggest some pathogenetic mechanism other than loss of glomerular HS chains in urine for the proteinuria of CNF.

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References

  1. Hallman N, Hjelt L (1959) Congenital nephrotic syndrome. J Pediatr 55: 152–162

    PubMed  Google Scholar 

  2. Norio R (1966) Heredity in the congenital nephrotic syndrome of the Finnish type. Ann Paediatr Fenn [Suppl 12]: 27: 1–94

    Google Scholar 

  3. Seppälä M, Rapola J, Huttunen NP, Aula P, Karjalainen O, Ruoslahti E (1976) Congenital nephrotic syndrome: prenatal diagnosis and genetic counseling by estimation of amniotic fluid and maternal serum alphafetoprotein. Lancet I: 123–125

    Article  Google Scholar 

  4. Mahan JD, Mauer SM, Sibley RK, Vernier RL (1984) Congenital nephrotic syndrome: evolution of medical management and results of renal transplantation. J Pediatr 105: 549–557

    PubMed  Google Scholar 

  5. Holmberg C, Jalanko H, Koskimies O, Leijala M, Salmela K, Eklund B, Ahonen J (1990) Renal transplantation in children with congenital nephrotic syndrome of the Finnish type. Transplant Proc 22: 158

    PubMed  Google Scholar 

  6. Huttunen NP, Rapola J, Vilska J, Hallman N (1980) Renal pathology in congenital nephrotic syndrome of Finnish type: a quantitative light microscopic study on 50 patients. Int J Pediatr Nephrol 1: 10–16

    PubMed  Google Scholar 

  7. Rapola J, Sariola H, Ekblom P (1984) Pathology of fetal congenital nephrosis: immunohistochemical and ultrastructural studies. Kidney Int 25: 701–707

    PubMed  Google Scholar 

  8. Autio-Harmainen H, Karttunen T, Risteli L, Risteli J, Rapola J (1985) Accumulation of laminin and type IV collagen in the kidney in congenital nephrosis. Kidney Int 27: 662–666

    PubMed  Google Scholar 

  9. Autio-Harmainen H, Rapola J (1983) The thickness of the glomerular basement membrane in congenital nephrotic syndrome of the Finnish type. Nephron 34: 48–50

    PubMed  Google Scholar 

  10. Rapola J, Huttunen NP, Hallman N (1992) Congenital and infantile nephrotic syndrome. In: Edelmann C (ed) Pediatric kidney disease, 2nd edn. Little Brown, Boston, p 1291

    Google Scholar 

  11. Autio-Harmainen H, Rapola J (1982) Renal pathology of fetuses with congenital nephrotic syndrome of the Finnish type. A qualitative and quantitative light microscopic study. Nephron 29: 158–163

    Google Scholar 

  12. Vernier RL, Klein DJ, Sisson SP, Mahan JD, Oegema TR, Brown DM (1983) Heparan sulfate-rich anionic sites in the human glomerular basement membrane. Decreased concentration in congenital nephrotic syndrome. N Engl J Med 309: 1001–1009

    PubMed  Google Scholar 

  13. Mahan JD, Sisson-Ross SS, Vernier RL (1989) Anionic sites in the human kidney: ex vivo perfusion studies. Mod Pathol 2: 117–124

    PubMed  Google Scholar 

  14. Vermylen C, Levin M, Mossman J, Barratt TM (1989) Glomerular and urinary heparan sulphate in congenital nephrotic syndrome. Pediatr Nephrol 3: 122–129

    PubMed  Google Scholar 

  15. Jadresic LP, Filler G, Barratt TM (1991) Urine glycosaminoglycans in congenital and acquired nephrotic syndrome. Kidney Int 40: 280–284

    PubMed  Google Scholar 

  16. Heuvel LPWJ van den, Born J van den, Jalanko H, Schröder CH, Veerkamp JH, Assmann KJM, Berden JHM, Holmberg C, Rapola J, Monnens LAH (1992) The glycosaminoglycan content of renal basement membranes in the congenital nephrotic syndrome of the Finnish type. Pediatr Nephrol 6: 10–15

    PubMed  Google Scholar 

  17. Rapola J (1987) Congenital nephrotic syndrome. Pediatr Nephrol 1: 441–446

    PubMed  Google Scholar 

  18. Whiteman P (1973) The quantitative determination of glycosaminoglycans in urine with Alcian blue 8GX. Biochem J 131: 351–357

    PubMed  Google Scholar 

  19. Galambos JT (1967) Effect of borate and sulfamate on the carbazole color of sugars. Anal Biochem 19: 119–132

    PubMed  Google Scholar 

  20. Lammi M, Tammi M (1988) Densitometric assay of nanogram quantities of proteoglycans precipitated on nitrocellulose membrane with Safranin O. Anal Biochem 168: 352–357

    PubMed  Google Scholar 

  21. Bradford MM (1976) A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal Biochem 72: 248–254

    Article  PubMed  Google Scholar 

  22. Michelacci YM, Glashan RQ, Schor N (1989) Urinary excretion of glycosaminoglycans in normal and stone forming subjects. Kidney Int 36: 1022–1028

    PubMed  Google Scholar 

  23. Gambaro G, Cicerello E, Mastrosimone S, Lavagnini T, Baggio B (1989) High urinary excretion of glycosaminoglycans: a possible marker of glomerular involvement in diabetes. Metabolism 38: 419–420

    PubMed  Google Scholar 

  24. Hotz P, Pilliod J, Boillat MA (1989) Glycosaminoglycans and urine flow rate. Nephron 51: 271–273

    PubMed  Google Scholar 

  25. Bower L, Warren C, Manley G (1992) Human serum and urine glycosaminoglycans in health and in patients with chronic renal failure. Ann Clin Biochem 29: 190–195

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Bacteriology and Immunology and Children's Hospital, University of Helsinki, Haartmaninkatu 3, P.O. Box 21, SF-00014, Helsinki, Finland

    Päivi Ljungberg

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  1. Päivi Ljungberg
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Ljungberg, P. Glycosaminoglycans in urine and amniotic fluid in congenital nephrotic syndrome of the Finnish type. Pediatr Nephrol 8, 531–536 (1994). https://doi.org/10.1007/BF00858117

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  • DOI: https://doi.org/10.1007/BF00858117

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