Abstract
In this paper, a boy is reported with the association of congenital analbuminemia (CAA) and steroid-sensitive idiopathic nephrotic syndrome (INS), two conditions resulting independently in reduced colloid oncotic pressure. The unique occurrence helps confirm earlier reports that albumin is not the exclusive factor responsible for maintaining colloid oncotic pressure.
References
Adamson O, Trachtman H, Tejani A (1986) Urinary protein electrophoresis patterns in childhood idiopathic nephrotic syndrome. Int J Pediatr Nephrol 7:181–186
Boner G, Cox AJ, Kelly DJ, Tobar A, Bernheim J, Langham RG, Cooper ME, Gilbert RE (2003) Does vascular endothelial growth factor (VEGF) play a role in the pathogenesis of minimal change disease? Nephrol Dial Transplant 18:2293–2299
Brocklebank T, Cooper EH, Richmond K (1991) Sodium dodecyl sulphate polyacrylamide gel electrophoresis patterns of proteinuria in various renal diseases of childhood. Pediatr Nephrol 5:371–375
Campagnoli M, Sala A, Labo S, Rossi A, Neuhaus TJ, Braegger CP, Minchiotti L, Galliano M (2005) Analbuminemia in a Swiss family is caused by a C --> T transition at nucleotide 4446 of the albumin gene. Clin Biochem 38:819–823
Deschênes G, Guigonis V, Doucet A (2004) Molecular mechanism of edema formation in nephrotic syndrome. Arch Pediatr 11:1084–1094
Fujihara CK, Arcos-Fajardo M, Brandão De Almeida Prado E, José Brandão De Almeida Prado M, Sesso A, Zatz R (2002) Enhanced glomerular permeability to macromolecules in the Nagase analbuminemic rat. Am J Physiol Renal Physiol 282:F45–F50
Ciba-Geigy (1979) Geigy Scientific Tables, 8th edn. Ciba-Geigy, Basel, Switzerland
Gossi B, Kleinert D, Gossi U (2000) A further case of analbuminemia. Schweiz Med Wochenschr 130:583–589
Kallee E (1996) Bennhold’s analbuminemia: a follow-up study of the first two cases (1953–1992). J Lab Clin Med 127:470–480
Kapur G, Valentini RP, Imam AA, Jain A, Mattoo TK (2007) Serum osmolal gap in patients with idiopathic nephrotic syndrome and severe edema. Pediatrics 119:e1404–e1407
Kemper MJ, Altrogge H, Ganschow R, Müller-Wiefel DE (2002) Serum levels of immunoglobulins and IgG subclasses in steroid sensitive nephrotic syndrome. Pediatr Nephrol 17(6):413–417
Koot BGP, Houwen R, Pot D-J, Nauta J (2004) Congenital analbuminaemia: biochemical and clinical implications. A case report and literature review. Eur J Pediatr 163:664–670
Nguyen MK, Kurtz I (2004) New insights into the pathophysiology of the dysnatremias: a quantitative analysis. Am J Physiol Renal Physiol 287:F172–F180
Palmer BF, Alpern RJ (1997) Pathogenesis of edema formation in the nephrotic syndrome. Kidney Int Suppl 59:S21–S27
Rostoker G, Behar A, Lagrue G (2000) Vascular hyperpermeability in nephrotic edema. Nephron 85:194–200
Russi E, Weigand K (1983) Analbuminemia. Klin Wochenschr 61:541–545
Taylor GM, Neuhaus TJ, Shah V, Dillon S, Barratt TM (1997) Charge and size selectivity of proteinuria in children with idiopathic nephrotic syndrome. Pediatr Nephrol 11:404–410
The Albumin web site. Home page at http://www.albumin.org
[No authors listed] (1981) The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr 98:561–564
Vande Walle JGJ, Donckerwolcke RA (2001) Pathogenesis of edema formation in the nephrotic syndrome. Pediatr Nephrol 16:283–293
Watkins S, Madison J, Galliano M, Minchiotti L, Putnam FW (1994) Analbuminemia: three cases resulting from different point mutations in the albumin gene. Proc Natl Acad Sci USA 91:9417–9421
Webb NJA, Watson CJ, Roberts ISD, Bottomley MJ, Jones CA, Lewis MA, Postlethwaite RJ, Brenchley PEC (1999) Circulating vascular endothelial growth factor is not increased during relapses of steroid-sensitive nephrotic syndrome. Kidney Int 55:1063–1071
Weinstock JV, Kawanishi H, Sisson J (1979) Morphologic, biochemical and physiologic alterations in a case of idiopathic hypoalbuminemia (analbuminemia). Am J Med 67:132–139
Wraight EP (1974) Capillary permeability to protein as a factor in the control of plasma volume. J Physiol 237:39–47
Author information
Authors and Affiliations
Corresponding author
Additional information
Interest: no interest declared.
Rights and permissions
About this article
Cite this article
Neuhaus, T.J., Stallmach, T. & Genewein, A. A boy with congenital analbuminemia and steroid-sensitive idiopathic nephrotic syndrome: an experiment of nature. Eur J Pediatr 167, 1073–1077 (2008). https://doi.org/10.1007/s00431-007-0620-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00431-007-0620-y