Abstract
The clinical classification of nephrotic syndrome (NS) is based on age at presentation. However, this classification is arbitrary because the majority of early onset NS has a genetic origin and has a widespread age of onset (from fetal life to several years). The aims of this review are to illustrate the knowledge accumulated on congenital nephrotic syndrome (CNS) in terms of genetics, classification, findings at histology and US–based on a review of the literature.
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References
Jalanko H (2009) Congenital nephrotic syndrome. Pediatr Nephrol 24:2121–2128
Ismaili K, Pawtowski A, Bayer O et al (2009) Genetic forms of nephrotic syndromes: a single center experience in Brussels. Pediatr Nephrol 24:287–294
Niaudet P, Gubler MC (2006) WT1 and glomerular diseases. Pediatr Nephrol 21:1653–1660
Jolly M, Goodburn S, Cox P et al (2003) Congenital nephropathy and ventriculomegaly: a report of 4 cases. Prenat Diagn 23:48–51
Mark K, Reis A, Zenker M (2006) Prenatal finding in four consecutive pregnancies with fetal Pierson syndrome, a newly defined congenital nephrosis syndrome. Prenat Diagn 26:262–266
Demmer L, Primack W, Loik V et al (1999) Frasier syndrome: a cause of focal segmental glomerulosclerosis in a 46, XX female. J Am Soc Nephrol 10:2215–2218
Cohen AH, Turner MC (1994) Kidney in Galloway-Mowat Syndrome: clinical spectrum with description of pathology. Kidney Int 45:1407–1415
Debiec H, Ronco P (2007) Feto-maternal allo-immunization with antenatal glomerulopathies. Ann NY Acad Sci 1110:559–566
Giani M, Edefonti A, Damiani B et al (1996) Nephrotic syndrome in a mother and her infant: relationship with cytomegalovirus infection. Pediatr Nephrol 10:73–75
Northrup M, Mendez-Castillo A, Brown JC et al (2003) Congenital nephrotic syndrome, Finnish type: sonographic appearance and pathologic correlation. J Ultrasound Med 22:1097–1099
Saraga M, Jääskeläinen J, Koskimies O (1995) Diagnostic sonographic changes in the kidneys of 20 infants with congenital nephrotic syndrome of the Finnish type. Eur Radiol 5:49–54
Salame H, Damry N, Vandehoudt K et al (2003) The contribution of ultrasound for the differential diagnosis of congenital and infantile nephrotic syndromes. Eur Radiol 13:2674–2679
Chaumoitre K, Brun M, Cassart M et al (2006) Differential diagnosis of fetal hyperechogenic cystic kidneys unrelated to renal tract anomalies: a multicenter study. Ultrasound Obstet Gynecol 28:911–917
Nortier JL, Debiec H, Tournay Y et al (2006) Neonatal disease in neutral endopeptidase alloimmunisation: lessons for immunological monitoring. Pediatr Nephrol 21:1399–1405
Habib R, Bois E (1973) Heterogeneity of early onset nephrotic syndromes in infants (nephrotic syndrome “in infants”). Anatomical, clinical and genetic study of 37 cases. Helv Pediatr Acata 28:91–107
Habib R, Gubler M, Antignac C et al (1990) Congenital or childhood nephrotic syndrome with diffuse mesangial sclerosis. Am Pediatr (Paris) 37:73–77
Debiec H, Guigonis V, Mougenot B et al (2002) Antenatal membranous glomerulonephritis due to anti-neutral endopeptidase antibodies. N Engl J Med 346:2053–2060
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Avni, E.F., Vandenhoute, K., Devriendt, A. et al. Update on congenital nephrotic syndromes and the contribution of US. Pediatr Radiol 41, 76–81 (2011). https://doi.org/10.1007/s00247-010-1793-5
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DOI: https://doi.org/10.1007/s00247-010-1793-5