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Pediatric Nephrology

, Volume 22, Issue 5, pp 661–669 | Cite as

Familial steroid-sensitive nephrotic syndrome in Southern Israel: clinical and genetic observations

  • Daniel LandauEmail author
  • Tal Oved
  • Dan Geiger
  • Luba Abizov
  • Hanna Shalev
  • Ruti Parvari
Original Article

Abstract

Reports on genetically informative steroid-responsive (sensitive) idiopathic nephrotic syndrome (SSNS) families are lacking. We studied an extended SSNS Bedouin (B) family with a high rate of consanguinity. The clinical presentation and steroid response of its 11 affected individuals were similar to those of sporadic SSNS (spontaneous remission towards puberty and minimal change disease by kidney biopsy). Genome-wide linkage analysis, using a 382 microsatellite-markers mapping set and additional markers adjacent to 80 candidate genes of the index family, did not support linkage to any chromosomal locus. Retrospective analysis of all additional children with SSNS treated by our institution in the past 20 years (n = 96, 50% of them of Jewish origin) revealed another five non-related B families with 2–3 first-degree cousins affected with SSNS in each. The overall familial SSNS rate among the B population (excluding the index family) was 28%, compared with 4% among Jews (Js) (OR 1.8–64, P < 0.005). There were more Bs with simple SSNS than there were Js (71% and 40%, respectively; OR 3.58, 95% CI 1.41–9.23, P < 0.01). In summary, SSNS in this index family was not linked to any of the presently known chromosomal loci nor predicted to be caused by mutation in any one of a list of genes associated with nephrotic syndrome (NS). The presence of other B families affected by SSNS supports the role for susceptibility genes enrichment, exposing highly consanguineous populations to an increased incidence of SSNS.

Keywords

Chromosome mapping Consanguinity Nephrosis Lipoid Arabs Jews 

Abbreviations

B

bedouin

FSGS

focal segmental glomerulosclerosis

GBM

glomerular basement membrane

J

jew

LOD

logarithmic odds

MCNS

minimal change nephrotic syndrome

NS

nephrotic syndrome

SSNS

steroid sensitive idiopathic nephrotic syndrome

Notes

Acknowledgment

Dan Geiger’s research was supported by the Israeli Ministry of Science. This study was partially supported by a research grant from the National Institute of Biotechnology in the Negev. We thank Prof. Ilana Shoham-Vardi for her help with the statistical calculations.

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Copyright information

© IPNA 2007

Authors and Affiliations

  • Daniel Landau
    • 1
    • 4
    Email author
  • Tal Oved
    • 2
  • Dan Geiger
    • 3
  • Luba Abizov
    • 2
  • Hanna Shalev
    • 1
    • 4
  • Ruti Parvari
    • 2
  1. 1.Department of Pediatrics, Faculty of Health SciencesBen Gurion University of the NegevBeer-ShevaIsrael
  2. 2.Department of Developmental Genetics and Virology, Faculty of Health SciencesBen Gurion University of the NegevBeer-ShevaIsrael
  3. 3.Computer Science DepartmentTechnionHaifaIsrael
  4. 4.Department of PediatricsSoroka Medical CenterBeer ShevaIsrael

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