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Lipoid proteinosis: Novel ECM1 pathogenic variants and intrafamilial variability in four unrelated Arab families.

Mingfeng LiJudith FischerSylvia SafwatWalaa ShomanYasmine El ChazliSvenja AlterCristina HasEbtesam Abdalla
Published in: Pediatric dermatology (2022)
Although all patients had characteristic manifestations of lipoid proteinosis, we observed intrafamilial phenotypic variability. Our data expand the pathogenic variant spectrum of ECM1 and also supports the fact that exon 6 is one of the most common hot spots of pathological variants in ECM1.
Keyphrases
  • end stage renal disease
  • extracellular matrix
  • copy number
  • ejection fraction
  • newly diagnosed
  • chronic kidney disease
  • peritoneal dialysis
  • prognostic factors
  • gene expression
  • big data