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Biallelic variants in GTPBP3: New patients, phenotypic spectrum, and outcome.

Francesca NardecchiaRosalba CarrozzoAlice InnocentiAlessandra TorracoValerio ZaccariaTeresa RizzaFrancesco PisaniEnrico BertiniVincenzo Leuzzi
Published in: Annals of clinical and translational neurology (2024)
Reviewed cases point toward clustering around two prevalent phenotypes: an early-onset presentation with severe fatal encephalopathy and a late milder presentation with global developmental delay/ID and cardiopathy, with the latter as, is the main feature. Our patients showed an intermediate phenotype with intrafamilial variability.
Keyphrases
  • early onset
  • end stage renal disease
  • ejection fraction
  • newly diagnosed
  • chronic kidney disease
  • peritoneal dialysis
  • prognostic factors
  • late onset
  • machine learning
  • gene expression
  • deep learning
  • case report