Severe trichothiodystrophy and cardiac malformation in a newborn carrying a novel GTF2H5 homozygous truncating variant

Clin Genet. 2023 Nov;104(5):604-606. doi: 10.1111/cge.14396. Epub 2023 Jun 25.

Abstract

We report a newborn patient with trichothiodystrophy-3 (TTD3) caused by a novel homozygous variant in the GTF2H5 gene. His severe phenotype included congenital ichthyosis, complex posterior cranial fossa anomaly, life-threatening infections, bilateral cryptorchidism, and, notably, a complex cardiac malformation, which is unprecedented in TTD3 patients.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Homozygote
  • Humans
  • Infant, Newborn
  • Male
  • Phenotype
  • Transcription Factors / genetics
  • Trichothiodystrophy Syndromes* / genetics

Substances

  • GTF2H5 protein, human
  • Transcription Factors