Case of 46,XX/47,XY, +21 chimerism in a newborn infant with ambiguous genitalia

Am J Med Genet. 1994 Feb 15;49(4):428-30. doi: 10.1002/ajmg.1320490415.

Abstract

We describe whole-body chimerism in a newborn infant with small phallus, pseudo-vaginal perineal hypospadias, and a bifid scrotum containing gonads. The human testis determining factor gene (SRY) was detected by PCR amplification. GTG-banding chromosome analysis in peripheral blood lymphocytes and cultured fibroblasts derived from right cubital skin showed a 46,XX/47,XY, +21 karyotype. Their ratios in each cell line were 294:5 and 178:7, respectively. QFQ-banding chromosome analysis documented 3 heteromorphic satellites on trisomic chromosomes 21 in the 47,XY, +21 cell line and a homozygous satellite pattern in the 46,XX cell line. Heteromorphic patterns of chromosomes 4, 13, 14, and 22 were also different between the two cell lines. To our knowledge, such disomy/trisomy chimeras have not been described previously.

Publication types

  • Case Reports

MeSH terms

  • Cells, Cultured
  • Chimera*
  • Chromosome Aberrations*
  • Chromosome Banding
  • Chromosome Disorders*
  • DNA-Binding Proteins / genetics
  • Genitalia / abnormalities*
  • Humans
  • Infant, Newborn
  • Karyotyping
  • Nuclear Proteins*
  • Polymerase Chain Reaction
  • Sex-Determining Region Y Protein
  • Transcription Factors*

Substances

  • DNA-Binding Proteins
  • Nuclear Proteins
  • SRY protein, human
  • Sex-Determining Region Y Protein
  • Transcription Factors