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Schematic representation of grading scheme for clinical classification of androgen insensitivity syndromes

Schematic representation of grading scheme for clinical classification of androgen insensitivity syndromes
Grades are numbered 1 to 7 in order of increasing severity (more defective masculinization).
  • Grade 1 – Typical virilization in utero
  • Grade 2 – Male phenotype with mild undervirilization, eg, isolated hypospadias
  • Grade 3 – Male phenotype with severe undervirilization, eg, small penis, perineoscrotal hypospadias, bifid scrotum and/or cryptorchidism
  • Grade 4 – Severe genital ambiguity, with intermediate phallic size, separated labioscrotal folds, and single perineal orifice
  • Grade 5 – Minimally virilized phenotype, with posterior labial fusion and clitoromegaly
  • Grade 6/7 – Typical female external genital appearance (grade 6 if pubic hair present in adulthood, grade 7 if no pubic hair in adulthood)
Reproduced with permission from: Quigley CA, De Bellis A, Marschke KB, et al. Androgen receptor defects: Historical, clinical, and molecular perspectives. Endocrine Reviews 1995; 16:271. Copyright © 1995 The Endocrine Society.
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