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Jacobs syndrome (triple x syndrome; xxx syndrome; super female syndrome)


Jacobs Syndrome (Triple X Syndrome; XXX Syndrome; Super Female Syndrome)

General: Caused by sex chromosomal anomaly with 44 autosom 434i81e al and 3X sex chromosomes; due to nondisjunction; often associated with autosomal trisomies; majority of cases are asymptomatic.

Ocular: Hypertelorism; epicanthus; mongoloid, slanted lid fissure; strabismus.

Clinical: Microcephaly; oligophrenia, occasionally with secondary amenorrhea; abnormal dentition; high-arched palate; hypogenitalism; occasionally mental retardation; early menopause.

Jacobs PA, et al. Evidence for the existence of the human 'superfemale.' Lancet 1959; 1:423.

Kohn G, et al. Trisomy X in three children. J Pediatr 1968; 72:248.




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