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Chromosome 10 ring syndrome. Ring chromosome 10 syndrome.

Incidence

Very rare syndrome. Sporadic.

Clinical Characteristics

A syndrome in which both ends of chromosome 10 have been lost (deletion) and the two broken ends have reunited to form a ring-shaped figure. Psychomotor retardation, craniofacial abnormalities (mainly microcephaly and eye and nose deformities), hypoplastic male genitalia, transverse palmar creases, and occasional heart anomalies are the usual abnormalities. The phenotype is related to the breakpoint and mosaicism resulting from the unstable nature of the ring at cell division.

Precipitants

None

Provocation Tests

None

Diagnostic Procedures

Karyotype. Ring chromosome 10.

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