Mosaic genome-wide paternal uniparental…

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Mosaic genome-wide paternal uniparental disomy syndrome

ORPHA:329813Malformation syndrome

Also called Androgenetic/biparental mosaicism · Genome-wide paternal uniparental disomy mosaicism · Mosaic genome-wide paternal UPD

What it is

A rare chromosomal anomaly characterized by a combination of paternal uniparental and biparental cell lineages, leading to variable clinical presentation that predominantly includes features of Beckwith-Wiedemann syndrome and increased risk of various tumors. In addition, features of Angelman syndrome and transient neonatal diabetes might be expected.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Antenatal, Neonatal
Classified as
Malformation syndrome

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

ICD-10 codes

Q95.8filed under a broader ICD-10 category

Cross-references

MONDO 0018007UMLS C5190828

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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