Wilson-Turner syndrome

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Wilson-Turner syndrome

ORPHA:3459Malformation syndrome

Also called WTS · X-linked intellectual disability-gynecomastia-obesity syndrome

What it is

Wilson-Turner syndrome (WTS) is a very rare X-linked multisystem genetic disease characterized by intellectual disability, truncal obesity, gynecomastia, hypogonadism, dysmorphic facial features, and short stature.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Childhood
Inheritance
X-linked dominant, X-linked recessive
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.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

HDAC8Disease-causing germline mutation(s) (loss of function)
LAS1LDisease-causing germline mutation(s)

ICD-10 codes

Q87.8filed under a broader ICD-10 category — shared with 581 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 5579MESH C536708MONDO 0010665OMIM 309585UMLS C1839736

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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