Juvenile Paget disease

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Juvenile Paget disease

ORPHA:2801Malformation syndrome

Also called Familial osteoectasia · Hereditary hyperphosphatasia · Hyperostosis corticalis deformans juvenilis · JPG

What it is

Juvenile Paget disease is a very rare form of Paget disease of the bone characterized by a general increase in bone turnover with increased bone resorption and deposition, resulting in cortical and trabecular thickening, and clinically presenting as progressive skeletal deformities, growth impairment, fractures, vertebral collapse, skull enlargement and sensorineural hearing loss.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Childhood
Inheritance
Autosomal 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

TNFRSF11BDisease-causing germline mutation(s)
TNFRSF11ACandidate gene tested

ICD-10 codes

M88.0filed under a broader ICD-10 category
M88.8filed under a broader ICD-10 category
M88.9filed under a broader ICD-10 category

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 2831MEDDRA 10078977MESH C537701MONDO 0009394OMIM 239000UMLS C0268414

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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