Childhood-onset hypophosphatasia

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Childhood-onset hypophosphatasia

ORPHA:247667Clinical subtype

Also called Childhood-onset Rathbun disease · Childhood-onset phosphoethanolaminuria

What it is

A rare, moderate form of hypophosphatasia (HPP) characterized by onset after six months of age and widely variable clinical features from low bone mineral density for age, to unexplained fractures, skeletal deformities, and rickets with short stature and waddling gait.

Key facts

Age of onset
Childhood, Infancy
Inheritance
Autosomal dominant, Autosomal recessive
Classified as
Clinical subtype

Recorded for the broader condition

Prevalence
<1 / 1 000 000 (China)Hypophosphatasia

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Gene

ALPLDisease-causing germline mutation(s) (loss of function)

ICD-10 codes

E83.3filed under a broader ICD-10 category — shared with 15 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 8735MESH C562440OMIM 241510UMLS C0220743

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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