Fabry's disease classification: Difference between revisions
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==Classification== | ==Classification== | ||
===Based upon | ===Based upon Phenotype=== | ||
{| class="wikitable" | |||
|+ | |||
! | |||
!Age of onset | |||
!Severity | |||
![[alpha-Gal A]] activity | |||
|- | |||
|Classic | |||
|Childhood (mostly) | |||
|severe | |||
|no activity or<1% of the normal mean | |||
|- | |||
|Atypical (later onset) | |||
|Third to seventh decades | |||
|less severe | |||
|2 to 30% of the normal mean | |||
|} | |||
<nowiki>*</nowiki>[[Heterozygous]] females can categorized in both groups based on the severity of the disease, from severe classic ones to less severe atypical and even no symptoms. | |||
*Based upon complications | *Based upon complications | ||
**Cardiac variant | **Cardiac variant | ||
**Renal variant | **Renal variant | ||
**Non-neuropathic form | **Non-neuropathic form | ||
Revision as of 19:24, 12 March 2022
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Fabry's disease Microchapters |
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Fabry's disease classification On the Web |
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American Roentgen Ray Society Images of Fabry's disease classification |
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Risk calculators and risk factors for Fabry's disease classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
Based upon Phenotype
| Age of onset | Severity | alpha-Gal A activity | |
|---|---|---|---|
| Classic | Childhood (mostly) | severe | no activity or<1% of the normal mean |
| Atypical (later onset) | Third to seventh decades | less severe | 2 to 30% of the normal mean |
*Heterozygous females can categorized in both groups based on the severity of the disease, from severe classic ones to less severe atypical and even no symptoms.
- Based upon complications
- Cardiac variant
- Renal variant
- Non-neuropathic form
- Neuropathic form
- Infantile form
- Juvenile form