Fabry's disease natural history, complications and prognosis: Difference between revisions
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*With out treatment, life span of heterozygote females will be at seventh decade and the main causes of death are cardiac dysfunction, cancer and stroke. | *With out treatment, life span of heterozygote females will be at seventh decade and the main causes of death are cardiac dysfunction, cancer and stroke. | ||
== Complications == | ==Complications== | ||
* Common complications that can develop as a result of Fabry's disease are: | *Common complications that can develop as a result of Fabry's disease are: | ||
** Heart problems including [[cardiomyopathy]], [[Cardiac arrhythmia|Arrhythmia]], [[Heart failure]] | **Heart problems including [[cardiomyopathy]], [[Cardiac arrhythmia|Arrhythmia]], [[Heart failure]] | ||
** [[Renal failure]] (ESRD) | **[[Renal failure]] (ESRD) | ||
** [[Peripheral neuropathy]] | **[[Peripheral neuropathy]] | ||
** [[Stroke|Strokes]] (such as TIA) | **[[Stroke|Strokes]] (such as TIA) | ||
* Complications that can develop as a result of the treatment of Fabry's disease with enzyme replacement therapy are: | *Complications that can develop as a result of the treatment of Fabry's disease with enzyme replacement therapy are: | ||
** '''[[Infusion reaction|Infusion reactions]]:''' [[Rigor|rigors]], [[fever]], etc. | **'''[[Infusion reaction|Infusion reactions]]:''' [[Rigor|rigors]], [[fever]], etc. | ||
** '''[[Seroconversion]]:''' presents of [[Anti-body|anti-bodies]] may lower the efficacy of [[ERT]]. | **'''[[Seroconversion]]:''' presents of [[Anti-body|anti-bodies]] may lower the efficacy of [[ERT]]. | ||
==Prognosis== | |||
* The prognosis of Fabry's disease improves with treatment. Without treatment, Fabry's disease will result in reduced life expectancy. <br /> | |||
==References== | ==References== | ||
[1] 1-903539-03-XFabry, Hermann, et al. Fabry disease : perspectives from 5 years of FOS. Oxford: PharmaGenesis, 2006. Print. | [1] 1-903539-03-XFabry, Hermann, et al. Fabry disease : perspectives from 5 years of FOS. Oxford: PharmaGenesis, 2006. Print. | ||
[[Category:Needs content]] | [[Category:Needs content]] | ||
Revision as of 09:36, 4 April 2022
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Homozygotes
- The symptoms of classic Fabry's disease usually develop in childhood or adolescents , and start with symptoms such as neuropathic pains, angiokeratomas, dyshidrosis, GI symptoms, and etc.
- Without treatment, lifespan of homozygote men will dramatically reduced to fifth decade and the main causes of deaths are renal failure, heart disease or stroke.
Heterozygote
- The symptoms of late-onset Fabry's disease in females and atypical variants are accrue in higher ages and are less severe.
- With out treatment, life span of heterozygote females will be at seventh decade and the main causes of death are cardiac dysfunction, cancer and stroke.
Complications
- Common complications that can develop as a result of Fabry's disease are:
- Heart problems including cardiomyopathy, Arrhythmia, Heart failure
- Renal failure (ESRD)
- Peripheral neuropathy
- Strokes (such as TIA)
- Complications that can develop as a result of the treatment of Fabry's disease with enzyme replacement therapy are:
- Infusion reactions: rigors, fever, etc.
- Seroconversion: presents of anti-bodies may lower the efficacy of ERT.
Prognosis
- The prognosis of Fabry's disease improves with treatment. Without treatment, Fabry's disease will result in reduced life expectancy.
References
[1] 1-903539-03-XFabry, Hermann, et al. Fabry disease : perspectives from 5 years of FOS. Oxford: PharmaGenesis, 2006. Print.