Amyloidosis is the accumulation on misfolded protein fibers in the body. This is very common condition associated with many of the chronic illness.
Haemodialysis-associated amyloidosis is a form of systemic
amyloidosis
Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weight ...
associated with
chronic kidney failure
Chronic kidney disease (CKD) is a type of kidney disease in which a gradual loss of kidney function occurs over a period of months to years. Initially generally no symptoms are seen, but later symptoms may include leg swelling, feeling tired, vom ...
.
Even if this is common in CKD patients with chronic regular dialysis, it can be also seen in patient with CKD but have never dialysed too.
Presentation
Long-term
haemodialysis
Hemodialysis, also spelled haemodialysis, or simply dialysis, is a process of purifying the blood of a person whose kidneys are not working normally. This type of dialysis achieves the extracorporeal removal of waste products such as creatinin ...
results in a gradual accumulation of
β2 microglobulin, a serum protein, in the blood.
It accumulates because it is unable to cross the dialysis filter.
Affected individuals usually present after 5 years of dialysis rarely before that. The tendency of haemodialysis-associated amyloidosis is to be articular in general affecting the joints.
Diagnosis
Prevention
The mainstay of management of the dialysis related amyloidosis is the prevention than the other type of treatment methods. Because most of the medical and surgical managements for this condition may not prevent the symptoms completely. Therefore we have to take adequate precautions to prevent future dialysis disequilibrium syndrome in CKD patients.
There are several steps in prevention of dialysis related amyloidosis.
* Use of high flux dialyzers
* Use of Beta 2 globulin absorber
* Preserve the residual kidney functions
* Early kidney transplant
In addition low copper dialysis is theorized to prevent or delay onset.
Management
Management of haemodialysis associated amyloidosis is symptomatic. Although there are lot of methods to prevent and delay the complications, probably the steroids and analgesics may helpful in the management of the condition.
However there are some surgical procedure to reduce the pain.
See also
*
Hepatoerythropoietic porphyria
Hepatoerythropoietic porphyria is a very rare form of hepatic porphyria caused by a disorder in both genes which code Uroporphyrinogen III decarboxylase (UROD).
It has a similar presentation to porphyria cutanea tarda (PCT), but with earlier onse ...
*
List of cutaneous conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against t ...
References
External links
https://academic.oup.com/ndt/article-pdf/13/suppl_1/58/9896967/130058.pdf
Amyloidosis
Skin conditions resulting from errors in metabolism
Renal dialysis
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