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https://www.selleckchem.com/products/pmsf-phenylmethylsulfonyl-fluoride.html Peripheral nervous system involvement in primary systemic amyloidosis is another important organ involvement in the spectrum of this disease entity. Early recognition may lead to an earlier diagnosis and treatment with improvement in prognosis.Early diagnosis of AL amyloidosis and appreciation of the nutritional and coagulation abnormalities associated with liver and gastrointestinal involvement are critically important in the treatment and management. In cases of severe malabsorption total parenteral nutrition can be extremely helpful as a bridge to organ improvement. Rarely the use of antifibrinolytic agents such as oral aminocaproic acid with transfusion support may control severe bleeding in patients with coagulation abnormalities. It is important to keep in mind that organ improvement should follow in lag phase after the reduction in the pathologic free light chain with treatment. Closely following light chain levels may permit brief holidays from treatment and enable periods of recovery before resuming therapy in patients with prompt early and deep hematologic responses.Kidney involvement in immunoglobulin-related amyloidosis (AIg) is common. Although patients with renal-limited AIg tend not to have the high mortality that patients with cardiac amyloidosis have, they do experience significant morbidity and impact on quality of life. The complexity of the pathogenesis remains incompletely understood. Models have been established to prognosticate and assess for the response to therapy. Patients with advanced renal impairment from immunoglobulin light chain amyloidosis still have poor renal prognosis, and better therapy is needed in order to preserve kidney function. Patients who develop end-stage renal disease can undergo renal replacement therapy with kidney transplantation.Amyloid light chain amyloidosis (AL) is the most commonly diagnosed systemic form of amyloidosis, resulting from
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