Renal amyloidosis

details about RENAL AMYLOIDOSIS in medical surgical nursing

1. NARAYANA HRUDAYALAYA COLLEGE OF NURSING PRESENTATION ON RENAL AMYLOIDOSIS Presented by Koyel T h n a d e r 1st p c b s c N H C O N

2. AMYLODOSIS : Amyloidosis is an abnormal protein that is usually produced in the bone marrow & can be deposited in any tissue organ .

3. RENAL AMYLODOSIS : The kidney is small & colour sectioned surface shows loss of corticomedullary distinction & pale , waxy transluencey .

4. - The amyloido deposits are seen mainly in the glomerullar c a p i l l a r y - The deposits are also present in pertibular connective tissue producing atrophic tubules & amyloid casts in the tubular lumina, & in the artial wall producing luminal narrowing .

5. Amyloid deposits damage the kidneys and make it harder for them to filter wastes & break down proteins . When the kidney become too damage they may no longer able to function well enough too maintain health , resulting kidney f a l i u r e

6. TYPES : 👉 A L amyloidosis ( primary amyloidosis ) 👉 AA amyloidosis ( secondery amyloidosis ) 👉 Heriditary amyloidosis ( Familial amyloidosis )

7. AL amyloidosis or primary amyloidosis : This is the most common from that occurs when the bone marrow produces too much certain fragment of antibody proteins , which builds up in the blood stream & can deposits in body tissue . AA amyloidosis or secondary amyloidosis : The develops along with a chronic infection or inflammatory disease , such as tuberculosis or rheumatoid arthritis .

8. Hereditary amyloidosis or familial amyloidosis : This is a genetic form passed down in families that often affects nerves & kidneys .

9. Pathogenesis of amyloidosis ▪️ For each of these amyloidogenic " precursor protein " - initially step in amyloid fibril formation is a misfolding event . ◾ Misfolding can result f r o m - proteolytic cleavage ( e.g-amyloid beta protein ) - an amnio acid substitution ( e.g-transthyretin [ TTR ] - intrinsic properties that becomes significant only at high serum concentration or in the presence of specific local factors .

10. ◾ Combination of these factors often determines the amyloidogenic potential of a particular protein . ◾ Regardless of the protein or the tigger for misfolding , the misfolded variants are highly prone to self aggregation . ◾ Self aggregation generates proto filaments that intract to from fibrils . ◾ Amyloid fibrils have a characteristics - beta pleated sheet configuration . - produces birefrigence under polarized light when stained with congo red dye . Factors that determine the organ distribution of amyloid deposits - not well understood .

11. How does amyloidosis cause renal disease : ◾ Disruption of tissue architecture by amyloid deposits . ◾ Amyloidogenic precursor proteins , folding intermediates , & proto filaments have toxicites that are independent of the amyloid deposits . - Lack of correlation between quantity of amyloid in tissue & organ dysfunction - detection of amyloidogenic precursor proteins in tissue in the absence of a m y l o i d - rapid improvement in makers of organ dysfunction after treatment induced reductions in precursor protein production .

12. Signs & symptoms : Signs and symptoms of amyloidosis are not usually experienced until the condition is advanced . ◾ Swelling in the ankles & l e g s ◾ Severe fatigue & weakens ◾ Shortness of b r e a t h ◾ Numbness , tungling, or pain in the hands or feet , especially pain in the wrist ( carpal tunnel syndrome ) ◾ Diarrhea , possibly with blood or constipation ◾ Feeling full quickly when eating & significant weight loss

13. Contd . . . . ◾ An enlarged tongue ◾ Skin changes , such as thickening or easy brushing , & purplish patches around the e y e s ◾ An irregular heart b e a t ◾ Difficulty swallowing .

14. Test & diagnosis : Diagnosis usually start with through medical history & physical examination . The following tests can be carried out : ◾ Laboratory tests with blood & urine to analyze for abnormal protein that can indicate amyloidosis . ◾ Biopsy is done by taking a sample tissue . Sample to check for signs of amyloidosis . The biopsy may be taken from the patients abnormal bone marro , or organ such as kidney . Tissue analysis can help determine the types

15. ◾ Imaging tests is carried by taking images of the organ affected by amyloidosis to help establish the extent of disease .

16. Treatment options for amyloidosis : Treatment of amyloidosis usually involves decreasing the protein that forms the amyloids , whuch is carried out depending on the organ affected . The treatment options included ◾ Use drug therapies of chemo therapy such s corticosteroids & a l k e r a n ◾ Dialysis if the kidney are failing ◾ Surgical & other procedures such a s - kidney or bone marrow transplant .

17. Preventive measure for amyloidosis : 1. Those who have hereditary amyloidosis should consider going to generic counseling to learn about the risks of passing the condition to their children . 2.studies suggest that the following dieatry choices such as limiting meat consumption taking fish oils supplements & vit c may help to prevent amyloidosis in people who are at high risk , or help slow the disease once it has developed .