Amyloidosis Kidney

Ganeval, D. Noel, L.H. Preud home, J.L. Droz, D. Grunfeld, J.P. Light-chain deposition disease its relation with AL-type amyloidosis. Kidney Int. 1984, 2d (1), 1-9. 

Lymphocytic inflammation of the urinary bladder was noted in mice chronically exposed to 250 or 500 mg/kg/day marine diesel fuel. Amyloidosis of the kidney was found to be secondary to dermatitis in mice chronically exposed to 500 mg/kg/day JP-5 (NTP/NIH 1986). 

The commonest causes of death in the Jamaican patients with multiple myelomatosis were bronchopneumonia and other infectious complications, a finding which is compatible with the secondary antibody deficiency syndrome and impaired cellular immunity which occurs in patients with this disease. Bleeding manifestations and renal failure were not uncommon findings, and myeloma kidney was observed in 66% of the cases. Skeletal involvement was observed, but in many cases the typical lesions had to be searched for. Amyloidosis was present in as many as 21% of the patients (Tl) and this may be associated with the high number of patients in Jamaica that are known to excrete Bence Jones protein in myeloma (Mil). 

Heterozygous carriers of functionally relevant mutations usually present with HDL cholesterol levels that are frequently below the fifth percentile. As would be expected, apoA-I levels are also frequently below the fifth percentile (i.e., < 1.05 g/1 and < 1.1 g/1 in Caucasian men and women, respectively). In most cases, heterozygous carriers of apoA-I variants do not present with specific clinical symptoms. An important exception are some structural apoA-I variants with amino acid substitutions in the amino terminus, which have been detected in patients with familial amyloidosis of the liver, the intestine, the kidney, the heart, peripheral nerves, and in the skin. In addition, some apoA-I variants like apoA-I L178P or L159P have been associated with increased risk of premature coronary heart disease or enhanced progression of carotid intima media thickness, whereas others did not show this association, or were even claimed to have reduced cardiovascular risk and advocated as possible agents for the treatment or prevention of atherosclerosis (notably apoA-I R173CMiiano) [22,43,53]. 

Kidney disease Upper respiratory tract infection Urinary tract infections Amyloidosis Tubular disease Glomerulonephritis... 

There are several other metabolic disorders, but because they are not very common, not much is known about them. For example, a disease known as amyloidosis results when enough amyloid protein builds up in one or more organs to cause the organ(s) to malfunction. The heart, kidneys, nervous system and gastrointestinal tract are most often affected. Amyloid (pronounced am -i-loyd) is an abnormal protein that may be deposited in any of the body s tissues or organs. This abnormal protein comes from cells in the bone marrow, so amyloidosis is known as a bone marrow disease. The bone marrow makes protective antibodies that protect against infection and disease. After they have served their function, these antibodies are broken down and recycled by... 

A physieal examination is necessary to find out if the organs are functioning properly. Blood, urine and bone marrow tests may also be done. A small tissue sample (biopsy) may be taken from the rectum, abdominal fat or bone marrow to determine if the person has amyloidosis. These biopsies are relatively minor procedures done in an outpatient setting with a local anesthetic. Occasionally, samples are taken from the liver, nerve, heart or kidney. This may require hospitalization and can help diagnose the specific oi an affected by amyloidosis. Blood or urine tests can detect the protein, but only bone marrow tests or other small samples of tissue can positively establish the di nosis of amyloidosis. 

AA amyloid appears in a generalized form and is mainly stored perireticularly in the kidney, spleen and liver. This kind of amyloidosis occurs as (1.) a congenital form in cases of familial Mediterranean fever, (2.) an idiopathic (= primary) form without any associated basic disease, and (i.) a reactive (= secondary) form in chronic inflammations or tumours (e.g. Hodgkin s disease) as well as in drug abuse and AIDS. 

A 63-year-old woman with rheumatoid arthritis was given intramuscular gold sodium thiomalate and began to have nausea, vomiting, anorexia, and watery diarrhea (71). A year later the watery diarrhea became more frequent (more than 10 times within a day) and she developed proteinuria. Biopsies from the stomach, duodenum, and kidney showed systemic amyloidosis. This was a rare case of secondary systemic amyloidosis associated with rheumatoid arthritis. It is not clear from the report what the role of gold was in this case. 

Heroin nephropathy/clinical course Amyloidosis associated with intravenous drug abuse HIV nephropathy and its relationship to heroin nephropathy Acute kidney injury due to drug-induced rhabdomyolysis Cocaine-induced renal disease 598 599 601 603 605... 

Crowleys, Feinfeld DA,JanisR. Resolution of nephrotic syndrome and lackof progression of heroin associated renal amyloidosis. Am J Kidney Dis 1989 13 333-335. 

Nephrogenic Diabetes Insipidus. Failure of the kidney to respond to normal or increased concentrations of AVP can cause NDI. In the majority of these patients, AVP is mcapable of stimulating cychc adenosine monophosphate (cAMP) formation. Two causes have been described for this disorder (1) mutation in the vasopressin receptor and (2) mutations in the aquaporin-2 water channels. Hie vasopressin receptor mutation form of NDI is an X-chromosome-linked disorder that mostly affects males. Females are more likely to have the aquaporin-2 water channel gene defect on chromosome 12,ql2-13, which produces an autosomal recessive disease. Acquired forms of NDI may be caused by metabolic disorders (hypokalemia, hypercalcemia, and amyloidosis), drugs (hthium, demeclocycline, and barbiturates), and renal diseases (polycystic disease and chronic renal failure). NDI may also be seen in the absence of these factors (idiopathic). 

Renal effects were not observed in dogs exposed intermittently to 25 ppm 1,1-dimethylhydrazine for 13-26 weeks (Rinehart et al. 1960). Mild renal effects including amyloidosis and mineralization were observed in hamsters exposed intermittently to 0.25 ppm hydrazine for 1 year (Vemot et al. 1985) however, no effects were noted in the kidneys of mice exposed intermittently to 1 ppm hydrazine for 1 year (Vemot et al. 1985). The findings of these animal studies are inconsistent with the severe effects observed in the human case study. However, more severe effects on the kidney have been observed in animals exposed to hydrazines by other routes (see Sections 2.2.2.2 and 2.4). 

Rats chronically fed selenite in the diet were reported to exhibit more frequent and more severe nephritis than those given equivalent amounts of selenate (Harr et al. 1967) however, the study authors did not quantify these observations or statistically compare data from the two groups. An increased incidence of amyloidosis of the major organs, including the kidneys, was observed in mice following lifetime exposure to sodium selenate or sodium selenite in drinking water at a level of 0.57 mg selenium/kg/day (Schroeder and Mitchener 1972). This effect was noted in 30% of control mice and 58% (p<0.001) of selenium-treated mice. Data for individual organs were not provided. 

Renal effects were also noted in a study conducted by Deelman (1962), in which mice were exposed dermally to an unspecified amount of tar applied 6-18 times over 6-7 weeks. "Seriously affected" kidneys were noted, although no details or data were presented. Ten groups of Swiss albino mice received topical applications of 10% benzene solutions of eight petroleum asphalts of known polynuclear aromatic hydrocarbon content (Wallcave et al. 1971). An additional group of 15 males and females were painted with benzene only and served as controls. Zones of approximately 1 square inch were shaved in the skin of the back of each animal and the solutions of asphalt were painted in this area 2 times/week with 25 mL of solution (approximately 2.5 mg asphalt per treatment). Amyloidosis was frequently observed in animals receiving asphalt, particularly in the kidney. The potential effect of benzene on the dermal absorption of asphalt components was not evaluated in this study. 

In AL amyloidosis, amyloid is formed from degradation products of the X or k light chains that deposit most frequently in the extracellular matrix of the kidney and the heart but also may deposit in the tongue. In other types of amyloidosis, the amyloid arises from other proteins and deposits in a characteristic organ. For example, the amyloid associated with chronic inflammatory conditions, such as tuberculosis or rheumatoid arthritis, is derived from an acute phase serum protein called serum amyloid Athat is produced by the liver in response to inflammation. It deposits most frequently in the kidney, and cardiac involvement is rare. 

Morgan CJ, Gelfand M, Atreya C, Miranker AD (2001) Kidney dialysis-assodated amyloidosis a molecular role for copper in fiber formation. J Mol Biol 309 339-345 Murphy RM (2002) Peptide aggregation in neurodegenerative disease. Annu Rev Biomed Eng 4 155-174 Mutter M, Vuilleumier S (1989) A chemical approach to protein design— template-assembled synthetic proteins (TASPs). Angew Chem Int Ed Engl 28 535-554... 

The use of high-flux membranes gained significant support in 1985 when Geyjo et al. [343] conclusively estahfished the link between the accumulation of P2M and a compfication of long-term dialysis called dialysis-related amyloidosis (DRA). As kidney failure progresses, P2M concentration in the extracellular compartments increases, often to levels... 

The physiology of amyloid pleural effusions is complex. AL amyloidosis can alter the function of several organs that could contribute to effusion formation including the heart (infiltrative cardiomyopathy—48% cases), kidneys (nephrotic syndrome—65% cases), and thyroid (hypothyroidism—4% cases) (20). 

Jadoul M, Garbar C, Noel H, et al. Histological prevalence of beta 2-microglobulin amyloidosis in hemodialysis a prospective post-mortem smdy. Kidney Int 1997 51 (6) 1928-1932. 

Biological Applications Calcium indicatois zinc indicators treating acute cell death, cerebral infarction, myocardial infarction, hepatonecrosis, kidney ischemic necrosis, necrotizing pancreatitis, amyloidosis, atherosclerosis, diseases characterized by calcification and/or plaque formation, osteoporosis, Paget s disease, heterotropic ossification, hypercalce-mia, cancer, inflammation, diabetes mellitus, epilepsy, epithelial disorders, glaucoma, HIV-associated conditions, respiratory disorders, streptococcal infection, viral diseases ... 

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