Kidney diseases

Abstract

The complement system, a central part of the innate immunity that serves as a first line of defense against foreign and altered host cells, is an extremely effective cell-killing and inflammation-provoking pathway. However, complement activation is a double-edged sword because uncontrolled stimulation can be highly detrimental to host tissues. [1][2][3] In order to avoid self-damage, a plethora of inhibitory mechanisms are known to prevent overwhelming activation at all stages of the complement cascade. The alternative pathway (AP) of complement is particularly significant for survival against invading pathogens and can be triggered by several other conditions, such as trauma, surgery, or pregnancy. Inappropriate AP activation may be

The human factor H protein family – an update

“…It is a chronic autoinflammatory disease, with several risk factors: predisposing genetic variations in complement genes, ageing, lifestyle, and environmental triggers and, despite intensive efforts, there is still no appropriate therapy curing the disease ( 61 , 62 ). C3 glomerulopathy (C3G) describes diseases with glomerular C3 deposition without the presence of Ig, the two main forms being dense deposit disease (DDD) and C3 glomerulonephritis ( 63 – 65 ). The Bruch’s membrane in the eye and the glomerular basement membrane in the kidney lack membrane-bound complement regulators, therefore they are vulnerable to complement activation and complement mediated damage due to dysfunction or deregulation of the soluble regulators FHL-1 and FH ( 66 ).…”

The complement alternative pathway in health and disease—activation or amplification?

“…Daina et al 38 review the extensive data linking the AP/AL with the pathogenesis of various kidney diseases. The two prototypical complement‐mediated kidney diseases are atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G).…”

“…Daina et al 38 demonstrating that production of complement proteins by kidney cells is a critical determinant of transplant rejection. 41 Although the liver is generally considered the source of most complement, extrahepatic production of complement proteins, such as by kidney tubular cells, creates a microenvironment which favors AP/AL activation.…”

A Worrying and Puzzling Case of Hypertension Presenting to the Emergency Department

“…A blood smear demonstrated red blood cells anisopoikilocytosis with schistocytes and increased (3%) helmet cells (Table 1 3). [3][4][5] When facing such phenotype, it is urgent to rule out thrombotic thrombocytopenic purpura (TTP), a condition with a dreadful course (Figure 4), 3,[6][7][8][9] which could be excluded by the normal values of ADAMTS 13. 10 The patient was treated with 1 unit of red blood cells, KCl supplementation, and antihypertensive treatment that allowed achievement of control of the high BP values over 4 days.…”

“…Collectively, the high BP values with worsening renal function and grade intravenous retinopathy indicated a hypertensive emergency, specifically malignant hypertension, with myocardial damage, impaired renal function and hemolysis, that is, hemolytic uremic syndrome (HUS), featuring a thrombotic microangiopathy (TMA; Figure 3). 3–5 When facing such phenotype, it is urgent to rule out thrombotic thrombocytopenic purpura (TTP), a condition with a dreadful course (Figure 4), 3,6–9 which could be excluded by the normal values of ADAMTS 13. 10…”