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α1-Antitrypsin deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
In addition to disease of the lungs, membranous glomerulonephritis has been observed histopathologically, and some of these patients have had signs of renal dysfunction [42]. Some evidence of glomerular disease is common at post-mortem examination in patients dying of liver disease. Immune complex disease is suggested by immunofluorescent evidence of α1-AT along with immunoglobulins and C3 on the glomerular basement membrane. A variety of inflammatory disorders have been associated with heterozygosity for the Z allele, including rheumatoid arthritis [43] and uveitis [44]. Severe panniculitis has been reported in 22 homozygotes [45, 46].
The kidneys
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
This disease occurs over a wide age range, although its peak incidence is in middle age. Idiopathic membranous glomerulonephritis shows a variable clinical course. Some cases may remit spontaneously but a significant proportion of patients pursue an indolent course for many months or years, ending in chronic renal failure. Treatment remains controversial. The effects of steroids and cytotoxic drugs on the course of the disease are uncertain and are still the subject of clinical trials.
Yi Shen An, a Chinese traditional prescription, ameliorates membranous glomerulonephritis induced by cationic bovine serum albumin in rats
Published in Pharmaceutical Biology, 2022
Yun-Li Zhao, Xiang-Hua Zhang, Feng Guo, Ying Wei, Jian-Hua Shang, Xiao-Dong Luo
Membranous glomerulonephritis (MGN) is a glomerular disease with distinct features in which the gradual accumulation of immunoglobulin and complement occurs in a discontinuous granular pattern in the subepithelial space, thus resulting in a marked increase in capillary permeability with proteinuria and nephrotic syndrome (Salant et al. 1980; Lai et al. 2015). Cationic bovine serum albumin (C-BSA)-induced glomerulonephritis, an animal model of immune complex glomerular injury, was previously developed to investigate the immunological mechanisms of human glomerulonephritis. This model has been used to investigate a variety of experimental animals and has been shown to exhibit features that are characteristic of human glomerulonephritis (Wu et al. 2009; Wang et al. 2016). Pathogenesis occurs due to the binding of positively charged C-BSA to negatively charged GBM, thus resulting in the formation of immune complexes (e.g., IgG, IgA, and IgM) in the glomeruli (Doi et al. 1984; Huang et al. 2013; Motiram et al. 2016). These complexes may trigger the complement system and result in glomerular injury, eventually leading to the induction of glomerulonephritis and impaired renal function (Couser 1985; Paccaud and Schifferli 1989). Approximately half of the patients with MGN exhibit positive staining for C3, predominantly C3c, a short-lived breakdown product of C3, thus indicating the ongoing formation of immune deposits and active disease (Nangaku et al. 2005).
A case of IgG4-related tubulointerstitial nephritis and membranous glomerulonephritis during the clinical course of gastric cancer: Imaging features of IgG4-related kidney disease
Published in Modern Rheumatology, 2019
Shigeto Horita, Hiroshi Fujii, Ichiro Mizushima, Yuhei Fujisawa, Satoshi Hara, Kazunori Yamada, Dai Inoue, Kenichi Nakajima, Kenichi Harada, Mitsuhiro Kawano
A renal biopsy showed diffuse dense lymphoplasmacytic cell infiltrates with scattered eosinophils and tubular atrophy within the renal interstitium. Periodic acid methenamine silver (PAM) staining revealed spike and bubbling formation in the glomeruli, and “bird’s-eye” pattern of fibrosis in the interstitium (Figure 2A–C). On IgG4 immunostaining, the number of IgG4+ plasma cells (PCs) was 31/ high power field (hpf) and IgG4/CD138 ratio >40% (Figure 2D). Immunofluorescence microscopy revealed granular deposits of IgG4, and to a lesser extent of IgG1, along the glomerular basement membrane (GBM) and tubular basement membrane (TBM). (Figure 3). Weak staining of C3 and faint staining of C1q were also detected in GBM and TBM. The patient was diagnosed with IgG4-TIN and membranous glomerulonephritis.
Intravenous immunoglobulins modify relapsing membranous glomerulonephritis after kidney transplantation: a case report
Published in Acta Clinica Belgica, 2018
Sanne Steyaert, Jo Van Dorpe, Anne Hoorens, Wim Van Biesen, Steven Van Laecke
Idiopathic membranous glomerulonephritis (IMGN) is the second most common cause of nephrotic syndrome in the Western adult population and a possible cause of end stage renal disease. Although a kidney biopsy is still essential for diagnosis, both serum and podocyte anti-phospholipase 2-receptor antibodies (APLA2R) have emerged as an additive diagnostic and predictive aid, with potential use before and after kidney transplantation [1]. In patients with primary glomerulonephritis these antibodies are positive in 80–85% of cases. Another 3–5% will express thrombospondin type-1 domain containing 7A (THSD7A), a different transmembrane protein found in the podocyt, while in the remaining 10–15% to date no autoantigens are identified [2]. Initiation of immunosuppressive therapy is dependent on clinical criteria such as the degree and the evolution of kidney dysfunction and proteinuria. Treatment options are corticosteroids, alkylating agents, rituximab, calcineurin inhibitors, and mycophenolate mofetil (MMF) with variable remission rates [1]. In the literature, the role of intravenous gamma-globulins (IVIG) in the treatment of this disease has been explored and a potential role outlined by nonrandomized trials [3,4].