scholarly journals Rapidly progressing glomerulonephritis in children

Author(s):  
T. P. Makarova ◽  
N. V. Kazan ◽  
E. E. Soldatova ◽  
L. I. Akhmadullina

Rapidly progressing glomerulonephritis in children is a rare but the most aggressive form of glomerulonephritis. This disease is clinically characterized by acute nephritic syndrome with rapidly progressive renal failure, morphologically it is characterized by the presence of extracapillary cellular or fibro-cellular crescent in more than 50% of glomeruli. Currently the literature describes only a few cases of clinical course of glomerulonephritis in children. The article presents the researchers’ observation of rapidly progressive glomerulonephritis type I (anti-BMC+) in a 12-year-old girl. Despite immunosuppressive therapy combined with plasmapheresis, the authors observed the progress of the disease resulting in chronic kidney disease stage V. The child received renal replacement therapy with peritoneal dialysis. The course was complicated by the development of severe hyperparathyroidism.

Antibodies ◽  
2020 ◽  
Vol 9 (2) ◽  
pp. 18
Author(s):  
Sophia Lionaki ◽  
Smaragdi Marinaki ◽  
Konstantinos Panagiotellis ◽  
Ioanna Tsoumbou ◽  
George Liapis ◽  
...  

Aim: Glomerular diseases (GD) associated with malignancies (AM, GDAM) have unique features, which are important to recognize, in the light of the progress made in cancer therapy. We aimed to describe the clinical and histopathological characteristics of patients with GDAM in relation to the presence of circulating autoantibodies, pointing to potential immune pathogenic pathways connecting cancer to GD. Materials and Methods: The included patients were studied retrospectively on the basis of a kidney biopsy proving GD and a related biopsy to establish the diagnosis of AM. We recorded patients’ demographics, serological and laboratory parameters, histopathological findings, and the type of malignancy, GD, and therapy. Results: In total, 41 patients with GDAM, with a mean age of 63.1 (±10.7) years, were studied. In 28 (68.3%) cases, GD was associated with a solid tumor, and in 13 (31.7%) patients with a lymphoid malignancy. The most frequent histopathological pattern was membranous nephropathy (43.9%). Overall, at the time of GD diagnosis, 17% of the patients were positive for antinuclear antibodies (ANA), and 12.2% for antineutrophil cytoplasmic autoantibodies (ANCA), all against myeloperoxidase (MPO). In addition, 93.3% of the patients who had membranous nephropathy were negative for transmembrane glycoprotein M-type phospholipase A2 receptor (PLA2R) antibody. Sixteen patients (39.0%) presented with acute nephritic syndrome, of whom five (31.25%) developed rapidly progressive glomerulonephritis. In a mean follow-up time of 36.1 (±28.3) months, nine (21.95%) patients ended up with end-stage kidney disease, and eight (19.5%) died. Conclusion: We found that 3.2% of patients who underwent a native kidney biopsy in our institution during the past decade, for any reason, were identified as having some type of GD associated with a malignancy. Serology indicated a significant presence of ANA or MPO-ANCA antibodies in patients with nephritic syndrome and the absence of PLA2R antibodies in patients with membranous nephropathy.


Author(s):  
Thomas Dowsett ◽  
Louise Oni

AbstractAnti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane. Evidence of anti-GBM antibodies in serum or histologically are required for diagnosis. Treatment in children is based on very limited adult data and often involves the use of acute apheresis to rapidly remove circulating factors coupled with intensive immunosuppression such as cyclophosphamide and intravenous corticosteroids. There is also an emerging role for the use of biologic agents such as B cell depletion. The evidence base in children with anti-GBM disease is extremely limited. Multi-centre international collaboration is required to provide insight into this disease, better describe its prognosis and work towards improving outcomes. This review article summarises the key features of this disease in children, highlights treatment options and considers areas of unmet need.


2019 ◽  
pp. 2-3

Impaired phosphate excretion by the kidney leads to Hyperphosphatemia. It is an independent predictor of cardiovascular disease and mortality in patients with advanced chronic kidney disease (stage 4 and 5) particularly in case of dialysis. Phosphate retention develops early in chronic kidney disease (CKD) due to the reduction in the filtered phosphate load. Overt hyperphosphatemia develops when the estimated glomerular filtration rate (eGFR) falls below 25 to 40 mL/min/1.73 m2. Hyperphosphatemia is typically managed with oral phosphate binders in conjunction with dietary phosphate restriction. These drugs aim to decrease serum phosphate by binding ingested phosphorus in the gastrointestinal tract and its transformation to non-absorbable complexes [1].


Diabetes ◽  
2020 ◽  
Vol 69 (Supplement 1) ◽  
pp. 1049-P
Author(s):  
ELVIRA GOSMANOVA ◽  
DARREN E. GEMOETS ◽  
LAURENCE S. KAMINSKY ◽  
CSABA P. KOVESDY ◽  
AIDAR R. GOSMANOV

2020 ◽  
Vol 24 (1) ◽  
pp. 60-66
Author(s):  
I. V. Lavrishcheva ◽  
A. Sh. Rumyantsev ◽  
M. V. Zakharov ◽  
N. N. Kulaeva ◽  
V. M. Somova

BACKGROUND. The lack of data on the epidemiology of presarcopenia/sarcopenia leads to an underestimation of the role of this condition in the structure of morbidity and mortality of haemodialysis patients in theRussian Federation. THE AIM: to study the epidemiological aspects of presarcopenia /sarcopenia in patients with chronic kidney disease stage 5d. PATIENTS AND METHODS. This study comprised 317 patients receiving programmed bicarbonate haemodialysis for 8.2 ± 5.1 years, among them 171 women and 146 men, the average age was 57.1 ± 11.3 years. The assessment of the presence of sarcopenia was performed using the method recommended by the European Working Group on Sarcopenia in Older People. RESULTS. The prevalence of presarcopenia was 0.7 % and sarcopenia 29.6 %. The incidence of skeletal muscle mass deficiency according to muscle mass index (IMM) was 30.3 %, 48.7 % showed a decrease in muscle strength according to dynamometry, and low performance of skeletal muscles according to 6 minute walk test was determined in 42.8 %. Sarcopenia patients were significantly characterized by lower body mass index, as well as higher body fat mass values. The duration of haemodialysis (χ2 = 22.376, p = 0.0001) and the patient's age (χ2 = 10.545 p = 0.014) were an independent risk factors for the development of sarcopenia. CONCLUSION. Sarcopenia is recorded more frequently in hemodialysis patients than presarcopenia. Its prevalence increases among patients of older age groups and with a hemodialysis duration of more than 5 years. The age and experience of dialysis make their independent contribution to the development of this syndrome.


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