Background: Recent studies have established a link between the development of preeclampsia (PE) and excessive activation or dysregulation of complement system. Investigating this system’s role may aid in the developing therapeutic strategies for treating PE.
Aims: To evaluate the levels of membrane attack complex (MAC) in blood serum during PE development in patients with chronic kidney disease (CKD) and the general population.
Materials and methods: The prospective observational study included 44 patients with PE: 17 women with a prior diagnosis of CKD and 27 women from the general population without a complicating somatic history. Soluble MAC levels was measured using an enzyme-linked immunoassay (HycultBiotech, Human Terminal Complement Complex Elisa Kit, HK32801).
Results: The incidence of mild and severe PE was similar between the groups. Among CKD patients, 29% (n=5) had mild PE and 71% (n=12) had severe PE, compared to 40% (n=11) and 60% (n=16), respectively. MAC levels were comparable between CKD and general population groups for mild PE (3933 [24676537] μU/ml vs 3202 [28134279] μU/ml [p=0.692]) and severe PE (4139 [38744458] μU/ml vs 4805 [32809594] μU/ml [p=0.378]), respectively. MAC levels did not differ significantly between mild and severe PE. In the general population, severe PE showed a trend toward higher MAC levels (4805 [32809594] μU/mL vs 3202 [28134279] μU/mL [p=0.054]). Severe PE was complicated by trombotiс microangiopathy (TMA) in 6 of 27 patients compared to 1 patient in the CKD group. Among patients with severe PE and TMA (7/44), MAC levels were significantly higher than in those without TMA (5345 [348713169] μU/ml vs 3933 [30574747] μU/ml [p=0.048]).
Conclusions: in PE patients, regardless of the development stage, severity or the presence CKD, MAC levels are elevated indicating hyperactivation of the complement system in this pregnancy complication. The highest MAC levels were observed in patients with TMA manifestations.

Aim. The study aimed to explore the relationship between disturbances in the ADAMTS13/von Willebrand factor (vWF)/platelet system and the risk of mortality in COVID-19 patients (n=90) with impaired renal function and thrombocytopenia. The retrospective analysis included patients treated between May 2020 and August 2022 at the nephrology department of the 52nd city Hospital in Moscow. Blood counts were assessed upon hospital admission.
Results. Median ADAMTS13 activity in discharged patients (n=62) was 91.0% of normal (95% CI 75.0‑105%), and in deceased patients (n=26) it was significantly reduced to 74.0% (95% CI 42‑84%; p<0.001). Conversely, vWF antigen level and its activity were significantly elevated in all patients but showed no difference between survivors and non-survivors. Platelet counts were 114×10³/μl (95% CI 103 to 128×10³/μl) in survivors and 43.0×10³/μl (95% CI 25.7 to 59.9×103/μl) in deceased patients (p<0.001). Platelets counts correlated positively with ADAMTS13 activity (Spearman coefficient rho = 0.514; p<0.001) were independ of vWF level or activity. Among patients undergoing hemodialysis (HD), lower ADAMTS13 activity and platelet counts were significantly associated with mortality. ROC curve analysis revealed that ADAMTS13 ≤79% and ≤53% increased the odds ratio (OR) for mortality in HD patients (n=58) to 8.53 (95% CI 2.12‑34.3; p=0.0025) and 27.6 (95% CI 3.11‑245; p=0.0029) respectively. A platelet count ≤75×10³/μl rised the OR to 10.9 (95% CI 3.00‑39.2; p=0.0003). In HD patients with acute kidney injury superimposed on chronic kidney disease (n=10), ADAMTS13 activity ≤79% increased the OR for mortalirty to 117 (95% CI 1.9‑7960; p=0.023). In contrast, no significant association between ADAMTS13 activity and mortality was observed in patients not undergoing HD or kidney transplant recipients. Survival probability in COVID-19 patients on HD with ADAMTS13 activity ≤53% was 3.22±0.92 times lower (p<0.01) after 35 days of hospital admission compared to patients with ADAMTS13 activity >53%.
Conclusion. These findings suggest that reduced ADAMTS13 activity and platelet counts are strong predictors of poor outcomes in COVID-19 patients undergoing hemodialysis.

Background: The quality of life (QoL) and the psychological burden of immunoglobulin A nephropathy (IgAN) have not been thoroughly studied.
Aim: To analyze QoL and complaints of patients with IgAN and to test the hypothesis that QoL scores are associated with clinical and demographic variables.
Patients and methods: The study included 221 patients with primary IgAN, confirmed by biopsy. None of patients had received immunosuppressive treatment. QOL was assessed using the Russian version of the Kidney Disease Quality of Life Short Form (KDQOL-SF) questionnaire, which includes general scales for measuring QoL independent of disease and kidney-specific scales. In additions to QoL parameters, the prevalence of patient complaints was examined. Routine clinical and demographic data were used as independent variables in multiple regression models.
Results: General QoL scores in patients with IgAN were comparable to those of conditionally healthy individuals. On CKD-specific scales, the highest scores were reported for "work status" and "symptoms/problems", while the lowest were for "burden of kidney disease". Key stressors included the disease itself, dietary restrictions, fatigue and impact of the disease on appearance. Female sex, older age, lower albumin level, and higher average blood pressure were independently associated with reduced QoL.
Conclusion: Although IgAN patients scored relatively high on most QoL scales, they endure a a significant psychological and social burden. Identifying and addressing the psychological and social factors influencing life under the strain of the disease can be crucial for for improving patient-centered outcomes in IgAN management.

C3 glomerulopathy (С3G) is a group of ultra-rare diseases with the incidence about 13 cases per 1 million population per year. Major role in the C3G pathogenesis play disturbances of the complement activation, deposition and degradation, resulting in the glomerular deposition of C3, which, in turn, leads to glomerular damage and inflammation in the kidney tissue. C3G commonly associated with the progressive course, poor kidney outcomes and high rate of recurrence after kidney transplantation. Efficacy of the current conventional approaches to C3G treatment, including nephroprotective measures and glucocorticoids and mycophenolic acid analogues is insufficient; the usage of targeted anti-B-cell therapy with rituximab also did not provide sustainable effect. Unsatisfactory results of the current clinical practice and a rapid progress in the development of new targeted medications recently lead to the active investigation of a number of molecules, targeting several factors of the complement cascade, which may enrich therapeutic armamentarium for the treatment of C3G and other glomerular diseases, associated with the complement dysregulation. Several studies, aiming the evaluation of blockade of various complement system components – C5, C5a receptor, factor D, factor B, C3, and mannose-binding lectin-associated serine proteases type 1 and type 2 for С3G treatment are currently in progress. This review of literature presents available data from the current clinical trials and discusses new options of the targeted treatment of C3G.

Congenital generalized lipodystrophy is a rare metabolic disease with autosomal recessive inheritance. It is characterized by a reduction of subcutaneous adipose tissue, ectopic lipid deposition, and the development of a number of metabolic disorders including insulin resistance diabetes mellitus, fatty liver degeneration and hepatitis, and arterial hypertension. One of clinical manifestation of the disease is kidney damage often leading to proteinuria of varying severity. However, the pathophysiological mechanisms underlying kidney damage in this condition are not yet fully understood. The aim of the study was to perform a clinical and morphological analysis of renal complications in a 32-year-old woman with Berardinelli-Seip syndrome and a rare heterozygous mutation in the AGPAT2 gene, a nucleotide substitution C.636C>T (L212L). This case report incorporated data from patient’s the medical history, intravital laboratory and instrumental studies, and both qualitative and quantitative morphological analysis of a nephrobiopsy sample. Clinical signs and morphological finding at light-optical and electron microscopic levels are presented, highlighting the reorganization of glomeruli tissue elements. The main clinical manifestations of kidney damage in this patient included arterial hypertension, progressive facial edema, swelling of the lower extremities and lumbar region, advanced nephrotic syndrome, and impaired nitrogen-excretory kidney function. Morphological examination did not reveal histological or ultrastructural signs of diabetic microangiopathy or nephropathy. Instead, findings included large glomeruli with a lobular configuration of the glomerular tuft, moderate mesangial expansion, segmental thickening of the capillary loop, and duplication of the glomerular basal membrane. There was deposition of immune complexes, primarily of the C3c complement fragment, along the capillary wall and in the paramesangial and mesangial regions, forming hyperdense osmiophilic ribbon-like and garland structures. These findings led to the classification of renal damage as C3 glomerulopathy. The study suggests that various mutations in the AGPAT2 gene may influence renal complications in Berardinelli-Seip syndrome, potentially contributing to a broader clinical spectrum, including the possibility of developing C3 glomerulopathy.

Introduction: Neonatal aortic thrombosis is a rare but serious condition with potentially severe outcomes. Approximately 80% of cases are linked to umbilical catheterization, while not non-catheter-related cases are classified as spontaneous. Clinical presentation varies with the location and severity of thrombosis, ranging from asymptomatic cases to life-threatening emergency such as cardiac or respiratory failure, necrotizing enterocolitis, limb gangrene, or acute kidney injury (AKI) renal artery involvement.
Materials and methods: This report describes a case of spontaneous abdominal aortic thrombosis in a newborn girl, resulting in AKI necessitating renal replacement therapy.
Results: A full-term newborn breastfed girl experiences clinical deterioration on day 11, presenting with lethargy, feeding refusal, macrohematuria, anuria on day 12. Ultrasound Doppler revealed bilateral renal artery thrombosis, with laboratory findings of hyperazotemia, hypernatremia, hemoconcentration, and 18% weight loss. Despite fluid replacement, electrolyte correction, anuria persisted, requiring peritoneal dialysis. CT angiography confirmed abdominal aortic thrombosis extending from the superior mesenteric artery to the common femoral arteries. Treatment included unfractionated heparin and tissue plasminogen activator (Alteplase), but kidney function did not recover, leaving the child dialysis-dependent.
Conclusions: This case highlights the rare occurrence of abdominal aortic thrombosis in the neonatal period, leading to AKI and chronic kidney disease (CKD) 5D due to cortical necrosis. Neonates are particularly vulnerable to thrombotic complications due to factors such a dehydration, hypernatremia, and polycythemia.