Background and Aims The disease diagnostics results and the efficacy of pathogenetic treatment when transferring from the original drug to the biosimilar were evaluated during clinical observation of an adult patient with aHUS. Method A 46-year-old patient has a history of hypertension since 2012 and maximum ABP of 230/130 mmHg. In 2016, newly diagnosed azotemia was identified (blood creatinine — 140 μmol/L, GFR by EPI — 54 mL/min/1.73m2). In 2018, the patient suffered an acute cerebrovascular accident in the vertebrobasilar area. On admission to Almazov National Medical Research Centre, blood test showed: creatinine – 260 μmol/L (GFR by EPI — 28 mL/min/1.73m2), urea – 11.52 mmol/L, LDH – 130 U/L, complement system C3 – 1.32 g/L, С4 – 0.37 g/L, hemoglobin – 144 g/L, platelets – 302*109/L; urinalysis showed: daily protein loss – 2.6 g/day, RBC – 0–1 per field. Taking into account the kidney damage, the renal fine-needle aspiration biopsy was performed, revealing histopathological findings typical of chronic thrombotic microangiopathy of extreme severity, arteriolar arteriosclerosis with total or subtotal luminal occlusion, extensive secondary perihilar segmental glomerulosclerosis (27%); and global glomerulosclerosis (55%). According to the study results, the secondary genesis of TMA was ruled out, as well as STEC-HUS and thrombotic thrombocytopenic purpura (TTP), systemic diseases (systemic lupus erythematosus, antiphospholipid syndrome (APS), scleroderma), malignancies, HIV infection, sepsis, malignant arterial hypertension, adverse drug events, or disseminated intravascular coagulation (DIC). Plasma ADAMTS-13 levels were also assessed. Its activity was 64% (normal range 93–113%) of ADAMTS-13 activity in the control plasma, thus the diagnosis of thrombocytopenic purpura was ruled out. Due to the absence of anti-CFH-antibodies the antibody origin of aHUS was also ruled out. To assess the contribution of additional factors promoting the development of TMA, the polymorphism of hemostasis genes was studied to reveal homozygous genotypes of platelet collagen receptors (ITGA2: 807 С/Е), heterozygous genotypes of fibrinogen genes (FGB: 455 G/А), folate cycle enzymes (methylenetetrahydrofolate reductase (MTHFR: 677 C/T), methionine synthase (MTR: 2756 A/G (D919G)), responsible for a pronounced tendency towards hyperhomocysteinemia. In the clinical case the diagnosis of aHUS was obvious, which was supported by the classical symptom cluster of TMA and histopathological verification in the absence of data suggestive of other pathological conditions. The peculiarity of this clinical case is the late diagnosis, long-standing course, and severity (complications such as an acute cerebrovascular accident in the vertebrobasilar area, target lesions, kidneys in particular). Results Taking into account the primary diagnosis, treatment with a standard dose of eculizumab was initiated. After three months of treatment the patient was transferred to therapy with Russian biosimilar of eculizumab. After the switch from the original drug, the therapy with eculizumab biosimilar continued to be associated with a positive trend including a gradual decline in azotemia level (blood creatinine – 115 μmol/L (GFR by EPI – 52 mL/min/1.73m2), blood urea – 7.10 mmol/L), proteinuria (daily protein loss – 0.6 g/day). The comprehensive treatment including antihypertensive therapy also led to the normalisation of ABP values. No adverse events associated with the therapy were noted. Conclusion The obtained results demonstrate high efficacy and safety of eculizumab biosimilar in the treatment of the adult patient with aHUS.
Background and Aims One of the complications of hemodialysis therapy (HD) is the development of protein-energy wasting (PEW), which is an independent predictor of morbidity and mortality in this cohort of patients. The prevalence of PEW in hemodialysis patients in different regions of the world varies from 15% to 75% but has not been practically studied in the Russian Federation. To assess the prevalence of protein-energy wasting in hemodialysis patients in the European region of the Russian Federation. Method 645 hemodialysis patients were examined in 9 hemodialysis centers in 5 regions of the European part of the Russian Federation. Among the patients there were 345 women and 300 men, the average age was 56.8 ± 12.8 years. The duration of hemodialysis therapy was 8.4 ± 5.3 years. The diagnosis of PEW was established in accordance with the criteria: biochemical criteria (serum albumin < 3.8 g per 100 ml; serum prealbumin (transthyretin) < 30 mg per 100 ml; serum cholesterol < 100 mg per 100 ml); low body weight, reduced total body fat, or weight loss; a decrease in muscle mass; and low protein or energy intakes. The level of blood albumin was determined using the BCG method (bromocresol green). Estimation of protein and calorie intake was performed using 3-day food diaries filled out by patients. Results Insufficient protein intake according to the International Society of Renal Nutrition and Metabolism (ISRNM) recommendations was observed in 5.5 % of patients, calories in 4.1 %, combined deficiency in protein and calorie intake in 2.7% of patients. The prevalence of PEW was 51.2 %. The relationship between age and gender was not identified. The lowest prevalence of PEW is observed in patients with a hemodialysis therapy experience of fewer than 5 years (25,4 %) and gradually increasing by 2.5 times in patients with survival rates on HD for more than 10 years (χ2 = 22,580 р = 0.0001). Conclusion The prevalence of protein-energy wasting in hemodialysis patients in the European region of the Russian Federation is 51,2 %.
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