Evaluation of the Possibility of Axial Psoriatic Arthritis Patients Meeting Classification Criteria for Axial Spondyloarthritis and Ankylosing Spondylitis.

The objective of the study was to analyze whether axial psoriatic arthritis (axPsA) patients meet classification criteria for axial spondyloarthritis (axSpA) and ankylosing spondylitis (AS). A total of 104 patients (66 men and 38 women) with PsA according to CASPAR criteria were examined, all patients had back pain. Patients were evaluated for presence of inflammatory back pain (IBP) by ASAS criteria. Back pain not meeting the ASAS criteria was taken to be chronic back pain (chrBP). Patients underwent hands, feet and pelvis, cervical spine and lumbar spine X-rays. Erosions, osteolysis, and juxta-articular new bone formation were evaluated. Definite radiographic sacroiliitis (d-rSI) was defined as bilateral grade ≥ 2 or unilateral grade ≥ 3. Nineteen patients without d-rSI underwent sacroiliac joints MRI. Ninety-three patients underwent HLA B27 examination. The number of patients who met the criteria for axSpA (ASAS) and the modified New York (mNY) criteria for AS was determined. IBP was identified in 67 (64.4%) patients; chrBP, in 37 (35.6%) patients; 31 (29.8%) patient were of older age (over 40) at the onset of IBP/chrBP; 57 (58.8%) patients had d-rSI; 6 (31.6%) patients had MRI-SI; syndesmophytes were detected in 57 (58.8%) cases. Among 40 patients without d-rSI, 19 (47.5%) had syndesmophytes. In 38/97 (39.2%) patients d-rSI was detected along with syndesmophytes, while 19/97 (19.6%) patients had isolated d-rSI without spondylitis, and 19/97 (19.6%) patients had isolated syndesmophytes without d-rSI. HLA B27 was present in 28 (30.1%) cases. 51 (55.4%) patients met criteria for axSpA. Forty-one (44.6%) patients did not meet criteria for axSpA; however, 27 (65.9%) of them had syndesmophytes. Forty-eight (48.5%) PsA patients met mNY criteria for AS. Among these patients, a set of specific features was revealed: 18 (37.5%) had no IBP, 18 (37.5%) were of older age (over 40) at the onset of IBP/chrBP, 34 (70.8%) had dactylitis, 38 (79.2%) had erosive polyarthritis, 23 (48.8%) had juxta-articular new bone formation, 14 (30.2%) had osteolysis, 23 (48.9%) had "chunky" non-marginal syndesmophytes, and 40 (82.6%) had nail psoriasis; 28 (66.6%) patients were HLA-B27 negative. Forty-five percent of axPsA patients do not meet criteria for axSpA. Characteristic features have been identified to differentiate axPsA from AS.

Growth Induction of Solid Ehrlich Ascitic Carcinoma in Mice after Proton Irradiation of Tumor Cells Ex Vivo.

This study presents data on the growth rate and frequency of induction of the solid form of Ehrlich's ascites carcinoma (EAC) in mice in the short and long term after inoculation of ascitic cells irradiated ex vivo with a proton beam in the dose range of 30-150 Gy. It was shown that the growth rate of solid tumors after inoculation of irradiated cells ex vivo coincided with the growth of tumors in the control group. The frequency of tumor induction in mice after inoculation of EAC cells irradiated at a dose of 30 Gy was 80%, 60 Gy-60%, 90 Gy-25%, and 120 Gy-10%; at irradiation at a dose of 150 Gy, no tumors appeared during the entire observation period. Thus, we determined the dose of proton radiation required to eliminate tumor cells and/or signaling factors that can lead to the induction of tumor growth of EAC in mice.

[Treatment of ventral hernias in patients with morbid obesity]. / Taktika lecheniya ventral'nykh gryzh u patsientov s morbidnym ozhireniem.

The review is devoted to the treatment of ventral hernias in patients with morbid obesity. This issue is important due to significant number of such patients and no unambiguous clinical recommendations. The advantages of simultaneous surgery (with bariatric intervention) are obvious, i.e. lower risk of postoperative hernia incarceration and no need for re-hospitalization with another intervention. High risk of bariatric population makes it necessary to minimize surgery time and surgical trauma. A staged approach with reducing body weight surgically or conservatively before hernia repair is often chosen. Hernia repair should be performed using laparoscopic or robotic techniques with obligatory use of mesh implants. Panniculectomy or abdominoplasty as the main surgery is a valid option. Currently, it is necessary to develop clear criteria for selecting patients with morbid obesity for staged and simultaneous treatment of ventral hernias.

[Cancer of terminal part of common bile duct in a patient with heterotaxy syndrome]. / Rak terminal'nogo otdela obshchego zhelchnogo protoka u patsienta s sindromom geterotaksii.

Case report is devoted to successful pancreatectomy for cancer of terminal part of common bile duct in a patient with heterotaxy syndrome. The main difficulties during dissection of pancreaticoduodenal complex arose due to anatomical disorientation and the lack of standard topographic and anatomical landmarks. Preoperative computed tomography with assessment of visceral vessel anatomy is essential in all patients with biliopancreaticoduodenal tumors. If heterotaxy syndrome is suspected, additional examination is required to detect other potential abnormalities and prepare for unusual situation.

[Diagnostic value of urinary biomarkers excretion in estimation of morphological lesions in patients with primary glomerulopathies].

AIM: To investigate the diagnostic value of biomarkers in assessing the severity of sclerotic and atrophic lesions in primary glomerulopathies. MATERIALS AND METHODS: One hundred patients were included in the study, according to the results of kidney biopsy in 9 (9%) cases minimal change disease was diagnosed, in 28 (28%) focal segmental glomerulosclerosis, in 26 (26%) membranous nephropathy and in 37 (37%) IgA nephropathy. The clinical course of nephropathy was evaluated, standard laboratory tests were performed, and urinary excretions of cystatin C, 1-microglobulin, 2-microglobulin and NGAL were measured. The degree of glomerulosclerosis was assessed quantitatively, tubulointerstitial sclerosis and tubular atrophy semiquantitatively. RESULTS: According to the results of linear correlations and ROC-analysis, urinary excretion of cystatin C and 1-microglobulin had diagnostic value for early degree of tubulointerstitial sclerosis (cut-off value 319.9 and 10.94 mg/day, respectively). Urinary excretion of 2-microglobulin reflected the initial degree of tubalar atrophy (cut-off value of 0.224 mcg/day), as well as tubulointerstitial sclerosis of various degrees of severity (cut-off value 0.224 and 0.240 mkg/day). NGAL urinary excretion was the only marker of early degree of glomerulosclerosis with its excretion of more than 1445.4 ng/day and tubular atrophy, with a severity of 50% or more (cut-off value 4897.8 ng/day). CONCLUSION: A comprehensive assessment of sclerotic and atrophic lesions in the renal parenchyma, can be performed using a panel of traditional (GFR, proteinuria) and specific biomarkers (1-, 2-microglobulins, cystatin C, NGAL) to implement a comprehensive, personalized approach, as well as to assess the prognosis of nephropathy. In addition, the evaluation of the panel of different biomarkers can be used in those clinical situations where kidney biopsy can not be performed.

[Retrospective clinical and morphological analysis of patients with AL amyloidosis (the 2008 to 2015 nephrobiopsies)]. / Retrospektivnyi kliniko-morfologicheskii analiz bol'nykh s AL-amiloidozom pochek (nefrobiopsii za period s 2008 po 2015 g.).

OBJECTIVE: To study correlations between the clinical signs of dysfunction and pathological structural changes in the renal parenchyma in a group of Russian patients with AL amyloidosis in 2008-2015. SUBJECTS AND METHODS: A total group (At) including Group 1 with AL (kappa + lambda light chains) (n=46) was divided into subgroups: 2λ) 40 patients with AL-lambda (AL-λ); 3κ) 6 patients with AL-kappa (AL-κ). All the patients underwent standard laboratory and instrumental studies: determinations of the peak systolic and diastolic blood pressures (SBP and DBP, respectively, mm Hg), glomerular filtration rate (GFR) (ml/min/1.73 m2) by the EPI equation, daily protein loss (g/day). Polyclonal antibodies against kappa and lambda light chains, AA component, and transthyretin (DAKO, Denmark) were used as immunomorphological markers. Light optical structural changes were semiquantitatively assessed, by ranking the following analyzed sign: interstitial focal sclerosis (FS), tubular atrophy (TA), interstitial inflammatory infiltration (II) semi-quantitatively (0 - no; 1 - < 25%; 2 - <50%, 3 - >50% of the volume of a histological compartment). Glomerulosclerosis (GS) was defined as the percentage of sclerotic glomeruli. The extent of amyloid depositions in the renal parenchyma structures was estimated according to the procedure proposed by Ying Yao et al., 2013. RESULTS: The AL group showed a female preponderance (65.21%). The patients' mean age was 62±11 years. There were no significant differences in daily proteinuria and the levels of serum creatinine, GFR, SBP, and DBP between the groups. The predominant clinical manifestation in the patients was nephrotic syndrome. A comparative analysis of the pathomorphological criteria for the spread of amyloid masses and the markers of fibroplastic processes revealed no statistically significant differences in the studied groups. Correlation analysis of the spread of AL deposits in the renal parenchyma in the patients of Group 1 and Subgroup 2λ, as well as laboratory data showed that there were significant (p<0.05) correlations with GFR, serum creatinine, unlike in Subgroup 3κ. At the same time, the analysis demonstrated that daily proteinuria had a significant positive correlation with VA, IA, GA, and TA values in Subgroup 3κ, unlike in Group 1 and Subgroup 2λ. Positive correlations were found between glomerulosclerosis and VA in Subgroup 2 λ and IA in Group 1. Sclerotic (FS and TA) changes in the tubular interstitium (TIN) were significantly positively correlated with all the indicators of AL (GA, VA, IA, TA) in the examinees in Group 1 and Subgroup 2λ, but not in Subgroup 3κ. Inflammatory TIN infiltration showed statistically significant (p<0.05) positive correlations with IA and VA in Group 1 and Subgroup 2λ and their absence in subgroup 3κ. CONCLUSION: : The retrospective analysis of nephrobiopsy specimens from of patients with AL amyloidosis revealed that kidney damage was mainly associated with the development of λ-associated AL amyloidosis. The clinical and laboratory parameters were correlated with the pathomorphological criteria for loading the renal parenchyma with amyloid masses. The findings suggest that there are clinical and morphological features of the subclasses of AL amyloidosis, which may be of value for predicting the course and progression of the disease.

[Monoclonal gammopathy of renal significance: consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment].

Monoclonal gammopathy of renal significance (MGRS) is a new nosology in modern nephrology and oncohematology. MGRS is defined as kidney injury due to nephrotoxic monoclonal immunoglobulin produced by the B-cell line clone which does not reach the hematological criteria for specific treatment initiation. Monoclonal proteins pathological effects on kidney parenchyma result in irreversible decline of kidney function till the end stage renal disease that in line with the position of International Consensus of hematologists and nephrologists determinates critical necessity for clone specific treatment in patients with MGRS despite the absence of hematological indications for treatment initiation. Main challenge of MGRS in Russian Federation is an inaccessibility of an in-time diagnostic and appropriate treatment for the great majority of patients due to the following reasons: 1) limited knowledge about the MGRS among hematologists and nephrologists; 2) lack of necessary diagnostic resources in most health-care facilities; 3) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. Consensus document comprises the opinion of experts leading nephrologists and hematologists of Russian Federation on the problem of MGRS including the incoherence in nosology classification, diagnostics approach and rationale for clone specific treatment. Consensus document is based on conclusions and agreements reached during the conference of leading nephrologists and hematologists of Russia which was held in the framework of symposia Plasma cell dyscrasias and lymphoproliferative diseases: modern approaches to therapy, 1516 of March 2019, Pavlov First Saint Petersburg State Medical University. The present Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses.

[Simultaneous surgery for complicated giant post-traumatic phrenic hernia in a patient with morbid obesity and diabetes mellitus type II]. / Opyt vypolneniya simul'tannoi operatsii po povodu oslozhnennoi gigantskoi posttravmaticheskoi diafragmal'noi gryzhi u patsienta s morbidnym ozhireniem i sakharnym diabetom II tipa.

Surgical treatment of post-traumatic right-sided phrenic hernia is associated with certain technical difficulties due to topographic and anatomical features. Morbid obesity combined with diabetes mellitus type II is one of the main factors complicating any surgical thoracic or abdominal surgery and further rehabilitation. We report simultaneous surgery for complicated post-traumatic right-sided phrenic hernia in a patient with morbid obesity and diabetes mellitus type II. Surgical correction of giant phrenic hernia facilitated further effective treatment of morbid obesity and concomitant diabetes mellitus type II.

[Quality of life as an efficiency index of surgical treatment of chronic pancreatitis]. / Kachestvo zhizni kak pokazatel' éffektivnosti khirurgicheskogo lecheniia khronicheskogo pankreatita.

OBJECTIVE: To evaluate quality of life in long-term postoperative period in patients with chronic pancreatitis. MATERIAL AND METHODS: There were 31 (21 males and 10 females) patients with complicated forms of chronic pancreatitis who underwent surgery in 2015-2017. Mean age was 49 (44; 53) years, body mass index - 22.4 (20.4; 24) kg/m2. HR-QOL was determined using the questionnaires SF-36, QLQ EORTS C30, VAS. Postoperative control was made no earlier than 6 months after surgery. Median follow-up 11.5 months. The data were analyzed using non-parametric methods. Quantitative data are shown as median with interquartile range. Differences between quantitative values were determined using the Mann-Whitney test. RESULTS: Physical status value (SF-36) before surgery was 30.5 (24.8; 37.5), after surgery - 50.8 (46.7; 56.5). Mental status value was 30.2 (26.7; 36.4) prior to surgery and 53.8 (48.7; 57.3) after operation. Improvement of QoL (QLQ C30) from 29.17 (0; 50.0) before surgery to 75.0 (54.2; 83.3) after operation was observed. VAS-score of pain severity was 8 (8; 10) prior to surgery and 3 (2; 5) after surgery. Differences were significant (p<0.05). CONCLUSION: Surgical treatment of complicated chronic pancreatitis significantly reduces pain and improves HR-QoL. However, recurrent symptoms of chronic pancreatitis in long-term period cannot be excluded due to short follow-up period (median less than one year). The further investigation is needed.

[The mistakes in diagnosis of pancreatic intraductal papillary mucinous tumor]. / Oshibki diagnostiki vnutriprotokovoi papilliarno-mutsinoznoi opukholi podzheludochnoi zhelezy.

It is presented surgical treatment of the patient with pancreatic intraductal papillary mucinous tumor who was under observation with diagnosis of chronic pancreatitis for a long time. Recurrent tumor with possible malignant transformation to carcinoma was suspected in 5 years after pancreaticoduodenectomy. It was supposed to perform pancreatic stump extirpation. Redo surgery did not reveal any tumor. The cause of recurrent pain was pancreatic duct obliteration at the level of anastomosis.

[Diagnosis and treatment of pancreatic head cancer followed by mesenteric-portal vein invasion]. / Diagnostika i lechenie raka golovki podzheludochnoi zhelezy, prorastaiushchego mezenteriko-portal'nyi segment ven.

AIM: To evaluate the outcomes of pancreaticoduodenectomy with mesenteric-portal vein resection for pancreatic head cancer. MATERIAL AND METHODS: Retrospective analysis included 124 patients with pancreatic head cancer for the period 2010-2017. Mesenteric-portal vein (MPV) invasion was diagnosed in 37 (29.8%) patients, tumor contact with superior mesenteric artery as a borderline resectable state was noted in 11 cases. All patients underwent pancreaticoduodenectomy with mesenteric-portal vein resection. RESULTS: Vein invasion was histologically confirmed in 19 (51.3%) out of 37 patients. At the same time, arterial invasion was absent in 11 patients with a borderline resectable tumor. CT-associated overdiagnosis of venous wall invasion was 6.4%, intraoperative overdiagnosis - 87.5%. R0-resection was achieved in 88.5% after conventional pancreaticoduodenectomy and in 78.4% after pancreaticoduodenectomy followed by MPV resection. Median survival was 17 months, 2-year survival - 41%. Among 11 patients with a borderline resectable tumor median survival was 11 months. Pancreaticoduodenectomy without vein resection was followed by 2-year survival near 68.1%. Differences were significant (p=0.02). CONCLUSION: Pancreaticoduodenectomy followed by MPV resection as the first stage of combined treatment of pancreatic head cancer is absolutely justified if circumferential involvement of the vein and contact with superior mesenteric artery or celiac trunk do not exceed 50%. Vein resection can provide R0-surgery in these cases.

[ONSTEP procedure for inguinal hernia repair]. / Allogernioplastika onstep v lechenii pakhovykh gryzh.

AIM: To evaluate early and long-term outcomes of ONSTEP procedure including their comparison with those of Liechtenstein hernia repair. MATERIAL AND METHODS: The study consists of 2 parts - retrospective and prospective. There were 472 patients in retrospective part. Prospective analysis included 30 patients after ONSTEP hernia repair and 30 patients after Liechtenstein procedure. RESULTS: Postoperative complications and chronic pain syndrome occurred in 2.9% and 0.4% of cases after ONSTEP surgery, respectively. Moreover, ONSTEP procedure was followed by 2 cases or recurrence. There were no any significant differences between ONSTEP and Liechtenstein procedures. CONCLUSION: ONSTEP technique is highly effective and safe procedure that can be used in inguinal hernia management.

[Current issues in pancreatic surgery]. / Aktual'nye voprosy khirurgii podzheludochnoi zhelezy.

AIM: To present own experience of pancreatic surgery and to analyze literature data for this issue. MATERIAL AND METHODS: We have analyzed work of abdominal surgery department over the last 5 years. Moreover, MEDLINE and RSCI databases regarding surgical treatment of pancreatic diseases were assessed. RESULTS: There were 456 pancreatectomies. Postoperative complications arose in 176 (38.6%) patients, 11 patients died (2.4%). According to world data, mortality after pancreatectomy reaches 10%. Only creation of specialized centers is proven way to improve the outcomes. CONCLUSION: Current medical assistance for pancreatic disease may be only achieved in specialized centers with large number of various pancreatic procedures. The organization of such centers is required throughout the country and certain accreditation criteria should be developed for this purpose. Targeted routing of patients to specialized pancreatology centers will be able to reduce incidence of diagnostic, tactical and technical errors.

[Wirsungorrhagia in cystic mucinous pancreasic tumor]. / Virsungorragiia pri kistoznoi mutsinoznoi opukholi podzheludochnoi zhelezy.

A rare case of virsungorrhagia in a 65 y/o patient with a mucinous tumor of the tail of the pancreas is presented. Recurrent gastrointestinal bleeding was associated with an arrosis of the splenic artery adjacent to the tumor, which required a two-step treatment - endovascular occlusion of the splenic artery and distal resection of the pancreas.

[Endovascular procedures in pancreatic surgery]. / Éndovaskuliarnye vmeshatel'stva v khirurgii podzheludochnoi zhelezy.

AIM: To evaluate an effectiveness of endovascular techniques in pancreatic surgery. MATERIAL AND METHODS: For the period 1995-2017 at Vishnevsky Institute of Surgery endovascular treatment (EVT) was applied in 51 patients with chronic pancreatitis complicated by false aneurysms (FA) and postoperative hemorrhage after pancreatectomy. Various methods of embolization and stenting were used in 24 and 11 cases respectively in order to exclude FA of celiac trunk and superior mesenteric artery from blood flow. Endovascular hemostasis for postoperative hemorrhage was carried out with embolization of damaged vessel in 11 patients. Stent-grafts were deployed in 5 patients with marginal defect of the wall of hepatic/superior mesenteric arteries. RESULTS: In all 35 patients with chronic pancreatitis complicated by false aneurysms EVT ensured thrombosis of the aneurysm's cavity. EVT was final in 14 patients with FA and absent communication with pancreatic duct. Radical surgical treatment was required after 7-10 days for FA communicated with pancreatic duct due to lysis of thrombotic masses by pancreatic enzymes. Spleen infarction was diagnosed in 3 patients. Two of them did not require treatment while 1 patient underwent splenectomy in view of splenic abscess. 1 patient died from liver cirrhosis followed by severe hepatic failure, death was not associated with bleeding. In 16 patients with post-pancreatectomy bleeding hemostasis was achieved in all cases with EVT. However, recurrent bleeding occurred in 2 patients who underwent successful redo endovascular intervention. Complications after EVT were observed in 2 patients: duodenal wall necrosis followed by fistula which was closed spontaneously (n=1); advanced intestinal infarction (n=1) followed by fatal outcome; pulsating hematoma within cubital fossa that required brachial artery ligation and autovenous bypass. CONCLUSION: EVT provides thrombosis of FAs of celiac trunk and superior mesenteric artery branches in patients with chronic pancreatitis, as well as hemostasis for postoperative bleeding after pancreatectomy.

[Pulmonary larval paragonimiasis mimicking lung cancer]. / Legochnyi larval'nyi paragonimoz, imitiruiushchii rak legkogo.

Larval paragonimiasis is a parasitic disease caused by lung fluke larvae. Unlike the classic form of paragonimiasis, the larval form occurs with a large number of clinical manifestations. However, this fact only complicates the diagnosis of larval paragonimiasis, for the abundance of clinical manifestations results in the misdiagnosis of other diseases. Another feature of this form of paragonimiasis is the tendency to generalize and mimic the clinical presentation of malignant neoplasms. The performed diagnostic measures failed to give an accurate view of the nature of the disease, therefore diagnostic thoracoscopy was carried out and biopsy specimens were taken from the subpleural region of dissemination, followed by urgent histologic examination suggestive of glandular cancer. This circumstance became the reason for atypical resection of the affected portion, which was done; however, the patient died from pulmonary thromboembolism on postoperative day 2. The main diagnosis of chronic pulmonary generalized paragonimiasis (cystic and pneumosclerotic phases) with neoplastic syndrome was posthumously made.

[Is medication possible for acute appendicitis?] / Vozmozhna li konservativnaia terapiia ostrogo appenditsita?

AIM: To study the possibility of antibacterial therapy for acute appendicitis as an alternative to surgical treatment. MATERIAL AND METHODS: For the period 1985-2010 diagnostic laparoscopy was performed in 5548 patients with suspected acute appendicitis. Acute phlegmonous or gangrenous appendicitis was observed in 2275 (41.0%) patients who underwent laparoscopic appendectomy. Other acute diseases occurred in 2458 (44.3%) patients. Acute catarrhal appendicitis or secondary inflammation of the appendix were diagnosed in 815 (14.7%) patients; they did not get appendectomy. In-hospital antibacterial therapy has been administered for 1-2 days followed by discharge for outpatient treatment. One patient with acute destructive appendicitis who refused surgery was treated with antibiotics. RESULTS: Antibacterial therapy in 815 patients with acute catarrhal appendicitis allowed to avoid surgical treatment. Complications (appendicular infiltration, abscess), repeated hospitalizations within 1 month were absent. Antibacterial treatment provided recovery in 1 patient with destructive appendicitis who refused surgical treatment. CONCLUSION: Patients with acute catarrhal appendicitis diagnosed with laparoscopy do not need for appendectomy and can be cured by antibacterial therapy.

Membranoproliferative glomerulonephritis in Russian population.

AIM: Analysis of etiology, clinical and morphological manifestations, approaches to therapy and prognosis of membranoproliferative glomerulonephritis (MPGN). MATERIALS AND METHODS: Cases of MPGN were retrospectively identified in the period 2000-2017 with subsequent analysis of etiology, clinical data and morphology (including deposits of immunoglobulins (Ig) and C3 complement fractions). The achievement of complete and partial remissions (PR, CR), overall survival, progression (by composite endpoint: decrease in the estimated GFR (eGFR) ≥50% from the baseline or eGFR <15 ml/min/1.73 m2 or the onset of dialysis). RESULTS: 214 cases of MPGN entered the study with the average age of 44±16 years. Most patients had nephrotic syndrome and significant hematuria. In 58.4% of cases, eGFR was <60 mL/min/1.73 m2, and every fifth patient had CKD 4 or 5 stages. The prevalence of MPGN among all biopsy-confirmed glomerulopathies was 9.3%. Idiopathic MPGN (iMPGN) was detected in 30.4% of cases, secondary MSGN (sMPGN) - in 69.6% (autoimmune diseases - 34.1%, infectious diseases - 16.4%, monoclonal gammopathies - 9.3%, complement-mediated damage - 9.8%). Ig+C3+MPGN was mainly associated with autoimmune diseases and infections; C3-glomerulopathy or thrombotic microangiopathy were most often causes of Ig-C3+MPGN; Ig-C3-/Ig+C3-MPGN had heterogeneous etiology. The median follow-up period was 28 [7; 37] months. The 10-year total cumulative patient and renal survival rates were 71 and 50%, respectively (without differences between sMPGN and iMPGN). The frequency of the PR/CR was 50% (iMPGN - 46.2%, sMPGN - 51.3%) depending on the etiology of the MPGN (p=0.049). The cumulative 10-year progression-free renal survival was nearly 100% in cases with PR/CR and 0% in non-responders. CONCLUSION: MPGN is a severe variant of glomerular damage with a heterogeneous etiological structure and an unfavorable prognosis. Targeted clinical and morphological diagnostics of MPGN allows to identify the cause of the disease in most cases. This approach is reliable for the adequate treatment choice and improvement of outcomes in MPGN.

Association of polymorphisms of HLA-DRB1 and TNF-308 G/A with radiographic joint damage in patients with early rheumatoid arthritis with high inflammatory activity, treated according to the principle of "Treat to target" (REMARKA study).

AIM: To clarify the association between HLA-DRB1 and TNFα (-308G>A) genes polymorphism and joint destruction/further progression during 12 months of the follow-up period (FUP) in patients with early (<6 months), active, predominantly antibodies to cyclic citrullinated peptide (ACCP) and rheumatoid factor (RF)-positive rheumatoid arthritis (RA) treated according to "Treat to target" strategy. MATERIALS AND METHODS: The study included 85 patients with early RA and duration of symptoms <6 months. All patients were initially assigned to subcutaneous methotrexate (MTX) with rapid dose escalation to 20-25 mg/week. Combination MTX + biological therapy, mainly adalimumab, was used when MTX was ineffective. Joint destruction was assessed by Sharp-Van der Heijde modification scoring method at baseline and after 12 months FUP. Real time polymerase chain reaction (PCR-RT) was used for TNFα gene polymorphism (-308G>A) genotyping. Low resolution PCR-RT with subsequent sequence-based typing of *04 were performed to study HLA-DRB1 gene polymorphism. The HLA-DRB1*01, *04:01, *04:04, *04:05, *04:08, *10 alleles were categorized as SE+ (Shared Epitope) alleles. RESULTS: As for TNFα gene polymorphism, it was demonstrated that the number of narrowings and total Sharp score values were almost twice as high at baseline in GG genotype carriers as compared to GA genotype carriers (р<0,005, and р<0,004 respectively). Similar association was found after 12mo FUP. The progression of joint destruction, assessed as the change (∆) in the number of erosions, joint space narrowings and the total score, was statistically significantly associated with HLA-DRB1*(SE) genotypes: the carriers of SE (SE+/SE+) double-dose had more advanced progression as compared to (SE+/SE-)/(SE-/SE-) carriers (р<0,028, р<0,019, р<0,035 respectively). CONCLUSION: Our data suggest that HLA-DRB1 (SE+) gene and TNFα (-308G>A) polymorphisms are associated with the progression of radiographic joint destruction in early, active RA patients managed according to "Treat to target" stratagy.

[Nephrological aspects of complement-mediated thrombotic microangiopathy]. / Nefrologicheskie aspekty komplement-oposredovannoi tromboticheskoi mikroangiopatii.

AIM: To analyze cases of complement-mediated thrombotic microangiopathy (C-TMA) in the daily practice of a hospital nephrology department, relative to the general aspects of the clinical course, therapy, and short-term outcomes. SUBJECTS AND METHODS: The prospective study conducted in the period June 1, 2015 to June 1, 2016 included 10 patients with C-TMA newly diagnosed during hospitalization. Standard demographic parameters, generally accepted clinical data showing the severity of TMA and the involvement of vital organs were recorded at admission. Short-term outcomes of therapy (plasma therapy and/or eculizumab therapy), such as achievement of hematological remission and the need for maintenance therapy and renal replacement therapy (RRT), were evaluate. RESULTS: The median time from the onset of symptoms of the disease to diagnosis in the analyzed cases was 5 months (range 1 week to 26 months). The incidence of C-TMA among the inpatients of nephrology departments was 6.4 per 1,000 patients per year. Five patients were admitted to a clinic with symptoms of obvious extrarenal organ disorders; 4 of them had multiple organ dysfunction. Evident clinical symptoms of renal dysfunction were detected in all the patients, which necessitated RRT in 8 out of the 10 patients. Renal biopsy was performed in 7 patients. All were found to have typical morphological manifestations of acute and chronic TMA. All the patients received plasma therapy; most of them had plasma exchanges; 4 patients took eculizumab. There were no fatal outcomes. All achieved hematological remission. Three of the 8 cases could achieve varying recovery of kidney function and stop dialysis. CONCLUSION: Analysis of this series of cases confirms the idea about the severity of TMA due to multiple organ dysfunction and the high risk of end-stage renal failure at late diagnosis. The key points of better renal prognosis are the earliest detection and treatment of the disease.