Allogeneic Hematopoietic Stem Cell Transplantation Activity in Inborn Errors of Immunity in Russian Federation.

Purpose: Allogeneic hematopoietic stem cell transplantation (HSCT) is an established therapy for many inborn errors of immunity (IEI). The indications for HSCT have expanded over the last decade. The study aimed to collect and analyze the data on HSCT activity in IEI in Russia.

[Questionnaire of the Ryzhikh National Medical Research Centre for Coloproctology is a new tool for assessing chronic pelvic pain and pelvic organ dysfunction].

The article presents the work of a multidisciplinary team of experts from various fields of medicine to optimize the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology» for use in clinical practice. The survey of respondents was conducted from June 28 to September 28, 2021. As a result of this survey, by repeatedly making edits and clarifications during communication with respondents, the final version was obtained, which allows assessing the patient's subjective sensations by the nature and localization of pelvic pain, sensitivity disorders and pelvic organ function.

[Recommendations for the Protocol of functional examination of the anorectal zone and disorders classification: the International Anorectal Physiology Working Group consensus and Russian real-world practice].

This manuscript summarizes consensus reached by the International Anorectal Physiology Working Group (IAPWG) for the performance, terminology used, and interpretation of anorectal function testing including anorectal manometry (focused on high-resolution manometry), the rectal sensory test, and the balloon expulsion test. Based on these measurements, a classification system for disorders of anorectal function is proposed. Aim to provide information about methods of diagnosis and new classification of functional anorectal disorders to a wide range of specialists general practitioners, therapists, gastroenterologists, coloproctologists all who face the manifestations of these diseases in everyday practice and determine the diagnostic and therapeutic algorithm.

[Rehabilitation program for patients with low anterior resection syndrome].

Unlabelled: Surgical treatment of rectal cancer and sphincter-preserving low anterior resection results to evacuation disorders («low anterior resection syndrome» - LARS). There are no clinical recommendations for the treatment of patients with LARS as well as a rehabilitation program for them.

Objective: To develop a rehabilitation program for patients with low anterior resection syndrome.

Clinical significance of cytogenetic changes in childhood T-cell acute lymphoblastic leukemia: results of the multicenter group Moscow-Berlin (MB).

The prognostic significance of genetic lesions in T-cell ALL still needs to be elucidated. Karyotyping and FISH were performed in samples from 120 patients with T-cell ALL registered in the trial Moscow-Berlin 2008. Most frequent rearrangements were TLX3 (N = 29; 24%) and TAL1 (N = 18; 15%), followed by KMT2A (N = 6; 5%), TLX1 (N = 5; 4.

A Conditioning Regimen with Plerixafor Is Safe and Improves the Outcome of TCRαβ and CD19 Cell-Depleted Stem Cell Transplantation in Patients with Wiskott-Aldrich Syndrome.

Our initial experience with hematopoietic stem cell transplantation (HSCT) from a matched unrelated donor (MUD; n = 12) or a haploidentical related donor (n = 6) with T cell receptor (TCR)αβ/CD19 graft depletion in patients with Wiskott-Aldrich syndrome (WAS) (n = 18) showed a dramatic decrease in the incidence of graft-versus-host disease (GVHD) and transplantation-related mortality, with an increased overall survival (OS) of 88.9%. Unfortunately, the treatment was associated with mixed myeloid donor chimerism and secondary graft dysfunction (severe thrombocytopenia, n = 2; graft rejection, n = 5).

Efficacy and safety of pathogen-reduced platelet concentrates in children with cancer: a retrospective cohort study.

Background: The safety of blood component transfusions is still of concern and the use of pathogen reduction (PR) methods is increasing. Limited data are available describing safety and efficacy of PR platelet (PLT) transfusions in children. We report the results of a retrospective evaluation of prophylactic transfusions of PR PLT units treated with riboflavin and ultraviolet light in pediatric patients with malignant disorders.

Diffuse scattering in metallic tin polymorphs.

The lattice dynamics of the metallic tin β and γ polymorphs has been studied by a combination of diffuse scattering, inelastic x-ray scattering and density functional perturbation theory. The non-symmorphic space group of the β-tin structure results in unusual asymmetry of thermal diffuse scattering. Strong resemblance of the diffuse scattering intensity distribution in β and γ-tin were observed, reflecting the structural relationship between the two phases and revealing the qualitative similarity of the underlying electronic potential.

[Annexin-5 as a biochemical marker of early vascular disorders under chronic disease of kidneys].

The study evaluated the significance of annexin-5 as a biochemical marker of atherosclerosis in patients with chronic disease of kidneys. The sample consisted of 46 patients with chronic disease of kidneys without clinical symptoms of atherosclerosis. The indicators of lipidogram, intima-media complex of carotid artery reactivity of vessels of microcirculatory channel and concentration of annexin-5 are determined.

[Effect of nitrogen source on gene expression of first steps of methanol utilization pathway in Pichia pastoris].

Pichia pastoris is a methylotrophic yeast that is widely used for the expression of heterologous proteins. Here, we have investigated the dependence of AOX1 and CAT1 expression on the source of nitrogen. It has shown that the expression of AOX1 and CAT1 depends on the source of nitrogen.

[Application of alpha1-adrenoblockers in treatment of ureteral calculi].

The results of evaluation of the efficacy of alpha1-adrenoblockers in treatment of ureteral calculi are presented. Comparative, prospective, placebo-uncontrolled nonrandomized single-center study was performed, which included 118 patients with solitary diagnosed calculi in various parts of ureter. After the pain relief, all the patients underwent conservative therapy aimed at spontaneous discharge of concretions.

[Age features of treatment of idiopathic membranoproliferative glomerulonephritis].

The aim of the study was to investigate the effectiveness of long application of the combined therapy by cytostatics and steroid hormones for patients with morphologically proved membranoproliferative glomerulonephritis depending on age. 51 patients younger 60 years and 27 patients older 60 years were investigated. Patients received therapy by steroid hormones and cytostatics within one year.

[Combined therapy of membranoproliferative glomerulonephritis in older patients].

The aim of the study was to investigate the effectiveness of combined long-term therapy by cytostatics and steroid hormones in older patients with membranoproliferative glomerulonephritis to halt renal failure progression. 27 patients older than 60 years with morphologically proved membranoproliferative glomerulonephritis have been treated. Nephrosclerosis was detected in 40% of studied patients according to results of kidney biopsies.

[Endothelial dysfunction and apoptosis at early stage of chronic kidney disease].

Aim: To study changes in apoptosis and endothelial function in patients with chronic kidney disease (CKD) stage I-IIIb (CKDsI-IIIb).

Material And Methods: A complex of biochemical, enzyme immunoassay and device investigations was used to examine 128 patients with CKDsI-IIIb.

Results: In CKD stage I reduction of endothelium-dependent vasodilation (EDV) was detected in 34% patients, in stage II -- in 52 %, in stage IIIa -- y 52 %, in stage IIIb - in 70%.

[Transcutaneous nephrolithotripsy in presenile and senile patients].

A total of 323 transcutaneous roentgenoendoscopic operations were made in 212 patients from January 2000 to December 2009 including 96 operations in 81 presenile and senile patients (87 and 9 operations, respectively). Transcutaneous nephrolithotripsy (TCNT) eliminated concrement from the kidney for one session in 59 (72.8%) patients.

[Transurethral contact electroimpulse ureteropyelolithotripsy].

Transurethral contact electroimpulse ureteropyelolithotripsy (TCEUT) was conducted in 161 patients aged 18 to 84 years (mean age 47 +/- 14 years) admitted to the urological department of Zhukovsky city hospital in 2006-2009. Female/male proportion 1.4:1.

[Choice of urinary tract drainage in different age groups of patients with urolithiasis].

Retro- and prospective analyses of 802 case histories of patients with nephrostomic drainage (n=272), ureteral catheter (n=27) and ureteral stent (n=503) treated for urolithiasis in the urological department of M.F. Vladimirsky Moscow Region Research Clinical Institute and Zhukovsky city hospital hospitalized in 1995 to 2009 made it possible to develop algorithms of choice of upper urinary tract drainage depending on clinical and laboratory indices.

[The level of endothelin-1 and reactivity of skin microvessels in patients with early stages of chronic kidney disease].

Aim: To evaluate endothelin-1 (ET-1) levels and to characterize reflex vasomotor reactions of skin vessels in distant exposure to cold in patients with chronic kidney disease (CKD) of stage I and II.

Material And Methods: Glomerular filtration rate (GFR) was calculated by MDRD formula for 40 healthy subjects (mean age 39.2 +/- 2.

[Diagnosis and treatment of urolithiasis in combination with infravesical obstruction in presenile and senile patients].

The analysis of the results of examination and treatment of 510 urolithiasis patients with organic infravesical obstruction (IVO) treated in the urological clinic of M. F. Vladimirsky Moscow Regional Research Clinical Institute and in the urological department of Zhukovsky city hospital from 1995 to 2009 made it possible to arrive at the following conclusions: the above patients have obstruction symptoms in 100% cases, irritative symptoms--in more than 2/3 of the patients; combined use of low invasive, endoscopic and open methods reestablishes urodynamics of the lower urinary tract in more than 90% patients; the decision on the treatment policy and techniques depends on severity of clinical symptoms caused by IVO and urolithiasis as well as disturbance of upper and lower urinary tract urodynamics.

[Recurrences of acute promyelocytic leukemia in children: experience with arsenic trioxide therapy and autologous hematopoietic cell transplantation].

Aim: To analyze the specific features of recurrences of acute promyelocytic leukemia (APL) in children after standard therapy with daunorubicin, cytosine arabinoside (Ara-C), all-trans retinoic acid (ATRA) and to develop further programmed treatment policy.

Subjects And Methods: The study included 9 patients with recurrent APL. The recurrences developed significantly more frequently in a very high-risk group (patients with minimal residual disease being preserved after the intensive therapy phase).

[Efficiency of the ALL-MB-2002 protocol in children with acute lymphoblastic leukemia].

Aim: To evaluate the efficiency of the original ALL-MB-2002 protocol within the multicenter study of treatment of acute lymphoblastic leukemia (ALL) in children.

Subjects And Methods: A total of 1873 primary patients with ALL aged 1 to 18 years, of whom 1544 patients were enrolled in this study, were notified at 36 clinics of Russia and Belarus from April 15, 2002, to January 1, 2008.

Results: With the median observation of 4.

[On the issue of methods of assessment of quality of life conditioned by health].

The results of the study of 444 schoolchildren from 8-10 degrees and aged from 13 to 16 years studying in the general education schools of Moscow and Vologda are discussed. It is proved that the questionnaire TACQOL can be applied among adolescents for quantitative evaluation of their quality of life conditioned by health and to get reliable, replicable and comparative results. The range of variation of indicators is established for all scales in norm and it permits to reveal children with lower quality of life on the background of medical social problems.

[Assessment of rate of infection with agents of bacterial infections in ticks captured on one of the Moscow park terrains].

Aim: To study the rate of infection of ticks captured one of the Moscow park terrains with bacteria (agents of tick borreliosis and anaplasmosis).

Materials And Methods: Rates of infection of dried ticks with agents of main tick-borne bacterial infections (tick borreliosis and anaplasmosis) were determined by nested PCR.

Results: In May-June 2006, 76 ticks (40 adult females, 36 adult, males) belonged to Ixodes ricinus species were captured by the method "on flag".