Pediatria. Journal named after G.N. Speransky最新文献

{"title":"Respiratory support in children with severe bronchopulmonary dysplasia. A bibliographical review","authors":"S. Mezhinsky, A. V. Mostovoy, A. L. Karpova, V. V. Gorev, D. Ovsyannikov, A. Zaplatnikov","doi":"10.24110/0031-403x-2024-103-1-146-152","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-146-152","url":null,"abstract":"Bronchopulmonary dysplasia (BPD) is a chronic lung disease of multifactorial etiology characterized by the formation of respiratory failure and regressing as the child grows. With a severe form of BPD, adequate and safe respiratory support is required for a long time. The mechanical properties of the lungs in these patients differ significantly from those in acute respiratory pathology, which should be taken into account when setting the parameters of the ventilator. Important task is to determine the optimal moment for the transition to a strategy of respiratory support for damaged lungs, which is characterized by slow dynamics and specific parameter settings. In the absence of recommendations based on high quality evidence, most specialists choose a personalized approach that takes into account the specificity of respiratory mechanics in patients with severe BPD. An important goal is to achieve respiratory comfort, which allows achieving optimal growth and recovery of respiratory system.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"9 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunological screening as infectious complications prediction method in pediatric cardiac surgery","authors":"E. A. Degtyareva, B. Mwela, D. Kudlay, D. Ovsyannikov, M. Kantemirova, A. Prodeus, A. I. Kim, I. E. Nefedova, T. V. Rogova, M. Tumanyan, O.V. Alexeeva, I. Korsunsky","doi":"10.24110/0031-403x-2024-103-1-87-94","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-87-94","url":null,"abstract":"Incidence of infectious complications in children with congenital heart disease, especially with cyanotic and complex defects, is higher than in the population, which is associated with both hemodynamic and immune disorders. Various types of primary immunodeficiencies in congenital heart disease and the dependence of immune disorders on the morphology of the defect, the severity of circulatory failure and arterial hypoxemia are described. The importance of diagnosing immunological disorders is emphasized to reduce the risk of infectious complications of cardiac surgery, reduce mortality and improve the outcomes of surgical interventions for congenital heart disease. The prognostic value of immune disorders identified by the TREC/KREC method for infectious complications of cardiac surgery has not been studied. The purpose of this research was to assess the possibility of predicting infectious complications of cardiac surgery in children with congenital heart disease based on screening using the TREC/KREC method. Material and methods used: preoperatively, 200 children with congenital heart disease aged 3 days to 12 months old were examined. Instrumental and laboratory methods were used including immunological screening for TREC/KREC DNA. Results: 123 (62.4%) were diagnosed with “acyanotic” congenital heart disease, 74 (37.5%) with various cyanotic congenital heart diseases and 10 (5%) with congenital heart diseases with Down syndrome, Di George syndrome, Williams syndrome or multiple malformations. 184 (92%) underwent various cardiac surgical interventions. Violations of T-cell immunity according to preoperative TREC/KREC screening were observed in 23.5% of cases including in all children with syndromic forms of congenital heart disease, multiple malformations, and significantly more often in cyanotic congenital heart disease, conotruncal defects, and those admitted in critical conditions. Infectious complications of cardiac surgery were observed significantly more often in this group than in children with normal T-cell immunity (in 36% and 3.6%, respectively). Conclusion: the prognostic value of TREC/KREC screening for targeted preparation and postoperative management in order to prevent infectious complications of cardiac surgery has been confirmed.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"9 26","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957712","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early childhood cardiological profile of premature infants with a birth weight below 2500 gram","authors":"P. Khodkevich, K. V. Kulikova, O. Fedorova, I. Deev, E. S. Kulikov","doi":"10.24110/0031-403x-2024-103-1-30-38","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-30-38","url":null,"abstract":"Purpose of the research was to establish the characteristics of the cardiovascular system in premature newborns with a birth weight below 2500 gram in their early childhood. Methods used: the data of 302 children born in 2014-2020 in the Tomsk Oblast Regional Perinatal Center named after I.D. Yevtushenko and the Maternity Hospital No. 4 (both located in Tomsk, Russia) were analyzed. The control group of 76 was formed from healthy full-term newborns. The comparison group of 226 consisted of premature infants with birth weight below 2500 gram with the three subgroups depending on birth weight: low (LBW), very low (VLBW) and extremely low body weight (ELBW). During the follow-up that lasted for up to 3 years after birth an anamnesis, physical examination and fixation of the results of paraclinical studies (ECG, EchoCG) were carried out. Results: the probability of having a cardiac diagnosis among children with ELBW at birth at the time of discharge from the obstetric institution was 7 times higher compared to children with LBW (OR=7.60; [CI 95% 1.6-35.3]); birth weight below 1500 gram was associated with a 7-fold greater likelihood of PDA persistence compared to children with LBW at birth (OR=7.44; [CI 95% 3.34-16.55]); in the comparison group at the different age periods the low EchoCG parameters of heart size were established, while normal values of the left ventricular ejection fraction were noted. Conclusion: children with ELBW at birth differed in their cardiological profile already in their early childhood.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"6 20","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bacteriological effectiveness of the laboratory water bath ECROSKHIM PE-4300 for the expressed breast milk pasteurization","authors":"O.V. Nodvikova, S.M. Askhadullina, O. Lukoyanova, T. Borovik, T. Kazyukova","doi":"10.24110/0031-403x-2024-103-1-166-173","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-166-173","url":null,"abstract":"Breastfeeding is commonly acknowledged as being the biological norm. In cases when a child cannot be latched to the breast, freshly expressed mother's milk is the first choice that in specific conditions may require pasteurization. In accordance with the Russian sanitary rules and regulations as of 2021, “human milk is processed by boiling it in a water bath,” which is extremely destructive for the most of its biologically active and important components. The global practice of thermal processing of human milk is its low-temperature pasteurization according to the Holder method for 30 minutes at the temperature of +62.5°C. Currently there are no Russian domestically produced breast milk (BM) pasteurizers whilst it is not always possible to purchase imported equipment as yet. The purpose of this research was to assess the bacteriological safety of BM pasteurization using the laboratory water bath PE-4300 domestically produced by “ECROSKHIM” in Saint Petersburg, Russia, which allows the reproducing of the Holder method. Materials and methods used: a prospective single-center study of 153 lactating mothers was conducted with 306 BM samples being subjected to bacteriological analysis prior to and after the pasteurization. Results: before pasteurization, the growth of opportunistic bacteria was detected in all BM samples mainly due to the growth of S. epidermidis. After pasteurization, 96.1% of BM samples turned out to be sterile. In the remaining 3.9%, the scant growth of S. epidermidis (2.6%), Streptococcus oralis (0.65%) and Acinetobacter (0.65%) was not clinically significant. Conclusion: BM pasteurization in the laboratory water bath ECROSKHIM PE-4300 demonstrated high bacteriological safety of the device’s final revision, which in its turn allowed Authors to recommend this method of processing of both maternal and donor BM in pediatric and perinatal facilities.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"7 42","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital and acquired forms of cytomegalovirus infection in infants","authors":"N. V. Rogozina, V.V. Vasilyev, I.V. Markin, R. A. Ivanova","doi":"10.24110/0031-403x-2024-103-1-105-113","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-105-113","url":null,"abstract":"Cytomegalovirus infection (CMVI) is a common viral disease. Differential diagnosis of congenital and acquired forms of the disease in infants is rather difficult. The purpose of this research was to describe the cytomegalovirus infection clinical features in infants depending on the management tactics. Materials and methods used: а retrospective study of 95 medical records of pediatric patients treated for CMVI in their first year of life. 3 groups were formed as follows: G1 of 40 with confirmed diagnosis of congenital CMVI, G2 (43) with an assumed diagnosis of congenital CMVI and G3 (12) with acquired CMVI (the comparison group). Results: the study of the clinical manifestations of both the congenital and the acquired CMVIs showed that the acquired form of the disease runs as manifested infection and is characterized by acute onset of the disease, moderate intoxication, increase in body temperature to febrile levels (33.3%), rhinitis, catarrhal tonsillitis and lymphadenitis (41.7%), bronchitis (16.7%) and neutropenia (50.0%). The incidence of detection of clinical manifestations associated with CNS lesions did not differ significantly between children with congenital CMVI and those with suspected congenital CMVI despite their detection at a later period (at the age of 3 to 6 months old): 30% and 34.9%, respectively, p=0.636. On the other hand, neutropenia was statistically significantly more common among children with suspected congenital CMVI (41.9%) than among those with congenital CMVI (6%), p=0.007. Conclusion: when CMV DNA is detected in the blood in infants, congenital CMVI cannot be excluded.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"12 20","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of epoetin alfa in very preterm infants with birthweight below 1500 gram","authors":"A. L. Karpova, A. V. Mostovoy, L.A. Anikeeva, A.P. Khoteeva, S.V. Semenova, L. N. Karpov, S.V. Martirosyan, A. Zaplatnikov","doi":"10.24110/0031-403x-2024-103-1-39-48","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-39-48","url":null,"abstract":"Purpose of the research was to evaluate the effect of the administration of epoetin alfa (Eralfon®️) after 8 days of life in very preterm infants with birthweight below 1500 gram and gestational age below 310/7 weeks on the anemia of prematurity, on hemoglobin levels in blood at the age of six months and on retinopathy of prematurity. Materials and methods used: a retrospective study of 105 very preterm infants was conducted at the two perinatal centers, L.A. Vorokhobov Moscow City Clinical Hospital No. 67 of the Moscow Department of Healthcare (Moscow, Russia) and Yaroslavl Oblast Regional Perinatal Center (Yaroslavl, Russia), who were divided into two groups, G1 (56) who did not receive epoetin alpha (EA) at 1 to 2 months of life (the so-called “no EA Group”) and were nursed at the Yaroslavl Oblast Regional Perinatal Center; and G2 (49) who received EA at 1 to 2 months of life (the “EA Group”) and were nursed at the L.A. Vorokhobov Moscow City Clinical Hospital No. 67 of the Moscow Department of Healthcare. In the “EA Group,” there was late (after the 8th day of life) EA administration at a dose of 200 units/kg at once subcutaneously 3 times per week for 6 weeks. Results: hemoglobin levels in peripheral blood on the first day of life had no statistically significant differences. The level of hemoglobin in peripheral blood at the age of 6 months was statistically significantly higher in preterm infants who had received EA at 1 to 2 months of life. The development of the anemia of prematurity was statistically significantly less frequently recorded in the “EA Group:” 40 out of 49 (82.0%) vs. 54 out of 56 (96.0%), p=0.022. Erythrocyte transfusion in the “no EA Group” was performed in 38 out of 56 (67.8%), 25 out of 49 (51.0%) in the “EA group,” p=0.079, OR 0.49 [0.22, 1.08], RR 0.71 [0.47, 1.06]. Children who were in need of high-frequency oscillatory ventilation (HFOV) have not only had statistically significantly more frequent need for erythrocyte transfusion but also statistically significantly more often developed BPD and retinopathy of prematurity at the stage that requires surgical intervention. Conclusion: late EA administration in preterm infants with birthweight below 1500 gram reduces the risk of the development of the anemia of prematurity, increases the level of hemoglobin in peripheral blood at the age of six months old and does not increase the risk for development of retinopathy of prematurity.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"3 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incidence and the nature of nasal soft tissue injury during non-invasive respiratory support in newborns and its predisposing factors. A retrospective cohort study","authors":"E. Shestak, V. Starkov, O. P. Kovtun, I. Vakhlova","doi":"10.24110/0031-403x-2024-103-1-77-86","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-77-86","url":null,"abstract":"Non-invasive respiratory support is the preferred method for treating respiratory problems in newborns. Whilst having significant advantages, non-invasive methods also have their own complications associated with trauma to the nasal soft tissues such as erythema, ecchymosis, edema, necrosis in the area of the columella and nasal septum and vestibular stenosis as well as long-term consequences in the form of deformation of the nose and nasal septum. However, research studies aimed at determining the incidence and the nature of these injuries are very few. The purpose of this research was to determine the incidence and the nature of damage to the nasal soft tissues in newborns as a result of non-invasive respiratory support and its predisposing factors. Materials and methods used: a single-center retrospective cohort study of 558 newborns who were admitted at the Yekaterinburg Clinical Perinatal Center (Yekaterinburg, Sverdlovsk Oblast, Russia) during 12 months in 2022 and received non-invasive respiratory support: continuous positive airway pressure (CPAP), low flow nasal cannula (LFNC), was conducted with 176 (31.5%) full-term (≥370 weeks of gestation) and 382 (68.5%) premature (˂370 weeks)/190 (34%) with low body weight, 84 (15%) with very low body weight and 47 (8.4%) with extremely low body weight. Thus, the incidence and the nature of injuries to the nasal soft tissues were determined during non-invasive respiratory support in the observed pediatric patients. Results: the incidence of nasal injuries was ca. 10% in the entire patient cohort. No injuries were recorded in full-term infants. Among premature infants, injuries occurred in 84% of cases with a gestational age (GA) of 240 to 276 weeks, 35% with GA of 280 to 296 weeks, below 5% with GA of 300 to 336 weeks and 1.8% with GA 340 to 366 weeks. Therefore, in the groups of children with GA 240 to 276 and 280 to 296 weeks the nasal injury was most common because it was 84% of all cases of nasal injuries registered during these two particular gestational periods with 100% of grades 2 and 3 injuries and 100% of injuries to the nasal passages. The duration of any type of non-invasive respiratory therapy statistically significantly increased the incidence of any grade of injury. The use of LFNC statistically significantly increased the risk of damage to the nasal passages (OR 2.492; 95% CI 1.576-3.942). Conclusion: nasal injuries and injuries to nasal passages during non-invasive respiratory therapy can occur in up to 10% of cases. The main predisposing factors are low birth weight, low GA and long duration of any type of non-invasive respiratory therapy.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"2 11","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957948","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital (antenatal) hydronephrosis-modifiable risk factor for chronic kidney disease in children: interdisciplinary approach to prevention of chronic disease. Bibliographical review and the Authors’ own data","authors":"G. Poretskova, O. A. Sedashkina, L. Mazur, S.S. Terekhin, J.A. Gasanov, M. A. Barskaya, V.N. Balashova, V.N. Kuznetsova, G. Makovetskaya","doi":"10.24110/0031-403x-2024-103-1-66-76","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-66-76","url":null,"abstract":"Congenital hydronephrosis (CHN) is the most common malformation of the kidney which leads to the development of chronic kidney disease (CKD) in children up to its terminal stage. Current clinical practices related to observation and analysis of long-term outcome of the disease and interdisciplinary collaboration are in the need for further improvement. The purpose of the research was to determine whether CHN is a modifiable risk factor for the onset and progression of CKD in children. Materials and methods used: a single-center cohort retrospective study of the results of antenatal screening of fetuses with congenital defects of organs and systems both with CAKUT syndrome and CHN was conducted at the Samara Oblast Regional Clinical Hospital named after V.D. Seredavin (Samara, Russia) in 2013-2022. Results: 13,484 fetuses were registered with congenital malformations of organs and systems, of which 2,818 (20.9% [20.2%; 21.6%]) with those of urinary organs. 390 were born alive with CHN (13.8% [12.6%; 15.2%]), of which 359 (92%) had surgical intervention using Heinz-Andersen method at the age of 37 [0.3-44.5] weeks: 208 (58%) boys/151 (42%), girls; unilateral CHN in 345 (96%) and bilateral in 14 (4%). Recovery occurred in 328 (91.4% [88.0%; 93 ,9%]) cases with CHN after the surgery. Based on the results of the research, an algorithm for interdisciplinary interaction of healthcare specialists was developed for routing of the pregnant women with fetal CAKUT syndrome. Conclusion: CHN is a modifiable risk factor for the development of CKD since 91.4% of children recovered in the long term.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"4 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of the effectiveness of the use of bacteriophages in the treatment of acute intestinal infections caused by opportunistic pathogens in infants","authors":"K. Ermolenko, L.A. Kirilenko, N. V. Gonchar, N. V. Skripchenko","doi":"10.24110/0031-403x-2024-103-1-114-123","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-114-123","url":null,"abstract":"Acute intestinal infections caused by opportunistic microflora (OPM) are a pressing problem in infants. The purpose of this research was to investigate the treatment of enterocolitis caused by OPM in infants using antibacterial drugs and their detection by bacteriophages. Materials and methods used: a single-center retrospective cohort study of 106 pediatric patients aged 1 month to 1 year old with mild to moderate severity of acute intestinal infections caused by OPM and treated with an antibacterial drug (nifuroxazide), a bacteriophage (complex liquid pyophage) or a combination thereof, who were divided into the three groups as follows: G1 (“Nifuroxazide,” n=39), G2 (“Pyophage,” n=33) and G3 (“Combined drugs,” n=34), was conducted at the Scientific and Research Institute for Children’s Infections of the Russian Federal Biomedical Agency (Saint Petersburg, Russia) in 2018-2021. Data on pathogen elimination and duration of symptoms in patients were analyzed. The criteria for the effectiveness of therapy were the normalization of temperature, stool and coprograms and a decrease in the OPM titer in feces to a level of ≤104 CFU/g. Results: achievements of all applications of successful therapy occurred more often in G3 compared to G1 (0.007). An increased risk of treatment failure was evidenced by patients having OPM in a stool at the level of over 109 CFU/g (RR=14,09 [2,84; 69,96]) and the “visible entire” presence of erythrocytes (RR=6.26 [2.51; 15.62]) and leukocytes (RR=5,51 [2,22; 13,67]) in a coprogram. The probability of a response to therapy when using the active substances Nifuroxazide+Pyophage was lower (0.120 [0.037, 0.389]) than with monotherapy with Pyophage (0.227 [0.074, 0.692]) or Nifuroxazide (2.678 [1.14, 6.297]) alone. Conclusion: combined therapy of acute intestinal infections caused by OPM in infants using the antibacterial drugs coupled with Pyophage increases the effectiveness of OPM elimination and the disease’s clinical and laboratory symptoms’ relief.","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"2 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957915","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Atypical hemolytic-uremic syndrome associated with antibodies to factor H","authors":"Khadizha M Emirova, T. Pankratenko, V.K. Vakhitov, O. Orlova, O. Chernysheva, A. Muzurov, D. Kudlay, O.V. Zaitseva, S. Mstislavskaya, T. Abaseeva, G. A. Generalova, P. Shatalov, P. V. Avdonin, P. P. Avdonin","doi":"10.24110/0031-403x-2024-103-1-123-137","DOIUrl":"https://doi.org/10.24110/0031-403x-2024-103-1-123-137","url":null,"abstract":"Atypical hemolytic-uremic syndrome (aHUS) is an ultra-rare life-threatening disease from the group of thrombotic microangiopathies (TMA) with genetic predisposition factors associated with uncontrolled activation of the alternative complement pathway. In 10% of cases aHUS is acquired and associated with antibodies to factor H (CFH-Ab-aHUS), among which 90% of cases are also determined by a deficiency of plasma proteins related to factor H (CFHR). Information on the course, therapeutic approaches and outcomes of CFH-Ab-aHUS varies Worldwide and is presented by series of clinical observations. The purpose of the research was to determine the patterns of the course and outcomes of CFH-Ab-aHUS to substantiate methods of effective treatment and improve prognosis. Methods used: retro- and prospective analysis of the course and outcomes of CFH-Ab-aHUS in 43 patients in Jan. 2010-Feb. 2022. The sample is continuous. In the development of microangiopathic hemolytic anemia (MAHA), thrombocytopenia and acute kidney injury (AKI), the diagnosis of CFH-Ab-aHUS was established after exclusion of other forms of TMAs and determination of immunoglobulin class G (IgG) in serum against factor H by enzyme-linked immunosorbent assay (ELISA-VIDITEST anti-complement factor H, Czech Republic). In 41 cases molecular genetic study by NGS (next generation sequencing) for aHUS panel and Sanger sequencing were performed. Results: the incidence of CFH-Ab-aHUS was 20.6% among all cases of aHUS. In 88.4% of children the disease debuted at the age of above 4 y/o. It was associated with a trigger (infections, vaccination, trauma, etc.) in 76.7% of cases. The median (Me) level of antibodies to factor H was 12400 [4187; 22181] AU/ml, which was more than 8 times higher than the normal upper limit. The acute period was characterized by the development of the TMA triad (MAGA, thrombocytopenia, AKI) in 100%, hemorrhagic syndrome (69.8%), arterial hypertension (69.8%), C3 consumption (77.8%) and dysfunction of more than 2 systems (83.7%). Neoliguric form of AKI was diagnosed in 44.2%, cardiac involvement in 65.1%, CNS in 55.8%, GI in 51.2%, pulmonary in 23.3%, and visual organ in 16.3% of cases. CFHR1/CFHR3 deletions were found in 87.8% (36/41) of cases (77.8% homozygous/ 22.2% heterozygous). Relapses were noted in 34.9%. Remission of CFH-Ab-aHUS was achieved in 11.6% of cases using plasma therapy and immunosuppressants, in 88.4% - with complement-blocking therapy (eculizumab). The outcome of the disease was characterized by the development of arterial hypertension (65.1%), CKD (60.5%), hypertrophic (25.6%) and dilated cardiomyopathy (16.3%). Conclusion: thus, in the Russin pediatric population with aHUS, every fifth child is diagnosed with CFH-Ab-aHUS associated with a risk of recurrence in every third child. The most important components of the management of patients with CFH-Ab-aHUS are its timely diagnosis and initiation of pathogenetic therapy (plasma therapy with immunosuppressants","PeriodicalId":503254,"journal":{"name":"Pediatria. Journal named after G.N. Speransky","volume":"12 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139957583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}