Current pediatrics最新文献

{"title":"Short-Term Outcomes of Simultaneous Inguinal Hernia Repair at Laparoscopic Appendectomy in Children: Cohort Study","authors":"V. Gavrilyuk, D. Severinov, YA Zubkova","doi":"10.15690/vsp.v23i1.2651","DOIUrl":"https://doi.org/10.15690/vsp.v23i1.2651","url":null,"abstract":"Background. Nowadays, simultaneous techniques have been widely adopted in abdominal pediatric surgery as they allow to perform surgical interventions for several diseases at the same time. However, the safety of such interventions requires further studies. Objective. The aim of the study is to analyze the short-term outcomes of simultaneous hernia repair in laparoscopic appendectomy in children. Methods. The study included data from patients hospitalized with suspected acute appendicitis in 2022–2023. The study group included children operated for acute appendicitis and who had signs of patent processus vaginalis. Percutaneous internal ring suturing (PIRS) was performed in all cases. The control group (no indirect inguinal hernia was revealed) was formed by pairwise matching of patients by gender, age (± 1 year), and appendicitis type. The surgery time, the blood loss volume, the beginning of enteral feeding, and the presence of postoperative complications were analyzed. Results. The compared groups (13 individuals each) were comparable in gender (38% boys), age (median age about 10 years), and appendicitis type (catarrhal — 31%, phlegmonous — 38%, gangrenous — 31%), and white cell count at admission, presence of serous exudate and omentum changes revealed during surgery. The beginning of enteral feeding, the stool, as well as total duration of hospital stay after surgery did not differ in two groups. Moreover, both groups were comparable in terms of surgery duration, intraoperative blood loss volume, pain syndrome severity. No pyo-inflammatory complications of abdominal cavity or postoperative wounds were revealed during the hospital stay. Conclusion. Simultaneous hernia repair via PIRS method is effective and safe method of surgical management of pediatric patients with combination of acute appendicitis and inguinal hernia.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140433018","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":"Combination Treatment for Severe Forms of Mucopolysaccharidosis, Type I (Hurler Syndrome): Case Report","authors":"N. D. Vashakmadze, N. V. Zhurkova, Marina A. Babaykina, Albina V. Dobrotok, O. Gordeeva, L. S. Namazova-Baranova","doi":"10.15690/vsp.v22i6.2701","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2701","url":null,"abstract":"Background. Hurler syndrome (mucopolysaccharidosis, type I) is a rare hereditary disease with chronic course. The main methods for Hurler syndrome management are hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). In recent years, combination treatment (ERT administration both before and after HSCT) has shown its efficacy in case of disease progression. Clinical case description. The presented clinical cases demonstrate the efficacy of ERT administration in patients with Hurler syndrome after HSCT: in the first clinical case due to the decrease in alpha-iduronidase activity 2 years after HSCT, in the second clinical case due to the aggravation of the patient's condition (cardiovascular and respiratory systems, hepatomegaly, although the level of enzyme and glycosaminoglycans in the patient's urine remained within normal values). Conclusion. Combination treatment including ERT not only before HSCT, but also in case of clinical state worsening after HSCT, plays significant role in stabilizing the patient's condition, preventing rapid progression of symptoms and development of life-threatening complications (especially cardiovascular ones).","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139623973","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":"Hereditary Amino Acid Metabolism Disorders and Urea Cycle Disorders: to Practicing Physician","authors":"N. V. Zhurkova, N. D. Vashakmadze, Nataliya S. Sergienko, Anastasiya N. Dudina, M. S. Karaseva, L. Selimzyanova, A. Y. Rachkova, Y. Kotalevskaya, Andrej N. Surkov","doi":"10.15690/vsp.v22i6.2700","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2700","url":null,"abstract":"Hereditary amino acid metabolism disorders (aminoacidopathies) are clinically and genetically heterogeneous group of hereditary metabolic diseases caused by enzymes deficiency involved in amino acid metabolism, that finally leads to progressive damage of central nervous system, liver, kidneys, and other organs and systems. Hereditary urea cycle disorders occur because of enzyme deficiency leading to impaired urea synthesis and hyperammoniemia in patients. The age of disease onset and clinical manifestations severity range from milder, intermittent forms to severe, manifesting in the first hours of life. Expanded neonatal screening (implemented in Russian Federation at 01.01.2023) allows to diagnose diseases from these groups in the first days of life, to prescribe timely pathogenetic therapy. Altogether it helps to prevent the development of disease severe complications. Raising awareness about hereditary aminoacidopathies and urea cycle disorders among pediatricians, neonatologists, neurologists, gastroenterologists, ophthalmologists is a topical issue of modern pediatrics.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139624854","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":"Belimumab in a Patient with Systemic Lupus Erythematosus with Juvenile Onset and Steroid-induced Diabetes: Clinical Case","authors":"M. Kaleda, I. Nikishina, Alesya V. Firsa","doi":"10.15690/vsp.v22i6.2649","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2649","url":null,"abstract":"Background. The management of children with systemic lupus erythematosus (SLE) is usually associated with lifelong systemic glucocorticoids administration and, thereby, high risk of serious side effects, including steroid-induced diabetes. The belimumab (B-lymphocyte stimulator inhibitor) administration significantly reduces the glucocorticoids dose, the risk and severity of steroid therapy complications. Clinical case description. The patient was diagnosed with SLE at the age of 16 years. Therapy with hydroxychloroquine and oral glucocorticoid at a high dose (methylprednisolone 56 mg per day) was initiated. Steroid-induced diabetes was diagnosed 1 month after the therapy start; avascular necrosis sites were revealed in 2 months. Mycophenolate mofetil made it possible to achieve the disease activity control. However, the belimumab was prescribed 5 months after diagnosis verification due to continuous insulin requirement and avascular necrosis progression. Conclusion. Belimumab is the only genetically engineered biologic drug approved for the treatment of children with SLE. As a result of its use, it was possible to stabilize the patient's condition quickly (within 3 months), to reduce significantly the dose of oral glucocorticoid, methylprednisolone (from 24 to 8 mg/day), to achieve remission of steroidinduced diabetes with further insulin withdrawal, and also to relieve avascular necrosis clinical symptoms.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139624673","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":"Analysis of the Treatment Efficacy in Late Diagnosis of Alport Syndrome in a Child: Clinical Case","authors":"S. Y. Volgina, N. A. Solovyeva, G. A. Kulakova, E. A. Kurmayeva, Liliya I. Mukhametdinova, E. L. Rashitova","doi":"10.15690/vsp.v22i6.2705","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2705","url":null,"abstract":"Background. Alport syndrome is a systemic, hereditary, progressive disease characterized by ultrastructural changes in the glomerular basement membrane caused by pathogenic variants of type IV collagen genes. The use of angiotensin-converting enzyme inhibitors (ACEI) for nephroprotection is effective at the microhematuria and/or albuminuria stage. Treatment tactics in case of nephrotic syndrome development in such patients remains the subject of discussion. Clinical case description. The patient was diagnosed with proteinuria at the neonatal period and hematuria at the age of one month. The hereditary nephritis was diagnosed at the age of 6 years; the ACEI was administered, however, the proteinuria continued to increase. The diagnosis was confirmed at the age of 8.5 years via the puncture nephrobiopsy: collagenopathy, type IV, focal segmental glomerular sclerosis. Moreover, chronic bilateral sensorineural hearing loss and bilateral myopic astigmatism were diagnosed. Ciclosporin A (125 mg/day) was additionally prescribed. The increase in the cystatin C, urea, uric acid, cholesterol levels in blood was mentioned after 14 months of treatment. These parameters decreased after reducing cyclosporine A dose to 100 mg/day, however, proteinuria has increased. Angiotensin II receptor blocker (candesartan 8 mg/day) was prescribed to enhance nephroprotective therapy at the age of 10 years 2 months. Another increase of the immunodepressant dose was performed at the age of 11, it led to decrease in the estimated glomerular filtration rate and increase of creatinine, cystatin C, urea, cholesterol, uric acid, and potassium levels in the blood. These changes were considered as cyclosporine-dependent. The dose of cyclosporine A was reduced to 125 mg/day, and to 100 mg/day from the age of 14. There was no progression of chronic kidney disease at the follow-up at the age of 15.5 years. Conclusion. Nephroprotective treatment of a child with Alport syndrome initiated after the development of nephrotic syndrome did not stop the chronic kidney disease progression. Whereas relatively high doses of ciclosporin A have reduced proteinuria but led to nephrotoxicity and cyclosporin dependence.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139625135","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":"Niemann-Pick Disease: Seven Questions about it","authors":"N. D. Vashakmadze, N. V. Zhurkova","doi":"10.15690/vsp.v22i6.2702","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2702","url":null,"abstract":"Acid sphingomyelinase deficiency is a rare hereditary disease caused by enzyme deficiency due to mutations in the SMPD1 gene. Decreased enzyme activity leads to accumulation of sphingomyelin in lysosomes. This disease is characterized by wide diversity of clinical manifestations: from infantile neurovisceral form (Niemann-Pick disease type A) with severe rapidly progressive neurodegeneration to chronic visceral form (Niemann-Pick disease type B) with almost no central nervous system involvement. There is also intermediate phenotype: chronic neurovisceral form (Niemann-Pick disease type A/B) that includes manifestations two other forms, both visceral and neurological. The disease course is most commonly progressive and multi-system due to sphingomyelin accumulation in cells of the monocyte-macrophage system, as well as in other cells such as hepatocytes.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139624879","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":"Brain Morphometry is an Advanced Method of Neuroimaging Mapping in Children","authors":"Alexey I. Firumyants, L. S. Namazova-Baranova, Georgiy A. Karkashadze, O. P. Kovtun, Viktor V. Dyachenko, N. Shilko, Elena N. Rudenko, Alexey V. Meshkov, Nataliya S. Sergienko, Yuliya V. Nesterova, Leonid M. Yatsick, Anastasya I. Rykunova","doi":"10.15690/vsp.v22i6.2707","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2707","url":null,"abstract":"The use of magnetic resonance imaging in morphometry, as quantitative assessment of brain parameters (thickness, surface area, volume), allows to detect changes in many neuropsichiatric conditions that were previously considered intact. This article provides data on neuroimaging brain morphometry and effective use of this method in neurosciences.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139624690","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":"Role of Calcineurin Inhibitors in the Management of Atopic Dermatitis in Children","authors":"K. Efendieva, L. S. Namazova-Baranova, Juliya G. Levina, V. Kalugina, A. Alekseeva, Elena V. Vishneva, K. Volkov","doi":"10.15690/vsp.v22i6.2666","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2666","url":null,"abstract":"Atopic dermatitis (AD) is a widespread chronic inflammatory skin disease that has a significant impact on various aspects of patient's life. This review presents modern view on AD pathophysiology, its correlation with other concomitant diseases, and covers practical aspects of external anti-inflammatory therapy implementation. The analysis of clinical studies has shown the significant role of calcineurin inhibitors in the effective treatment of AD in children from its first manifestations at early age.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139626482","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":"Breast Milk Jaundice","authors":"I. Belyaeva, E. Bombardirova, Elena O. Kurnatovskaya","doi":"10.15690/vsp.v22i6.2656","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2656","url":null,"abstract":"The results of modern studies on breast milk jaundice pathogenesis, that is common form of neonatal jaundice, are presented. The major risk factors and diagnostic methods (excluding other forms of jaundice syndrome at recurrent and lingering jaundice in newborns) were defined. The main therapy approaches for breast milk jaundice were presented. Issues of phototherapy indications were discussed. The feasibility of maintaining breastfeeding in case of breast milk jaundice development was proved. This condition requires the attention of pediatricians despite the relatively favorable course and outcomes. The need to continue studies on pathogenetic therapy of such patients was noted.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139625550","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":"Supplemental Feeding Implementation: Window of Opportunities for the Intestinal Microbiota Development and Immune Responses Modulation","authors":"I. Belyaeva, L. S. Namazova-Baranova, E. Bombardirova, Regina A. Shukenbayeva, T. Turti","doi":"10.15690/vsp.v22i6.2663","DOIUrl":"https://doi.org/10.15690/vsp.v22i6.2663","url":null,"abstract":"This review summarizes stages of intestinal microbiota development in infant and immune responses modulation associated to these stages. The leading role of breastfeeding in the optimal microbiota and associated immune responses development during the first half of child’s life is presented. The biological feasibility of supplemental feeding implementation at the second window of opportunity (4–6 months) is justified, as well as role of supplementation products (including cereal) in adult microbiota development.","PeriodicalId":10867,"journal":{"name":"Current pediatrics","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139626461","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}