Central European Journal of Immunology最新文献

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Hematopoietic stem cell transplantation in a patient with activated phosphoinositide 3‑kinase δ syndrome: A case report and literature review 活化磷酸肌酸 3- 激酶 δ 综合征患者的造血干细胞移植:病例报告与文献综述
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-12-21 DOI: 10.5114/ceji.2023.133949
Mateusz P. Łyżwa, Karolina Kędziora, Natalia Kałamarz, Jowita Frączkiewicz, Anna Panasiuk, Joanna Owoc-Lempach, Barbara Piątosa, Marcin Hennig, Ninela Irga-Jaworska, Krzysztof Kałwak
{"title":"Hematopoietic stem cell transplantation in a patient with activated phosphoinositide 3‑kinase δ syndrome: A case report and literature review","authors":"Mateusz P. Łyżwa, Karolina Kędziora, Natalia Kałamarz, Jowita Frączkiewicz, Anna Panasiuk, Joanna Owoc-Lempach, Barbara Piątosa, Marcin Hennig, Ninela Irga-Jaworska, Krzysztof Kałwak","doi":"10.5114/ceji.2023.133949","DOIUrl":"https://doi.org/10.5114/ceji.2023.133949","url":null,"abstract":"Activated phosphoinositide 3-kinase δ syndrome (APDS) is a recently described disease characterized by recurrent infections, lymphoproliferation with a high risk of malignancy, early-onset cytopenia, and a propensity for autoimmune diseases. Hematopoietic stem cell transplantation (HSCT) has proven to be an effective treatment method; however, the recovery process after HSCT is prolonged and accompanied by complications. In this study, we present the case of a patient with APDS type 1. Despite showing signs of immunodeficiency at the age of 6 months, it took almost 6 years to reach a definitive diagnosis. The patient experienced recurrent infections, often accompanied by anemia requiring transfusions, and multifocal nonmalignant lymphoproliferation. Only after receiving the appropriate diagnosis was it possible to implement proper and accurate treatment. HSCT was performed when the patient was 6 years old, leading to significant improvement in his condition. At the 17-month post-HSCT follow-up, the boy is asymptomatic and in good general health, although close monitoring continues due to mixed chimerism and delayed humoral immune recovery. Applying HSCT before the patient develops malignancy contributes to expanding the use of HSCT as a treatment option for APDS type 1.","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"12 1","pages":""},"PeriodicalIF":1.3,"publicationDate":"2023-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139910742","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The role of inflammatory and remodelling biomarkers in patients with non-small cell lung cancer 炎症和重塑生物标志物在非小细胞肺癌患者中的作用
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-12-13 DOI: 10.5114/ceji.2023.133725
Hemn Abdalla Omer, Christer Janson, Kawa Amin
{"title":"The role of inflammatory and remodelling biomarkers in patients with non-small cell lung cancer","authors":"Hemn Abdalla Omer, Christer Janson, Kawa Amin","doi":"10.5114/ceji.2023.133725","DOIUrl":"https://doi.org/10.5114/ceji.2023.133725","url":null,"abstract":"<b>Introduction:</b><br/>Biomarkers play a crucial role in evaluating the prognosis, diagnosis, and monitoring of non-small cell lung cancer (NSCLC). The aim of this study was to compare the levels of inflammatory and remodelling biomarkers among patients with NSCLC and healthy controls (HCs) and to investigate the correlation between these biomarkers.<br/><br/><b>Material and methods:</b><br/>Blood samples were taken from 93 NSCLC and 84 HCs. Each sample was analysed for the inflammatory biomarkers transforming growth factor β1 (TGF-β1), mothers against decapentaplegic homolog 2 (SMAD2) and the remodelling biomarkers Wingless-related integration site (Wnt3a) and β-catenin (CTNN-β1).<br/><br/><b>Results:</b><br/>The patients with NSCLC had significantly higher levels of all the measured biomarkers. In the NSCLC patients, TGF-β1 correlated significantly with SMAD2 (<i>r</i> = 0.34, <i>p</i> = 0.0008), Wnt3a (<i>r</i> = 0.328, <i>p</i> = 0.0013), and CTNN-β1 levels (<i>r</i> = 0.30, <i>p</i> = 0.004). SMAD2 correlated significantly with CTNN β1 (<i>r</i> = 0.546, <i>p</i> = 0.0001) and Wnt3a (<i>r</i> = 0.598, <i>p</i> = 0.0001). CTNN-β1 level also correlated with the level of Wnt3a (<i>r</i> = 0.61, <i>p</i> = 0.0001). No correlation was found between biomarkers and symptom scores.<br/><br/><b>Discussion:</b><br/>In this study, patients with NSCLC had higher inflammatory and remodelling biomarker levels than HCs. In the NSCLC, there were significant associations between inflammatory and remodelling biomarkers. This indicates that measuring biomarkers could be valuable in the workup of NSCLC patients.<br/><br/><b>Conclusions:</b><br/>Our investigation showed that inflammatory and remodelling biomarkers might play a role in future immunologic response and pharmacologically targeted NSCLC therapy.<br/><br/>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"69 1","pages":""},"PeriodicalIF":1.3,"publicationDate":"2023-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139924137","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Association of human leukocyte antigen class II alleles with epithelial cell apoptosis and extracellular matrix production in acute COVID-19 人类白细胞抗原 II 类等位基因与急性 COVID-19 上皮细胞凋亡和细胞外基质生成的关系
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-12-12 DOI: 10.5114/ceji.2023.133684
Ieva Vanaga, Oksana Kolesova, Aleksandrs Kolesovs, Gunta Sture, Elvira Hagina, Jelena Storozenko, Liene Nikitina-Zake, Ludmila Viksna
{"title":"Association of human leukocyte antigen class II alleles with epithelial cell apoptosis and extracellular matrix production in acute COVID-19","authors":"Ieva Vanaga, Oksana Kolesova, Aleksandrs Kolesovs, Gunta Sture, Elvira Hagina, Jelena Storozenko, Liene Nikitina-Zake, Ludmila Viksna","doi":"10.5114/ceji.2023.133684","DOIUrl":"https://doi.org/10.5114/ceji.2023.133684","url":null,"abstract":"<b>Introduction:</b><br/>Pathogenic mechanisms and long-term consequences of COVID-19 require attention in studies on SARS-CoV-2. The association of the severity of COVID-19 with genetic factors, such as human leukocyte antigen (HLA) genes, remains underexplored. Our study assessed the relationships between HLA class II alleles and COVID-19 severity and blood-based indicators of systemic inflammation and organ damage, serum markers of epithelial cell apoptosis such as caspase-cleaved CK18 fragment M30 (CK18-M30) and the extracellular matrix product hyaluronic acid (HA).<br/><br/><b>Material and methods:</b><br/>The study included 101 hospitalized COVID-19 patients (mean age 60 ±14 years). Clinical tests were performed at admission to the hospital. The levels of CK18-M30 and HA were detected in serum by enzyme-linked immunosorbent assay (ELISA). HLA typing was performed in HLA-DRB1, -DQA1, and -DQB1 loci by the polymerase chain reaction with low-resolution sequence-specific primers.<br/><br/><b>Results:</b><br/>Sixty-one patients had a non-severe and 40 had a severe or critical disease course (following the WHO definition). The severity was associated with older age, male gender, higher HA, CK18-M30, and some indicators of inflammation. Despite the lack of direct association between HLA alleles and the severity of COVID-19, the presence of HLA-DRB1*04 and 12 alleles in the genotype was associated with lowered or elevated HA, respectively. The HLA-DQB1*03:01 allele was associated with lowered CK18-M30, aspartate aminotransferase, and ferritin. In addition, HLA-DQB1*06:01 was associated with elevated alanine aminotransferase.<br/><br/><b>Conclusions:</b><br/>Associations of HLA class II alleles with markers of epithelial cell apoptosis and extracellular matrix production indirectly support the influence of HLA genes on acute COVID-19 severity.<br/><br/>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"2 1","pages":""},"PeriodicalIF":1.3,"publicationDate":"2023-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139923883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Newly diagnosed seropositive rheumatoid arthritis in a 52-year-old man superimposed on long-standing ankylosing spondylitis during secukinumab treatment 新诊断的血清阳性类风湿关节炎在52岁的男性叠加长期强直性脊柱炎在secukinumab治疗期间
4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.132066
Jaroslaw Nowakowski, Anna Tomoń, Dorota Telesińska-Jasiówka
{"title":"Newly diagnosed seropositive rheumatoid arthritis in a 52-year-old man superimposed on long-standing ankylosing spondylitis during secukinumab treatment","authors":"Jaroslaw Nowakowski, Anna Tomoń, Dorota Telesińska-Jasiówka","doi":"10.5114/ceji.2023.132066","DOIUrl":"https://doi.org/10.5114/ceji.2023.132066","url":null,"abstract":"rheumatoid arthritis (ra) and ankylosing spondylitis (as) are severe chronic inflammatory joint diseases with different immune-mediated mechanisms playing a role in their pathogenesis. rheumatoid arthritis is an erosive arthritis of peripheral joints and as is a spondyloarthropathy affecting mainly sacroiliac and spinal joints leading to excessive bone formation and ankylosis. The coexistence of rA and As in the same patient is rare. Presented here is a 52-year-old patient with long-standing As with bilateral ankylosis of sacroiliac joints who developed peripheral symmetric polyarthritis while being treated with the interleukin 17 inhibitor secukinumab introduced due to secondary inefficacy of the tu - mor necrosis a inhibitor etanercept. He was finally diagnosed with seropositive rA coexisting with As and treatment was changed to the Janus kinase inhibitor tofacitinib. eventually, remission was sustained with use of the interleukin 6 inhibitor tocilizumab. This is the first case of rA developing during anti-interleukin 17 therapy. Although tocilizumab lacks efficaciousness in As, in this case therapy was succesful as the rA-driving cytokine mechanism possibly prevailed.","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"41 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135059027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Immunotherapy of B cell lymphoma with CD22-redirected CAR NK-92 cells. cd22重定向CAR - NK-92细胞对B细胞淋巴瘤的免疫治疗
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.126672
Xiaopeng Tian, Ruixi Zhang, Huimin Qin, Xiangru Shi, Wenhui Qi, Dongpeng Jiang, Tingting Zhu, Aining Sun
{"title":"Immunotherapy of B cell lymphoma with CD22-redirected CAR NK-92 cells.","authors":"Xiaopeng Tian,&nbsp;Ruixi Zhang,&nbsp;Huimin Qin,&nbsp;Xiangru Shi,&nbsp;Wenhui Qi,&nbsp;Dongpeng Jiang,&nbsp;Tingting Zhu,&nbsp;Aining Sun","doi":"10.5114/ceji.2023.126672","DOIUrl":"https://doi.org/10.5114/ceji.2023.126672","url":null,"abstract":"<p><strong>Introduction: </strong>Chimeric antigen receptor (CAR)-NK cells are considered safer than CAR-T cells due to their short lifetime and production of lower toxicity cytokines. By virtue of unlimited proliferative ability in vitro, NK-92 cells could be utilized as the source for CAR-engineered NK cells. CD22 is highly expressed in B cell lymphoma. The goal of our study was to determine whether CD22 could become an alternative target for CAR-NK-92 therapy against B cell lymphoma.</p><p><strong>Material and methods: </strong>We first generated m971-BBZ NK-92 that expressed a CAR for binding CD22 in vitro. The expression of CAR was assessed by flow cytometric analysis as well as immunoblotting. The cytotoxicity of the m971-BBZ NK-92 cells towards target lymphoma cells was determined by the luciferase-based cytolysis assay. The production of cytokines in CAR NK-92 cells in response to target cells was evaluated by ELISA assay. Lastly, the cytolytic effect was evaluated by the cytolysis assay mentioned above following irradiation. The level of inhibitory receptor of CAR-expressing cells was assessed by flow cytometry.</p><p><strong>Results: </strong>CD22-specific CAR was expressed on m971-BBZ NK-92 cells successfully. m971-BBZ NK-92 cells efficiently lysed CD22-expressing lymphoma cells and produced large amounts of cytokines after coculture with target cells. Meanwhile, irradiation did not apparently influence the cytotoxicity of m971-BBZ NK-92 cells. Inhibitory receptor detection exhibited a lower level of PD-1 in m971-BBZ NK-92 cells than FMC-63 BBZ T cells after repeated antigen stimulation.</p><p><strong>Conclusions: </strong>Our data show that adoptive transfer of m971-BBZ NK-92 could serve as a promising strategy for immunotherapy of B cell lymphoma.</p>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"48 1","pages":"1-13"},"PeriodicalIF":1.3,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/17/d1/CEJI-48-50550.PMC10189576.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9930543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Trigeminal neuralgia occurring after the third dose of Pfizer BioNTech COVID-19 vaccine. Complication or coincidence? An illustrative case report and literature review. 三叉神经痛发生在第三剂辉瑞BioNTech COVID-19疫苗后。复杂还是巧合?一篇说明性病例报告及文献回顾。
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.125309
Kacper Chrostowski, Michał Piasecki, Joanna Bielewicz
{"title":"Trigeminal neuralgia occurring after the third dose of Pfizer BioNTech COVID-19 vaccine. Complication or coincidence? An illustrative case report and literature review.","authors":"Kacper Chrostowski,&nbsp;Michał Piasecki,&nbsp;Joanna Bielewicz","doi":"10.5114/ceji.2023.125309","DOIUrl":"https://doi.org/10.5114/ceji.2023.125309","url":null,"abstract":"<p><p>The coronavirus disease 2019 pandemic is an ongoing concern for medical care worldwide. Since its emergence, multiple COVID-19 vaccines have been designed, allowing for more effective control of the pandemic. COVID-19 vaccines, like any other form of medical intervention, may cause adverse and unforeseen side effects, varying in frequency and severity. Determining a correlation between the occurring symptoms and the vaccination is often a challenging task, requiring multiple data sources and reported cases. So far, there have been multiple reports of trigeminal neuralgia developing after COVID-19 vaccination. A 36-year-old woman was admitted to the Emergency Ward due to chronic pain attacks in the left side of her face. The pain appeared two months ago, on the day following the vaccination using the third dose of the Pfizer BioNTech COVID-19 vaccine. At the Neurology Department she was diagnosed with trigeminal neuralgia. Based on the lack of any obvious causes, relation to the vaccination, and other similar reports, we assumed that the trigeminal neuralgia was a complication of the vaccination. Hospital treatment consisted of oxcarbazepine, dexamethasone and pregabalin. Treatment was successful, with transient episodes of exacerbation. Six months after the onset of the disorder the patient remains without pain. We believe that the presented case supports the possibility of trigeminal neuralgia occurring in relation to the Pfizer BioNTech COVID-19 vaccine administration. Additional reports may further contribute to establishing a certain link.</p>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"48 1","pages":"75-80"},"PeriodicalIF":1.3,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d1/14/CEJI-48-50183.PMC10189574.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9930538","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
The role of monocytes in malaria infection. 单核细胞在疟疾感染中的作用。
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.126650
Keh Min Xuan, Nurhidanatasha Abu Bakar, Khairul Mohd Fadzli Mustaffa, Maryam Azlan
{"title":"The role of monocytes in malaria infection.","authors":"Keh Min Xuan,&nbsp;Nurhidanatasha Abu Bakar,&nbsp;Khairul Mohd Fadzli Mustaffa,&nbsp;Maryam Azlan","doi":"10.5114/ceji.2023.126650","DOIUrl":"https://doi.org/10.5114/ceji.2023.126650","url":null,"abstract":"<p><p>Malaria remains one of the most common human infections worldwide. In endemic areas, malaria is a leading cause of morbidity and mortality and it imposes significant socioeconomic burdens on the people affected. Monocytes are part of the immune system controlling parasite burden and protecting the host against malaria infection. Monocytes play their protective roles against malaria via phagocytosis, cytokine production and antigen presentation. Though monocytes are crucial for clearance of malaria infection, they have also been shown to cause adverse clinical outcomes. In this review, we discuss recent findings regarding the role of monocytes in malaria via mechanisms such as parasite detection and clearance, pro-inflammatory activities, and activation of other immune components. We also highlight the role of different monocyte subsets, and other myeloid cells that are involved in malaria infection. However, more investigations are required in order to explore the exact roles of these monocytes in malaria infection.</p>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"48 1","pages":"54-62"},"PeriodicalIF":1.3,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/7b/fc/CEJI-48-50545.PMC10189572.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9930541","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Molecular regulatory mechanism of LILRB4 in the immune response. LILRB4在免疫应答中的分子调控机制。
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.125238
Haiyin Liu, Jun Yang, Jing Zhang, Peiyue Zhang, Mengting Zhang, Chaojun Yang, Li Liu, Cuiyuan Huang, Wei Wang, Yuhong Zhai, Jian Yang
{"title":"Molecular regulatory mechanism of LILRB4 in the immune response.","authors":"Haiyin Liu,&nbsp;Jun Yang,&nbsp;Jing Zhang,&nbsp;Peiyue Zhang,&nbsp;Mengting Zhang,&nbsp;Chaojun Yang,&nbsp;Li Liu,&nbsp;Cuiyuan Huang,&nbsp;Wei Wang,&nbsp;Yuhong Zhai,&nbsp;Jian Yang","doi":"10.5114/ceji.2023.125238","DOIUrl":"https://doi.org/10.5114/ceji.2023.125238","url":null,"abstract":"<p><p>Immune diseases are caused by the imbalance of immune regulation. This imbalance is regulated by many factors, both negative and positive. Leukocyte immunoglobulin-like receptor B4 (LILRB4) is a member of leukocyte immunoglobulin-like receptors (LILRs). LILRs are expressed constitutively on the surface of multiple immune cells which associate with membrane adaptors to signal through multi- ple cytoplasmic immunoreceptor tyrosine-based inhibitory motifs (ITIMs) or immunoreceptor tyro-sine-based activation motifs (ITAMs). Through ITIM, LILRB4 could recruit the src homology domain type-2-containing tyrosine phosphatase 1 or 2 (SHP-1 or SHP-2) into the cell membrane. In addition, many factors can induce the expression of LILRB4, such as vitamin D, interferon and so on. Studies have demonstrated that LILRB4 had a negative regulatory role in various of immune diseases. The present review intends to expound the structure and function of LILRB4, as well as its regulators and receptors in the immune cells, so as to provide a theoretical basis for immune disease therapy.</p>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"48 1","pages":"43-47"},"PeriodicalIF":1.3,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c9/5e/CEJI-48-50171.PMC10189573.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9933154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A rare cause of steroid-resistant nephrotic syndrome - a case report. 类固醇抵抗性肾病综合征的罕见病因- 1例报告。
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.127534
Paulina Kuran, Emilia Platos, Małgorzata Mizerska-Wasiak, Małgorzata Pańczyk-Tomaszewska
{"title":"A rare cause of steroid-resistant nephrotic syndrome - a case report.","authors":"Paulina Kuran,&nbsp;Emilia Platos,&nbsp;Małgorzata Mizerska-Wasiak,&nbsp;Małgorzata Pańczyk-Tomaszewska","doi":"10.5114/ceji.2023.127534","DOIUrl":"https://doi.org/10.5114/ceji.2023.127534","url":null,"abstract":"<p><p>Steroid resistance is a common condition occurring in children with nephrotic syndrome. Until now, over 50 genes involved in steroid-resistant nephrotic syndrome (SRNS) pathogenesis have been identified, among which the most prevalent are NPHS1, NPHS2, CD2AP, and PTPRO. The patterns of inheritance of SRNS are autosomal recessive, autosomal dominant, or mitochondrial, and tissues of those patients show focal segmental glomerulosclerosis (FSGS) signs in histopathological image analysis. We present a case of a 6-year-old girl who was admitted to the pediatric nephrology department due to nephrotic range proteinuria and edema of the lower leg. We started therapy with prednisone at a dose of 45 mg (60 mg/m<sup>2</sup>), enalapril as a nephroprotection, and antihistamines as an additional treatment. During in-patient treatment, we detected increased blood pressure. Due to persistent proteinuria in spite of 6-week treatment with steroids at the maximal dose, we confirmed disease resistance to steroids. Additionally, FSGS signs were confirmed in kidney biopsy samples. After genetic screening for SRNS and detection of the rare gene mutation NUP93 we reduced prednisone but maintained nephroprotective treatment and administered cyclosporin A. The girl remains currently under the care of nephrologists with normal arterial blood pressure, trace proteinuria in follow-up examination, and normal kidney function. NUP93 mutation is extremely rare; therefore few cases have been described to date. The onset of the symptoms in all pediatric patients appeared before the age of 8 and they developed end stage kidney disease (ESKD). They might manifest symptoms from the other systems.</p>","PeriodicalId":9694,"journal":{"name":"Central European Journal of Immunology","volume":"48 2","pages":"158-162"},"PeriodicalIF":1.3,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/48/a2/CEJI-48-50695.PMC10485693.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10220431","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Effects of theophylline combined with inhaled corticosteroids on patients with moderate and severe asthma and changes of T lymphocyte subsets in peripheral blood. 茶碱联合吸入皮质类固醇对中重度哮喘患者外周血T淋巴细胞亚群的影响。
IF 1.3 4区 医学
Central European Journal of Immunology Pub Date : 2023-01-01 DOI: 10.5114/ceji.2023.127843
Xiaozhen Cai, Rong Rong, Yidan Huang, Xiaowen Pu, Nanhai Ge
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