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  1. Article: Editorial: Recent advances in pediatric red blood cells disorders.

    Canciani, Gabriele / Palumbo, Giuseppe / Brewin, John / Rossi, Francesca / Ceglie, Giulia

    Frontiers in pediatrics

    2024  Volume 12, Page(s) 1403651

    Language English
    Publishing date 2024-04-03
    Publishing country Switzerland
    Document type Editorial
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2024.1403651
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Genome editing for sickle cell disease: still time to correct?

    Ceglie, Giulia / Lecis, Marco / Canciani, Gabriele / Algeri, Mattia / Frati, Giacomo

    Frontiers in pediatrics

    2023  Volume 11, Page(s) 1249275

    Abstract: Sickle cell disease (SCD) is an inherited blood disorder, due to a single point mutation in the β-globin gene ( ...

    Abstract Sickle cell disease (SCD) is an inherited blood disorder, due to a single point mutation in the β-globin gene (
    Language English
    Publishing date 2023-11-02
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2023.1249275
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  3. Article ; Online: Prognosis of COVID-19 in Patients with Liver and Kidney Diseases: An Early Systematic Review and Meta-Analysis.

    Oyelade, Tope / Alqahtani, Jaber / Canciani, Gabriele

    Tropical medicine and infectious disease

    2020  Volume 5, Issue 2

    Abstract: The mortality and severity in COVID-19 is increased in patients with comorbidities. The aim of this study was to evaluate the severity and mortality in COVID-19 patients with underlying kidney and liver diseases. We retrieved data on the clinical ... ...

    Abstract The mortality and severity in COVID-19 is increased in patients with comorbidities. The aim of this study was to evaluate the severity and mortality in COVID-19 patients with underlying kidney and liver diseases. We retrieved data on the clinical features and primary composite end point of COVID-19 patients from Medline and Embase which had been released from inception by the April 16, 2020. The data on two comorbidities, liver diseases and chronic kidney disease, were pooled and statistically analysed to explain the associated severity and mortality rate. One hundred and forty-two abstracts were screened, and 41 full articles were then read. In total, 22 studies including 5595 COVID-19 patients were included in this study with case fatality rate of 16%. The prevalence of liver diseases and chronic kidney disease (CKD) were 3% (95% CI; 2-3%) and 1% (95% CI; 1-2%), respectively. In patients with COVID-19 and underlying liver diseases, 57.33% (43/75) of cases were severe, with 17.65% mortality, while in CKD patients, 83.93% (47/56) of cases were severe and 53.33% (8/15) mortality was reported. This study found an increased risk of severity and mortality in COVID-19 patients with liver diseases or CKD. This will lead to better clinical management and inform the process of implementing more stringent preventative measures for this group of patients.
    Keywords covid19
    Language English
    Publishing date 2020-05-15
    Publishing country Switzerland
    Document type Journal Article ; Review
    ISSN 2414-6366
    ISSN (online) 2414-6366
    DOI 10.3390/tropicalmed5020080
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  4. Article ; Online: CAR-T Therapy for Pediatric High-Grade Gliomas: Peculiarities, Current Investigations and Future Strategies.

    Antonucci, Laura / Canciani, Gabriele / Mastronuzzi, Angela / Carai, Andrea / Del Baldo, Giada / Del Bufalo, Francesca

    Frontiers in immunology

    2022  Volume 13, Page(s) 867154

    Abstract: High-Grade Gliomas (HGG) are among the deadliest malignant tumors of central nervous system (CNS) in pediatrics. Despite aggressive multimodal treatment - including surgical resection, radiotherapy and chemotherapy - long-term prognosis of patients ... ...

    Abstract High-Grade Gliomas (HGG) are among the deadliest malignant tumors of central nervous system (CNS) in pediatrics. Despite aggressive multimodal treatment - including surgical resection, radiotherapy and chemotherapy - long-term prognosis of patients remains dismal with a 5-year survival rate less than 20%. Increased understanding of genetic and epigenetic features of pediatric HGGs (pHGGs) revealed important differences with adult gliomas, which need to be considered in order to identify innovative and more effective therapeutic approaches. Immunotherapy is based on different techniques aimed to redirect the patient own immune system to fight specifically cancer cells. In particular, T-lymphocytes can be genetically modified to express chimeric proteins, known as chimeric antigen receptors (CARs), targeting selected tumor-associated antigens (TAA). Disialoganglioside GD2 (GD-2) and B7-H3 are highly expressed on pHGGs and have been evaluated as possible targets in pediatric clinical trials, in addition to the antigens common to adult glioblastoma - such as interleukin-13 receptor alpha 2 (IL-13α2), human epidermal growth factor receptor 2 (HER-2) and erythropoietin-producing human hepatocellular carcinoma A2 receptor (EphA2). CAR-T therapy has shown promise in preclinical model of pHGGs but failed to achieve the same success obtained for hematological malignancies. Several limitations, including the immunosuppressive tumor microenvironment (TME), the heterogeneity in target antigen expression and the difficulty of accessing the tumor site, impair the efficacy of T-cells. pHGGs display an immunologically cold TME with poor T-cell infiltration and scarce immune surveillance. The secretion of immunosuppressive cytokines (TGF-β, IL-10) and the presence of immune-suppressive cells - like tumor-associated macrophages/microglia (TAMs) and myeloid-derived suppressor cells (MDSCs) - limit the effectiveness of immune system to eradicate tumor cells. Innovative immunotherapeutic strategies are necessary to overcome these hurdles and improve ability of T-cells to eradicate tumor. In this review we describe the distinguishing features of HGGs of the pediatric population and of their TME, with a focus on the most promising CAR-T therapies overcoming these hurdles.
    MeSH term(s) Child ; Glioblastoma ; Humans ; Immunotherapy, Adoptive/methods ; Liver Neoplasms ; Pediatrics ; Receptors, Chimeric Antigen/genetics ; Tumor Microenvironment
    Chemical Substances Receptors, Chimeric Antigen
    Language English
    Publishing date 2022-05-04
    Publishing country Switzerland
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2022.867154
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  5. Article: Modelling Pancreatic Neuroendocrine Cancer: From Bench Side to Clinic.

    Ney, Alexander / Canciani, Gabriele / Hsuan, J Justin / Pereira, Stephen P

    Cancers

    2020  Volume 12, Issue 11

    Abstract: Pancreatic neuroendocrine tumours (pNETs) are a heterogeneous group of epithelial tumours with neuroendocrine differentiation. Although rare (incidence of <1 in 100,000), they are the second most common group of pancreatic neoplasms after pancreatic ... ...

    Abstract Pancreatic neuroendocrine tumours (pNETs) are a heterogeneous group of epithelial tumours with neuroendocrine differentiation. Although rare (incidence of <1 in 100,000), they are the second most common group of pancreatic neoplasms after pancreatic ductal adenocarcinoma (PDAC). pNET incidence is however on the rise and patient outcomes, although variable, have been linked with 5-year survival rates as low as 40%. Improvement of diagnostic and treatment modalities strongly relies on disease models that reconstruct the disease ex vivo. A key constraint in pNET research, however, is the absence of human pNET models that accurately capture the original tumour phenotype. In attempts to more closely mimic the disease in its native environment, three-dimensional culture models as well as in vivo models, such as genetically engineered mouse models (GEMMs), have been developed. Despite adding significant contributions to our understanding of more complex biological processes associated with the development and progression of pNETs, factors such as ethical considerations and low rates of clinical translatability limit their use. Furthermore, a role for the site-specific extracellular matrix (ECM) in disease development and progression has become clear. Advances in tissue engineering have enabled the use of tissue constructs that are designed to establish disease ex vivo within a close to native ECM that can recapitulate tumour-associated tissue remodelling. Yet, such advanced models for studying pNETs remain underdeveloped. This review summarises the most clinically relevant disease models of pNETs currently used, as well as future directions for improved modelling of the disease.
    Language English
    Publishing date 2020-10-28
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2527080-1
    ISSN 2072-6694
    ISSN 2072-6694
    DOI 10.3390/cancers12113170
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  6. Article ; Online: Heart rate variability in patients with cirrhosis: a systematic review and meta-analysis.

    Oyelade, Tope / Canciani, Gabriele / Carbone, Gabriele / Alqahtani, Jaber S / Moore, Kevin / Mani, Ali R

    Physiological measurement

    2021  Volume 42, Issue 5

    Abstract: ... ...

    Abstract Background
    MeSH term(s) Heart ; Heart Rate ; Humans ; Liver Cirrhosis/diagnosis ; Severity of Illness Index
    Language English
    Publishing date 2021-06-17
    Publishing country England
    Document type Journal Article ; Meta-Analysis ; Systematic Review
    ZDB-ID 1149545-5
    ISSN 1361-6579 ; 0967-3334
    ISSN (online) 1361-6579
    ISSN 0967-3334
    DOI 10.1088/1361-6579/abf888
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

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    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
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  7. Article: Prognosis of COVID-19 in Patients with Liver and Kidney Diseases: An Early Systematic Review and Meta-Analysis

    Oyelade, Tope / Alqahtani, Jaber / Canciani, Gabriele

    Trop. Med. Infect. Dis.

    Abstract: The mortality and severity in COVID-19 is increased in patients with comorbidities. The aim of this study was to evaluate the severity and mortality in COVID-19 patients with underlying kidney and liver diseases. We retrieved data on the clinical ... ...

    Abstract The mortality and severity in COVID-19 is increased in patients with comorbidities. The aim of this study was to evaluate the severity and mortality in COVID-19 patients with underlying kidney and liver diseases. We retrieved data on the clinical features and primary composite end point of COVID-19 patients from Medline and Embase which had been released from inception by the April 16, 2020. The data on two comorbidities, liver diseases and chronic kidney disease, were pooled and statistically analysed to explain the associated severity and mortality rate. One hundred and forty-Two abstracts were screened, and 41 full articles were then read. In total, 22 studies including 5595 COVID-19 patients were included in this study with case fatality rate of 16%. The prevalence of liver diseases and chronic kidney disease (CKD) were 3% (95% CI; 2-3%) and 1% (95% CI; 1-2%), respectively. In patients with COVID-19 and underlying liver diseases, 57.33% (43/75) of cases were severe, with 17.65% mortality, while in CKD patients, 83.93% (47/56) of cases were severe and 53.33% (8/15) mortality was reported. This study found an increased risk of severity and mortality in COVID-19 patients with liver diseases or CKD. This will lead to better clinical management and inform the process of implementing more stringent preventative measures for this group of patients.
    Keywords covid19
    Publisher WHO
    Document type Article
    Note WHO #Covidence: #276862
    Database COVID19

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  8. Article ; Online: Prognosis of COVID-19 in Patients with Liver and Kidney Diseases

    Oyelade, Tope / Alqahtani, Jaber / Canciani, Gabriele

    Tropical medicine and infectious disease, 5(2):80

    An Early Systematic Review and Meta-Analysis

    2020  

    Abstract: The mortality and severity in COVID-19 is increased in patients with comorbidities. The aim of this study was to evaluate the severity and mortality in COVID-19 patients with underlying kidney and liver diseases. We retrieved data on the clinical ... ...

    Abstract The mortality and severity in COVID-19 is increased in patients with comorbidities. The aim of this study was to evaluate the severity and mortality in COVID-19 patients with underlying kidney and liver diseases. We retrieved data on the clinical features and primary composite end point of COVID-19 patients from Medline and Embase which had been released from inception by the April 16, 2020. The data on two comorbidities, liver diseases and chronic kidney disease, were pooled and statistically analysed to explain the associated severity and mortality rate. One hundred and forty-two abstracts were screened, and 41 full articles were then read. In total, 22 studies including 5595 COVID-19 patients were included in this study with case fatality rate of 16%. The prevalence of liver diseases and chronic kidney disease (CKD) were 3% (95% CI; 2–3%) and 1% (95% CI; 1–2%), respectively. In patients with COVID-19 and underlying liver diseases, 57.33% (43/75) of cases were severe, with 17.65% mortality, while in CKD patients, 83.93% (47/56) of cases were severe and 53.33% (8/15) mortality was reported. This study found an increased risk of severity and mortality in COVID-19 patients with liver diseases or CKD. This will lead to better clinical management and inform the process of implementing more stringent preventative measures for this group of patients.
    Keywords COVID-19 ; necrosis ; nonalcoholic steatohepatitis ; chronic kidney disease ; hepatitis B and C ; cirrhosis ; alcohol-related liver disease ; SARS-CoV-2 ; covid19
    Language English
    Publishing country de
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article: Heart Rate Turbulence Predicts Survival Independently From Severity of Liver Dysfunction in Patients With Cirrhosis.

    Oyelade, Tope / Canciani, Gabriele / Bottaro, Matteo / Zaccaria, Marta / Formentin, Chiara / Moore, Kevin / Montagnese, Sara / Mani, Ali R

    Frontiers in physiology

    2020  Volume 11, Page(s) 602456

    Abstract: Background: Reduced heart rate variability (HRV) is an independent predictor of mortality in patients with cirrhosis. However, conventional HRV indices can only be interpreted in individuals with normal sinus rhythm. In patients with recurrent premature ...

    Abstract Background: Reduced heart rate variability (HRV) is an independent predictor of mortality in patients with cirrhosis. However, conventional HRV indices can only be interpreted in individuals with normal sinus rhythm. In patients with recurrent premature ventricular complexes (PVCs), the predictive capacity of conventional HRV indices is compromised. Heart Rate Turbulence (HRT) represents the biphasic change of the heart rate after PVCs. This study was aimed to define whether HRT parameters could predict mortality in cirrhotic patients.
    Materials and methods: 24 h electrocardiogram recordings were collected from 40 cirrhotic patients. Turbulence Onset was calculated as HRT indices. The enrolled patients were followed up for 12 months after the recruitment in relation to survival and/or transplantation.
    Results: During the follow-up period, 21 patients (52.5%) survived, 12 patients (30%) died and 7 patients (17.5%) had liver transplantation. Turbulence Onset was found to be strongly linked with mortality on Cox regression (Hazard ratio = 1.351,
    Conclusion: This study provides further evidence of autonomic dysfunction in cirrhosis and suggests that HRT is reliable alternative to HRV in patients with PVCs.
    Language English
    Publishing date 2020-12-09
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2564217-0
    ISSN 1664-042X
    ISSN 1664-042X
    DOI 10.3389/fphys.2020.602456
    Database MEDical Literature Analysis and Retrieval System OnLINE

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