15 research outputs found
The Clinical Impact of Methotrexate-Induced Stroke-Like Neurotoxicity in Paediatric Departments: An Italian Multi-Centre Case-Series
IntroductionStroke-like syndrome (SLS) is a rare subacute neurological complication of intrathecal or high-dose (>= 500 mg) Methotrexate (MTX) administration. Its clinical features, evoking acute cerebral ischaemia with fluctuating course symptoms and a possible spontaneous resolution, have elicited interest among the scientific community. However, many issues are still open on the underlying pathogenesis, clinical, and therapeutic management and long-term outcome. Materials and MethodsWe retrospectively analyzed clinical, radiological and laboratory records of all patients diagnosed with SLS between 2011 and 2021 at 4 National referral centers for Pediatric Onco-Hematology. Patients with a latency period that was longer than 3 weeks between the last MTX administration of MTX and SLS onset were excluded from the analysis, as were those with unclear etiologies. We assessed symptom severity using a dedicated arbitrary scoring system. Eleven patients were included in the study. ResultsThe underlying disease was acute lymphoblastic leukemia type B in 10/11 patients, while fibroblastic osteosarcoma was present in a single subject. The median age at diagnosis was 11 years (range 4-34), and 64% of the patients were women. Symptoms occurred after a mean of 9.45 days (+/- 0.75) since the last MTX administration and lasted between 1 and 96 h. Clinical features included hemiplegia and/or cranial nerves palsy, paraesthesia, movement or speech disorders, and seizure. All patients underwent neuroimaging studies (CT and/or MRI) and EEG. The scoring system revealed an average of 4.9 points (+/- 2.3), with a median of 5 points (maximum 20 points). We detected a linear correlation between the severity of the disease and age in male patients. ConclusionsSLS is a rare, well-characterized complication of MTX administration. Despite the small sample, we have been able to confirm some of the previous findings in literature. We also identified a linear correlation between age and severity of the disease, which could improve the future clinical management
Acute dacryocystitis with giant lacrimal abscess: a case report
We report a case of a 4-year-old girl with acute dacryocystitis complicated with giant lacrimal abscess who underwent open dacryocystectomy as resolutive surgery
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Co-designing an intervention to strengthen COVID-19 vaccine uptake in Congolese migrants in the UK (LISOLO MALAMU) : a participatory qualitative study protocol
INTRODUCTION: Migrants positively contribute to host societies yet experience barriers to health and vaccination services and systems and are considered to be an underimmunised group in many European countries. The COVID-19 pandemic has highlighted stark inequities in vaccine uptake, with migrants facing access and informational barriers and lower vaccine confidence. A key challenge, therefore, is developing tailored vaccination interventions, services and systems which account for and respond to the unique drivers of vaccine uptake in different migrant populations. Participatory research approaches, which meaningfully involve communities in co-constructing knowledge and solutions, have generated considerable interest in recent years for those tasked with designing and delivering public health interventions. How such approaches can be used to strengthen initiatives for COVID-19 and routine vaccination merits greater consideration. METHODS AND ANALYSIS: LISOLO MALAMU ('Good Talk') is a community-based participatory research study which uses qualitative and coproduction methodologies to involve adult Congolese migrants in developing a tailored intervention to increase COVID-19 vaccine uptake. Led by a community-academic coalition, the study will involve (1) semistructured in-depth interviews with adult Congolese migrants (born in Democratic Republic of Congo, >18 years), (2) interviews with professional stakeholders and (3) codesign workshops with adult Congolese migrants. Qualitative data will be analysed collaboratively using reflexive thematic analysis, and behaviour change theory will be used in parallel to support the coproduction of interventions and make recommendations across socioecological levels. The study will run from approximately November 2021 to November 2022. ETHICS AND DISSEMINATION: Ethics approval was granted by the St George's University Research Ethics Committee (REC reference: 2021.0128). Study findings will be disseminated to a range of local, national and international audiences, and a community celebration event will be held to show impact and recognise contributions. Recommendations for implementation and evaluation of prototyped interventions will be made
Preliminary Study on β3-Adrenoreceptor as Predictor Marker of Relapse in Ewing Sarcoma Patients
Ewing sarcoma (EWS) is a paediatric aggressive malignant tumour of bones and soft tissues. Multidisciplinary chemotherapies, surgical resection, and radiation represent the only strategies counteracting the disease, however spreading and relapse of disease still remain a clinical issue. Circulating tumour cells (CTCs) are an important feature of EWS but the prognostic significance has not been, yet, clarified. CTCs have been found both in patients with localized disease and in those who recur or metastasize. The identification of markers that can detect recurrences and metastasis remains an important challenge for research. Unfortunately, even most of patients with localized cancer relapsed and the reason has not yet been fully understood. In this clinical study on EWS patients, we evaluated the expression of CD99 antigen and beta-3 adrenergic receptor (β3-AR) on CTCs and bioptic derived cells by flow cytometry. The preliminary data revealed a higher β3-AR expression on cells derived from metastatic or relapsed patients, suggesting a role for the β3-AR as a possible predictive maker of disease recurrence in both patients with metastatic and localized disease
Activated Phosphoinositide 3-Kinase Delta Syndrome 1: Clinical and Immunological Data from an Italian Cohort of Patients
Activated phosphoinositide 3-kinase delta syndrome 1 (APDS-1) is a recently described inborn error of immunity caused by monoallelic gain-of-function mutations in the PIK3CD gene. We reviewed for the first time medical records and laboratory data of eight Italian APDS-1 patients. Recurrent sinopulmonary infections were the most common clinical feature at onset of disease. Seven patients presented lymphoproliferative disease, at onset or during follow-up, one of which resembled hemophagocytic lymphohistiocytosis (HLH). Genetic analysis of the PIK3CD gene revealed three novel mutations: functional testing confirmed their activating nature. In the remaining patients, the previously reported variants p.E1021K (n = 4) and p.E525A (n = 1) were identified. Six patients were started on immunoglobulin replacement treatment (IgRT). One patient successfully underwent hematopoietic stem cell transplantation (HSCT), with good chimerism and no GVHD at 21 months post-HSCT. APDS-1 is a combined immune deficiency with a wide variety of clinical manifestations and a complex immunological presentation. Besides IgRT, specific therapies targeting the PI3K delta pathway will most likely become a valid aid for the amelioration of patients' clinical management and their quality of life