Hospital de Pediatría S.A.M.I.C. "Prof. Dr. Juan P. Garrahan", commonly known as Hospital Garrahan, is a public hospital in Buenos Aires, Argentina, located in the neighborhood of Parque Patricios. Since it opened on 25 August 1987, it has become the leading public, free and high-complexity pediatric hospital in the country.It is a highly specialized medical center, with over 3000 employees, state-of-the-art technology and progressive cares. It is jointly funded by the federal government of Argentina (80%) and by the Buenos Aires City Government (20%).
muerte súbita a mediano y a largo plazos. Las técnicas propuestas para ofrecer flujo sistémico a la coronaria izquierda (CI) son diversas, incluido el bypass, pero sin duda el tratamiento óptimo es la transferencia del botón coronariano a la arteria aorta, es decir, el reimplante coronario.
Background and aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability. This current study seeks to stratify and assign a score to each IDRF based on its impact on surgical risk. Methods: This collaborative study entailed retrospective collection of patient data from NB operations conducted between 2016 and 2020, together with the computation of a Surgical Complexity Index (SCI) for every patient. The SCI values obtained were correlated with preoperatively identified IDRFs. Mann-Whitney statistical test was employed to assign a relevance score to the association between individual IDRFs and SCI. Results: 14 centers contributed 427 neuroblastoma index cases meeting study inclusion criteria. 303 patients had 1 or more IDRFs. The presence and number of IDRFs in all patients significantly correlated with the SCI value (p < 0.0001). The most frequently encountered IDRF was renal pedicle involvement. There was a significant association observed between clusters of abdominal IDRFs and the occurrence of post-operative complications (p < 0.05), while a weak association link was found with intra-operative complications. A relevance score for individual IDRFs allowed their stratification based on surgical risk. The most relevant IDRFs were extension within two anatomical body compartments, infiltration of the hepato-pancreatic block, encasement of the superior mesenteric artery and coeliac axis, and tumor compressing the trachea. Conclusions: This current study has facilitated the assignment of a relevance score to each IDRF, correlating it with surgical risks. Considering this stratification of surgical risk alongside oncologic risk as defined by the neuroblastoma treatment patient risk grouping should facilitate a more precise definition of surgical objectives and the optimal conditions favoring gross tumor resection. (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.
Vagus nerve stimulation (VNS) is a neuromodulatory treatment for refractory epilepsy, involving electrical stimulation of the vagus nerve via a subcutaneously implanted pulse generator. We present a step-by-step description of the VNS implantation technique, incorporating tips and tricks from an epilepsy surgeon with over 20 years of experience (MB). Proper patient selection, meticulous surgical technique, and comprehensive postoperative care, supported by educational resources such as surgical technique articles, videos, and simulators, are crucial for minimizing complications and optimizing patient outcomes.
Abstract Background Lower respiratory tract infection (LRTI) is a common cause of admission and antibiotic (ATB) overuse in children. Adequate use of diagnostic methods and ATB prescription in intensive care units (ICU) remain a challenge for antimicrobial stewardship programs. Aims to evaluate the impact of a multilevel intervention to improve diagnostic testing and ATB use in children with LRTI admitted to ICU. Methods Quasi-experimental, before-after study. Patients admitted to ICU from April to June 2023 (Pre Intervention period: PreI) and July to October 2023 (Post Intervention: PostI) with community acquired LRTI were included. Intervention consisted in educational activities, daily review and feedback of ATB prescription, development and dissemination of an institutional Guideline of ATB use in LTRI, including a Clinical Score to distinguish viral from bacterial pneumonia. Results 95 patients were included. Median age was 11 months (IQR 4-19).Fifty eight (61%) had any underlying condition, including: previous bronchiolitis N:16 (17%), Asthma N:8(8%) and Neurological Disorders N:7 (7%). Clinical diagnosis upon ICU admission was: bronchiolitis (N:42, 44%), asthmatic crisis (N: 33, 35%) and Pneumonia (N: 11, 12%). A viral etiology was documented in 76 patients (80%). The most common was RSV (N: 52, 55%) followed by Rhinovirus (N: 19, 20%). Viral coinfection was detected in 15 patients (16%). Sixty-three patients were admitted during PreI and 32 during PostI. Children admitted to ICU during PreI were more frequently healthy (PreI: 48% vs PostIn 22%, p 0.015), with bronchiolitis as admission diagnosis (PreI 56% vs PostI 23%, p 0.002) and confirmed respiratory infection (PreI 87% vs PostI 66%, p 0.01). In PreI more Blood cultures (PreI 61% vs PostI 51%, p 0.06) and tracheal aspirate cultures (PreI 48% vs PostI 22%, p 0.04) were performed. Empirical ATB were started on admission in 39 patients (62%) during PreI, and in 13 patients (41%) in PostI (p: 0.04). Adequate ATB prescription increased from 14% in the PreI to 37% in the PostI (p 0.01). Patients in PostI had shorter hospital length of stay (8 vs 14 days, p 0.02) Conclusion A multilevel intervention in children with LRTI on admission to ICU improved the use of blood and tracheal aspirate cultures and ATB prescription. Length of stay decreased in PostI. Disclosures All Authors: No reported disclosures
The HECW2 gene, essential for neurodevelopment, plays a critical role in maintaining cellular homeostasis and regulating key pathways in the nervous system. Deleterious variants in the HECW2 gene have been associated with developmental delay, intellectual disability, hypotonia and epilepsy, as well as dysmorphic features. We present the case of an infant with a novel variant in HECW2 with an unusual clinical presentation and a progressive disease course, showing three successive electroclinical patterns, consisting of burst suppression characteristic of early infantile developmental and epileptic encephalopathy, subcontinuous myoclonic seizures and infantile epileptic spasms syndrome without hypsarrhythmia, occurring over a short period of time. This case expands the clinical spectrum associated with this gene and highlights the intrafamilial phenotypic variability. It also underscores the importance of personalized therapeutic strategies, as demonstrated by the use of perampanel in this patient. These findings emphasize the need to include HECW2 as a possible aetiology in the diagnostic evaluation of developmental and epileptic encephalopathies.
