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- Physical rehabilitation in Brazilian pediatric intensive care units: a multicenter point prevalence study(2023) REDIVO, Juliana; KANNAN, Harini; SOUZA, Andreia Aparecida Freitas; COLLETI JUNIOR, José; KUDCHADKAR, Sapna Ravi; HORIGOSHI, Nelson Kazunobu; COSTA, Graziela de Araújo; CASTILHO, Taísa Roberta Ramos de; PERON, Paula Peres Domingues; SCARANTO, Walter Perez; MEDEIROS, Daniela Nasu Monteiro; MATSUMOTO, Toshio; ALMEIDA, Carlos Gustavo de; OLIVEIRA, Felipe Rezende Caino de; BRANDÃO, Marcelo Barciela; LIMA-SETTA, Fernanda; PRATA-BARBOSA, Arnaldo; XAVIER, Glaciele Nascimento; ANDRADE, Livia Barbosa de; AGUIAR, Agda Ultra de; COUTINHO, Marcos Paulo Galdino; CASTRO, Roberta Esteves Viera de; LANDY, Glazia André; BALANIUC, Suzana Lopes Bonfim; YAMAGUCHI, Ricardo SilveiraABSTRACT Objective: To determine the prevalence and factors associated with the physical rehabilitation of critically ill children in Brazilian pediatric intensive care units. Methods: A 2-day, cross-sectional, multicenter point prevalence study comprising 27 pediatric intensive care units (out of 738) was conducted in Brazil in April and June 2019. This Brazilian study was part of a large multinational study called Prevalence of Acute Rehabilitation for Kids in the PICU (PARK-PICU). The primary outcome was the prevalence of mobility provided by physical therapy or occupational therapy. Clinical data on patient mobility, potential mobility safety events, and mobilization barriers were prospectively collected in patients admitted for ≥ 72 hours. Results: Children under the age of 3 years comprised 68% of the patient population. The prevalence of therapist-provided mobility was 74%, or 277 out of the 375 patient-days. Out-of-bed mobility was most positively associated with family presence (adjusted odds ratios 3.31;95%CI 1.70 - 6.43) and most negatively associated with arterial lines (adjusted odds ratios 0.16; 95%CI 0.05 - 0.57). Barriers to mobilization were reported on 27% of patient-days, the most common being lack of physician order (n = 18). Potential safety events occurred in 3% of all mobilization events. Conclusion: Therapist-provided mobility in Brazilian pediatric intensive care units is frequent. Family presence was high and positively associated with out-of-bed mobility. The presence of physiotherapists 24 hours a day in Brazilian pediatric intensive care units may have a substantial impact on the mobilization of critically ill children.
- Performance and labor conditions of physiotherapists in Brazilian intensive care units during the COVID-19 pandemic. What did we learn?(2023) RESENDE, Andressa Sabrina de Oliveira; BACCI, Suzi Laine Longo dos Santos; PAULA, Ítalo Ribeiro; PEREIRA, Leandro Alves; JOHNSTON, Cíntia; LUSZCZYNSKI, Valéria Cabral Neves; AZEVEDO, Vívian Mara Gonçalves de OliveiraABSTRACT Objective: To describe the role of physiotherapists in assisting patients suspected to have or diagnosed with COVID-19 hospitalized in intensive care units in Brazil regarding technical training, working time, care practice, labor conditions and remuneration. Methods: An analytical cross-sectional survey was carried out through an electronic questionnaire distributed to physiotherapists who worked in the care of patients with COVID-19 in Brazilian intensive care units. Results: A total of 657 questionnaires were completed by physiotherapists from the five regions of the country, with 85.3% working in adult, 5.4% in neonatal, 5.3% in pediatric and 3.8% in mixed intensive care units (pediatric and neonatal). In intensive care units with a physiotherapists available 24 hours/day, physiotherapists worked more frequently (90.6%) in the assembly, titration, and monitoring of noninvasive ventilation (p = 0.001). Most intensive care units with 12-hour/day physiotherapists (25.8%) did not apply any protocol compared to intensive care units with 18-hour/day physiotherapy (9.9%) versus 24 hours/day (10.2%) (p = 0.032). Most of the respondents (51.0%) received remuneration 2 or 3 times the minimum wage, and only 25.1% received an additional payment for working with patients suspected to have or diagnosed with COVID-19; 85.7% of them did not experience a lack of personal protective equipment. Conclusion: Intensive care units with 24-hour/day physiotherapists had higher percentages of protocols and noninvasive ventilation for patients with COVID-19. The use of specific resources varied between the types of intensive care units and hospitals and in relation to the physiotherapists’ labor conditions. This study showed that most professionals had little experience in intensive care and low wages.
- OCCULT AND SEMI-OCCULT CONSTIPATION IN CHILDREN WITH MONOSYMPTOMATIC OR NON MONOSYMPTOMATIC ENURESIS(2023) MAFFEI, Helga Verena L; VIDOLIN, Eliana; REIS, Joceara Neves dos; FREITAS, Marcia de; CABRAL, Beatriz Helena; TRIGO-ROCHA, FlavioABSTRACT Background: Functional constipation and enuresis frequently coexist. Constipation treatment often results in resolution or improvement of the enuresis. However, besides the classical presentation, patients can present with occult constipation (OC) diagnosed in complementary evaluation; in addition, semi-occult constipation (SOC) can be detected by means of a detailed questionnaire. Objective: To quantify OC and SOC frequency in children with monosymptomatic or non monosymptomatic enuresis (MNE or NMNE). Methods: Otherwise healthy children/adolescents, with enuresis refractory to behavioral therapy and denying constipation after simple questions, answered a structured bowel habit questionnaire and were submitted to a plain abdominal radiological exam. Constipation was classified considering the Boston diagnostic criteria (to allow diagnosis at initial stages), and fecal loading in the X-ray quantified ≥10 by the Barr score. Children with constipation received a standardized treatment (except 26 “pilot” children). Results: Out of 81 children, 80 aged 9.34±2.07 years, 52.5% male, were diagnosed with constipation: 30 OC, 50 SOC; 63.75% had MNE, 36.25% NMNE (six NMNE without behavioral therapy). Demographic data and the Barr score were similar for OC and SOC, but SOC children experienced significantly more constipation complications (retentive fecal incontinence and/or recurrent abdominal pain). Not showing the Bristol Stool Scale (BSS) to 24 “pilot” children, or absence of constipation symptoms accompanying BSS predominantly type 3, in 13 children, did not significantly impact the detection of constipation by the Barr score. Children identifying BSS 3 or ≤2 had similar results. Twenty-eight children, with adequate follow-up after treatment, improved or recovered from constipation at 44 of their 52 follow-up visits. Conclusion: In patients with MNE or NMNE refractory to behavioral therapy, and who initially denied constipation after simple questions, a detailed questionnaire based on the Boston diagnostic criteria detected SOC in 61.7%, and the radiological Barr score revealed fecal loading (OC) in 37.0% of them.
- Exploring the Brazilian pediatric palliative care network: a quantitative analysis of a survey data(2023) FERREIRA, Esther Angelica Luiz; VALETE, Cristina Ortiz Sobrinho; BARBOSA, Silvia Maria de Macedo; COSTA, Graziela de Araujo; MOLINARI, Poliana Cristina Carmona; IGLESIAS, Simone Brasil de Oliveira; CASTRO, Ana Cristina Pugliese deObjective: The aim of this study was to identify the characteristics of services in Brazil that compound the Brazilian Pediatric Palliative Care (PPC) Network. Methods: An online survey was conducted among representatives from PPC services. A total of 90 services from Brazil completed the online survey and answered a questionnaire about the services characterization, health professionals working in PPC, access to opioid prescription and education, and research in PPC.Results: In total, 80 services (88.9%) were created after 2010, 52 (57.9%) were in the southeast region, 56 (62.2%) were in public hospitals, 63 (70%) had up to 100 beds, and 57 (63.3%) were at the tertiary level. Notably, 88 (97.8%) had a physician in the team and 68 (75.5%) dedicated part-time to PPC. Also, 33 (36.7%) revealed concern with the care of health professionals and 36 (40%) reported difficulty or no access to opioid prescription. Research studies were reported to be conducted in 29 (32.2%) services.Conclusions: This mapping points out to a concentration of PPC services in the southeast region, with part-time professional dedication, and the need to improve professionals' care. Difficulty in opioid access was reported. It is necessary to extend PPC participation to other Brazilian regions, increase time dedicated to PPC, improve professionals' care and improve access to opioid prescription.
- POLR1A variants underlie phenotypic heterogeneity in craniofacial, neural, and cardiac anomalies(2023) SMALLWOOD, Kelly; WATT, Kristin E. N.; IDE, Satoru; BALTRUNAITE, Kristina; BRUNSWICK, Chad; INSKEEP, Katherine; CAPANNARI, Corrine; ADAM, Margaret P.; BEGTRUP, Amber; BERTOLA, Debora R.; DEMMER, Laurie; DEMO, Erin; DEVINSKY, Orrin; GALLAGHER, Emily R.; SACOTO, Maria J. Guillen; JECH, Robert; KEREN, Boris; KUSSMANN, Jennifer; LADDA, Roger; LANSDON, Lisa A.; LUNKE, Sebastian; MARDY, Anne; MCWALTERS, Kirsty; PERSON, Richard; RAITI, Laura; SAITOH, Noriko; SAUNDERS, Carol J.; SCHNUR, Rhonda; SKORVANEK, Matej; SELL, Susan L.; SLAVOTINEK, Anne; SULLIVAN, Bonnie R.; STARK, Zornitza; SYMONDS, Joseph D.; WENGER, Tara; WEBER, Sacha; WHALEN, Sandra; WHITE, Susan M.; WINKELMANN, Juliane; ZECH, Michael; ZEIDLER, Shimriet; MAESHIMA, Kazuhiro; STOTTMANN, Rolf W.; TRAINOR, Paul A.; WEAVER, K. NicoleHeterozygous pathogenic variants in POLR1A, which encodes the largest subunit of RNA Polymerase I, were previously identified as the cause of acrofacial dysostosis, Cincinnati-type. The predominant phenotypes observed in the cohort of 3 individuals were craniofacial anomalies reminiscent of Treacher Collins syndrome. We subsequently identified 17 additional individuals with 12 unique heterozygous variants in POLR1A and observed numerous additional phenotypes including neurodevelopmental abnormalities and structural cardiac defects, in combination with highly prevalent craniofacial anomalies and variable limb defects. To understand the pathogenesis of this pleiotropy, we modeled an allelic series of POLR1A variants in vitro and in vivo. In vitro assessments demonstrate variable effects of indi-vidual pathogenic variants on ribosomal RNA synthesis and nucleolar morphology, which supports the possibility of variant-specific phenotypic effects in affected individuals. To further explore variant-specific effects in vivo, we used CRISPR-Cas9 gene editing to reca-pitulate two human variants in mice. Additionally, spatiotemporal requirements for Polr1a in developmental lineages contributing to congenital anomalies in affected individuals were examined via conditional mutagenesis in neural crest cells (face and heart), the second heart field (cardiac outflow tract and right ventricle), and forebrain precursors in mice. Consistent with its ubiquitous role in the essential function of ribosome biogenesis, we observed that loss of Polr1a in any of these lineages causes cell-autonomous apoptosis resulting in embryonic malformations. Altogether, our work greatly expands the phenotype of human POLR1A-related disorders and demonstrates variant-specific effects that provide insights into the underlying pathogenesis of ribosomopathies.
- Effect of manual hyperinflation with versus without positive end-expiratory pressure on dynamic compliance in pediatric patients following congenital heart surgery: A randomized controlled trial(2023) GUIMARAES, Jessica Camara; SILVA, Thalis Henrique da; ARAGON, Davi Casale; JOHNSTON, Cintia; GASTALDI, Ada Clarice; CARLOTTI, Ana P. C. P.Background: We aimed to compare the effect of manual hyperinflation with versus without positive end-expiratory pressure (PEEP) on dynamic compliance of the respiratory system in pediatric patients undergoing congenital heart surgery; to assess the safety of the technique in this population.Methods: This was a randomized controlled trial conducted at the pediatric intensive care unit (PICU) of a tertiary-care hospital. Patients admitted to the PICU following cardiac surgery and receiving postoperative mechanical ventilation were randomized to the experimental or control group. Patients in the experimental group (n = 14) underwent manual hyperinflation with a PEEP valve set at 5 cm H2O, once daily, during the first 48 hours after surgery. Patients allocated to the control group (n = 16) underwent manual hyperinflation without PEEP, at the same time points. Lung mechanics was assessed before (T0) and 5 minutes (T5) after manual hyperinflation. The primary endpoint was dynamic compliance. Secondary outcomes included oxygen saturation index, duration of mechanical ventilation, length of stay, 28-day mortality and safety.Results: Demographic and clinical characteristics were comparable in both groups. There was no significant difference in dynamic compliance between times in each group (Day 1: (mean) 0.78 vs 0.81 and 0.70 vs 0.77; Day 2: 0.85 vs 0.78 and 0.67 vs 0.68 mL/kg/cm H2O, in experimental and control groups, respectively; P > .05). Mean deltas of dynamic compliance were not significantly different between groups. The proportion of patients extubated <72 hours after surgery was similar in experimental and control groups (43% vs 50%, respectively; P = .73). Oxygen saturation index, length of stay, and 28-day mortality were not significantly different between groups. None of the patients had hemodynamic instability.Conclusions: Manual hyperinflation was safe and well tolerated in pediatric patients following surgery for congenital heart disease. No significant change in dynamic compliance of the respiratory system or in oxygenation was observed with the use of manual hyperinflation with or without PEEP in this population.
- Small and large bowel anatomy is associated with enteral autonomy in infants with short bowel syndrome: A retrospective cohort study(2024) SANDY, Natascha S.; ROBERTS, Amin J.; WALES, Paul W.; TOMA, Ricardo K.; BELZA, Christina; DOGRA, Harween; EVANS, Helen M.; GATTINI, Daniela; HIND, Jonathan; MERCER, David; POVONDRA, Jill M.; TURNER, Justine; YAP, Jason; WONG, Theodoric; AVITZUR, YaronBackground: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA.Methods: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015. The cohort was stratified based on the achievement of EA. Statistical testing was completed using t-test, chi-square, Cox proportional hazards regression model, and Kaplan-Meier analysis.Results: EA was achieved in 229 patients. In the multivariable analysis, the percentage of residual LB (hazard ratio [HR] = 1.02; 95% CI = 1.01-1.02) and SB (HR = 1.01; 95% CI = 1.01-1.02) length, presence of the ileocecal valve (HR = 2.02; 95% CI=1.41-2.88), and not coming from a high-volume transplantation center (HR = 2.42; 95% CI = 1.68-3.49) were positively associated with EA, whereas a negative association was seen with the presence of stoma at the time when shortest remnant was documented (HR = 0.72; 95% CI = 0.52-1.00). EA achievement was significantly different between the anatomical subgroups (log-rank test P < 0.001) with an EA rate of 80.4% in infants with >= 50% SB and LB (median time 209 days); 62.5% with >= 50% SB and <50% LB (397 days); 58.3% with <50% SB and >= 50% LB (1192 days), and 25.9% with <50% SB and LB. Necrotizing enterocolitis (NEC) was not associated with a better achievement of EA (NEC vs other etiologies: log-rank test P = 0.33).Conclusions: Overall, 62% of infants with IF secondary to SBS achieved EA over a mean time of follow-up of 2.3 years. A colon length of >50% can compensate for the loss of small bowel (<50%) and account for similar EA rates as those in children with residual SB > 50%.
- Malnutrition and Nutrition Support in Latin American PICUs: The Nutrition in PICU (NutriPIC) Study(2023) CAMPOS-MINO, Santiago; FIGUEIREDO-DELGADO, Artur; ZARATE, Patricia; ZAMBERLAN, Patricia; MUNOZ-BENAVIDES, Eliana; COSS-BU, Jorge A.OBJECTIVES: To characterize the nutritional status of children admitted to Latin American (LA) PICUs and to describe the adequacy of nutrition support in reference to contemporary international recommendations. DESIGN: The Nutrition in PICU (NutriPIC) study was a combined point-prevalence study of malnutrition carried out on 1 day in 2021 (Monday 8 November) and a retrospective cohort study of adequacy of nutritional support in the week preceding. SETTING: Four-one PICUs in 13 LA countries. PATIENTS: Patients already admitted to the PICU of 1 month to 18 years old on the study day were included in the point-prevalence study. For the retrospective arm, we included patients receiving nutritional support on the study day and with a PICU length of stay (LOS) greater than or equal to 72 hours. Exclusion criteria were being a neonate, conditions that precluded accurate anthropometric measurements, and PICU LOS greater than 14 days. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 316 patients screened, 5 did not meet age criteria. There were 191 of 311 patients who were included in the point-prevalence study and underwent anthropometric evaluation. Underweight and length for age less than -2 z scores were present in 42 of 88 children (47.7%) and 41 of 88 children (46.6%) less than 24 months old, and 14 of 103 (13.6%) and (23/103) 22.3% of 103 children greater than or equal to 24 months, respectively. Evidence of obesity (body mass index > 2 z score) was present in 7 of 88 children (5.7%) less than 24 months old and 13 of 103 children (12.6%) greater than or equal to 24 months. In the 115 of 311 patients meeting criteria for the retrospective arm, a total of 98 patients reported complete nutritional data. The 7-day median (interquartile range) adequacy for delivered versus recommended enteral energy and protein requirement was 114% (75, 154) and 99% (60, 133), respectively. CONCLUSIONS: The NutriPIC study found that in 2021 malnutrition was highly prevalent especially in PICU admissions of less than 24 months old. Retrospectively, the 7-day median nutritional support appears to meet both energy and protein requirements.
- Real-world data of Brazilian adults with X-linked hypophosphatemia (XLH) treated with burosumab and comparison with other worldwide cohorts(2024) VAISBICH, Maria Helena; CILLO, Antonio Cesar Paulillo de; SILVA, Barbara Campolina C.; DALVA, Catarina Brasil; CARVALHO, Erico Higino de; ALMEIDA, Juliana M. C. M. de; MARQUES, Larissa L. M.; RIBEIRO, Marcia; SILVA, Mauro Borghi M. da; MEDEIROS, Paula Frassinetti V. de; MENDES, Pedro HenriqueBackground: Disease-related variants in PHEX cause XLH by an increase of fibroblast growth factor 23 (FGF23) circulating levels, resulting in hypophosphatemia and 1,25(OH)(2) vitamin D deficiency. XLH manifests in early life with rickets and persists in adulthood with osseous and extraosseous manifestations. Conventional therapy (oral phosphate and calcitriol) improves some symptoms, but evidence show that it is not completely effective, and it can lead to nephrocalcinosis (NC) and hyperparathyroidism (HPT). Burosumab (anti-FGF23 antibody) has shown to be effective and safety in the clinical trials. Methods: The current real-world collaborative study evaluated genetic, clinical and laboratory data of XLH Brazilian adult patients treated with burosumab. Results: Nineteen unrelated patients were studied. Patients reported pain, limb deformities and claudication, before burosumab initiation. 78% of them were previously treated with conventional therapy. The severity of the disease was moderate to severe (15 patients with score >5). At the baseline, 3 patients presented NC (16.7%) and 12 HPT (63%). After 16 +/- 8.4 months under burosumab, we observed a significant: increase in stature (p = 0.02), in serum phosphate from 1.90 +/- 0.43 to 2.67 +/- 0.52 mg/dL (p = 0.02); in TmP/GFR from 1.30 +/- 0.46 to 2.27 +/- 0.64 mg/dL (p = 0.0001), in 1,25 (OH)(2) D from 50.5 +/- 23.3 to 71.1 +/- 19.1 pg/mL (p = 0.03), and a decrease in iPTH from 86.8 +/- 37.4 pg/mL to 66.5 +/- 31.1 (p = 0.002). Nineteen variants were found (10 novel). HPT tended to develop in patients with truncated PHEX variants (p = 0.06). Conclusions: This study confirms the efficacy and safety of burosumab on XLH adult patients observed in clinical trials. Additionally, we observed a decrease in iPTH levels in patients with moderate to severe HPT at the baseline.
- Body composition in pediatric patients(2023) ZAMBERLAN, Patricia; MAZZONI, Beatriz P.; BONFIM, Maria A. C.; VIEIRA, Rafaela R.; TUMAS, Rosana; DELGADO, Artur F.Undernutrition is highly prevalent in children who are critically ill and is associated with increased morbidity and mortality, including a higher risk of infection due to transitory immunological disorders, inadequate wound healing, reduced gut function, longer dependency on mechanical ventilation, and longer hospital stays compared with eutrophic children who are critically ill. Nutrition care studies have proposed that early interventions targeting nutrition assessment can prevent or minimize the complications of undernutrition. Stress promotes an acute inflammatory response mediated by cytokines, resulting in increased basal metabolism and nitrogen excretion and leading to muscle loss and changes in body composition. Therefore, the inclusion of body composition assessment is important in the evaluation of these patients because, in addition to the nutrition aspect, body composition seems to predict clinical prognosis. Several techniques can be used to assess body composition, such as arm measurements, calf circumference, grip strength, bioelectrical impedance analysis, and imaging examinations, including computed tomography and dual-energy x-ray absorptiometry. This review of available evidence suggests that arm measurements seem to be well-established in assessing body composition in children who are critically ill, and that bioelectrical impedance analysis with phase angle, handgrip strength, calf circumference and ultrasound seem to be promising in this evaluation. However, further robust studies based on scientific evidence are necessary.
- Physician Perceptions of and Barriers to Pediatric Palliative Care for Children With Cancer in Brazil(2023) MCNEIL, Michael J.; GODFREY, Alyssa; LOGGETTO, Patricia; OLIVEIRA JUNIOR, Andre de; JOB, Godwin; BOLDRINI, Erica; MURRA, Glaucia Regina Costa; AYUB, Dileiny Antunes Geronutti; LIMA, Allan Francisco Oliveira de; ALMEIDA, Aline Esmeraldo Andrade de; GARCIA, Julia Lopes; AMARAL, Anna Beatriz Costa Neves do; FONSECA, Ieda Cristina Cunha Ferreira e; FRIEDRICH, Paola; METZGER, Monika L.; DEVIDAS, Meenakshi; AGULNIK, Asya; BAKER, Justin N.PURPOSE Early integration of pediatric palliative care (PPC) for children with cancer is critical to improving the quality of life of both the patient and family. Understanding physician perceptions of palliative care and perceived barriers to early integration is necessary to develop PPC in Brazil. METHODS The Assessing Doctors' Attitudes on Palliative Treatment survey was modified for use in Brazil. The survey was open from January 2022 to June 2022 and distributed to physicians of all specialties from participating institutions who treat children with cancer. Statistical analysis was complemented by qualitative analysis of open-ended responses. RESULTS A total of 272 respondents participated. Most respondents reported access to PPC experts for consultation (77.2%) and 34.5% indicated previous palliative care training. Physician knowledge of PPC was generally aligned with WHO guidance (median alignment, 93.0%; range, 80.5%-98.2%). However, about half (53.3%) felt comfortable addressing physical needs of patients receiving PPC, 35.3% addressing emotional needs, 25.8% addressing spiritual needs, and 33.5% addressing grief and bereavement needs. Most respondents (65.4%) felt palliative care should be involved from diagnosis, but only 10.3% stated that this occurred in their setting. The most important barriers identified were physician discomfort (89.0%), limited physician knowledge (88.6%), and lack of homebased services (83.8%). CONCLUSION Despite a strong understanding of the role of palliative care, physicians in Brazil reported low confidence delivering PPC to children with cancer. Additionally, physicians generally believed that PPC should be integrated earlier in the disease trajectory of children with cancer. This work will direct educational and capacity building initiatives to ensure greater access to high-quality PPC for children with cancer in Brazil to address patient and family suffering.
- Data-driven, cross-disciplinary collaboration: lessons learned at the largest academic health center in Latin America during the COVID-19 pandemic(2024) RITTO, Ana Paula; ARAUJO, Adriana Ladeira de; CARVALHO, Carlos Roberto Ribeiro de; SOUZA, Heraldo Possolo De; FAVARETTO, Patricia Manga e Silva; SABOYA, Vivian Renata Boldrim; GARCIA, Michelle Louvaes; KULIKOWSKI, Leslie Domenici; KALLAS, Esper Georges; PEREIRA, Antonio Jose Rodrigues; COBELLO JUNIOR, Vilson; SILVA, Katia Regina; ABDALLA, Eidi Raquel Franco; SEGURADO, Aluisio Augusto Cotrim; SABINO, Ester Cerdeira; RIBEIRO JUNIOR, Ulysses; FRANCISCO, Rossana Pulcineli Vieira; MIETHKE-MORAIS, Anna; LEVIN, Anna Sara Shafferman; SAWAMURA, Marcio Valente Yamada; FERREIRA, Juliana Carvalho; SILVA, Clovis Artur; MAUAD, Thais; GOUVEIA, Nelson da Cruz; LETAIF, Leila Suemi Harima; BEGO, Marco Antonio; BATTISTELLA, Linamara Rizzo; DUARTE, Alberto Jose da Silva; SEELAENDER, Marilia Cerqueira Leite; MARCHINI, Julio; FORLENZA, Orestes Vicente; ROCHA, Vanderson Geraldo; MENDES-CORREA, Maria Cassia; COSTA, Silvia Figueiredo; CERRI, Giovanni Guido; BONFA, Eloisa Silva Dutra de Oliveira; CHAMMAS, Roger; BARROS FILHO, Tarcisio Eloy Pessoa de; BUSATTO FILHO, GeraldoIntroduction The COVID-19 pandemic has prompted global research efforts to reduce infection impact, highlighting the potential of cross-disciplinary collaboration to enhance research quality and efficiency.Methods At the FMUSP-HC academic health system, we implemented innovative flow management routines for collecting, organizing and analyzing demographic data, COVID-related data and biological materials from over 4,500 patients with confirmed SARS-CoV-2 infection hospitalized from 2020 to 2022. This strategy was mainly planned in three areas: organizing a database with data from the hospitalizations; setting-up a multidisciplinary taskforce to conduct follow-up assessments after discharge; and organizing a biobank. Additionally, a COVID-19 curated collection was created within the institutional digital library of academic papers to map the research output.Results Over the course of the experience, the possible benefits and challenges of this type of research support approach were identified and discussed, leading to a set of recommended strategies to enhance collaboration within the research institution. Demographic and clinical data from COVID-19 hospitalizations were compiled in a database including adults and a minority of children and adolescents with laboratory confirmed COVID-19, covering 2020-2022, with approximately 350 fields per patient. To date, this database has been used in 16 published studies. Additionally, we assessed 700 adults 6 to 11 months after hospitalization through comprehensive, multidisciplinary in-person evaluations; this database, comprising around 2000 fields per subject, was used in 15 publications. Furthermore, thousands of blood samples collected during the acute phase and follow-up assessments remain stored for future investigations. To date, more than 3,700 aliquots have been used in ongoing research investigating various aspects of COVID-19. Lastly, the mapping of the overall research output revealed that between 2020 and 2022 our academic system produced 1,394 scientific articles on COVID-19.Discussion Research is a crucial component of an effective epidemic response, and the preparation process should include a well-defined plan for organizing and sharing resources. The initiatives described in the present paper were successful in our aim to foster large-scale research in our institution. Although a single model may not be appropriate for all contexts, cross-disciplinary collaboration and open data sharing should make health research systems more efficient to generate the best evidence.
- Glioneuronal and Neuronal Tumors: Who? When? Where? An Update Based on the 2021 World Health Organization Classification(2023) AYRES, A. S.; BANDEIRA, G. A.; FERRACIOLLI, S. F.; TAKAHASHI, J. T.; MORENO, R. A.; GODOY, L. F. de Souza; CASAL, Y. R.; LIMA, L. G. C. A. de; FRASSETO, F. P.; LUCATO, L. T.Neuronal and glioneuronal tumors usually have a benign course and may have typical imaging characteristics, allowing their diagnosis based on MR imaging findings. The most common lesions are dysembryoplastic neuroepithelial tumors and gangliogliomas, which have typical imaging characteristics. The fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System, recently published in 2021, places greater emphasis on molecular markers to classify tumors of the CNS, leading to extensive changes in the classification of tumors, including neuronal and glioneuronal tumors. The 2021 revision included 3 new tumors types: multinodular and vacuolating neuronal tumor, diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (a provisional type), and myxoid glioneuronal tumor. Following these recent changes in the World Health Organization classification, we aimed to review the main imaging features of these lesions in relation to their histopathologic and molecular features. Learning Objectives: To list the neuronal and glioneuronal tumors; recognize the main imaging findings and histologic characteristics of neuronal and glioneuronal tumors; know the typical location of each neuronal and glioneuronal tumor; and become familiar with the main molecular alterations of neuronal and glioneuronal tumors to better understand their behavior
- WT1 Pathogenic Variants are Associated with a Broad Spectrum of Differences in Sex Development Phenotypes and Heterogeneous Progression of Renal Disease(2022) FERRARI, M. T. M.; WATANABE, A.; SILVA, T. E. Da; GOMES, N. L.; BATISTA, R. L.; NISHI, M. Y.; PAULA, L. C. P. De; COSTA, E. C.; COSTA, E. M. F.; CUKIER, P.; ONUCHIC, L. F.; MENDONCA, B. B.; DOMENICE, S.Wilms' tumor suppressor gene 1 (WT1) plays an essential role in urogenital and kidney development. Heterozygous germline pathogenic allelic variants of WT1 have been classically associated with Denys-Drash syndrome (DDS) and Frasier syndrome (FS). Usually, exonic pathogenic missense variants in the zinc finger region are the cause of DDS, whereas pathogenic variants affecting the canonic donor lysine-threonine-serine splice site in intron 9 cause FS. Phenotypic overlap between WT1 disorders has been frequently observed. New WT1 variant-associated phenotypes, such as 46,XX testicular/ovarian-testicular disorders of sex development (DSD) and primary ovarian insufficiency, have been reported. In this report, we describe the phenotypes and genotypes of 7 Brazilian patients with pathogenic WT1 variants. The molecular study involved Sanger sequencing and massively parallel targeted sequencing using a DSD-associated gene panel. Six patients (5 with a 46,XY karyotype and 1 with a 46,XX karyotype) were initially evaluated for atypical genitalia, and a 46,XY patient with normal female genitalia sought medical attention for primary amenorrhea. Germ cell tumors were identified in 2 patients, both with variants affecting alternative splicing of WT1 between exons 9 and 10. Two pathogenic missense WT1 variants were identified in two 46,XY individuals with Wilms' tumors; both patients were <1 year of age at the time of diagnosis. A novel WT1 variant, c.1453_1456 (p.Arg485Glyfs∗14), was identified in a 46,XX patient with testicular DSD. Nephrotic proteinuria was diagnosed in all patients, including 3 who underwent renal transplantation after progressing to end-stage kidney disease. The expanding phenotypic spectrum associated with WT1 variants in XY and XX individuals confirms their pivotal role in gonadal and renal development as well as in tumorigenesis, emphasizing the clinical implications of these variants in genetic diagnosis.
- Avoid re-interpreting fluid bolus recommendations for low-income settings(2023) RANJIT, Suchitra; KISSOON, Niranjan; ARGENT, Andrew; INWALD, David; VENTURA, Andrea Maria Cordeiro; JABORINSKY, Roberto; SANKAR, Jhuma; SOUZA, Daniela Carla de; NATRAJ, Rajeswari; OLIVEIRA, Claudio Flauzino De; SAMRANSAMRUAJKIT, Rujipat; JAYASHREE, Muralidharan; SCHLAPBACH, Luregn J.
- Imaging of pediatric skull lytic lesions: A review(2024) SANTOS, Mariana; CUNHA, Bruno; ABREU, Vasco; FERRACIOLLI, Suely; GODOY, Luis; MURAKOSHI, Rodrigo; AMARAL, Lazaro Luis Faria; CONCEICAO, CarlaSkull lesions in pediatric population are common findings on imaging and sometimes with heterogeneous manifestations, constituting a diagnostic challenge. Some lesions can be misinterpreted for their aggressiveness, as with larger lesions eroding cortical bone, containing soft tissue components, leading to excessive and, in some cases, invasive inappropriate etiological investigation. In this review, we present multiple several conditions that may present as skull lesions or pseudolesions, organized by groups (anatomic variants, congenital and development disorders, traumatic injuries, vascular issues, infectious conditions, and tumoral processes). Anatomic variants are common imaging findings that must be recognized by the neuroradiologist. Congenital malformations are rare conditions, such as aplasia cutis congenita and sinus pericranii, usually seen at earlier ages, the majority of which are benign findings. In case of trauma, cephalohematoma, growing skull fractures, and posttraumatic lytic lesions should be considered. Osteomyelitis tends to be locally aggressive and may mimic malignancy, in which cases, the clinical history can be the key to diagnosis. Vascular (sickle cell disease) and tumoral (aneurismal bone cyst, eosinophilic granuloma, metastases) lesions are relatively rare lesions but should be considered in the differential diagnosis, in the presence of certain imaging findings. The main difficulty is the differentiation between the benign and malignant nature; therefore, the main objective of this pictorial essay is to review the main skull lytic lesions found in pediatric age, describing the main findings in different imaging modalities (CT and MRI), allowing the neuroradiologist greater confidence in establishing the differential diagnosis, through a systematic and simple characterization of the lesions.
- Requirements for Brazilian Outpatient Centers of Excellence in Hidradenitis Suppurativa: Consensus(2023) BELLEI, Ericles Andrei; SHIROZAKI, Miriam Emi Makinodan; PUGLIA, Ana Lia Pradella; CARVALHO, Andre Vicente Esteves de; RIQUENA, Barbara; VALLILO, Camilla Carlini; BARROS, Danilo de; TAMASHIRO, Erika Yumi; CINTRA, Gabriela; DUARTE, Gleison Vieira; RIVITTI-MACHADO-MACHADO, Maria Cecilia da Matta; MAGALHAES, Renata Ferreira; NASCIMENTO, Ricardo Figueiredo do; TUNALA, Roberto; SILVA, Roberto da; CESAR, Wagner Guimaraes Galvao; THIES, Felipe GaruttiBackground: Hidradenitis suppurativa (HS) is a chronic skin condition. Its complexity and impact on patients highlight the need for multidisciplinary care that can address the physical, psychological, and social aspects. Centers of excellence can ideally provide the necessary infrastructure, resources, and expertise to effectively treat HS. However, there are still no consolidated models of centers of excellence in HS, and establishing their foundations is an intricate research challenge. Purposely, design and co-creation as innovation techniques are helpful approaches to this type of research. Methods: In this study, we conducted a co-creation with consensus among HS specialists to propose the criteria and requirements to establish outpatient centers of excellence of HS in Brazil. We followed a linear process with mixed methods in 6 stages. Results: The process resulted in 10 categories for establishing outpatient centers, including their respective requirements, rationale, and classification. The categories include onboarding and welcoming; infrastructure and procedures; infusion therapy; flows and referrals; staffing; disease management; metrics during diagnosis; metrics during treatment; awareness and advocacy; research and education. Discussion: The idealized outpatient centers can play a role in the complete multidisciplinary treatment for HS and advancing the science of healthcare services by providing a focus for research, training, and translation of findings into practice.
- Remote Monitoring for Seizures During Therapeutic Hypothermia in Neonates With Hypoxic-Ischemic Encephalopathy(2023) VARIANE, Gabriel Fernando Todeschi; DAHLEN, Alex; PIETROBOM, Rafaela Fabri Rodrigues; RODRIGUES, Daniela Pereira; MAGALHAES, Mauricio; MIMICA, Marcelo Jenne; LLAGUNO, Nathalie Salles; LEANDRO, Danieli Mayumi Kimura; GIROTTO, Paula Natale; SAMPAIO, Leticia Brito; MEURS, Krisa Page VanIMPORTANCE Neonates with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia (TH) frequently experience seizures, which are associated with adverse outcomes. Efforts to rapidly identify seizures and reduce seizure burden may positively change neurologic and neurodevelopmental outcomes. OBJECTIVE To describe the onset, treatment, and evolution of seizures in a large cohort of newborns with HIE during TH assisted by a telehealth model and remote neuromonitoring approach. DESIGN, SETTING, AND PARTICIPANTS This was a prospective, observational, multicenter cohort study performed between July 2017 and December 2021 in 32 hospitals in Brazil. Participants were newborns with HIE meeting eligibility criteria and receiving TH. Data were analyzed from November 2022 to April 2023. EXPOSURE Infants with HIE receiving TH were remotely monitored with 3-channel amplitude integrated electroencephalography (aEEG) including raw tracing and video imaging, and bedside clinicians received assistance from trained neonatologists and neurologists. MAIN OUTCOMES AND MEASURES Data on modified Sarnat examination, presence, timing and seizure type, aEEG background activity, sleep-wake cycling, and antiepileptic drugs used were collected. Descriptive statistical analysis was used with independent t test, chi 2, Mann-Whitney test, and post hoc analyses applied for associations. RESULTS A total of 872 cooled newborns were enrolled; the median (IQR) gestational age was 39 (38-40) weeks, 518 (59.4%) were male, and 59 (6.8%) were classified as having mild encephalopathy by modified Sarnat examination, 504 (57.8%) as moderate, and 180 (20.6%) as severe. Electrographic seizures were identified in 296 newborns (33.9%), being only electrographic in 213 (71.9%) and clinical followed by electroclinical uncoupling in 50 (16.9%). Early abnormal background activity had a significant association with seizures. Infants with flat trace had the highest rate of seizures (58 infants [68.2%]) and the greatest association with the incidence of seizures (odds ratio [OR], 12.90; 95% CI, 7.57-22.22) compared with continuous normal voltage. The absence of sleep-wake cycling was also associated with a higher occurrence of seizures (OR, 2.22; 95% CI, 1.67-2.96). Seizure onset was most frequent between 6 and 24 hours of life (181 infants [61.1%]); however, seizure occurred in 34 infants (11.5%) during rewarming. A single antiepileptic drug controlled seizures in 192 infants (64.9%). The first line antiepileptic drug was phenobarbital in 294 (99.3%).
CONCLUSIONS AND RELEVANCE In this cohort study of newborns with HIE treated with TH, electrographic seizure activity occurred in 296 infants (33.9%) and was predominantly electrographic. Seizure control was obtained with a single antiepileptic drug in 192 infants (64.9%). These findings suggest neonatal neurocritical care can be delivered at remote limited resource hospitals due to innovations in technology and telehealth. - Establishment of oral microbiome in very low birth weight infants during the first weeks of life and the impact of oral diet implementation(2023) VANZELE, Pedro A. R.; SPARVOLI, Luiz Gustavo; CAMARGO, Patricia P. de; TRAGANTE, Carla R.; BEOZZO, Glenda P. N. S.; KREBS, Vera L. J.; CORTEZ, Ramon V.; TADDEI, Carla R.Very low birth weight (VLBW) infants, mostly preterm, have many barriers to feeding directly from the mother's breast, and need to be fed alternatively. Feeding is a major influencer in oral microbial colonization, and this colonization in early life is crucial for the promotion of human health. Therefore, this research aimed to observe the establishment of oral microbiome in VLBW infants during their first month of life through hospitalization, and to verify the impact caused by the implementation of oral diet on the colonization of these newborns. We included 23 newborns followed during hospitalization and analyzed saliva samples collected weekly, using 16S rRNA gene sequencing. We observed a significant decrease in richness and diversity and an increase in dominance over time (q-value < 0.05). The oral microbiome is highly dynamic during the first weeks of life, and beta diversity suggests a microbial succession in early life. The introduction of oral diet does not change the community structure, but affects the abundance, especially of Streptococcus. Our results indicate that although time is related to significant changes in the oral microbial profile, oral feeding benefits genera that will remain colonizers throughout the host's life.
- A randomized, double-blind, non-inferiority trial comparing the immunogenicity and safety of two seasonal inactivated influenza vaccines in adults(2023) VANNI, Tazio; SALOMAO, Maria da Grada; VISCONDI, Juliana Yukari Kodaira; BRAGA, Patricia Emilia; SILVA, Anderson da; PIORELLI, Roberta de Oliveira; SANTOS, Joane do Prado; GATTAS, Vera Lucia; LUCCHESI, Maria Beatriz Bastos; OLIVEIRA, Mayra Martho Moura de; KOIKE, Marcelo Eiji; CAMPOS, Lucia M. A.; COELHO, Eduardo B.; WECKX, Lily Yin; LARA, Amanda Nazareth; PAIVA, Terezinha M.; TIMENETSKY, Maria do Carmo S. T.; PRECIOSO, Alexander RobertoBackground: To enhance the production and availability of influenza vaccines in different regions of the world is paramount to mitigate the global burden of this disease. Instituto Butantan developed and man-ufactured an embryonated egg-based inactivated split-virion trivalent seasonal influenza vaccine as part of a technology transfer partnership with Sanofi Pasteur.Methods: This is a phase IV, randomized, double-blind, active-controlled, multicenter clinical trial includ-ing adults 18-60 and > 60 years recruited during the 2019 southern hemisphere influenza season. Subjects were randomized 1:1 to receive either the Sanofi Pasteur Trivalent Seasonal Influenza Vaccine (SP-TIV) or Instituto Butantan Trivalent Seasonal Influenza Vaccine (IB-TIV). Hemagglutinin inhibition antibody titers were assessed pre-vaccination and 21 days post-vaccination.Results: 624 participants were randomized and vaccinated. In both intention-to-treat and per-protocol analysis, non-inferiority of the SP-TIV versus IB-TIV was demonstrated for the three influenza strains. In the per-protocol analysis, the SP-GMT/IB-GMT ratios for H1N1, H3N2, and B were 0.9 (95%CI, 0.7- 1.1), 1.2 (95%CI, 1.0-1.4), and 1.1 (95%CI, 0.9-1.3), respectively. Across vaccination groups, the most com-mon adverse reactions (AR) were limited to the injection-site, including pain and tenderness. The major-ity of the ARs were graded 1 and/or 2 and lasted less than one day. No serious adverse reaction was observed.Conclusion: This study demonstrated the non-inferiority of the immunogenicity of a single-dose of Instituto Butantan versus a single dose of the Sanofi Pasteur Seasonal Trivalent Influenza Vaccine in adults. Both vaccines were well tolerated and presented similar safety profiles.(c) 2023 Elsevier Ltd. All rights reserved.