Long-term outcomes and patterns of relapse in patients with bilateral Wilms tumor or bilaterally predisposed unilateral Wilms tumor, a report from the COG AREN0534 study.

The objective of this study is to report the long-term timing and patterns of relapse for children enrolled in Children's Oncology Group AREN0534, a multicenter phase III clinical trial conducted from 2009 to 2015. Participants included children with bilateral Wilms tumor (BWT) or unilateral WT with genetic predisposition to develop BWT followed for up to 10 years. Smoothed hazard (risk) functions for event-free survival (EFS) were plotted so that the timing of events could be visualized, both overall and within pre-specified groups. Two hundred and twenty-two children (190 BWT and 32 unilateral WT with BWT predisposition) were followed for a median of 8.6 years. Fifty events were reported, of which 48 were relapse/progression. The overall 8-year EFS was 75% (95% confidence interval: 69%-83%). The highest risk for an EFS event was immediately after diagnosis with a declining rate over 2 years. A second peak of events was observed around 4 years after diagnosis, and a small number of events were reported until the end of the follow-up period. In subset analyses, later increases in risk were more commonly observed in patients with female sex, anaplastic histology, negative lymph nodes or margins, and favorable histology Wilms tumor patients with post-chemotherapy intermediate risk. Among relapses that occurred after 2 years, most were to the kidney. These patterns suggest that late events may be second primary tumors occurring more commonly in females, although more investigation is required. Clinicians may consider observation of patients with BWT beyond 4 years from diagnosis.

Bladder Embryonal Rhabdomyosarcoma Presenting in the Perinatal Period: Report of Two Consecutive Cases.

Bladder embryonal rhabdomyosarcomas (ERMS) are malignant soft tissue tumors that rarely present in the perinatal period. Herein, we report 2 cases that were diagnosed in the newborn period (one with concomitant posterior urethral valves [PUV]), managed with chemotherapy and surgical resection to minimize the need for radiation.

Redo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy-epispadias complex complications. Technical principles and pearls based on case scenarios.

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

Current surgical approaches to pediatric renal tumors.

Pediatric renal tumors are among the most common pediatric solid malignancies. Surgical resection is a key component in the multidisciplinary therapy for children with kidney tumors. Therefore, it is imperative that surgeons caring for children with renal tumors fully understand the current standards of care in order to provide appropriate surgical expertise within this multimodal framework. Fortunately, the last 60 years of international, multidisciplinary pediatric cancer cooperative group studies have enabled high rates of cure for these patients. This review will highlight the international surgical approaches to pediatric patients with kidney cancer to help surgeons understand the key differences and similarities between the European (International Society of Pediatric Oncology) and North American (Children's Oncology Group) recommendations.

Pediatric Urology Providers' Perceptions and Attitudes on Transitional Urologic Care in a Single-Payer Health Care System.

INTRODUCTION: Advances in health care have improved outcomes for pediatric patients with congenital neurourological conditions, highlighting the need for an effective transition from pediatric to adult care. This study investigates Canada's transitional urologic care within its single-payer health care system, focusing on the active members of Pediatric Urologists of Canada and their practices, perceptions, and attitudes toward transitional urologic care. METHODS: A survey was distributed to 35 Pediatric Urologists of Canada members from July 2023 to January 2024, which collected data on transitional care practices and available transitional urology clinics. It also focused on a service overview, covering aspects such as the responder's geographic and institutional affiliations, clinical practice characteristics involving transitional care, perceived challenges, and strategies for care enhancement. RESULTS: Nationwide engagement emphasized a commitment to improving transitional care, with a significant proportion of respondents (64%) having over 10 years of practice, reflecting substantial experience in addressing transitional care challenges. The survey identified 4 dedicated transitional care clinics, 3 of which are active, pointing to concerted efforts toward specialized service provision. The major challenges identified include the lack of adult transitional care initiatives and governmental support. Recommendations for improvement should focus on standardizing transition protocols and boosting patient education. The survey also underscored the necessity of protocolized care for spina bifida-neurogenic bladder and complex urogenital conditions. CONCLUSIONS: This study highlights the active efforts and existing challenges within Canada's transitional urologic care system, particularly emphasizing operational transitional care clinics as a crucial step forward in catering to transitioning patients' needs.

Bilateral Wilms tumor with anaplasia: A report from the Children's Oncology Group Study AREN0534.

INTRODUCTION: The purpose of this study is to examine the outcomes in children with anaplastic bilateral Wilms tumor (BWT) from study AREN0534 in order to define potential prognostic factors and areas to target in future clinical trials. METHODS: Demographic and clinical data from AREN0534 study patients with anaplasia (focal anaplasia [FA], or diffuse anaplasia [DA]) were compared. Event-free survival (EFS) and overall survival (OS) were reported using Kaplan-Meier estimation with 95% confidence bands, and differences in outcomes between FA and DA compared using log-rank tests. The impact of margin status was analyzed. RESULTS: Twenty-seven children who enrolled on AREN0534 had evidence of anaplasia (17 DA, 10 FA) in at least one kidney and were included in this analysis. Twenty-six (96%) had BWT. Nineteen percent had anaplastic histology in both kidneys (four of 17 DA, and one of 10 FA). Forty-six percent with BWT had bilateral nephron-sparing surgery (NSS); one child who went off protocol therapy, eventually required bilateral completion nephrectomies. Median follow-up for EFS and OS was 8.6 and 8.7 years from enrollment. Four- and 8-year EFS was 53% [95% confidence interval (CI): 34%-83%] for DA; 4-year EFS was 80% [95% CI: 59%-100%], and 8-year EFS 70% [95% CI: 47%-100%] for FA. Three out of 10 children with FA and eight out of 17 children with DA had events. EFS did not differ statistically by margin status (p = .79; HR = 0.88). Among the six children who died (five DA, one FA), all experienced prior relapse or progression within 18 months. CONCLUSION: Events in children with DA/FA in the setting of BWT occurred early. Caution should be taken about interpreting the impact of margin status outcomes in the context of contemporary multimodal therapy. Future targeted investigations in children with BWT and DA/FA are needed.

MR Imaging of Pediatric Neuroblastoma: Is Gadolinium Enhancement Necessary for Evaluation of Image-Defined-Risk Factors?

Background: Pre-treatment stratification and outcomes of neuroblastoma patients often depend on the assessment of image-defined risk factors (IDRFs) on MR Imaging, usually using Gadolinium-contrast materials which are cautioned in pediatrics. We aimed to address whether gadolinium contrast-enhanced sequences are necessary to identify the presence/absence of IDRFs. Methods: Patients with neuroblastoma with MR imaging were retrospectively identified from 2005 to 2021. Ninety confirmed IDRFs were evaluated in 23 patients. Corresponding MR studies were anonymized, randomized, and independently evaluated by 3 fellowship-trained pediatric radiologists. Each radiologist assessed the studies twice. At the first reading, all enhanced sequences were omitted, while in the second reading, the full study with enhanced sequences were included. Consensus reading was obtained among readers. Inter- and intra-rater agreements using Kappa statistics (κ) as well as the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of non-enhanced MR in assessing IDRFs with respect to enhanced MR were calculated. Results: There were substantial (ĸ: 0.64-0.73) intra-reader agreements, and moderate to substantial (ĸ: 0.57-0.62) inter-reader agreements among radiologists in identifying IDRFs using non-enhanced MR. Non-enhanced MR had a sensitivity of 87.8% (95% CI [79-94]), specificity of 93% (89-96), PPV of 82.3 (73-89), NPV of 95.4 (92-98), and accuracy of 91.6 (88-94) in identifying IDRFs. However, 5/23 patients (21.7%) had a change in staging with the inclusion of contrast sequences. Conclusion: Although contrast sequences have a role in IDRF assessment, the majority can be adequately assessed on MR without gadolinium-contrast enhancement. Validation in a larger cohort is an important next step.

Da Agenda ambiental à Vigilância Ambiental: um percurso histórico e biopolítico / From the Environmental Agenda to Environmental Surveillance a historical and biopolitical path

A presente tese se propõe a traçar uma genealogia da Vigilância Ambiental no Brasil. Partindo de um conjunto de chaves interpretativas da obra do filósofo Michel Foucault, congregamos um amplo leque de fontes documentais para compreender os primeiros sinais de uma incipiente medicina preventiva e sua agenda eminentemente ambiental, ainda em um Brasil pré-imperial, e observamos suas transformações em paridade com o desenvolvimento de nossa sociedade - buscando aí encontrar o mote biopolítico, dado que a construção da nação exigia medidas sanitárias que garantissem a saúde da população, ainda que não necessariamente por questões humanitárias. Acompanhamos a transição da agenda ambiental na saúde pública, que leva em conta os impactos do meio ambiente na existência humana para o movimento reverso, quando nos damos conta de que a humanidade está provocando danos possivelmente irreversíveis ao planeta - e como essa nova fase tem afetado nossa saúde. De maneira crítica e reflexiva, discutimos a formação e a dimensão biopolítica da Vigilância Ambiental na atualidade, dispondo de maiores e melhores recursos tecnológicos, mas atuando em uma governamentalidade neoliberal de redução de direitos - que afetam, inclusive, o direito à saúde

This thesis aims to trace the genealogy of Enviromental Surveillance in Brazil. From the standpoint a set of interpretative concepts of the philosopher Michel Foucault, we convey a vast array of documental sources to comprehend the first signs of an incipient preventive care and its respectable environmental agenda, still in a pre-imperial Brazil, and we observe its transformations parallel to the development of our society - aiming to find the biopolitical mote, given that the creation of the nation required sanitation methods that could guarantee the health of the population, even though not by strictly humanitarian reasons. We follow the transtition of the environmental agenda in our public health, which takes into consideration the impact of the environment in the human existence to the reverse movement, when we come to the realization that humanity has been causing possibly irreversible damage to the environment - and due to this also affecting our health. In a critic and self-reflecting way, we discuss the formation and the dimension of biopolitics of the Enviromental Surveillance in current day society, having access to a vast array of newer and better technological resources, but acting in a neoliberal governmentality of deprivation of rights, that also affect the right to health

Aspectos biopolíticos da Vigilância Ambiental / Biopolitical aspects of environmental surveillance

Resumo Este artigo analisa, em uma perspectiva biopolítica, alguns aspectos da temática ambiental na Vigilância em Saúde, mais especificamente na área de Vigilância Ambiental. Tal análise nos remete a noções de biopolítica e governamentalidade elaboradas por Michel Foucault que servem de chaves conceituais para a discussão. Para produzir a análise bibliográfica, utilizamos os principais documentos técnicos e legislações da área de Vigilância Ambiental, Vigilância Ambiental em Saúde e Vigilância em Saúde Ambiental, três denominações utilizadas para o objeto de estudo durante o seu desenvolvimento. Ao separar os documentos, efetuamos a comparação e crítica dos mesmos, atentos aos aspectos biopolíticos explícitos ou implícitos. A pesquisa é parte de uma tese de doutorado que construiu uma genealogia da área da Vigilância Ambiental. No trajeto histórico da formação da Vigilância Ambiental, aspectos biopolíticos foram se delineando na construção das ações de saúde da área. Primeiro, descrevemos brevemente a formação da área; posteriormente, realizamos uma leitura de alguns aspectos da biopolítica que parece estar implícita nas práticas de saúde que vêm caracterizando a Vigilância Ambiental, destacando uma visão das suas implicações na prática do serviço.

Abstract This article analyzes, from a biopolitical perspective, some aspects of environmental theme in Health Surveillance, more specifically in the area of Environmental Surveillance. This analysis brings us notions of biopolitics and governmentality elaborated by Michel Foucault that serve as conceptual keys for the discussion. To produce the bibliographic analysis, we used the main technical documents and legislation of the Environmental Surveillance, Environmental Health Surveillance and Environmental Health Surveillance, three denominations used for the object of study during its development. In separating the documents, we compare and critique them, paying attention to explicit or implicit biopolitical aspects. The research is part of a doctoral thesis that built a genealogy of Environmental Surveillance. In the historical course of the formation of Environmental Surveillance, biopolitical aspects were outlined in the construction of health actions in the area. First, we briefly describe the formation of the area; then we read some aspects of biopolitics that seem to be implicit in the health practices that have characterized Environmental Surveillance, highlighting a view of its implications for service practice.

Monocular surgery to correct large-angle esotropia: a 10-year follow-up study / Cirurgia monocular para correção de esotropias de grande ângulo: seguimento de 10 anos

ABSTRACT Purpose: To conduct a 10-year follow-up on the surgical correction of large-angle esotropia using monocular surgery, verifying surgical stability over time in both adults and children, and investigating the presence of consecutive exotropia among reviewed patients. Methods: The angles of deviation in primary position were measured using prism cover testing in patients with good vision in both eyes and the Krimsky method in those with severe amblyopia. Among the 46 patients who underwent surgery, 40 were evaluated 10 years post-surgery. Student's t-test and McNemar's test were used for statistical analyses. Results: No significant differences in the angles of deviation in primary position were found between measurements taken 6 months and 10 years post-surgery (p=0.922), as well as between children and adults (p=0.767). Among the 40 reviewed patients, only five presented with exotropia, all of which were small (the largest being XT 15Δ). Therefore, large consecutive exotropia over time was not observed. Conclusion: Our results suggest that monocular surgery to correct large-angle esotropia using large medial rectus recessions and broad lateral rectus resections was viable and safe in both adults and children over short and long terms.

RESUMO Objetivo: Conduzir um acompanhamento de 10 anos de correção cirúrgica de esotropias de grande ângulo com cirurgia monocular, verificando a estabilidade cirúrgica ao longo do tempo em adultos e crianças, investigando a presença de exotropias consecutivas entre os pacientes revisados. Métodos: Os ângulos de desvio em posição primária foram medidos usando teste de cobertura prismática em pacientes com boa visão em ambos os olhos e o método de Krimsky naqueles com ambliopia severa. Entre os 46 pacientes operados, 40 foram avaliados 10 anos após a cirurgia. O teste t-Student e o de McNemar foram usados para análises estatísticas. Resultados: Não foram encontradas diferenças significativas nos ângulos de desvio em posição primária entre as medidas realizadas 6 meses e 10 anos pós-operatórios (p=0,922, assim como entre crianças e adultos (p=0,767). Entre os 40 pacientes revisados, apenas cinco apresentaram exotropias, todas pequenas (sendo o maior XT 15Δ). Portanto grandes exotropias consecutivas ao longo do tempo não foram observadas. Conclusão: Nossos resultados sugerem que a cirurgia monocular para corrigir a esotropias de grande ângulo usando amplos recuos do reto medial e grandes ressecções do reto lateral foi viável e segura tanto em adultos quanto em crianças em curto como a longo prazo.

The relationship between low birth weight and exposure to inhalable particulate matter / Relação entre baixo peso ao nascer e exposição ao material particulado inalável / Relación entre el bajo peso al nacer y la exposición a partículas inhalables

Atmospheric pollution is a global public health problem. The adverse effects of air pollution are strongly associated with respiratory and cardiovascular diseases and, to a lesser extent, with adverse pregnancy outcomes. This study analyzes the relationship between exposure to PM10 and low birth weight in the city of Santo André, São Paulo State, Brazil. We included babies born to mothers resident in Santo André between 2000 and 2006. Data on daily PM10 levels was obtained from the São Paulo State Environmental Agency. We performed descriptive analysis and logistic regressions. The prevalence rate of low birth weight was 5.9%. There was a dose-response relationship between PM10 concentrations and low birth weight. Exposure to the highest quartile of PM10 (37,50µg/m³) in the third trimester of pregnancy increased the risk of low birth weight by 26% (OR: 1.26; 95%CI: 1.14-1.40) when compared to the first quartile. The same effect was observed in the remaining trimesters. This effect was observed for ambient particle concentrations that met the current air quality standards.

Em todo o mundo a poluição atmosférica é um problema de saúde pública. Os efeitos adversos relacionados aos poluentes atmosféricos estão fortemente associados com doenças respiratórias e cardiovasculares e, em menor grau, com os resultados adversos da gravidez. O objetivo do trabalho foi avaliar a relação entre PM10 e baixo peso ao nascer no Município de Santo André, São Paulo, Brasil. Foram incluídos no estudo recém-nascidos de mães residentes em Santo André (2000-2006). A Agência Ambiental do Estado de São Paulo forneceu dados diários de PM10. Realizou-se análise descritiva e de regressão logística. A prevalência de baixo peso ao nascer foi de 5,9%. Observou-se uma relação dose-resposta entre as concentrações de PM10 e baixo peso ao nascer. As concentrações de PM10 no quartil mais alto (37,50µg/m³) no terceiro trimestre gestacional aumentaram o risco de baixo peso ao nascer em 26% (OR = 1,26; IC95%: 1,14-1,40) quando comparadas com o primeiro quartil. O mesmo efeito foi observado nos demais trimestres. Esse efeito foi verificado mesmo com as concentrações de partículas dentro dos padrões de qualidade do ar.

La contaminación del aire en todo el mundo es un problema de salud pública. Los efectos adversos relacionados con los contaminantes del aire están fuertemente asociados con enfermedades respiratorias y cardiovasculares, pero en menor medida con los resultados adversos del embarazo. En este estudió se evaluó la relación entre PM10 y bajo peso al nacer en el municipio de Santo André, São Paulo, Brasil. Se incluyeron en el estudio los recién nacidos de madres residentes en Santo André (2000-2006). La Agencia ambiental del Estado de São Paulo informó de los índices diarios de PM10. Además, se realizó un análisis descriptivo y por regresión logística. La prevalencia de bajo peso al nacer fue de un 5,9%. Hubo una relación dosis-respuesta entre las concentraciones de PM10 y bajo peso al nacer. Las concentraciones de PM10 en el cuartil más alto (37,50µg/m³) en el tercer trimestre aumentaban el riesgo gestacional de bajo peso al nacer en un 26% (OR = 1,26; IC95%: 1,14-1,40), en comparación con el primer cuartil. El mismo efecto se observó en los trimestres restantes. Este efecto fue observado incluso en partículas con concentraciones que están dentro de los estándares del aire.

Carcinoma folicular de tireóide: estudo retrospectivo / Follicular thyroid carcinoma: retrospective study

OBJETIVOS: Abordar o diagnóstico, tratamento e evolução do carcinoma folicular da tireóide. MÉTODOS: Análise retrospectiva dos dados de 38 pacientes submetidos à tireoidectomia por carcinoma folicular puro, num período de 10 anos no HC-FMUSP. O tempo médio de seguimento foi de três anos e três meses. Nove pacientes eram do sexo masculino (23,7 por cento) e 29 do sexo feminino (76,3 por cento), com idades entre 19 e 87 anos (média=49,5). RESULTADOS: Em 17 (58,6 por cento) dos doentes, observou-se nódulo único à ultra-sonografia, e 23 (79,3 por cento) tinham nódulos frios à cintilografia. Sintomatologia esteve presente em 33 pacientes (86,8 por cento). A punção aspirativa por agulha fina (PAAF), realizada em 27 pacientes, revelou padrão folicular em 24 (88,9 por cento), carcinoma papilífero em 2 (7,4 por cento) e bócio em 1 (3,7 por cento). Tireoidectomia total foi o tratamento final em 34 pacientes e esvaziamento cervical foi realizado em três casos. Apenas 5 (13,1 por cento) obtiveram confirmação diagnóstica ao exame de congelação intra-operatória. Houve 2 (5,2 por cento) óbitos pela doença e 5 (13,1 por cento) pacientes apresentam-se vivos com doença. O aumento da tireoglobulina (TG) correlacionou-se com o aparecimento de metástase em 100 por cento dos casos. CONCLUSÕES: Concluímos que pacientes com carcinoma folicular de tireóide geralmente apresentam-se com nódulo único ou predominante ao primeiro exame, cuja PAAF é de padrão folicular. O exame de congelação raramente confirma o diagnóstico. Em nosso serviço, o tratamento de escolha é a tireoidectomia total, permitindo um seguimento mais adequado e confiável do paciente e prevenindo o crescimento de lesões subclínicas no lobo contralateral. A evolução geralmente é favorável.

BACKGROUND: Unlike papillary carcinoma, there are limited data regarding diagnosis, treatment and follow-up of patients with follicular thyroid carcinoma. METHODS: We retrospectively analyzed data on 38 patients submitted to thyroidectomy for exclusive follicular carcinoma over a 10 years period. Mean follow-up was 39 months (114 months the longest). Nine patients were males (23,7 percent) and 29 females (76,3 percent). Mean age was 49.5 (19 to 87 years). RESULTS: Thirty-three (86,8 percent) patients presented at least one symptom such as dyspnea, dysphagia or hoarseness. Seventeen (58,6 percent) patients had a solitary nodule shown by ultrasonography and 23 (79,3 percent) had "cold" nodules by scintigraphy. Twenty-seven patients underwent were submitted to fine-needle aspiration biopsy (FNA), which revealed a follicular neoplasm (benign or malignant) in 24 (88,9 percent) patients, goiter in 1(3,7 percent) and papillary carcinoma in 2 cases (7,4 percent). Total thyroidectomy was the final treatment in 34 patients and neck dissection operation was required in 3 cases. Only 13,1 percent of the patients were diagnosed as having follicular carcinoma by intraoperative frozen section. Two patients died from the tumor and 5 patients remain alive with the tumor all of them with distant metastasis. Elevation of serum levels of thyroglobulin correlated with metastasis in 100 percent of those cases. CONCLUSIONS: We conclude that follicular thyroid carcinoma usually presents as a solitary nodule diagnosed as a follicular neoplasm by FNA biopsy. Intra-operatory frozen section rarely confirms the diagnosis. O treatment of choice for thyroid follicular cancer total thyroidectomy followed by complementary therapy (radioiodine therapy) if necessary. We advocate total thyroidectomy because it is assumited with a better follow-up and avoids growth of subclinical lesions on the opposite lobe. Outcome is good in the majority of cases.