Hyperbaric Medicine in Pediatrics — reality of a Portuguese reference center / Medicina Hiperbárica em Pediatria — realidade de um centro de referência português

ABSTRACT Objective: To identify and characterize the population of Pediatric patients referred to our hyperbaric oxygen therapy center. Methods: Retrospective and observational study, including pediatric patients treated with hyperbaric oxygen therapy, from 2006 to 2021, at the hyperbaric medicine reference center in the north of Portugal. Variables of interest were extracted from electronic medical records. Results: Our study included 134 patients. The most frequent reasons for referral were carbon monoxide poisoning (n=59) and sudden sensorineural hearing loss (n=41). In 75 cases (56%), treatment was initiated in an urgent context. Symptom presentation at Emergency Department varied among patients, the most frequent being headache and nausea/vomiting. Concerning carbon monoxide poisoning, the most common sources were water heater, fireplace/brazier, and boiler. Regarding adverse effects, it was identified one case of intoxication by oxygen and four cases of middle ear barotrauma. Conclusions: The most frequent cause for referral was carbon monoxide poisoning. All patients evolved favorably, with few side effects being reported, emphasizing the safety of this therapy. While most pediatricians may not be aware of the potential benefits arising with hyperbaric oxygen therapy, it is of upmost importance to promote them, so that this technique is increasingly implemented.

RESUMO Objetivo: Identificar e caracterizar a população de casos pediátricos encaminhados para o nosso centro de oxigenoterapia hiperbárica. Métodos: Estudo retrospetivo e observacional, que incluiu doentes pediátricos tratados com oxigenoterapia hiperbárica, de 2006 a 2021, no centro de referência de medicina hiperbárica do norte de Portugal. As variáveis de interesse foram extraídas dos processos clínicos eletrônicos. Resultados: O nosso estudo incluiu 134 casos. Os motivos de encaminhamento mais frequentes foram intoxicação por monóxido de carbono (n=59) e surdez súbita neurossensorial (n=41). Em 75 casos (56%) o tratamento foi iniciado em contexto de urgência. Os sintomas de apresentação à admissão variaram entre os diferentes casos, sendo os mais frequentes cefaleias e náuseas/vômitos. No que diz respeito à intoxicação por monóxido de carbono, as fontes mais comuns foram o aquecedor, lareira/braseiro e caldeira. Com relação aos efeitos adversos, foram identificados um caso de intoxicação por oxigênio e quatro casos de barotrauma do ouvido médio. Conclusões: A causa mais frequente de encaminhamento foi a intoxicação por monóxido de carbono. Todos os pacientes evoluíram favoravelmente e foram registrados poucos efeitos adversos, o que enfatiza a segurança desta terapia. Uma vez que a maioria dos pediatras pode não estar informada sobre os potenciais benefícios da oxigenoterapia hiperbárica, é de extrema importância promovê-los para que esta técnica seja cada vez mais implementada.

Caracterización de pacientes con enfermedades asociadas a inmunosupresión con infecciones por Cryptosporidium spp. atendidos en un hospital pediátrico referencial de Argentina, 2018-2023 / Description of the characteristics of patients with diseases associated with immunosuppression and Cryptosporidium spp. infection seen at a referral children's hospital in Argentina, 2018­2023

Cryptosporidium spp. es un protozoario productor de diarrea. Los pacientes inmunocomprometidos pueden desarrollar formas clínicas graves y persistentes. Se describen las características de pacientes con enfermedad de base asociada a inmunosupresión (EAI) con infección por Cryptosporidium spp. (IC) atendidos en un hospital pediátrico referencial de Argentina entre los años 2018 y 2023. Se analizaron datos demográficos, EAI, características de la diarrea y coinfecciones. Se incluyeron 30 pacientes con EAI e IC. La mayoría registró trasplante de órgano sólido, neoplasia hematológica e inmunodeficiencia primaria. Dieciocho presentaron diarrea persistente al momento del diagnóstico. Seis pacientes registraron coinfecciones. Se debe considerar la criptosporidiosis en el diagnóstico diferencial de enfermedad diarreica aguda o persistente en niños con distintos tipos de EAI, como el trasplante de órgano sólido, neoplasias hematológicas e inmunodeficiencias primarias.

Cryptosporidium spp. is a diarrhea-causing protozoan. Immunocompromised patients may develop severe and persistent clinical forms. Here we describe the characteristics of patients with an underlying disease associated with immunosuppression (DAI) and Cryptosporidium spp. infection seen at a referral children's hospital in Argentina between 2018 and 2023. Demographic data, DAI, diarrhea characteristics, and co-infections were analyzed. A total of 30 patients with DAI and cryptosporidiosis were included. Most of them had undergone a solid organ transplant, had a hematologic neoplasm, or primary immunodeficiency. Persistent diarrhea was observed in 18 patients at the time of diagnosis. Co-infections were recorded in 6 patients. Cryptosporidiosis should be considered in the differential diagnosis of acute or persistent diarrhea in children with different types of DAI, such as solid organ transplant, hematologic neoplasms, and primary immunodeficiencies.

Agenesia de la vesícula biliar: una serie de casos multicéntrica y revisión de la literatura / Agenesis of the gallbladder: A multicenter case series and review of the bibliography

La agenesia de la vesícula biliar es una entidad rara en pediatría con una evolución normalmente silente, y representa un desafío diagnóstico para el médico que enfrenta estos casos por primera vez. Algunos pacientes pueden, sin embargo, presentar síntomas que simulan otras patologías del árbol biliar, y muchos de ellos son operados ante esta sospecha. Sin embargo, el diagnóstico oportuno de esta entidad permite llevar a cabo un tratamiento médico que muchas veces es suficiente para resolver el problema del paciente. Si bien es una condición benigna, los pacientes suelen presentar otras malformaciones asociadas que son más graves en naturaleza y que deben investigarse activamente para poder derivarlos a los especialistas de manera oportuna. Presentamos nuestra experiencia en el diagnóstico y tratamiento de estos pacientes, así como una breve revisión de la literatura. Esperamos que sea de utilidad para el médico que encuentre un caso similar.

Gallbladder agenesis is a rare condition in pediatrics that is usually asymptomatic and represents a diagnostic challenge for physicians seeing these cases for the first time. Some patients may, however, present with symptoms that mimic other diseases of the bile ducts, and many of them undergo surgery due to such suspicion. Still, a timely diagnosis of gallbladder agenesis allows for medical treatment that is often sufficient to resolve the patient's problem. Although it is a benign condition, patients often present with other associated, more serious malformations and should be actively studied for a timely referral to other specialists. Here we describe our experience with the diagnosis and treatment of these patients and a brief review of the bibliography. We hope it will be helpful for physicians facing similar cases.

Linfohistiocitosis hemofagocítica en niño con virus de inmunodeficiencia humana en contexto de coinfecciones virales oportunistas / Hemophagocytic lymphohistiocytosis in a child with human immunodeficiency virus in the setting of opportunistic viral co-infections

La linfohistiocitosis hemofagocítica (LHH) es una entidad rara que se caracteriza por un estado hiperinflamatorio secundario a la activación desregulada del sistema inmune con compromiso multisistémico. Puede ser primaria o hereditaria, o estar desencadenada por diversas enfermedades. La mortalidad sin tratamiento oportuno es del 50 % de los casos. Se presenta el caso de una paciente de 1 año y 8 meses con diagnóstico reciente de infección por virus de inmunodeficiencia humana en estadio sida. Cursó internación para estudio e inicio de tratamiento antirretroviral durante la cual presentó múltiples intercurrencias infectológicas e inmunológicas. Se destacan dos episodios de linfohistiocitosis hemofagocítica en contexto de inmunodeficiencia adquirida no controlada y coinfecciones oportunistas. El objetivo de este reporte es destacar la importancia de la sospecha de LHH para un diagnóstico y tratamiento pertinente

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by a hyperinflammatory state secondary to dysregulated immune activity with multisystem involvement. HLH may be primary or hereditary, or triggered by various diseases. Mortality without a timely treatment reaches 50% of the cases. Here we describe the case of a 1-year and 8-month-old female patient with a recent diagnosis of human immunodeficiency virus infection in the AIDS stage. She was hospitalized for assessment and initiation of antiretroviral therapy during which she developed multiple intercurrent infectious and immune conditions. Two episodes of hemophagocytic lymphohistiocytosis in the setting of uncontrolled acquired immunodeficiency and opportunistic co-infections stand out. The objective of this case report is to highlight the importance of suspecting HLH for a relevant diagnosis and treatment.

Aumento de la frecuencia de escorbuto en niños con selectividad alimentaria: serie de casos / Increase in the frequency of scurvy in children with food selectivity: A case series

El escorbuto es una enfermedad producida por déficit de vitamina C. Aunque es poco frecuente, en los últimos años observamos un incremento de casos en niños con trastornos de la conducta alimentaria. Sus manifestaciones son variadas, ya que esta vitamina actúa como cofactor en numerosos procesos, como la síntesis de colágeno. Las manifestaciones cutáneas características son las petequias, equimosis e hiperqueratosis. El compromiso mucoso se manifiesta como gingivitis con hipertrofia, hemorragias y pérdida de piezas dentarias. El diagnóstico es clínico y puede confirmarse mediante la determinación de la vitamina C plasmática. El objetivo de este trabajo es describir una cohorte de pacientes diagnosticados en los últimos años, manifestaciones clínicas y hallazgos en relación con su conducta alimentaria y trastornos del neurodesarrollo.

Scurvy is a disease caused by vitamin C deficiency. Although rare, in recent years, the number of scurvy cases in children with eating disorders has increased. Its manifestations are varied because vitamin C is a cofactor in numerous processes, such as collagen synthesis. The typical skin manifestations include petechiae, bruising, and hyperkeratosis. Mucosal involvement manifests as gingivitis with hypertrophy, bleeding, and loss of teeth. The diagnosis is based on clinical findings and may be confirmed by measuring plasma vitamin C levels. The objective of this study was to describe a cohort of patients diagnosed with scurvy in recent years, its clinical manifestations, and findings in relation to their eating behavior and neurodevelopmental disorders.

Tendência temporal de internação por queimadura, na faixa etária 0-14 anos, no Brasil, 2012-2022 / Time trend of hospitalization due to burn, in the age group of 0-14 years, in Brazil, 2012-20

Introdução: Queimaduras são lesões teciduais causadas pelo contato com fontes de calor. Representam um problema de saúde pública global. Em crianças causam grandes impactos. A gravidade e intensidade das queimaduras estão relacionadas ao agente etiológico e suas consequências são um grande obstáculo para o paciente. Método: O estudo analisou dados de internações hospitalares por queimaduras em crianças de 2012 a 2022, utilizando informações do SIH-SUS. Foram calculadas taxas de internações por 100.000 habitantes para cada ano, considerando variáveis dependentes. Os pesquisadores utilizaram coeficientes padronizados e regressão linear simples para analisar os dados. Resultados: Incluíram-se dados de 91.091 internações por queimaduras em crianças, 0-14 anos. Verificou-se estabilidade na taxa geral de internações (taxa média 17,963; ß=0,119; p=0,163). No sexo feminino houve tendência de aumento (taxa média 14,346; ß=0,169; p=0,029); no masculino de estabilidade (taxa média 21,426; ß=0,069; p=0,504). O sexo feminino comportou-se com estabilidade em todas as faixas etárias; o masculino com aumento na faixa 0-4 anos (taxa média 42,264; ß=0,613; p=0,003), estabilidade na faixa 5-9 anos (taxa média 14,189; ß=-0,21; p=0,867) e redução na faixa 10-14 anos (taxa média 9,871; ß= -0,328; p=0,007). A Região Sul demonstrou tendência de aumento (taxa média 26,952; ß=1,091; p=0,001). Conclusão: Houve estabilidade na taxa geral de internações. O sexo feminino tendeu ao aumento e o masculino à estabilidade. Houve estabilidade nas regiões brasileiras, exceto no Sul.

Introduction: Burns are tissue injuries caused by contact with heat sources. They represent a global public health problem. They have major impacts on children. The severity and intensity of burns are related to the etiological agent and their consequences are a major obstacle for the patient. Method: The study analyzed data on hospital admissions for burns in children from 2012 to 2022, using information from SIH-SUS. Hospitalization rates per 100,000 inhabitants were calculated for each year, considering dependent variables. The researchers used standardized coefficients and simple linear regression to analyze the data. Results: Data from 91,091 hospitalizations for burns in children, 0-14 years old, were included. There was stability in the general hospitalization rate (mean rate 17.963; ß=0.119; p=0.163). In females there was an increasing trend (mean rate 14.346; ß=0.169; p=0.029); in males, there was stability (mean rate 21.426; ß=0.069; p=0.504). Females behaved with stability in all age groups; the male with an increase in the range 0-4 years (mean rate 42.264; ß=0.613; p=0.003), stability in the range 5-9 years (mean rate 14.189; ß=-0.21; p=0.867) and reduction in the range 10-14 years (mean rate 9.871; ß= -0.328; p=0.007). The South Region demonstrated an increasing trend (mean rate 26.952; ß=1.091; p=0.001). Conclusion: There was stability in the general hospitalization rate. The female sex tended towards increase and the male towards stability. There was stability in Brazilian regions, except in the South.

Utilidad de la tomografía de impedancia eléctrica para la programación del ventilador mecánico en paciente crítico pediátrico, una alternativa dinámica y no invasiva: revisión de dos casos clínicos / Utility of electrical impedance tomography for mechanical ventilator programming in pediatric critical patients, a dynamic and non-invasive alternative: a review of two clinical cases

La tomografía por impedancia eléctrica (TIE) es una modalidad de monitorización funcional respiratoria por imagen, no invasiva y libre de radiación, que permite visualizar en tiempo real la ventilación pulmonar regional y global en pacientes adultos y pediátricos conectados a Ventilación Mecánica (VM). OBJETIVO: Se describe la utilidad de la TIE en dos pacientes críticos pediátricos, en quienes no fue factible realizar medición de mecánica pulmonar, como herramienta para el ajuste de parámetros ventilatorios. CASOS CLÍNICOS: Se presentan dos pacientes pediátricos de 27 y 11 meses con condiciones clínicas diferentes, conectados a VM, en quienes se utilizó la TIE como método de monitoreo de la distribución pulmonar y titulación de la presión positiva al final de la espiración (PEEP) óptima, con el objetivo de obtener una ventilación pulmonar más homogénea. Se presentan mediciones funcionales con diferentes niveles de PEEP y valores de distribución en las distintas regiones de interés (ROI), además de un flujograma de situaciones en las que la TIE podría resultar útil para el ajuste ventilatorio. CONCLUSIÓN: La información funcional proporcionada por la TIE, permitió monitorizar de forma dinámica la VM y optimizar los parámetros ventilatorios, facilitando la implementación de estrategias de protección pulmonar en ambos pacientes, imposibilitados de realizar una medición estática de la mecánica respiratoria.

The Electrical Impedance Tomography (EIT) is a non-invasive and radiation-free respiratory functional imaging monitoring modality that allows real-time visualization of regional and global lung ventilation in adult and pediatric patients connected to mechanical ventilation (MV). OBJECTIVE: This paper describes the utility of EIT in two critical pediatric patients for whom measuring pulmonary mechanics was not feasible. EIT is used as a tool for adjusting ventilatory parameters. CLINICAL CASES: Two pediatric patients aged 27 and 11 months, with different clinical conditions, connected to MV are presented. EIT was used to monitor lung distribution and titrate the optimal Positive End-Expiratory Pressure (PEEP), to achieve more homogeneous lung ventilation. Functional measurements are presented with different PEEP levels and distribution values in different regions of interest (ROI), along with a flowchart illustrating situations where EIT could be useful for ventilatory adjustment. CONCLUSION: The functional information provided by EIT, allowed dynamic monitoring of MV, optimizing ventilatory parameters and facilitating the implementation of lung protective strategies in both patients, unable to undergo static respiratory mechanics measurements.

Asociación de experiencias adversas en la infancia con síntomas de asma en niños. Revisión bibliográfica / Association of adverse childhood experiences with asthma symptoms in children. A literature review

Las experiencias adversas en la infancia (EAI) tienen un impacto considerable en la salud física y mental de los niños y adolescentes. Esta revisión bibliográfica se centra en la asociación entre estas experiencias y la incidencia de síntomas asmáticos, así como en las alteraciones inmunológicas en la población pediátrica. Los estudios revisados muestran una correlación significativa entre la exposición a EAI y el asma, destacando la importancia de un enfoque biopsicosocial para su manejo. Además, se discuten las implicancias clínicas y las precauciones necesarias al interpretar estos hallazgos.

Adverse childhood experiences (ACEs) have a significant impact on the physical and mental health of children and adolescents. This literature review focuses on the association between these experiences and the incidence of asthma symptoms, as well as immunological alterations in the pediatric population. The reviewed studies show a significant correlation between ACE exposure and asthma, highlighting the importance of a biopsychosocial approach for its management. Additionally, the clinical implications and necessary precautions when interpreting these findings are discussed.

Acute kidney injury: the experience of a tertiary center of Pediatric Nephrology / Insuficiência renal aguda: experiência de um centro terciário de Nefrologia Pediátrica

ABSTRACT Introduction: Acute kidney injury (AKI) is an abrupt deterioration of kidney function. The incidence of pediatric AKI is increasing worldwide, both in critically and non-critically ill settings. We aimed to characterize the presentation, etiology, evolution, and outcome of AKI in pediatric patients admitted to a tertiary care center. Methods: We performed a retrospective observational single-center study of patients aged 29 days to 17 years and 365 days admitted to our Pediatric Nephrology Unit from January 2012 to December 2021, with the diagnosis of AKI. AKI severity was categorized according to Kidney Disease Improving Global Outcomes (KDIGO) criteria. The outcomes considered were death or sequelae (proteinuria, hypertension, or changes in renal function at 3 to 6 months follow-up assessments). Results: Forty-six patients with a median age of 13.0 (3.5-15.5) years were included. About half of the patients (n = 24, 52.2%) had an identifiable risk factor for the development of AKI. Thirteen patients (28.3%) were anuric, and all of those were categorized as AKI KDIGO stage 3 (p < 0.001). Almost one quarter (n = 10, 21.7%) of patients required renal replacement therapy. Approximately 60% of patients (n = 26) had at least one sequelae, with proteinuria being the most common (n = 15, 38.5%; median (P25-75) urinary protein-to-creatinine ratio 0.30 (0.27-0.44) mg/mg), followed by reduced glomerular filtration rate (GFR) (n = 11, 27.5%; median (P25-75) GFR 75 (62-83) mL/min/1.73 m2). Conclusions: Pediatric AKI is associated with substantial morbidity, with potential for proteinuria development and renal function impairment and a relevant impact on long-term prognosis.

RESUMO Introdução: Insuficiência renal aguda (IRA) é uma deterioração abrupta da função renal. A incidência de IRA pediátrica está aumentando em todo o mundo, em ambientes críticos e não críticos. Nosso objetivo foi caracterizar apresentação, etiologia, evolução e desfechos da IRA em pacientes pediátricos internados em um centro de atendimento terciário. Métodos: Realizamos estudo retrospectivo observacional de centro único de pacientes com idade entre 29 dias a 17 anos e 365 dias internados em nossa Unidade de Nefrologia Pediátrica, de janeiro de 2012 a dezembro de 2021, com diagnóstico de IRA. A gravidade da IRA foi categorizada de acordo com os critérios do Kidney Disease Improving Global Outcomes (KDIGO). Os desfechos considerados foram óbito ou sequelas (proteinúria, hipertensão ou alterações na função renal em avaliações de acompanhamento de 3 a 6 meses). Resultados: Incluímos 46 pacientes com idade mediana de 13,0 (3,5-15,5) anos. Cerca de metade (n = 24; 52,2%) apresentou um fator de risco identificável para o desenvolvimento de IRA. Treze pacientes (28,3%) eram anúricos; todos foram classificados como IRA KDIGO 3 (p < 0,001). Quase um quarto (n = 10; 21,7%) dos pacientes necessitaram de terapia renal substitutiva. Aproximadamente 60% (n = 26) apresentou pelo menos uma sequela, sendo proteinúria a mais comum (n = 15; 38,5%; mediana (P25-75) da relação proteína/creatinina urinária 0,30 (0,27-0,44) mg/mg), seguida de taxa de filtração glomerular (TFG) reduzida (n = 11; 27,5%; mediana (P25-75) da TFG 75 (62-83) mL/min/1,73 m2). Conclusões: A IRA pediátrica está associada à morbidade substancial, com potencial para desenvolvimento de proteinúria e comprometimento da função renal e impacto relevante no prognóstico de longo prazo.

Percutaneous kidney biopsies in children: a 24-year review in a tertiary center in northern Portugal / Biópsias renais percutâneas em crianças: uma revisão de 24 anos em um centro terciário no norte de Portugal

Introduction: Percutaneous kidney biopsy (KB) is crucial to the diagnosis and management of several renal pathologies. National data on native KB in pediatric patients are scarce. We aimed to review the demographic and clinical characteristics and histopathological patterns in children who underwent native percutaneous KB over 24 years. Methods: Retrospective observational study of patients undergoing native percutaneous KB in a pediatric nephrology unit between 1998 and 2021, comparing 3 periods: period 1 (1998-2005), period 2 (2006-2013), and period 3 (2014-2021). Results: We found that 228 KB were performed, 78 (34.2%) in period 1, 91 (39.9%) in period 2, and 59 (25.9%) in period 3. The median age at KB was 11 (7-14) years. The main indications for KB were nephrotic syndrome (NS) (42.9%), hematuria and/or non-nephrotic proteinuria (35.5%), and acute kidney injury (13.2%). Primary glomerulopathies were more frequent (67.1%), particularly minimal change disease (MCD) (25.4%), IgA nephropathy (12.7%), and mesangioproliferative glomerulonephritis (GN) (8.8%). Of the secondary glomerulopathies, lupus nephritis (LN) was the most prevalent (11.8%). In group 1, hematuria and/or non-nephrotic proteinuria were the main reasons for KB, as opposed to NS in groups 2 and 3 (p < 0.01). LN showed an increasing trend (period 1-3: 2.6%-5.3%) and focal segmental glomerular sclerosis (FSGS) showed a slight decreasing trend (period 1-3: 3.1%-1.8%), without statistical significance. Conclusions: The main indication for KB was NS, which increased over time, justifying the finding of MCD as main histological diagnosis. LN showed an increase in incidence over time, while FSGS cases did not increase.

RESUMO Introdução: A biópsia renal (BR) percutânea é fundamental para diagnóstico e manejo de diversas patologias renais. Dados nacionais sobre BR nativa em pacientes pediátricos são escassos. Nosso objetivo foi revisar características demográficas, clínicas e padrões histopatológicos em crianças submetidas a BR percutânea nativa ao longo de 24 anos. Métodos: Estudo observacional retrospectivo de pacientes submetidos a BR percutâneas nativas em unidade de nefrologia pediátrica entre 1998 e 2021, comparando três períodos: período 1 (1998-2005), período 2 (2006-2013), período 3 (2014-2021). Resultados: Constatamos que foram realizadas 228 BR, 78 (34,2%) no período 1, 91 (39,9%) no período 2, 59 (25,9%) no período 3. A idade mediana na BR foi 11 (7-14) anos. As principais indicações para BR foram síndrome nefrótica (SN) (42,9%), hematúria e/ou proteinúria não nefrótica (35,5%), lesão renal aguda (13,2%). Glomerulopatias primárias foram mais frequentes (67,1%), principalmente doença de lesão mínima (DLM) (25,4%), nefropatia por IgA (12,7%), glomerulonefrite mesangioproliferativa (GN) (8,8%). Das glomerulopatias secundárias, nefrite lúpica (NL) foi a mais prevalente (11,8%). No grupo 1, hematúria e/ou a proteinúria não nefrótica foram os principais motivos para BR, ao contrário da SN nos grupos 2 e 3 (p < 0,01). A NL apresentou tendência crescente (período 1-3: 2,6%-5,3%) e a glomeruloesclerose segmentar focal (GESF) apresentou leve tendência decrescente (período 1-3: 3,1%-1,8%), sem significância estatística. Conclusões: A principal indicação para BR foi SN, que aumentou ao longo do tempo, justificando o achado de DLM como principal diagnóstico histológico. A NL apresentou aumento na incidência ao longo do tempo, enquanto os casos de GESF não aumentaram.

Drenaje venoso anómalo. Vena cava superior izquierda persistente que drena en la aurícula derecha y vena cava superior derecha que drena en la aurícula izquierda: reporte de un caso / Anomalous Venous Drainage. Persistent Left Superior Vena Cava Draining into the Right Atrium and Right Superior Vena Cava Draining into the Left Atrium: Case Report

Introducción: Entre 0,3-0,5% de niños nacidos presentan una vena cava superior izquierda persistente, lo que lo hace una de las malformaciones congénitas más frecuentes del drenaje venoso. El drenaje de esta en la aurícula derecha, además del drenaje de la vena cava superior derecha en la aurícula izquierda, es extremadamente infrecuente. Caso Clínico: Se presenta el caso de un infante de 8 meses asintomático que es llevado a consulta por presentar desaturaciones. El examen físico es normal. El ecocardiograma de contraste con solución salina muestra una vena cava superior izquierda persistente que drena en la aurícula derecha y un retorno anómalo de la vena cava superior derecha. Se realiza una corrección quirúrgica y evoluciona de forma favorable. Conclusión: La presentación en simultáneo de una vena cava superior izquierda persistente que drena en la aurícula derecha y una vena cava derecha que drena en la aurícula izquierda es extremadamente rara. La mayoría de los casos registrados se diagnosticaron de manera incidental en personas adultas al no presentar síntomas.

Introduction: Between 0.3-0.5% of children born have a persistent left superior vena cava, which makes it one of the most frequent congenital malformations of venous drainage. Drainage of this vein into the right atrium, in addition to drainage of the right superior vena cava into the left atrium, is extremely rare. Clinical case: We present a case of an asymptomatic 8-month-old infant who was taken to the clinic for desaturations. Physical examination was normal. The contrast echocardiogram with saline solution showed a persistent left superior vena cava draining into the right atrium and an anomalous return of the right superior vena cava. Surgical correction was performed and the patient evolved favorably. Conclusion: The simultaneous presentation of a persistent left superior vena cava draining into the right atrium and a right superior vena cava draining into the left atrium is extremely rare. Most of the reported cases were diagnosed incidentally in adults in the absence of symptoms.

El tumor inflamatorio de Pott: una complicación infrecuente de la sinusitis. Reporte de caso / Pott's puffy tumor: A rare complication of sinusitis. A case report

Una complicación infrecuente de la sinusitis frontal es el tumor inflamatorio de Pott. Se manifiesta como una tumefacción en la frente por la presencia de un absceso subperióstico secundario a una osteomielitis del hueso frontal. El diagnóstico oportuno permite un tratamiento intensivo médico y quirúrgico precoz, esencial para evitar complicaciones intracraneales graves. Se presenta el caso de un varón de 12 años con un tumor inflamatorio de Pott como complicación de una pansinusitis. Representó un desafío diagnóstico; sin embargo, la instauración del tratamiento oportuno permitió una evolución clínica favorable.

A rare complication of frontal sinusitis includes Pott's puffy tumor. It manifests as a swelling of the forehead due to the presence of a subperiosteal abscess secondary to osteomyelitis of the frontal bone. A timely diagnosis allows for an early, intensive medical and surgical treatment, which is critical to prevent serious intracranial complications. Here we describe the case of a 12-year-old boy with Pott's puffy tumor as a complication of pansinusitis. This case was a diagnostic challenge; however, a timely treatment allowed for a favorable clinical course.

Síndrome de la costilla deslizante en la edad pediátrica. Serie de casos / Slipping rib syndrome in childhood. A case series

Entre las causas de dolor torácico, la costilla deslizante presenta baja prevalencia, antecedentes traumáticos y manejo controvertido. Este síndrome merece ser incluido en el diagnóstico diferencial de causas de dolor torácico en niños. Al no asociarse a traumatismos previos y la deformidad de cartílagos, nos induce a pensar en una alteración en el desarrollo costal, al margen de la etiología traumática típica en adultos. Se presenta una serie de pacientes pediátricos intervenidos por costilla deslizante en un centro de referencia entre 2001 y 2022. Se incluyeron nueve pacientes, con un rango de edades de 11 a 16 años. Solo dos casos describen traumatismo previo. Todos presentan un inicio súbito de dolor toracoabdominal intenso. Los pacientes fueron intervenidos mediante resección abierta de cartílagos costales afectos, con resolución del dolor.

Among the causes of chest pain, slipping rib has a low prevalence, usually with a history of trauma, and its management is controversial. Slipping rib syndrome should be included in the differential diagnosis of causes of chest pain in children. When not associated with previous trauma and cartilage deformity, it is necessary to consider an alteration in rib development, regardless of the typical traumatic etiology in adults. Here we describe a series of pediatric patients with slipping rib seen at a referral hospital between 2001 and 2022. Nine patients aged 11 to 16 years were included. Only 2 had a history of trauma. All patients described a sudden onset of severe thoracic abdominal pain. The patients underwent open resection of the affected costal cartilages, with resolution of pain.

Tumores del sistema nervioso central. Clasificación de la Organización Mundial de la Salud 2021. Hacia un cambio de paradigma / Tumors of the central nervous system. Classification of the World Health Organization 2021. Towards a paradigm shift

El estudio de los tumores del sistema nervioso central (SNC) resulta ser un tema de gran consideración y su conocimiento reviste una alta importancia en la práctica médica. Las clasificaciones de las neoplasias del SNC comenzaron a mediados del siglo XIX hasta que en 1979 la Organización Mundial de la Salud (OMS) publicó la primera edición de una sistemática útil con el objetivo de establecer un lenguaje común para todas las especialidades médicas. Al día de hoy, 5 ediciones actualizaron la taxonomía neoplásica. La quinta edición del año 2021 consolida el cambio de paradigma dado por los avances moleculares, si bien todavía la transición se encuentra en proceso entre la caracterización morfológica y la biológica molecular. En este artículo, se analizan las nuevas modificaciones incorporadas en las diferentes familias tumorales más frecuentes en pediatría haciendo hincapié en aquella información de utilidad para el médico pediatra en su práctica diaria y la consulta multidisciplinaria.

The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19 th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published.The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.

Cáncer De Laringe En Adolescencia Y Virus De Papiloma Humano: Reporte De Un Caso

Introducción: El carcinoma de células escamosas de cabeza y cuello, es una malignidad rara en la población pediátrica. Su incidencia ha ido en aumento en las últimas décadas y los principales factores de riesgo descritos son inmunosupresión y transmisión vertical del virus del papiloma humano (VPH). Presentación del caso: presentamos a un paciente masculino de 14 años con historia de 2 años de disfonía intermitente que luego progreso a estridor laríngeo por lo cual se le realizo traqueostomía con micro laringoscopia directa, donde se evidenció una lesión de aspecto verrucoso y bordes irregulares comprometiendo hemilaringe derecha y paralizándola. Se le toma biopsia y se reporta carcinoma de células escamosas moderadamente invasor (p16 positivo). Luego de evaluación multidisciplinaria, tratándose de una enfermedad localmente avanzada, se decide tratamiento con quimio radioterapia definitiva con intención de preservación orgánica. Conclusión: debido a lo poco común del cáncer de laringe en la población pediátrica y la poca evidencia que hay en cuanto a opciones de tratamiento, limita nuestro conocimiento de esta patología solo a reporte de casos y revisiones de la literatura. consideramos necesario realizar este reporte de caso para agregar información sobre esta patología poco común (provisto por Infomedic International)

Introduction: Squamous cell carcinoma of the head and neck is a rare malignancy in the pediatric population. Its incidence has been increasing in the last decades and the main risk factors described are immunosuppression and vertical transmission of human papillomavirus (HPV). Case presentation: we present a 14-year-old male patient with a 2-year history of intermittent dysphonia that later progressed to laryngeal stridor, for which he underwent tracheostomy with direct micro laryngoscopy, where a lesion of verrucous aspect and irregular borders was evidenced, compromising the right hemilaringeal region and paralyzing it. A biopsy was taken and a moderately invasive squamous cell carcinoma (p16 positive) was reported. After multidisciplinary evaluation, being a locally advanced disease, it was decided to treat with definitive chemo-radiotherapy with the intention of organ preservation. Conclusion: due to the rarity of laryngeal cancer in the pediatric population and the lack of evidence regarding treatment options, our knowledge of this pathology is limited to case reports and literature reviews. We consider it necessary to make this case report to add information on this uncommon pathology. (provided by Infomedic International)

Compromiso de arteria renal en paciente con arteritis de Takayasu: reporte de caso y revisión de literatura

Introducción: La arteritis de Takayasu (TAK) es una enfermedad idiopática granulomatosa de grandes vasos que afecta predominantemente a la aorta, ramas arteriales principales y pulmonares. La prevalencia es mayor en adultos que en niños, con una incidencia de 1,1 por millón de personas. Su etiología sigue siendo poco conocida. Caso Clínico: Se presenta el caso de un paciente pediátrico con pérdida de la masa renal izquierda por disminución del calibre de arteria renal ipsilateral meses después del debut de la Arteritis de Takayasu como aneurisma de aorta abdominal. El paciente cumple criterios de TAK según clasificación EULAR/PRINTO/PReS basados en anomalía angiográfica, hipertensión arterial y elevación reactantes de fase aguda. La TAK presenta afectación sistémica y alteraciones cardiovasculares (75-85%), hipertensión en más del 80% de los casos, con estenosis de la arteria renal en casi el 50% de los pacientes. Conclusiones: El tratamiento general de la TAK está dirigido a controlar la inflamación vascular y prevenir el daño orgánico irreversible. A pesar de un diagnóstico temprano con tratamiento oportuno y agresivo el seguimiento en la enfermedad de Takayasu es imprescindible dado que la progresión de esta puede seguirse dando. Deben establecerse estrategias para asegurar la adherencia al seguimiento. (provided by Infomedic Intl.)

Introduction: Takayasu's arteritis (TAK) is an idiopathic granulomatous large vessel disease predominantly affecting the aorta, main arterial branches and pulmonary arteries. The prevalence is higher in adults than in children, with an incidence of 1.1 per million people. Its etiology remains poorly understood. Case Report: We present the case of a pediatric patient with loss of left renal mass due to decreased ipsilateral renal artery caliber months after the debut of Takayasu's arteritis as abdominal aortic aneurysm. The patient meets TAK criteria according to EULAR/PRINTO/PReS classification based on angiographic abnormality, arterial hypertension and elevated acute phase reactants. TAK presents systemic involvement and cardiovascular alterations (75-85%), hypertension in more than 80% of cases, with renal artery stenosis in almost 50% of patients. Conclusions: The general treatment of TAK is aimed at controlling vascular inflammation and preventing irreversible organ damage. Despite early diagnosis with timely and aggressive treatment, follow-up in Takayasu's disease is imperative as progression may still occur. Strategies should be established to ensure adherence to follow-up. (provided by Infomedic Intl.)

Desafío Diagnóstico

Niña 6 años, originaria de Darién, sin antecedentes personales de interés. Inicia cuadro de dolor y cojera de miembro inferior izquierdo con edema en región posterior de tobillo izquierdo. Los familiares lo relacionan con haber estado de excursión en un río de Darién días previos. Consultan en varias ocasiones en centros de salud de la zona, donde pautan tratamiento ambulatorio y colocación de férula. (provisto por Infomedic International)

6-year-old girl, originally from Darien, with no personal history of interest. She begins with pain and lameness of the left lower limb with edema in the posterior region of the left ankle. Family members relate it to having been hiking in a river in Darien the previous days. They consulted several times in health centers in the area, where they prescribed outpatient treatment and splinting. (provided by Infomedic International)

Tumores en ovario en pediatría / Ovarian tumors in pediatrics

Los tumores de ovario en la edad pediátrica son raros, representan 1-5 % de los tumores infantiles, con una incidencia anual de 2,6 casos por cada 100.000 pacientes. La mayoría son benignos y se tratan de quistes funcionales, sin embargo, entre 10-20 % son malignos y generalmente se presentan en adolescentes; estos últimos, se dividen en 3 grupos: tumores epiteliales, germinales, y estromales o de células sexuales. Método: Estudio retrospectivo de tipo transversal, observacional, no experimental. Se analizaron los pacientes con diagnóstico de tumor de ovario, ingresados en el Servicio de Cirugía Pediátrica del Hospital de Niños "Dr. José Manuel de los Ríos", entre el 1 de enero de 2017 y 01 de julio de 2022. Resultados: 18 pacientes incluidos en el estudio, con edad media de 8,23 años (DE 4,77); los síntomas más frecuentes presentados al momento del ingreso fueron: aumento de volumen abdominal (52,94 %, 9 pacientes), y dolor abdominal (35,29 %, 6 pacientes), entre otros. Reporte patológico: 2 pacientes con quistes de ovario funcional (11,76 %) y 16 pacientes con tumor neoplásico (88,23 %), de los cuales 8 fueron germinales (53,33 %), 5 tumores epiteliales (33,33 %) y 2 pacientes con linfoma (13,33 %). Conclusión: Los tumores de ovario en general tienen una edad promedio de presentación de 8 años y los tumores neoplásicos se presentaron principalmente en adolescentes, siendo el tipo histológico más frecuente el tumor germinal y dentro de este grupo el teratoma quístico maduro. (AU)

Ovarian tumors in pediatric age are rare, representing 1-5 % of childhood tumors, with an annual incidence of 2.6 cases per 100,000 patients. Most of them are benign and functional cysts; however, between 10-20 % are malignant and generally occur in teenagers; the latter are divided into 3 groups: epithelial, germinal, and stromal or sex cell tumors. Methods: Retrospective, cross-sectional, observational, non-experimental study. Patients with a diagnosis of ovarian tumor, admitted to the Pediatric Surgery Service of the Children's Hospital "Dr. José Manuel de los Ríos", between January 01, 2017 and July 01, 2022, were analyzed. Results: 18 patients included in the study, with mean age 8.23 years (SD 4.77); the most frequent symptoms presented at admission were: increased abdominal volume (52.94 %, 9 patients), and abdominal pain (35.29 %, 6 patients), among others. Pathological report: 2 patients with functional ovarian cysts (11.76 %) and 16 patients with neoplastic tumor (88.23 %), of which 8 were germinal (53.33 %), 5 epithelial tumors (33.33 %) and 2 patients with lymphoma (13.33 %). Conclusion: Ovarian tumors in general have an average age of presentation of 8 years and neoplastic tumors occurred mainly in teenagers, the most common histological type being the germ cell tumor and within this group the mature cystic teratoma. (AU)

[Detection of microorganisms and resistance genes from positive blood cultures using the FilmArray® BCID2 panel: Experience in a pediatric hospital]. / Detección de microorganismos y genes de resistencia en hemocultivos positivos mediante FilmArray® panel BCID2: experiencia en un hospital pediátrico.

The rapid identification of microorganisms that cause bacteremia and their possible resistance markers are extremely important for the timely initiation of effective antibiotic therapy. The FilmArray® panel BCID2 (an automated rapid multiplex PCR assay) detects microorganisms and resistance genes from positive blood cultures within one hour. The aim of this study was to compare the results obtained from the FilmArray® Panel BCID2 and conventional culture in pediatric patients, as well as the reporting times of both methods. Sixty (60) FilmArray® results were included in the analysis. BCID2 showed high agreement with culture in the identification of microorganisms in monomicrobial bacteremias. However, in polymicrobial blood cultures, BCID2 detected a greater number of microorganisms compared to conventional culture, specifically,1 Staphylococcus aureus, 3 Staphylococcus epidermidis, 1 Enterococcus faecium, 2 Klebsiella oxytoca, 1 Acinetobacter calcoaceticus-baumannii complex, 1 Bacteroides fragilis and 1 Haemophilus influenzae. Furthermore, 88.3% (95%CI: 78.7-94.8) of the FilmArray® results coincided with conventional culture, while in polymicrobial bacteremias, BCID2 detected a greater number of microorganisms with respect to conventional culture [70% (95%CI: 39.3-91.5)]. The agreement of resistance genes was good with a few exceptions (one ESBL was not detected by FilmArray® and one S. aureus strain was characterized as methicillin-resistant by BCID2 and methicillin-sensitive by culture). When comparing the time elapsing since the blood culture was reported as positive up to the results were obtained, BCID2 had a median of 2h 50min (IQR of 1 h 58min to 9h 27min) while the conventional culture had a median of 45h 20min (IQR of 24h 57min to 63h 50min).

Necesidades de cuidados paliativos en el contexto del cáncer infantil en un hospital de tercer nivel de atención / Necessidades de cuidados paliativos no contexto do câncer infantil em um hospital terciário / Palliative care needs in the context of childhood cancer in a tertiary care hospital

Introducción: La Organización Mundial de la Salud destaca que los cuidados paliativos mejoran la calidad de vida de pacientes niños y adultos frente a enfermedades potencialmente mortales. Metodología: Estudio de tipo observacional, prospectivo, transversal y descriptivo. Incluyó a 120 pacientes oncológicos de 8 a 18 años, junto a sus cuidadores y enfermeras asignadas. Se utilizó el instrumento NECPAL 3.1 y se analizaron los datos con SPSS versión 25. Resultados: La mediana de edad de los pacientes pediátricos fue de 11 años; la leucemia linfoblástica aguda ocupó el primer lugar de morbilidad con el 52.5%. El 78.3% de los participantes fueron NECPAL positivos. Se encontró que el 85% tenía dos o más síntomas persistentes, predominantemente dolor, cansancio y náuseas. Además, el 87.5% experimentó malestar emocional, con preocupaciones significativas sobre aspectos económicos (47.5%), familiares (70.8%) y emocionales (48.3%). Discusión: Resaltan investigaciones previas de Levin et al., Alvarado et al. y Vanegas et al., entre otros, sobre el sufrimiento y los síntomas persistentes en pacientes oncológicos, así como la relevancia del apoyo familiar en los cuidados paliativos. Conclusiones: Es imperativo un abordaje continuo y multidisciplinario para abordar los síntomas físicos, emocionales y sociales en pacientes pediátricos con cáncer. Este enfoque integral no solo mejora la calidad de vida durante el tratamiento, sino que también proporciona un apoyo crucial durante la fase terminal, garantizando una atención óptima a lo largo del proceso

Introduction: The World Health Organization emphasizes that palliative care improves the quality of life of children and adult patients in the face of life-threatening illnesses. Methodology: Observational, prospective, cross-sectional and descriptive study. Included 120 oncology patients aged 8 to 18 years alongside their assigned nurses and caregivers. The NECPAL 3.1 tool was used, and the data were analyzed with SPSS version 25. Results: The median age of the pediatric patients was 11 years old. With 52.5%, the acute lymphoblastic leukemia ranked first in morbidity. 78.3% of the participants were NECPAL positive. 85% were found to have two or more persistent symptoms, mainly pain, fatigue, and nausea. In addition, 87.5% experienced emotional discomfort, with significant concerns about economic (47.5%), familiar (70.8%) and emotional (48.3%) aspects. Discussion: Previous research by Levin et al., Alvarado et al., Vanegas et al. and Vanegas et al., among others, on distress and persistent symptoms in oncology patients as well as the importance of family support in palliative care. Conclusions: It is imperative to address continuously and in a multidisciplinary manner the physical, emotional and social symptoms in pediatric cancer patients. This integral approach not only improves the quality of life during treatment, but also provides crucial support during the terminal stage, guaranteeing optimal care throughout the process

Introdução: A Organização Mundial da Saúde aponta que os cuidados paliativos melhoram a qualidade de vida dos pacientes, tanto das crianças quanto dos adultos, diante de doenças potencialmente mortais. Metodologia: Estudo de tipo observacional, prospectivo, transversal e descritivo. Ele incluiu 120 pacientes oncológicos da faixa etária entre 8 e 10 anos, bem como suas enfermeiras e cuidadores atribuídos. Foi utilizado o instrumento NECPAL 3,1. Além disso, foram analisados os dados com o programa SPSS, versão 25. Resultados: A média da faixa etária dos pacientes pediátricos foi de 11 anos; a leucemia linfoblástica aguda ocupou o primeiro lugar de morbidez, com 52,5%. Segundo os critérios NECPAL, 78,3% dos participantes foram positivos. Se achou que 85% tinham dois ou mais sintomas persistentes, nomeadamente dor, cansaço e náuseas. Além disso, 87,5% experimentaram mal-estares emocionais, com preocupações significativas no que diz respeito a aspetos econômicos (47,5%), familiares (70,8%) e emocionais (48,3%). Discussão: Destacam pesquisas prévias de Levin et al., Alvarado et al. e Vanegas et al., entre outras, sobre o sofrimento e os sintomas persistentes em pacientes oncológicos, bem como o relevo do apoio familiar nos cuidados paliativos. Conclusões: É imperativo ter uma abordagem contínua e multidisciplinar para defrontar os sintomas físicos, emocionais e sociais dos pacientes pediátricos com câncer. Isto deve ser feito integralmente, não só para melhorar a qualidade de vida durante o tratamento, mas também para brindar apoio crucial durante a fase terminal. Deve se garantir uma atenção ótima no decurso do processo