Case report: Multisystem inflammatory syndrome in children associated with COVID-19, macrophage activation syndrome, and incomplete Kawasaki disease.

Background: Multisystem inflammatory syndrome in children (MIS-C), is a severe complication of coronavirus disease 2019 (COVID-19), characterized by persistent fever, systemic inflammatory response, and organ failure. MIS-C with a history of COVID-19 may share clinical features with other well-defined syndromes such as macrophage activation syndrome, Kawasaki disease, hemophagocytic syndrome and toxic shock syndrome. Case 1: An 11-year-old male with a history of hypothyroidism and precocious puberty with positive antibody test for COVID-19 was admitted for fever, poor general condition, severe respiratory distress, refractory shock, and multiple organ failure. His laboratory examination showed elevated inflammatory parameters, and bone marrow aspirate showed hemophagocytosis. Case 2: A 13-year-old male with a history of attention deficit hyperactivity disorder and cognitive delay presented clinical manifestations of Kawasaki disease, fever, conjunctival congestion, exanthema, and hyperemia in oral mucosa, tongue, and genitals, with refractory shock and multiple organ failure. Reverse transcriptase polymerase chain reaction (RT-PCR) and antibodies for COVID-19 were negative, inflammation parameters were elevated, and bone marrow aspirate showed hemophagocytosis. Patients required intensive care with invasive mechanical ventilation, vasopressor support, intravenous gamma globulin, systemic corticosteroids, low molecular weight heparin, antibiotics, and monoclonal antibodies and, patient 2 required renal replacement therapy. Conclusions: Multisystemic inflammatory syndrome in children can have atypical manifestations, and identifying them early is very important for the timely treatment and prognosis of patients.

[Unilateral congenital pulmonary lymphangiectasia in a preterm infant]. / Linfangiectasia pulmonar congénita unilateral en un neonato prematuro.

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Linfangiectasia pulmonar congénita unilateral en un neonato prematuro / Unilateral congenital pulmonary lymphangiectasia in a preterm infant

La linfangiectasia pulmonar congénita (LPC) unilateral es una enfermedad extremadamente rara de los vasos linfáticos pulmonares. OBJETIVO: presentar un caso de LPC en un recién nacido prematuro. CASO CLÍNICO: recién nacido masculino, prematuro, con insuficiencia respiratoria severa a las 2 horas de vida extrauterina, recibió tratamiento con surfactante exógeno, catecolaminas y ventilación de alta frecuencia oscilatoria (VAFO). La tomografía axial computarizada (TAC) de tórax reveló bulas y atrapamiento de aire de pulmón izquierdo, el estudio histopatológico describió dilatación quística de los canales linfáticos broncoalveolares. Se diagnosticó LPC unilateral secundaria. La evolución clínica hasta los 19 meses de edad fue normal y la TAC de tórax mostró escasas bulas enfisematosas. CONCLUSIONES: La LPC debe ser uno de los diagnósticos diferenciales en neonatos con dificultad respiratoria inexplicable. El pronóstico dependerá del tipo de LPC y de la afectación pulmonar.

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Balance de líquidos y lesión renal aguda en el choque séptico / Fluid balance and acute kidney injury in septic shock

Resumen: Introducción: En el paciente con choque séptico, la administración excesiva de líquidos puede incrementar la morbilidad y mortalidad. El objetivo de este estudio fue evaluar la asociación entre el balance de líquidos, la lesión renal aguda y la mortalidad en pacientes con choque séptico. Métodos: Se realizó un estudio de casos y controles en una unidad de terapia intensiva pediátrica. Se comparó el balance de líquidos en las primeras 72 h y la presencia de lesión renal aguda en pacientes con diagnóstico de choque séptico que fallecieron contra pacientes que sobrevivieron a la misma patología. Se realizó un análisis univariado y multivariado. Resultados: Se incluyeron 45 casos y 45 controles en el análisis. La mortalidad se asoció con riesgo pediátrico de mortalidad (PRISM) ≥ 26 puntos (RM 7.5, IC 95% 2.8-18.7; p = 0.000), disfunción orgánica logística pediátrica (PELOD) ≥ 24 puntos (RM 11.0, IC 95% 4.1-29.4; p = 0.000), creatinina ≥ 0.65 mg/dl (RM 5.6, IC 95% 2.2-13.9; p = 0.000), lactato ≥ 2.5 mmol/l (RM 2.5, IC 95% 1.1-5.9; p = 0.033), SvO2 < 60% (RM 4.6, IC 95% 4.5-4.5; p = 0.001), balance positivo > 9% en 72 h (RM 4.3, IC 95% 1.6-11.7; p = 0.003), lesión renal aguda (RM 5.7, IC 95% 2.2-15.1; p = 0.000). En el modelo multivariado, PRISM ≥ 26 y PELOD ≥ 24 puntos permanecieron significativas. Conclusiones: En los pacientes que fallecieron por choque séptico, el modelo multivariado mostró una asociación con PRISM ≥26 y PELOD ≥24 y una tendencia hacia la asociación con SvO2 <60% y balance de líquidos positivo >9%.

Abstract: Background: In patients with septic shock, excessive fluid administration can lead to increased morbidity and mortality. The aim of this study was to evaluate the association between fluid balance, acute kidney injury and mortality in patients with septic shock. Methods: A study of cases and controls was conducted in a Pediatric Intensive Care Unit. The fluid balance in the first 72 h and the presence of acute kidney injury was compared in patients diagnosed with septic shock who died against patients who survived the same condition. Univariate and multivariate analyses were performed. Results: Forty-five cases and forty-five controls were included in the analysis. Mortality was associated with Pediatric Risk of Mortality (PRISM III) ≥ 26 points (OR 7.5, 95% CI 2.8-18.7; p = 0.000), Pediatric Logistic Organ Dysfunction (PELOD) ≥ 24 points (OR 11.0, 95% CI 4.1-29.4; p = 0.000), creatinine ≥ 0.65 mg/dl (OR 5.6, 95% CI 2.2-13.9; p = 0.000), lactate ≥ 2.5 mmol/l (OR 2.5, 95% CI 1.1-5.9; p = 0.033), SvO2 < 60% (OR 4.6, 95% CI 4.5-4.5; p = 0.001), positive balance > 9% in 72 h (OR 4.3, 95% CI 1.6-11.7; p = 0.003), acute kidney injury (OR 5.7, 95% CI: 2.2-15.1; p = 0.000). In the multivariate model, the values of PRISM ≥26 and PELOD ≥24 points were significant. Conclusions: In patients who died due to septic shock, the multivariate model showed an association with PRISM ≥26 and PELOD ≥24 and a trend toward association with SvO2 <60% and positive balance of liquids > 9%.

[Fluid balance and acute kidney injury in septic shock]. / Balance de líquidos y lesión renal aguda en el choque séptico.

BACKGROUND: In patients with septic shock, excessive fluid administration can lead to increased morbidity and mortality. The aim of this study was to evaluate the association between fluid balance, acute kidney injury and mortality in patients with septic shock. METHODS: A study of cases and controls was conducted in a Pediatric Intensive Care Unit. The fluid balance in the first 72h and the presence of acute kidney injury was compared in patients diagnosed with septic shock who died against patients who survived the same condition. Univariate and multivariate analyses were performed. RESULTS: Forty-five cases and forty-five controls were included in the analysis. Mortality was associated with Pediatric Risk of Mortality (PRISM III) ≥ 26 points (OR 7.5, 95% CI 2.8-18.7; p=0.000), Pediatric Logistic Organ Dysfunction (PELOD) ≥ 24 points (OR 11.0, 95% CI 4.1-29.4; p=0.000), creatinine ≥ 0.65mg/dl (OR 5.6, 95% CI 2.2-13.9; p=0.000), lactate ≥ 2.5 mmol/l (OR 2.5, 95% CI 1.1-5.9; p=0.033), SvO2 < 60% (OR 4.6, 95% CI 4.5-4.5; p=0.001), positive balance > 9% in 72h (OR 4.3, 95% CI 1.6-11.7; p=0.003), acute kidney injury (OR 5.7, 95% CI: 2.2-15.1; p=0.000). In the multivariate model, the values of PRISM ≥26 and PELOD ≥24 points were significant. CONCLUSIONS: In patients who died due to septic shock, the multivariate model showed an association with PRISM ≥26 and PELOD ≥24 and a trend toward association with SvO2 <60% and positive balance of liquids > 9%.

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans / Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis

La histiocitosis de células de Langerhans pulmonar es una patología intersticial en la que existe un acumulo de células histiocíticas específicas a nivel pulmonar. El neumotórax espontáneo es una complicación reconocida de histiocitosis de células de Langerhans pulmonar y es secundario a la destrucción del parénquima pulmonar con cambios quísticos asociados. Reportamos el caso de un niño de 2 años con neumotórax espontáneo bilateral recurrente, con una tomografía axial computada de tórax con infiltrado intersticial, fibrosis, lesiones quísticas e imágenes bullosas. El diagnóstico fue establecido por examen histológico e inmunohistoquímica de tejido de biopsia pulmonar con anticuerpos CD1 y S100 positivos. El niño recibió tratamiento con prednisona y etopósido, con buena respuesta clínica y tomográfica.

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans / Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis

La histiocitosis de células de Langerhans pulmonar es una patología intersticial en la que existe un acumulo de células histiocíticas específicas a nivel pulmonar. El neumotórax espontáneo es una complicación reconocida de histiocitosis de células de Langerhans pulmonar y es secundario a la destrucción del parénquima pulmonar con cambios quísticos asociados. Reportamos el caso de un niño de 2 años con neumotórax espontáneo bilateral recurrente, con una tomografía axial computada de tórax con infiltrado intersticial, fibrosis, lesiones quísticas e imágenes bullosas. El diagnóstico fue establecido por examen histológico e inmunohistoquímica de tejido de biopsia pulmonar con anticuerpos CD1 y S100 positivos. El niño recibió tratamiento con prednisona y etopósido, con buena respuesta clínica y tomográfica.(AU)

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.(AU)

[Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis]. / Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans.

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans. / [Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis].

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.

Algoritmo de tratamiento de la cetoacidosis diabética en pediatría: [revisión] / Treatment algorithm of diabetic ketoacidosis in pediatric: [review]

La cetoacidosis diabética (CAD) es la complicación más importante de la diabetes mellitus. La piedra angular para el diagnóstico de la CAD son la historia clínica y la exploración física, en ellas generalmente encontramos los factores precipitantes y podemos clasificar el estado de hidratación del paciente. Los estudios de laboratorio son de gran utilidad para monitorizar la hiperglucemia, el estado ácido-base y el desequilibrio electrolítico inicial. La terapia inicial durante la primera hora es administración de líquidos intravenosos, generalmente cristaloides, con revaloraciones del estado de hidratación y de los niveles séricos de potasio antes de comenzar el tratamiento con insulina, que debe hacerse en la segunda hora. El monitoreo de la glucemia y de los electrolitos séricos es la base para un tratamiento exitoso en la CAD, sobre todo para evitar el edema cerebral, que es la complicación más seria. Afortunadamente, dicha complicación es rara y uno de los factores de riesgo asociados es el tratamiento inadecuado; si bien se han propuesto otros factores de riesgo, no están totalmente identificados.

Guía para la determinación de muerte cerebral en el niño / Guide for the determination of brain death in children

La muerte cerebral (MC) es definida como la pérdida irreversible de la función cerebral incluyendo las del tallo cerebral. Un dilema frecuente en la Unidades de Cuidados Intensivos Pediátricos es el hecho de cómo podemos definir y diagnosticar la MC, bajo una variedad de circunstancias entre ellas la edad de desarrollo. Los criterios de MC estableccidos en adultos han sido aceptados en forma general por múltiples comisiones médicas; pero la aplicabilidad de esos criterios para edades pediátricas no ha sido definitvamente establecido. Esta guía hace énfasis en los períodos de observación de acuerdo a las edades pediátricas, diagnóstico clínico, incluyendo prueba de apnea, así como las principales pruebas confirmatorias para MC y sus criterios