La cinética de la citrulina y la arginina y su valor como factor pronóstico en pacientes pediátricos críticamente enfermos. Respuesta de los autores / The value of the kinetics of citrulline andarginine as a prognostic factor in critically ill paediatric patients. Author's reply

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Indicadores pronósticos clínicos en el posoperatorio de cirugía cardiovascular pediátrica y su relación con la cinética del estrés oxidativo / Prognostic indicators after cardiac surgery in children and their relationship with the oxidative stress response

Objetivos. Analizar la evolución de parámetros de estrés oxidativo en el posoperatorio de cirugía cardiovascular pediátrica y correlacionarlos con diferentes indicadores clínicos pronósticos. Material y métodos. Treinta niños, de entre un mes y 14 años, peso > 5 kg, sometidos a circulación extracorpórea. Se obtuvieron muestras preoperatoria, posoperatoria inmediata y tras 18-20 h. Se analizó la capacidad de peroxidación lipídica de las membranas celulares mediante la cuantificación de productos de reacción con el ácido tiobarbitúrico, cuyo principal representante es el malondialdehído; se cuantificó el contenido celular de glutatión total, oxidado y reducido (representantes de la respuesta antioxidante). Se analizaron las variables clínicas que permitieran establecer una puntuación para el síndrome de respuesta inflamatoria sistémica asociado a circulación extracorpórea. Resultados. Treinta pacientes con una mediana de edad de 4,1 años (rango intercuartílico [RIC]: 2,7; 8,0); el 62,1% eran niñas; mediana de desviaciones estándar de peso −0,39 (RIC: −0,76; 0,24), de talla −0,22 (RIC: −0,74; 0,27) y de IMC −0,43 (RIC: −1; 0,45). Mediana de tiempo quirúrgico 79 min (RIC: 52,5; 125,5), mediana de pinzamiento 38,5 min (RIC: 22; 59). Aumentó el malondialdehído y disminuyó el glutatión en ambos momentos posoperatorios, con clara correlación directa, estadísticamente significativa, del tiempo de circulación extracorpórea con el porcentaje de descenso de glutatión total entre preoperatorio y posoperatorio inmediato y entre el preoperatorio y el posoperatorio tardío. Hubo una correlación estadística entre los niveles de glutatión total tras 18-20 h posoperatorias y el tiempo de duración de la ventilación mecánica y la pertenencia al grupo de síndrome de respuesta inflamatoria sistémica. Conclusiones. La circulación extracorpórea activa mediadores inflamatorios, máximo tras el pinzamiento aórtico, mejorando tras 24 h, siendo dependiente de los tiempos quirúrgicos. El desarrollo de respuesta inflamatoria está asociado a una mayor duración de la ventilación mecánica, una estancia más prolongada en Cuidados Intensivos, puntuaciones mayores del Modelo de Aristóteles y tiempos más largos quirúrgicos. Los que no cumplen criterios de respuesta inflamatoria tienen más niveles de glutatión en el posoperatorio tardío (AU)

Objectives. To analyse the trend in lipid peroxidation and antioxidant response as key markers of oxidative stress after paediatric cardiovascular surgery, and compare them with other internationally accepted clinical prognostic indicators. Patients and methods. A prospective study was conducted on 30 children aged one month to 14 years, weight > 5 kg, undergoing cardiopulmonary bypass surgery. Blood samples were taken just before the intervention, immediately after surgery, and after 18-20 h. Cell membrane lipid peroxidation was analysed by quantifying malondialdehyde, as well as measuring total glutathione (oxidized and reduced), as representatives of antioxidant response. An analysis was also performed on clinical variables for establishing a score for the systemic inflammatory response syndrome associated with cardiopulmonary bypass. Results. The study included 30 children with a mean age of 4.1 years old (interquartile range [IQR]: 2.7; 8.0). Of these, 62.1% were girls. The standard deviation of the median weight was −0.39 (IQR: −0.76; 0.24), the median height was −0.22 (IQR: −0.74; 0.27), and the median BMI was −0.43 (IQR: −1; 0.45). The final surgery times were divided into 2 parts: total time of extracorporeal circulation, with a mean of 79 min (IQR: 52.5; 125.5), and the clamping time, a measurement included in the previous figure with a mean value of 38.5 min (IQR: 22; 59). Malondialdehyde increased and glutathione decreased in postoperative time, with clear, statistically significant direct correlation between time of extracorporeal circulation and percentage decrease in total glutathione between preoperative and immediate postoperative time, and a decline between the preoperative and late postoperative. There was a statistical correlation between total glutathione levels at 18-20 h postoperatively and the duration of mechanical ventilation and inflammatory systemic response syndrome. Conclusions. Surgery with extracorporeal circulation performed in children activates inflammatory mediators, being maximum after aortic clamping, and improving after the first 24 h. The level of oxidative stress activation depends on surgical times. The development of systemic inflammatory response syndrome is associated with longer duration of mechanical ventilation, longer stay in intensive care, higher scores in the Aristotle model and longer surgical times. Those who do not meet criteria for inflammatory response have higher levels of glutathione in first 24 h (AU)

Prognostic indicators after cardiac surgery in children and their relationship with the oxidative stress response.

OBJECTIVES: To analyse the trend in lipid peroxidation and antioxidant response as key markers of oxidative stress after paediatric cardiovascular surgery, and compare them with other internationally accepted clinical prognostic indicators. PATIENTS AND METHODS: A prospective study was conducted on 30 children aged one month to 14 years, weight>5 kg, undergoing cardiopulmonary bypass surgery. Blood samples were taken just before the intervention, immediately after surgery, and after 18-20 h. Cell membrane lipid peroxidation was analysed by quantifying malondialdehyde, as well as measuring total glutathione (oxidized and reduced), as representatives of antioxidant response. An analysis was also performed on clinical variables for establishing a score for the systemic inflammatory response syndrome associated with cardiopulmonary bypass. RESULTS: The study included 30 children with a mean age of 4.1 years old (interquartile range [IQR]: 2.7; 8.0). Of these, 62.1% were girls. The standard deviation of the median weight was -0.39 (IQR: -0.76; 0.24), the median height was -0.22 (IQR: -0.74; 0.27), and the median BMI was -0.43 (IQR: -1; 0.45). The final surgery times were divided into 2 parts: total time of extracorporeal circulation, with a mean of 79 min (IQR: 52.5; 125.5), and the clamping time, a measurement included in the previous figure with a mean value of 38.5 min (IQR: 22; 59). Malondialdehyde increased and glutathione decreased in postoperative time, with clear, statistically significant direct correlation between time of extracorporeal circulation and percentage decrease in total glutathione between preoperative and immediate postoperative time, and a decline between the preoperative and late postoperative. There was a statistical correlation between total glutathione levels at 18-20 h postoperatively and the duration of mechanical ventilation and inflammatory systemic response syndrome. CONCLUSIONS: Surgery with extracorporeal circulation performed in children activates inflammatory mediators, being maximum after aortic clamping, and improving after the first 24h. The level of oxidative stress activation depends on surgical times. The development of systemic inflammatory response syndrome is associated with longer duration of mechanical ventilation, longer stay in intensive care, higher scores in the Aristotle model and longer surgical times. Those who do not meet criteria for inflammatory response have higher levels of glutathione in first 24h.

Errores innatos del metabolism en un hospital pediátrico. Diferencias sustanciales entre la era pre y postcribado neonatal ampliado / Innate errors of metabolism in a pediatric hospital. Substantial differences between the extended neonatal pre- and post screening era

Introducción. Las aminoacidopatías son enfermedades metabólicas hereditarias (EMH) que sin diagnóstico ni tratamiento precoz pueden producir consecuencias graves, llegando incluso a la muerte. La introducción del programa de cribado neonatal mediante espectrometría de masas en tándem (MS/MS) pretende mejorar el pronóstico. El objetivo del estudio es comparar la evolución clínica de pacientes diagnosticados de aminoacidopatías en fase clínica frente a los resultantes del cribado neonatal. Material y métodos. Estudio descriptivo retrospectivo de pacientes diagnosticados de metabolopatías entre Enero de 2002 y Junio de 2015. El cribado ampliado de EIM mediante MS/MS se está empleando en nuestro centro desde abril de 2010. Resultados. Han sido diagnosticados en nuestra unidad en estos 13 años 245 casos de EMH. Agrupándolos por patologías: 152 trastornos del metabolismo de las proteínas, 27 trastornos del metabolismo de los carbohidratos, 33 trastornos del metabolismo lipídico, 12 enfermedades lisosomales, 2 enfermedades peroxisomales, 4 defectos congénitos de glicosilación de proteínas, 10 casos de enfermedad de Wilson y 5 de deficiencia de alfa-1 antitripsina. Del total de la serie, 19 pacientes son de origen magrebí (7,7%) y un 52 % de sexo femenino. De los casos que debutaron en cuidados intensivos pediátricos la mayoría de los pacientes requirieron apoyo agresivo, incluyendo ventilación mecánica y terapia de eliminación extracorpórea (7 diálisis peritoneal, 6 hemofiltración veno-venosa continua), así como fármacos vasoactivos. De todos los pacientes, sufrieron datos de shock 15 niños, fallo multiorgánico 6, grave insulto neurológico 8, coagulopatía 3 y fallo hepático agudo 3. Discusión. La descompensación aguda de una metabolopatía, como en otros EIM, es una emergencia metabólica que debemos diagnosticar y tratar precozmente, por su elevada morbimortalidad. La instauración del cribado ampliado ha logrado el tratamiento en fase presintomática y la identificación precoz de las descompensaciones agudas, lo cual ha contribuido al descenso de las mismas y a una clara reducción de mortalidad. Hay niños ya diagnosticados por cribado que pueden necesitar ingreso por descompensación y otros sin posibilidad de cribado que pueden requerir ingreso por debut (AU)

Introduction. Amino acid disorders are hereditary metabolic diseases (HMD) that may cause serious consequences, even death, without diagnosis or early treatment. The introduction of the neonatal screening program using tandem mass spectrometry (MS/MS) aims to improve the prognosis. This study has aimed to compare the clinical course of patients diagnosed of amino acid disorders in the clinical phase versus the results of neonatal screening. Material and methods. Retrospective descriptive study of patients diagnosed of metabolic disorders between January 2002 and June 2015. The extended screening of EIM by MS/ MS has been used in our center since April 2010. Results. A total of 245 cases of HMD has been diagnosed in our unit during these 13 years. Grouped by conditions: 152 protein metabolism disorders, 27 carbohydrate metabolism disorders, 33 lipid metabolism disorders, 12 lysosomal diseases, 2 peroxisomal diseases, 4 protein glycosylation congenital defects, 10 cases of Wilson disease and 5 alpha-1 antitrypsin deficiency. Nineteen out of the entire series were of origin Maghreb (7.7%) and 52% were women. Of the cases initiating in pediatric intensive care, most of the patients required aggressive support, including mechanical ventilation and extracorporeal elimination therapy (7 peritoneal dialysis, 6 continuous venovenous hemofiltration) and vasoactive drugs. Fifteen of all the patients suffered shock data, 6 multiorgan failure, 8 severe neurological insult, 3 coagulopathy, and 3 severe liver failure. Discussion. Acute decompensation of a metabolic disorder, as in other EIM is a metabolic emergency that should be diagnosed and treated early, due to its elevated morbidity- mortality. Initiation of extended screening has achieved treatment in the presymptomatic phase and early identification of acute decompensations, which has contributed to their decrease and to a clear reduction of mortality. There are children who have already been diagnosed by screening that may need hospitalization due to decompensations and others without possibility of screening that may require admission due to debut (AU)

La cinética de la citrulina y la arginina y su valor como factor pronóstico en pacientes pediátricos críticamente enfermos / Citrulline and arginine kinetics and its value as a prognostic factor in pediatric critically ill patients

INTRODUCCIÓN: Se han documentado bajas concentraciones de citrulina y arginina plasmáticas en niños en diversas condiciones patológicas. Hipótesis: La cinética de citrulina y arginina plasmáticas durante la enfermedad crítica pediátrica se correlaciona con parámetros evolutivos clínicos y bioquímicos. PACIENTES Y MÉTODOS: Estudio observacional unicéntrico prospectivo en pacientes de 7 días a 14 años ingresados en Unidad de Cuidados Intensivos Pediátricos (PICU). Los datos bioquímicos y clínicos fueron recogidos al ingreso, a las 12 h, a las 24 h, al 3.er y al 7.° día. RESULTADOS: Un total de 44 pacientes críticamente enfermos fueron incluidos y un grupo control de 51 niños sanos. La citrulina desciende de forma significativa (p < 0,05) a las 12 h de ingreso con niveles bajos mantenidos hasta el día 7, comenzando un aumento progresivo después. La arginina ya está descendida a las 6h, aunque tiene una subida más precoz (día 3). La disminución de citrulina al tercer día se correlaciona directamente con la arginina. Hay correlación entre la elevación de la citrulina al 7.° día con menor duración de ventilación mecánica, menor estancia en PICU y menos complicaciones. Los niveles de citrulina bajos al 7° día aún descendidos el día 7 se asocian con un mayor aumento de PCR y procalcitonina en primeras 24 h. La disminución de arginina en las primeras 12 h se correlaciona inversamente con estancia más larga, mayor número de complicaciones y aumento de reactantes de fase aguda en día 3. CONCLUSIONES: Hay disminución de arginina y citrulina en los primeros días de la enfermedad crítica, con recuperación al 3.er y 7.° día, respectivamente, y existe una relación entre mayor disminución y peor evolución

INTRODUCTION: Low concentrations of plasma citrulline and arginine have been reported in children under various pathological conditions. Hypothesis: Plasma citrulline and arginine levels undergo different kinetics during the early days of critical illness in children according to the severity of symptoms and can be correlated with other clinical and laboratory parameters associated with the SIR. PATIENTS AND METHODS: A single-center prospective observational study in patients 7 days to 14 years admitted to pediatric intensive care unit (PICU). Citrulline and arginine blood levels (blood in dry paper, analysis by mass spectrometry in tandem), acute phase reactants and clinical data were collected on admission, at 12 h, 24 h, 3 and 7 days. RESULTS: A total of 44 critically ill patients were included and control group was formed by 42 healthy children. The citrulline and arginine kinetic analysis showed: 1) Citrulline falls significantly (P<.05) at 12 h of admission; levels remain low until day 7 and begin progressive increase again. 2) Arginine is already lowered at 6h, although an earlier rise occurs (3rd day). 3. The decrease of citrulline in the first 3 days of admission positively correlates with arginine kinetics. Bivariate analysis showed: 1) Correlation of elevated citrulline on the 7th day with shorter duration of mechanical ventilation, lower PICU stay and lower occurrence of complications. The levels of citrulline still descended at day 7 are associated with increased CRP/procalcitonin elevation at first 24 h. 2) The greatest decrease of arginine in the first 12 h is associated with a longer PICU stay and greater number of complications and increase of acute phase reactants at 3 days. CONCLUSIONS: There are decreased levels of arginine and citrulline in the first days at PICU, with recovery at the 3rd and 7th day respectively, and a relationship between a greater decrease and a worse outcome and between a longer income and a higher serum CRP/procalcitonin

[Citrulline and arginine kinetics and its value as a prognostic factor in pediatric critically ill patients]. / La cinética de la citrulina y la arginina y su valor como factor pronóstico en pacientes pediátricos críticamente enfermos.

INTRODUCTION: Low concentrations of plasma citrulline and arginine have been reported in children under various pathological conditions. HYPOTHESIS: Plasma citrulline and arginine levels undergo different kinetics during the early days of critical illness in children according to the severity of symptoms and can be correlated with other clinical and laboratory parameters associated with the SIR. PATIENTS AND METHODS: A single-center prospective observational study in patients 7 days to 14 years admitted to pediatric intensive care unit (PICU). Citrulline and arginine blood levels (blood in dry paper, analysis by mass spectrometry in tandem), acute phase reactants and clinical data were collected on admission, at 12 h, 24 h, 3 and 7 days. RESULTS: A total of 44 critically ill patients were included and control group was formed by 42 healthy children. The citrulline and arginine kinetic analysis showed: 1) Citrulline falls significantly (P<.05) at 12 h of admission; levels remain low until day 7 and begin progressive increase again. 2) Arginine is already lowered at 6h, although an earlier rise occurs (3rd day). 3. The decrease of citrulline in the first 3 days of admission positively correlates with arginine kinetics. Bivariate analysis showed: 1) Correlation of elevated citrulline on the 7th day with shorter duration of mechanical ventilation, lower PICU stay and lower occurrence of complications. The levels of citrulline still descended at day 7 are associated with increased CRP/procalcitonin elevation at first 24 h. 2) The greatest decrease of arginine in the first 12 h is associated with a longer PICU stay and greater number of complications and increase of acute phase reactants at 3 days. CONCLUSIONS: There are decreased levels of arginine and citrulline in the first days at PICU, with recovery at the 3rd and 7th day respectively, and a relationship between a greater decrease and a worse outcome and between a longer income and a higher serum CRP/procalcitonin.

Bronquiolitis obliterante postinfecciosa con presentación radiológica como pulmón hiperclaro unilateral / Postinfective obliterative bronchiolitis presenting as a hyperlucent lung

El síndrome de Swyer-James McLeod, o pulmón hiperclaro unilateral idiopático, es una entidad fundamentalmente radiológica caracterizada por una hiperclaridad de uno o más lóbulos, o de un pulmón entero. El tamaño pulmonar puede ser normal o reducido. Existe una desviación mediastínica hacia el lado afectado durante la inspiración, con disminución del movimiento diafragmático homolateral; se observa una escasa vascularización del pulmón, con un patrón broncográfico peculiar, que puede acompañarse, como en este caso, de bronquiectasias. Se presenta el caso de un varón diagnosticado a los 2 meses de vida de bronquiolitis, que evolucionaría hacia el desarrollo de este síndrome(AU)

Swyer-James McLeod syndrome or unilateral hyperlucent lung is basically a radiologic entity, defined by one or more hyperlucent lung lobus, or a complete hyperlucent lung. Lung size may be normal or reduced. There is a mediastinal deviation towards the affected hemithorax during inhalation and a limited diaphragmatic movement. Poor lung vascularization is also appreciated and a characteristic bronchographyc pattern. It may be associated to bronchiectasias as it happened in our patient. We introduce a case of a two months old male infant diagnosed of serious RSV + bronchiolitis, needing intensive care(AU)

Lavados colónicos anterógrados para el estreñimiento refractario en niños sin encefalopatía / Antegrade colonic enemas for intractable constipation in non-mentally retarded children

Revisión retrospectiva de las cecostomías realizadas para lavados anterógrados colónicos. Doce apendicocecostomías desde enero de 2002 a febrero de 2008, 9 apendicostomías en pacientes con mielomeningocele y 3 cecostomías en niños con estreñimiento crónico no orgánico sin encefalopatía ni retraso mental. De los 9 primeros pacientes, de entre 3 y 13 años, 8 tuvieron muy buena evolución y uno requirió retirada por mal empleo familiar. A un niño de 7 años, por lo demás sano, con estreñimiento crónico desde los 10 meses, pese a laxantes múltiples (varias tandas de desimpactación y dilatación anal bajo anestesia), con estudio morfofuncional normal, se le realizó, hace 5 años, cecostomía; persistió cierta tendencia a impactación pero con buena calidad de vida. Otro niño, previamente sano, de 12 años de edad, presentaba incontinencia fecal diaria asociada a estreñimiento desde los 3 años, con manometría anorrectal normal y biopsia con leve displasia neuronal; se le realizó cecostomía hace 3 años, con mejoría evidente y menor trastorno emocional secundario. El último caso de cecostomía tenía 8 años, con cuadro similar al anterior y se realizó procedimiento endoscópico con botón de Chait, con lo que mejoró francamente el cuadro. La progresión del estreñimiento rebelde a edad adulta tiene un impacto negativo en la adaptación social y el estado emocional del paciente, y puede alterar la vida familiar. Los lavados anterógrados colónicos proporcionan independencia y mejoran la calidad de vida. Se necesitan realizar en más pacientes para poder establecer verdaderos datos de efectividad (AU)

A descriptive review of 12 patients who underwent appendicocecostomy or caecostomy for antegrade colonic lavage from January 2002 to February 2008. There were 9 appendicocecostomies performed patients from 3 to 13 years suffering from myelomeningocele, of which 8 of them had a very good outcome, with one case with drawn due to poor use by the family. Three caecostomies were performed in non-mentally retarded constipated children. One was an otherwise healthy 7 year-old boy with hards tools since he was 10 months old, in spite of multiple laxative treatments, with normal morphology and function. He had a percutaneous caecostomy five years ago, with some improvement and a good quality of life, but still some occasional partial impactions. Another healthy 12 year-old boy with daily constipation associated faecal incontinence since he was 3 years old (normal manometry and rectal biopsy with signs of mild neuronal dysplasia)had a percutaneous caecostomy performed three years ago, with improvement in the faecal incontinence and better psychological out come. The last caecostomy patient was an 8 year-old boy, with a similar clinical history and good progress in last three years after placing a Chait’s button using an endoscopic procedure. Stubborn constipation continuing in to adult life has a negative impact on the social and emotional adaptation of the paediatric patient, affecting family interactions. Antegrade colonic lavage allows independence and improves the quality of life in patients affected by recurrent faecal impactions. This technique needs to be performed on more patients to find out its true effectiveness (AU)

[Antegrade colonic enemas for intractable constipation in non-mentally retarded children]. / Lavados colónicos anterógrados para el estreñimiento refractario en niños sin encefalopatía.

A descriptive review of 12 patients who underwent appendicocecostomy or caecostomy for antegrade colonic lavage from January 2002 to February 2008. There were 9 appendicocecostomies performed patients from 3 to 13 years suffering from myelomeningocele, of which 8 of them had a very good outcome, with one case withdrawn due to poor use by the family. Three caecostomies were performed in non-mentally retarded constipated children. One was an otherwise healthy 7 year-old boy with hard stools since he was 10 months old, in spite of multiple laxative treatments, with normal morphology and function. He had a percutaneous caecostomy five years ago, with some improvement and a good quality of life, but still some occasional partial impactions. Another healthy 12 year-old boy with daily constipation associated faecal incontinence since he was 3 years old (normal manometry and rectal biopsy with signs of mild neuronal dysplasia) had a percutaneous caecostomy performed three years ago, with improvement in the faecal incontinence and better psychological outcome. The last caecostomy patient was an 8-year-old boy, with a similar clinical history and good progress in last three years after placing a Chait's button using an endoscopic procedure. Stubborn constipation continuing into adult life has a negative impact on the social and emotional adaptation of the paediatric patient, affecting family interactions. Antegrade colonic lavage allows independence and improves the quality of life in patients affected by recurrent faecal impactions. This technique needs to be performed on more patients to find out its true effectiveness.