Encefalopatía grave y deficiencia de vitamina B12: reversibilidad tras la terapia nutricional / Severe encephalopathy and vitamin B12 deficiency: reversibility after nutritional therapy

INTRODUCCIÓN: la vitamina cobalamina es esencial para el buen funcionamiento del SNC, la hematopoyesis y la síntesis de ADN. Su déficit es frecuentemente secundario a la anemia perniciosa o a las dietas vegetarianas estrictas. CASO CLÍNICO: lactante varón de 18 meses con disminución del nivel de conciencia y trastorno del movimiento (temblor y movimientos coreiformes) de horas de duración. Como antecedentes, presenta retraso de la adquisición de los hitos motores (no bipedestación, lenguaje monosilábico) y pérdida progresiva de los mismos en las últimas semanas (sostén cefálico y sedestación). Alimentado mediante lactancia materna desde el nacimiento. La familia sigue una alimentación vegetariana. En las pruebas de neuroimagen, la TC y la RM craneales muestran atrofia supratentorial generalizada de ambas sustancias y de los ganglios basales. Se inicia tratamiento con vitamina B12 intramuscular, aumentando sus niveles. Posteriormente se procede a la diversificación alimentaria y la administración de cianocobalamina oral, con normalización clínica al cabo de 6 meses y radiológica al cabo de 7 meses. DISCUSIÓN: recalcamos la importancia de la suplementación con B12 durante el embarazo y la lactancia tanto en la mujer como en el lactante

INTRODUCTION: the vitamin cobalamin is essential for a proper functioning of the CNS, hematopoiesis, and DNA synthesis. Its deficiency is frequently secondary to pernicious anemia or strict vegetarian diets. CASE REPORT: an 18-month-old male infant presented with a decreased level of consciousness and movement disorder (tremor and choreiform movements) of several hours' standing. He had a history of delayed acquisition of motor milestones (not standing, monosyllabic language), and progressive loss of these over the last few weeks (head support and sitting). He had been breastfed from birth. His family has a vegetarian diet. In neuroimages, cranial CT and MRI scans showed generalized supratentorial atrophy involving both matters and the basal ganglia. Treatment was started with intramuscular vitamin B12, which increased its levels. Subsequently, the patient was subjected to dietary diversification and oral cyanocobalamin, with clinical normalization after 6 months and radiological normalization after 7 months. DISCUSSION: we emphasize the importance of vitamin B12 supplementation during pregnancy and lactation in vegetarian mothers and their infants

[Severe encephalopathy and vitamin B12 deficiency: reversibility after nutritional therapy]. / Encefalopatía grave y deficiencia de vitamina B12: reversibilidad tras la terapia nutricional.

INTRODUCTION: Introduction: the vitamin cobalamin is essential for a proper functioning of the CNS, hematopoiesis, and DNA synthesis. Its deficiency is frequently secondary to pernicious anemia or strict vegetarian diets. Case report: an 18-month-old male infant presented with a decreased level of consciousness and movement disorder (tremor and choreiform movements) of several hours' standing. He had a history of delayed acquisition of motor milestones (not standing, monosyllabic language), and progressive loss of these over the last few weeks (head support and sitting). He had been breastfed from birth. His family has a vegetarian diet. In neuroimages, cranial CT and MRI scans showed generalized supratentorial atrophy involving both matters and the basal ganglia. Treatment was started with intramuscular vitamin B12, which increased its levels. Subsequently, the patient was subjected to dietary diversification and oral cyanocobalamin, with clinical normalization after 6 months and radiological normalization after 7 months. Conclusions: we emphasize the importance of vitamin B12 supplementation during pregnancy and lactation in vegetarina mothers and their infants.

INTRODUCCIÓN: Introducción: la vitamina cobalamina es esencial para el buen funcionamiento del SNC, la hematopoyesis y la síntesis de ADN. Su déficit es frecuentemente secundario a la anemia perniciosa o a las dietas vegetarianas estrictas. Caso clínico: lactante varón de 18 meses con disminución del nivel de conciencia y trastorno del movimiento (temblor y movimientos coreiformes) de horas de duración. Como antecedentes, presenta retraso de adquisición de hitos motores (no bipedestación, lenguaje monosilábico) y pérdida progresiva de los mismos en lúltimas semanas (sostén cefálico y sedestación). Alimentado mediante lactancia materna desde el nacimiento. La familia sigue una alimentación vegetariana. En las pruebas de neuroimagen, la TC y la RM craneales muestran atrofia supratentorial generalizada de ambas sustancias y de los ganglios basales. Se inicia tratamiento con vitamina B12 intramuscular, aumentando sus niveles. Posteriormente se procede a la diversificación alimentaria y la administración de cianocobalamina oral, con normalización clínica al cabo de 6 meses y radiológica al cabo de 7 meses. Conclusiones: recalcamos la importancia de la suplementación con B12 durante el embarazo y lactancia tanto en la mujer como en el lactante.

Niveles de neopterina y síndrome de respuesta inflamatoria sistémica en pacientes críticos pediátricos / Neopterin levels and systemic inflammatory response syndrome in pediatric critically ill patients

Introducción: La neopterina y biopterina, subproductos de reacciones redox, son cofactores en la producción de óxido nítrico. Hipótesis: La neopterina y biopterina plasmáticas sufren evolución diferente durante los primeros días de una enfermedad crítica en pediatría. Métodos: Estudio prospectivo observacional monocéntrico en pacientes de 7 días-14 años ingresados en UCIP con criterios de SRIS. Se recogieron, al ingreso y a las 24 h, los niveles de neopterina y biopterina, otros reactantes de fase aguda y datos clínicos. Resultados: Se analizó a 28 pacientes, el 78,9% varones, de 5,04 años (RIQ 1,47-10,26), con PRISM II 2,0% (RIQ 1,1-5,0), ventilación mecánica (VM) en 90% (36,8% >24 h), duración de VM de 6,0 h (RIQ 3,7-102,0), ingreso en UCIP de 5,0 días (RIQ 2,7-18,7), media de VIS máximo de 0 (RIQ 0-14). La neopterina inicial fue de 2,3 ± 1,2 nmol/l y a las 24 h de 2,3 ± 1,4 nmol/l. La biopterina basal fue 1,3 ± 0,5 nmol/l y a las 24h 1,4 ± 0,4 nmol/l. La neopterina fue significativamente mayor en estancia > 6 días (p = 0,02), VM > 24h (p = 0,023) y con complicaciones (p = 0,05). La neopterina se correlaciona de forma directa con la duración de VM (rho = 0,6; p = 0,011), la estancia en UCIP (rho = 0,75; p < 0,0001) y el VIS (rho = 0,73; p = 0,001). Adicionalmente, la biopterina se correlaciona directamente con el PRISM (rho = 0,61; p = 0,008) y la cifra de leucocitos (rho = 0,88; p = 0,002). Discusión: Existe un ascenso de neopterina con mayor estancia, mayor VIS, VM más duradera y aparición de complicaciones, lo que refleja una activación del sistema inmune celular en los más graves (AU)

Introduction: Neopterin and biopterin are sub-products of redox reactions, which act as cofactors of enzymes responsible for nitric oxide production. The hypothesis is presented that plasma neopterin and biopterin evolve differently during the first days in a critically ill child. Methods: A single-centre prospective observational study was conducted on patients 7 days to 14 years admitted to our Paediatric Intensive Care Unit (PICU) and that met Systemic inflammatory response syndrome (SIRS) criteria. Neopterin and biopterin levels, as well as other acute phase reactants, were collected at admission and at 24 h. Results: A total of 28 patients were included, of which 78.9% were male, The median age was 5.04 years (interquartile range [IQR] 1.47-10.26), and PRISM II 2.0% (IQR 1.1-5.0). Mechanical ventilation (MV) was used in 90% of patients, with a median duration of 6.0 hrs (IQR 3.7-102.0). The median length of stay in PICU was 5.0 days (IQR 2.7-18.7), maximum VIS mean of 0 (IQR 0-14). Baseline neopterin level was 2.3±1.2 nmol/l and at 24 h it was 2.3±1.4 nmol/l. Baseline biopterin was 1.3±0.5 nmol/l and 1.4±0.4 nmol/l at 24 h. Neopterin levels were significantly higher in patients with PICU length of stay > 6 days (P=.02), patients who needed MV >24 h (P=.023), and those who developed complications (P=.05). Neopterin correlates directly and is statistically significant with the duration of MV (rho=.6, P=.011), PICU length of stay (rho=.75, P<.0001), and VIS (rho=.73, P=.001). Additionally, biopterin directly correlates with the PRISM (rho=.61, P=.008). Discussion: There is a higher neopterin level when there is a longer PICU stay, higher VIS score, longer time on MV, and occurrence of complications, indicating the involvement of an activation of the cellular immune system (AU)

[Neopterin levels and systemic inflammatory response syndrome in pediatric critically ill patients]. / Niveles de neopterina y síndrome de respuesta inflamatoria sistémica en pacientes críticos pediátricos.

INTRODUCTION: Neopterin and biopterin are sub-products of redox reactions, which act as cofactors of enzymes responsible for nitric oxide production. The hypothesis is presented that plasma neopterin and biopterin evolve differently during the first days in a critically ill child. METHODS: A single-centre prospective observational study was conducted on patients 7 days to 14 years admitted to our Paediatric Intensive Care Unit (PICU) and that met Systemic inflammatory response syndrome (SIRS) criteria. Neopterin and biopterin levels, as well as other acute phase reactants, were collected at admission and at 24 h. RESULTS: A total of 28 patients were included, of which 78.9% were male, The median age was 5.04 years (interquartile range [IQR] 1.47-10.26), and PRISM II 2.0% (IQR 1.1-5.0). Mechanical ventilation (MV) was used in 90% of patients, with a median duration of 6.0 hrs (IQR 3.7-102.0). The median length of stay in PICU was 5.0 days (IQR 2.7-18.7), maximum VIS mean of 0 (IQR 0-14). Baseline neopterin level was 2.3±1.2 nmol/l and at 24 h it was 2.3±1.4 nmol/l. Baseline biopterin was 1.3±0.5 nmol/l and 1.4±0.4 nmol/l at 24 h. Neopterin levels were significantly higher in patients with PICU length of stay > 6 days (P=.02), patients who needed MV >24 h (P=.023), and those who developed complications (P=.05). Neopterin correlates directly and is statistically significant with the duration of MV (rho=.6, P=.011), PICU length of stay (rho=.75, P<.0001), and VIS (rho=.73, P=.001). Additionally, biopterin directly correlates with the PRISM (rho=.61, P=.008). DISCUSSION: There is a higher neopterin level when there is a longer PICU stay, higher VIS score, longer time on MV, and occurrence of complications, indicating the involvement of an activation of the cellular immune system.

Estrés oxidativo en el postoperatorio de cirugía cardiovascular pediátrica / Oxidative stress response after cardiac surgery in children

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Acute alithiasic cholecystitis: a not so rare disease.

INTRODUCTION: Acute acalculous cholecystitis (AAC) occurs more frequently in critically ill patients, in the immediate postoperative period, after trauma or extensive burns. It has a high rate of morbidity and mortality. Ischemia, infection and vesicular stasis are determinants in its pathogenesis. MATERIAL AND METHOD: Retrospective study including all cases of AAC diagnosed in our pediatric intensive care unit between January 1997 and December 2012. RESULTS: We included 7 patients, all associated with viral or bacterial infection. All of them suffered from abdominal pain, mainly localized in the right upper quadrant, jaundice and dark urine. Abdominal ultrasound showed thickening and hypervascularity of the gallbladder wall in all cases. The outcome was satisfactory without surgery in all patients. CONCLUSIONS: The clinical presentation is oligosymptomatic within severe systemic diseases. The AAC should be suspected in the appearance of any abdominal pain with jaundice/dark urine and hypertransaminasemia in patients suffering from critical or serious infections.

Colecistitis aguda alitiásica en pediatría. Una patología no tan rara / Acute alithiasic cholecystitis: a not so rare disease

Introducción: la colecistitis aguda alitiásica (CAA) se da con mayor frecuencia en pacientes críticos, en el periodo postoperatorio inmediato, tras traumatismos o quemaduras extensas. Tiene una alta tasa de morbimortalidad. La isquemia, la infección y la estasis vesicular son determinantes en su patogenia. Material y método: estudio retrospectivo que incluye todos los casos de CAA diagnosticados en la unidad de cuidados intensivos de nuestro centro en el periodo comprendido entre enero de 1997 y diciembre de 2012. Resultados: se incluyen a 7 pacientes, todos asociaron infección viral o bacteriana. Todos debutaron con dolor abdominal localizado en hipocondrio derecho, ictericia y coluria. La ecografía abdominal en todos los casos demostró engrosamiento e hipervascularización de la pared vesicular. La evolución fue satisfactoria en todos los casos sin necesidad de cirugía. Conclusiones: la presentación del cuadro es oligosintomática en el seno de enfermedades sistémicas de gravedad variable. La CAA se debe sospechar ante todo cuadro de dolor abdominal con ictericia/coluria e hipertransaminasemia en pacientes críticos o que cursan infecciones graves (AU)

Introduction: Acute acalculous cholecystitis (AAC) occurs more frequently in critically ill patients, in the immediate postoperative period, after trauma or extensive burns. It has a high rate of morbidity and mortality. Ischemia, infection and vesicular stasis are determinants in its pathogenesis. Material and method: Retrospective study including all cases of AAC diagnosed in our pediatric intensive care unit between January 1997 and December 2012. Results: We included 7 patients, all associated with viral or bacterial infection. All of them suffered from abdominal pain, mainly localized in the right upper quadrant, jaundice and dark urine. Abdominal ultrasound showed thickening and hypervascularity of the gallbladder wall in all cases. The outcome was satisfactory without surgery in all patients. Conclusions: The clinical presentation is oligosymptomatic within severe systemic diseases. The AAC should be suspected in the appearance of any abdominal pain with jaundice/dark urine and hypertransaminasemia in patients suffering from critical or serious infections (AU)

Estudio de la agregación plaquetaria en el postoperatorio de cirugía cardiovascular mediante circulación extracorpórea en edad pediátrica / Effects of cardiac surgery with extracorporeal circulation on platelet aggregation in children

Introducción: Hay aspectos no bien aclarados en el postoperatorio de cirugía extracorpórea infantil, como la importancia de las alteraciones de la agregación plaquetaria. Objetivo: Analizar las alteraciones de agregación plaquetaria presentes en el postoperatorio de circulación extracorpórea pediátrico y ver su evolución temporal en primeras 24 h. Material y métodos: Estudio analítico, de cohortes, prospectivo, observacional en niños de un mes a 14 años, sometidos a cirugía cardiaca mediante circulación extracorpórea entre 2010-2011. Muestras de sangre previo a la intervención (PRE), tras 1 h del desclampaje (PO1) y tras 18-20 h (PO2). Se analiza la agregación plaquetaria inducida por colágeno, araquidónico y ADP, valorando su correlación con los tiempos quirúrgicos y de clampaje aórtico. Resultados: Treinta pacientes; mediana 4,1 años (IQ: 2,7; 8,0); 62,1% niñas; mediana de desviaciones estándar de peso -0,39 (IQ: -0,76; 0,24), de talla -0,22 (IQ: -0,74; 0,27) y de IMC -0,43 (IQ: -1; 0,45). Mediana de tiempo quirúrgico 79 min (IQ: 52,5; 125,5), mediana de clampaje 38,5 min (IQ: 22, 59). La agregación inducida por colágeno es menor en PO1 (15,20 ± 5,07) que en PRE (28,60 ± 4,22) y en PO2 (20,60 ± 3,98) empieza a ascender, pero aún es menor que en PRE; igual pasa con la inducida por araquidónico (25,00 ± 2,94; 14,10 ± 1,52; 19,50 ± 1,43) y por ADP (22,90 ± 1,66; 12,90 ± 1,52; 18,00 ± 2,49). Conclusiones: La circulación extracorpórea infantil genera disfunción plaquetaria grave, máxima en postoperatorio inmediato y aún persistente tras 24 h, independiente de tiempos de cirugía o clampaje; esto puede facilitar el sangrado en postoperatorio e influir en el uso de hemoderivados (AU)

Introduction: Some aspects of Cardiac Surgery with Extracorporeal Circulation in children are still not clear, one of which is impaired platelet aggregation. Objective: To analyze the Influence of Extracorporeal Circulation on changes in platelet aggregation in children < 15 years in our center, and to observe these changes over time in first 24 hours. Material and Methods: Analytical, cohort, prospective, observational study in children aged 1 month to 14 years, weight > 5 kg, undergoing cardiac surgery using cardiopulmonary bypass between 2010 and 2011. Blood samples were taken just before the intervention (PRE), after 1 h of declamping (PO1), and after 18-20 h (PO2). Platelet aggregation induced by collagen, arachidonic acid‘, and ADP were measured, assessing their correlation with surgery times and aortic clamping. Results: A total of 30 patients were included, with a median age of 4.1 years (IQR:2.7,8.0), 62.1% female, median weight of standard deviations of -0.39 (IQR:-0.76,0.24) of size -0.22 (IQR:- 0.74,0.27) and BMI -0.43 (IQR:-1,0,45). Median surgery time 79 min (IQR:52.5,125.5), clamping median 38.5 min (IQR:22,59). Collagen induced aggregation is lower in PO1 (15.20 ± 5.07) than in PRE (28.60 ± 4.22), and rises again in PO2 (20.60 ± 3.98), but is still less than in PRE; similarlywith arachidonic acid (25.00 ± 2.94, 14.10 ± 1.52, 19.50 ± 1.43), and ADP (22.90 ± 1.66, 12.90 ± 1.52, 18.00 ± 2.49). Conclusions: Cardiopulmonary bypass activates inflammatory mediators and causes a severe platelet dysfunction, which is maximum in immediate postoperative, and persistent even after 24 hours, regardless of surgery and clamping times, which may lead to postoperative bleeding and determine the use of blood and even furher surgery (AU)