Estrategias de fluidoterapia racional en Cuidados Intensivos Pediátricos / Strategies of rational fluid therapy in Pediatric Intensive Cares

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Paciente pediátrico crítico. Cuidados Intensivos Pediátricos, Hospital Infantil La Paz / Critical pediatric patient. Pediatric intensive cares, Hospital Infantil La Paz

Los Cuidados Intensivos Pediátricos (CIPs) iniciaron su actividad en el Hospital Infantil La Paz en el año 1074. El servicio actual dispone de 16 camas, cuatro de cuidados medios, ocho de cuidados intensivos y cuatro de aislamiento con exclusa y filtros HEPA (High Efficiency Particulate Air). En los últimos 6 años (2006-2010) se han atendido 3.674 pacientes, media de 612 pacientes año, con una ocupación media anual del 83,4% y mortalidad media anual del 4,9%. Al ser considerado un servicio polivalente, se ha establecido una estrecha colaboración prácticamente con la totalidad de los servicios y especialidades pediátricas médico-quirúrgicas del hospital, con mayor o menor frecuencia según las patologías asistidas, destacando entre las patologías asistidas, destacado entre las patologías quirúrgicas los pacientes con asistencia ventricular (Berlin Hear), ECMO (Extracorporeal Membrane Oxygenation) y trasplante cardiaco; así como tratamiento psotoepratorio de los trasplantes hepáticos, renales, intestinales y multiviscerales. Entre las líneas de trabajo, hay que destacar el tratamiento intracoronario con células progenitoras autólogas de médula ósea en pacientes con miocardiopatía dilatada e insuficiencia cardíaca; creación de un equipo mixto, médico y de enfermería para el abordaje guiado por ecografía, de accesos venosos centrales insertados periféricamente; registro de una patente europea de válvula de cierre ultrarrápido y sin fugas para respiradores; utilización a nivel pediátrico de la tecnología ventilatoria NAVA (Neurally Adjusted Ventilatory Asist); monitorización hemodinámica con el monitor PiCCO2 (Pulsion Medical System); la promoción de la ecuación médica basada en la simulación de ata fidelidad y la implantación de la asistencia ventricular externa en Pediatría (AU)

The Pediatric Intensive Care Unit of La Paz Universitary Hospital, was founded in 1974. It is now a polyvalent medical-surgical tertiary unit, equipped with 4 intermediate care beds, 8 intensive care beds and 4 isolation beds with airlock and HEPA filters for the treatment of transplanted and immunocompromised patients. In the last 6 years, 3674 patients have been treated (mean 612 patients/year) with a mean occupancy rate of 83,4% and a mortality rate of 4,9&. As the polyvalent unit it is, PICU staff maintains close and daily contact and cooperation with almost all the rest of the medical and surgical departments of the hospital. In this regard Critically ill cardiac patients are admitted in the PICU ( for the treatment with ECMO and ventricular assist devices (EXCOR Berlin Heart), as well as post-operative patients after cardiac, liver, renal, intestinal and multivisceral transplantation. Of all the achievements and current lines of work of the department, it can be highlighted the intracoronary treatment with autologous bone marrow derived progenitor cells in patients with dilated cardiomyopathy, the implantation development of a pediatric ventricular assist program, the echo guided cannulation of peripherally inserted central venous catheter by a especially trained intravenous therapy team, the registration of a European patent for a ultrarapid shutoff respirator valve, the incorporation of NAVA mode for mechanically ventilated patients and the PiCCO2 monitor for the hemodynamic monitorization of patient with cardiovascular dysfunction, and the promotion of medical education based on high-fidelity simulation (AU)

[Pyroglutamic acidemia associated with acetaminophen]. / Acidosis piroglutámica asociada a paracetamol.

We report a case of pyroglutamic acidemia probably related to acetaminophen administration. A 16-month boy recovering from hemolytic uremic syndrome abruptly developed unexplained high anion gap metabolic acidosis requiring hemodialysis. Septic shock, lactic acidosis and salicylate intoxication were ruled out. Betahydroxybutyrate and acetoacetate levels were within the normal range. No osmolarity gap or high amino acid levels were found. Urine and blood pyroglutamic acid levels were 392 mmol/mol creatinine (reference range: 9-55) and 9.8 mmol/L (reference range<0.16), respectively. The patient was receiving acetaminophen. We conclude that pyroglutamic acidosis should be considered in patients receiving acetaminophen who abruptly develop high anion gap metabolic acidosis not attributable to more common causes.

Acidosis piroglutámica asociada a paracetamol / Pyroglutamic acidemia associated with acetaminophen

El objetivo del manuscrito es comentar un caso de acidosis piroglutámica probablemente relacionada con la administración de paracetamol. Un niño de 16 meses en fase de recuperación de un síndrome hemolítico urémico desarrolló bruscamente acidosis metabólica con anión gap aumentado que precisó hemodiálisis. El paciente no presentaba signos sugerentes de sepsis y la acidosis láctica y la intoxicación por salicilatos se descartaron. Las concentraciones de betahidroxibutirato, acetoacetato, aminoácidos séricos y el osmol gap fueron normales. La determinación urinaria y sérica de ácido piroglutámico fue patológica: 392 mmol/mol creatinina (rango de referencia: 9-55) y 9,8 mmol/l (referencia < 0,16), respectivamente. El paciente estaba recibiendo paracetamol. La acidosis piroglutámica se debe descartar en el paciente que esté recibiendo paracetamol y desarrolle de forma brusca acidosis metabólica con anión gap aumentado sin otra causa precipitante

We report a case of pyroglutamic acidemia probably related to acetaminophen administration. A 16-month boy recovering from hemolytic uremic syndrome abruptly developed unexplained high anion gap metabolic acidosis requiring hemodialysis. Septic shock, lactic acidosis and salicylate intoxication were ruled out. Betahydroxybutyrate and acetoacetate levels were within the normal range. No osmolarity gap or high amino acid levels were found. Urine and blood pyroglutamic acid levels were 392 mmol/mol creatinine (reference range: 9-55) and 9.8 mmol/L (reference range < 0.16), respectively. The patient was receiving acetaminophen. We conclude that pyroglutamic acidosis should be considered in patients receiving acetaminophen who abruptly develop high anion gap metabolic acidosis not attributable to more common causes

[Fisher's index as indicator of severity and its course in the postoperative period following liver transplantation in children]. / Indice de Fisher como indicador de gravedad y su evolución en el postoperatorio inmediato del trasplante hepático en niños.

OBJECTIVES: To study the evolution of Fisher index during the early postoperative period after liver transplantation in children. PATIENTS AND METHODS: We have studied 32 liver transplants performed on 26 pediatric patients, during the first week of the post-operative period. In all cases the BCAA/AAA quotient was determined before transplant, at the time of PICU admission, and on days 1, 2, 3, 4, 5, 6 and 7 after transplant. RESULTS: Compared to control group values, the Fisher index for these patients is significantly lower at pre-transplant (p < 0,0001), upon admission (p < 0,001), and days 1 (p < 0,0001), 2 (p < 0,0001) and 3 (p < 0, 0001). Comparison between non-survivors and survivors shows a significant reduction in the first group on the index in days 1 (p < 0,02), 3 (p < 0,02), 4 (p < 0,005), 5 (p < 0,002), 6 (p < 0,03) and 7 (p < 0,01) of post-transplant. CONCLUSIONS: The evolution of the Fisher index can be useful as an indication of the severity of the condition of these kinds of patients, which would help to establish more aggressive treatment to improve prognosis.

Índice de fisher como indicador de gravedad y su evolución en el postoperatorio inmediato del trasplante hepático en niños / Fisher's index as an indicator of severity and its evolution in the postoperative period following liver transplantation in children

OBJETIVO: Estudiar la evolución del índice de Fisher y su utilidad como indicador de gravedad, durante el postoperatorio inmediato de pacientes pediátricos sometidos a un trasplante de hígado. PACIENTES Y MÉTODOS: El estudio comprende 32 trasplantes de hígado realizados en 26 niños. En todos los casos el índice BCAA/AAA fue determinado antes del trasplante, al ingreso postrasplante y los días 1, 2, 3, 4, 5, 6 y 7 después del trasplante. RESULTADOS: Comparado con el grupo control, el índice de Fisher de los pacientes fue significativamente más bajo en el pretrasplante (p < 0,0001), al ingreso (p < 0,001) y los días 1 (p < 0,0001), 2 (p < 0,0001) y 3 (p < 0,0001). Al comparar el grupo de pacientes vivos con el grupo de pacientes fallecidos, se evidencia una disminución significativa en el grupo de pacientes fallecidos los días 1 (p < 0,02), 3 (p < 0,02), 4 (p < 0,005), 5 (p < 0,002), 6 (p < 0,03) y 7 (p < 0,01). CONCLUSIÓN: La evolución del índice de Fisher puede servir como parámetro indicador de gravedad en pacientes pediátricos sometidos a un trasplante de hígado, lo que permitiría establecer un tratamiento más intensivo que pudiera mejorar el pronóstico de estos enfermos (AU)

[Amino acid changes in blood of children with severe liver disease. The evaluation of differences according to distinct physiopathology]. / Alteraciones de los aminoácidos en sangre en niños con enfermedad hepática severa. Valoración de las diferencias en relación a la distinta fisiopatología.

OBJECTIVE: The aim of this study was to know the serum amino acid profiles in children with terminal hepatic diseases and to assess the differences between the two main physiopathological groups of hepatic damage: cholestasis and cellular necrosis. PATIENTS AND METHODS: We studied twenty-six pediatric patients with severe hepatic diseases admitted to the Pediatric Intensive Care Unit. Patients were divided into two groups according to the predominant hepatic lesion: cellular damage (fourteen children) and cholestasis damage (twelve cases). RESULTS: Overall, there is a significant increase in the aromatic amino acids (AAA) phenylalanine (p < 0.04) and tyrosine (p < 0.0003) and a decrease in the branched-chain amino acids (BCAA) leucine, isoleucine and valine (p < 0.00001), with a reduction in the BCAA/AAA ratio (p < 0.00001). However, we found a significant decrease in glutamine, cysteine, taurine, serine, threonine, tryptophan, total amino acids and essential amino acids, together with higher levels of glutamic acid, ornithine and citrulline, which reflects a more complex metabolic disturbance. The group with cholestatic damage shows very low taurine levels (p < 0.0003). Patients with predominantly cellular damage have higher increases in tyrosine (p < 0.01), phenylalanine and hydroxyproline (p < 0.01). CONCLUSIONS: These findings may help us to better understand the complex physiopathology of amino acid metabolism in different liver diseases. Moreover, the extremely low levels of taurine found prompted us to recommend additional dietary support particularly in children with cholestatic hepatopathy.

[Status asthmaticus; experience in a pediatric intensive care unit]. / Status asmático: experiencia en una unidad de cuidados intensivos pediátricos.

Thirty children presenting status asthmaticus in fifty episodes were studied during a period of three and a half years. All of them showed acute respiratory failure secondary to asthma. The purpose was to evaluate treatment results and complications compared with a previous similar study conducted in our unit. We have noticed that there is a lower number of admissions for SA in our ICU and a different seasonal tendency. Precious treatment of asthmatic crisis selective inhaled beta 2 bronchodilators can prevent the ICU admission of an asthmatic patient.

[Alpha 1-antitrypsin deficiency with histologic expression of metabolic disease of carbohydrates]. / Déficit de alpha-1-antitripsina con expresividad histológica de enfermedad metabólica de hidratos de carbono.

A two months old male affected by alpha-1-AT PiZZ deficiency with severe transient neonatal cholestasis is presented. Two hepatic biopsies were practiced in neonatal period. There was no evidence of PAS positive globules, but an intense univacuolar steatosis and a rossetoid transformation of hepatocytes were observed. Both findings are identical to those found in the histopathologic study of the liver in certain metabolic diseases such as fructosemia and galactosemia. A third biopsy practiced at an age of two years confirmed diagnosis of alpha-1-AT deficit since presence of PAS positive globules was established. It must be pointed out that histopathological findings show great variability among different patients with alpha-1-AT deficit in the neonatal period, as well as the infrequent presence of PAS positive globules in hepatic biopsies of those c during the first months of life.