[Interhospital transportation of the severe pediatric patient in Catalonia]. / Transporte interhospitalario del niño crítico en Cataluña.

OBJECTIVE: To describe the organization of the interhospital transport of the pediatric patients in Catalonia. DESIGN: Description of the different phases of organization, of the organizational structure, both of the bases and of the coordinating center, and of the evolution of the model. SCOPE: System of medical emergencies and hospital net of Catalonia. PERIOD: between 1996 and 2003. PATIENTS: Patients moved by the pediatric teams. INTERVENTIONS: Analysis of the documentation used in the transports realized by the system of medical emergencies. VARIABLES OF INTEREST: Clinical information, hours and times of transport, as well as the way used for the transport. RESULTS. A total of 6,110 pediatric transports were realized by an annual average of 777. The 94% of the movements carried out for ambulances and 6% with helicopter. The number of transport was growing to lengths of the years of study. CONCLUSIONS: The model followed in the last years in Catalonia bases on the specialization of the pediatric interhospital transport. The pediatric teams contribute a value added to the own transport.

Transporte interhospitalario del niño crítico en Cataluña / Interhospital transportation of the severe pediatric patient in Catalonia

Objetivo. Describir la organización del transporte interhospitalario del niño crítico en Cataluña. Diseño. Descripción de las diferentes fases de organización, de la estructura organizativa, tanto de las bases como del centro coordinador, y de la evolución del modelo. Ámbito. Sistema de emergencias médicas y red hospitalaria de Cataluña. Período entre 1996 y 2003. Pacientes. Pacientes trasladados por los equipos pediátricos. Intervenciones. Análisis de la documentación empleada en los traslados realizados por el sistema de emergencias médicas. Variables de interés. Datos clínicos, horas y tiempos de traslado, así como el modo empleado para el traslado. Resultados. Se realizaron 6.110 traslados pediátricos con un promedio anual de 777. El 94% de los traslados se realizaron por ambulancias terrestres y el 6% con medio aéreo. El número de transportes fue creciendo a lo largo de los años de estudio. Conclusiones. El modelo seguido en los últimos años en Cataluña se basa en la especialización del transporte pediátrico interhospitalario. Los equipos pediátricos aportan un valor añadido al propio transporte

Objective. To describe the organization of the interhospital transport of the pediatric patients in Catalonia. Design. Description of the different phases of organization, of the organizational structure, both of the bases and of the coordinating center, and of the evolution of the model. Scope. System of medical emergencies and hospital net of Catalonia. Period: between 1996 and 2003. Patients. Patients moved by the pediatric teams. Interventions. Analysis of the documentation used in the transports realized by the system of medical emergencies. Variables of interest. Clinical information, hours and times of transport, as well as the way used for the transport. Results. A total of 6,110 pediatric transports were realized by an annual average of 777. The 94% of the movements carried out for ambulances and 6% with helicopter. The number of transport was growing to lengths of the years of study. Conclusions. The model followed in the last years in Catalonia bases on the specialization of the pediatric interhospital transport. The pediatric teams contribute a value added to the own transport

Bronquiolitis: factores predictivos de la duración del ingreso hospitalario / Predictive factors of duration of hospital admission for bronchiolitis

Objetivo. Determinar qué parámetros son más útiles en el momento del ingreso de niños con bronquiolitis para prever la duración de la estancia hospitalaria. Metodología. Estudio retrospectivo por revisión de historias clínicas. Se incluyen los pacientes ingresados en nuestro centro por bronquiolitis durante el año 1999. Los enfermos se dividen en dos grupos según la duración de la estancia hospitalaria: el grupo I con una estancia de 3 días o menos y el II, con más de 3 días. Se analizan datos clínicos, analíticos y radiológicos según el protocolo realizado para este estudio. Resultados. Se incluyen un total de 271 pacientes, 73 pertenecen al primer grupo y 198 al segundo. Se objetivan diferencias estadísticamente significativas en la duración de la estancia hospitalaria con relación al antecedente de prematuridad, la edad del paciente, la existencia de VRS positivo, la presencia de fiebre, la puntuación en la escala de gravedad para bronquiolitis HSJD, el hallazgo de alteraciones radiológicas, la realización de analítica y la necesidad de tratamiento con antibióticos, oxígeno y alimentación por sonda nasogástrica. No hay diferencias en cuanto al sexo, los antecedentes de cardiopatía o displasia broncopulmonar (DBP), los resultados analíticos y la necesidad de tratamiento con broncodilatadores. La presencia de tres de los siguientes factores de riesgo: prematuridad, VRS positivo, fiebre, radiografía de tórax alterada, edad 6, tiene una sensibilidad del 63.5% y una especificidad del 79.5% como predictor de la duración superior a 72 horas de un ingreso por bronquiolitis. Si se tienen en cuenta sólo dos de los factores, aumenta la sensibilidad hasta el 89.8% en detrimento de la especificidad, que baja al 46.6%. Conclusión. Analizar conjuntamente determinados factores de riesgo en el momento del ingreso de una bronquiolitis mejora las previsiones en cuanto a la duración de la estancia en el hospital y facilita una distribución más adecuada de los recursos disponibles

Objectives. To determine the clinical factors that may help anticipate the length of hospital stay in children with bronchiolitis. Methods. Retrospective study based on the analysis of medical records of all children admitted to St Joan de Deu Hospital (SJDH) with the diagnosis of bronchiolitis in 1999. Patients were divided into two groups according to the duration of the admission. Group I: children who were hospitalized for >= 3 days. Group II: children who were hospitalised for > 3 days. Clinical, laboratory, and radiological data were reviewed. Results. 271 cases (Group I, 73; Group II, 198) were reviewed. We identified a significant relationship between the length of stay and the following parameters: prematurity, age, respiratory syncitial virus (RSV) infection, fever, high score in the SJDH bronchiolitis scale, abnormal imaging, need for laboratory evaluation, administration of antibiotics, need for oxygen supplementation, and need for nasogastric tube feeding. No relationship with duration of admission was found for gender, presence of congenital heart disease, bronchopulmonary dysplasia (BPD), results of laboratory evaluation, and need for bronchodilators. These results indicate a sensitivity of 63.5% and a specificity of 79.5% as predictor of admission > 72 hours if the patient has 3 of the following risk factors: prematurity, RSV infection, fever, radiological abnormalities, age < 2 months, and more than 6 points in the SJDH bronchiolitis score at the time of admission. The presence of two risk factors increases sensitivity to 89.8%, but the specificity decreases to 46.6%. Conclusion. The analysis of risk factors at the time of admission of patients with bronchiolitis may help in anticipating the duration of stay and facilitates the allocation of resources

[Multiple intracranial tuberculomas in infancy]. / Tuberculomas intracraneales múltiples en la infancia.

INTRODUCTION: Tuberculous involvement of the CNS is most frequent in children aged between 6 months and 6 years, although it may occur at any age. It may present as meningoencephalitis, basal arachnoiditis or intracranial tuberculomas. Whilst meningitis is typical of infancy, tuberculomas and arachnoiditis are commoner in adults. It has been estimated that tuberculomas make up 3% of the cases of neurotuberculosis. The increasing use of CAT and MR has been a great help for diagnosis of this serious complication of tuberculosis. CLINICAL CASE: A 5 month old patient presented with tuberculous meningitis which had been treated with streptomycin, isoniazid, pyrazinamide and rifampicin at the usual dosage. One month later, after good initial progress, triventricular hydrocephaly was diagnosed and a ventriculoperitoneal shunt inserted. Three months after this, there was an episode of intracranial hypertension. Cranial CAT showed considerable zones of hypodense parenchyma without ventricle dilatation. On MR there were multiple, disseminated, rounded areas which were hyperintense on T2 and compatible with intracranial tuberculomas. After fresh insertion of a ventricular shunt, the patient progressed but still had a residual right hemiparesia and retarded development. CONCLUSIONS: Although intracranial tuberculomas usually occur in adults, they may be seen in children following meningoencephalitis. Occasionally, following a good initial response to tuberculostatic drugs, tuberculomas appear, although not present before, as happened in our patient. This usually occurs within the first three months, and although the mechanism is unknown, it is believed to be due to the accumulation of lymphocytes and macrophages at preexisting microscopic foci when treatment is started.

Tuberculomas intracraneales múltiples en la infancia / Multiple intracranial tuberculomas in infancy

Introducción. La afectación del sistema nervioso central por tuberculosis es más frecuente en niños entre 6 meses y 6 años, aunque puede ocurrir a cualquier edad. Puede manifestarse como meningoencefalitis, aracnoiditis basal o tuberculoma/s intracraneal/es. Mientras la meningitis es propia de la infancia, los tuberculomas y la aracnoiditis son más frecuentes en el adulto. Se calcula que el tuberculoma representa el 3 por ciento de las neurotuberculosis. El uso creciente de TAC y la RM ha sido de gran ayuda diagnóstica para esta grave complicación de la tuberculosis. Caso clínico. Paciente de 5 meses que presentó una meningitis tuberculosa tratada con estreptomicina, isoniacida, piracinamida y rifampicina en dosis habituales. Un mes después, y tras una buena evolución inicial, presentó una hidrocefalia triventricular por lo que se colocó un drenaje ventriculoperitoneal. A los tres meses, presentó un episodio de hipertensión intracraneal. La TAC craneal mostró amplias zonas de hipodensidad parenquimatosa sin dilatación ventricular y la RM múltiples y diseminadas áreas redondeadas, hiperintensas en T2 compatibles con tuberculomas intracraneales. Tras colocar nuevamente el drenaje ventricular, la evolución fue hacia una hemiparesia derecha residual y retraso del desarrollo. Conclusiones. Aunque los tuberculomas intracraneales son propios de la edad adulta, pueden aparecer en niños tras una meningoencefalitis. En ocasiones, tras una buena respuesta inicial con tuberculostáticos aparecen tuberculomas que no existían previamente, como en el caso de nuestra paciente. Generalmente esto ocurre en los primeros tres meses y aunque el mecanismo es desconocido se cree debido al acúmulo de linfocitos y macrófagos sobre focos microscópicos preexistentes una vez instaurado el tratamiento (AU)

[Neurological form of onset in haemophagocytic lymphohistiocytosis]. / Forma de debut neurológico de la linfohistiocitosis hemofagocítica.

INTRODUCTION: Haemophagocyte lymphohistiocytosis (HLH) is a hematological disorder, autosomal recessive and in which there is benign proliferation of histiocytes with intense phagocytic activity of hematopoietic cells. The clinical features include fever, pancytopenia, coagulation disorders, liver dysfunction, the presence of histiocytes and haemophagocytes in the bone marrow, lymph nodes, spleen and liver. The nervous system is always involved and sooner or later patients develop a nervous system disorder with variable symptoms which may include irritability, disorders of consciousness, convulsions, ataxia, nystagmus or signs of intracranial hypertension. CLINICAL CASE: Onset of the disease showing purely neurological features is rare. We therefore describe the case of an 8 month old baby with HLH with a purely neurological condition involving irritability, horizontal rapid eye movements and vertical saccadic movements of both eyes and focal convulsive seizures. Initial complementary examinations were normal, except for study of the CSF with a lowered protein level and cells (monocytes). Finding hepatosplenomegaly and pallor, together with the laboratory investigations, made it advisable to do a bone marrow punch biopsy to detect haemophagocytes which would be diagnostic of HLH. In spite of chemotherapy there was rapid neurological deterioration, with alterations of the white matter and hydrocephaly which required insertion of a ventriculo-peritoneal shunt. The patient died when he was 10 months old. CONCLUSIONS: The cases of HLH in which cerebromeningeal disorders alone precede systemic symptoms are extremely rare. Hence the interest in reporting this case, so that it may be borne in mind in other cases of acute neurological onset. In this case initially there was encephalitis alone, but this was rapidly followed by systemic complications.

Forma de debut neurológico de la linfohistiocitosis hemofagocítica / Neurological form of onset in haemophagocytic lymphohistiocytosis

Introducción. La linfohistiocitosis hemofagocítica (LHH) es una enfermedad hematológica, autosómica recesiva, en la que se produce una proliferación benigna de histiocitos con intensa actividad fagocítica de células hematopoyéticas. Cursa con fiebre, pancitopenia, trastornos de la coagulación, disfunción hepática, presencia de histiocitos y hemofagocitos en médula ósea, nódulos linfáticos, bazo e hígado. El compromiso del sistema nervioso es constante y todos los pacientes desarrollan más tarde o más temprano un cuadro neurológico que puede manifestarse con síntomas variables como irritabilidad, trastornos de conciencia, convulsiones, ataxia, nistagmo o signos de hipertensión intracraneal. Caso clínico. El debut de la enfermedad con un cuadro exclusivamente neurológico es poco común y por ello presentamos la observación de un lactante de 8 meses con LHH que presenta un cuadro exclusivamente neurológico, en forma de irritabilidad y movimientos oculares rápidos horizontales y sacádicos verticales de ambos ojos y crisis convulsivas focales. Los exámenes complementarios iniciales fueron normales, excepto el estudio del LCR que reveló hiperproteinorraquia y celularidad (monocitos). La evidencia de sustancia blanca con áreas de edema, necrosis y atrofia cortical [1,3,5]. una hepatoesplenomegalia y palidez, junto a la analítica, aconsejó practicar una punción biopsia de médula ósea que detectó hemofagocitos, diagnóstico de LHH. A pesar del tratamiento quimioterápico se produjo un rápido deterioro neurológico, con alteraciones en la sustancia blanca y acompañado de hidrocefalia que requirió derivación ventriculoperitoneal. El paciente falleció a los 10 meses. Conclusión. Los casos de LHH en los que el compromiso cerebromeníngeo exclusivo precede a los síntomas sistémicos es extremadamente raro, de aquí el interés en comunicarlo y tenerlo en cuenta frente a un paciente con una clínica neurológica aguda, en este caso exclusivamente de encefalitis pero que se complicó rápidamente con manifestaciones sistémicas (AU)

[Neuromuscular pathology in a critical pediatric patient]. / Patología neuromuscular del paciente crítico en pediatría.

OBJECTIVE: To describe and provide diagnosis guidelines for the neuromuscular pathology of the pediatric critical patients, manifested as extubation difficulty, based in our experience. CLINICAL CASES: A retrospective study has been performed on three patients in the Pediatric Intensive Care Unit that were diagnosed by using clinical, analytical and electromyographical findings. In the three patients the presence of the disorder was suspected due to the extubation difficulty and the hypotony. All them received vecuronium as neuromuscular blockage while dexamethasone was provided to one of them due to a nodal tachycardia. Myopathic causes were discarded in view of the normally of the muscular enzymes. The electromyography showed an axonal disorder in all three child. Neither lumbar puncture nor muscular biopsy were performed in any of them. CONCLUSIONS: The three patients were diagnosed for a drug neuropathy (neuromuscular blocked and/or corticotheraphy). There were described another causes of the critical patient polyneuropathy in the literature, but we didn't find any of them.

Fatal haemorrhagic infarct in an infant with homocystinuria.

Thrombotic and thromboembolic complications are the main causes of morbidity and mortality in patients with homocystinuria. However, it is unusual for thrombosis and infarction to be the presenting feature leading to investigation for homocystinuria and cerebrovascular lesions in the first year of life. We describe a previously healthy 6-month-old infant who presented with a large middle-cerebral-artery territory infarction and died of massive brain swelling. Homocystinuria due to cystathionine beta-synthase (CBS) deficiency was diagnosed by metabolite analysis and confirmed by enzymatic activity measurement in a postmortem liver biopsy. Homocystinuria should be considered in the differential diagnosis of venous or arterial thrombosis, regardless of age, even in the absence of other common features of the disease. We recommend systematic metabolic screening for hyperhomocysteinemia in any child presenting with vascular lesions or premature thromboembolism.

[Percutaneous catheterization of jugular and subclavian vein (author's transl)]. / Cateterización percutánea de las venas yugular interna y subclavia en pediatría.

During a three-year period, central venous catheterization was performed in 83 children by percutaneous approach through internal jugular or and subclavian veins. Internal jugular vein was catheterized in 62 children, 40 of them were under one year old and 16 were within one to five years. Complications were seen only in two occasions: accidental punction of carotid artery. Subclavian vein was used in 21 patients: 7 or the children were under one year, 7 children were between one to five years and 7 children were over five years of age. A pneumothorax and a perforation of the subclavian vein were the complications of this last group. Indications, technics and complications of central venous catheterization--either internal jugular or subclavian--are described emphasizing the advantages that they offer as compared to traditional venisection.