Leucoencefalopatía posterior reversible en niño con cardiopatía congénita y estenosis traqueal / Posterior reversible leukoencephalopathy in child with congenital heart disease and tracheal stenosis

La leucoencefalopatía posterior reversible (LPR) es un síndrome clínico-radiológico, infrecuente en la edad pediátrica, que se caracteriza por una rápida instauración de cefalea, alteraciones visuales y del nivel de conciencia, hipertensión arterial y crisis convulsivas. Presentamos el caso de un paciente de 13 años con presentación atípica del cuadro y describimos la fisiopatología y las lesiones radiológicas, destacando el característico carácter reversible del cuadro y la importancia de la sospecha clínica y el diagnóstico precoz para el adecuado manejo de estos pacientes (AU)

Reversible posterior leukoencephalpathy syndrome (RPLS) is a clinical-radiological syndrome that is uncommon in the pediatric age. It is characterized by rapid onset of headache, visual and level of awareness alterations, high blood pressure and seizures. We present the case of a 13 year old male patient with atypical presentation of the picture. We describe the pathophysiology and radiological lesions, stressing the characteristic reversible character of the picture and the importance of the clinical suspicion and early diagnosis for the adequate management of these patients (AU)

Infective endocarditis in congenital heart disease: a frequent community-acquired complication.

BACKGROUND: Infective endocarditis (IE) is a severe complication in patients with congenital heart disease (CHD). Epidemiology, etiology, and outcome in this group are different to those of patients with acquired heart disease. METHODS: We reviewed all cases of proven and probable IE (Duke's criteria) diagnosed in our center during the last two decades. RESULTS: We observed 45 cases of IE in patients with CHD (age range 8 months to 35 years); these represented 5.5 % of all the episodes of IE in our institution during the study period. The most frequent CHD were ventricular septal defect (31 %), tetralogy of Fallot (19 %), and atrioventricular septal defect (11 %). Twenty cases of IE (44 %) were recorded in patients with non-corrected native-valve CHD. Of the 24 patients with prosthetic-valve IE, post-operative acquisition during the first 6 months was confirmed in 11 patients (range 4-110 days). IE was community-acquired in 62 % of cases. Streptococcus spp. were the most frequent etiologic agents (33 %), followed by Staphylococcus spp. (32 %). Surgery was required to treat IE in 47 % of patients (52 % in prosthetic-valve IE and 41 % in native-valve IE, p = ns). In comparison to native-valve IE, prosthetic-valve IE was significantly more nosocomial-acquired (61 vs. 14 %, p = 0.002), presented a higher heart failure rate at diagnosis (39 vs. 9 %, p = 0.035), and developed more breakthrough bacteremia episodes (19 vs. 0 %, p = 0.048). Global mortality was 24 % (75 % in patients with prosthetic-valve IE who required surgery and 0 % in patients with native-valve IE who required surgery, p = 0.001). Multivariate analysis excluding breakthrough bacteremia (100 % mortality in this condition) confirmed that nosocomial IE [odds ratio (OR), 23.7; 95 % confidence interval (CI), 2.3-239.9] and the presence of heart failure at diagnosis of IE (OR, 25.9; 95 % CI, 2.5-269.6) were independent factors associated with mortality. CONCLUSION: Half of all cases of IE in patients with CHD occurred in patients with non-corrected native-valve CHD and two-thirds were community-acquired. Streptococcus spp. were the most frequent etiological agents. Patients with prosthetic-valve IE present a worse outcome, especially those requiring surgery. Breakthrough bacteremia, nosocomial IE, and heart failure are independent factors of mortality in patients with CHD presenting IE.

[Care of the critically ill child with heart disease in Spain]. / La asistencia al niño crítico con cardiopatía en España.

INTRODUCTION: The objective of the present study is to present the organization of the resources of paediatric cardiac critical care in Spain. PATIENTS AND METHODS: Data were collected through questionnaires sent by e-mail to Spanish PICUs. RESULTS: 22 PICUs were enrolled. The median number of beds were 9.5 (4-18 beds). Total cardiac admissions represented a 20 % of total PICUs admissions per year, firstly for congenital heart defects, and secondly for respiratory problems. Cardiac surgical activities were carried out in 16 centres, centralized in PICU in 10 cases. Mechanical support of the myocardium was performed in 7 PICUs. A total of 10 participating PICUs considered echocardiograph training necessary and also an increase in the amount of activity for better results. CONCLUSIONS: Paediatric cardiac critical care involves a significant use of resources, including PICUs with no surgical activity. This study is useful for detecting common problems and for improving clinical care.

La asistencia al niño crítico con cardiopatía en España / Care of the critically ill child with heart disease in Spain

Introducción: Los niños con cardiopatía constituyen una causa frecuente de ingreso en UCIP. El objetivo de este estudio es conocer la organización de su asistencia en España. Pacientes y métodos: Se elaboró un cuestionario que se envió por correo electrónico a todas las UCIP englobadas en la Sociedad Española de Cuidados Intensivos Pediátricos. Resultados: Contestaron la encuesta 22 UCIP, con una mediana de camas de 9,5 (rango 4-18). Los ingresos de niños críticos con cardiopatía representaron el 20 % del total de ingresos anuales en las UCIP con actividad cardioquirúrgica y hasta el 10 % en UCIP sin dicha actividad. Las causas de ingreso más frecuentes fueron las cardiopatías congénitas (coartación aórtica y defectos de septo) y, en segundo lugar, problemas no cardiológicos (fundamentalmente infecciones respiratorias). Asisten el postoperatorio de cirugía cardíaca infantil 16 UCIP (4 unidades tienen programa de trasplante cardíaco pediátrico), 10 de ellas de forma centralizada en su centro. Un total de 7 unidades disponen de medios de soporte mecánico miocárdico; 10 de las UCIP encuestadas consideraron muy importante adquirir formación en ecocardiografía, así como la agrupación de los pacientes en áreas especializadas. Conclusiones: La atención al niño crítico con cardiopatía supone una utilización importante de recursos en las UCIP, incluidas aquellas que no atienden postoperatorios cardíacos. Este tipo de estudios permite identificar limitaciones comunes y favorecer la asistencia de este tipo de pacientes (AU)

Introduction: The objective of the present study is to present the organization of the resources of paediatric cardiac critical care in Spain. Patients and methods: Data were collected through questionnaires sent by e-mail to Spanish PICUs. Results: 22 PICUs were enrolled. The median number of beds were 9.5 (4-18 beds). Total cardiac admissions represented a 20 % of total PICUs admissions per year, firstly for congenital heart defects, and secondly for respiratory problems. Cardiac surgical activities were carried out in 16 centres, centralized in PICU in 10 cases. Mechanical support of the myocardium was performed in 7 PICUs. A total of 10 participating PICUs considered echocardiograph training necessary and also an increase in the amount of activity for better results. Conclusions: Paediatric cardiac critical care involves a significant use of resources, including PICUs with no surgical activity. This study is useful for detecting common problems and for improving clinical care (AU)

Encefalopatía tras sedación prolongada con midazolam / Encephalopathy after prolonged sedation with midazolam

La analgesia y la sedación son terapias necesarias para el manejo adecuado del niño críticamente enfermo. El midazolam es una benzodiacepina, ampliamente utilizada en las Unidades de Cuidados Intensivos Pediátricos, a pesar de que su uso continuado produce tolerancia y dependencia. Tras su retirada puede aparecer síndrome de abstinencia y están descritas otras alteraciones neurológicas no claramente relacionadas con la deprivación del mismo. Presentamos el caso de una niña que, tras las retirada de sedación prolongada con midazolam y fentanilo, presentó un cuadro de desconexión de medio, hipertonía generalizada, movimientos estereotipados faciales, automatismos orales, ausencia de fijación de la mirada y de movimientos intencionales, estrabismo y, posiblemente, alucinaciones. La paciente no tuvo síntomas típicos de síndrome de abstinencia y se descartaron otras causas de encefalopatía. Las alteraciones se resolvieron espontáneamente en 3 semanas por lo que la sintomatología se atribuyó a un efecto adverso relacionado con la administración prolongada de midazolam

Analgesia and sedation are essential parts of the management of teh critically ill child. Midazolam is a widely used benzodiazepine in the Pediatric Intensive Care Units, but its use produces tolerance and dependence. After its discontinuation, abstinence syndrome can appear and also there are reported other neurological abnormalities not clearly related with the withdrawal. We report the case of a girl that after discontinuation of prolonged sedation with midazolam and fentanyl showed diminished level of consciousness generalized hipertony, facial grimacing, poor visual tracking, absence of coordinated movements, strabismus and possibly hallucinations. The patient did not present symptoms or signs suggesting withdrawal syndrome. Other causes of encephalopathy were ruled out. All abnormalities were spontaneously resolved in 3 weeks, and were attributed to an adverse effect of prolonged midazolam infusion

[Ventilation in special situations. Mechanical ventilation in congenital cardiopathies and pulmonary hypertension]. / Ventilación en situaciones especiales. Ventilación mecánica en cardiopatías congénitas e hipertensión pulmonar.

The cardiovascular and respiratory systems act as a functional unit. Mechanical ventilation modifies pulmonary volumes, which generates changes in autonomic nervous system reactivity and provokes tachy- or brady-cardia (depending on the tidal volume used). Mechanical ventilation also decreases cardiac filling volumes (pre-load) and alters pulmonary vascular resistances. In addition, intrathoracic pressures are enlarged, which usually produces a decrease in right atrium filling and an increase in right ventricle afterload. If coronary flow is impaired, myocardial contractility is reduced. However, if cardiac failure is present, mechanical ventilation is especially beneficial because it corrects hypoxia and respiratory acidosis, decreases the work of breathing, and improves stroke volume. Mechanical ventilation in congenital heart diseases is indicated either as lifesaving support or as physiopathological treatment to modify the ratio between pulmonary and systemic flow. As a general rule, if excessive pulmonary blood flow is present, the aim of respiratory support is to increase pulmonary vascular resistance by using high levels of airway pressure and even by delivering FiO2<21%. When there is low pulmonary flow, the lowest possible intrathoracic pressures should be used, especially in cases of pulmonary hypertension, which will also require high FiO2. However, mechanical ventilation has adverse effects and consequently it must be stopped as early as possible, once the child is stable and requires minimal cardiopulmonary support. Weaning can even be performed in the operating room, when the surgical procedure is finished. When this is not possible, weaning should be performed in the pediatric intensive care unit. Because there are no criteria for successful withdrawal of mechanical support in congenital heart disease, general pediatric criteria should be used.

Adolescente con pérdida brusca de la conciencia y crisis comicial tras traumatismo craneoencefálico / Sudden loss of consciousness and comitial crisis after head injury in a teenager

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Prognosis of patients with acute renal failure without cardiopathy.

BACKGROUND: The outcome for children with acute renal failure (ARF) may be poor. However, relatively few published studies have considered prognosis of these patients. METHODS: We prospectively studied, from 1978 to 1998, 92 such children without heart disease to try to identify risk factors for mortality. RESULTS: Forty five per cent of children with tumours, shock, and other causes died compared with none of those with a primary urinary tract related problem. ARF did not seem to be the cause of death in any case. Univariate analysis showed that in the non-primary urinary problem group (55 cases), patients with hypotension, high values of BUN or creatinine, or who needed mechanical ventilation or dialysis, had a poor outcome. Multivariate analysis showed that probability of death can be estimated using the following score: -0.02 + 0.28 (hypotension) + 0.19 (ventilation) + 0.27 (dialysis) + 0.01 (BUN). CONCLUSIONS: Mortality of patients with ARF was related to aetiology, the need for dialysis and/or ventilator use, hypotension, and BUN values.

Hipernatremia grave por administración accidental de sal común / Fatal hypernatremia due to exogenous salt intake

La hipernatremia es una alteración electrolítica común, aunque rara vez se debe a un exceso absoluto de sodio. La hipernatremia grave debida a ingestión de sal común, bien de forma accidental o bien como forma de maltrato infantil, es una entidad infrecuente pero que conlleva una alta mortalidad, así como dificultades para conseguir un tratamiento terapéutico óptimo. Apesar de ser fácilmente reconocible mediante las pruebas de laboratorio, la determinación de su etiología es con frecuencia muy difícil de establecer. Es importante tener presente que, incluso una ingesta sorprendentemente pequeña de sal puede provocar una hipernatremia aguda grave con desenlace fatal. Se describen 2casos de intoxicación salina grave en 2niñas de 20y 7meses de edad, respectivamente, cuya primera manifestación fue hipernatremia grave y convulsiones tras la ingesta accidental de sal común. En el caso1, la administración inadvertida de sal común, en lugar de azúcar, a dos yogures provocó una hipernatremia aguda y estado convulsivo. En el caso2, un error en la preparación de la solución de rehidratación oral (agua saturada con sal) originó un cuadro de convulsiones, coagulopatía de consumo y trombosis del seno venoso longitudinal. Ambos casos fallecieron con el diagnóstico de muerte encefálica (AU)

[Fatal hypernatremia due to accidental administration of table salt]. / Hipernatremia grave por administración accidental de sal común.

Hypernatremia is a common electrolyte abnormality, but it is rarely attributable to excess sodium. Hypernatremia due to exogenous salt intake, caused either by accidental ingestion or as a form of child abuse, is rare, difficult to manage and results in high mortality. Although hypernatremia is easily recognized by laboratory tests, its etiology is often difficult to determine. A surprisingly small amount of salt intake can result in a fatal outcome. We report two cases of severe salt intoxication in two girls, aged 20 and 7 months, who presented with severe hypernatremia. Both had seizures after accidental salt ingestion. In the first case, salt instead of sugar was inadvertently added to two yoghurts, leading to hypernatremia and convulsions. In the second case, a mistake in the preparation of salt-saturated water as an oral rehydration solution provoked seizures, coagulopathy and longitudinal venous sinus thrombosis. Both cases developed encephalic death. We discuss the clinical course and the difficulties in the treatment of these cases in the context of the available literature.

Age-related increase in expression of TGF-beta1 in the rat kidney: relationship to morphologic changes.

In the kidney, aging is characterized by the development of structural changes, including glomerulosclerosis and interstitial fibrosis. Transforming growth factor-beta1 (TGF-beta1) is known to play a critical role in the genesis of these alterations in pathologic conditions. The present experiments were designed to test the hypothesis that TGF-beta1 may be involved in the development of age-related histopathologic changes in rat kidney, and that captopril, an angiotensin-converting enzyme inhibitor, may influence the progression of glomerular and interstitial lesions. In this study, 3-, 18-, 24-, and 30-mo-old rats were examined, and an age-related increase in urinary protein excretion was found; plasma creatinine and systolic BP did not change. Significant structural changes, including glomerular sclerosis and interstitial fibrosis, were found in the group of aged rats (24- and 30-mo-old). Immunostaining for TGF-beta in the renal cortex interstitium was increased in the group of 24-mo-old rats, with a parallel increase in TGF-beta1 mRNA expression, measured with reverse-transcription PCR. Captopril-treated animals showed a statistically significant decrease in urinary protein excretion but no significant changes in BP. Moreover, captopril reduced the extent of interstitial fibrosis, but did not affect the degree of glomerulosclerosis. A significant inhibition of TGF-beta1 mRNA expression was observed in the captopril-treated animals. These findings suggest that TGF-beta1 may act as a fibrogenic growth factor that could be responsible, at least partially, for the renal interstitial fibrosis associated with aging. Treatment with captopril might delay the progression of these lesions.

Captopril inhibits pp60(c-src) tyrosine phosphorylation in cultured human mesangial cells.

The present experiments were devoted to analyzing the mechanisms involved in the captopril-dependent inhibition of human mesangial cell proliferation. Studies were performed in cultured human mesangial cells incubated with captopril, an angiotensin II-converting enzyme inhibitor with antioxidant properties, lisinopril, a non-antioxidant angiotensin II-converting enzyme inhibitor, and tocopherol, a pure antioxidant. Both angiotensin II-converting enzyme inhibitors significantly inhibited fetal calf serum-induced [3H]thymidine uptake by human mesangial cells, in a dose- and time-dependent manner, an effect which was not observed with tocopherol. The antiproliferative effect of captopril and its ability to block tyrosine phosphorylation of human mesangial cells proteins were significantly greater than those of lisinopril. Moreover, captopril significantly prevented the fetal calf serum-induced tyrosine phosphorylation of pp60(c-src). The present results suggest that the antiproliferative ability of captopril does not completely depend on its angiotensin II-converting enzyme inhibitor properties, pointing to a possible interaction of the drug with the intracellular mechanisms responsible for the transmission of the proliferative signals.