Early post-operative neurodevelopment and visual assessment in neonates with congenital heart disease undergoing cardiac surgery.

OBJECTIVE: Assessment of neurobehavior and visual function of newborns with congenital heart disease during the post-operative period to identify infants at risk of neurodevelopmental and visual impairment. STUDY DESIGN: Prospective study that included 45 newborns who underwent cardiac surgery. Newborn Behavioral Observations test (NBO) and "ML Battery of Optotypes" were used for assessment. RESULTS: The median NBO global score was 2.4 [2.1-2.6]. Total days of oral morphine [p = 0.005] and total days of sedation [p = 0.009] were strongly related to abnormal evaluations. Time of cerebral regional oxygen saturation (CrSO2) under 40% during surgery and increased lactate were related to abnormal motor evaluation. Only 14.5% of patients presented pathological results in visual evaluation. CONCLUSIONS: We have demonstrated alterations in attention, autonomic, motor, and oral motor function. Duration of sedative medication, time of CrSO2 under 40% during surgery, and increased lactate are the most important risk factors. No significant visual impairment was detected.

Treatment with bisphosphonates in severe hypercalcemia due to subcutaneous fat necrosis in an infant with hypoxic-ischemic encephalopathy.

Subcutaneous fat necrosis (SCFN) is a rare entity that occurs generally in term or post-term newborns exposed to perinatal stressing factors. These cutaneous lesions appear during the first weeks of life and their potential complications, such as hypercalcemia, determine the prognosis. We present a full-term newborn with SCFN lesions that appeared at the age of 12 days and who, 1 week later, developed moderate hypercalcemia. In our patient, the standard treatment was not enough to normalize calcemia and, in order to prevent secondary effects, etidronate therapy was initiated and it successfully normalized calcium levels. When SCFN is diagnosed, it is important to detect early hypercalcemia and treat it aggressively. This case provides further evidence of etidronate as an alternative and effective treatment for moderate-severe hypercalcemia.

Hipotermia cerebral moderada en la encefalopatía hipóxico-isquémica. Experiencia en el primer año de su puesta en marcha / Moderate cerebral hypothermia in hypoxic-ischaemic encephalopathy: Experience after one year

Introducción: La hipotermia cerebral moderada ha probado ser una intervención eficaz para reducir la mortalidad y la discapacidad mayor en los neonatos con encefalopatía hipóxico-isquémica (EHI) moderada-grave. Objetivos: Describir la experiencia en el primer año de su utilización y valorar la factibilidad y seguridad de esta intervención. Métodos: Revisión de los 20 pacientes con EHI moderada-grave tratados con hipotermia corporal total en la Agrupación Sanitaria Hospital Sant Joan de Déu-Hospital Clínic, entre enero de 2009 y junio de 2010. Resultados: Durante este periodo ingresaron 50 neonatos con EHI perinatal, en 26 de ellos moderada-grave. Un total de 20 neonatos recibieron hipotermia (13 con EHI grave y 7 moderada). En todos ellos se encontró un antecedente de riesgo de hipoxia-isquemia perinatal y algún signo clínico de EHI. Quince neonatos presentaron convulsiones clínicas y/o en el registro electroencefalográfico. La temperatura rectal se mantuvo en 33,5±0,5°C en el 76,5% de las determinaciones para los neonatos con control manual de la temperatura y en el 93,6% para los manejados con servocontrol (p<0,0001). El recalentamiento se realizó en una mediana de 10,5 horas. No se produjo ninguna complicación potencialmente grave relacionada con la hipotermia. Fallecieron 7 neonatos (35%), todos ellos con EHI grave. Conclusiones: No se han apreciado dificultades en ninguna de las fases de esta intervención terapéutica ni se ha registrado ninguna complicación potencialmente grave relacionada con ella. Tanto el control manual de la temperatura como su servocontrol son eficaces para mantener la temperatura diana, pero esta muestra una menor variabilidad con el equipo servocontrolado(AU)

Introduction: Moderate cerebral hypothermia has been shown to be an effective intervention in decreasing mortality and major disabilities in infants with moderate-severe hypoxic-ischaemic encephalopathy (HIE). Objectives: To describe our experience within the first year of implementation, and to evaluate the feasibility and safety of this intervention. Methods: Retrospective study of 20 patients with moderate-severe HIE treated with whole body hypothermia in the Agrupación Sanitaria Hospital Sant Joan de Déu-Hospital Clínic, between January 2009 and June 2010.ResultsDuring this period, 50 patients with perinatal HIE, 26 of them moderate- severe, were admitted to our units. Twenty patients received hypothermia (13 with severe and 7 with moderate HIE). All of them had at least one risk factor for perinatal hypoxia-ischaemia, and clinical signs of HIE. Fifteen had clinical and/or EEG seizures. Core temperature was maintained at 33.5±0.5°C in 76.5% of determinations for infants cooled with a manual control device, and in 93.6% for those cooled with a servo-controlled device (P<0,0001). Re-warming took a median time of 10.5hours. No potentially severe complications related to hypothermia were observed. Seven patients (35%) died, all of them with severe HIE. Conclusions: There were no difficulties in any of the steps of this intervention, and no potentially severe complications related to it were recorded. Both manual and servo-control methods are equally effective on maintaining the target temperature, although temperature shows less variability using the servo-controlled equipment(AU)

[Moderate cerebral hypothermia in hypoxic-ischaemic encephalopathy: Experience after one year]. / Hipotermia cerebral moderada en la encefalopatía hipóxico-isquémica. Experiencia en el primer año de su puesta en marcha.

INTRODUCTION: Moderate cerebral hypothermia has been shown to be an effective intervention in decreasing mortality and major disabilities in infants with moderate-severe hypoxic-ischaemic encephalopathy (HIE). OBJECTIVES: To describe our experience within the first year of implementation, and to evaluate the feasibility and safety of this intervention. METHODS: Retrospective study of 20 patients with moderate-severe HIE treated with whole body hypothermia in the Agrupación Sanitaria Hospital Sant Joan de Déu-Hospital Clínic, between January 2009 and June 2010. RESULTS: During this period, 50 patients with perinatal HIE, 26 of them moderate- severe, were admitted to our units. Twenty patients received hypothermia (13 with severe and 7 with moderate HIE). All of them had at least one risk factor for perinatal hypoxia-ischaemia, and clinical signs of HIE. Fifteen had clinical and/or EEG seizures. Core temperature was maintained at 33.5 ± 0.5°C in 76.5% of determinations for infants cooled with a manual control device, and in 93.6% for those cooled with a servo-controlled device (P<.0001). Re-warming took a median time of 10.5 hours. No potentially severe complications related to hypothermia were observed. Seven patients (35%) died, all of them with severe HIE. CONCLUSIONS: There were no difficulties in any of the steps of this intervention, and no potentially severe complications related to it were recorded. Both manual and servo-control methods are equally effective on maintaining the target temperature, although temperature shows less variability using the servo-controlled equipment.

Phosphorylation of HtrA2 by cyclin-dependent kinase-5 is important for mitochondrial function.

The role of the serine protease HtrA2 in neuroprotection was initially identified by the demonstration of neurodegeneration in mice lacking HtrA2 expression or function, and the interesting finding that mutations adjacent to two putative phosphorylation sites (S142 and S400) have been found in Parkinson's disease patients. However, the mechanism of this neuroprotection and the signalling pathways associated with it remain mostly unknown. Here we report that cyclin-dependent kinase-5 (Cdk5), a kinase implicated in the pathogenesis of several neurodegenerative diseases, is responsible for phosphorylating HtrA2 at S400. HtrA2 and Cdk5 interact in human and mouse cell lines and brain, and Cdk5 phosphorylates S400 on HtrA2 in a p38-dependent manner. Phosphorylation of HtrA2 at S400 is involved in maintaining mitochondrial membrane potential under stress conditions and is important for mitochondrial function, conferring cells protection against cellular stress.

Cauterization of meso-ovarian vessels, a new model of intrauterine growth restriction in rats.

Intrauterine growth restriction (IUGR) remains an important cause of perinatal morbidity and mortality. Both IUGR and low birth weight have been identified as risk factors for increased incidence of cardiovascular disease, dyslipemia, and other diseases in the adulthood. Several animal models have been developed to study the underlying mechanisms of IUGR and its later consequences, with utero-placental ischemia by uterine artery ligation (UAL) being the most frequently used in rats. The relevance of this model lies in the fact that it induces altered placental perfusion, the main cause of IUGR in humans in Western countries. However, there is also controversy over the grade and homogeneity of IUGR obtained. In this study, we propose a new animal model of IUGR related to placental ischemia through the cauterization of meso-ovarian vessels. We aimed to test the feasibility of meso-ovarian vessel cauterization (CMO), and to compare it with uterine artery ligation (UAL). The CMO group had similar incidence of perinatal mortality, percentage of IUGR, and evolution of body weight during early extrauterine life to the UAL group, indicating that both methods are similarly efficient for inducing IUGR. Moreover, both of them affect the ratio of fetal to placental weight, and the weight of vital organs, supporting the hypothesis of a fetal compensatory response or "brain- and heart-sparing effect". Both operative models suffer approximately 50% perinatal mortality, implying that they are both more efficient in the production of IUGR when C-section is performed. On the other hand, CMO was significantly faster to perform than UAL and seemed to produce a more uniform ischemia throughout the uterus than the UAL method, resulting in a more homogeneous group of IUGR pups.

Reducing errors in the management of hyperbilirubinaemia: validating a software application.

OBJECTIVE: To verify the usefulness and reliability of a software tool we developed to help to apply the American Academy of Pediatrics (AAP) Guidelines 2004 on hyperbilirubinaemia according to the infant's age in hours and their clinical risk factors. DESIGN: Randomised, cross-over, controlled trial with 20 simulated clinical cases comparing the "manual" application of the guidelines with our software application. PARTICIPANTS: Fifteen doctors (eight final-year residents and seven consultants) from two hospitals in Spain. MAIN OUTCOME MEASURES: Major errors (defined a priori as any deviation from the AAP guidelines that involve a risk of morbidity or mortality for the patient), minor errors (those that cause discomfort and/or, in extremely rare cases, morbidity) and time spent. RESULTS: Fifteen doctors each managed 20 simulated cases, half by using the guidelines alone and half using the software tool. Without the software application, 42 "minor" errors were made. With it, only 25 errors were made. "Major" errors also decreased from 10 to 2 with the software. As a group, the residents benefited most; they made an average of 1.8 errors fewer per 10 cases. Use of the software reduced the time taken by the residents to resolve the cases, although the mean reduction in time was not significant for the group of consultants. CONCLUSIONS: The use of simulated clinical cases revealed many errors in the routine management of hyperbilirubinaemia. The software helped clinicians make fewer errors and saved time for residents, but not consultants.

Síndrome de neck-tongue, una entidad que se debe recordar / Neck-Tongue syndrome, a disorder to remember

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[Cholesterol is still high. So what do we do now? Treatment of uncontrolled hypercholesterolaemia over a year]. / El colesterol sigue alto. Y ahora qué hacemos? Tratamiento de la hipercolesteremia no controlada a lo largo de un año.

OBJECTIVE: To find the effectiveness of lipid-lowering treatment, based on normal clinical practice in primary care, on lipid control of patients with clear hypercholesterolaemia (HC). Design. Semi-experimental before-and-after intervention study. SETTING: Urban health centre. PARTICIPANTS: 187 patients known to have lipaemia, with total or LDL cCholesterol (cLDL) above 270 and 190 mg/dl, respectively.Intervention. Normal clinical practice for twelve months in nine primary care clinics. MAIN MEASUREMENTS: The lipid profile and lipid-lowering treatment were recorded at the start of the study and after twelve months. Lipid control (as a function of cLDL) was evaluated as optimal, acceptable or deficient, as a function of the cardiovascular risk, following the criteria of the Spanish Arteriosclerosis Society (1994). RESULTS: In 27% of cases, no visit relating to HC was recorded by the patient s doctor. The number of patients treated with lipid-lowering drugs grew from 50 to 98 (27 vs 52%, p < 0,005), fundamentally at the expense of statin treatment. After twelve months, there were significant drops in the plasma concentration of cLDL (12%, 95%CI, 9 to 15%) and in the percentage of patients with deficient control, which fell from the initial 91% to 61% (p < 0.005), although only 16% reached optimal control. CONCLUSIONS: After a year, under conditions of normal clinical practice, there was an increase in the use of lipid-lowering drugs and improvement in lipid control, though a bit over half the patients (61%) with clear hypercholesterolaemia maintained concentrations requiring treatment.

El colesterol sigue alto. ¿Y ahora qué hacemos? Tratamiento de la hipercolesteremia no controlada a lo largo de un año / Cholesterol is still high. So what do we do now? treatment of uncontrolled hypercholesterolaemia over a year

Objetivo. Conocer la efectividad sobre el control lipídico del tratamiento hipolipemiante, basado en la práctica clínica habitual en atención primaria, en pacientes con hipercolesteremia manifiesta. Diseño. Intervención semiexperimental, antes-después. Emplazamiento. Centro de salud urbano. Participantes: 187 pacientes dislipémicos conocidos, con colesterol total o colesterol LDL (cLDL) > 270 o 190 mg/dl, respectivamente .Intervención. Práctica clínica habitual durante 12 meses en 9 consultas de atención primaria. Mediciones principales. Se registró el perfil lipídico y el tratamiento hipolipemiante al inicio del estudio y al cabo de 12 meses. El control lipídico (en función del cLDL) se evaluó como óptimo, aceptable y deficiente en función del riesgo cardiovascular según los criterios de la Sociedad Española de Arteriosclerosis (1994).Resultados. En un 27 por ciento de casos no se registró ninguna visita relacionada con la hipercolesteremia por su médico. El número de pacientes tratados con hipolipemiantes creció de 50 a 98 (27 frente a 52 por ciento; p < 0,005), fundamentalmente a expensas del uso de estatinas. Tras 12 meses, se observaron descensos significativos en la concentración plasmática del cLDL (12 por ciento; IC del 95 por ciento, 9-15 por ciento) y del porcentaje de pacientes con control deficiente, que descendió del 91 por ciento inicial al 61 por ciento (p < 0,005), aunque sólo un 16 por ciento alcanzó un control óptimo. Conclusiones. Tras un año, con las condiciones de práctica clínica habitual, se observó un incremento en el uso de hipolipemiantes y una mejoría en el control lipídico, aunque algo más de la mitad de los pacientes (61 por ciento) con hipercolesteremia manifiesta permanecen con concentraciones tributarias de tratamiento (AU)

[Guidelines of the Spanish Society of Cardiology for clinical practice in exercise testing]. / Guías de práctica clínica de la Sociedad Española de Cardiología en pruebas de esfuerzo.

Most exercise testing is performed in adults with known or suspected ischemic heart disease. In the last few years cardiac imaging techniques have been applied in this field, improving the information obtained with the procedure. However, the exceptions to this rule are emerging rapidly not only in healthy people (asymptomatic individuals, athletes, handicapped people) but also in cardiac patients (advanced congestive heart failure, hypertension, rhythm disorders, congenital heart disease, etc.). All the-se issues justify the need for a multidisciplinary consensus document in Spain. This paper reviews and updates the methodological aspects of the stress test, including those related to oxygen consumption measurements. The main aim of this review was to determine the role of exercise testing in the evaluation of ischemic heart disease as well as the applications of imaging stress testing. The usefulness of this test in other non-ischemic cardiac disorders and in selected subsets of healthy people is also reviewed.

Lipoprotein(a) and the significance of the association between platelet glycoprotein IIIa polymorphisms and the risk of premature myocardial infarction.

Platelet glycoprotein IIb/IIIa may be involved in the pathogenesis of myocardial infarction as the key element in platelet aggregation and as the binding site of lipoprotein(a) to platelets, inhibiting plasminogen binding and activation. Recently, a strong association between the P1A2 polymorphism of the glycoprotein IIIa gene and acute coronary thrombosis has been reported. although this has not been confirmed. In an associated study, we determined plasma lipoprotein levels, the apo E genotype and the P1A genotype in 250 males under 55 years with myocardial infarction and they were compared with 250 age- and sex-matched controls. Patients showed an over-representation of the epsilon3/4 genotype with respect to the control group. We found that there were no differences in the allelic frequency of P1A2 between case patients and age-matched controls (chi2 = 0.05, P = 0.92) and that subjects bearing the P1A2 allele showed higher plasma lipoprotein(a) concentration than p1A1/P1A1 individuals. Therefore, in this population there is no association between carriage of p1A2 allele and increased risk of myocardial infarction but the carriage of P1A2 is associated with higher plasma Lp(a) concentration.

The treatment of refractory depression using paroxetine with lithium augmentation.

1. The case is described of a 68-year-old woman with DSM-III-R major depression who was treatment-resistant to paroxetine. 2. Low dose lithium addition, at a plasma level of 0.2 mM, led to a rapid and sustained remission of her illness.

The Mediterranean-type diet: is there a need for further modification?

The effects on plasma lipoproteins of four fat-modified diets were assessed in 11 nuns in a contemplative order in the Mediterranean region of Spain. Diet 1 [high polyunsaturated fatty acid (PUFA), low monounsaturated fatty acid (MUFA), low ratio of PUFAs to saturated fatty acids (P:S)] and diet 3 (low PUFA, high MUFA, low P:S) induced significant, directly comparable reductions in total plasma (12% and 13%, respectively) and low-density-lipoprotein (LDL) cholesterol (24% and 19%, respectively). Diet 2 [high PUFA, high MUFA, low saturated fatty acid (SFA), high P:S] induced greater decrements (23% and 30% in total plasma and LDL cholesterol, respectively). Diet 4 (low PUFA, low MUFA, high SFA, low P:S) induced a significant increase in LDL cholesterol of 11%. No significant changes in high-density-lipoprotein cholesterol were observed with these diets. Because the effects of PUFAs and MUFAs are comparable, no recommendations on modifying the habitual, high-MUFA-containing Mediterranean diet need be made other than, perhaps, a reduction in the overall intake of SFAs.

[Acute myocardial infarct in a young woman addicted to cocaine]. / Infarto agudo de miocardio en una joven adicta a la cocaína.

A 28-year-old female, addict to cocaine and with acute myocardial infraction is reported. The patient was obese and smoked 17 cigarette packs per year. She had been consuming about 250 mg of cocaine daily for the last months until 2 weeks before admission. Coronary angiography showed a 100% stenosis of the left anterior descending artery. Although the vasospastic effect of cocaine on the pathogenesis of coronary ischemia in addicts is well known, most patients reported in the literature show coronary artery stenosis. This may lead to hypothesize about a likely atherogenic role of cocaine.