Evaluation of the vasoactive-inotropic score, mid-regional pro-adrenomedullin and cardiac troponin I as predictors of low cardiac output syndrome in children after congenital heart disease surgery.

OBJECTIVE: To determine the predictive value of the inotropic score (IS) and vasoactive-inotropic score (VIS) in low cardiac output syndrome (LCOS) in children after congenital heart disease surgery involving cardiopulmonary bypass (CPB), and to establish whether mid-regional pro-adrenomedullin (MR-proADM) and cardiac troponin I (cTn-I), associated to the IS and VIS scores, increases the predictive capacity in LCOS. DESIGN: A prospective observational study was carried out. SETTING: A Paediatric Intensive Care Unit. PATIENTS: A total of 117children with congenital heart disease underwent CPB. Patients were divided into two groups: LCOS and non-LCOS. INTERVENTIONS: The clinical and analytical data were recorded at 2, 12, 24 and 48h post-CPB. Logistic regression was used to develop a risk prediction model using LCOS as dependent variable. MAIN OUTCOME MEASURES: LCOS, IS, VIS, MR-proADM, cTn-I, age, sex, CPB time, PIM-2, Aristotle score. RESULTS: While statistical significance was not recorded for IS in the multivariate analysis, VIS was seen to be independently associated to LCOS. On the other hand, VIS>15.5 at 2h post-CPB, adjusted for age and CPB timepoints, showed high specificity (92.87%; 95%CI: 86.75-98.96) and increased negative predictive value (75.59%, 95%CI: 71.1-88.08) for the diagnosis of LCOS at 48h post-CPB. The predictive power for LCOS did not increase when VIS was combined with cTn-I >14ng/ml at 2h and MR-proADM >1.5nmol/l at 24h post-CPB. CONCLUSIONS: The VIS score at 2h post-CPB was identified as an independent early predictor of LCOS. This predictive value was not increased when associated with LCOS cardiac biomarkers. The VIS score was more useful than IS post-CPB in making early therapeutic decisions in clinical practice post-CPB.

[Neurological complications in patients receiving solid organ transplants]. / Complicaciones neurológicas en trasplantados de órgano sólido.

INTRODUCTION: Neurological complications (NC) are a significant cause of morbidity and mortality in paediatric patients receiving solid organ transplants. Our aim was to describe the experience of our hospital with NC in paediatric patients receiving heart, lung and liver transplants. PATIENTS AND METHODS: A retrospective study was conducted on 140 paediatric patients who received a solid organ transplant during the period 2000-2011. RESULTS: A total of 23 paediatric solid organ transplant recipients (16.4% of cases), with a median age of 6 years, had NC. The symptoms were, in order of frequency: acute symptomatic seizures (12 patients); acute encephalopathy (11 patients); neuromuscular weakness (4 children), tremor (4 children), headache (2 children), neuropathic pain (2 children), and visual disturbances (2 children). The aetiologies of NC were: the neurotoxicity of the immunosuppressive drugs (12 patients), post-hypoxic-ischaemic encephalopathy (6 patients), infections (2 cases), mechanical compression of peripheral nerve during surgery (2 cases), and a metabolic complication (1 case). The five patients who met the criteria of posterior reversible encephalopathy syndrome had a favourable outcome. Seven patients died, four of them due to hypoxic-ischaemic encephalopathy. CONCLUSIONS: NC are common in paediatric patients receiving heart, liver, lung, and renal transplants, with acute symptomatic seizures and acute encephalopathy being the most common clinical signs. No differences were found in the NC with the different types of transplants. Neurotoxicity of the immunosuppressive drugs and hypoxic-ischaemic encephalopathy were the main causes of NC, having different management and outcomes. The prognosis was favourable in most of the patients, except for those who had moderate or severe post-hypoxic-ischaemic damage.

[Clinical characteristics of patients with infection due to Influenza A (H1N1) 2009 and critical pathology]. / Características clínicas de pacientes con infección por Influenza A (H1N1) 2009 y patología crítica.

OBJECTIVE: Retrospective, observational study in children hospitalized due to Influenza A (H1N1) between October 2009 and February 2010, emphasizing on those who required intensive therapy. MATERIAL AND METHODS: Risk factors, symptoms, course, complications, mortality, time of disappearance of RT-PCR Influenza A (H1N1), average length of stay and duration of treatment with oseltamivir, were studied, comparing critically ill patients (group I) with the rest of hospitalized patients (group II). RESULTS: A total of 38 children, with a mean age of 5.03±4.6 years were admitted during the study period. Six patients required intensive care, 83.3% had risk factors for influenza A (H1N1) compared with 62.5% in group II. Fever > 38°C was the predominant symptom (92.1%). Respiratory symptoms were significantly more frequent in group I (83.3% vs. 33.6; P<.01). Two critically ill patients required mechanical ventilation due to ARDS (acute respiratory distress syndrome). One patient with exacerbation of chronic respiratory failure responded favourably to high-flow oxygen. One patient developed fulminant myocarditis and required ECMO (Extracorporeal Membrane Oxygenation) because of secondary cardiogenic shock, which developed into multiple organ dysfunction and brain death. Oseltamivir therapy was given for an average of 8.6 days in group I and 4.5 days in group II (P<.05), checking the negativity of RT-PCR Influenza A (H1N1) in 7±2 days. The average stay was significantly higher in group I. Two patients admitted to PICU died. CONCLUSIONS: The presence of risk factors increases the likelihood of unfavourable outcome: high mortality among patients requiring intensive care. Monitoring of RT-PCR Influenza A (H1N1) could help establish the duration of isolation measures.

[Terlipressin as rescue therapy in hypercyanotic spells of Fallot tetralogy]. / Terlipresina como tratamiento de rescate de las crisis hipoxémicas en la tetralogía de fallot.

Hypercyanotic spells, very common in Fallot tetralogy, are a medical emergency and can cause neurological damage or even death. They are produced by a dramatic decrease in pulmonary blood flow, with increased right-left shunt and a drop in systemic arterial saturation. Treatment includes alpha.-agonists (such as methoxamine or phenylephrine), which increase peripheral resistance, forcing the entry of blood through the infundibulum. However, they are not available in our environment. An alternative, still not described, could be terlipressin, a potent vasoconstrictor. We report a three months old infant, with Fallot tetralogy and hypoplastic pulmonary branches, who developed a severe hypoxaemic crisis during postoperative palliative surgery (Blalock-Taussig), no responsive to standard therapy. The administration of terlipressin in three hypercyanotic spells, was effective and reversed them, with a significant and sustained increase in arterial saturation. Although there is still no evidence, terlipressin may be an alternative to alpha.-agonists in these patients.

[Fatal poisoning by accidental inhalation of aluminium phosphide]. / Intoxicación letal por inhalación accidental de fosfuro alumínico.

The cases of two 6 and 9-year-old sisters with lethal poisoning by inhalation of aluminium phosphide, after its inadequate use in a rural environment, are described. The clinical symptoms consisted of sudden vomiting, cardiac arrhythmias, shock, dyspnea, pulmonary edema/acute respiratory distress, metabolic acidosis and hepatic dysfunction, and the patients died in spite of advanced life support. Although an early diagnosis might theoretically improve the poisoning outcome, its high lethality rate and the absence of a specific antidote, efforts must be directed towards prevention and restricting its use as pesticide and being aware of its toxicity.

[Invasive mechanical ventilation in cystic fibrosis: Influence in lung transplant]. / Ventilación mecánica invasiva en fibrosis quística: influencia en el trasplante pulmonar.

INTRODUCTION: Invasive mechanical ventilation (IMV) in patients with advanced cystic fibrosis (CF) is a relative contraindication for lung transplant (LT) in adults, although there is currently no data on children. PATIENTS AND METHODS: An 8-year retrospective study on 21 children with CF who underwent LT was performed, analysing their results as they were receiving (n = 8) or not (n = 13) IMV pretransplant. Demographic and surgical data, postoperative course, lung function and survival (immediate and 1-year) were compared between both groups. The role of the IMV pretransplant as a postoperative risk factor was estimated (odds ratio) and Kaplan Meier survival study was performed in both groups. RESULTS: No differences in patient age, sex and nutritional parameters were observed between both groups. Those on IMV who received LT required more frequent and longer bypass, more need for tracheotomy, a higher number of rejection episodes per patient and multiorgan failure, longer PICU stay and longer time on IMV than those who were not on IMV when LT was received. Nevertheless, no differences could be found regarding graft function and immediate and 1-year survivals (62.5% vs. 92.3% with and without IMV respectively). On the other hand, long-term survival was significantly lower than in patients on IMV. CONCLUSIONS: In our experience, children with CF on IMV who receive LT have more complicated surgery and immediate postoperative course. Though immediate and 1-year results and survivals may be encouraging, medium and long-term ones are significantly lower.

[Organisation of paediatric resident rotations by specific areas]. / Organización de las rotaciones del residente de pediatría por las áreas específicas.

INTRODUCTION: Pediatric resident training is based on rotations. The tutor and the head of the area are responsible for planning the rotation. MATERIAL AND METHODS: An organisational model of rotation for a Pediatric residents program is presented. It includes educational objectives, theoretical contents, abilities and attitudes that must be acquired by the resident, training activities to achieve such objectives, strategies of stimulus and evaluation tools. COMMENTS: The aim of this model is to improve the resident teaching-learning process and help in the planning of resident training for those in charge of pediatric areas.

[Infant botulism]. / Botulismo del lactante.

We report a case of botulism in a 40 day old infant. The patient presented a descending flaccid paralysis requiring mechanical ventilation for 12 days. She is the first European patient treated with Human Botulism Immune Globulin. A few weeks later a second case of infant botulism was detected in our geographical region in Southern Spain. We emphasise the importance of an early diagnosis and treatment with Human Botulism Immune Globulin to decrease morbidity.

[Möbius syndrome and an apparently life-threatening event]. / Síndrome de Möbius y episodio aparentemente letal.

Möbius syndrome is characterized by congenital facial weakness with impairment of ocular abduction. A subgroup of these patients have associated apneas because of involvement of brainstem respiratory centers located slightly lateral to the abducens nuclei. We report a 7-month old infant admitted to the pediatric intensive care unit because of an episode of cyanosis, hypotonia and unresponsiveness. The patient then became respirator dependent afterwards. On examination, facial diplegia, impairment of ocular abduction and hypotonia were evident. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in brainstem tegmentum. At the age of 11 months he was discharged but required a home ventilator. He died 5 months later due to an infection. Möbius syndrome is associated with central respiratory dysfunction. The finding of abnormal signal intensity in brainstem tegmentum on MRI is a possible predictor of apnea in these patients.

[Artificial nutrition in pediatric intensive care units]. / Nutrición artificial en las unidades de cuidados intensivos pediátricos.

OBJECTIVE: To perform an epidemiologic study of artificial nutrition in critically-ill pediatric patients. PATIENTS AND METHODS: A multicenter, prospective and descriptive study was conducted in 23 Spanish intensive care units (ICU) (18 pediatric ICUs and five pediatric/neonatal ICUs) over a 1-month period. Artificial nutrition (AN) was required by 165 critically-ill patients (21.4 %). Data on diagnosis, severity, treatment, type of nutrition administered and complications were analyzed. RESULTS: A total of 54.4 % of the participants were younger than 1 year, 19.4 % were aged between 1 and 5 years old, 15.7 % between 5 and 10 years old and 13.4 % were older than 10 years. ICU mean length stay was 11 days. One hundred six patients were administered enteral nutrition (EN): 67.9 % continuous nasogastric EN, 27.4 intermittent nasogastric EN, 16 % nasojejunal EN, 2.8 % gastrostomy EN. Eighty patients required parenteral nutrition (PN): 86.3 % central PN, 20 % peripheral PN. No significant differences were found between patients with EN and PN in mean energy intake, days receiving AN, diagnosis at admission to the ICU, disease severity (measured by PRISM III) or intensive support techniques. The EN group required greater inotropic support. Patients undergoing mechanical ventilation had equal mortality independent of the type of AN. The most common complications in EN were: 17.9 % emesis, 13.2 % abdominal distension, 11.3 % diarrhea, 4.7 % gastric residual volumes, and 6.6 % hypokalemia. In PN complications consisted of: 5 % catheter related infection, 1.3 % thrombophlebitis, 7.5 % hyponatremia, 3.8 % hypoglycemia, 6.3 % hypophosphatemia and 3.8 % hypertriglyceridemia. CONCLUSIONS: EN provides critically-ill children with adequate energy intake and is well tolerated. Therefore, if there are no contraindications, EN should be the system of choice in the critically-ill patient requiring AN.

[Mechanical ventilation in pediatrics (III). Weaning, complications and other types of ventilation. High-frequency ventilation]. / Ventilación mecánica en pediatría (III). Retirada de la ventilación, complicaciones y otros tipos de ventilación. Ventilación de alta frecuencia.

In the era of lung-protective ventilation strategies, high frequency oscillatory ventilation (HFOV) has attracted renewed interest and its use has dramatically increased in neonatal and pediatric intensive care units. HFOV is able to reduce ventilator-induced lung injury by limiting the incidence of volutrauma, atelectrauma, barotrauma and biotrauma. During HFOV, adequate oxygenation and ventilation is achieved by using low tidal volumes and small pressure swings at supraphysiologic frequencies. Unlike other high-frequency ventilation modes, HFOV has an active expiration phase. HFOV constitutes a safe and successful ventilation mode for managing pediatric patients with respiratory insufficiency refractory to optimized conventional mechanical ventilation and provides better results when initiated early. However, the elective use of HFOV requires further studies to identify its benefits over conventional modes of mechanical ventilation and to support its routine use as a first line therapy. In the present article, the Respiratory Working Group of the Spanish Society Pediatric Critical Care reviews the main issues in the pediatric application of HFOV. In addition, a general practical protocol and specific management strategies, as well as the monitoring, patient care and other special features of the use of HFOV in the pediatric setting, are discussed.

[Sildenafil in the treatment of pulmonary hypertension]. / Sildenafilo en el tratamiento de la hipertensión pulmonar.

Pulmonary hypertension (PHT) is a rare entity that is difficult to treat. Prognosis is poor. Sildenafil, a selective inhibitor of type 5 phosphodiesterase, has been proposed among the many treatments available for primary and secondary pulmonary hypertension. We report our experience with an infant with pulmonary hypertension due to congenital mitral stenosis and persistent ductus arteriosus, who developed congestive cardiac failure with persistent PHT despite surgical correction. Conventional treatment was unsuccessful and the patient was treated with sildenafil. The clinical course was satisfactory, allowing extubation and withdrawal of vasoactive drugs; pulmonary and left atrial pressure decreased and the patient was discharged. She is currently being treated on an outpatient basis with oral sildenafil and shows satisfactory hemodynamic status. We review alternatives to conventional treatments for pulmonary hypertension with special reference to pediatrics.

[Fatal outcome after severe cerebellar infarction due to spontaneous dissection of the vertebral artery]. / Evolución fatal tras infarto cerebeloso masivo por disección espontánea de arteria vertebral.

Cerebellar infarction due to vertebrobasilar ischemia in spontaneous vertebrobasilar dissection is infrequent in children and adolescents. The commonest pathogenic mechanism of cerebellar infarction is arterial occlusion. Although usually favorable, outcome can be fatal, as in the present case. A previously healthy 4-year-old girl was admitted to the emergency room with sensory failure and trembling. The previous week she had suffered from a transitory ischemic attack that resolved spontaneously. On admission to the emergency department, the Glasgow Coma Scale score was 10/15 and she presented irregular polypneic breathing, anisocoric reactive pupils, enhanced reflexes, lower limb clonus and bilateral pyramidal signs. Cranial computed tomography revealed marked hypodensity in both cerebellar hemispheres and white supratentorial substance with obliteration of the quadrigeminus cisterns. Cerebral angiography showed left vertebral artery dissection with distal pseudoaneurysm and marked basilar occlusion. Despite aggressive antiedemic treatment and early instauration of intravenous continuous sodium heparin infusion, the patient developed a bilateral cerebellar infarction and supratentorial hydrocephalus. Brain death was diagnosed after 48 hours. Postmortem pathologic examination revealed previous thrombus the in superior left cerebellar artery and basilar artery occlusion. We discuss the absence of predisposing factors and the transitory ischemic attack in this patient 1 week before she developed massive cerebellar infarction.

[Prolonged Guillain-Barré syndrome]. / Síndrome de Guillain-Barré prolongado.

Outcome in Guillain-Barré syndrome (GBS) in childhood is usually favorable although prolonged and severe forms may develop. We report the case of a 12-year-old boy with rapidly ascending, slowly-progressive GBS with severe cranial nerve and autonomic dysfunction. Diagnosis was based on clinical presentation, electromyography, cerebrospinal fluid analysis and plasmatic antiganglioside GM1 antibodies. Early treatment with an initial loading dose of 2 g/kg of intravenous gammaglobulins over 5 days was given with a significant but slow clinical improvement. A repeat infusion of gammaglobulins was given two months later. Response was satisfactory with motor, sensory and autonomic dysfunction recovery. A third course of immunoglobulins 28 days later produced little benefit. At the present time, 5 months after the onset of the disease, flaccid paralysis of the lower limbs and the distal portion of the upper limbs persists but autonomic instability has disappeared. The patient has been decannulated and is following an ambulatory rehabilitation program. Although repeated intravenous immunoglobulins may be useful in the treatment of prolonged GBS, the key issue is excellent intensive care unit management. Further research examining potentially more effective treatment such as beta-interferon and immunosuppressive agents is required.

[External ventricular assist devices as circulatory support in patients with dilated cardiomyopathy]. / Asistencia ventricular externa como soporte circulatorio en un paciente con miocardiopatía dilatada.

Ventricular assist devices have demonstrated their utility in patients with intractable cardiac failure, both as support until complete myocardial recovery and as a bridge to transplantation. Specific pediatric pneumatic paracorporeal systems can be applied even in infants. Long-term survival has been reported although experience is limited. We report the case of an 8-year-old boy with dilated cardiomyopathy awaiting cardiac transplantation. The patient developed profound cardiogenic shock with multiorgan failure while being evaluated for heart transplantation. He was given biventricular assistance with the MEDOS-HIA system (MEDOS-Helmholtz Institute). Maximum stroke volume ventricles of 25 and 22 ml were used, achieving a cardiac output of 2.2 l/min in both ventricles. The patient was supported with ventricular assistance for 9 days, but multiple organ failed to improve and transplantation became impossible. Progressive loss of peripheral circulatory resistance unresponsive to treatment developed and ventricular assistance was discontinued. The previous severe shock and advanced and progressive multiorgan failure could be responsible for the poor outcome of our patient despite maintenance of adequate cardiac output. Nevertheless, the use of ventricular assist devices is a real therapeutic alternative in children with severe cardiogenic shock, allowing them to recover completely or undergo heart transplantation. Patient selection, the choice of a system of appropriate size, and early implantation seem to be the cornerstones for obtaining good results.

[Convulsive status secondary to star anise poisoning in a neonate]. / Crisis convulsiva secundaria a intoxicación por anís estrellado en un lactante.

Despite its potential toxicity, the use of star anise infusions for the treatment of infant colic is firmly rooted in our culture. In the last year, several cases of infants with acute neurological and gastrointestinal symptoms related to the ingestion of large quantities of star-shaped anise have been reported in Spain. We report a 1-month old girl who was admitted to our emergency room with convulsive status after ingesting a considerable amount of star anis administered to relieve possible infant colic. The patient required three repeat doses of diazepam to control the seizures. Because no abnormalities were found and outcome was favorable without anticonvulsant therapy, the clinical symptoms can be attributed to star anise ingestion, which contains potent neurotoxins such as veranisatins. The etiopathogenesis of this process and star anise toxicity are discussed and the need for the regulation of natural products with pharmacological effects which are freely dispensed from drug stores is stressed. Finally, the regulations of the Ministry of Health and Consumption for 2001 concerning the withdrawal of all anise-containing products are presented.