[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.

Terlipresina como tratamiento de rescate de las crisis hipoxímicas en la tetralogía de fallot / Terlipressin as rescue therapy in hypercyanotic spells of fallot tetralogy

Las crisis hipoxémicas, especialmente frecuentes en la tetralogía de Fallot, son una emergencia médica, que pueden originar secuelas neurológicas o incluso la muerte. Se producen por una disminución drástica del flujo pulmonar, con aumento de shunt derecha-izquierda y caída de la saturación arterial sistémica. Su tratamiento incluye α-agonistas (como metoxamina, fenilefrina), que incrementan las resistencias periféricas, forzando la entrada de sangre por el infundíbulo. Sin embargo, en nuestro medio no están comercializados. Una alternativa no descrita aún podría ser terlipresina, un potente vasoconstrictor. Se presenta un lactante de 3 meses de edad, con tetralogía de Fallot e hipoplasia de ramas pulmonares, que desarrolló crisis hipoxémicas graves durante el postoperatorio de cirugía paliativa (Blalock-Taussig), sin respuesta al tratamiento habitual. La administración de terlipresina, hasta en 3 episodios, consiguió revertir las mismas, con un incremento significativo de la saturación arterial. Aunque no existe evidencia actualmente, la terlipresina podría ser una alternativa a los α-agonistas en estos pacientes (AU)

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 α-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 α-agonists in these patients (AU)

[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.

[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.

[Hypoxic-ischemic coma in children. Factors related to the prognosis]. / Coma hipóxico-isquémico en niños. Factores relacionados con su pronóstico.

OBJECTIVE: The purpose of this study was to analyze the etiology, evolution and predictive value of clinical variables and complementary explorations in children admitted to a PICU with hypoxic-ischemic coma. PATIENTS AND METHODS: A retrospective review (1991-1996) of 24 children with hypoxic-ischemic coma admitted to the pediatric ICU of a reference hospital was performed. Clinical data on admission and results of complementary explorations (EEG and neuroimaging techniques) were collected and the findings compared according to the evolution and underlying pathophysiological mechanism. RESULTS: Non-reactive and dilated pupils, existence of neurohypophyseal disorders and isoelectric EEGs were exclusively found in dead patients. A score of 3-4 on the Glasgow Coma Score was obtained more frequently in groups with the worse evolution (exitus and surviving with sequelae), as well as positive findings with neuroimaging techniques and the appearance of seizures, though without meaningful differences. More significant was the existence of associated problems, which was more frequent in groups with a negative evolution. The differences among the pathophysiological groups were not meaningful. Global mortality in the series studied was 41.6%, with 29.2% of the survivors having neurological sequelae and 29.2% being healthy. CONCLUSIONS: Hypoxic-ischemic coma in children has a high mortality and morbility. The prognosis for each patient is based on the clinical facts and complementary explorations. No isolated data allows the establishment of the final outcome with certainty.