[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.

[22Q11 microdeletion syndrome: cardiorespiratory symptoms and fibrobronchoscopy]. / Síndrome de microdeleción 22q11: manifestaciones cardiorrespiratorias y utilidad de la fibrobroncoscopia.

The 22q11 deletion syndrome is a frequent contiguous-gene deletion syndrome. This disorder has a broad spectrum of phenotypic manifestations. It includes various syndromes such as DiGeorge syndrome. The most frequent clinical manifestations are congenital cardiac defects, characteristic facies, palate malformations, hypoparathyroidism, immunodeficiency due to thymic hypoplasia, growth retardation, and behavioural and psychiatric problems. Among the symptoms observed, many patients suffer from respiratory insufficiency or failure. The origin is often multifactorial. Structural airway abnormalities are frequently found in this syndrome. In many of these patients the malformation is mild or non-existent, and remains asymptomatic. However, in some cases it can cause a severe respiratory insufficiency, being diagnosed when other disorders are ruled out. These cases illustrate the importance of early visualisation of the airway by fibrobronchoscopy in the management of the patient with 22q11 deletion syndrome who has recurrent respiratory difficulties.

[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.

[Medical pericarditis: study of 19 cases]. / Pericarditis médicas: estudio de 19 casos.

19 cases of medical pericarditis admitted to the PICU from 1978 to 1989 were reviewed, to asses the diagnostic and therapeutical approach. The overall incidence is low, 0.32% of the patients admitted to the PICU in that time period. We have found neither tuberculous nor purulent pericarditis, with a 42% of idiopatic forms. Echocardiography was the best method for syndromic diagnosis. The ethiological diagnosis of pericarditis was made, in most cases, by means of non invasive methods or in association with the underlying disease. The cualitative analysis of the pericardial fluid was not useful for the ethiological diagnosis, therefore, we suggest pericardiocentesis should be performed mainly as a therapeutical tool. There were no correlation between the amount of fluid obtained on the first pericardiocentesis and the later incidence of the pericardial effusion. Biopsy brought no ethiological specificity in the cases it was performed.

[Acute bulbar palsy due to myelomeningocele and Chiari II malformation]. / Parálisis bulbar aguda en un caso de mielomeningocele y malformación de Chiari tipo II.

We describe a case of a 10-year-old girl with myelomeningocele and type II Chiari malformation. She presented with an acute bulbar palsy requiring mechanical ventilation. Surgical decompression of the brainstem was performed and, because of a phrenic nerve palsy, surgical plication of the right diaphragm was also required. The patient s evolution was favourable. We discuss the various forms of clinical presentation of this pathology as well as its management. The importance of early diagnosis and surgical correction are also stressed as a key factor for favourable evolution.

[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.

[The first national program of pediatric lung transplantation: the experience in pediatric intensive care]. / Primer programa nacional de trasplante pulmonar infantil: experiencia en cuidados intensivos pediátricos.

OBJECTIVE: The aim of this study was to analyze the postoperative progress and medical management in the Pediatric Intensive Care Unit (PICU) of patients that underwent bilateral lung transplant. PATIENTS AND METHODS: From April 1997 to June 1998, 10 pediatric lung transplants were performed at the Hospital Reina Sofía (Córdoba, Spain). There were 4 males and 6 females (mean age 11.5 years, range 5 to 15 years). Indications for transplantation were cystic fibrosis (n = 9) and one pulmonary fibrosis secondary to viral infection. Before the transplant, two patients required mechanical ventilation for acute respiratory decompensation and one patient was ventilator-dependent. Immunosuppression consisted of corticosteroids, azathioprine and cyclosporine or tacrolimus. Post-transplantation management included early extubation, when possible, optimal fluid balance to prevent lung edema, low aggressive mechanical ventilation and adequate treatment of complications, such as rejection and infection. RESULTS: There were no peri-operative mortalities. The mean stay in the PICU was 28 days (median: 17 days) and the mean time on mechanical ventilation was 19 days (median: 5.5 days). The most frequent complications were rejection (n = 8), hyperglycemia (n = 6), renal failure (n = 4), arterial hypertension (n = 4) and respiratory infections (n = 3). There were no airway complications. CONCLUSIONS: Even if the post-operative period in pediatric lung transplant patients is difficult, the results have been good with an important improvement in the quality of life of these patients has been achieved.

[Status asthmaticus; experience in a pediatric intensive care unit]. / Status asmático: experiencia en una unidad de cuidados intensivos pediátricos.

Thirty children presenting status asthmaticus in fifty episodes were studied during a period of three and a half years. All of them showed acute respiratory failure secondary to asthma. The purpose was to evaluate treatment results and complications compared with a previous similar study conducted in our unit. We have noticed that there is a lower number of admissions for SA in our ICU and a different seasonal tendency. Precious treatment of asthmatic crisis selective inhaled beta 2 bronchodilators can prevent the ICU admission of an asthmatic patient.

[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.