[ECMO: experience in paediatrics]. / ECMO: experiencia en edad pediátrica.

INTRODUCTION: ECMO (Extracorporeal Membrane Oxygenation) provides a vital support to patients with supposed reversible respiratory and/or cardiac failure, in whom conventional support techniques have been previously unsuccessful. OBJECTIVES: To determinate the criteria used in our hospital to put paediatric patients on ECMO, compare their clinical course depending on their pathology (respiratory failure, congenital heart disease or sepsis) and identify the sequelae attributable to this technique. MATERIAL AND METHOD: A retrospective review of clinical records of all patients on ECMO support in our centre, excluding those presenting typically in neonatal period. RESULTS: ECMO was used on 16 patients from June 2001 to January 2007, of which 50% were males. The median age was 7 months (from 21 days to 11 years). The reason for starting ECMO was respiratory failure in 11 cases (oxygenation index >40 and/or alveolar-arterial oxygen gradient >605), congenital heart disease in 2 and sepsis in 3 (due to shock unresponsive to adequate resuscitation). The median time to starting ECMO from PICU admission was 3.58 days (from 12h to 9 days). Venovenous cannulation was used initially in 8 patients, but 5 of them needed venoarterial ECMO later. The technique was used for a mean of 8 days (from 1 to 28 days). The main complication was the isolation of bacteria in different cultures (8 patients). The overall survival was 50% (6 patients with respiratory failure and both patients submitted to cardiac surgery). Extracorporeal support was withdrawn in 7 children because their clinical situation was irreversible. Another patient died seven days after successful decannulation. We have not found any serious sequel among survivors that could be attributable to this technique. CONCLUSIONS: Survival among children supported with ECMO in our hospital is similar to that recorded by the ELSO in 2004, although the prognosis depends on the initial pathology. There are different criteria for starting this technique depending on the underlying diseases: respiratory index of poor prognosis in patients with respiratory failure, haemodynamic instability in those with sepsis or cardiac failure after cardiovascular surgery. We have not found any serious sequel among the survivors which could be attributable to this technique.

[Congenital hyperekplexia as a cause of neonatal hypertonia]. / Hiperekplexia congénita como causa de hipertonía neonatal.

INTRODUCTION: Hyperekplexia is a disease characterized by an exaggerated startle reflex in front of minimal tactile and/or acoustic stimuli, that usually goes with high muscular tone, except when the patient is sleep. When the patient grows, high muscular tone decreases and then appear sudden hypertonic episodes that make fall the patient. Presence of inguinal and/or umbilical hernia is common, as well as sudden infant death. Neurophysiological findings can give guidance to diagnosis. It's an autosomal dominant transmission disease, but sporadic cases can appear. Clonazepam is the drug of choice, obtaining a marked improvement in a lot of patients. CLINICAL CASE AND CONCLUSIONS: Although rare, hyperekplexia is a diagnosis we must think about when we see an hypertonic newborn, and this is the reason why we report this case recently diagnosed in our hospital.

Pulmonary vascular abnormalities and ventilation-perfusion relationships in mild chronic obstructive pulmonary disease.

Morphologic changes in pulmonary muscular arteries may modify the mechanisms that regulate the pulmonary vascular tone and contribute to maintaining an adequate ventilation-perfusion (VA/Q) matching in patients with chronic obstructive pulmonary disease (COPD). To analyze the relationships between the abnormalities of pulmonary muscular arteries and the degree of VA/Q inequality, and to assess the effect of these abnormalities on the changes in VA/Q relationships induced by oxygen breathing, we studied a group of patients with mild COPD undergoing resective lung surgery. According to the degree of airflow obstruction and the increase in VA/Q mismatch produced by 100% O2 breathing (delta logSD Q), patients were divided into three groups: (A) patients with normal lung function, (B) patients with airflow obstruction and a high response to oxygen (delta logSD Q > 0.4), and (C) patients with airflow obstruction and a low response to oxygen (delta logSD Q < 0.4). Pulmonary arteries in Groups B and C showed narrower lumens and thicker walls than in Group A. These morphologic changes were produced mainly by an enlargement of the intimal layer and were more pronounced in Group C than in Group B. The assessment of intimal area as a function of artery diameter showed that the increase in intima in Group C took place predominantly in arteries with small diameters (< 500 microns). The mean intimal area on each subject correlated with both the PaO2 value (r = -0.46, p < 0.05) and the overall index of VA/Q mismatching (r = 0.51, p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Controlled diet in phenylketonuria may cause serum carnitine deficiency.

Serum carnitine levels may be reduced in patients with phenylketonuria (PKU) owing to low carnitine intake, deficient carnitine synthesis and acylcarnitine production from phenylalanine metabolites. In order to investigate the possible carnitine deficiency we determined serum carnitine in its different forms and the precursors and cofactors involved in its synthesis in a group of patients with PKU or hyperphenylalaninaemia. Free, total and acylcarnitine values were significatively reduced only in PKU patients with Phe-restricted diet which had not been supplemented with carnitine. Acylcarnitine/free carnitine ratio and all the other parameters studied were normal in all patients. We conclude that the low serum carnitine levels in PKU patients with a strict diet are a consequence of the low carnitine intake.

Fine needle aspiration of myofibroblastoma of the breast in a man. A report of two cases.

The cytopathologic features of fine needle aspiration biopsy of two myofibroblastomas of the breast in men are described. The cytologic findings consisted of monomorphic spindle cells with ovoid, grooved nuclei, isolated or in clusters, with an ill-defined, short fascicular pattern and abundant acellular, myxoid-appearing material. Atypical features were absent. No epithelial elements were present. We emphasize the value of aspiration cytology in association with clinical and mammographic data to establish a definitive diagnosis of this uncommon entity. This benign tumor should be considered in pure spindle cell, nontypical fine needle aspiration biopsy specimens of breast masses with sharp demarcation from the surrounding tissue, especially in men.

[Meningitis in pediatrics. Clinical and epidemiological study of 173 cases]. / Meningitis en pediatría. Estudio clínico y epidemiológico de 173 casos.

BACKGROUND: To know the incidence, clinical presentation, differential diagnosis and resistance level to antibiotics in pediatric meningitis. METHODS: 173 cases of meningitis (bacterial: 69, viral: 104) have been prospectively followed during 1988 according to a previously established clinical and laboratory protocol. RESULTS: Meningitis attack rate was 60 cases/100,000 children younger than 15 years per year (meningococcal: 25/100,000, Haemophilus: 2/100,000). Mortality was 1.4%. 40% of bacterial meningitis received previous antibiotic treatment. Sensitivity of culture, Gram stain, and direct antigen detection by latex and EIA was 79%, 61.7%, 20.5% and 34%, respectively. Bacterial and viral differential diagnosis, by the application of Boyer's score was 95% sensitive and 98% specific. Four out of six cases of Haemophilus meningitis were ampicillin and chloramphenicol resistant; 39% of meningococcus had their penicillin susceptibility decreased between 2 and 8 times, although no therapeutic failures were seen. CONCLUSIONS: Laboratory parameters might not separate bacterial and viral meningitis at early stages of illness. Gram stain is an excellent and sensitive method of bacterial detection in CSF. Moderate resistance to penicillin in meningococcus is very frequent but clinical failures are not yet present.

Adenosquamous carcinoma of the esophagus. A case report.

A case of adenosquamous carcinoma of the esophagus is presented. This carcinoma is a typical neoplasm of the upper aerodigestive tract almost exclusively originating in squamous epithelium in continuity with minor salivary glands. It is a very rare tumor in the esophagus, and is often diagnosed as mucoepidermoid carcinoma. The differential diagnosis between them is important due to the better prognosis of the last entity. The histology and electron microscopy is described and the literature is reviewed.

[Acute fulminant myocarditis in a young man of 19]. / Miocarditis aguda fulminante en un joven de 19 años.

We report the case of a 19-year-old male who developed an acute myocarditis, with restrictive hemodynamic pattern and cardiogenic shock, and who died 48 hours after hospital admission. We discuss the characteristics of this uncommon form of presentation of acute myocarditis, and the importance of early diagnosis and treatment.