Miguel Félix

Foreign bodies in the airway: a quarter of a century's experience.

INTRODUCTION Foreign body (FB) aspiration in children is a common and potentially dangerous situation that can be associated to significant morbidity. AIMS To characterise the FB aspiration in children cases at the Hospital Pediátrico de Coimbra over a twenty five year period. STUDY DESIGN This study was based on the retrospective analysis of all clinical files of children who were diagnosed with foreign body aspiration January 1982 to December 2006. RESULTS Foreign body aspiration was confirmed in 316 children. The incidence was higher during the first twelve years of the study (64%). Around two thirds of the children were male (206) and the sample was aged 6 months to 12 years. Most children were younger than 3 years old (83%). In 88% of cases a choking episode was noticed while an early diagnosis (<24h) was obtained in only 39%. The most frequently described signs and symptoms were unilateral diminished breath sounds and cough. In 7% of cases no symptoms were described. The most frequently recorded radiology finding was focal hyperinflation (42%) and in 22% the chest x-ray was unremarkable. Treatment was exclusively by rigid bronchoscopy. Complications related to the bronchoscopy removal were described in 22 cases. Most aspirated FB were of vegetable origin (75%). The majority of FB was lodged in the right bronchial tree. Postremoval flexible bronchoscopy was performed in 116 cases. CONCLUSION An unnoticed FB aspiration and absence of and/or non-specific initial symptoms may contribute to a late diagnosis. The significant reduction in the number of cases over the later years may be related to the implementation of preventive strategies.

Long-term ventilation in children: ten years later.

INTRODUCTION Home mechanical ventilation (HMV) represents a treatment option for patients with chronic respiratory failure and has changed prognosis and survival of many disorders in children. The aim of this study was to characterize a group of children on long-term mechanical ventilation (LTMV) for a period longer than 10 years. METHODS A retrospective analysis was carried out including patients on LTMV for more than 10 years (LTMV-10) in a tertiary pediatric hospital. STATISTICAL ANALYSIS PASW Statistics 18(®). RESULTS Thirty-one children (61% female) belong to the LTMV-10 group. Median age at the beginning of ventilatory support was 3 years (birth to 13 years). Main indications for assisted ventilation were neuromuscular disease (n=12, 39%), metabolic disease (n=7, 23%) and central hypoventilation (n=6, 19%). Volume ventilation was used in 2 children, and positive pressure ventilation in the others, mainly bilevel positive airway pressure (n=25, 81%). Invasive ventilation via tracheostomy was used since the beginning in four cases, and subsequently in two other children. The mean time of ventilatory support was 146 months and the maximum was 219 months. Respiratory morbidity was the most frequent cause of hospitalization and the annual rate of such episodes was 0.17 per child. Global mortality rate was 19%. CONCLUSIONS HMV programs provide necessary and safe assistance for children with severe chronic respiratory failure. As shown in our series, it is possible to be kept on this respiratory support modality for long periods with good compliance and a small number of hospitalizations.

Atrofia muscular espinhal – Apoio ventilatório não invasivo em pediatria

The deterioration of the respiratory function in children suffering from degenerative neuromuscular disease is the main cause of the high mortality rate associated with these diseases. Noninvasive ventilation (NIV) has reduced the morbidity and mortality due to respiratory insufficiency in these children. However, the use of support ventilation in some cases of spinal muscular atrophy (SMA) is still controversial. A retrospective study of 22 patients suffering from SMA who were followed up in the Paediatric Hospital of Coimbra is presented: 7 of type I, 11 of type II, and 4 of type III. In 17 of these cases, non-invasive ventilation by mask was begun, and in 3 of them NIV was applied for prophylactic purposes. The 7 children with SMA type I began NIV when they were 13 months of age on average (3 months-3 years); 5 of them died, between 1 and 15 months after the beginning of the ventilation. Of the 11 children with SMA type II, 8 were submitted to NIV and one died 22 months later. Three of the children in this group began NIV in a prophylactic way, and in all of them a decrease in the thoracic deformity was observed. Of the 4 patients of type III, 2 of them were submitted to non-invasive ventilation. In all of the symptomatic cases, a decrease in the frequency and severity of respiratory infections was observed, after ventilation was started. The respiratory support with NIV may improve the quality of life of children suffering from SMA as well as prolong their life expectancies. In SMA type I, whose clinical manifestations are precocious and whose prognostic is very serious, the application of this support has been debated.

Corpos estranhos na via aérea: Experiência de um quarto de século

Introduction: Foreign body (FB) aspiration in children is a common and potentially dangerous situation that can be associated to significant morbidity. Aims: To characterise the FB aspiration in children cases at the Hospital Pediatrico de Coimbra over a twenty five year period. Study design: This study was based on the retrospective analysis of all clinical files of children who were diagnosed with foreign body aspiration January 1982 to December 2006. Results: Foreign body aspiration was confirmed in 316 children. The incidence was higher during the first twelve years of the study (64%). Around two thirds of the children were male (206) and the sample was aged 6 months to 12 years. Most children were younger than 3 years old (83%). In 88% of cases a choking episode was noticed while an early diagnosis (< 24 h) was obtained in only 39%. The most frequently described signs and symptoms were unilateral diminished breath sounds and cough. In 7% of cases no symptoms were described. The most frequently recorded radiology finding was focal hyperinflation (42%) and in 22% the chest x-ray was unremarkable. Treatment was exclusively by rigid bronchoscopy. Complications related to the bronchoscopy removal were described in 22 cases. Most aspirated FB were of vegetable origin (75%). The majority of FB was lodged in the right bronchial tree. Postremoval flexible bronchoscopy was performed in 116 cases. Conclusion: An unnoticed FB aspiration and absence of and/or non-specific initial symptoms may contribute to a late diagnosis. The significant reduction in the number of cases over the later years may be related to the implementation of preventive strategies. Rev Port Pneumol 2008; XIV (6): 787-802

WITHDRAWN: Long-term ventilation in children: Ten years later

This article has been withdrawn for editorial reasons because the journal will be published only in English. In order to avoid duplicated records, this article can be found at http://dx.doi.org/10.1016/j.rppnen.2014.03.017. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.

Cystic Fibrosis Newborn Screening in Portugal: PAP Value in Populations with Stringent Rules for Genetic Studies

Newborn screening (NBS) for cystic fibrosis (CF) has been shown to be advantageous for children with CF, and has thus been included in most NBS programs using various algorithms. With this study, we intend to establish the most appropriate algorithm for CF-NBS in the Portuguese population, to determine the incidence, and to contribute to elucidating the genetic epidemiology of CF in Portugal. This was a nationwide three-year pilot study including 255,000 newborns (NB) that were also screened for congenital hypothyroidism (CH) and 24 other metabolic disorders included in the Portuguese screening program. Most samples were collected in local health centers spread all over the country, between the 3rd and 6th days of life. The algorithm tested includes immunoreactive trypsinogen (IRT) determination, pancreatitis associated protein (PAP) as a second tier, and genetic study for cases referred to specialized clinical centers. Thirty-four CF cases were confirmed positive, thus indicating an incidence of 1:7500 NB. The p.F508del mutation was found in 79% of the alleles. According to the results presented here, CF-NBS is recommended to be included in the Portuguese NBS panel with a small adjustment regarding the PAP cut-off, which we expect to contribute to the improvement of the CF-NBS performance. According to our results, this algorithm is a valuable alternative for CF-NBS in populations with stringent rules for genetic studies.

P25 - Hypersensitivity/adverse reactions to antituberculosis drugs – a case report

Case report A ten years old boy was admitted for pleural tuberculosis (negative cultures, positive quantiferon assay, and father under treatment for tuberculosis). On the tenth day of treatment with isoniazid, rifampicin and pyrazinamide he develloped an exuberant urticarial rash, facial oedema, fever, myalgias, oliguria and, later, conjunctival hyperemia. Laboratory results included low platelet count, hypoalbuminemia and hyponatremia. Gradual improvement occured after suspension of treatment. One month later, drugs were gradualy re-introduced (1 drug/week) but several reactions occurred: anaphylaxis 5 hours after rifampicin administration and generalized macular rash after pyrazinamide. Both drugs were suspended. Isoniazid caused an initial light generalized macular exanthema. Streptomycin, ethambutol and ciprofloxacin were introduced and well tolerated. In subsequent evaluation the child was asymptomatic, with normal analytic results and radiologic improvement. After 6 months of successful treatment, he was tested in detail for reactions to the implicated drugs.