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Introduction: Long-term home oxygen therapy is indicated for patients with chronic hypoxemia. We intend to describe pediatric population on long-term home oxygen therapy followed-up at Pediatric Respiratory Unit of a tertiary care hospital between 2003-2012 and to compare with previous 1991-2000 review; to verify conformity with international and national recommendations and need for specific pediatric national guidelines, non-existent in Portugal., Material and Methods: Retrospective, descriptive and comparative study based on clinical files review. Review the guidelines for oxygen therapy in pediatric population., Results: We studied 86 patients (59.3% males). The median age at the beginning of oxygen therapy was 0.0 (0.0-216.0) months, with a median duration of 15.0 (3.0-223.0) months. The most frequent diagnosis was bronchopulmonary dysplasia (53.5%), followed by bronchiolitis obliterans (14.0%), neurologic disorders (10.5%), cystic fibrosis (8.1%), miscellaneous syndromes (5.8%), sickle-cell disease (3.5%), other neonatal lung diseases (2.3%) and interstitial lung diseases (2.3%). Are maintained on follow-up 53 (61.6%) patients, 38 on oxygen therapy; 12 (13.9%) died. The median time of follow-up was 39.5 (1.0-246.0) months, minim on other neonatal lung diseases and maximum on cystic fibrosis. Comparing with previous review, this shows a relative increase in bronchiolitis obliterans and bronchopulmonary dysplasia patients, with increased duration in the latter, and inclusion of neurologic and hematologic patients., Discussion: Prescription of long-term oxygen therapy in pediatric age mainly occurs in specific diseases of infants and pre-school aged. Neurologic and hematologic patients represent new indications, similarly to international publications., Conclusion: The knowledge of national reality and pediatric orientations are needed for care plans and rational prescription.

Introduction: Obstructive sleep apnea (OSA) affects approximately 1-3% of pediatric population and is associated with significant morbidity. As adenotonsillar hypertrophy (ATH) is its primary cause in children, elective adenotonsillectomy is the first treatment of choice. Noninvasive ventilation (NIV) has been increasingly considered as an option, mainly for children with complex diseases, ineligible or waiting for surgeries, or after surgery failure., Objectives: To describe the experience in the management of children with complex OSA, and to evidence the feasibility and advantages of NIV., Methods: This was a retrospective cohort study of 68 children on NIV, in whom complex OSA was the main indication for ventilation, in a Pediatric Respiratory Unit at a University Hospital between January 1997 and March 2012. Demographic and clinical data were collected on the underlying diagnosis, therapeutic interventions prior to NIV, NIV related issues and outcome., Results: Forty (59%) children were male, median age at starting NIV was 6 years and 7 months, with interquartile range (IQR) of 15-171 months. Twenty-two (32%) were infants and 25 (37%) adolescents. The most common diagnosis was congenital malformations and genetic disorders in 34 (50%) patients. Nine patients had cerebral palsy, 8 were post treatment for central nervous system tumors and 6 had inborn errors of metabolism. Three children had ATH and three obesity. The majority of patients (76%) had exclusively obstructive OSA and started CPAP. Ten patients had minor complications. Twenty-two patients stopped NIV due to clinical improvement, 8 were non-compliant and 8 patients died. NIV median duration was 21.5 months (IQR: 7-72)., Conclusions: NIV is feasible and well tolerated by children with OSA associated with complex disorders, and has been shown to have few complications even in infants and toddlers., (Copyright © 2013 Sociedade Portuguesa de Pneumologia. Published by Elsevier España. All rights reserved.)

Unlabelled: Despite advances in non-invasive ventilation techniques, tracheostomy is still indicated in children with serious airway obstruction or with the need for long-term ventilation. Alternatives should be studied before deciding to tracheostomise as complications exist, despite the simple procedure. The aims of this study were the identification of tracheostomised children followed in a tertiary care paediatric department, the characterisation of primary disease, the indications leading to tracheostomy and any complications. We present a case review of tracheostomised children followed in our department. Sixteen tracheostomised children were followed, median age of 4.5 months, neuromuscular disease - 5, cerebral palsy - 3 and pulmonary or airway disease - 7. Indications were long-term invasive ventilation - 12, subglottic or tracheal stenosis - 3 and laryngomalacia - 1. The most frequent complications found were persistent bacterial colonisation, accidental decannulation and obstruction. There was a tracheostomy- related death in 1 child., Outcome: 9 children maintained tracheostomy, 5 were successfully decannulated and 2 children died while on tracheostomy. Tracheostomised children usually have complex disease that requires a multidisciplinary team and should be followed-up at a specialised reference centre.

Introduction: Pleural effusions can complicate pneumonias in children and adolescents and are usually associated with a long hospital stay and increased morbidity., Aims: To characterise a population of patients with parapneumonic pleural effusion and to establish possible prognostic factors on admission based on clinical, imaging and analytical data. To correlate treatment options with the outcome., Methods: Case review of patients under 18 years old with parapneumonic pleural effusion, admitted between July 1997 - June 2004 (7 years)., Results: 118 patients were included, 60% male, with mean age 7 years. The incidence of pleural effusion increased throughout the period of the study. The admissions occurred predominantly in autumn and winter. On admission 60% of patients had respiratory distress and 39% chest pain. In 40% loculations were found on admission and were associated with longer hospital stay, longer course of antibiotic therapy and more frequent need for surgery. Thoracentesis was performed in 72% of patients (mean pH pleural fluid 7.24). The aetiologic agent was identified in 17% of cases: Streptococcus pneumoniae (five), Staphylococcus aureus (four) and Streptococcus pyogenes (four). In our study, 52% of patients underwent pleural drainage and 18% surgery. Median length of hospital stay was 15 days with mean 16.4 days (2 - 51)., Discussion: Factors associated with worse prognosis were respiratory distress, loculations, empyema, low pH in pleural fluid, glucose or proteins in pleural fluid, high lactic dehydrogenase level in pleural fluid and high serum C-reactive protein. Pleural drainage and/or surgery can shorten hospital stay and improve outcome., Conclusion: Complicated parapneumonic pleural effusions are managed successfully in centres with experience in the different types of procedure that might be necessary.

Bronchiolitis obliterans (BO) is a rare disease in immunocompetent children that usually occurs after infection of the lower airways. While a diagnosis of BO was usually confirmed by lung biopsy, identification of prior lung lesion plus a typical clinical course and a suggestive chest X-ray and CT scan have replaced the need for more invasive procedures. The authors reviewed the clinical records of 10 BO patients, followed in the Outpatients Paediatric Pulmonology Unit from January 1997 to December 2002, to identify the most common aetiology, clinical and radiological profiles, treatment and course. All patients maintained cough and/or wheezing after the initial acute episode. 80% had failure to thrive at the time of the diagnosis, mean age 16 months. Viral pneumonia was the main initial event (5 adenovirus, 3 respiratory syncytial virus, 1 parainfluenza virus, 1 unknown). Lung biopsies were not performed as clinical and radiological presentations were typical of BO. The follow-up (mean 36 months) revealed clinical resolution in 3 children and persistent symptoms in 6. One patient had progressive respiratory failure and died. Prompt recognition of the diagnosis with supportive treatment that included oxygen therapy and an aggressive nutrition plan helped to improve the clinical state of the children.

The authors describe the clinical case of a 15 year-old adolescent with recurrent bilateral chylothorax leading to multiple hospitalizations. As in other cases described the diagnosis was made on the basis of classical clinical features and characteristic CT scan. Most cases of pulmonary lymphangiectasis, described in the literature, are lethal in the neonatal period. In this case the girl has a reasonable life quality, in spite of multiple hospitalizations. Lower limb lymphedema, facial anomalies (flat face, palpebral edema, tooth anomalies and small mouth) and mild development retardation allow us to consider our case as the first portuguese case of Hennekam Syndrome. Molecular studies will allow, in the future to explain most of the clinical manifestations and to include other patients in the same clinical entity.

Lung abscess is a rare entity in childhood, but leads to high morbidity and long length of hospitalisation. It appears commonly in children with risk factors or other disease, have a good response to antibiotic therapy and it is rarely necessary the use of invasive techniques. Authors present two cases of lung abscess in children, one of them with risk factors to aspiration, that improved with antibiotic therapy and make a revision about this entity in infancy.

This work is a retrospective study of latrodectism in the State of Bahia, Brazil, from August 1980 to July 1990. The data concerning the accidents were obtained from file cards at the Antivenom Information Center of Bahia (AVICB). Latrodectus curacavienis was the ethiologic agent identified in 28% of the arachnid accidents. The major incidence was registered in urban area (57%) affecting men (70%) more than women, with 10 to 29 year-old age group (58%). Local pain (56%), erythematous papula (29%) and light oedema (17%) were the principal local symptoms. Pain in the limbs (29%), tremor and rigidities (29%), sweating (28%), limbs and arms paresthesia (21%) and abdominal pain (17%) were systemic ones. The treatment was mainly symptomatic (67%) and antivenin serum was used in 21% of the cases. After serotherapy, 64% of the patients left the hospital within less than 24 hours.

Objective: The authors studied 14 children with myocarditis in order to correlate clinical and laboratorial data with evolution., Design: Retrospective study of all cases with myocarditis admitted during a 2 year period., Setting: Unidade de Cardiologia Pediátrica do HSJ., Patients: 14 patients, aged 7 to 34 months, with the diagnosis of myocarditis on the ground of biopsy or necropsy findings. The clinical data analysed were: medical history, physical examinations, hematological and biochemical studies, chest X ray, electro and echocardiogram, virus and immunological studies and cardiac catheterization., Results: All the patients had congestive heart failure and 10 had a history of a previous viral infection. Those who survived the first week were submitted to hematological and biochemical studies, chest X Ray, electrocardiogram, echocardiogram, virological and immunological studies and cardiac catheterization. All patients were treated with captopril and furosemide, 11 received dobutamine and 6 an immunosuppressive regime. Six patients died in the first week and one, nine months after diagnosis. One patient had an unfavourable course and the other six improved clinically., Conclusions: The authors were not able to find any correlation between laboratorial data and the disease outcome. Age less than one year and an abrupt onset were associated with 100% mortality. Immunosuppression was associated with improvement in 66% of the patients.

The authors present a case report of hypoglycemic tumor (insulinoma) of the pancreas. The tumor had benign histology and the greatest volume among the tumors of this type described in the literature.