Organization of Home Mechanical Ventilation in Portugal: Characterization of current centers and a pathway to uniformization.

INTRODUCTION: Home Mechanical Ventilation (HMV) is increasing worldwide. OBJECTIVE: Characterization of the Portuguese HMV Units. METHODS: The HMV Team Group of the Portuguese Pulmonology Society prepared a questionnaire that was sent by e-mail addressed to Pneumology Department Directors throughout the country, and the responses were then analyzed. The results enabled a provisional classification of the Units, which followed specific criteria. RESULTS: Thirty centers were surveyed, of which 60% (18) sent the answers to the questionnaire. As for the results obtained, only one center was considered as a basic unit. Most centers (14/18) were considered specialized units. 3/18 centers were classified as highly complex multidisciplinary units. Of the 12 centers that did not answer the questionnaire, one refused to do it and another center was in transition period. CONCLUSIONS: Analysis of the results reveals the high number of patients treated with HMV in Portugal, supports the importance of creating protocols to standardize HMV countrywide, and audit its practice through the creation of a national register.

Portuguese consensus document for the management of alpha-1-antitrypsin deficiency.

Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic disorders, although it remains underdiagnosed. Whereas at international level there are several areas of consensus on this disorder, in Portugal, inter-hospital heterogeneity in clinical practice and resources available have been adding difficulties in reaching a diagnosis and in making therapeutic decisions in this group of patients. This raised a need to draft a document expressing a national consensus for AATD. To this end, a group of experts in this field was created within the Portuguese Pulmonology Society - Study group on AATD, in order to elaborate the current manuscript. The authors reviewed the existing literature and provide here general guidance and extensive recommendations for the diagnosis and management of AATD that can be adopted by Portuguese clinicians from different areas of Medicine. This article is part of a supplement entitled "Portuguese consensus document for the management of alpha-1-antitrypsin deficiency" which is sponsored by Sociedade Portuguesa de Pneumologia.