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Objective: Home mechanical ventilation is used with patients with severe, chronic respiratory failure, a condition that has a serious impact on quality of life. The aim of this study was to produce a translation and cultural adaptation of the Severe Respiratory Insufficiency. Questionnaire for the Spanish population, the first health-related quality of life questionnaire specifically designed for patients receiving home mechanical ventilation., Methods: Four bilingual German-Spanish translators were used to translate and back-translate the questionnaire. Meetings were held with the translators following each step of the translation process to produce a single version that could be used in the next step. At the end of the process, the questionnaire was piloted to assess its comprehensibility. A scoring system using a scale of 1 (lowest) to 10 (highest) was used to rate both translation difficulty and the naturalness of the language produced. The equivalence of the original and translated items was also evaluated., Results: Three Spanish versions of the questionnaire were produced. Task difficulty was rated as quite low: the mean (SD) ratings were 1.4 (0.6) for translation and 2.2 (1.1) for back translation. The naturalness of the translated items was rated as very high, with scores improving with the successive versions (version 1, 8.4; version 2, 8.7; version 3, 9.1; P< .001). Thirty of the questionnaire items (61.2%) were judged to be fully equivalent, 13 (26.5%) to be similar, and 6 (12.2%) to be non-equivalent. The meaning conveyed by 5 of the items was changed or clarified during piloting., Conclusions: The translation of the questionnaire using the translation-back-translation procedure has produced a version that is both comparable to the original and accessible to the Spanish population. Its validity is currently being tested in a multicenter study.

We report the case of a Duchenne muscular dystrophy patient with good bulbar function but severely decreased forced vital capacity (9%) and spontaneous peak cough flow (PCF) (2.35 L/s). The patient needed continuous noninvasive ventilation (NIV) consisting of a volumetric ventilator with a nighttime nasal mask and a daytime mouthpiece. He also required application of manually assisted coughing techniques by insufflation with a resuscitation bag and chest thrust (manually assisted PCF after maximum insufflation capacity of 4.33 L/s). An episode of serious food aspiration was resolved by his main caregiver through NIV and manually assisted coughing. Bronchoscopy under sedation using NIV with a lip seal connection to his volumetric ventilator later revealed that no material remained. This case exemplifies the potential role of skilled respiratory management in some neuromuscular diseases.

Introduction: In spite of the great variability in clinical signs in the mitochondrial disorders, reports of respiratory disorders in these conditions are purely anecdotal, and almost all occurred in patients with clinical data of the illness. We present a case in which respiratory insufficiency was the first sign of a mitochondrial disorder., Case Report: A 74 year old patient, with no significant clinical history, had two episodes of severe acute respiratory insufficiency without cardiopulmonary causes to justify it. The patient was investigated for neuromuscular disorders and had a muscle biopsy compatible with mitochondrial myopathy., Conclusion: The occurrence of respiratory failure in patients with mitochondrial disorders is exceptional, is not related to the degree of deterioration of the respiratory function tests and is potentially reversible.

Objective: Protein-energy malnutrition (PEM) constitutes an important complication in children submitted to intensive care treatment for respiratory failure (RF). This complication is not usually evaluated in assessment protocols. Nutrition assessment in a Pediatric Intensive Care Unit (PICU) was performed in children with respiratory failure., Patients and Methods: A prospective study involving 65 children (1-158 months of age) with RF at admission to the PICU was carried out. The patients studied were divided into four groups according to their etiology: Group I (n = 29) included those with acute bronchial pathology; Group II (n = 17) those with pneumonia: Group III (n = 11) those in the post-operative period following cardiac surgery; and Group IV (n = 8) those patients with multiple-organ dysfunction. All patients in Groups I, II, and III had chronic diseases. Anthropometrical and nutritional evaluation included weight, height and body mass index (w/h2)., Results: The weight for age was low (59%). Height deficit was observed in a significantly lower percentage of the patients (13.1%). The body mass index showed values in relationship to the 50th percentile that varied between 83.4 +/- 23.1% and 97.1 +/- 4.7%. In 68.9% of the patients the criteria of global malnutrition were met according to the classification of McLaren and Read., Conclusions: The data confirm that PEM is frequent in patients with RF in the PICU and emphasize the usefulness of anthropometrical evaluation as an important, simple and non-invasive method of nutritional evaluation. Early and intensive nutritional intervention can reduce or prevent the vicious circle of malnutrition in RF patients hospitalized in the PICU.