Long-term ventilation in children: Ten years later

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(A) The sequence of figures represent the adequate position to perform the scan: at the right angle to the patient's axis of vision, in a good alignment, closely adapted to the face and the pupil in the center of LCD screen. (B) The sequence of figures presents the pupil measurement phases: targeting phase (1), ready phase (2) and measurement phase. (C) Pupillometer display of one measurement results." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. Couto, D. Silva, P. Santos, S. Queirós, L. Delgado, A. Moreira" "autores" => array:6 [ 0 => array:2 [ "Iniciales" => "M." "apellidos" => "Couto" ] 1 => array:2 [ "Iniciales" => "D." "apellidos" => "Silva" ] 2 => array:2 [ "Iniciales" => "P." "apellidos" => "Santos" ] 3 => array:2 [ "Iniciales" => "S." "apellidos" => "Queirós" ] 4 => array:2 [ "Iniciales" => "L." "apellidos" => "Delgado" ] 5 => array:2 [ "Iniciales" => "A." "apellidos" => "Moreira" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X0873215915856126?idApp=UINPBA00004E" "url" => "/08732159/0000002100000001/v0_201604141151/X0873215915856126/v0_201604141151/en/main.assets" ] "itemAnterior" => array:16 [ "pii" => "X087321591585610X" "issn" => "08732159" "doi" => "10.1016/j.rppnen.2014.02.004" "estado" => "S300" "fechaPublicacion" => "2015-01-01" "documento" => "article" "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/" "subdocumento" => "fla" "cita" => "Rev Port Pneumol. 2015;21:11-5" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 9616 "formatos" => array:3 [ "EPUB" => 270 "HTML" => 8077 "PDF" => 1269 ] ] "en" => array:11 [ "idiomaDefecto" => true "titulo" => "The overall impact of COPD (CAT) and BODE index on COPD male patients: correlation?" 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"apellidos" => "Ladeira" ] 1 => array:2 [ "Iniciales" => "T." "apellidos" => "Gomes" ] 2 => array:2 [ "Iniciales" => "A." "apellidos" => "Castro" ] 3 => array:2 [ "Iniciales" => "C." "apellidos" => "Ribeiro" ] 4 => array:2 [ "Iniciales" => "M." "apellidos" => "Guimarães" ] 5 => array:2 [ "Iniciales" => "N." "apellidos" => "Taveira" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/X087321591585610X?idApp=UINPBA00004E" "url" => "/08732159/0000002100000001/v0_201604141151/X087321591585610X/v0_201604141151/en/main.assets" ] "en" => array:14 [ "idiomaDefecto" => true "titulo" => "Long-term ventilation in children: Ten years later" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "16" "paginaFinal" => "21" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "C. Cancelinha, N. Madureira, P. Mação, P. Pleno, T. Silva, M.H. Estêvão, M. Félix" "autores" => array:7 [ 0 => array:4 [ "Iniciales" => "C." "apellidos" => "Cancelinha" "email" => array:1 [ 0 => "candidacancelinha@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor1" ] ] ] 1 => array:3 [ "Iniciales" => "N." "apellidos" => "Madureira" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] 1 => array:2 [ "etiqueta" => "b" "identificador" => "affb" ] ] ] 2 => array:3 [ "Iniciales" => "P." "apellidos" => "Mação" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] 3 => array:3 [ "Iniciales" => "P." "apellidos" => "Pleno" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] 4 => array:3 [ "Iniciales" => "T." "apellidos" => "Silva" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] 5 => array:3 [ "Iniciales" => "M.H." "apellidos" => "Estêvão" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] 1 => array:2 [ "etiqueta" => "b" "identificador" => "affb" ] ] ] 6 => array:3 [ "Iniciales" => "M." "apellidos" => "Félix" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "affa" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Pediatric Pulmonology Unit, Pediatric Hospital, Coimbra University and Hospital Centre, Coimbra, Portugal" "etiqueta" => "a" "identificador" => "affa" ] 1 => array:3 [ "entidad" => "Sleep and Ventilation Laboratory, Pediatric Hospital, Coimbra University and Hospital Centre, Coimbra, Portugal" "etiqueta" => "b" "identificador" => "affb" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor1" "etiqueta" => "*" "correspondencia" => "Corresponding author. candidacancelinha@gmail.com" ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig1" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385611fig1.jpg" "Alto" => 843 "Ancho" => 1586 "Tamanyo" => 81996 ] ] "descripcion" => array:1 [ "en" => "Number of LTMV-10 children initiated on respiratory support per year." ] ] ] "textoCompleto" => "<a name="sec0005" class="elsevierStyleCrossRefs"></a>IntroductionIn the last two decades there has been an increase of children requiring long-term mechanical ventilation (LTMV) and, in many countries, programs of home mechanical ventilation (HMV) have been developed.<a href="#bib1" class="elsevierStyleCrossRefs">1</a>, <a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib4" class="elsevierStyleCrossRefs">4</a>, <a href="#bib5" class="elsevierStyleCrossRefs">5</a>, <a href="#bib6" class="elsevierStyleCrossRefs">6</a> Several factors have contributed to this: advances in neonatal and pediatric care with improved survival of children with acute respiratory insufficiency, technological advances and development of suitable home equipment, and increased use of polysomnography (PSG) to diagnose sleep disordered breathing.<a href="#bib5" class="elsevierStyleCrossRefs">5</a>, <a href="#bib7" class="elsevierStyleCrossRefs">7</a> Furthermore, pressure to reduce hospital stay duration and awareness that this environment is inappropriate for children, has resulted in increased number of patients being discharged on ventilatory support.<a href="#bib8" class="elsevierStyleCrossRefs">8</a>In this context, HMV, mainly non-invasive ventilation (NIV), provides domiciliary health care for patients with chronic respiratory failure. HMV combines significant change in family life, increased survival rate, quality of life improvement and decrease in hospital costs.<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib7" class="elsevierStyleCrossRefs">7</a>, <a href="#bib9" class="elsevierStyleCrossRefs">9</a>, <a href="#bib10" class="elsevierStyleCrossRefs">10</a>, <a href="#bib11" class="elsevierStyleCrossRefs">11</a>In 1993, a HMV program for patients with chronic respiratory failure began in our hospital, with a multidisciplinary team formed by pediatric pulmonology physicians and nurses, psychologists and social workers. In the last 20 years, more than 300 children have been supported with HMV.The aim of this study was to characterize the group of children and adolescents on HMV for a period longer than 10 years followed in our hospital and analyze compliance and impact of HMV. Patients on LTMV transferred to other institutions before a 10 years of follow-up, were excluded, although some may have kept ventilatory support.<a name="sec0010" class="elsevierStyleCrossRefs"></a>Materials and methodsAn observational study was performed through data collection and retrospective review of medical records of pediatric patients on HMV for longer than 10 years (LTMV-10) followed in our hospital. Starting point for follow-up was day one of ventilatory support and end point was the moment of data collection conclusion (31st August 2013), patient's transferal or death.Data collected included patient's age, gender, diagnosis, age at beginning of ventilatory support, indications for its use, ventilatory technique and mode of ventilation provided, previous overnight oximetry or PSG, length of time on HMV, patient's compliance and outcome.Data were collected and analyzed through PASW Statistics 18®.<a name="sec0015" class="elsevierStyleCrossRefs"></a>ResultsSince 1993, 31 children were under HMV for a period longer than 10 years and were followed in our hospital. Nine patients (29%) started HMV between 1993 and 1998 and 22 (71%) between 1999 and 2003 (<a href="#f0005" class="elsevierStyleCrossRefs">Figure 1</a>). The patients were categorized according to diagnosis: neuromuscular disorders (NMD) – 12 (39%), metabolic diseases – 7 (23%), central hypoventilation – 6 (19%), genetic syndromes – 2 (6%) and other neurological disorders – 4 (13%) (<a href="#t0005" class="elsevierStyleCrossRefs">Table 1</a>). Nineteen patients (61%) were female. Gender was equally distributed among all diagnostic groups.<a name="f0005" class="elsevierStyleCrossRefs"></a><img src="420v21n01-90385611fig1.jpg" alt="Number of LTMV-10 children initiated on respiratory support per year."></img>Figure 1. Number of LTMV-10 children initiated on respiratory support per year.Table 1. Diagnosis and age at starting of ventilatory support.<a name="t0005" class="elsevierStyleCrossRefs"></a><table><tr align="left"><td>Diagnosis</td><td>Total</td><td>Age median (min–max)</td></tr><tr align="left"><td>Neuromuscular disease</td><td>12</td><td> </td></tr><tr align="left"><td>CMD</td><td>5</td><td>6 yr (1–8 yr)</td></tr><tr align="left"><td>SMA type II</td><td>4</td><td>1.5 yr (11 mo to 9 yr)</td></tr><tr align="left"><td>SMA type III</td><td>1</td><td>2 yr</td></tr><tr align="left"><td>DMD</td><td>1</td><td>10 yr</td></tr><tr align="left"><td>Congenital myopathy</td><td>1</td><td>6 yr</td></tr><tr align="left"><td colspan="3"> </td></tr><tr align="left"><td>Metabolic disease</td><td>7</td><td> </td></tr><tr align="left"><td>MPS</td><td>5</td><td>11 yr (3–12 yr)</td></tr><tr align="left"><td>GSD type II</td><td>2</td><td>12 yr (11–13 yr)</td></tr><tr align="left"><td colspan="3"> </td></tr><tr align="left"><td>Central hypoventilation</td><td>6</td><td> </td></tr><tr align="left"><td>Congenital (CCHS)</td><td>5</td><td>0 mo</td></tr><tr align="left"><td>Acquired</td><td>1</td><td>2 yr</td></tr><tr align="left"><td colspan="3"> </td></tr><tr align="left"><td colspan="3">Genetic syndrome</td></tr><tr align="left"><td>Prader-Willi syndrome</td><td>2</td><td>3.5 yr (3–4 yr)</td></tr><tr align="left"><td colspan="3"> </td></tr><tr align="left"><td>Other neurological disorders</td><td>4</td><td> </td></tr><tr align="left"><td>Hypoxic ischemic encephalopathy + BPD</td><td>1</td><td>0 mo</td></tr><tr align="left"><td>Cervical spinal cord trauma</td><td>1</td><td>7 yr</td></tr><tr align="left"><td>Corpus callosum agenesis</td><td>1</td><td>3 yr</td></tr><tr align="left"><td>Unknown neurodegenerative disease</td><td>1</td><td>8 yr</td></tr></table>Legend: CCHS – congenital central hypoventilation syndrome; CMD – congenital muscular dystrophy; SMA – spinal muscular atrophy; DMD – Duchenne muscular dystrophy; MPS – mucopolysaccharidosis; GSD – glycogen storage disease; BPD – bronchopulmonar dysplasia; M – months; Y – years. The average age of beginning ventilatory support was three years, ranging from birth to 13 years depending on the diagnosis (<a href="#f0010" class="elsevierStyleCrossRefs">Figure 2</a>). Congenital central hypoventilation syndrome (CCHS) children were subsequently on NIV, starting between 11 days and 5 months of age, with the exception of one case that stayed on invasive ventilation (tracheostomy) since birth.<a name="f0010" class="elsevierStyleCrossRefs"></a><img src="420v21n01-90385611fig2.jpg" alt="Age at ventilatory support starting, according to congenital diagnosis. Legend: CCHS – congenital central hypoventilation syndrome; CMD – congenital muscular dystrophy; SMA – spinal muscular atrophy; DMD – Duchenne muscular dystrophy; MPS – mucopolysaccharidosis; GSD – glycogen storage disease."></img>Figure 2. Age at ventilatory support starting, according to congenital diagnosis. Legend: CCHS – congenital central hypoventilation syndrome; CMD – congenital muscular dystrophy; SMA – spinal muscular atrophy; DMD – Duchenne muscular dystrophy; MPS – mucopolysaccharidosis; GSD – glycogen storage disease.Ventilatory support was started electively in 17 (55%) children (<a href="#t0010" class="elsevierStyleCrossRefs">Table 2</a>). In 12 patients, this decision was supported by a full PSG or nocturnal oximetry: PSG showed hypoventilation in 3 congenital muscular dystrophy (CMD), 1 congenital myopathy and 1 mucopolysaccharidosis (MPS) and obstructive sleep apnea (OSA) in 2 MPS, 1 Duchenne muscular dystrophy (DMD) and 1 corpus callosum agenesis; recurrent desaturations on oximetry in 1 CMD, 1 Prader-Willi syndrome (PWS) and 1 glycogen storage disease (GSD) type II. In three children with spinal muscular atrophy (SMA) type II, ventilatory support was started in the first two years of life on a prophylactic basis. Two other patients (CMD and SMA type II) started ventilatory support due to clinical symptoms of hypoventilation.Table 2. Onset of ventilation – interface and mode of ventilation.<a name="t0010" class="elsevierStyleCrossRefs"></a><table><tr align="left"><td>Diagnosis</td><td>Total</td><td colspan="2">Starting</td><td colspan="2">Interface</td><td colspan="3">Mode of ventilation</td></tr><tr align="left"><td> </td><td> </td><td>Elective</td><td>Non-elective</td><td>Mask</td><td>Tracheostomy</td><td>BPAP</td><td>CPAP</td><td>Volume</td></tr><tr align="left"><td colspan="9">Neuromuscular disease</td></tr><tr align="left"><td>CMD</td><td>5</td><td>5</td><td>–</td><td>5</td><td>–</td><td>5</td><td>–</td><td>–</td></tr><tr align="left"><td>SMA type II</td><td>4</td><td>4</td><td>–</td><td>4</td><td>–</td><td>4</td><td>–</td><td>–</td></tr><tr align="left"><td>SMA type III</td><td>1</td><td>–</td><td>1</td><td>1</td><td>–</td><td>1</td><td>–</td><td>–</td></tr><tr align="left"><td>DMD</td><td>1</td><td>1</td><td>–</td><td>1</td><td>–</td><td>0</td><td>1</td><td>–</td></tr><tr align="left"><td>Congenital myopathy</td><td>1</td><td>1</td><td>–</td><td>1</td><td>–</td><td>1</td><td>–</td><td>–</td></tr><tr align="left"><td colspan="9"> </td></tr><tr align="left"><td colspan="9">Metabolic disease</td></tr><tr align="left"><td>MPS</td><td>5</td><td>3</td><td>2</td><td>5</td><td>–</td><td>2</td><td>2</td><td>1</td></tr><tr align="left"><td>GSD type II</td><td>2</td><td>1</td><td>1</td><td>2</td><td>–</td><td>2</td><td>–</td><td>–</td></tr><tr align="left"><td colspan="9"> </td></tr><tr align="left"><td colspan="9">Central hypoventilation</td></tr><tr align="left"><td>CCHS</td><td>5</td><td>–</td><td>5</td><td>4</td><td>1</td><td>4</td><td>–</td><td>1</td></tr><tr align="left"><td>Acquired</td><td>1</td><td>–</td><td>1</td><td>–</td><td>1</td><td>1</td><td>–</td><td>–</td></tr><tr align="left"><td colspan="9"> </td></tr><tr align="left"><td colspan="9">Genetic syndrome</td></tr><tr align="left"><td>Prader-Willi syndrome</td><td>2</td><td>1</td><td>1</td><td>2</td><td>–</td><td>1</td><td>1</td><td>–</td></tr><tr align="left"><td colspan="9"> </td></tr><tr align="left"><td colspan="9">Other neurological disorders</td></tr><tr align="left"><td>Hypoxic ischemic encephalopathy + BPD</td><td>1</td><td>–</td><td>1</td><td>–</td><td>1</td><td>1</td><td>–</td><td>–</td></tr><tr align="left"><td>Cervical spinal cord trauma</td><td>1</td><td>–</td><td>1</td><td>1</td><td>–</td><td>1</td><td>–</td><td>–</td></tr><tr align="left"><td>Corpus callosum agenesis</td><td>1</td><td>1</td><td>–</td><td>1</td><td>–</td><td>1</td><td>–</td><td>–</td></tr><tr align="left"><td>Unknown neurodegenerative disease</td><td>1</td><td>–</td><td>1</td><td>–</td><td>1</td><td>1</td><td>–</td><td>–</td></tr></table>Legend: BPD – bronchopulmonary dysplasia; CCHS – congenital central hypoventilation syndrome; CMD – congenital muscular dystrophy; SMA – spinal muscular atrophy; DMD – Duchenne muscular dystrophy; MPS – mucopolysaccharidosis; GSD – glycogen storage disease; BPD – bronchopulmonary dysplasia. Fourteen patients (45%) had ventilatory support initiated in emergency situations. Six started in the first days of life (<a href="#t0005" class="elsevierStyleCrossRefs">Table 1</a>) and could not be weaned from ventilation and four (2 MPS, 1 NMD and 1 GSD type II) began during an acute respiratory illness. The remaining encloses one PWS with hypercapnic coma, one neurodegenerative disease requiring tracheostomy and two previously healthy children (sequelae of pneumococcal meningitis and cervical spinal cord trauma).LTMV was started mainly by NIV in most children (n = 27, 87%). Invasive ventilation through tracheostomy was initiated in four children (<a href="#f0015" class="elsevierStyleCrossRefs">Figure 3</a>). During follow-up, two more patients required tracheostomy: one MPS with irreversible airway obstruction and one CMD before surgery for scoliosis.<a name="f0015" class="elsevierStyleCrossRefs"></a><img src="420v21n01-90385611fig3.jpg" alt="Ventilatory technique on onset and follow-up. Legend: LTMV-10 – Long-term mechanical ventilation for 10 years; T – tracheostomy; NIV – non-invasive ventilation; NIV-16–20 h – NIV for 16–20 h; NIV-sleep – NIV only during sleep."></img>Figure 3. Ventilatory technique on onset and follow-up. Legend: LTMV-10 – Long-term mechanical ventilation for 10 years; T – tracheostomy; NIV – non-invasive ventilation; NIV-16–20 h – NIV for 16–20 h; NIV-sleep – NIV only during sleep.Seven children (five on NIV and the two with tracheostomy) needed to increase the daily time of ventilation to 16–20 h or full day.Volume ventilation was used in two of the first children included and positive pressure ventilation in all the other cases, mainly bilevel positive airway pressure (BPAP) – 81% (<a href="#t0010" class="elsevierStyleCrossRefs">Table 2</a>). Continuous positive airway pressure (CPAP) was used in the four children with OSA. Three patients (1 DMD and 2 MPS) needed a subsequent change of mode of ventilation to BPAP due to association of alveolar hypoventilation.Other support equipment was provided according to patient’ needs: oxygen saturation monitor, humidification system, suction device, mechanical in-exsufflator and self-inflating resuscitation bag.During the last five years of follow-up, 8 (26%) patients had 5–10 respiratory infections, 12 (39%) had less than 5 and 11 (35%) had none. In the same period, 12 (39%) patients required hospitalization, mainly due to chronic respiratory failure agudization (6 NMD). The average number of hospitalizations was 0.17 per patient per year (maximum 1 per year). The first child enrolled in the study (MPS) was hospitalized 16 times during the year prior to ventilatory support initiation, 13 of which in the pediatric intensive care unit. The improvement of respiratory status after starting ventilation meant no need for admissions since then.The mean length of LTMV was 146 months (maximum 219 months). Six children (19%) had a fatal outcome: sudden death (CCHS and MPS), acute respiratory failure (MPS), septic shock (corpus callosum agenesis), cardio-respiratory arrest (CCHS) and unknown cause (neurodegenerative disease). Twelve children made the transition to adult respiratory services. One patient was lost to follow-up.Twelve children are currently relying on HMV and being followed in our hospital. The follow-up is performed every 3–12 months in outpatient clinics or during a programmed hospital admission. A multidisciplinary approach is performed in each evaluation, reinforcing the importance of ventilatory support, identifying and correcting problems, monitoring compliance and treating comorbidities. The overall compliance rate, based on technical ventilatory reports, is higher than 95% in each patient. Nine of the 12 children attend regular school.<a name="sec0020" class="elsevierStyleCrossRefs"></a>DiscussionHome LTMV represents a valid and proven alternative to long-term hospitalization of children with chronic respiratory failure. Its main advantages are more humanized environment for these patients, reduction in hospital-acquired infection and decrease in health care costs.<a href="#bib6" class="elsevierStyleCrossRefs">6</a>, <a href="#bib12" class="elsevierStyleCrossRefs">12</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a>Since the beginning of the HMV program in our hospital, the number of children treated has increased substantially, a trend also seen in other series.<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib4" class="elsevierStyleCrossRefs">4</a>, <a href="#bib5" class="elsevierStyleCrossRefs">5</a>, <a href="#bib14" class="elsevierStyleCrossRefs">14</a>, <a href="#bib15" class="elsevierStyleCrossRefs">15</a>One key difference between our series and others international reports are the diagnosis groups. This is related to the 10 years duration of ventilatory support. In our study there were no children with craniofacial abnormalities, airway malacia or bronchopulmonary dysplasia. These situations can improve with time due to physical growth or evolution of the disease, and so, ventilatory support is temporary.<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib6" class="elsevierStyleCrossRefs">6</a>, <a href="#bib14" class="elsevierStyleCrossRefs">14</a>, <a href="#bib15" class="elsevierStyleCrossRefs">15</a> In our first published series, concerning the use of domiciliary NIV during a six years’ period, 20% of 40 children included had suspended ventilation because they had improved or were cured: trachea/broncho malacia, macroglossia, diaphragmatic paresis and adenotonsillar hypertrophy associated with other situations.<a href="#bib16" class="elsevierStyleCrossRefs">16</a> The main disorders included in the present study, such as NMD and CCHS, are much less likely to allow discontinuation of ventilatory support.<a href="#bib11" class="elsevierStyleCrossRefs">11</a>, <a href="#bib12" class="elsevierStyleCrossRefs">12</a>, <a href="#bib15" class="elsevierStyleCrossRefs">15</a>We highlight how young the children of this series group were: 23% were younger than 1 year, compared with less than 10% in the Italian<a href="#bib12" class="elsevierStyleCrossRefs">12</a> and French<a href="#bib7" class="elsevierStyleCrossRefs">7</a> series. The median age (3 years) is also lower than in other studies (6–7 years).<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a> This may be affected by the 4 CCHS patients who began NIV during the first year of life, which was not observed in other series.<a href="#bib7" class="elsevierStyleCrossRefs">7</a>, <a href="#bib12" class="elsevierStyleCrossRefs">12</a> Although a few studies have demonstrated beneficial effects from the long term use of NIV technique in CCHS course, including in children aged from 5 weeks,<a href="#bib10" class="elsevierStyleCrossRefs">10</a>, <a href="#bib17" class="elsevierStyleCrossRefs">17</a> its early use is not consensual. According to recent literature, these children should initiate ventilation through tracheostomy in order to achieve optimal oxygenation and neurocognitive development.<a href="#bib18" class="elsevierStyleCrossRefs">18</a>NMD patients started HMV in different ages, as seen in other series, with median ages varying between 2 and 8 years.<a href="#bib11" class="elsevierStyleCrossRefs">11</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a> In children with SMA, the prophylactic respiratory support with NIV, also seen in our series, may improve quality of life, as well as prolong life expectancy.<a href="#bib10" class="elsevierStyleCrossRefs">10</a>, <a href="#bib19" class="elsevierStyleCrossRefs">19</a> In SMA type I, due to its more severe prognosis, it is more difficult to balance the ethical concepts of beneficence, non-maleficence, autonomy and distributive justice.<a href="#bib20" class="elsevierStyleCrossRefs">20</a>Six children (22%) were aged 10 years or more at onset of HMV. This group included not only children with NMD such as DMD, but also children with metabolic diseases (MPS, GSD type II) surviving through childhood. This is similar to other series,<a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib5" class="elsevierStyleCrossRefs">5</a> although cystic fibrosis is also a common diagnosis in adolescents on LTMV.<a href="#bib7" class="elsevierStyleCrossRefs">7</a> Surviving childhood, along with a cohort of young people requiring initiation of respiratory support in their teenage years, has led to a growing number of patients on HMV requiring transfer of care to adult services.<a href="#bib15" class="elsevierStyleCrossRefs">15</a> Adult medicine physicians are now faced with medical conditions they were not previously acquainted with. In our hospital, there is a transitional program to the adult services, ensuring a smooth transfer. Regarding DMD, the only patient included was initiated on CPAP at ten years old, due to obesity associated to OSA. Some years later he was switched to BPAP. Weakness of respiratory muscles usually evolves in crescendo in adolescence, but at an early stage, mainly if obesity or adenotonsillar enlargement is associated, OSA may appear and a respiratory support may be needed.<a href="#bib17" class="elsevierStyleCrossRefs">17</a>, <a href="#bib21" class="elsevierStyleCrossRefs">21</a>In our series, NIV technique was used in a higher percentage than in most of other reports (26–97%).<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib4" class="elsevierStyleCrossRefs">4</a>, <a href="#bib12" class="elsevierStyleCrossRefs">12</a>, <a href="#bib14" class="elsevierStyleCrossRefs">14</a>, <a href="#bib15" class="elsevierStyleCrossRefs">15</a> The major advantages of NIV, compared to invasive technique with tracheostomy, include greater patient comfort, simpler application, use and care, reduced incidence of complications<a href="#bib7" class="elsevierStyleCrossRefs">7</a> and higher survival rates.<a href="#bib15" class="elsevierStyleCrossRefs">15</a> The successful use of NIV has been demonstrated in children with OSA.<a href="#bib10" class="elsevierStyleCrossRefs">10</a>, <a href="#bib16" class="elsevierStyleCrossRefs">16</a> Furthermore, its use in children with NMD has become much more common after studies showing clear benefit in terms of outcomes and patient acceptability.<a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib10" class="elsevierStyleCrossRefs">10</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a>, <a href="#bib22" class="elsevierStyleCrossRefs">22</a>, <a href="#bib23" class="elsevierStyleCrossRefs">23</a>Positive pressure ventilation was used in most cases partially because these ventilators were more available and affordable. Volume-targeted ventilation is sometimes recommended in patients with NMD,<a href="#bib7" class="elsevierStyleCrossRefs">7</a> but for most children pressure-targeted machines work well and are simple to use.<a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a>, <a href="#bib24" class="elsevierStyleCrossRefs">24</a>, <a href="#bib25" class="elsevierStyleCrossRefs">25</a> The choice of the mode of ventilation depends on the medical team experience, but it is also the reflection of national ventilator company activity and other logistical or reimbursement policies.<a href="#bib1" class="elsevierStyleCrossRefs">1</a>Similarly to other studies, respiratory support was frequently initiated during an acute exacerbation.<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib7" class="elsevierStyleCrossRefs">7</a>, <a href="#bib12" class="elsevierStyleCrossRefs">12</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a>, <a href="#bib15" class="elsevierStyleCrossRefs">15</a> These data emphasize the need to discuss LTMV during outpatient visits prior to the development of respiratory failure. In our series, the number of NMD starting ventilator support electively (86%) was higher than in other studies (approximately 50%).<a href="#bib7" class="elsevierStyleCrossRefs">7</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a>Compliance to HMV was virtually 100%, a bit higher than in other series (86–97%).<a href="#bib3" class="elsevierStyleCrossRefs">3</a>, <a href="#bib7" class="elsevierStyleCrossRefs">7</a> As described by Ottonello et al., management of these patients requires an understanding of the specific technology involved in diagnosis and long-term care. In addition, a multidisciplinary approach is often warranted because of the complexity of the medical problems encountered in this children.<a href="#bib12" class="elsevierStyleCrossRefs">12</a>Our results agree with published data confirming low morbidity for home care ventilator dependent children.<a href="#bib4" class="elsevierStyleCrossRefs">4</a>, <a href="#bib5" class="elsevierStyleCrossRefs">5</a>, <a href="#bib11" class="elsevierStyleCrossRefs">11</a>, <a href="#bib13" class="elsevierStyleCrossRefs">13</a> A small number of hospitalizations was seen in the majority of patients. This fact and the possibility of attending school are good indicators of improvement in quality of life.<a href="#bib11" class="elsevierStyleCrossRefs">11</a>The overall mortality rate in our study was 19% (10–20% in other series).<a href="#bib2" class="elsevierStyleCrossRefs">2</a>, <a href="#bib4" class="elsevierStyleCrossRefs">4</a>, <a href="#bib12" class="elsevierStyleCrossRefs">12</a>, <a href="#bib14" class="elsevierStyleCrossRefs">14</a> Timely initiation of ventilatory support in children with NMD, as recommended in the recent British guidelines, may have influenced results.<a href="#bib25" class="elsevierStyleCrossRefs">25</a><a name="sec0025" class="elsevierStyleCrossRefs"></a>ConclusionHMV is expanding in pediatric population. As shown in our series, it is possible to keep on this respiratory support modality for long periods with good compliance, small number of hospitalizations and social reintegration. Close monitoring with a specialized team and a muldisciplinary approach, is essential for long term HMV success.<a name="sec0030" class="elsevierStyleCrossRefs"></a>Ethical disclosures<a name="sec0035" class="elsevierStyleCrossRefs"></a>Protection of human and animal subjectsThe authors declare that no experiments were performed on humans or animals for this study.<a name="sec0040" class="elsevierStyleCrossRefs"></a>Confidentiality of dataThe authors declare that they have followed the protocols of their work center on the publication of patient data and that all the patients included in the study received sufficient information and gave their written informed consent to participate in the study.<a name="sec0045" class="elsevierStyleCrossRefs"></a>Right to privacy and informed consentThe authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.<a name="sec0050" class="elsevierStyleCrossRefs"></a>Conflicts of interestThe authors have no conflicts of interest to declare.Received 16 January 2014 Accepted 28 March 2014 Corresponding author. candidacancelinha@gmail.com" "pdfFichero" => "320v21n01a90385612pdf001.pdf" "tienePdf" => true "PalabrasClave" => array:1 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec640999" "palabras" => array:4 [ 0 => "Long-term mechanical ventilation" 1 => "Home" 2 => "Chronic disease" 3 => "Ten years" ] ] ] ] "tieneResumen" => true "resumen" => array:1 [ "en" => array:1 [ "resumen" => "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." ] ] "multimedia" => array:6 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385611fig1.jpg" "Alto" => 843 "Ancho" => 1586 "Tamanyo" => 81996 ] ] "descripcion" => array:1 [ "en" => "Number of LTMV-10 children initiated on respiratory support per year." ] ] 1 => array:8 [ "identificador" => "fig2" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385611fig2.jpg" "Alto" => 1555 "Ancho" => 2903 "Tamanyo" => 210523 ] ] "descripcion" => array:1 [ "en" => "Age at ventilatory support starting, according to congenital diagnosis. Legend: CCHS ¿ congenital central hypoventilation syndrome; CMD ¿ congenital muscular dystrophy; SMA ¿ spinal muscular atrophy; DMD ¿ Duchenne muscular dystrophy; MPS ¿ mucopolysaccharidosis; GSD ¿ glycogen storage disease." ] ] 2 => array:8 [ "identificador" => "fig3" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "420v21n01-90385611fig3.jpg" "Alto" => 1807 "Ancho" => 2170 "Tamanyo" => 206135 ] ] "descripcion" => array:1 [ "en" => "Ventilatory technique on onset and follow-up. Legend: LTMV-10 ¿ Long-term mechanical ventilation for 10 years; T ¿ tracheostomy; NIV ¿ non-invasive ventilation; NIV-16¿20 h ¿ NIV for 16¿20 h; NIV-sleep ¿ NIV only during sleep." ] ] 3 => array:6 [ "identificador" => "fig4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "Number of LTMV-10 children initiated on respiratory support per year." ] ] 4 => array:6 [ "identificador" => "fig5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "Age at ventilatory support starting, according to congenital diagnosis. Legend: CCHS – congenital central hypoventilation syndrome; CMD – congenital muscular dystrophy; SMA – spinal muscular atrophy; DMD – Duchenne muscular dystrophy; MPS – mucopolysaccharidosis; GSD – glycogen storage disease." ] ] 5 => array:6 [ "identificador" => "fig6" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "descripcion" => array:1 [ "en" => "Ventilatory technique on onset and follow-up. Legend: LTMV-10 – Long-term mechanical ventilation for 10 years; T – tracheostomy; NIV – non-invasive ventilation; NIV-16–20 h – NIV for 16–20 h; NIV-sleep – NIV only during sleep." ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:25 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Patterns of home mechanical ventilation use in Europe: results from the Eurovent survey. 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Corresponding author. candidacancelinha@gmail.com

, N.. Madureiraa,b, P.. Maçãoa, P.. Plenoa, T.. Silvaa, M.H.. Estêvãoa,b, M.. Félixa

a Pediatric Pulmonology Unit, Pediatric Hospital, Coimbra University and Hospital Centre, Coimbra, Portugal

b Sleep and Ventilation Laboratory, Pediatric Hospital, Coimbra University and Hospital Centre, Coimbra, Portugal

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    "textoCompleto" => "<a name="sec0005" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Introduction</span><p class="elsevierStylePara">In the last two decades there has been an increase of children requiring long-term mechanical ventilation &#40;LTMV&#41; and&#44; in many countries&#44; programs of home mechanical ventilation &#40;HMV&#41; have been developed&#46;<a href="&#35;bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a> Several factors have contributed to this&#58; advances in neonatal and pediatric care with improved survival of children with acute respiratory insufficiency&#44; technological advances and development of suitable home equipment&#44; and increased use of polysomnography &#40;PSG&#41; to diagnose sleep disordered breathing&#46;<a href="&#35;bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> Furthermore&#44; pressure to reduce hospital stay duration and awareness that this environment is inappropriate for children&#44; has resulted in increased number of patients being discharged on ventilatory support&#46;<a href="&#35;bib8" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">8</span></a></p><p class="elsevierStylePara">In this context&#44; HMV&#44; mainly non-invasive ventilation &#40;NIV&#41;&#44; provides domiciliary health care for patients with chronic respiratory failure&#46; HMV combines significant change in family life&#44; increased survival rate&#44; quality of life improvement and decrease in hospital costs&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib9" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">9</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">10</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">11</span></a></p><p class="elsevierStylePara">In 1993&#44; a HMV program for patients with chronic respiratory failure began in our hospital&#44; with a multidisciplinary team formed by pediatric pulmonology physicians and nurses&#44; psychologists and social workers&#46; In the last 20 years&#44; more than 300 children have been supported with HMV&#46;</p><p class="elsevierStylePara">The aim of this study was to characterize the group of children and adolescents on HMV for a period longer than 10 years followed in our hospital and analyze compliance and impact of HMV&#46; Patients on LTMV transferred to other institutions before a 10 years of follow-up&#44; were excluded&#44; although some may have kept ventilatory support&#46;</p><a name="sec0010" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Materials and methods</span><p class="elsevierStylePara">An observational study was performed through data collection and retrospective review of medical records of pediatric patients on HMV for longer than 10 years &#40;LTMV-10&#41; followed in our hospital&#46; Starting point for follow-up was day one of ventilatory support and end point was the moment of data collection conclusion &#40;31st August 2013&#41;&#44; patient&#39;s transferal or death&#46;</p><p class="elsevierStylePara">Data collected included patient&#39;s age&#44; gender&#44; diagnosis&#44; age at beginning of ventilatory support&#44; indications for its use&#44; ventilatory technique and mode of ventilation provided&#44; previous overnight oximetry or PSG&#44; length of time on HMV&#44; patient&#39;s compliance and outcome&#46;</p><p class="elsevierStylePara">Data were collected and analyzed through PASW Statistics 18<span class="elsevierStyleSup">&#174;</span>&#46;</p><a name="sec0015" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Results</span><p class="elsevierStylePara">Since 1993&#44; 31 children were under HMV for a period longer than 10 years and were followed in our hospital&#46; Nine patients &#40;29&#37;&#41; started HMV between 1993 and 1998 and 22 &#40;71&#37;&#41; between 1999 and 2003 &#40;<a href="&#35;f0005" class="elsevierStyleCrossRefs">Figure 1</a>&#41;&#46; The patients were categorized according to diagnosis&#58; neuromuscular disorders &#40;NMD&#41; &#8211; 12 &#40;39&#37;&#41;&#44; metabolic diseases &#8211; 7 &#40;23&#37;&#41;&#44; central hypoventilation &#8211; 6 &#40;19&#37;&#41;&#44; genetic syndromes &#8211; 2 &#40;6&#37;&#41; and other neurological disorders &#8211; 4 &#40;13&#37;&#41; &#40;<a href="&#35;t0005" class="elsevierStyleCrossRefs">Table 1</a>&#41;&#46; Nineteen patients &#40;61&#37;&#41; were female&#46; Gender was equally distributed among all diagnostic groups&#46;</p><a name="f0005" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"><img src="420v21n01-90385611fig1.jpg" alt="Number of LTMV-10 children initiated on respiratory support per year&#46;"></img></p><p class="elsevierStylePara">Figure 1&#46; Number of LTMV-10 children initiated on respiratory support per year&#46;</p><p class="elsevierStylePara">Table 1&#46; Diagnosis and age at starting of ventilatory support&#46;</p><a name="t0005" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"></p><table><tr align="left"><td>Diagnosis</td><td>Total</td><td>Age median &#40;min&#8211;max&#41;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Neuromuscular disease</span></td><td>12</td><td>&#160;</td></tr><tr align="left"><td>CMD</td><td>5</td><td>6 yr &#40;1&#8211;8 yr&#41;</td></tr><tr align="left"><td>SMA type II</td><td>4</td><td>1&#46;5 yr &#40;11 mo to 9 yr&#41;</td></tr><tr align="left"><td>SMA type III</td><td>1</td><td>2 yr</td></tr><tr align="left"><td>DMD</td><td>1</td><td>10 yr</td></tr><tr align="left"><td>Congenital myopathy</td><td>1</td><td>6 yr</td></tr><tr align="left"><td colspan="3">&#160;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Metabolic disease</span></td><td>7</td><td>&#160;</td></tr><tr align="left"><td>MPS</td><td>5</td><td>11 yr &#40;3&#8211;12 yr&#41;</td></tr><tr align="left"><td>GSD type II</td><td>2</td><td>12 yr &#40;11&#8211;13 yr&#41;</td></tr><tr align="left"><td colspan="3">&#160;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Central hypoventilation</span></td><td>6</td><td>&#160;</td></tr><tr align="left"><td>Congenital &#40;CCHS&#41;</td><td>5</td><td>0 mo</td></tr><tr align="left"><td>Acquired</td><td>1</td><td>2 yr</td></tr><tr align="left"><td colspan="3">&#160;</td></tr><tr align="left"><td colspan="3"><span class="elsevierStyleItalic">Genetic syndrome</span></td></tr><tr align="left"><td>Prader-Willi syndrome</td><td>2</td><td>3&#46;5 yr &#40;3&#8211;4 yr&#41;</td></tr><tr align="left"><td colspan="3">&#160;</td></tr><tr align="left"><td><span class="elsevierStyleItalic">Other neurological disorders</span></td><td>4</td><td>&#160;</td></tr><tr align="left"><td>Hypoxic ischemic encephalopathy&#160;&#43;&#160;BPD</td><td>1</td><td>0 mo</td></tr><tr align="left"><td>Cervical spinal cord trauma</td><td>1</td><td>7 yr</td></tr><tr align="left"><td>Corpus callosum agenesis</td><td>1</td><td>3 yr</td></tr><tr align="left"><td>Unknown neurodegenerative disease</td><td>1</td><td>8 yr</td></tr></table><p class="elsevierStylePara">Legend&#58; CCHS &#8211; congenital central hypoventilation syndrome&#59; CMD &#8211; congenital muscular dystrophy&#59; SMA &#8211; spinal muscular atrophy&#59; DMD &#8211; Duchenne muscular dystrophy&#59; MPS &#8211; mucopolysaccharidosis&#59; GSD &#8211; glycogen storage disease&#59; BPD &#8211; bronchopulmonar dysplasia&#59; M &#8211; months&#59; Y &#8211; years&#46;<br></br></p><p class="elsevierStylePara">The average age of beginning ventilatory support was three years&#44; ranging from birth to 13 years depending on the diagnosis &#40;<a href="&#35;f0010" class="elsevierStyleCrossRefs">Figure 2</a>&#41;&#46; Congenital central hypoventilation syndrome &#40;CCHS&#41; children were subsequently on NIV&#44; starting between 11 days and 5 months of age&#44; with the exception of one case that stayed on invasive ventilation &#40;tracheostomy&#41; since birth&#46;</p><a name="f0010" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"><img src="420v21n01-90385611fig2.jpg" alt="Age at ventilatory support starting&#44; according to congenital diagnosis&#46; Legend&#58; CCHS &#8211; congenital central hypoventilation syndrome&#59; CMD &#8211; congenital muscular dystrophy&#59; SMA &#8211; spinal muscular atrophy&#59; DMD &#8211; Duchenne muscular dystrophy&#59; MPS &#8211; mucopolysaccharidosis&#59; GSD &#8211; glycogen storage disease&#46;"></img></p><p class="elsevierStylePara">Figure 2&#46; Age at ventilatory support starting&#44; according to congenital diagnosis&#46; Legend&#58; CCHS &#8211; congenital central hypoventilation syndrome&#59; CMD &#8211; congenital muscular dystrophy&#59; SMA &#8211; spinal muscular atrophy&#59; DMD &#8211; Duchenne muscular dystrophy&#59; MPS &#8211; mucopolysaccharidosis&#59; GSD &#8211; glycogen storage disease&#46;</p><p class="elsevierStylePara">Ventilatory support was started electively in 17 &#40;55&#37;&#41; children &#40;<a href="&#35;t0010" class="elsevierStyleCrossRefs">Table 2</a>&#41;&#46; In 12 patients&#44; this decision was supported by a full PSG or nocturnal oximetry&#58; PSG showed hypoventilation in 3 congenital muscular dystrophy &#40;CMD&#41;&#44; 1 congenital myopathy and 1 mucopolysaccharidosis &#40;MPS&#41; and obstructive sleep apnea &#40;OSA&#41; in 2 MPS&#44; 1 Duchenne muscular dystrophy &#40;DMD&#41; and 1 corpus callosum agenesis&#59; recurrent desaturations on oximetry in 1 CMD&#44; 1 Prader-Willi syndrome &#40;PWS&#41; and 1 glycogen storage disease &#40;GSD&#41; type II&#46; In three children with spinal muscular atrophy &#40;SMA&#41; type II&#44; ventilatory support was started in the first two years of life on a prophylactic basis&#46; Two other patients &#40;CMD and SMA type II&#41; started ventilatory support due to clinical symptoms of hypoventilation&#46;</p><p class="elsevierStylePara">Table 2&#46; Onset of ventilation &#8211; interface and mode of ventilation&#46;</p><a name="t0010" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"></p><table><tr align="left"><td>Diagnosis</td><td>Total</td><td colspan="2">Starting</td><td colspan="2">Interface</td><td colspan="3">Mode of ventilation</td></tr><tr align="left"><td>&#160;</td><td>&#160;</td><td>Elective</td><td>Non-elective</td><td>Mask</td><td>Tracheostomy</td><td>BPAP</td><td>CPAP</td><td>Volume</td></tr><tr align="left"><td colspan="9"><span class="elsevierStyleItalic">Neuromuscular disease</span></td></tr><tr align="left"><td>CMD</td><td>5</td><td>5</td><td>&#8211;</td><td>5</td><td>&#8211;</td><td>5</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td>SMA type II</td><td>4</td><td>4</td><td>&#8211;</td><td>4</td><td>&#8211;</td><td>4</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td>SMA type III</td><td>1</td><td>&#8211;</td><td>1</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td>DMD</td><td>1</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>0</td><td>1</td><td>&#8211;</td></tr><tr align="left"><td>Congenital myopathy</td><td>1</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td colspan="9">&#160;</td></tr><tr align="left"><td colspan="9"><span class="elsevierStyleItalic">Metabolic disease</span></td></tr><tr align="left"><td>MPS</td><td>5</td><td>3</td><td>2</td><td>5</td><td>&#8211;</td><td>2</td><td>2</td><td>1</td></tr><tr align="left"><td>GSD type II</td><td>2</td><td>1</td><td>1</td><td>2</td><td>&#8211;</td><td>2</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td colspan="9">&#160;</td></tr><tr align="left"><td colspan="9"><span class="elsevierStyleItalic">Central hypoventilation</span></td></tr><tr align="left"><td>CCHS</td><td>5</td><td>&#8211;</td><td>5</td><td>4</td><td>1</td><td>4</td><td>&#8211;</td><td>1</td></tr><tr align="left"><td>Acquired</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>1</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td colspan="9">&#160;</td></tr><tr align="left"><td colspan="9"><span class="elsevierStyleItalic">Genetic syndrome</span></td></tr><tr align="left"><td>Prader-Willi syndrome</td><td>2</td><td>1</td><td>1</td><td>2</td><td>&#8211;</td><td>1</td><td>1</td><td>&#8211;</td></tr><tr align="left"><td colspan="9">&#160;</td></tr><tr align="left"><td colspan="9"><span class="elsevierStyleItalic">Other neurological disorders</span></td></tr><tr align="left"><td>Hypoxic ischemic encephalopathy&#160;&#43;&#160;BPD</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>1</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td>Cervical spinal cord trauma</td><td>1</td><td>&#8211;</td><td>1</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td>Corpus callosum agenesis</td><td>1</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr><tr align="left"><td>Unknown neurodegenerative disease</td><td>1</td><td>&#8211;</td><td>1</td><td>&#8211;</td><td>1</td><td>1</td><td>&#8211;</td><td>&#8211;</td></tr></table><p class="elsevierStylePara">Legend&#58; BPD &#8211; bronchopulmonary dysplasia&#59; CCHS &#8211; congenital central hypoventilation syndrome&#59; CMD &#8211; congenital muscular dystrophy&#59; SMA &#8211; spinal muscular atrophy&#59; DMD &#8211; Duchenne muscular dystrophy&#59; MPS &#8211; mucopolysaccharidosis&#59; GSD &#8211; glycogen storage disease&#59; BPD &#8211; bronchopulmonary dysplasia&#46;<br></br></p><p class="elsevierStylePara">Fourteen patients &#40;45&#37;&#41; had ventilatory support initiated in emergency situations&#46; Six started in the first days of life &#40;<a href="&#35;t0005" class="elsevierStyleCrossRefs">Table 1</a>&#41; and could not be weaned from ventilation and four &#40;2 MPS&#44; 1 NMD and 1 GSD type II&#41; began during an acute respiratory illness&#46; The remaining encloses one PWS with hypercapnic coma&#44; one neurodegenerative disease requiring tracheostomy and two previously healthy children &#40;sequelae of pneumococcal meningitis and cervical spinal cord trauma&#41;&#46;</p><p class="elsevierStylePara">LTMV was started mainly by NIV in most children &#40;<span class="elsevierStyleItalic">n</span>&#160;&#61;&#160;27&#44; 87&#37;&#41;&#46; Invasive ventilation through tracheostomy was initiated in four children &#40;<a href="&#35;f0015" class="elsevierStyleCrossRefs">Figure 3</a>&#41;&#46; During follow-up&#44; two more patients required tracheostomy&#58; one MPS with irreversible airway obstruction and one CMD before surgery for scoliosis&#46;</p><a name="f0015" class="elsevierStyleCrossRefs"></a><p class="elsevierStylePara"><img src="420v21n01-90385611fig3.jpg" alt="Ventilatory technique on onset and follow-up&#46; Legend&#58; LTMV-10 &#8211; Long-term mechanical ventilation for 10 years&#59; T &#8211; tracheostomy&#59; NIV &#8211; non-invasive ventilation&#59; NIV-16&#8211;20&#160;h &#8211; NIV for 16&#8211;20&#160;h&#59; NIV-sleep &#8211; NIV only during sleep&#46;"></img></p><p class="elsevierStylePara">Figure 3&#46; Ventilatory technique on onset and follow-up&#46; Legend&#58; LTMV-10 &#8211; Long-term mechanical ventilation for 10 years&#59; T &#8211; tracheostomy&#59; NIV &#8211; non-invasive ventilation&#59; NIV-16&#8211;20&#160;h &#8211; NIV for 16&#8211;20&#160;h&#59; NIV-sleep &#8211; NIV only during sleep&#46;</p><p class="elsevierStylePara">Seven children &#40;five on NIV and the two with tracheostomy&#41; needed to increase the daily time of ventilation to 16&#8211;20&#160;h or full day&#46;</p><p class="elsevierStylePara">Volume ventilation was used in two of the first children included and positive pressure ventilation in all the other cases&#44; mainly bilevel positive airway pressure &#40;BPAP&#41; &#8211; 81&#37; &#40;<a href="&#35;t0010" class="elsevierStyleCrossRefs">Table 2</a>&#41;&#46; Continuous positive airway pressure &#40;CPAP&#41; was used in the four children with OSA&#46; Three patients &#40;1 DMD and 2 MPS&#41; needed a subsequent change of mode of ventilation to BPAP due to association of alveolar hypoventilation&#46;</p><p class="elsevierStylePara">Other support equipment was provided according to patient&#8217; needs&#58; oxygen saturation monitor&#44; humidification system&#44; suction device&#44; mechanical in-exsufflator and self-inflating resuscitation bag&#46;</p><p class="elsevierStylePara">During the last five years of follow-up&#44; 8 &#40;26&#37;&#41; patients had 5&#8211;10 respiratory infections&#44; 12 &#40;39&#37;&#41; had less than 5 and 11 &#40;35&#37;&#41; had none&#46; In the same period&#44; 12 &#40;39&#37;&#41; patients required hospitalization&#44; mainly due to chronic respiratory failure agudization &#40;6 NMD&#41;&#46; The average number of hospitalizations was 0&#46;17 per patient per year &#40;maximum 1 per year&#41;&#46; The first child enrolled in the study &#40;MPS&#41; was hospitalized 16 times during the year prior to ventilatory support initiation&#44; 13 of which in the pediatric intensive care unit&#46; The improvement of respiratory status after starting ventilation meant no need for admissions since then&#46;</p><p class="elsevierStylePara">The mean length of LTMV was 146 months &#40;maximum 219 months&#41;&#46; Six children &#40;19&#37;&#41; had a fatal outcome&#58; sudden death &#40;CCHS and MPS&#41;&#44; acute respiratory failure &#40;MPS&#41;&#44; septic shock &#40;corpus callosum agenesis&#41;&#44; cardio-respiratory arrest &#40;CCHS&#41; and unknown cause &#40;neurodegenerative disease&#41;&#46; Twelve children made the transition to adult respiratory services&#46; One patient was lost to follow-up&#46;</p><p class="elsevierStylePara">Twelve children are currently relying on HMV and being followed in our hospital&#46; The follow-up is performed every 3&#8211;12 months in outpatient clinics or during a programmed hospital admission&#46; A multidisciplinary approach is performed in each evaluation&#44; reinforcing the importance of ventilatory support&#44; identifying and correcting problems&#44; monitoring compliance and treating comorbidities&#46; The overall compliance rate&#44; based on technical ventilatory reports&#44; is higher than 95&#37; in each patient&#46; Nine of the 12 children attend regular school&#46;</p><a name="sec0020" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Discussion</span><p class="elsevierStylePara">Home LTMV represents a valid and proven alternative to long-term hospitalization of children with chronic respiratory failure&#46; Its main advantages are more humanized environment for these patients&#44; reduction in hospital-acquired infection and decrease in health care costs&#46;<a href="&#35;bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a></p><p class="elsevierStylePara">Since the beginning of the HMV program in our hospital&#44; the number of children treated has increased substantially&#44; a trend also seen in other series&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib14" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">14</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a></p><p class="elsevierStylePara">One key difference between our series and others international reports are the diagnosis groups&#46; This is related to the 10 years duration of ventilatory support&#46; In our study there were no children with craniofacial abnormalities&#44; airway malacia or bronchopulmonary dysplasia&#46; These situations can improve with time due to physical growth or evolution of the disease&#44; and so&#44; ventilatory support is temporary&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib6" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">6</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib14" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">14</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a> In our first published series&#44; concerning the use of domiciliary NIV during a six years&#8217; period&#44; 20&#37; of 40 children included had suspended ventilation because they had improved or were cured&#58; trachea&#47;broncho malacia&#44; macroglossia&#44; diaphragmatic paresis and adenotonsillar hypertrophy associated with other situations&#46;<a href="&#35;bib16" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">16</span></a> The main disorders included in the present study&#44; such as NMD and CCHS&#44; are much less likely to allow discontinuation of ventilatory support&#46;<a href="&#35;bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">11</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a></p><p class="elsevierStylePara">We highlight how young the children of this series group were&#58; 23&#37; were younger than 1 year&#44; compared with less than 10&#37; in the Italian<a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a> and French<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> series&#46; The median age &#40;3 years&#41; is also lower than in other studies &#40;6&#8211;7 years&#41;&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a> This may be affected by the 4 CCHS patients who began NIV during the first year of life&#44; which was not observed in other series&#46;<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a> Although a few studies have demonstrated beneficial effects from the long term use of NIV technique in CCHS course&#44; including in children aged from 5 weeks&#44;<a href="&#35;bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">10</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib17" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">17</span></a> its early use is not consensual&#46; According to recent literature&#44; these children should initiate ventilation through tracheostomy in order to achieve optimal oxygenation and neurocognitive development&#46;<a href="&#35;bib18" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">18</span></a></p><p class="elsevierStylePara">NMD patients started HMV in different ages&#44; as seen in other series&#44; with median ages varying between 2 and 8 years&#46;<a href="&#35;bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">11</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a> In children with SMA&#44; the prophylactic respiratory support with NIV&#44; also seen in our series&#44; may improve quality of life&#44; as well as prolong life expectancy&#46;<a href="&#35;bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">10</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib19" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">19</span></a> In SMA type I&#44; due to its more severe prognosis&#44; it is more difficult to balance the ethical concepts of beneficence&#44; non-maleficence&#44; autonomy and distributive justice&#46;<a href="&#35;bib20" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">20</span></a></p><p class="elsevierStylePara">Six children &#40;22&#37;&#41; were aged 10 years or more at onset of HMV&#46; This group included not only children with NMD such as DMD&#44; but also children with metabolic diseases &#40;MPS&#44; GSD type II&#41; surviving through childhood&#46; This is similar to other series&#44;<a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a> although cystic fibrosis is also a common diagnosis in adolescents on LTMV&#46;<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> Surviving childhood&#44; along with a cohort of young people requiring initiation of respiratory support in their teenage years&#44; has led to a growing number of patients on HMV requiring transfer of care to adult services&#46;<a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a> Adult medicine physicians are now faced with medical conditions they were not previously acquainted with&#46; In our hospital&#44; there is a transitional program to the adult services&#44; ensuring a smooth transfer&#46; Regarding DMD&#44; the only patient included was initiated on CPAP at ten years old&#44; due to obesity associated to OSA&#46; Some years later he was switched to BPAP&#46; Weakness of respiratory muscles usually evolves <span class="elsevierStyleItalic">in crescendo</span> in adolescence&#44; but at an early stage&#44; mainly if obesity or adenotonsillar enlargement is associated&#44; OSA may appear and a respiratory support may be needed&#46;<a href="&#35;bib17" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">17</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib21" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">21</span></a></p><p class="elsevierStylePara">In our series&#44; NIV technique was used in a higher percentage than in most of other reports &#40;26&#8211;97&#37;&#41;&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib14" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">14</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a> The major advantages of NIV&#44; compared to invasive technique with tracheostomy&#44; include greater patient comfort&#44; simpler application&#44; use and care&#44; reduced incidence of complications<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> and higher survival rates&#46;<a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a> The successful use of NIV has been demonstrated in children with OSA&#46;<a href="&#35;bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">10</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib16" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">16</span></a> Furthermore&#44; its use in children with NMD has become much more common after studies showing clear benefit in terms of outcomes and patient acceptability&#46;<a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib10" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">10</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib22" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">22</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib23" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">23</span></a></p><p class="elsevierStylePara">Positive pressure ventilation was used in most cases partially because these ventilators were more available and affordable&#46; Volume-targeted ventilation is sometimes recommended in patients with NMD&#44;<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> but for most children pressure-targeted machines work well and are simple to use&#46;<a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib24" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">24</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib25" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">25</span></a> The choice of the mode of ventilation depends on the medical team experience&#44; but it is also the reflection of national ventilator company activity and other logistical or reimbursement policies&#46;<a href="&#35;bib1" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">1</span></a></p><p class="elsevierStylePara">Similarly to other studies&#44; respiratory support was frequently initiated during an acute exacerbation&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib15" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">15</span></a> These data emphasize the need to discuss LTMV during outpatient visits prior to the development of respiratory failure&#46; In our series&#44; the number of NMD starting ventilator support electively &#40;86&#37;&#41; was higher than in other studies &#40;approximately 50&#37;&#41;&#46;<a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a></p><p class="elsevierStylePara">Compliance to HMV was virtually 100&#37;&#44; a bit higher than in other series &#40;86&#8211;97&#37;&#41;&#46;<a href="&#35;bib3" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">3</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib7" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">7</span></a> As described by Ottonello et al&#46;&#44; management of these patients requires an understanding of the specific technology involved in diagnosis and long-term care&#46; In addition&#44; a multidisciplinary approach is often warranted because of the complexity of the medical problems encountered in this children&#46;<a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a></p><p class="elsevierStylePara">Our results agree with published data confirming low morbidity for home care ventilator dependent children&#46;<a href="&#35;bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib5" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">5</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">11</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib13" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">13</span></a> A small number of hospitalizations was seen in the majority of patients&#46; This fact and the possibility of attending school are good indicators of improvement in quality of life&#46;<a href="&#35;bib11" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">11</span></a></p><p class="elsevierStylePara">The overall mortality rate in our study was 19&#37; &#40;10&#8211;20&#37; in other series&#41;&#46;<a href="&#35;bib2" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib4" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">4</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib12" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">12</span></a><span class="elsevierStyleSup">&#44; </span><a href="&#35;bib14" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">14</span></a> Timely initiation of ventilatory support in children with NMD&#44; as recommended in the recent British guidelines&#44; may have influenced results&#46;<a href="&#35;bib25" class="elsevierStyleCrossRefs"><span class="elsevierStyleSup">25</span></a></p><a name="sec0025" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Conclusion</span><p class="elsevierStylePara">HMV is expanding in pediatric population&#46; As shown in our series&#44; it is possible to keep on this respiratory support modality for long periods with good compliance&#44; small number of hospitalizations and social reintegration&#46; Close monitoring with a specialized team and a muldisciplinary approach&#44; is essential for long term HMV success&#46;</p><a name="sec0030" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Ethical disclosures</span><a name="sec0035" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Protection of human and animal subjects</span><p class="elsevierStylePara">The authors declare that no experiments were performed on humans or animals for this study&#46;</p><a name="sec0040" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Confidentiality of data</span><p class="elsevierStylePara">The authors declare that they have followed the protocols of their work center on the publication of patient data and that all the patients included in the study received sufficient information and gave their written informed consent to participate in the study&#46;</p><a name="sec0045" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Right to privacy and informed consent</span><p class="elsevierStylePara">The authors have obtained the written informed consent of the patients or subjects mentioned in the article&#46; The corresponding author is in possession of this document&#46;</p><a name="sec0050" class="elsevierStyleCrossRefs"></a><span class="elsevierStyleSectionTitle">Conflicts of interest</span><p class="elsevierStylePara">The authors have no conflicts of interest to declare&#46;</p><p class="elsevierStylePara">Received 16 January 2014 <br></br>Accepted 28 March 2014 </p><p class="elsevierStylePara">Corresponding author&#46; candidacancelinha&#64;gmail&#46;com</p>"
    "pdfFichero" => "320v21n01a90385612pdf001.pdf"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Introduction</span><br/><p class="elsevierStylePara">Home mechanical ventilation &#40;HMV&#41; represents a treatment option for patients with chronic respiratory failure and has changed prognosis and survival of many disorders in children&#46; The aim of this study was to characterize a group of children on long-term mechanical ventilation &#40;LTMV&#41; for a period longer than 10 years&#46;</p><span class="elsevierStyleSectionTitle">Methods</span><br/><p class="elsevierStylePara">A retrospective analysis was carried out including patients on LTMV for more than 10 years &#40;LTMV-10&#41; in a tertiary pediatric hospital&#46; Statistical analysis&#58; PASW Statistics 18<span class="elsevierStyleSup">&#174;</span>&#46;</p><span class="elsevierStyleSectionTitle">Results</span><br/><p class="elsevierStylePara">Thirty-one children &#40;61&#37; female&#41; belong to the LTMV-10 group&#46; Median age at the beginning of ventilatory support was 3 years &#40;birth to 13 years&#41;&#46; Main indications for assisted ventilation were neuromuscular disease &#40;<span class="elsevierStyleItalic">n</span>&#160;&#61;&#160;12&#44; 39&#37;&#41;&#44; metabolic disease &#40;<span class="elsevierStyleItalic">n</span>&#160;&#61;&#160;7&#44; 23&#37;&#41; and central hypoventilation &#40;<span class="elsevierStyleItalic">n</span>&#160;&#61;&#160;6&#44; 19&#37;&#41;&#46; Volume ventilation was used in 2 children&#44; and positive pressure ventilation in the others&#44; mainly bilevel positive airway pressure &#40;<span class="elsevierStyleItalic">n</span>&#160;&#61;&#160;25&#44; 81&#37;&#41;&#46; Invasive ventilation via tracheostomy was used since the beginning in four cases&#44; and subsequently in two other children&#46; The mean time of ventilatory support was 146 months and the maximum was 219 months&#46; Respiratory morbidity was the most frequent cause of hospitalization and the annual rate of such episodes was 0&#46;17 per child&#46; Global mortality rate was 19&#37;&#46;</p><span class="elsevierStyleSectionTitle">Conclusions</span><br/><p class="elsevierStylePara">HMV programs provide necessary and safe assistance for children with severe chronic respiratory failure&#46; As shown in our series&#44; it is possible to be kept on this respiratory support modality for long periods with good compliance and a small number of hospitalizations&#46;</p>"
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Article information

ISSN: 08732159
Original language: English

DOI:10.1016/j.rppnen.2014.03.017

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Year/Month	Html	Pdf	Total

2024 November	16	8	24
2024 October	88	49	137
2024 September	107	28	135
2024 August	139	39	178
2024 July	147	48	195
2024 June	117	25	142
2024 May	109	51	160
2024 April	104	29	133
2024 March	104	35	139
2024 February	67	29	96
2024 January	71	28	99
2023 December	65	25	90
2023 November	60	34	94
2023 October	53	30	83
2023 September	78	31	109
2023 August	69	28	97
2023 July	49	37	86
2023 June	56	19	75
2023 May	101	24	125
2023 April	68	26	94
2023 March	96	29	125
2023 February	108	31	139
2023 January	51	19	70
2022 December	85	20	105
2022 November	130	49	179
2022 October	105	46	151
2022 September	64	39	103
2022 August	94	40	134
2022 July	81	57	138
2022 June	69	43	112
2022 May	72	37	109
2022 April	58	42	100
2022 March	46	55	101
2022 February	48	43	91
2022 January	47	49	96
2021 December	38	40	78
2021 November	52	45	97
2021 October	56	46	102
2021 September	47	53	100
2021 August	75	43	118
2021 July	56	40	96
2021 June	83	49	132
2021 May	63	32	95
2021 April	212	88	300
2021 March	139	36	175
2021 February	134	26	160
2021 January	105	20	125
2020 December	87	12	99
2020 November	83	31	114
2020 October	83	20	103
2020 September	124	18	142
2020 August	135	45	180
2020 July	151	25	176
2020 June	136	22	158
2020 May	132	28	160
2020 April	135	12	147
2020 March	127	15	142
2020 February	113	25	138
2020 January	137	31	168
2019 December	147	43	190
2019 November	124	15	139
2019 October	152	29	181
2019 September	166	50	216
2019 August	262	41	303
2019 July	254	35	289
2019 June	258	15	273
2019 May	287	44	331
2019 April	220	32	252
2019 March	223	16	239
2019 February	191	9	200
2019 January	195	28	223
2018 December	113	4	117
2018 November	43	1	44
2018 October	53	17	70
2018 September	33	5	38
2018 August	30	21	51
2018 July	26	18	44
2018 June	27	15	42
2018 May	24	21	45
2018 April	50	24	74
2018 March	42	22	64
2018 February	12	9	21
2018 January	18	21	39
2017 December	20	18	38
2017 November	28	20	48
2017 October	13	13	26
2017 September	19	13	32
2017 August	19	11	30
2017 July	17	14	31
2017 June	21	19	40
2017 May	21	23	44
2017 April	13	4	17
2017 March	15	14	29
2017 February	6	3	9
2017 January	6	9	15
2016 December	17	12	29
2016 November	14	4	18
2016 October	9	4	13
2016 September	2	1	3
2016 August	4	3	7
2016 July	7	12	19
2016 June	16	10	26
2016 May	12	4	16
2016 April	46	16	62
2016 March	75	35	110
2016 February	67	25	92
2016 January	52	25	77
2015 December	50	16	66
2015 November	48	16	64
2015 October	58	26	84
2015 September	40	23	63
2015 August	31	12	43
2015 July	30	9	39
2015 June	20	13	33
2015 May	46	25	71
2015 April	63	34	97
2015 March	98	34	132
2015 February	80	47	127

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