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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Amyotrophic lateral sclerosis &#40;ALS&#41; is a devastating disease involving both upper and lower motor neurons&#46; It is the most common form of motor neuron disease&#44; affecting approximately 1&#46;2 to 1&#46;8&#47;100&#44;000 individuals&#46; Clinically&#44; ALS is characterised by progressive muscular weakness leading to loss of the ability to move and speak and&#44; in almost all patients&#44; causes death between 3 and 5 years from diagnosis&#46; Cortical and cognitive functions remain essentially unchanged&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Respiratory failure is the most common cause of morbidity and mortality in these patients&#46; Reduction in respiratory muscle strength with ineffective alveolar ventilation and difficult airway secretions clearance may lead to chronic respiratory insufficiency &#40;CRI&#41; and potentially life-threatening problems&#46; Long term mechanical ventilation &#40;LTMV&#41; delivered either invasively or non invasively is the main therapeutic intervention to support the respiratory muscle function and to increase life expectancy and health-related quality of life &#40;QOL&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Literature evaluating survival of ALS patients receiving LTMV via tracheostomy is scarce and contains conflicting reports&#44; with considerable variation among studies as to the time between starting this treatment and death&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Quality control of the equipment used and risk management are important aspects of LTMV in order to ensure that patients receive the prescribed ventilatory support safely and accurately&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;5</span></a> Deaths due to malfunction of mechanical ventilators are reported but forensic autopsy records are rare&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> We report the case of a 69-year-old trachostomised&#44; ventilator-dependent ALS female patient&#44; found dead by her husband&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Case report</span><p id="par0020" class="elsevierStylePara elsevierViewall">A 69-year-old female had been diagnosed as having had ALS for 2 years&#46; She had been 24&#47;24<span class="elsevierStyleHsp" style=""></span>h ventilator-dependent&#44; under tracheostomy LTMV for 18 month after a previous 9-month period of mask ventilation&#46; Lung function test at initiation of NIV showed mild restriction &#40;slow vital capacity &#40;SVC&#41;&#58; 61&#37; pred&#44; forced expiratory volume at 1<span class="elsevierStyleHsp" style=""></span>s &#40;FEV1&#41;&#58; 60&#37; pred&#44; FEV1&#47;SVC&#58;99&#37;&#41;&#46; Tracheostomy had been electively performed after progressive worsening of gas exchanges &#40;PaCO2 59<span class="elsevierStyleHsp" style=""></span>mmHg&#41; symptoms and lack of compliance to NIV&#46; In fact&#44; the patient had not tolerated NIV for more than 2<span class="elsevierStyleHsp" style=""></span>h per night&#46; She was also on enteral nutrition &#40;EN&#41; through percutaneous gastrostomy &#40;PEG&#41;&#46; In the last 12 months the patients underwent two admissions to the emergency department for non-respiratory causes&#44; and had no major problem in the last four months before she was found dead in her bed by her husband&#46; No resuscitation maneuver was performed&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The night before&#44; her husband had checked ventilator and EN pump and had found nothing unusual&#46; Early in the morning he was woken up by the alarm of the EN pump&#58; he realized that his wife was not breathing and pulseless&#46; The ventilator was in &#8220;stand- by&#8221; mode&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">At autopsy&#44; gross examination did not reveal any trauma sign but showed brain oedema and congestion and lung congestion&#46; Pulmonary histological examination revealed a slightly thickening of peribronchial interstitial space&#44; alveolar overdistension with alveolar rupture and diffuse alveolar haemorrhage&#44; alveolar capillary congestion &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 1</a>&#41;&#46; Focal microhemorrhages were detected also in lungs&#44; kidneys&#44; liver&#44; heart&#44; brain and adrenal glands tissue &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 2</a>&#41;&#46; Oxicological examination was negative&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Analysis of the internal memory of the ventilator&#44; showed that during the night&#44; several voltage drops had happened&#46; Specific technical tests revealed malfunction of the ventilator internal battery&#44; unable to provide the necessary voltage had caused the ventilator stop&#46; Battery malfunction also reduced the alarm volume which was not heard by caregiver&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">Respiratory failure is the most common cause of death in ALS patients&#46; In this case&#44; cause of death was attributed to ventilator pump failure related to acute asphyxia&#46; Asphyxia is a broad term including a variety of conditions resulting in interference with oxygen uptake or utilization&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> particularly in the brain&#46; In the forensic context&#44; death typically results from mechanically induced cerebral hypoxia&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">All ventilators provided with an expiratory valve allow the patient to breath in room air in case of ventilator malfunctioning&#46; The valve always remains open when the ventilator stops&#46; Only a severe valve malfunctioning can explain asphyxia in a patient still actively breathing&#46; However the likelihood of this valve malfunctioning is very low because in case of valve membrane rupture &#40;so called mushroom valve&#41; the valve leaks still allow the patients to breath in room air&#46; But in this case it is not likely that our 24&#47;24 hour ventilatory-dependent patient would still have the residual strength for breathing activity and this would explain the acute asphyxia&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Pulmonary histological examination revealed thickening of peribronchial interstitial space&#44; alveolar over-distension&#44; break of inter-alveolar walls and diffuse alveolar haemorrhages that can also go along with the hypothesis of barotraumas in a long term ventilated patient&#46; In the present case histological pattern showed high grade of acute pulmonary emphysema allowing us to hypothesize long-term barotrauma effect&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Ventilator-dependent patient management&#44; including periodic check and maintenance of the ventilator&#44; is crucial&#46; A survey covering 16 European countries showed that&#58; &#40;1&#41; ventilator servicing was mainly carried out by external companies&#44; with a servicing frequency ranging 3&#8211;12 months&#59; &#40;2&#41; interaction between servicing companies and prescribers was limited&#59; &#40;3&#41; participation of centres in equipment quality control was poor&#59; and &#40;4&#41; centres were insufficiently aware of vigilance systems&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Chatwin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> examined the nature of calls to a home support helpline to identify patient&#47;equipment problems and strategies to minimise risk for patients&#44; healthcare teams and manufacturers&#46; From adult and paediatric patients with neuromuscular disease&#44; chronic obstructive pulmonary disease or chest wall disease receiving LTMV&#44; and all calls to a dedicated respiratory support telephone hotline in a six-month period were analysed&#46; Out of 1211 patients 1119 received non-invasive ventilation&#44; 12 tracheostomy ventilation&#59; 149 had two ventilators for 24-h ventilator dependency&#46; There was a mean of 528 daytime calls per month and 14 calls a month at night&#46; Following 188 calls&#44; a home visit was performed&#59; these identified a technical problem that could either be solved in the patient&#39;s home in 64&#37; or required replacement or new parts in 22&#37; of cases&#46; In 25 calls in which no mechanical fault was identified&#44; 13 patients were either found to be unwell or required hospital admission&#46; Authors conclude that patients using LTMV have a substantial need for assistance&#44; with most technical problems being resolved simply&#46; Where no fault was found during an equipment check&#44; the patient themselves may be unwell and should receive early clinical evaluation&#46; The patient may have mistaken clinical deterioration for an equipment problem&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Ethical disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Protection of human and animal subjects</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Confidentiality of data</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Right to privacy and informed consent</span><p id="par0070" class="elsevierStylePara elsevierViewall">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></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Conflicts of interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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            0 => "Amyotrophic lateral sclerosis"
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            0 => "Esclerose lateral amiotr&#243;fica"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Background</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Amyotrophic lateral sclerosis &#40;ALS&#41; is a fatal&#44; progressive&#44; neurodegenerative disease and most patients affected die of respiratory compromise and&#47;or pneumonia within 2&#8211;3 years of diagnosis&#46; As ALS progresses&#44; ventilator assistance is required&#46; In the end stages of the disease&#44; patients suffer from respiratory failure and may become ventilator-dependent&#46; Deaths due to malfunction of mechanical ventilators are reported but there are very few forensic autopsy records&#46; We report the case of a 69-year-old ALS female ventilator-dependent&#44; trachostomised patient who was found dead by her husband&#44; with the ventilator in &#8220;stand-by&#8221; mode&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Method</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A forensic autopsy was performed&#46; Samples of internal organs were taken for histological and toxicological examination&#46; The ventilator internal memory was also analysed and tested in order to find possible malfunction&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Gross examination did not reveal any sign of trauma but showed brain and lung congestion&#46; Pulmonary histological examination revealed thickening of peribronchial interstitial space&#44; alveolar over-distension&#44; break of inter-alveolar walls and diffuse alveolar haemorrhages&#46; Focal microhemorrhages were also detected in other organs&#46; Analysis of the ventilator internal memory showed that during the night of death&#44; there had been several voltage drops&#46; Specific tests revealed malfunction of the internal battery which was unable to provide the necessary voltage&#44; as a consequence the ventilator switched off&#44; stopping ventilation&#46; Battery malfunction reduced the volume of the ventilator alarm&#44; which was not heard by the caregiver&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Histological pattern&#44; with acute pulmonary emphysema and focal polivisceral haemorrhages&#44; is strongly suggestive of a death due to &#8220;acute&#8221; asphyxia&#46; The authors also discuss the need for strict supervision and follow up of these ventilatory dependent patients and their devices&#46;</p></span>"
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        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introdu&#231;&#227;o</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A Esclerose Lateral Amiotr&#243;fica &#40;ELA&#41; &#233; uma doen&#231;a fatal&#44; progressiva e neurodegenerativa e a maioria dos doentes afectados morrer&#227;o de falha respirat&#243;ria e&#47;ou pneumonia 2 ou 3 anos ap&#243;s o diagn&#243;stico&#46; &#192; medida que a ELA progride torna-se necess&#225;ria a assist&#234;ncia ventilat&#243;ria&#46; Nos est&#225;gios finais da doen&#231;a&#44; os doentes sofrem de insufici&#234;ncia respirat&#243;ria e podem tornar-se dependentes do ventilador&#46; S&#227;o conhecidas mortes devido ao mau funcionamento de ventiladores mec&#226;nicos mas existem poucos registos forenses de tal situa&#231;&#227;o&#46; Relatamos o caso de uma doente de 69 anos com ELA&#44; traqueostomizada e dependente do ventilador&#44; que foi encontrada morta pelo seu marido com o ventilador em modo de espera &#40;&#171;stand-by&#187;&#41;&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">M&#233;todo</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Foi realizada uma aut&#243;psia forense&#46; Foram recolhidas amostras dos &#243;rg&#227;os internos para exame toxicol&#243;gico e histol&#243;gico&#46; A mem&#243;ria interna do ventilador foi tamb&#233;m analisada e testada de modo a descobrir uma poss&#237;vel avaria&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">O exame macrosc&#243;pico n&#227;o revelou qualquer sinal de trauma mas indicou congest&#227;o cerebral e pulmonar&#46; O exame pulmonar histol&#243;gico revelou o espessamento do espa&#231;o intersticial peribr&#244;nquico&#44; sobredistens&#227;o alveolar&#44; quebra de paredes interalveolares e hemorragias alveolares difusas&#46; Foram detetadas micro-hemorragias focais noutros &#243;rg&#227;os&#46; A an&#225;lise da mem&#243;ria interna do ventilador mostrou que&#44; durante a noite da morte&#44; houve diversas quedas de tens&#227;o&#46; Testes espec&#237;ficos revelaram o mau funcionamento da bateria interna que n&#227;o conseguiu fornecer a tens&#227;o necess&#225;ria&#44; consequentemente o ventilador desligou-se&#44; parando a ventila&#231;&#227;o&#46; O mau funcionamento da bateria reduziu o volume do alarme do ventilador&#44; que n&#227;o foi ouvido pelo prestador de cuidados&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclus&#227;o</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">O padr&#227;o histol&#243;gico&#44; com enfisema pulmonar agudo e hemorragias focais poliviscerais&#44; &#233; fortemente indicador de morte devido a asfixia &#171;aguda&#187;&#46; Os autores discutem tamb&#233;m a necessidade de uma supervis&#227;o rigorosa e seguimento destes doentes dependentes do ventilador e dos seus equipamentos&#46;</p></span>"
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