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High flow nasal oxygen (HFNO) is proven to be effective in non-COVID-19 hypoxemic respiratory failure. In the beginning of the COVID-19 pandemic, HFNO was quickly introduced into daily clinical practice, although the evidence of its effectiveness in COVID-19 was limited. Randomized controlled trials suggest that HFNO has no effect on survival. However, HFNO may lead to less intubations in comparison with conventional oxygen therapy. The evidence of HFNO use in patients with do-not-intubate orders remains very limited. However, in these patients, improvement in comfort could be an important argument to start treatment with HFNO. Additional research is needed to make an evidence based consideration about the clinical use of HFNO in COVID-19 care.

Conventional oxygen therapy could be delivered via nasal prongs, face mask, venturi mask, or non-rebreather mask. High flow nasal cannula (HFNC) produces a higher flow of FiO2. This higher flow creates a positive pressure on the higher respiratory tract. Collapsed alveoli could be opened due to this positive pressure and CO2 would be eliminated. This improves the oxygenation and reduces the total work of breathing. Continuous positive pressure support (CPAP) realizes a continuous positive expiratory pressure to the respiratory tract, which opens the alveoli and reduces the pre-load by reducing the venous return. Non-invasive ventilation (NIV) improves ventilation and reduces the work of breathing. Initiating use of HFNC, CPAP or NIV can prevent intubation but the response should be carefully evaluated after 1-2 hours to not delay intubation.

COVID-19 patients admitted to the Intensive Care Unit may develop painful range of motion restrictions of the large joints due to heterotopic ossifications. Here we describe two patients who developed restricted and painful passive and active mobility of the hips, shoulders and elbows after mechanical ventilation because of respiratory failure due to COVID-19 pneumonia. Conventional radiography showed extensive heterotopic ossifications. Retrospectively, alkaline phosphatase levels were elevated. It is likely that local and systemic factors contribute to the development of heterotopic ossifications. Early diagnosis is important to provide complementary non-pharmacological interventions (gentle passive mobilization) and medication (non-steroidal anti-inflammatory drugs, such as indomethacin). If pain and limited joint mobility remain present, surgical removal of ectopic bone could be considered. Future trials are needed to systematically map the prevalence of heterotopic ossifications in COVID-19 patients who were admitted to the Intensive Care Unit, andto evaluate whether prophylactic treatment with non-steroidal anti-inflammatory drug is of relevance.

Hospitalization for corona virus disease 2019 (COVID-19) may be followed by complications after discharge. We aimed to evaluate mortality, readmission rate, and readmission characteristics after hospitalization with COVID-19.A retrospective cohort study METHODS: Inclusion of all patients hospitalized for COVID-19 between March 1, 2020, and June 1, 2020 in Zuyderland Medical Centre, The Netherlands. Main outcome measures were mortality and readmission after hospitalization. Univariate and multivariate regression analysis were performed to identify risk factors for death and readmission.A total of 769 patients hospitalized with COVID-19 (mean age 70 ± 14 years; 39% female) were included in the study. In-hospital mortality was 22.4% , as such 596 patients were discharged alive and followed after discharge with a median of 80 days (IQR 66-91). Total mortality after discharge was 6.4% (n=38) and readmission rate was 11.7% (n=70). Main reasons for readmission were respiratory insufficiency (31%), arterial and venous thrombotic events (16%) or related to a chronic comorbidity (14%). Mortality rates were higher in older patients and patients who experienced delirium during hospital stay. Risk factors for readmission were male sex, discharge to a long-term care facility and COPD.1 out of 6 COVID-19 positive patients died or was readmitted after discharge. This shows an ongoing vulnerability of COVID-19 patients. Physicians and policy makers should consider this high rate when making decisions on discharge, hospital-capacity planning, and patient monitoring after discharge.

COVID-19 is a treacherous disease, in which infected patients who appear to fare well can deteriorate rapidly, mostly due to respiratory failure. For general practitioners (and other first-line responders), a clinical evaluation at any given time merely provides a snapshot of the patient's condition. Therefore, frequent monitoring is warranted in at-risk patients. However, there is no one-size-fits-all approach for monitoring, treatment and referral decisions. This is particularly the case in patients with advanced age. In this article, through the use of case examples, we aim to provide guidance when facing difficult management decisions in patients with (suspected) COVID-19.

In the Netherlands, an increasing number of patients are dependent on domiciliary ventilation. General practitioners and elderly care physicians caring for these patients are increasingly confronted with problems related to chronic ventilation. Most patients die due to progression of respiratory failure, however, patients may ask their physician to electively withdraw their assisted ventilation. According to the Dutch Medical Treatment Contracts Act, withdrawal of domiciliary ventilation at the request of a patient constitutes normal medical care and concerns neither the assessment of, nor the compliance with, a request for euthanasia. Currently, there is no Dutch guidance or guideline containing practical advice regarding the medical, ethical, organisational and supportive aspects of withdrawal of domiciliary ventilation. This paper addresses the planning necessary for the patient and between treating professionals, for the organisation and implementation of withdrawal of domiciliary ventilation at the patient's home, a nursing home or hospice.

The antisynthetase syndrome (AS) is a rare auto-immune disease characterised by inflammatory myopathies, interstitial lung disease, inflammatory arthritis, Mechanic's Hands and Raynaud phenomenon. AS infrequently presents with life-threatening lung disease as its primary or sole manifestation. By means of two clinical case reports, an overview is given of recent advances in diagnosis and treatment of AS-related interstitial lung disease. We emphasise the importance of considering AS as a differential diagnosis if lung disease does not respond to standard treatment in a relatively young patient, certainly if there is even a mild suggestion of myopathy, arthritis or cutaneous involvement.

Patients with decreased exercise tolerance and orthopnoea are often referred to an internist, a cardiologist or a pulmonologist. These symptoms can also be caused by weakness of the respiratory muscles, as an indication of a neuromuscular disease. If these symptoms are not recognized as such, this may result in a delay in timely diagnosis.An 82-year-old man had suffered from decreased exercise tolerance for 18 months. For the last months he had been sleeping upright and had lost 20 kg in weight. Analyses by the cardiologist and the internist had not led to a definitive diagnosis. He was finally brought to the emergency department with loss of consciousness and hypercapnic respiratory insufficiency. Neurological examination was suggestive of motor neuron disease such as progressive spinal muscular atrophy or amyotrophic lateral sclerosis. The patient died within 24 hours of admission.Patients with symptoms resulting from respiratory muscle weakness are commonly referred to non-neurological specialists, leading to a delay in diagnosis and treatment of an underlying neuromuscular disease.

More and more patients with chronic respiratory insufficiency are eligible for home mechanical ventilation. As a result, medical doctors are increasingly confronted with said patients. The treatment goal of this supporting therapy is to improve quality of life and possibly life extension. Centres for home mechanical ventilation decide on the indication, whereas the clinical setup of the ventilator takes place in a hospital setting. Staff in the Dutch Gelderse Vallei hospital have been initiating - both non-invasive and invasive - home mechanical ventilation for various patient groups for the last 4 years. This is a labour-intensive process during which numerous issues can occur. Experience and expertise is necessary for a successful process. A multidisciplinary approach and accessible consultation between stakeholders is of great importance when setting up home mechanical ventilation for these patients.

5-10% of all oncological patients will be admitted to ICU at some point.- Medical oncological patients are usually admitted due to respiratory insufficiency or sepsis.- Mortality among medical-oncological ICU patients has decreased to an average of 40%, with a wide spread per tumour type.- The main prognostic factors for ICU survival are: clinical condition, severity of the acute condition, tumour type, and some specific oncological conditions.- This article describes directive ICU admission criteria, subdivided into three categories of medical oncological patients. Although not validated, these criteria give guidance to clinicians in deciding whether ICU admission is a responsible course of action.- In patients with an uncertain prognosis an ICU trial can be useful.- ICU admission criteria are largely based on expert opinion, as sufficient scientific evidence is lacking. A multidisciplinary decision process is therefore important.- Ideally, decision-making about ICU treatment should take place at an early stage in primary health care or outpatient clinic; this is called Advance Care Planning.

Barbiturate intoxication is potentially lethal. With the availability of the newer anticonvulsants the use of barbiturates in treating epilepsy has decreased significantly, with a concurrent decrease in the incidence of overdose with these medications. There have, however, been recent alarm signals from governmental sources concerning the increase in the Internet purchase of illegal medications, including barbiturates, for use in attempted suicide.Here we describe two patient cases involving barbiturate intoxication with amobarbital and thiopental, respectively. They had both obtained the barbiturates via the Internet. Both patients were comatose and showed signs of respiratory depression; one of them was also haemodynamically unstable. Both patients recovered fully following intensive supportive therapy.In patients with coma, respiratory depression, absence of brainstem reflexes and shock with no evident cause one should be aware of the possibility of barbiturate intoxication, even when there is no indication that these have been prescribed or that the patient has direct or indirect access to barbiturates. Prompt, optimal supportive therapy will give a good chance of full somatic recovery.

Acute intensive care (IC) triage involves a challenging decision-making process. Physicians are required to make life or death decisions about an unfamiliar patient within a short time frame. An 84-year-old female was admitted to the stroke unit following an extensive cerebral infarction. The intensive care unit (ICU) physician was consulted because of a suspected severe abdominal sepsis even though ICU treatment had never previously been discussed. A 77-year-old female with a previous history of myocardial infarction and severe COPD developed acute respiratory failure on the ward, and was admitted to the ICU for support by a mechanical ventilator. The family felt this was an inappropriate course of treatment, considering her former poor quality of life. When physicians are confronted with sudden deterioration of the patient's clinical condition without advanced care planning a limited-time IC treatment trial is often initiated, possibly leading to inappropriate ICU admissions. ICU treatment options should preferably be discussed beforehand; preliminary background information regarding the patient's wishes is essential for adequate decision-making.

Baclofen is increasingly prescribed for alcohol dependency. Subsequently, the risk of self-intoxication with this medicinal product is increasing.A 23-year-old man with a history of alcohol dependence was admitted to our hospital after self-intoxication with 2700 mg baclofen and 330 mg mirtazapine. Respiratory insufficiency as a result of the baclofen intoxication required intubation and admission to the ICU. During the first day, despite the use of sedatives, the patient became intermittently agitated and aggressive. In the following days, he developed severe delirium, probably due to baclofen withdrawal. The reintroduction of baclofen quickly resolved these symptoms.In the case of baclofen, in practice it is difficult to differentiate between intoxication and withdrawal. To prevent potentially severe withdrawal symptoms, we recommend reintroduction of baclofen when the first signs of restlessness and agitation arise following intoxication.

Three patients with myotonic dystrophy (MD) developed respiratory failure. The first was a 55-year-old man with MD who had been admitted elsewhere with pneumonia and respiratory failure. After discharge he was re-admitted with respiratory failure due to respiratory pump failure. Ultimately, he was given a tracheostomy and was ventilated during the night. The second patient was a 38-year-old man who was admitted to the intensive care unit when he could not be weaned off the ventilator following cholecystectomy for symptomatic gall stones. It appeared that he had partial respiratory failure due to MD and overweight. Ultimately he was able to be weaned during the day with nightly non-invasive ventilation. The third patient was a 55-year-old woman who was admitted with recurrent pneumonia and respiratory failure. She appeared to have chronic respiratory failure due to MD. She was ventilated at night via a tracheostomy. All three patients reported a gradual decline in physical and mental performance in the years preceding this acute episode. All three experienced a remarkable recovery of performance after the institution of mechanical ventilatory support. Respiratory failure that develops over a number of years can be easily missed in aging patients with slowly progressing neuromuscular disease. Timely recognition may lead to improved survival and quality of life by the application of non-invasive ventilatory support.

OBJECTIVE: To find out which patients with Duchenne muscular dystrophy are eligible for starting home mechanical ventilation and what the survival rate is.DESIGN: Retrospective.METHOD: In 48 patients with Duchenne muscular dystrophy who were treated with home ventilation from 1987, the results were assessed in the follow-up visit in February 2005. Initially, ventilation was only given through a tracheotomy (TPPV), but after starting up a multidisciplinary neuromuscular consultation, non-invasive ventilation (NIPPV) was offered in an earlier stage of the disease. The following data were derived from the outpatient medical record: indication for ventilation, vital capacity (VC), arterial blood gas values, duration of ventilation up to February 2005, survival and causes of death.RESULTS: 15 patients died. The 5-year survival rate was 75% from the start of mechanical ventilation and 67% (18/27) of the patients were still living at home at the time of the follow-up visit. The most important causes of death were cardiomyopathy (5/15) and tracheal bleeding (3/15). The group of patients who started ventilation before 1995 (n = 17) had a significantly smaller VC than the group (n = 31) who started after the neuromuscular consultation was set up. The PaCO2 during daytime was significantly higher in the group that started ventilation before 1995 compared to the group that started later.CONCLUSION: Home mechanical ventilation can be implemented effectively in patients with Duchenne dystrophy, with a 5-year survival of 75%.

Rib fractures resulting from blunt thoracic trauma occur frequently in the elderly. Even though these are usually due to a low impact trauma mechanism (e.g. a fall from standing height), rib fractures are associated with significant morbidity and mortality in the elderly patient. This unfavourable clinical course is most often caused by respiratory insufficiency due to lung oedema or pneumonia. This cases series presents three elderly patients who sustained multiple rib fractures and were subsequently admitted to a level I trauma centre for treatment and discusses diagnostic challenges and different treatment approaches for this population. More specifically, age-specific indications and contra-indications for intravenous or epidural analgesia and operative rib fixation are discussed. In addition, we show that a multidisciplinary approach, involving a trauma surgeon, anaesthetist, intensive care physician and physiotherapist, is paramount for the prevention of potentially lethal complications.

The decision to move to a form of mechanical ventilation in patients with acute respiratory failure due to an acute exacerbation of COPD is influenced by expectations about survival and quality of life after discharge from the ICU. Physicians tend to be too pessimistic about the survival outcome of an ICU stay with invasive mechanical ventilation. The forced expiratory volume in 1 second (FEV1) is not an adequate prognostic parameter. In order to prevent undertreatment of patients with respiratory failure due to an exacerbation of COPD, knowledge of prognostic parameters and quality of life in these patients is very important. End of life care should be integrated into the standard care of COPD patients.

In three mechanically ventilated patients ventilatory and circulatory complications resulted from high levels of intrinsic positive end-expiratory pressure (PEEPi): progressive pulmonary hyperinflation due to impairment of the expiration. PEEPi was initially not considered as the cause of shock and low tidal volumes and/or high inflation pressures. In a 74-year-old man the circulation deteriorated further when hand bagging was started in an attempt to improve his ventilatory condition; after reduction of the respiration rate, he recovered well. In a 40-year-old woman with relapsing polychondritis sedation helped to reduce the respiratory rate so as to restore sufficient expiratory time. A 59-year-old woman developed acute exacerbation of severe chronic obstructive pulmonary disease, and went into shock during interhospital ambulance transport; she was stabilized after recognition of PEEPi and adjustment of the setting of the ventilator. Detection of PEEPi (e.g. by the finding of a deep inflation level on physical examination) is more important than exact measurement of PEEPi. If PEEPi is detected, the ventilator should be set at PEEP at 80-90% of PEEPi, low frequency (e.g. 8/min) and a long expiratory time, and high inspiratory flow.

A 20-year-old woman presented with abdominal pain, purpura on the extremities, and arthralgia. The diagnosis of Henoch-Schönlein purpura was made based on granular IgA deposits in the vessels in a skin biopsy. Three weeks after onset of these symptoms, she developed glomerulonephritis and diffuse alveolar haemorrhage; she developed respiratory failure and needed mechanical ventilation. She recovered upon treatment with corticosteroids. Diffuse alveolar bleeding has occasionally been reported in adults with Henoch-Schönlein purpura; this is the first report from the Netherlands.

Respiratory insufficiency developed in a man aged 68 after cardiac surgery and in a man aged 60 with COPD and a history of cigarette smoking after an attack of 'flu', while in a woman aged 70 with non insulin-dependent diabetes mellitus it had been present for years. All three had bilateral diaphragmatic paralysis. The diagnosis is based on the triad orthopnoea, paradoxical abdominal movements during respiration in the recumbent position and a decrease of the vital capacity in the horizontal as compared with the sitting position. The patients' physical condition could be improved with the aid of (noninvasive) ventilatory support.

In two women aged 65 and 49 years and a man aged 64 years, severe respiratory failure developed and a pulmonary disease was suspected. They also had a minor systolic murmur. At further investigation no pulmonary cause for the disease could be established. Pulmonary artery catheterization revealed increased pulmonary artery and wedge pressures and transthoracic and transoesophageal echocardiography revealed mitral valve insufficiency. Two patients had valve surgery, the third received medication. Respiratory failure is a common problem in an intensive care unit. If this condition is caused by mitral valve insufficiency the clinical picture is not always that of acute left ventricular failure with hydrostatic pulmonary oedema due to backward failure. In patients with respiratory failure due to mitral valve pathology initial diagnostic problems may be overcome by combining the findings of pulmonary artery catheterization and transthoracic and transoesophageal echocardiography.

Three patients, a man aged 71 and two women aged 47 and 54, were admitted for chronic obstructive pulmonary disease and cardiac failure. All three had thoracic deformities, owing to earlier pneumonectomy with thoracoplasty because of pulmonary tuberculosis, congenital kyphoscoliosis, and infant poliomyelitis respectively. Such patients are at risk of developing chronic respiratory insufficiency because of chronic alveolar hypoventilation: muscle power decreasing with age gradually fails to meet the increased respiratory labour. Often, the respiratory insufficiency is not noticed because the problems are ascribed to secondary chronic obstructive pulmonary disease or cardiac failure. The first sign of imminent respiratory insufficiency is nocturnal carbon dioxide accumulation. Therapy consists of respiratory assistance at night by positive air pressure ventilation via a nose mask.

An 8-month-old boy with a medical history of prematurity and intubation presented with a severe inspiratory wheeze, caused by subglottic cysts.

Currently, more than 2200 patients in the Netherlands receive chronic ventilatory support. In the majority of patients this leads to increased survival without any complications. Nevertheless, in case of ventilatory support via a mask, problems such as skin irritation, leakage and claustrophobia can occur. In case of tracheostomy, it can lead to increased pulmonary secretion. Diaphragm pacing with an external pacemaker might be an attractive alternative to prevent these symptoms as it can replace ventilatory support by mask or tracheostomy. Current indications are patients with spinal cord injury or with congenital central hypoventilation syndrome who are chronically respiratory insufficient. In our experience, patients can be completely or partially weaned from mechanical ventilation when using the diaphragm pacer. In the Netherlands, the technique is only performed at the University Medical Center Groningen.

Healthcare professionals are seeing an increasing number of morbidly obese patients. Medical interventions are often difficult to perform in these patients. In acute situations this can lead to major problems.Assistance from a mobile medical team was requested for a 42-year-old male weighing 350 kg with severe respiratory failure. Transporting the patient was problematic due to his build. The hospital to which he was brought after hours of delay lacked the appropriate space and resources for morbidly obese patients. Since the condition of the patient deteriorated, intubation and mechanical ventilation were required. Despite additional equipments for problematic airway access, the procedure failed and the patient died due to respiratory failure.Designating centres for care, also in the acute situations, of morbidly obese patients is recommended, to improve the care of these patients and to prevent disasters.

Early recognition of Guillain-Barré syndrome (GBS) is crucial to anticipate and adequately respond to possible respiratory insufficiency. Young children with GBS frequently have non-specific complaints and are more difficult to examine, which may cause a significant delay in diagnosing GBS. We present 3 children, all boys, aged 22 months, 7 years and 4 years respectively, with GBS in whom the diagnosis was missed upon admission, resulting in a failure to appreciate the risk of acute respiratory insufficiency. Two children had to undergo emergency intubation, and one of these cases had a fatal outcome. Young children with GBS often present with pain and refusal to walk, or with difficulty swallowing, and are often initially misdiagnosed with e.g. tonsillitis or coxitis. These nonspecific symptoms can be a first sign of a progressive polyradiculoneuropathy and should prompt a full neurological examination and timely referral to a paediatric neurological centre with Intensive Care facilities.

Lung volume recruitment can improve peak cough flows and respiratory compliance in patients who either do or do not require mechanical ventilation. There are several lung volume recruitment techniques: air stacking, glossopharyngeal breathing and mechanical insufflation-exsufflation with cough assist devices. The principle of lung volume recruitment is based on the insufflation of air in the lungs after maximal inspiration. In air stacking, a manual resuscitation bag is used for insufflation. Glossopharyngeal breathing requires the use of oropharyngeal and laryngeal muscles by the patient. The mechanical cough assist device exsufflates the air after insufflation. These techniques may prevent pulmonary complications, hospital admission and tracheotomy in patients with a reduced ability to cough, a proclivity towards atelectasis and recurrent airway infections. The combination of long-term mechanical ventilation with lung volume recruitment has led to further improvement in the prognosis of chronic respiratory failure. More patients may potentially benefit from lung volume recruitment than only those being converted from short-term to long-term mechanical ventilation.

To assess the effect of a local guideline advising elective caesarean section without maternal comorbidity at a gestational age ofor= 39+0 weeks.Retrospective cohort study.Children born by elective caesarean section in the period 2003-2007 at the VUmc with a gestational ageor= 37+0 weeks and without maternal comorbidity were included. Respiratory complications, length of hospital stay, admission to the neonatal intensive care unit (NICU), respiratory support and medication were recorded from charts of admitted children. These data were compared with data collected in 1994-1998 before implementation of the local guideline.In 2003-2007, 501 children were born from 486 elective caesarean sections. In 1994-1998, 333 children were born from 324 elective caesarean sections. In 2003-2007, mean maternal age was younger, local anaesthesia more frequent, elective caesarean section was more often performed ator= 39+0 weeks (p0.001) and the birth weight was higher. In 2003-2007, fewer infants were admitted to an NICU than in 1994-1998 (6/501 versus 17/333, p0.001), of whom fewer infants were born with gestational ageor= 39+0 weeks (50% versus 94%, p = 0.04). In 2003-2007, respiratory morbidity was lower in the group with gestational age 38+0/7-38+6 weeks than in 1994-1998. This is explained by the higher number of elective caesarean sections performed at gestational age of 38+4/7-38+6 weeks in 2003-2007 (63%) compared with 1994-1998 (28%; p0.001). After correction for confounders, the odds ratio for developing respiratory morbidity in 2003-2007 was 0.27 compared with 1994-1998 (95% CI: 0.10-0.70).Implementation of the guideline reduced elective caesarean sections before a gestational age of 39+0 weeks and consequently reduced neonatal respiratory morbidity.

Two neonates were born via elective caesarean section, after 38 2/7 and 38 0/7 weeks of gestation, respectively. They developed serious respiratory complications: a pneumothorax and respiratory insufficiency, respectively, for which they were transferred to a neonatal intensive care unit and were mechanically ventilated. If a caesarean section is performed before 39 0/7 weeks of gestation, the risk of respiratory complications, such as idiopathic respiratory distress syndrome or wet lung disease, is increased. Despite this scientific evidence, elective caesarean sections continue to be planned before 39 weeks of gestation.

The classic clinical presentation of anti-GBM disease, or Goodpasture's disease, is pulmonary haemorrhage in combination with rapidly progressive glomerulonephritis. However, presenting symptoms vary considerably. To illustrate this, this article describes 3 male patients with anti-GBM disease, aged 25, 27 and 25 years respectively. The first patient presented with massive haemoptysis without glomerulonephritis, the second patient presented with a microcytic anaemia, caused by subclinical alveolar bleeding, and the third patient presented with rapidly progressive glomerulonephritis in combination with respiratory failure. All 3 patients required mechanical ventilation but did survive. Two patients progressed to end-stage renal disease (ESRD). Earlier recognition might prevent respiratory failure and ESRD. Therefore, despite heterogeneous clinical presentation, immediate recognition of anti-GBM disease is vitally important in view of the potentially lethal consequences if left untreated.

Three patients with myotonic dystrophy (MD) developed respiratory failure. The first was a 55-year-old man with MD who had been admitted elsewhere with pneumonia and respiratory failure. After discharge he was re-admitted with respiratory failure due to respiratory pump failure. Ultimately, he was given a tracheostomy and was ventilated during the night. The second patient was a 38-year-old man who was admitted to the intensive care unit when he could not be weaned off the ventilator following cholecystectomy for symptomatic gall stones. It appeared that he had partial respiratory failure due to MD and overweight. Ultimately he was able to be weaned during the day with nightly non-invasive ventilation. The third patient was a 55-year-old woman who was admitted with recurrent pneumonia and respiratory failure. She appeared to have chronic respiratory failure due to MD. She was ventilated at night via a tracheostomy. All three patients reported a gradual decline in physical and mental performance in the years preceding this acute episode. All three experienced a remarkable recovery of performance after the institution of mechanical ventilatory support. Respiratory failure that develops over a number of years can be easily missed in aging patients with slowly progressing neuromuscular disease. Timely recognition may lead to improved survival and quality of life by the application of non-invasive ventilatory support.

While developments in molecular biology have been giving rise to breakthroughs in diagnosis, management and promising treatment approaches for neuromuscular disorders, there has been a silent revolution in ventilatory support of patients with a range of neuromuscular disorders with respiratory insufficiency. In a Dutch retrospective study on ventilatory support in 48 boys with Duchenne muscular dystrophy, a 5-year survival rate of 75% was found, with 67% of the patients still living with their relatives. These data are in line with reports from Great Britain. The significant impact of ventilatory support on the natural history of various neuromuscular disorders with respiratory insufficiency is a major breakthrough.

To find out which patients with Duchenne muscular dystrophy are eligible for starting home mechanical ventilation and what the survival rate is.Retrospective.In 48 patients with Duchenne muscular dystrophy who were treated with home ventilation from 1987, the results were assessed in the follow-up visit in February 2005. Initially, ventilation was only given through a tracheotomy (TPPV), but after starting up a multidisciplinary neuromuscular consultation, non-invasive ventilation (NIPPV) was offered in an earlier stage of the disease. The following data were derived from the outpatient medical record: indication for ventilation, vital capacity (VC), arterial blood gas values, duration of ventilation up to February 2005, survival and causes of death.15 patients died. The 5-year survival rate was 75% from the start of mechanical ventilation and 67% (18/27) of the patients were still living at home at the time of the follow-up visit. The most important causes of death were cardiomyopathy (5/15) and tracheal bleeding (3/15). The group of patients who started ventilation before 1995 (n = 17) had a significantly smaller VC than the group (n = 31) who started after the neuromuscular consultation was set up. The PaCO2 during daytime was significantly higher in the group that started ventilation before 1995 compared to the group that started later.Home mechanical ventilation can be implemented effectively in patients with Duchenne dystrophy, with a 5-year survival of 75%.

A preterm neonate, with a gestational age of 30 1/7 weeks, was born after a period of prolonged rupture of the membranes and a retroplacental haematoma causing vaginal bleeding. During admission to the neonatal intensive-care unit, mechanical ventilation was indicated because of acute respiratory failure following blood aspiration, which was causing oxygenation and ventilation problems. Endotracheal surfactant was administered and, because of persistent pulmonary hypertension of the newborn (PPHN), NO-inhalation therapy was started. A quick recovery was seen and two days post partum the patient could be extubated. Blood aspiration may cause acute respiratory problems and PPHN, with quick recovery after effective mechanical ventilation, surfactant and NO-inhalation therapy.

Two girls developed symptoms of wheezing which started shortly after birth. The symptoms did not respond to bronchodilators. At the age of 5 months, the first infant developed severe respiratory distress with decreased left-sided breathing sounds on auscultation. The chest X-ray showed left-sided hyperinflation. Bronchoscopy revealed isolated malacia of the left main stem bronchus. The second patient, who had a history ofcor vitium, was referred to a paediatric pulmonologist in an academic hospital for chronic coughing and wheezing. Bronchoscopy and CT angiogram, performed at the age of 14 months, revealed tracheal malacia due to compression from a right descending aortic arch. Broncho- and tracheomalacia are disorders which may rarely result in severe respiratory distress. These disorders should be considered when unexplained symptoms of wheezing or coughing are present in young infants, especially if the symptoms start shortly after birth and persist without signs of viral infection.

A girl and a boy, who both presented with recurrent respiratory infections from birth, were referred to a paediatrician at the age of 2.5 years: they were diagnosed with cystic fibrosis (CF). The girl died from respiratory insufficiency at the age of 6 years and the boy at the age of 13 years from pulmonary aspiration. A further girl and boy who presented with abnormal faeces and failure to thrive were referred to the paediatrician at the ages of 2.5 months with haematomas and 2 weeks with anaemia respectively, as a result of vitamin deficiencies due to malabsorption. They too had CF. The girl had a brain haemorrhage in the meantime that left her with serious impairments. The boy recovered. A delay in diagnosing CF is not uncommon, as the symptoms of CF are hard to differentiate from those of common childhood diseases. However, this diagnostic delay can result in serious organ damage. Current treatment of CF has a predominantly prophylactic character and aims at maintaining normal growth and nutritional status as well as at preventing or postponing chronic bacterial infection of the lower respiratory tract. This treatment is most effective when it is started before any organ damage has occurred: a state that can only be achieved when patients with CF are identified shortly after birth. Therefore, it is important to add CF-screening to the neonatal screening program.

Two women aged 54 and 69 years with high spinal cord injuries and tetraparesis developed hypothermia (30.4 and 29.4 degrees C, respectively); normalization was accomplished by external heating using insulation blankets. Even moderately lowered environmental temperatures may lead to hypothermia in these patients with abnormal temperature sensation, incapacity to compensate and autonomous dysfunction. The symptoms are aspecific and the differential diagnosis is extensive. Recommendations for prevention of hypothermia include daily measurement of the core temperature, adjustment of the environmental temperature and humidity of the air, adequate closing and avoidance of vasodilator drugs.

A 65-year-old man with chronic obstructive pulmonary disease who had frequently been treated with prednisone for recurrent exacerbations was admitted to hospital with a pneumothorax. This proved to be a complication of a chronic necrotizing pulmonary aspergillosis. Despite maximal therapy the patient died 15 months after diagnosis from total respiratory insufficiency, the end stage of the bullous emphysema.

A 17-year-old boy presented with acute respiratory insufficiency based on high airway obstruction due to a tracheal schwannoma.

A 50-year-old woman who received a prescription for hydrochlorothiazide for 'swollen ankles', developed respiratory distress due to acute pulmonary oedema within a few hours after she took the first dose. She was treated with artificial respiration during 3 days and with dopamine and circulatory volume suppletion. She recovered completely. Patient had stopped earlier use of hydrochlorothiazide because of respiratory discomfort. The described adverse effect is rare, but because of the high use of this drug it is important to recognize it.

Amyotrophic lateral sclerosis is a progressive neurological disorder. It is characterised by selective motor-neuron degeneration in the cortex, brainstem, and spinal cord. Consequently, patients suffer from muscle weakness and usually die within 3-5 years after diagnosis from respiratory insufficiency. About 5-10% of the patients have a family history of ALS, the remaining are classified as sporadic ALS. There is only limited information about genetic susceptibility factors in sporadic ALS. Some patients with familial ALS have mutations in the gene encoding for copper/zinc superoxide dismutase, a protein involved in scavenging superoxide radicals. This results in a toxic gain of function. Mutations in the gene coding for alsin, ALS2, have been shown to be responsible for an autosomal recessive form of juvenile ALS.

A 32-year-old man was presented with severe hypothermia and respiratory insufficiency after submersion in water. The ECG showed Osborn waves and a prolonged QRS and QT duration which normalized after correction of the hypothermia.

Two female patients aged 88 and 82 who were being treated for constipation with lactulose, developed life-threatening dilatation of the bowel. Both underwent surgery. One of them was found to have cancer of the bowel; she had an uneventful postoperative recovery. No mechanical abnormalities were found in the second patient but she died due to respiratory insufficiency following aspiration. Lactulose is one of the most frequently prescribed laxatives. In the colon it is metabolized by bacteria into short-chain fatty acids, hydrogen and carbon dioxide. The resulting production of gas in the colon can contribute to a non-toxic megacolon, particularly in patients with delayed intestinal passage.

A 67-year-old male presented with abdominal pain followed by respiratory distress. Imaging revealed a right-sided Bochdalek's hernia. At thoracotomy two days later, an incarcerated small bowel segment was removed, with a protracted post-operative course. In adults, a Bochdalek's hernia is rare and usually asymptomatic. However, when symptoms do occur, incarceration of the intestinal contents is frequent and accompanied by high mortality. The severely protracted course after delayed treatment of a symptomatic Bochdalek's hernia described here illustrates the importance of early recognition and surgical intervention.

Two boys, aged 2 and 11 years, presented with fever and muscle weakness that resulted in respiratory insufficiency. A physical examination and additional tests confirmed the diagnosis 'myasthenia'. Acetyl cholinesterase-inhibitor therapy had a favourable effect. Myasthenia is a diagnosis that should be considered for every child presenting with muscle weakness of unknown origin.

In two patients (men aged 64 and 62 years, respectively) with amyotrophic lateral sclerosis (ALS) tracheostomy ventilation was initiated following (imminent) acute respiratory failure; in one patient this was done because advance directives were lacking, while in the other non-invasive ventilation (NIPPV) was no longer an option. A third ALS patient, a woman aged 36 years, already had chronic respiratory failure when she presented at the local centre for home mechanical ventilation. As a result, the placement of a percutaneous endoscopic gastrostomy (PEG) was impossible. Instead, she had to use a nasopharyngeal feeding tube, which diminished the effectiveness of her NIPPV. The fourth patient, a man aged 60 years, was referred in good time and hence had sufficient time to consider domiciliary ventilation. Following the placement of a PEG he started NIPPV electively. In patients with ALS, domiciliary ventilation should be discussed early in the course of the disease. Advance directives with regard to domiciliary ventilation are important, not only to avoid undesired (invasive) ventilation, but also with respect to the placement of a PEG and the appropriate use of oxygen and morphine.

Chronic lung disease of the neonate sometimes occurs as a residual condition following respiratory distress in preterm infants. Improvements in neonatal intensive care treatment will in future lead to a greater number of children surviving chronic lung disease and reaching adulthood. The symptoms of the disease are hypoxaemia, hypercapnia, tachypnoea, subcostal and intercostal retractions, fluid retention, a reduced exertion tolerance and hyperreactive airways. The treatment after the first weeks of life is symptomatic and consists of: providing supplemental oxygen via a nasal mask or cannula (0.1-1 l/min); rapid downward adjustment of oxygen therapy may lead to more complaints and poorer growth; a normal fluid therapy; if there is a tendency towards fluid retention, then diuretic therapy is indicated and in severe cases fluid restriction as well; in the case of bronchial hyperreactivity: inhaled corticosteroids (the lowest effective dose for a period of several months) and a trial treatment with beta-agonists; in the case of persistent complaints or functional limitations, lung function tests can distinguish obstructive and restrictive disorders; vaccinations according to the national programme; consider vaccinations against influenza (age: 6-12 months) and respiratory syncytial virus (age2 years).

A 38-year-old man presented with respiratory insufficiency due to a pneumothorax on the left side and giant bullae on the right side.