Obstructive sleep apnea therapy.

In clinical practice, oral appliances are used primarily for obstructive sleep apnea patients who do not respond to continuous positive airway pressure (CPAP) therapy. We hypothesized that an oral appliance is not inferior to CPAP in treating obstructive sleep apnea effectively. We randomly assigned 103 individuals to oral-appliance or CPAP therapy. Polysomnography after 8-12 weeks indicated that treatment was effective for 39 of 51 persons using the oral appliance (76.5%) and for 43 of 52 persons using CPAP (82.7%). For the difference in effectiveness, a 95% two-sided confidence interval was calculated. Non-inferiority of oral-appliance therapy was considered to be established when the lower boundary of this interval exceeded -25%. The lower boundary of the confidence interval was -21.7%, indicating that oral-appliance therapy was not inferior to CPAP for effective treatment of obstructive sleep apnea. However, subgroup analysis revealed that oral-appliance therapy was less effective in individuals with severe disease (apnea-hypopnea index > 30). Since these people could be at particular cardiovascular risk, primary oral-appliance therapy appears to be supported only for those with non-severe apnea.

[18 years experience with mechanical ventilation in patients with Duchenne muscular dystrophy]. / 18 jaar ervaring met chronische beademing bij patiënten met spierdystrofie van Duchenne.

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%.

[From the poliomyelitis epidemic to the founding of artificial respiration centres, intensive care units and centres for home mechanical ventilation]. / Van de poliomyelitisepidemie naar de oprichting van beademingscentra, intensivecareafdelingen en centra voor thuisbeademing.

In 1952, Copenhagen was confronted with a poliomyelitis epidemic that involved the respiratory musculature in large numbers of patients. The anaesthetist B. Ibsen, who established carbon dioxide intoxication due to severe hypoventilation as the cause of death, proposed that the patients be treated by tracheostomy and positive pressure respiration in order to achieve better ventilation than with an iron lung. In the Netherlands, it was decided to organise the control ofthe epidemics on a nationwide basis. Various hospitals were asked to set up artificial respiration centres. In addition, the Beatrix Fund was set up in order to collect money for combating poliomyelitis. The epidemic reached the Netherlands in 1956. In Groningen University Medical Centre, 74 patients were admitted, of whom 36 had to be ventilated. In two cases, the mechanical ventilation could not be stopped and one of these was ultimately discharged home with chronic ventilation in 1960, thus becoming the first patient in the Netherlands to be given mechanical ventilation at home. The mechanical ventilation centres developed into the intensive care units as we know them today. Most of the forms of treatment now in use are based on the techniques thought up and elaborated by the pioneers working in the mechanical ventilation centres. The latest development in this series is the development of centres for home mechanical ventilation.

Pneumatosis intestinalis and active cytomegaloviral infection after lung transplantation. Groningen Lung Transplant Group.

Pneumatosis intestinalis occurred in a patient with a primary cytomegaloviral (CMV) infection with pneumonitis 6 weeks after single lung transplantation for primary pulmonary hypertension. The possible causal relationship between pneumatosis intestinalis, an uncommon disorder with an obscure pathogenesis, and active CMV infection has been observed before; however, to our knowledge, this is the first report of this combination after lung transplantation. The patient had no abdominal complaints, and after treatment of the CMV infection, the pneumatosis intestinalis resolved spontaneously. The early diagnosis of active CMV infection and the prevention of unnecessary abdominal surgery were essential in this case.

Acid maltase deficiency: treatment of respiratory insufficiency with cuirass respirator.

Three patients with the late-onset form of acid maltase deficiency showed a gradual weakening of proximal limb and trunk muscles leading to severe respiratory insufficiency. Considerable deterioration of pulmonary function occurred owing to a vicious cycle of hypoventilation and exhaustion. Treatment by nocturnal cuirass ventilation with tailor-made shells was successful. The muscle weakness still progressed very slowly but there were no more respiratory problems.

[Risk of respiratory insufficiency caused by thoracic rigidity]. / Gevaar voor respiratoire insufficiëntie door starre thorax.

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.

[Treatment of the obstructive sleep-apnea syndrome in adults]. / Behandeling van het obstructieve slaapapneusyndroom bij volwassenen.

When treating the obstructive sleep-apnoea syndrome (OSAS), conservative management and the correction of treatable stenoses in the upper airway should be considered first. If these measures are neither effective nor applicable, then continuous positive airway pressure (CPAP) is the preferred treatment. Surgical interventions should only be considered after failure of non-surgical treatment modalities. Pharmacological management of OSAS is usually only indicated as a form of supplementary treatment in specific patients. Oral-appliance therapy appears to be of value in the management of OSAS and, in specific groups of patients, seems likely to offer a good alternative to CPAP in the future.

[Orthopnea: not always of cardiac origin]. / Orthopneu: niet altijd cardiaal bepaald.

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.

[Dyspnea caused by bullae in a muscular dystrophy patient on chronic intermittent ventilatory support]. / Dyspneu door bullae bij een chronisch intermitterend beademde patiënt met spierdystrofie.

A 65-year-old patient, ex-smoker, with facioscapulohumeral muscular dystrophy (FSHD) had been on home non invasive ventilatory support for three years when he experienced gradual increase of dyspnoea. The chest radiograph showed large bullae occupying most of the right hemithorax, with compression of lung tissue, mediastinal shift, and compression of the left lower lobe. Bullectomy resulted in rapid clinical and radiographic improvement. This is the first report of beneficial effects of emergency bullectomy in FSHD. Bullectomy has proved most successful in patients with localized bullae and compression of surrounding lung tissue. Patients with respiratory infections and bronchiectasis benefit less.

Amyloid tumour resected by laser therapy.

We report a patient presenting with dyspnoea, cough and fever with a middle lobe atelectasis. Amyloid deposits in the bronchial wall caused almost complete obstruction of the middle lobe bronchus. The patient was treated with neodymium yttrium aluminium garnet (NdYAG) laser photoresection resulting in complete clearance of the middle lobe bronchus. Laser therapy has to be considered as first-line therapy for patients with endobronchial amyloidosis.

No response improvement after sequential chemotherapy for small cell lung cancer.

In a phase II study in patients with small cell lung cancer (SCLC) the combination of cyclophosphamide, cisplatinum and etoposide was found to be active, the response rate was 91% (30% CR, 61% PR) in the whole group. In 40 limited disease patients 19 CR (48%) and 20 PR (50%) were seen, whereas in 30 extensive disease patients only 2 CR (7%) and 23 PR (77%) were reached. Adding a second combination of doxorubicin, vincristine and procarbazine resulted in response improvement in only two patients. Median response duration was 41 weeks in CR patients and 30 in PR patients (p less than 0.01). Median survival was 66 in CR and 45 weeks in PR patients (p less than 0.002). Performance score and disease stage were found to be good prognostic factors. Four patients (6%) are disease-free at 2 1/2 years. The value of sequential chemotherapy for SCLC is probably minimal in view of the lack of response improvement.