[Double aortic arch: clinical aspects, diagnosis and therapy in children and adults]. / Doppelter Aortenbogen. Klinik, Diagnose und Therapie bei Kindern und Erwachsenen.

Double aortic arch is a rare vascular anomaly which usually causes tracheal and esophageal compression in the first few months of life. During the last 30 years, 7 children, 2 to 24 months old, and one 29-year-old woman with double aortic arches have been treatedatour institution. Symptoms, diagnosis and treatment of these patients were evaluated. Dyspnoe, stridor, recurrent pulmonary infections, feeding problems and failure to thrive were the leading symptoms. Despite typical symptoms from early childhood, the diagnosis was missed in our adult patient. Typical compression of the esophageus and the trachea was visualized by esophagography by 7 and bronchoscopy/-graphy by 6 patients. Angiography was performed in all children, whereas magnetic resonance angiography and computed tomography were done in the adult patient. Resection of the smaller aortic arch, left in 3 and right in 5, through a right or a left posterolateral thoracotomy was uncomplicated and fully resolved the symptoms in all patients. Typical symptoms in early childhood should lead to prompt diagnosis and surgical treatment of double aortic arch. Surgical resection of the smaller aortic arch should also be performed in oligosymptomatic patients to prevent complications later. Preoperative angiography can be replaced by the less invasive magnetic resonance imaging and computed tomography.

[Nasal CPAP therapy in obstructive sleep apnea syndrome: patient compliance]. / Nasale CPAP-Therapie beim obstruktiven Schlafapnoesyndrom: Patienten-Compliance.

We aimed in 186 patients with obstructive sleep apnoea, consecutively treated with nasal CPAP between January 1990 and December 1997, to evaluate compliance with nCPAP therapy and to explore factors influencing compliance. At 3 to 6 months (K1), 1 to 2 years (K2) and more than 3 years (K3) patients were reexamined regarding clinical conditions, the mean time of nCPAP usage per night, nCPAP pressure, and body mass index (BMI). The regularly conducted controls consisted of checking mask fitting, polygraphy with automatic pressure titration and once, mostly at K1, polysomnography. Compliance with nCPAP was considered to be sufficient more than 4 hours of usage per night. At K1, 9 patients had stopped therapy, 5 had moved away, 6 had changed therapy, 6 had died, and 41 had not been treated long enough to have a follow-up at K1. Thus we were able to reexamine 119 patients with ongoing nCPAP therapy. We found no correlation between indices of severeness of sleep apnoea (apnoea/hypopnea index, mean low of night time oxygen saturation, nCPAP pressure, and BMI) and compliance. At K1 87 patients (73%) had sufficient nCPAP compliance. All of them showed sufficient compliance at K2 and K3 too. We conclude that a sufficient compliance at an initial control implies sufficient compliance later on. 32 patients (27%) showed insufficient compliance at K1. Of this group 41% (13 patients) improved compliance at K2. This result underlines the value of a second instruction in nCPAP therapy when patient compliance was lacking initially.

Functional upper airway obstruction and chronic irritation of the larynx.

Wheezing and dyspnoea are typical symptoms of asthma but can also be found in diseases of the extrathoracic airways. Functional upper airway obstruction may imitate, as well as complicate asthma. Functional upper airway obstruction was first described as a conversion disorder in young females with inspiratory stridor. Subsequently, it was found that functional upper airway obstruction was more often a secondary phenomenon in chronic asthma also involving the expiratory laryngeal airflow. During a period of 15 months, we diagnosed six cases of functional upper airway obstruction. Five patients were female and one male, and four were also asthmatics. Three cases showed chronic sinusitis with postnasal drip (PND) and/or gastro-oesophageal reflux. Both disorders may irritate the larynx. Treatment of sinusitis and gastro-oesophageal reflux led to a significant improvement of dyspnoea in all three of these patients. In asthma refractory to treatment and in the case of an asthmatic exacerbation without obvious cause, functional upper airway obstruction should be excluded to avoid unnecessary treatment with systemic steroids. Some of the possible causative factors of functional upper airway obstruction, such as postnasal drip and gastro-oesophageal reflux, are easily treatable.

[Rehabilitation of cystic fibrosis in adulthood]. / Rehabilitation bei zystischer Fibrose im Erwachsenenalter.

By the example of 2 adult patients with cystic fibrosis (CF) problems of pulmonary manifestation of CF and its complications are discussed. In both patients disease manifested late. Life expectancy of CF-patients has markedly increased over the last decades. In the adult distinct complications appear more often such as exacerbation of bronchitis due to pseudomonas aeruginosa, colonisation of air ways by Burkholderia cepacia or by atypical mycobacteria, development of allergic bronchopulmonary aspergillosis, bronchiectases and spontaneous pneumothorax. Today, even in adults with chronic bronchitis of unknown origin the possibility of CF has to be considered. Optimized treatment under clinical conditions, in combination with a physical training program and a diet rich in calories will ameliorate subjective physical performance as well as objective pulmonary function and work capacity. Regular rehabilitation programs contribute to better compliance. In patients with CF direct transition from pediatric to pneumologic/internistic treatment for adults is mandatory in order to optimize expectancy and quality of life.

[Peak-flow monitoring in asthmatic patients: indications and therapeutic implications of the measurements with reference to the 'traffic-light system']. / Peak-flow-Kontrollen bei Asthmatikern: Indikationen und therapeutische Implikationen der Messung unter Berücksichtigung des Ampelschemas.

Peak flow meters do not allow to perform lung function analysis in the private practice. However, they can be used for the diagnosis of reversible airway obstruction, to search for exacerbation factors in asthma, and to monitor drug therapy in chronic asthma. Peak flow measurements provide the means for self-assessment and self-management of asthma by the patients themselves allowing treatment to be optimized and guaranteeing a better quality of life. The plotting of daily pre- and post-bronchodilator peak flow values enables the patient to understand the pathophysiologic changes in his respiratory tract. Therapeutic implications are easily recognized and can be performed without delay by the patient himself on the basis of the so-called "traffic light system".

[Dangerous complication of transtracheal oxygen therapy with the SCOOP(R) system]. / Gefährliche Komplikation der transtrachealen Sauerstofftherapie mit dem SCOOP(R)-System.

A portable oxygen system in combination with transtracheal O2 delivery (SCOOP(R)) permits patients with respiratory failure optimal mobility and facilitates longterm oxygen therapy. This report describes a 70 year old female with COPD that developed acute respiratory distress 18 days after inserting PRESCOOP(R) and 11 days after changing to SCOOP 1 catheter. Catheter stripping had not revealed any pathology. Flexible bronchoscopy showed a mucus ball at the catheter tip leading to a 80% stenosis of the trachea. Trials to remove the ball with forceps and a loop were not successful until a rigid bronchoscop was inserted. Up to 10% of patients develop mucus ball formation with SCOOP 1 catheter which remains in situ for 6 weeks. In patients with high risk of mucus formation (high O2 flow, viscous mucus, low FEV1) the manufacturers of SCOOP recommend catheter stripping. We consider a control bronchoscopy being safer 1 week after changing from PRESCOOP to SCOOP because one patient has been reported to have died of this complication and our patient has developed a near fatal situation.

Short-course therapy of pulmonary tuberculosis: doctor's compliance.

OBJECTIVE: The present study, conducted from 1986 to 1991, investigated the accuracy of treatment monitoring performed by practitioners in the out-patient treatment of pulmonary tuberculosis. DESIGN: All patients with smear or culture positive pulmonary tuberculosis, receiving 6-month short-course chemotherapy with Isoniazid (H), Rifampicin (R) and Pyrazinamide (Z), were included. Treatment had been initiated either in our unit, or shortly before, with discharge after an average time of 8 weeks. Practitioners were sent a flow sheet advising how to perform standardized ambulatory controls. RESULTS: Forty-three patients were included in the study, four of whom did not visit their practitioner after discharge. It must be assumed that they did not complete treatment. The average follow-up time of the remaining 39 patients was 159 weeks. In 13 cases the treatment was prolonged without obvious reason. For four patients who had not converted to a negative sputum culture at discharge, negativity was never been proven thereafter. Objective means of assessing patient compliance by H-urine strip testing were undertaken only in three cases. X-ray controls were performed adequately in 44.4% of the cases. By contrast monthly checks of liver enzymes had been done only in 23.1% of the patients. CONCLUSION: We conclude that even a highly standardized protocol of short-course tuberculosis treatment requires a certain level of experience and compliance on the part of the practitioner. We accordingly recommend that every case of pulmonary tuberculosis should be reviewed at least twice by a chest physician.

[Therapy-resistant asthma: causes and therapy]. / Therapierefraktäres Asthma: Ursachen und Therapie.

Despite the introduction of new and potent antiasthmatic drugs, a minority of asthma patients, remains without response to therapy. Some of the patients with asthma refractory to therapy actually do not have asthma at all, but suffer from another syndrome mimicking asthma. Asthma may also exist in combination with a syndrome mimicking asthma (e.g. vocal cord dysfunction). Patients may then get treated with high doses of corticosteroids unnecessarily, because the symptoms due to vocal cord dysfunction are misinterpreted as being asthmatic. Sometimes asthma is complicated by an independent factor (e.g. gastroesophageal reflux), which does not respond to antiasthmatic drugs. In all cases of asthma being unresponsive to therapy, the diagnosis of asthma must be questioned, and other complicating factors need to be excluded. The patient's compliance should be encouraged, and correct handling of modern inhalation devices has to be monitored. Patients with refractory asthma should also undergo flexible bronchoscopy to exclude morphologic changes of the bronchial system and to obtain mucosal biopsies.

[Spontaneous course of allergic bronchopulmonary aspergillosis]. / Spontanverlauf einer allergischen bronchopulmonalen Aspergillose.

This case report illustrates the spontaneous course of allergic bronchopulmonary aspergillosis (ABPA). The x-ray images cover a time-frame of nearly 20 years. All of the main criteria of ABPA are fulfilled. Despite intermittent oral corticosteroid treatment, recurrent eosinophilic infiltrates of the lungs occurred. New strategies in diagnosis and treatment of ABPA are discussed.