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We present a 28-year-old women with a 3 yr history of duodenal ulcers. Following four treatment attempts to eradicate helicobacter pylori she was admitted because of gastric outlet obstruction and a weight loss of 20 kg within the last two years. Endoscopy and x-ray showed a circular inflammatory stenosis of the proximal duodenum extending over 8 cm. Additionally, chest x-ray showed a circumscript infiltrate in the third segment of the right lung. Mycobacterial infection could be excluded. Ileocolonoscopy and small intestinal follow-through beyond the duodenum were unremarkable, and Zollinger-Ellison-syndrome was ruled out. Bronchopulmonary histology showed intramucosal epithelioid-cell granulomas and bronchiolitis obliterans. Because the patient did not improve under conservative therapy a Billroth-II-resection was carried out. Histologically the resected specimen showed Crohn-like lesions. Postoperatively, severe peripheral arthritis was treated by steroids over 6 weeks. At follow-up the patient regained 20 kg and was free of symptoms without any medication. The pulmonary infiltrate had subsided almost completely. In summary, this extremely rare coincidence of isolated stenosing duodenal Crohn's disease and pulmonary involvement was successfully treated by Billroth-II-resection. This course of disease is compatible with the hypothesis that Crohn's disease may be maintained by antigens derived from ingested food.

Data on lung volumes and changes in flow-volume spirometry at high altitude are few and do not provide comprehensive assessment of the occurring changes. This study characterizes alterations of the forced expiratory flow-volume curve (FEFV-curve) and lung volumes at increasing altitude.FEFV-curve and lung volumes at increasing altitude were characterized by daily assessment of peak expiratory flow (PEF), forced expiratory volume in one second (FEV1), forced vital capacity (FVC) and maximal expiratory flow rates (MEF 25, 50, 75) at 25%, 50% and 75% of the FEFV-curve with a portable spirometer (turbinometric method) three times a day during an expedition to Mustagh Ata (7545m) in 15 healthy mountaineers.With increasing altitude FVC and FEV1 were reduced by up to 25% (74.8% / 74.6% of baseline) and MEF25 was reduced to 81.5% of baseline values. PEF initially increased up to 4451m and returned to baseline values above 5000m. After descent below 2000m, all values normalized within one day. There were weak negative correlations between AMSS and FEV1, FVC and PEF (r = -0.23, p0.001).We found increasing pulmonary restriction at high altitude without a marked reduction of PEF. Assessment of the FEFV-curve at high altitudes with a portable spirometer is a practical method reflecting the true field situation and may provide clinically relevant information (impending pulmonary edema).

Early lung cancer screening failed to reduce lung cancer mortality. New techniques such as autofluorescence bronchoscopy (AF) and the identification of specific genetic alteration might change future outcomes of lung cancer screening. It was the aim of our study to combine p53 analysis with white-light bronchoscopy (WL) or WL and AF to improve the diagnostic yield in a series of 36 patients with histologically proven lung cancer, pulmonary metastasis or suspected lung cancer. - Endobronchial sites were analysed by WL (n = 71), AF (Storz) (n = 34), histopathology (n = 71) and p53 mutations were examined by SSCP analysis on additional biopsies (n = 69). The overall frequency of cancerous lesions was 19, of which 14 were macroscopically visible lesions. The addition of p53 and autofluorescence improved the yield to 17 of 19 cases. In 7 preinvasive lesions (dysplasia/metaplasia) 4 were identified macroscopically and 5 of 7 lesions by all 3 methods. In the WL/p53 group the diagnostic yield was 7 of 9 cancerous lesions compared to 10 of 10 cancerous lesions in the AF group. It should be noted that all methods were associated with false positive results. However, the combination of conventional with autofluorescence bronchoscopy and mutation analysis is a promising approach which is applicable to clinical routine and may be further enhanced by the inclusion of a panel of markers of tumour progression.

In adult patients with cystic fibrosis bone metabolism may be altered by multiple mechanisms, such as abnormal calcium homeostasis, malnutrition, chronic inflammation or inactivity in the course of respiratory failure. In contrast to the high prevalence of osteoporosis in CF patients before lung transplantation, data from different CF collectives show great variation. It was the purpose of our cross-sectional study to determine changes in bone metabolism by measuring bone mineral density, and assessing calcium metabolism and clinical characteristics in adult patients (mean age 32 years) with cystic fibrosis.Bone mineral density (quantitative digital radiography), parameters of calcium homeostasis and clinical characteristics were determined in 34 adult patients with cystic fibrosis.The mean age of the study population was 32 years (range 20 to 47; 21m: 13f). 13 patients had normal T-values (mean bone mineral density in young adults), whereas 11 patients (32 %) had osteopenia and 10 (29 %) had osteoporosis. Calcium homeostasis was abnormal in only one case. In contrast T-values were positively correlated with a low body mass index (p = 0.01) and a low one-second forced expiratory volume (FEV1) (p0.05).Decreased mineral bone density is a frequent complication in adult patients with cystic fibrosis, but does not occur inevitably even in long-standing disease (up to 47 years). In our cohort measurable alterations of calcium homeostasis could be avoided by consistent substitution policy. The main determinants of a low T-value were poor nutritional status, lowered serum calcium or phosphate concentrations and severely impaired lung function as indicator of the progression of the disease. Screening of adult patients with CF can be recommended especially in presence of malnutrition or poor lung function.

Assessment of the therapeutic potential of tracheobronchial stenting for obstructive tracheobronchial disease, in-vivo comparison of different stent types and development of helpful criteria for choosing the suitable stent type.Prospective case analysis. Between 1993 and 1999 53 stents were implanted into the tracheobronchial system of 39 consecutive patients with benign or malignant airway obstruction. Every single stent (26 Strecker Stents, 18 Wallstents, 6 Accuflex Nitinolstents, 1 Dumon-, 1 Rüsch- and 1 Palmazstent) was recorded in an unified database. Analysis comprised clinical effectiveness, lung function if possible, relevant complications and radiologic follow-up parameters. The probability of their remaining within the tracheobronchial system, of their remaining undislocated and uncompressed was calculated using Kaplan-Meier analysis for three stent types.Stent placement proved itself to be an effective treatment in 86 % of the patients. Resistance could be normalized in 9/9 patients. Kaplan-Meier analysis clearly revealed a higher probability for the Wall- and Nitinolstent to remain within the tracheobronchial system and to remain uncompressed. Dislocation also occurred more rarely. Explantation of the Wallstent, however, if desired, was much more difficult compared to the Strecker stent. The Wallstent also occasionally led to the formation of granulation tissue especially at the proximal stent end and, as such, required reintervention.Any of the 3 stent types proved to be an effective therapeutic option in the management of obstructive tracheobronchial disease. Choise of the stent type should be determined through definition of the therapeutic intention. It is useful to distinguish between (a) benign stenosis, (b) malignant stenosis but curative therapeutic situation and (c) malignant stenosis in a palliative therapeutic situation with limited life expectancy. In spite of its superior mechanical properties the Wallstent is rather suited for a palliative situation because explantation may be difficult. The Strecker Stent requires more reinterventions but removal is easy to perform. The Nitinolstent possibly represents a reasonable compromise.

Long segment malacia of the trachea or main stem bronchi in children is not always suitable for surgical correction; patients may therefore remain ventilator-dependent and/or experience severe obstructive crises. We treated 7 children (ages, 4 months to 9 years) with extreme structural central airway obstruction with stent implantations. Six were mechanically ventilated; 5 had frequent life-threatening obstructive spells requiring deep sedation or paralysis. Diagnoses were: syndrome-associated tracheobronchomalacia (n = 4), malignancy infiltrating the carina (n = 1), congenital tracheal stenosis (n = 1), and tracheobronchial compression by a malpositioned aorta (n = 1). Six tracheal and 13 bronchial stents were endoscopically placed. The prostheses included mesh titan (n = 5), the newer shape memory material nitinol (n = 13), and 1 Y-shaped carina stent. Follow-up was reported for 7 weeks to 72 months. All patients showed marked improvement of their respiratory obstruction. Six children were weaned at least temporarily from ventilation. No significant bleeding, stenosis, or perforation was observed. Seven stents were changed after up to 14 months. Three children are well and at home. In 2 children airway stabilization was successful, but they later died from causes unrelated to stent placement, and 2 children died due to generalized airway disease. Soft metal mesh airway stents can offer a therapeutic option in life-threatening inoperable obstruction of the trachea and main stem bronchi in children.

Bronchopulmonary infections caused by trichomonads have been reported mainly in patients with pre-existing pulmonary or debilitating disease (e.g. bronchial carcinoma, lung abscess, bronchiectasis). Pulmonary trichomoniasis is most often due to infection with Trichomonas tenax, usually regarded as a harmless commensal of the human mouth, and may rarely be caused by other trichomonas species. A 45 year old female presented with a dry cough, exertional dyspnoea and malaise. These symptoms persisted for 6 months regardless of anti-inflammatory and anti-obstructive inhalative therapy. Sarcoidosis of the lungs, diagnosed 20 years prior, had been asymptomatic since and there was no coexistent disease. Laboratory data revealed increased ACE-levels (90 IE/ml) and lung function showed bronchial hyperreactivity on histamine challenge. No other abnormalities were found (chest x-ray, bronchoscopy, lung function test, blood count and serum calcium). The diagnosis was based on the cytological identification of numerous trophozoites of T. tenax in the bronchoalveolar lavage. Therapy with oral metronidazol for 40 days led to complete recovery from symptoms and normalisation of ACE serum levels. The patient has remained well for 12 months since. The pathogenicity of oral trichomonads in the non-immunocompromised host remains uncertain. Our patient had no known medical risk factors by comparison with published cases. The case illustrates the clinical relevance of pulmonary trichomoniasis in an otherwise healthy person.

In May 1984 a 58-year-old woman presented with a broad spectrum of clinical symptoms including malaise, arthralgia, hemoptysis and dyspnea, proteinuria and hematuria and a vasculitic necrotizing rash. Bronchial biopsies revealed subglottic granulomatous lesions and renal biopsies showed necrosis, extracapillary proliferation and crest formation, confirming the diagnosis of Wegener granulomatosis. Positive c-ANCA and anti-proteinase 3 subfraction (anti-PR3) titers were first analysed in 1991. Clinical remission was achieved by standard immunosuppressive therapy and renal function was stabilised. Several minor relapses were treated with pulsed intravenous cyclophosphamide but the symptoms could not be completely controlled. Eight years after the onset of disease, a dramatic increase in anti-PR3 titers was observed (34438 U/ml, normal range10, ELISA), followed 3 months later by a clinically apparent relapse. Immunosuppressive therapy was reinstituted without clinical improvement. At this point plasmapheresis resulted in an amelioration of clinical symptoms as well as a reduction in anti-PR3 titers. Concomitant immunosuppressive therapy was administered with oral corticosteroids. Forty days later anti-PR3 titers increased, reaching 75000 U/ml twelve months later, however this time without associated clinical symptoms. During the following months the patient had a further transient deterioration of pulmonary and renal function due to secondary bacterial infection which was successfully treated with antibiotics. A nephritic sediment was not present during these episodes. Curiously, the anti-PR3 titers have remained excessively elevated for the last three years.

Earlier observations on impaired in vitro effects of aldosterone on lymphocytic sodium and potassium pointed to the involvement of a defective nongenomic rather than genomic effector in pseudohypoaldosteronism. In this study, we investigated nongenomic aldosterone action in five patients with pseudohypoaldosteronism with regard to a rapid increase of free intracellular calcium [Ca2+]i in cultured nasal epithelial cells, assumably reflecting calcium influx through calcium channels. Patients were defined by episodes of salt loss despite high plasma aldosterone and renin levels. Four unaffected members of the families and four independent subjects served as controls. Considering an aldosterone-induced increase of [Ca2+]i by at least 10 nm as positive response, only 12% of cells from patients were responsive compared with 25% in normal subjects (P0.05). In terms of absolute changes, mean increase of [Ca2+]i was 1.6 +/- 1.1 nm in the patients (range-1-4) and 9.5 +/- 2.7 nm (P0.025) in the controls (range 1-25). Basal [Ca2+]i was not different between both groups (167 +/- 5 vs. 169 +/- 8 nm, mean +/- SE). These findings show an impaired nongenomic mineralocorticoid effector in patients with pseudohypoaldosteronism, which is in line with a defective sodium channel as shown recently by molecular cloning, and also with the fact that the classical, genomic intracellular receptor is structurally normal in these patients.

Tracheobronchial endoluminal reconstruction and stenting has become a valuable palliative tool in adults with intrathoracic tumors compromising the airways. Tracheobronchial balloon dilatation has been recently used in children and even neonates. We report a case of severe airway obstruction requiring emergency intubation and artificial ventilation in a 5-year-old child with intrathoracic recurrence of a rhabdomyosarcoma. Endoscopic balloon dilatation through the endotracheal tube with subsequent implantation of a non self-expanding metal mesh stent was used successfully, allowing extubation and discharge of the child from ICU.

Remote high dose rate brachytherapy is an effective local treatment modality for central lung tumors and has the potential to improve survival time. Optimal dose and fractionation schemes have not been identified yet. We conducted a prospective randomized study to compare two treatment schedules in terms of survival time, local tumor control, and possible complications.Group 1 received 4 brachytherapies with a dose of 3.8 Gy (at a 10-mm depth) on a weekly basis, and group 2 received 2 treatments with 7.2 Gy (at a 10-mm depth) at a 3-week interval. At a depth of 5 mm, the calculated doses would be 8 and 15 Gy. This study is still ongoing. Here we report interim results.Ninety-three patients with advanced cancer were included in the study; 44 were in group 1 and 49, in group 2. Both groups were comparable regarding age, sex, tumor stage, Karnofsky performance status, and histologic findings.A mean total irradiation dose of 13.4 +/- 5.2 Gy for group 1 and 13.7 +/- 4.4 for group 2 were applied (calculated at 10 mm from the source axis, equivalent to 27.9 Gy in group 1 and 28.5 Gy in group 2 at a 5-mm depth).The 1-year survival rate was 11.4% in group 1 and 20.4% in group 2. No significant difference in survival time was found, but mean survival was longer in group 2 (49 weeks) than in group 1 (26 weeks). Local control after 3 months was comparable in both groups. Fatal hemoptysis occurred at a similar rate in group 1 (22.2%) and in group 2 (21.1%).High-dose rate brachytherapy with 2 x 7.2 Gy with a 3-week interval is equivalent to a 4 x 3.8-Gy regimen on a weekly basis. The shorter treatment schedule is more convenient for patients, does not cause more side effects, and provides an equal local tumor control.

Background The new generation nebuliser PARI eFlow® rapid allows a highly efficient aerosol delivery at reduced inhalation time. However, lung function data during long-term use of this device are not available until now. Methods 70 clinically stable adult cystic fibrosis patients participated in this observation study. Lung function tests were performed prospectively 12 weeks after and again 9 to 12 months after switching the inhalation device from a conventional jet nebulizer to the PARI eFlow® rapid. Lung function data were collected retrospectively from the visits 1 year as well as 12 weeks prior to the switch-over. Lung function data for all time points were only available for 59 patients. Treatment time and patient's satification were recorded for both conventional and new nebuliser in all 70 patients. Results After 1 year of inhalation with eFlow® rapid, the mean change in FEV1% was -- 1.4% (n = 59 patients). The decrease in FEV1 was smaller than the change in FEV1 after 1 year of inhalation with the conventional jet nebuliser (control period, -3.1%), although this difference was not statistically significant. The same effect was seen in MEF25[%] '(-2.6% with conventional nebuliser compared to --1.6% after eFlow® rapid). Concerning the FVC, there was a greater improvement after 1 year of inhalation with the eFlow® rapid than with the jet nebuliser (+ 2.9% vs. +1.1%). For PEF%, there was an increase during the control period, whereas after inhalation with eFlow® rapid there was a decrease (+1.1% vs. --2.9%). All changes were not significantly different. The eFlow® rapid reduced total daily inhalation time by two-thirds (conventional nebuliser: 31.1 min/day; eFlow® rapid: 10.2 min/day, n = 70 patients) Conclusion Inhalation with the new nebuliser eFlow rapid does not alter FEV1, FVC or PEF significantly after 1 year of inhalation. The treatment time could be reduced significantly by the eFlow® rapid.

The paper compares the preoperative localization of stenosis in the lacrimal passage after digital dacryocystography with the operative findings in 23 eyes of 22 patients in the years 1988 to 1991 at the Munich University Eye Hospital. In a very high percentage (95.6%) there is a positive correlation. The discussion shows the advantages of the method and includes a review of relevant literature.

The results of a prospective follow-up study comprising 52 patients with central tumours of the lung submitted to endoluminal irradiation are reported. Additional external irradiation was possible in 30 patients (group A), but not in 22 patients (group B). Satisfactory immediate results were observed, together with a one-year survival rate of 39.3% (group A) and 12.5% (group B).

Orally or intravenously administered beta 2-adrenergic drugs have been found to inhibit esophageal motor function. Since inhalation of these drugs results in less systemic side effects, the present double-blind study was designed to investigate the influence of inhalation of the beta 2-adrenergic agonist albuterol (salbutamol) on esophageal motor function and gastroesophageal reflux in ten healthy volunteers. Esophageal motor function was recorded using a pneumohydraulically perfused multilumen manometry tube. Twenty-four-hour pH profiles were measured while the volunteers were ambulatory using a combined glass electrode connected to a portable recorder. Inhalation decreased neither lower esophageal sphincter pressure nor esophageal peristaltic amplitudes. Gastroesophageal reflux was similar on both occasions during inhalation of albuterol (3.1% [range, 1.0% to 25.5%] median upright time, with esophageal pH less than 4; and 0.1% [range, 0.0% to 10.7%] supine time) and during placebo treatment (3.6% [range, 1.6% to 19.8%] upright and 0.0% [range, 0.0% to 2.5%] supine time). Our data support the study of inhalation of beta 2-adrenergic drugs in asthmatic patients with accompanying gastroesophageal reflux.