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Four out of eleven patients--none of them HIV positive--who received treatment for non-Hodgkin lymphoma by the MACOP-B protocol between June 1989 and February 1990 were taken ill during or shortly after the conclusion of the course with fulminant pneumonia necessitating artificial ventilation. In three cases Pneumocystis carinii was identified as the pathogen, and in one patient the diagnosis of pneumocystosis seemed probable. The mean cumulative doses given before the outbreak of pneumonia were as follows: cyclophosphamide 2753 +/- 1161 mg, methotrexate 1590 +/- 667 mg, bleomycin 36 +/- 16.8 mg and prednisone 4378 +/- 1734 mg. The mean haemoglobin concentration was 10.7 +/- 0.5 g/dl, leucocyte count 5250 +/- 2100/microliters, lymphocyte count 1300 +/- 300/microliters and lactate dehydrogenase 227 +/- 34 U/l. The cumulative doses and laboratory findings in the seven patients not affected by pneumocytosis were not significantly different. The patients with pneumonia were supported by mechanical ventilation for 6-26 days and treated with large doses of corticosteroids and co-trimoxazole. One patient died after 17 days' ventilation. Three patients were successfully weaned from the ventilator. Chemotherapy protocols such as MACOP-B predispose to acute Pneumocystis pneumonia. The risk of infection is independent of the cumulative doses of the drugs employed. For this reason, prophylaxis with co-trimoxazole is normally mandatory.

Basic problem and objective In rare instances Pneumocystis carinii pneumonia (PCP) can occur in a cystic form, characterized by an acute course. The correct diagnosis is often missed. An analysis of a large cohort of patients with PCP was undertaken to determine the incidence and clinical manifestations of this form of the disease. Patients and methods Data on all HIV-positive patients on the hospital registry, hospitalized with the diagnosis of PCP between January 1988 and July 1996, were retrospectively analysed. To be included in the study, PCP had to have been confirmed microscopically on bronchial lavage or histologically by lung biopsy. The chest radiogram and (if available) computed tomogram of these patients were examined for possible cystic changes, and clinical as well as laboratory findings recorded. Results Five of 180 patients with PCP had cystic changes (2.8%). The interval between first symptoms and diagnosis ranged from 30 to 200 days (median: 32 days). All five patients (age: 24-49 years; all males) had a far advanced immunological disorder (median CD4 count: 10/microliter) and were in stage C3 (Centers for Disease Control). Three of the five patients had prophylactically inhaled pentamidine (300 mg/month). Bronchial lavage was false-negative in three of the five cases. One patient had a spontaneous pneumothorax. Lactate dehydrogenase levels were normal or only slightly elevated (207-417 U/l; median 305 U/l). Both the cavities and thin-walled cysts were demonstrated radiologically, but were more visible on CT than on the X-ray film. All patients responded well to the medication with cotrimoxazole, but the prognosis was poor with a survival time of only 0.3 to 16 months. Conclusion PCP should be included in the differential diagnosis when HIV patients have cystic lung changes. Invasive diagnostic methods are often required to avoid wrong diagnosis and superfluous therapeutic interventions.

HISTORY A 65-year-old woman was admitted because of dyspnea at rest and peripheral edema due to scleroderma-associated pulmonary fibrosis and hypertension, as well as Raynaud's phenomenon. DIAGNOSTIC FEATURES, TREATMENT AND COURSE She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 |PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome. CONCLUSION PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.

Unilateral recurrent nerve paralysis leads to glottic insufficiency, causing a significant lack of vocal ability. In contrast, bilateral palsies present with stridor on inspiration due to glottic stenosis. Most of the underlying lesions are iatrogenic, with thyroid surgery being the single most important causative factor. However, a variety of different reasons can lead to such a condition. Whenever aetiology is uncertain a complete diagnostic work-up is mandatory. Indirect laryngoscopy confirms the diagnosis. Laryngeal electromyography is of great value because it differentiates between paralysis and ankylosis of the cricoarytaenoid joint. Moreover, in many cases laryngeal electromyography provides a reliable prognosis of clinical outcome. While unfavorable results can be predicted with high accuracy, correct prognosis of complete recovery is more difficult. Speech therapy is the treatment of choice in case of unilateral recurrent nerve palsy. Only if a significant glottal gap persists medialization procedures may become useful for voice improvement. Endoscopic as well as open approaches are available for this purpose. Bilateral recurrent nerve palsies need to be addressed surgically in the vast majority of cases. Today, a variety of endoscopic techniques for widening the glottic airway are available. Compared to permanent tracheostomy these procedures have much less impact on the patient's quality of life and should be preferred whenever possible. Inevitably, voice quality is traded for airway normalisation. However, modern surgical techniques accomplish very tolerable phonatory results.

Background . Six patients known to have inoperable esophageal carcinoma presented with stridor due to both malignant tracheal stenosis (n = 6) and bilateral vocal cord paralysis. Two patients also had respiratory-digestive fistula. Methods . Patency was restored by endotracheal stenting plus unilateral cordectomy. Four patients had immediate relief. Two patients required enlargement of the cord incision. One of them declined reoperation and underwent tracheotomy. Results . Stent function was uneventful. There was no dislodgement or mucous impaction. Fistula seal was complete. There was no aspiration through the new-shaped glottic orifice. Peak expiratory flow increased from 24.4% ± 9.7% predicted normal before to 40.5% ± 13.7% after the procedure, whereas the dyspnea score decreased from 74.2 ± 12.7 to 24.2 ± 14.0. Conclusions . Restoration of airway continuity in serial laryngotracheal stenoses using a combined approach is a feasible technique in end-stage cancer patients. It effectively relieves respiratory distress and ensures voice preservation. In addition, it may avoid the risks of tracheotomy.

Our objective was to assess the effectiveness of transoral laser cordectomy and laser arytenoidectomy and to compare the results with a view to respiratory and phonatory function and deglutition. Twenty-eight patients with bilateral vocal cord paralysis were included in a prospective study. Eighteen patients had cordectomy, and 10, arytenoidectomy. Lung function tests and voice analysis were performed preoperatively and postoperatively. Subclinical aspiration was determined by endoscopic evaluation of the larynx during deglutition. The results were compared to determine the relative effectiveness of both surgical methods. How volume spirograms documented equally improved flow rates in both groups. The final voice evaluation revealed that maximum phonation time, peak sound pressure levels, and frequency range were reduced in all 28 patients, but the phonatory results varied considerably in each group. Subclinical aspiration was noticed in 5 of 10 patients after arytenoidectomy, but in none of 18 patients after cordectomy. Four of 6 previously tracheostomized patients were decannulated within 2 weeks after surgery, while the other 22 patients had no perioperative tracheostomies. We conclude that transoral laser cordectomy and arytenoidectomy are equally effective and reliable in the management of the restricted airway. Phonatory outcome is not predictable with either surgical procedure. Cordectomy is easier and faster to perform, and subclinical aspiration is not encountered with this procedure.

In contrast to the effects of cigarette smoke on T-lymphocyte subsets in the airways, it has not yet been determined whether smoking has immunomodulatory effects on surface antigens of peripheral blood T-lymphocytes and, if that is the case, whether these effects differ in smokers with and without chronic obstructive pulmonary disease (COPD). The present authors have, therefore, examined the expression of the surface activation marker CD28, the levels of cytotoxic effector lymphocytes (CD27-/CD45RA+) and the expression of the lung type (Tc)1-specific chemokine receptor CXCR(3)+ on peripheral blood CD8+ T-lymphocytes. The present authors have also studied the chemotactic activity of CD8+ T-lymphocytes on monocyte chemotactic protein (MCP)-1 and compared 13 nonsmoking controls, 12 smokers with COPD and 14 smokers without airflow limitation. There was a decrease in the total count of CD8+ T-cells and an increase in the CD4+/CD8+ ratio in smokers with COPD compared with smokers without COPD and controls. Expression of the Tc1-specific chemokine receptor CXCR(3)+ by CD8+ T-cells was increased in smokers with COPD compared with smokers without COPD and controls. The expression of activated and of cytotoxic effector CD8+ T-cells in smokers with and without COPD showed an increase compared with controls. CD8+ T-cells from smokers with and without COPD showed a decrease in chemotactic activity to MCP-1 compared with controls. In conclusion, chronic obstructive pulmonary disease may be a systemic immunomodulatory disease associated with the modification of surface antigens in blood CD8+ T-lymphocytes.

Objectives/Hypothesis: Airway stents have recently been used to establish and maintain patent airways in patients with malignant central airway obstruction, but insertion modalities remain controversial to date. The study seeks to determine the role of suspension laryngoscopy in interdisciplinary airway stenting. Study Design: Retrospective, single-institution analysis of a case series treated by a multidisciplinary airway team. Methods: Ninety-three consecutive patients with malignant obstruction of the trachea and/or tracheobronchial bifurcation underwent endotracheal stenting through a suspension laryngoscopy approach for the relief of impending respiratory distress. Feasibility, mortality, survival, and complications were analyzed as main outcome measures. Results: Stenting through a suspension laryngoscopy approach was feasible 91 of 93 patients (97.8%). Fifteen patients needed repeated stenting, and in all, 121 stents were implanted during the observation period. This approach allowed for the repeated insertion of rigid bronchoscopes of graded sizes to establish an airway and for precise stent positioning. Optical instruments and stent introducer systems could easily be used while adequate ventilation was continuously maintained. Silicone stents of maximal size were inserted without injury of the vocal cords during intubation. Median survival for all patients was 8 months. No intraoperative airway complications were observed, and no patient died secondary to stenting. Conclusions: Suspension laryngoscopy and jet ventilation provide an ideal setting for the precise placement of tracheal and bifurcation airway stents. Laryngologists should actively participate in interdisciplinary airway stenting programs.

Six patients known to have inoperable esophageal carcinoma presented with stridor due to both malignant tracheal stenosis (with additional respiratory-digestive tract fistula in 2 patients) and bilateral vocal cord paralysis. Patency was restored by endotracheal stenting plus unilateral cordotomy. Four patients had immediate relief. Two patients required enlargement of the vocal cord incision. One of them declined reoperation and underwent tracheostomy. The stent function was uneventful, with no dislodgment or mucus impaction. The fistula seal was complete, with no aspiration through the newly shaped glottic orifice. The peak expiratory flow increased from 24.4% ± 9.7% of predicted normal before the procedure to 40.5% ± 13.7% after the procedure. Restoration of airway continuity in serial laryngotracheal stenoses by a combined approach is a feasible technique in end-stage cancer patients. It effectively relieves respiratory distress and ensures voice preservation. In addition, it may avoid the risks of tracheotomy.

Objectives: To evaluate a newly developed bronchoscopic technique for the assessment of intratracheal pressures. Study Design: In situ measurement of central airway resistance in 20 consecutive spontaneously breathing subjects. Thirteen patients had benign glottic or subglottic stenosis. Seven patients without central airway disease served as normal control subjects. Methods: A pressure catheter was introduced into the trachea via the working channel. The pressure swing was measured upstream and downstream of the stenosis. Central airflow was monitored simultaneously using a commercial pneumotachograph attached to a mouthpiece. Data acquisition frequency was 500 Hz. Prestenotic and poststenotic inspiratory and expiratory resistances could be calculated and displayed from the raw data off-line. Results: Inspiratory and expiratory resistances measured in mid-trachea or below the stenosis (subglottic) were 0.36 ± 0.13 and 0.35 ± 0.13 kPa·s/L for the control subjects (C), 1.11 ± 0.47 and 0.65 ± 0.26 kPa·s/L for patients who did not need to be operated on (NOOP), 7.11 ± 7.19 and 3.35 ± 2.25 kPa·s/L respectively for those who required surgical correction (OP). Supraglottic inspiratory and expiratory resistances for C were 0.22 ± 0.09 and 0.25 ± 0.06 kPa·s/L, for NOOP 0.15 ± 0.10 and 0.14 ± 0.11 kPa·s/L, and for OP 0.26 ± 0.13 and 0.24 ± 0.07 kPa·s/L respectively. The cut-off point for surgical correction was estimated to be >2.5 kPa·s/L of inspiratory resistance. Concurrent expiratory values showed a considerable overlap between OP and NOOP. No correlation could be established between local resistance values and dyspnea score. Conclusions: In situ subglottic flow-pressure tracing in spontaneously breathing patients who present with benign obstruction of the upper airways is well tolerated and may help to identify patients who need surgical correction.

Chronic eosinophilic pneumonia (CEP) is a rare disease characterized by a progressive symptomatic deterioration of more than 1 month, pulmonary infiltrates with eosinophils, and a dramatic response to corticosteroid treatment. We describe a patient with CEP who presented with right upper lung infiltrates with evidence of cavitation.

Background: Although widely established in the management of malignant airway lesions, currently available tracheobronchial prostheses made of silicone have their drawbacks resulting from rigidity and wall thickness. Therefore we present clinical follow-up data obtained with a novel thin-walled expandable prototype silicone airway stent (Polyflex stent, Willy Rusch AG, Kernen, Germany) in 19 patients. Methods: Seventeen of 19 patients had tracheobronchial complications of infiltrating cancer: five had respiratory-digestive fistulas, 14 had mixed-type obstructions (mucosal infiltration plus extrinsic compression), and two had diffuse tracheal hemorrhages from the tumor surface (three patients had more than one complication). Two of 19 patients had benign postintubation stricture and malacia. Overall, 33 stents were implanted either simultaneously or in a consecutive manner. Scanning electron microscopy was performed both on prototype stents and on other available silicone stents for comparison. Results: The treatment improved the patients' clinical condition substantially. The mechanical properties of the new prosthesis were excellent. Important stent-associated side effects were early mucus retention ( n = 7), infolding of the inner silicone layer ( n = 2), and stent dislodgment ( n = 2). As of February 1997, 10 patients have died of causes unrelated to stent placement. Seven patients with malignant airway disease are still alive from 2 weeks up to 7 months after initial treatment. Scanning electron microscopy of explanted and unused prototypes suggested that an extremely ragged luminal microstructure may contribute to the firm adhesion of secretory material and that technical smoothing of the surface avoids such complications. Conclusions: The novel self-expandable silicone airway stent may be a promising addition to commonly used stent types. Short-term and medium-term management of fistulas, tumor surface bleeding, and strictures (malignant and benign) is satisfactory. Scanning electron microscopy of stents provides information on peculiar features of microstructure and material that may be of use in clinical research and technical innovation. (J Thorac Cardiovasc Surg 1997;114:527-34)