[Importance of medical history in diagnosis of respiratory insufficiency in patients suffering from neuromuscular diseases and thoracic deformities]. / Stellenwert der Anamnese in der Diagnostik der respiratorischen Insuffizienz bei Patienten mit neuromuskulären Erkrankungen und Thoraxdeformitäten.

Patients suffering from neuromuscular diseases and thoracic deformities may develop global respiratory failure during their illness. We wanted to judge clinical parameters and information from the patients' medical history to reliably, quickly and noninvasively diagnose a ventilatory failure. Therefore we evaluated 105 situations with and without mechanical ventilation from 29 patients with indication for noninvasive nocturnal mask ventilation. 6 clinical parameters (e.g. heart rate, oxygen saturation, relative vital capacity), 2 test results (pH and partial pressure of carbon dioxide (pCO2)) and 6 parameters from the patients' medical history (e.g. nycturia, frontal headache in the morning, breathlessness) were investigated. After statistical evaluation we could show a relation between heart rate and pCO2 (Spearman's correlation: r = 0.331, p = 0.001, n = 105; one-tailed significance: r = 0.335, p = 0.038, n = 29). Significant differences between the groups of nycturia incidence indicate a tight relation between the incidence of nycturia and the height of hypercapnia levels (ANOVA--analysis of variance: p = 0.001). Using logistic regression we could show that information regarding medical history, especially nycturia, frontal headache and indrawings, gives important indications for global respiratory failure (sensitivity 97.62-100%, specificity 57.14-76.19%). Pathogenesis needs to be elaborated further.

[Variability of ventilation parameters of home ventilation equipment]. / Variabilität von Beatmungsparametern bei Heimbeatmungsgeräten.

The performance of pressure- and volume controlled ventilators used for invasive and non-invasive ventilation in the home were tested on a patient lung model. In order to determine the influence of tidal volume preset, breathing rate, resistance, compliance and leakage to the variability of delivered tidal volume and peak airway pressure a factorial plan with adapted analysis of variance was used. The influence of tidal volume preset, compliance and leakage to the delivered tidal volume is significant. The peak airway pressure depends hardly on the influence factors. All tested ventilators meet the legal demands. But in some clinical situations there are considerable deviations of the breathing parameters depending on the brand. In conclusion ventilators of different brands are not interchangeable.

[Effects of laparotomy on respiratory function in patients without and with pre-existing respiratory compromise]. / Auswirkungen der Laparotomie auf die Atemfunktion bei Patienten ohne und mit respiratorischer Vorschädigung.

The direct effect of the upper laparotomy on the ventilation were examined in 23 patients without and in 22 patients with preexisting disorders of breathing. During the first 3 postoperative days the parameters FVC and FEV1 were decreased for 50-20% in an inverse relation to the preexisting degree of respiratory dysfunction and the arterial PO2 decreased about 2 kPa (15 mm Hg). Clear hypoxaemia (without hypercapnia) were only found in the group with a high degree of preexisting respiratory dysfunction (FEV1 less than 1.4 l). Especially pretreatment and aftercare are recommended for this patients in the case of laparotomy.

[Extramedullary hematopoiesis -- a differential diagnosis of intrathoracic paravertebral tumors]. / Extramedulläre Blutbildung als Differenzialdiagnose einer paravertebralen Raumforderung.

BACKGROUND: Intrathoracic tumors always suggest a malignant clinical picture in the first instance. Especially in the case of paravertebral masses an extramedullary hematopoiesis is possible. It is regarded as compensation mechanism for reduced blood cell formation. PATIENTS: In four patients' radiographic examination of the lung partially bilateral paravertebral masses where detected by chance. In two cases, a chronic anemia existed, one patient suffered from an extended hemangiomatosis of the right brachium and the right hemithorax. In one case, no hematological disease could be diagnosed. RESULTS: The computed tomography revealed malignancy-simulating tumors with partially necrotic pattern. In all cases, transthoracal biopsy showed cytological evidence of typical differentiated bone marrow with a regular hematopoiesis. As the extramedullary hematopoiesis of these four patients was diagnosed by chance and no symptoms or local complications existed, no specific therapy was necessary. In one of these cases a stable radiographic outcome for more than ten years is evident. CONCLUSIONS: Especially in the case of patients with chronic anemia the existence of extramedullary hematopoiesis has to be considered upon occurrence of paravertebral tumors. An hematological disorder as cause may as well be absent. Due to the suspicion of malignancy a morphologic diagnosis is always necessary, but some clinical and radiologic features may indicate a benign process. A progression of the size can be unrecognized for ten years, so a therapeutic intervention is needed only in case of symptoms like myelocompression.

Histologically proven extrinsic allergic alveolitis with severe obstructive pulmonary emphysema

Anamnesis: 61-year old man with progressive shortness of breath on exercise. Cough and expectoration during the last 6 years. - Exposure: Driver of cereals, massive exposure to mouldy and pest contaminated grains. Gave up his profession in 1979 due to dyspnea with short (2-3 h) latency after exposure. Since 1980 intermittent exposure during occasional jobs; renewed symptomatology. Aspergillus fumigatus detected on agar plates inoculated with material from wet areas in bathroom and kitchen. - Clinical symptoms: Barely audible vesicular breathing, barrel-shaped thorax, inspiratory-intercostal retraction. - Bodyplethysmography: Obstructive pulmonary emphysema with FEV1 0.8 l, TLC 7.8 l, RV/TLC relation 67%. - Precipitin-detection: Significantly increased IgG against Fusarium. Other moulds including Aspergillus: negative; thermophilic actinomycetes: negative; pigeon and chicken: negative; Ouchterlony with native material from patients flat: negative. - CT including HR-CT: Bilateral-substantial emphysema, no bullae, no ground glass-opacity, no signs for interstitial lung diseases, no mediastinal enlargement of lymph nodes. - Alpha-1-Antitrypsin: 1.67 to 2.3 g/l (normal range), phenotype M1. - Histology: In resected material from right-side lung-volume-resection detection of pulmonary emphysema as well as lymphocyte infiltration and numerous epitheloid cell granulomas with Langhans'giant cells without caseation assessed as residues of an exogenous allergic alveolitis. - Conclusion: In a patient with lung volume reduction surgery due to severe emphysema histologically a persistent exogenous allergic alveolitis was detected, which might have caused the emphysema.

Increased susceptibility of a carrier of X-linked chronic granulomatous disease (CGD) to Aspergillus fumigatus infection associated with age-related skewing of lyonization.

Chronic granulomatous disease (CGD) is an inherited disorder characterized by the inability of phagocytes to generate normal amounts of superoxide (O2-), leaving patients susceptible to life-threatening infections. It was previously assumed that once carriers of the X-linked form of CGD were found to have 30% or more of functionally normal neutrophils, they would be free of risk for infection because the lyonization ratio was believed to be constant. Our report strongly contradicts this assumption. A 45-year-old X-CGD carrier had approximately 40% of normal neutrophils in her peripheral blood at age 21 years. Recently, she contracted a life-threatening pulmonary infection with Aspergillus fumigatus. After recovery, the ratio of normal-to-nonfunctional neutrophils was re-evaluated. She was found to have only 6-8% of normal neutrophils, suggesting that a striking decrease in the number of normal cells over the past 25 years was the reason for an increased susceptibility to Aspergillus infection. We conclude that age-related acquired skewing of the lyonization ratio can result in an increased susceptibility to life-threatening infections in X-CGD carriers.

Detection of M. tuberculosis DNA in sarcoidosis: correlation with T-cell response.

The pathogenetic role of Mycobacterium tuberculosis (M. tuberculosis) in tuberculosis is well defined, whereas its role in sarcoidosis is controversial. In sarcoidosis, activation of T-helper cells is observed, which is comparable to tuberculosis. The aim of this study was first to investigate whether M. tuberculosis DNA could be retrospectively detected in samples of patients with clinically verified sarcoidosis by polymerase chain reaction (PCR) and second, to analyze the relationship between M. tuberculosis DNA positive samples and T-cell response in sarcoidosis patients. Formalin fixed paraffin-embedded lung tissues or cell sediments of bronchoalveolar lavage, respectively, from 65 patients with sarcoidosis and lung tissues from 40 tuberculosis patients were investigated by means of different PCR assays in comparison to control samples. The primers used were derived from insertion sequence IS 986/6110 specific for the M. tuberculosis complex (123 bp PCR) and from the gene encoding the 38 kDa protein antigen b (419 bp PCR). The 123 bp assay yielded a specificity of 97% and a sensitivity of 95%. In contrast, the 419 bp PCR method showed a lower sensitivity of only 8% likely because of possible DNA degradation during fixation and embedding procedures of the tissue and the fact that this PCR uses a single copy element as target. We amplified the M. tuberculosis complex specific 123 bp fragment in 64% of samples from sarcoidosis patients. The specificity of PCR products in these cases was confirmed by DNA sequencing. Interestingly in the M. tuberculosis positive sarcoidoses, we found increased serum levels of soluble interleukin-2 receptor in correlation to the sarcoidosis stages (p < 0.05). In conclusion, the determination of M. tuberculosis by PCR alone does not permit a differentiation between sarcoidosis and tuberculosis. However these results support the contention that M. tuberculosis may play a pathogenetic role at least in the part of sarcoidosis patients with elevated interleukin-2 receptor values.