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Biomedical subjects

R Ferlinz

Publications and source records attributed to R Ferlinz.

At least 19 recordsLinked to original sources

[Clinical late manifestation of Ondine's syndrome with pronounced primary, especially sleep related alveolar hypoventilation].

We report on a very rare case of a primary, alveolar hypoventilation syndrome (Ondine's Curse syndrome) in a female patient who was first treated at the age of 26 years as a result of an influenza infection; however, the case history revealed a decreased performance in comparison to similar aged children from earliest childhood onwards as well as an intermittent zyanosis of the lips and distal extremities. Other diseases which could explain a global respiratory insufficiency and/or pulmonary arterial hypertension were excluded.

Adult

[Experimental determination of equivalent percentage changes in measurement parameters of dose-response relations with the inhaled methacholine provocation test].

For the experimental determination of equivalent changes of the measurement parameters in the non-specific inhalative provocation test with metacholine, 26 persons with exogenous allergic asthma bronchiale were examined during the symptom-free interval. The inhalative provocation test was performed as dose-response relationship. Linear regressions were calculated with the data obtained. The percentage change of the measurement parameters that occurred with a quantity of 400 micrograms of aerosolised methacholine was calculated on the basis of the regression equation. These changes correspond to equivalent changes of the individual measurement parameters compared with each other. Generally, a drop in FEV1 by 20% is demanded for a positive provocation test. If this value is defined as "gold standard", the measured data allow derivation of the equivalent changes. Our studies showed that an increase of Rt by 195% (triplication), a sGaw drop by 44%, a PEF drop by 20% and a drop of MEF75, MEF50 by 30% each, compared with the original value, is equivalent to a significant change in the sense of a positive inhalative provocation test.

Adolescent

Lung-restricted activation of the alveolar macrophage/monocyte system in pulmonary sarcoidosis.

An activation of T-cells that is restricted to the lung has been demonstrated in pulmonary sarcoidosis. The role of blood monocytes (MO) and alveolar macrophages (AM) in this concept of compartmentalized inflammation has not yet been evaluated. In order to elucidate this question, we measured the release of tumor necrosis factor alpha (TNF alpha) and interleukin-1 (IL-1) by peripheral blood mononuclear cells (PBMNC) and AM in 43 patients with sarcoidosis (32 with active, 11 with inactive disease) without therapy and correlated the spontaneous monokine release to parameters of the T-cell alveolitis and the course of the disease. TNF alpha as well as IL-1 were spontaneously released by AM of the active group, i.e., 2,385 +/- 735 pg/ml/10(8) cells/24 h and 7/12 (IL-1+/total), respectively. Autologous PBMNC were quiescent, releasing only baseline levels of any monokine. AM were not activated in the inactive group, releasing 500 +/- 212 pg/ml/10(6) cells/24 h TNF alpha, whereas 1/5 were IL-1-positive (p less than 0.05 in both comparisons), which is within the range of the control group. Kinetic experiments revealed that the TNF alpha gene of AM is activated in vivo, resulting in TNF alpha mRNA-positive, TNF alpha-releasing cells that, cultured in vitro, regulate the TNF alpha gene transcription down and cease to release TNF alpha. Interestingly, there is no stringent correlation between the spontaneous release of TNF alpha by AM and signs of T-cell activation as soluble interleukin-2 (IL-2) receptor serum concentration, release of IL-2, and expression of IL-2 receptor by alveolar T-cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans

Fibrosing alveolitis responsive to corticosteroids following Legionnaires' disease pneumonia.

Two male patients ages 54 and 58 years had persisting pneumonia with dry cough, dyspnea, weight loss, and fever up to 39 degrees C that did not respond to erythromycin treatment. There was extensive restrictive impairment of ventilation and loss of diffusing capacity for carbon monoxide. Histologic examination of the basal pulmonary infiltrates showed fibrosing alveolitis. Serologic titers indicated that the patients had suffered from Legionella pneumophila infection. We believe that Legionella had caused the fibrosing alveolitis since there was absence of any other causative agents or factors. Both patients responded to corticosteroid treatment with rapid clinical improvement but delayed radiologic regression.

Humans

[Pulmonary infiltrations with eosinophilia (pulmonary eosinophilia)].

The pulmonary eosinophilias are characterised by radiographic lung shadows with either a peripheral blood eosinophilia of more than 450/microliter or histologic abnormalities consisting of both interstitial and intraalveolar accumulations of eosinophils and macrophages. We describe the clinical features, radiographic changes, results of bronchoscopy and follow-up studies of three women with chronic eosinophilic pneumonia of unknown aetiology. In all patients the illness resolved rapidly after treatment with corticosteroids, however one patient experienced a second episode after treatment withdrawal. We demonstrate the wide differential diagnosis of the syndrome of pulmonary eosinophilia.

Adult

[Primary pulmonary nodular amyloidosis and multiple emphysematous bullae in Sjögren syndrome].

The authors report on a very rare case of an isolated primary nodular pulmonary amyloidosis with multiple emphysematous bullae in Sjögren's syndrome. The circular foci present in both lungs in disseminated form were immunohistochemically speaking amyloid deposits of the AL-lambda type. There were no pointers to other organ manifestations or monoclonal immunoglobulins in the serum and/or urine.

Amyloid

Spontaneous monokine release by alveolar macrophages in chronic sarcoidosis.

In pulmonary sarcoidosis an activation of alveolar T lymphocytes and alveolar macrophages (AM) has been demonstrated. There is evidence that in contrast to acute disease a heightened T-cell response cannot be observed in the chronic phase of sarcoidosis. The role of AM in the inflammatory process of chronic sarcoidosis is not yet intensively evaluated. To address this question we measured the release of tumor necrosis factor alpha (TNF alpha) and interleukin-1 (IL-1) by AM of 39 patients with chronic sarcoidosis (duration greater than 4 years; 30 active, 9 inactive diseases) without therapy and correlated the monokine release with parameters of T-cell alveolitis and the course of the disease. The T4/T8 ratio was higher in the active than in the inactive group without reaching statistical significance. TNF alpha as well as IL-1 is spontaneously released by AM of the active group 2,099 +/- 518 pg/ml TNF alpha/10(6) cells/24 h and 8/13 (IL-1+/total) respectively. In the inactive group the AM release 375 +/- 246 pg/ml TNF alpha/10(6) cells/24 h which is in the range of the control and 1 out of 5 patients was IL-1-positive. There was no correlation between the monokine release and any parameter of T-cell alveolitis. These data support the hypothesis that the inflammatory process in chronic sarcoidosis is dominated by the activity of AM and that this activity determines the course of the disease.

Adult

Correlation of clinical and immunologic parameters of the inflammatory activity of pulmonary sarcoidosis.

The evaluation of activation markers such as T4/T8 ratio and HLA-DR expression of lymphocytes of bronchoalveolar lavage (L-BAL) is an important clinical approach for the staging of sarcoidosis. However, it is not known to what extent this is paralleled by an exaggerated lymphocyte function. We investigated the dependence of L-BAL activation markers on the production of interleukin-2 (IL-2) by L-BAL and on the soluble IL-2 receptor serum level (sIL-2R) in 116 patients with sarcoidosis. In none of the combinations tested was a correlation between the two groups of parameters found; r less than 0.5, upper 90% confidence limit of r less than 0.8. Interestingly, IL-2 production is independent of HLA-DR+ T4 L-BAL, and sIL-2R production is independent of the percentage of IL-2+ L-BAL. Our data indicate that the L-BAL activation markers and the functional activity of T-cells represent independent phenomena.

Adult

[Diabetes insipidus in sarcoidosis].

Sarcoidosis is a generalized disorder which involves the central nervous system in 5 per cent of patients presenting with the disease. We describe the case of a 22-year-old man who developed central diabetes insipidus with a daily urine production of 14,81 seven weeks after diagnosis of pulmonary sarcoidosis and parotid gland enlargement. Computed tomography showed a contrast enhancement of pituitary gland and stalk, no other intracranial manifestations were demonstrated. Intranasal administration of synthetic antidiuretic hormone (ADH) reduced urine volume to normal levels immediately. After discontinuation of 15 months therapy with corticosteroids and administration of ADH for 29 months spontaneous urine volume didn't exceed 31. The patient has been free of symptoms since discontinuation of therapy.

Administration, Intranasal

Assessment of local cellular immunity in lung cancer by bronchoalveolar lavage.

Small cell lung cancer (SCLC) is the most malignant of the pulmonary neoplasms and is associated with a poor local cellular immune response. 16 patients with non small cell lung cancer (NSCLC) and 11 patients with SCLC underwent bronchoalveolar lavage (BAL) in the lung which harbored the tumor in order to investigate the lymphocyte surface antigens utilizing the immunoperoxidase technique. Analysis of blood lymphocytes was performed in parallel. 8 patients with previous sarcoidosis in complete remission who underwent BAL and 10 normal blood donors served as controls. Among blood lymphocytes the CD3+, CD4+ and CD16+ cell populations were elevated significantly and the T4/T8 ratio was elevated in NSCLC patients, but only CD16+ were augmented in SCLC. Cell populations expressing the activation markers transferrin (TF) receptor, interleukin-2 (IL-2) receptor and the very late antigen VAL-1 were also increased in NSCLC, while SCLC was associated with antigen distributions similar to controls. No differences between the cohorts were seen in the expression of human leukocyte antigen (HLA)-DR. In BAL the population of CD3+ and CD4+ cells were reduced in SCLC and the T4/T8 ratio was diminished in contrast to controls and NSCLC patients, whereas these two latter groups did not differ from each other. The distribution pattern of CD16, TF receptor and IL-2 receptor in the study groups resembled that of cells of the blood stream, but CD16+ natural killer cells were additionally down regulated to control values in SCLC. No differences were seen in the distribution of VLA-1. HLA-DR+ cells were clearly elevated in both cancer groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenocarcinoma

Oxygen radical production by alveolar inflammatory cells in idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic inflammatory interstitial lung disease characterized by the accumulation of alveolar macrophages (AMs) and neutrophils in the lower respiratory tract, parenchymal cell injury, and fibrosis of the alveolar structure. Reactive oxygen intermediates (ROI) are claimed to be a major cause of tissue damage in IPF; however, the source of ROI has not been unequivocally identified. AMs, as well as neutrophils, are capable of releasing these agents. The contributions of these possible sources are not known. To address this question, we evaluated the spontaneous and stimulated (PMA or zymosan) ROI release of total bronchoalveolar cells and isolated AMs in 14 patients with IPF by means of luminol-enhanced chemiluminescence. Bronchoalveolar lavage (BAL) cells from 17 individuals without any signs of inflammation served as controls. In comparison with the controls, the spontaneous as well as the stimulated ROI release of total BAL cells in IPF are markedly increased (20,763.9 +/- 5,079.3 versus 2,509.5 +/- 300.6 counts/10 s/2.10(5) cells, spontaneously, IPF versus control; 106,819.3 +/- 33,802.8 versus 8,919 +/- 1,357.9 PMA induced; 41,597.1 +/- 8,442.6 versus 6,223.8 +/- 1,025.1 zymosan induced, p less than 0.001). Measurement of the ROI release of purified AMs revealed that these cells produce the bulk part of ROI released by BAL cells (84%). In spite of the fact that, on a per cell basis, the ROI release of neutrophils is 1.7-fold of that of AMs, there is no correlation between the ROI production of total BAL cells and the percentage of neutrophils in BAL, demonstrating a minor role of these cells in the generation of the total ROI burden in IPF.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

[Oral therapy with beta-2-sympathomimetic drugs: comparative study of the bronchospasmolytic effect of tulobuterol and fenoterol in patients with reversible airway obstruction].

In a multicentre, single-blind study, the bronchospasmolytic effect of a single oral dose of tulobuterol 2.0 mg and of fenoterol 2.5 mg was compared in 26 patients suffering from reversible airways obstruction. The bronchospasmolysis achieved with the oral application of tulobuterol and fenoterol is qualitatively satisfactory and reliable, the effect of tulobuterol persisting for a longer period than that of fenoterol.

Administration, Oral