A manifestation of sarcoidosis in a woman with Marfan's syndrome.
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Biomedical subjects
Publications and source records attributed to M Drent.
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In the following case report the determination of serum lactate dehydrogenase (LDH)-a simple though non-specific test-reflects changes of disease activity and clinical improvement during treatment with cyclophosphamide and a tapered dose of prednisone from 100 to 0 mg daily in a 43-year-old woman with idiopathic pulmonary fibrosis. The trend observed in this case indicates that the serum LDH-activity may be directly proportional to the extent of this diffuse inflammatory pulmonary disease.
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During the six months from January-June 1994, 10 cases of severe and 11 of less severe pulmonary infection caused by Pseudomonas aeruginosa were diagnosed in patients with chronic obstructive airways disease. Possible sources were evaluated. P. aeruginosa was isolated from four of the 22 nebulizers tested. The relationship of isolates from the patients and nebulizers was confirmed by sero- and phage-typing, and by arbitrarily-primed polymerase chain reaction (AP-PCR). Three types were identified and the distribution of types in patients with severe infection was as follows (one patient had a multiple infection). Type I was isolated from two nebulizers and from sputa, and/or blood and/or bronchial protected specimen brush samples or bronchial lavage fluid from four patients. Type II came from the sputa of three patients and a third nebulizer; and type III from sputa and/or blood of four further patients and another nebulizer. The data provided evidence for the relation between P. aeruginosa as a cause of infection and the contamination of the nebulizers. When nebulizer mouthpieces were changed every 24 h and sterilized between patients, no more contamination occurred, and the outbreak ceased.
Recently, we showed that it is possible to distinguish between three common interstitial lung diseases (ILD) with similarities in clinical presentation by using a number of selected variables derived from bronchoalveolar lavage fluid (BALF) analysis. The aim of this study was to develop a more general discriminant model, based on polychotomous logistic regression analysis. The 277 patients involved in the study belonged to diagnostic groups with sarcoidosis (n = 193), extrinsic allergic alveolitis (EAA; n = 39), and idiopathic pulmonary fibrosis (IPF; n = 45). The diagnosis had been established independently of the BALF-analysis results. The variables used to discriminate among these patient groups were the yield of recovered BALF, total cell count, and percentages of alveolar macrophages, lymphocytes, neutrophils, and eosinophils. In order to test the predictive power of the logistic model, we used 128 patients having sarcoidosis (n = 91), EAA (n = 5), or IPF (n = 32) from another hospital. In this test set the agreement of predicted with actual diagnostic-group membership was the same as in the learning set in which the logistic model was fitted: 94.5% of the cases were correctly classified. A validated computer program based on the polychotomous logistic regression model can be used to predict the diagnosis for an arbitrary patient with information provided by BALF analysis, and is thought to be of diagnostic value in patients suspected of having ILD.
This review describes the usefulness of monitoring the activity level of lactate dehydrogenase (LDH) and its isoenzyme pattern as indicators of pathological conditions in the lungs, such as cell damage or inflammation. Cytoplasmatic cellular enzymes, like LDH, in the extracellular space, although of no further metabolic function in this space, are still of benefit because they serve as indicators suggestive of disturbances of the cellular integrity induced by pathological conditions. Since LDH is an enzyme present in essentially all major organ systems, serum LDH activity is abnormal in a large number of disorders. Although the increase in total serum LDH activity is rather nonspecific, it is proposed that measurement of LDH activity levels and its isoenzyme pattern in pleural effusion and, more recently, in bronchoalveolar lavage fluid may provide additional information about lung and pulmonary endothelial cell injury.
A 30 year old man developed lipoid pneumonia after an injection of lamp oil. In addition to "foamy" alveolar macrophages, bronchoalveolar lavage (BAL) fluid analysis showed an increased number of neutrophils. Moreover, lactate dehydrogenase (LDH) and alkaline phosphatase activities were elevated. The increase seen in LDH activity both in serum and BAL fluid was accompanied by shifts in the isoenzyme pattern in similar directions for both fluids. These findings suggest a pulmonary source for the temporary serum as well as BAL fluid LDH increase. This case indicates the usefulness of bronchoalveolar lavage fluid analysis as a probe to detect pulmonary injury caused by a pneumotoxicant and, probably, to monitor recovery or deterioration.
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The aim of this study was to identify characteristic features in bronchoalveolar lavage fluid (BALF) samples of patients with tuberculosis, non-Hodgkin's or Hodgkin's disease and to investigate whether these differences facilitate the distinction of those disorders from sarcoidosis presenting with a similar clinical picture. Nonsmoker patients with histologically verified sarcoidosis (n = 29), tuberculosis (n = 6) proven by positive culture, non-Hodgkin's disease, (n = 6) or Hodgkin's disease (n = 7), both histologically verified, were investigated by BAL. A control group consisted of subjects without any pulmonary history. The presence of CD4+ and CD8+ T lymphocytes, as well as the CD4/CD8 ratio in BALF, aided in the differentiation between the various groups. Patients with malignant lymphomas had the lowest CD4/CD8 ratio in BALF, as well as in peripheral blood, and occasionally, plasma cells were present in BALF samples. The most important feature of BALF analysis in tuberculosis was detection of the causal microbial agent. In conclusion, although malignant lymphomas and tuberculosis require histologic evaluation and a positive culture, respectively, for diagnosis, BALF analysis may be of additional value in distinguishing those disorders from sarcoidosis.
Two patients presented with severe side effects of theophylline. Due to the theophylline intoxication patient A developed ischaemic hepatitis, which made haemodialysis treatment necessary to accelerate the elimination of the drug from the body. The intoxication caused immobilisation and total dependence with respect to the daily activities of patient B. In neither case was the association between mood disorders--like depression, agitation and anxiety--and an overdose of theophylline recognised. Administration of theophylline should be monitored with regular measurements of the serum theophylline concentration, because of its narrow therapeutic margin and the serious toxic side effects.
BACKGROUND: Plasma cells are usually absent in bronchoalveolar lavage (BAL) fluid. Extrinsic allergic alveolitis is associated with increased numbers of T and B lymphocytes in BAL fluid, as well as the presence of a few plasma cells. The aim of this study was to investigate whether there is a relationship between the presence of plasma cells and other cells, and immunoglobulin levels in BAL fluid of patients with extrinsic allergic alveolitis. METHODS: Thirty non-smoking patients with extrinsic allergic alveolitis who had a bronchoalveolar lavage 2-7 days after their last exposure to the causative antigen were selected, retrospectively. RESULTS: Patients suffering from extrinsic allergic alveolitis with plasma cells in the BAL fluid (n = 18) had increased absolute numbers of lymphocytes, eosinophils and mast cells, a decreased percentage of alveolar macrophages and lower CD4/CD8 ratio, as well as higher immunoglobulin levels, when compared with patients with extrinsic allergic alveolitis having no plasma cells in the BAL fluid (n = 12). CONCLUSIONS: The results suggest a relationship between the presence of plasma cells and the other constituents in BAL fluid and a more intense alveolitis. In addition there was a positive relationship between the number of plasma cells in BAL fluid and immunoglobulin levels. These data support the concept of local production of immunoglobulins by plasma cells in the lung following antigen exposure in susceptible individuals.
The aim of this study was to investigate whether the demonstration of plasma cells (PC), which are normally absent in bronchoalveolar lavage (BAL) fluid, facilitates differentiation among pulmonary disorders. Initial BAL fluid samples of 1,260 patients were analyzed. In 83 of these, PC were found. Of these 83, 47 were obtained from individuals suffering from extrinsic allergic alveolitis (EAA). The number of PC in BAL fluid from EAA patients was found related to the time between antigen exposure and BAL. Drug-induced pneumonitis appeared to be another disorder with a high percentage of cases with PC in the BAL fluid (35.7 percent). Therefore, we conclude that determination of PC in BAL fluid has differential diagnostic value in discriminating among interstitial lung diseases of various origins. However, the exact role of BAL fluid and PC and the link to clinical manifestations of these diseases needs further investigation.
One hundred patients with histologically verified sarcoidosis were studied. They were divided into three groups, based on their clinical presentation and smoking status. Group A consisted of patients whose disease was detected by routine chest x-ray film, without symptoms; group B included those with respiratory and general constitutional symptoms; and group C included patients with erythema nodosum and/or arthralgia and hilar lymphadenopathy. Group A showed an increased CD4/CD8 ratio of 4.7 +/- 1.1; group B, 8.0 +/- 1.2; and group C counted for the highest ratio of 10.7 +/- 1.5. Cigarette smoking modifies the immunologic bronchoalveolar lavage (BAL) fluid sample profile, since alveolitis was less pronounced in smokers. In addition, BAL fluid samples obtained from sarcoidosis patients with hilar lymphadenopathy showed the most characteristic features of alveolitis, suggesting a disseminated instead of a local immune response. Therefore, the clinical presentation of sarcoidosis and the smoking status of a sarcoidosis patient are crucial for interpreting individual lavage analysis results.
The aim of the present study was to investigate whether bronchoalveolar lavage (BAL) cell profile and immunoglobulin levels from patients with extrinsic allergic alveolitis (EAA) were related to the time elapsed between last antigen exposure and BAL. For this purpose, an analysis was performed of BAL fluid (BALF) obtained from 59 nonsmoking EAA patients at various time-points after termination of antigen exposure and BAL. BALF early after antigen provocation (group 1: < 24 h) contained high absolute and relative numbers of lymphocytes, neutrophils, eosinophils and mast cells, and a low relative number of alveolar macrophages. When obtained after recent antigen exposure (group 2: 2-7 days), BALF showed high numbers of lymphocytes, plasma cells and mast cells, and high levels of immunoglobulins M, G and A (IgM, IgG and IgA). In BAL obtained one week or more after the final antigen exposure, (Group 3: 8-30 days; Group 4: 1-12 months) the distribution of all constituents showed a tendency to return to normal values, with the exception of the lymphocytes. These results demonstrate that BAL cell profile and immunoglobulin levels in EAA are highly dependent on the time-point at which the material is obtained in relation to the last exposure to the causative antigen.
The aim of this study was to investigate the possibility of distinguishing between patients with similarities in clinical presentation, suffering from three frequently occurring interstitial lung diseases, by means of discriminant analysis, using a number of selected variables derived from bronchoalveolar lavage fluid (BALF) analysis. The study involved all 277 patients, who had an initial bronchoalveolar lavage (BAL) in the period 1980-1990. These patients belonged to the following diagnostic groups: sarcoidosis (n = 193), subacute extrinsic allergic alveolitis (EAA) (n = 39) and idiopathic pulmonary fibrosis (IPF) (n = 45). Thirty healthy volunteers were used as controls. Cellular and non-cellular constituents of BALF were evaluated. Variables, which could be used to discriminate among the three diagnostic groups were: yield of recovered BALF, total cell count, and percentages of alveolar macrophages, lymphocytes, polymorphonuclear neutrophils, eosinophils and plasma cells in BALF. When the set of data used to predict the membership of patients to diagnostic groups (test set) was the same as that in which the discriminant analysis was performed (learning set), 93% of the cases were correctly classified. This percentage decreased to 90%, however, when the test set was different from the learning set. It is possible to discriminate among patients with sarcoidosis, EAA or IPF with these selected variables. It appears that bronchoalveolar lavage (BAL) is useful as an adjunct in concert with other diagnostic methods.
Two patients are described with severe MVS, pulmonary venous hypertension and enlarged mediastinal, pulmonary and hilar lymph nodes. These enlargements were diagnosed on a preoperative chest CT. After MV replacement these enlarged lymph nodes nearly all resolved. The lymphadenopathy should be considered to be secondary to MVS with pulmonary venous hypertension.