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Biomedical subjects

G Steinkamp

Publications and source records attributed to G Steinkamp.

At least 55 records · Page 3Linked to original sources

Resting energy expenditure before and after treatment for Pseudomonas aeruginosa infection in patients with cystic fibrosis.

To investigate whether metabolic rates change after antipseudomonal treatment, resting energy expenditure (REE) was measured in 29 patients with cystic fibrosis (CF) aged 5-27 y before and after a 2-wk course of intravenous antibiotics for the treatment of chronic Pseudomonas aeruginosa infection. Before therapy, mean (+/- SD) REE was increased to 119.0 +/- 11.4% of the predicted normal value (REE%). Seventeen of the 29 patients were classified as having increased baseline REE% (> 115% of predicted, group I), whereas 12 patients had resting energy expenditures within the normal range (group N). After antipseudomonal therapy, mean REE% decreased significantly by 6.2% of predicted. This decline was negatively correlated with the baseline REE% (r = 0.70, P < 0.001). It was most pronounced (-11.3%) in group I whereas patients of group N showed no major changes. These results suggest that antipseudomonal therapy can reduce increased energy requirements of moderately ill CF patients with chronic Pseudomonas aeruginosa infection.

Adolescent↗

[Mucoviscidosis. Therapeutic strategies and survival].

The life expectancy of patients with cystic fibrosis (CF) has increased considerably during the last decades due to improved therapy; one third of German CF patients has now grown into adult-hood. The present paper describes the main therapeutic principles: oral antistaphylococcal treatment, therapy of chronic bronchopulmonary infection with Pseudomonas aeruginosa using intravenous, inhaled or--in adults--oral antibiotics, and improved regimens for the treatment of malnutrition. Finally, survival data from different CF centres are presented.

Anti-Bacterial Agents↗

[Mucoviscidosis--not a topic for pneumonologists?].

Although cystic fibrosis (CF) is the most common lethal hereditary disease, adult physicians remain to be relatively unfamiliar with this disease. In Germany, more than 25 per cent of the 4,000 CF patients are adults, but are treated by paediatricians. The present paper aims to focus the pulmonologist's attention to this problem. A brief review is given on aspects of genetics, pathophysiology, diagnosis, and treatment. Centralized treatment by an experienced CF clinic is important not only for children, but also for adults, and the contribution of adult pulmonologists to the care of these patients is urgently needed.

Combined Modality Therapy↗

Nosocomial acquisition of Pseudomonas aeruginosa by cystic fibrosis patients.

During a 4-year period, at least 12 of 40 patients with cystic fibrosis (CF) who were newly colonized with Pseudomonas aeruginosa had acquired it at CF recreation camps, clinics, or rehabilitation centers. After introduction of hygienic precautions at the CF clinic, only a single episode of nosocomial transmission of P. aeruginosa was detected at the CF ward during the subsequent 2 years.

Child↗

[Antibiotic inhalation in cystic fibrosis. A review of the literature].

Although antibiotic aerosols are widely used for the long-term treatment of chronic Pseudomonas aeruginosa infection in patients with cystic fibrosis, there are no clear therapeutic guidelines for the use of inhaled antibiotics due to the lack of large, well-controlled studies. This paper will review the current knowledge on the deposition and pharmacokinetics, the indication, clinical benefit and possible adverse reactions of inhaled antibiotics.

Administration, Inhalation↗

[Mucoviscidosis].

Explore the source record for details and available documents.

Anti-Bacterial Agents↗

Generation and metabolism of leukotrienes in granulocytes of patients with cystic fibrosis.

We studied the generation and metabolism of lipoxygenase products in peripheral granulocytes from children suffering from cystic fibrosis (CF). Peripheral granulocytes were stimulated at different times (days) before and during anti-infectious treatment with the Ca ionophore (7.5 microM, 5 and 20 min), opsonized zymosan (2 mg) and arachidonic acid (50 microM); the amount of lipoxygenase products in the cell supernatants was determined by high performance liquid chromatography. Granulocytes from patients with CF, compared to an age-matched control group, showed an increased omega-oxidation of the synthesized leukotriene (LTB4) into 20-OH- and 20-COOH-LTB4 after stimulation with the Ca ionophore (ratio of LTB4 versus omega-oxidated products in patients with CF: 0.77 +/- 0.07, mean +/- SEM, n = 11; control group: 1.07 +/- 0.1, n = 11, p less than 0.01) whereas the combined amounts of LTB4 and its omega-oxidated products did not differ significantly. A comparable profile was observed with opsonized zymosan. Stimulation of the cells with the Ca ionophore combined with arachidonic acid led to a significantly increased formation of lipoxygenase products in the patient group, whereas only a slight enhancement was observed in the control group. During the 14-day anti-infectious treatment a normalization of the altered pattern was observed. 12-Hydroxyeicosatetraenoic acid (12-HETE) production from platelets within the granulocyte fraction was significantly depressed in the CF group compared to the controls (38.5 +/- 12.5 versus 339 +/- 93 ng/5 +/- 10(6) cells, p less than 0.005). Within the CF group a strong correlation between the release of LTB4 and its metabolites, the production of 12-HETE and clinical (e.g. pO2, FEV1) and laboratory findings (e.g. IgE and IgG levels, C-reactive protein) was established. Our data suggest that the inflammatory process in patients with CF is associated with an alteration of the lipoxygenase pathway of granulocytes which correlates with the clinical signs of inflammation.

12-Hydroxy-5,8,10,14-eicosatetraenoic Acid↗

[Stabilization of lung function in cystic fibrosis during long-term tube feeding via a percutaneous endoscopic gastrostomy].

To determine whether long-term enteral feedings can improve nutritional status and lung function parameters in patients with cystic fibrosis (CF), 11 patients (8 female, 3 male, age 7 to 23 years) received a percutaneous endoscopic gastrostomy (PEG) since February 1988. All patients were moderately to severely affected and extremely malnourished with a relative underweight of 15% or more. After 9 months of supplemental nocturnal feeding, mean body weight improved from 25.8 +/- 4.7 kg (mean, standard deviation) to 31.6 +/- 7.4 kg (p = 0.003); mean relative underweight decreased from 20.7 +/- 3.4% to 10.4 +/- 8.6% (p = 0.02). Lung function, which was measured either prior to or without any intravenous antibiotics, improved considerably: mean vital capacity increased from 39.5% of the predicted value to 46.8% after 9 months (p = 0.01), the mean peak flow rate improved from 39.5% to 50.8% (p = 0.01). This improvement in lung function is probably secondary to the increased muscle mass of the patients. Long-term nocturnal feedings via a percutaneous gastrostomy tube improve nutritional status and lung function severely in malnourished patients with CF.

Adolescent↗

Increased resting energy expenditure in malnourished patients with cystic fibrosis.

Two recent studies (Shepherd 1988, Buchwals 1988) suggest that the malnutrition of CF may be associated with an energy-requiring defect leading to increased resting energy expenditure (REE). We investigated REE in relation to lean body mass (LBM) in 8 malnourished children with CF prior to the insertion of a gastrostomy tube. Two groups of age-matched (N1, n = 6) and weight-matched (N2, n = 5) healthy children served as controls. REE was measured by indirect calorimetry (Deltatrac Metabolic Monitor, Datex Instruments, Finland); FFM was determined by means of bioelectric impedance (BIA 103, RJL Systems, Detroit, U.S.A.). Malnourished patients with CF had higher expenditures than predicted by the Harris-Benedict equation (+32.7%). REE per kg LBM per day was markedly increased (+38.5%) compared to weight-matched controls. These results suggest that malnourished patients with CF have higher energy requirements than would be expected by the use of standard equations for caloric expenditure.

Basal Metabolism↗

[The effect of ambulatory care on the course of cystic fibrosis].

We analyzed the long-term follow-up of 97 patients with cystic fibrosis, who had been treated in our outpatient clinic for at least two years. Clinical, laboratory and lung function parameters were evaluated. At the first visit to the clinic, the patients were 0.1 to 25 years old (median: 4.6 years); CF had been diagnosed 2.9 years (median) earlier. Up to the age of 10 years, mean values for weight for height, vital capacity, pO2 and serum IgG were normal; however, during puberty the values declined into the pathological range. From the first to the second year of centralized treatment, the percentage of patients with underweight decreased from 42% to 31%. Abnormal IgG was found in 56% of patients during the first and in 42% of patients during the second year. No deterioration of the mean values was observed during the following five years, reflecting no progression of the disease. Centralized treatment of CF patients influences the course of this disease favourably. To permit the best possible prognosis for each patient, all children with CF should be transferred to a CF clinic soon after diagnosis has been established.

Adolescent↗

Long-term tobramycin aerosol therapy in cystic fibrosis.

The long-term efficacy and safety of aminoglycoside aerosol therapy for Pseudomonas aeruginosa colonization/infection in cystic fibrosis has not been fully investigated. In the present study, 14 patients with cystic fibrosis, ages 8-19 years (mean: 13.3 years), received tobramycin aerosol therapy for a mean duration of 20 months. Eighty milligrams of a tobramycin solution were inhaled twice daily after physiotherapy via a jet nebulizer. After 1 year, weight for height increased significantly by 2.9% of the predicted normal, and the Kraemer clinical score increased by 2.1 points (P less than 0.05). The frequency of hospital admissions decreased from 2.0 to 1.3 per patient, respectively, during the years before and after the study onset. The antibody response to P. aeruginosa elastase, exotoxin A, and alkaline phosphatase showed a reduction in serum titers against one or more enzymes in eight patients. The best long-term results after 12-38 months of treatment were obtained in moderately ill children. No evidence of ototoxicity or renal damage was observed. Although intermittent bacterial resistance occurred in five patients after 10-21 months of tobramycin inhalation, this was not associated with clinical deterioration. The study demonstrates the safety and clinical efficacy of long-term tobramycin aerosol therapy. Double-blind studies with larger patient cohorts are required to determine the value of aminoglycoside inhalation as an adjunct to the established therapeutic regimens.

Administration, Inhalation↗

Treatment of pseudomonas aeruginosa colonisation in cystic fibrosis.

To test whether early treatment could postpone the chronic colonisation of the respiratory tract with mucoid strains of Pseudomonas aeruginosa in patients with cystic fibrosis, we performed a pilot study in 28 patients aged 2 to 18 years. A two week course of azlocillin (150 mg/kg/day) and tobramycin (10 to 15 mg/kg/day) was given after a mean duration of P aeruginosa colonisation of five months (range one to 11 months). Weight for height increased significantly by 3.5% (SEM 0.7%) of the predicted normal after chemotherapy. The eradication of P aeruginosa that was achieved in 18 children directly after hospital treatment was only temporary. Samples from only 10 and five patients remained negative three and six months after treatment, respectively. Five children remained free from P aeruginosa for a prolonged period of 14 to 32 months. We conclude that, apart from the clinical improvement in all patients, some children might benefit from early antipseudomonas treatment with respect to the bacteriological outcome. Most children, however, experience only a temporary reduction in colonisation. Further investigations in form of controlled clinical trials seem justified.

Adolescent↗

Histamine release from basophils in cystic fibrosis.

We determined the histamine release from basophils in patients suffering from cystic fibrosis (CF) (median 17.2 years of age) and compared the data with an age-matched group of healthy donors. No significant differences in the basophil counts were determined between the CF and control groups. However, the absolute histamine content per basophil was elevated in the CF group (2.6 +/- 0.4 pg/basophil versus 1.4 +/- 0.2, mean +/- s.e.m., n = 15/10, P less than 0.004). Stimulation of basophils with the Ca ionophore (7.5 microM) and anti-IgE (10(-2) of a stock preparation) leads to a significantly higher release of histamine per basophil in CF patients as compared to healthy donors (Ca ionophore: 1.6 +/- 0.2 versus 0.9 +/- 0.2, mean +/- s.e.m., P less than 0.008; anti-IgE: 0.45 +/- 0.007 versus 0.28 +/- 0.04, P less than 0.02). These data indicate that basophils in CF may have a greater potential to release mediators although their releasability, expressed as the percentage of histamine release of the total histamine content, does not differ significantly compared to the healthy donors (62.1 +/- 8.3% versus 60.2 +/- 13%, mean +/- s.e.m.). Within the 14-day period of intravenous antibiotic treatment of the pulmonary infection (in 14 out of 15 cases P. aeruginosa was isolated from sputa samples) histamine release per basophil and total histamine content decreased to normal levels (day 1 2.6 +/- 0.3 versus 1.8 +/- 0.3, P less than 0.05). This decline was accompanied by an improvement of the clinical condition of the patient and reduction of P. aeruginosa isolates in sputa (n = 8). In contrast, in three patients with sustained P. aeruginosa colonization of the upper airways and impaired lung function histamine levels remained elevated. Our data demonstrate that the histamine content of basophils, as well as the release of histamine, is increased in patients with cystic fibrosis and correlates with the clinical signs of the chronic infection.

Adolescent↗

Pulmonary resection for localized bronchiectasis in cystic fibrosis. Report of three cases and review of the literature.

Three children with cystic fibrosis and localized bronchiectasis were treated by surgical removal of the affected lobe, after attempts to clear the obstructed bronchus by medical therapy and bronchoscopy with suction and lavage had been unsuccessful. Three to six years after surgery, the results are excellent. Pulmonary disease is mild in all patients, shown by chest radiographs and pulmonary function tests. A review of 102 cases reported in the literature demonstrates that pulmonary surgery is effective and can be performed safely in selected patients.

Bronchiectasis↗

Long-term tobramycin aerosol therapy of chronic Pseudomonas aeruginosa infection in patients with cystic fibrosis.

Fourteen cystic fibrosis patients (aged 9 to 20 years), chronically infected with Pseudomonas aeruginosa (PA), inhaled 80 mg (2 ml) tobramycin twice daily, because a significant clinical deterioration had occurred. Mean duration of aerosol therapy was 20.3 months (range, 11 to 38 months). There were no toxic side effects and no significant systemic absorption, 50 of 70 tobramycin serum levels being below the minimal detectable value of 0.1 mg/l. After 12 months of therapy there was an improvement of lung function, Po2, and weight. The frequency of intravenous therapy was reduced. Tobramycin resistance occurred in 5.8% of all PA strains after 10 to 29 months and did not represent a clinical problem. In a few patients PA was eradicated from sputum.

Administration, Inhalation↗

Renal function in cystic fibrosis: proteinuria and enzymuria before and after tobramycin therapy.

Proteinuria and enzymuria were measured in 27 patients with Cystic Fibrosis before and after tobramycin therapy. Prior to treatment, kidney function was normal in 23 patients. Four patients showed a pathological proteinuria and two haematuria. Renal biopsy in one patient showed segmental basement membrane alterations on electron microscopy; there were no immunoglobulin deposits. During intravenous therapy with tobramycin (10 mg/kg per day) and azlocillin (100 mg/kg per day) mean urinary N-acetyl-beta-D-glucosaminidase (NAG) excretion rose six-fold and mean urinary alaninaminopeptidase excretion increased ten-fold. After cessation of therapy, enzymuria rapidly returned to pretreatment values in all 14 patients. Aerosol tobramycin therapy in four patients did not affect urinary excretion of NAG. It can be concluded that tobramycin did not cause persistent renal damage in our patients, whether given intravenously or as an aerosol.

Acetylglucosaminidase↗