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Biomedical subjects

G Kusenbach

Publications and source records attributed to G Kusenbach.

At least 37 records · Page 2Linked to original sources
PubMed1991

[Long-term fungal culture of sputum from patients with cystic fibrosis].

Mycological examination of sputum from 121 patients with cystic fibrosis by means of long-term culture (4 weeks) revealed the occurrence of Candida albicans in low quantities in 70%, Aspergillus fumigatus in 30%, and Exophiala/Wangiella dermatitidis in 9% of the examined patients. A. fumigatus causes frequently the development of allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. The predisposing factors for colonization with the otherwise seldom recovered fungus E. dermatitidis in these patients and the consequences of these findings are discussed. In conclusion, long-term fungal cultures are advocated for specimens from CF patients.

Adolescent↗
PubMed1990

Histamine release test in comparison to standard tests in diagnosis of childhood allergic asthma.

An allergen-specific histamine release test (HR) was investigated in 93 children with a history of extrinsic asthma and suspected allergy to house dust mite or fungi. For both allergy groups the correlations between HR and the bronchial provocation test were better than those between the inhalation test and prick test or RAST. It is concluded that, especially in childhood where performance of a bronchial provocation should be avoided because of its potential risk and required hospitalization, the HR test can represent a significant diagnostic aid.

Adolescent↗
PubMed1989

The adrenergic system in lymphocytes from children with cystic fibrosis.

Several in vivo and in vitro studies have suggested that children suffering from cystic fibrosis (CF) might have a general defect of beta-adrenoceptors on the cell surface which might account for an unbalanced secretory process. In order to investigate if this view holds true, we determined the beta-adrenoceptor density and affinity on lymphocytes by means of radioligand studies using 125-iodo-cyano-pindolol (125-ICYP) in 20 children with CF. Cyclic AMP (cAMP) response was also investigated after specific beta-adrenoceptor stimulation with isoprenaline (IPN) and after direct stimulation of the adenylate cyclase with forskolin in lymphocytes. Children with CF and controls have identical numbers and affinities of beta-adrenoceptors on lymphocytes. The cyclic AMP response was identical in CF- and in age-matched control children regardless whether adenylate cyclase was stimulated directly or via beta-adrenoceptors. In conclusion, the data support the view that no general adrenoceptor or adenylate cyclase defect exists in CF. As several studies have found abnormal reactions to adrenergic stimuli in CF patients, we presume that there is a defect beyond the level of adrenergic receptors and cAMP which remains to be identified.

Adenylyl Cyclases↗
PubMed1989

[Surgical correction of bronchial stenosis: increased somatic development following elimination of the stenosis].

A 5 year old girl suffered from nearly total stenosis of the bronchus intermedius and exspiratory collaps of both main bronchi. Because of preterm birth she was postnatally intubated and mechanically ventilated for 6 weeks. Her somatic development was significantly retarded in comparison with the monozygotic twin, and pulmonary impairment increased again in the last preoperative year. By the combination of latest surgical technics (pericardial patch, interposition of a costal segment, fibrine glue) the bronchial abnormalities could be corrected successfully. In the first year after surgery catch-up growth was observed in the girl.

Aprotinin↗
PubMed1988

Glucocorticoid receptors in mononuclear blood cells and their correlation to endogenous and exogenous corticoids in healthy and asthmatic children.

The number and affinity of glucocorticoid binding sites in peripheral mononuclear cells (MNC) of asthmatic and healthy children were determined by a whole cell (3H)dexamethasone binding assay at 37 degrees C. Using HPLC determination, corresponding serum levels of non-protein-bound (free) cortisol, whole cortisol and cortisone as well as urine excretion of free cortisone and cortisol were assessed. The average number of binding sites (BS) per cell and the dissociation constant (KD) respectively, in atopic asthmatics (7768 +/- 666 BS/MNC resp. KD = 17.2 +/- 2 nM) did not differ from the values measured in our control group (8333 +/- 691 BS/MNC resp. 25.4 +/- 4.8 nM). Within the age range 1 month-15.8 years neither age-dependent changes nor sex-related differences in the number of binding sites or the KD values could be detected. Active or currently inactive asthmatics, and patients under different antiasthmatic drug regimes, had similar binding sites on MNC. No differences in serum levels of cortisol, cortisone and free cortisol or in free cortisol and free cortisone of 24-h urine samples were found between healthy children and asthmatics. After a short course of prednisolone therapy for an acute severe asthmatic attack the number of glucocorticoid binding sites in peripheral MNC decreased to an average of 4632 +/- 421 BS/MNC, whereas the dissociation constant did not change significantly (14.5 +/- 3.6 nM). The corticoid-hormone pattern in the serum, 24-h urine excretion, and the normal number and affinity of glucocorticoid receptors on peripheral MNC suggest that there is no primary, general impairment of glucocorticoid metabolism in asthmatic children.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

Effects of the antiallergic drug ketotifen on bronchial resistance and beta-adrenoceptor density of lymphocytes in children with exercise-induced asthma.

In a parallel, double-blind, placebo-controlled study we investigated the effect of the antiallergic drug Ketotifen on bronchial resistance (determined body plethysmographically) and beta 2-adrenoceptor density of lymphocytes (determined by 125-iodo-cyano-pindolol binding) in asthmatic children suffering from exercise-induced asthma (EIA). In order to provoke EIA a test which involved running for 7 min was performed with 22 asthmatic children. 13 age-matched children not suffering from asthma served as controls. Control children showed a 30% increase in the number but not in the affinity of beta-adrenoceptors in response to a 7-min run. Asthmatic children with EIA showed no difference in the number and affinity of beta-adrenoceptors under resting conditions when compared with the controls. In contrast to what has been observed with control children, however, no increase in the number of beta-adrenoceptors in response to exercise was observed. A 1-week treatment with placebo neither affected the bronchoconstriction nor the number of lymphocyte beta 2-adrenoceptors. Ketotifen (2 X 1 mg/day for 1 week) protected asthmatic children from EIA, at least in part, and restored the up-regulation of beta-adrenoceptors in response to exercise in 9 of 14 children with EIA. However, no correlation between the serum concentration of Ketotifen and the number of binding sites could be found. It is therefore concluded that the preventive effect of Ketotifen in EIA might also involve mechanisms other than a recovery of the beta-adrenergic system.

Adolescent↗
PubMed1987

Steady state pharmacokinetics, metabolism and pharmacodynamics of theophylline in children after unequal twice-daily dosing of a new sustained-release formulation.

This was an open-label study in 19 children aged 9-13 years, weighing 27-44 kg, with bronchial asthma. Twenty-four-hour steady-state concentrations of theophylline and its metabolites 1,3-dimethyl uric acid, 3-methyl xanthine and 1-methyl uric acid were assessed after daily dosing of 600 mg (ca 18 mg/kg/day) of the sustained-release theophylline micro-pellet sprinkle system BY158K, for 4 days. The dosing regimen used was an unequal twice-daily dose of 200 mg in the morning after breakfast and 400 mg in the evening after dinner. Twenty-four-hour peak expiratory flow (PEF) profiles were compared before treatment and at steady-state, along with lung function parameters after bronchial provocation. Mean values +/- SD (n = 16) of the steady-state characteristics were Cmin 6.8 +/- 2.1 mg/l, Cmax 14.5 +/- 4.8 mg/l and Cav 10.5 +/- 2.9 mg/l, the plateau time was 11.7 +/- 4.8 hr and peak-trough fluctuation and swing were 72 +/- 21 and 118 +/- 52%, respectively. There was an excellent reproducibility of theophylline pre-dose levels at corresponding time points of the 24-hr sampling period [r = 0.864 (p less than 0.001)]. Mean values +/- SD of the 24 hr average serum metabolite levels were 0.9 +/- 0.2 mg/1 for 1,3-dimethyl uric acid, 0.6 +/- 0.1 mg/1 for 3-methyl xanthine and 0.4 +/- 0.1 mg/1 for l-methyl uric acid. Lung function (n = 17) following bronchial provocation, improved in 10 children after theophylline treatment of 4 days, remained stable in 2 patients and deteriorated in 5 patients. Serum theophylline profiles and PEF profiles ran largely in parallel over the 24-hr period. Six children exhibited typical theophylline induced side-effects, headache (n = 3), nausea (n = 4), dizziness (n = 1), vomiting (n = 4), sleep disturbances (n = 1), pallor (n = 1) and tremor (n = 1), necessitating in 3 children one dose omission/reduction (n = 2) or subsequent dose reduction (n = 1). It has been shown that a twice daily dosing regimen with unequal doses of anhydrous theophylline (BY158K) is well suited to this population of fast metabolisers. The patients were well protected throughout the day, including the critical early morning hours.

Asthma↗
PubMed1986

[Postnatal development of the sympathoadrenergic system in premature and newborn infants].

Autonomic regulatory mechanisms and some metabolic functions are predominantly influenced by the sympathetic nervous system. Premature and mature newborns show a high variability and a low adaptability of the sympathetic nervous system. In order to investigate whether sympathetic systems are completely developed at birth or underly a postnatal maturation process we have determined plasma levels of adrenaline and noradrenaline as well as the density and affinity of alpha- and beta-adrenoceptors on thrombocytes and lymphocytes in pre- and mature newborns and adults. Catecholamines were determined by means of a radioenzymatic method, number and affinity of adrenoceptors by use of the radioactive labelled antagonists 3-H-Yohimbine and 125-I-Cyano-Pindolol. Beta-adrenoceptor responsiveness was assessed by measurement of cyclic AMP in lymphocytes before and after stimulation of beta-adrenoceptors by isoprenaline. A linear relationship occurred between the gestational age and the number of adrenoceptors on lymphocytes, whereas the alpha-adrenoceptors on thrombocytes showed no age dependency. The basal content of cyclic AMP and the accumulation in response to beta-adrenoceptor stimulation by isoprenaline was significantly lower in newborns than in adults. Since noradrenaline and adrenaline plasma levels were not significantly different in newborns and adults it is assumed that the low density of beta-adrenoceptors in premature and mature newborns is due to a postnatal maturation and not to a "down regulation" by circulating catecholamines. Our results suggest that an immaturity of beta-adrenoceptors is involved in the poorly developed adaptive control in newborns.

Adult↗
PubMed1986

[Defects of IgG subclasses as a cause of severe, recurrent respiratory tract infection].

IgG Immunoglobulins can be differentiated into four subclasses with different structures and functions. Partial or complete defects of one or two subclasses can be related to an impaired immune defence. We describe four children with severe recurrent bacterial airway infections. Two children had developed bronchiectasia following recurrent bronchopulmonary inflammation. Prior to diagnosis of IgG subclass deficiency other common causes of recurrent airway infections were excluded. Defects of IgG 2 or IgG 4 antibodies as well as of both classes were found with compensatory elevation of IgG 1 and IgG 3. In repeated sputum cultures haemophilus influenzae and staphylococcus aureus were isolated. This might be due to an impaired antibody production against special antigens as alpha-toxin of staphylococcus or capsular polysaccharide of haemophilus influenzae. The four cases demonstrate that in children with severe recurrent airway infections including bronchiectasia and otitis media defects of IgG subclasses have to be considered. Diagnosis should be proved by repeated determinations of blood levels after exclusion of other common causes for infections. Diminution of IgG subclasses without clinical symptoms of airway infections is also possible. If diagnosis seems to be certain intravenous substitution with 7 s gammaglobulin beside symptomatic antibiotic therapy is recommended.

Child↗
PubMed1986

[Peculiarities of drug therapy in childhood].

The age of the patient is one of the major determinants of drug efficacy due to certain anatomical, physiological, biochemical and behavioral characteristics of different age groups of the pediatric population. Qualitative and quantitative differences in pharmacokinetics and pharmacodynamics of drugs should be considered before dosage regimens can be established. Differences may occur in each age group, but may be even greater in newborns. Nevertheless, the maturation from the newborn towards adulthood proceeds through a continuum and thus involves also other age groups. Pediatricians are aware of this situation. But only for a small fraction of drugs used in newborns and infants clinical-pharmacological data are available, and drug instructions often contain disclaimer statements against the use in children. In pediatric intensive care units newborns and infants often receive drugs which have not been evaluated before within this age group. According to epidemiological data it has to be assumed that 30% of neonates in neonatal care units develop adverse side effects, which may originate from the drug itself or from drug interactions. Drug trials are completed or underway when new approaches to therapeutic interventions are developed for pediatric disease states. Examples for the development of new therapeutic interventions are for instance the use of theophylline for the prevention of apnoes in prematures, the use of indomethacin for the closure of a patent ductus arteriosus, or the specific advances of cancer chemotherapy in childhood. However, there is no doubt that a complete interplay between clinical, ethical and legal aspects renders the task of studying the effects of drugs in children more difficult than in the case of adults.

Apnea↗
PubMedDate not supplied

[Taurine supplementation in cystic fibrosis (CF): effect on vitamin E absorption kinetics].

Oral vitamin E (Vit.E) bioavailability is reduced in CF patients especially in case of malnourishment. Both exocrine pancreatic insufficiency and an altered bile acid composition showing an elevated glycine taurine ratio of conjugated bile acids which is due to excessive loss of bile acids in the stools may contribute to this observation. Because taurine supplementation reduces the glycine/taurine ratio of bile acids in duodenal juice of CF-patients it was the objective of this study to evaluate the effect of taurine supplementation on Vit.E absorption kinetics. Oral Vit.E tolerance tests (50 mg/kg) were performed before and after 3 months of taurine supplementation (30 mg/kg/day) in 11 CF patients (ages 7 to 22 years) under fasting conditions. Bodyweight and or weight for height of all patients were below the 25th percentile. Doses of all medications except antibiotics were kept unchanged during the study. Any additional Vit.E supplementation was stopped 14 days prior to each test. Serum Vit.E levels were measured over a 24 hour period. Determination of serum Vit.E concentrations was performed with a HPLC fluorescence technique. The glycine/taurine ratio in serum served as compliance parameter and dropped in all but one patients. Baseline Vit.E concentrations and serum Vit.E/total lipids ratios in serum considered as parameters of the Vit.E status increased significantly. Both the maximal Vit.E concentrations in serum and the areas under the oral absorption curves showed a significant increase with taurine supplementation. This study shows that the Vit.E status of malnourished CF patients can be improved with taurine supplementation due to improved Vit.E absorption kinetics.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗
PubMedDate not supplied

[Tuberculosis and cystic fibrosis].

Mycobacterial infections are rarely reported in Cystic Fibrosis patients although they quite often develop predisposing risk factors such as underweight, secondary diabetes mellitus and chronic inflammatory pulmonary disease. Furthermore glucocorticoid therapy is mandatory in some patients. CF heterozygotes are said to have a selective advantage due to an increased host resistance against Mycobacterium tuberculosis. In this survey 1926 CF patients were investigated for the incidence of tuberculin conversion and manifest infection with mycobacterium tuberculosis in the Federal Republic of Germany (FRG). The results do not support the hypothesis of increased host resistance nor do they show any evidence of a higher risk for tuberculosis in CF. Implications for prophylactic, diagnostic and therapeutic measures are discussed in accordance to the recent epidemiologic data of tuberculosis in the FRG.

Adolescent↗
PubMedDate not supplied

[Intracranial arterial aneurysm in children].

Over a period of 13 years 5 children aged 8 to 11 were operated on intracranial arterial aneurysms. Special features of these aneurysms in children were accidents in the anamnesis of three children and one localization at the internal carotid artery bifurcation and another one at a peripheral artery. It is unusual to have fusiform dilatations at the basis of two aneurysms and one large aneurysm among 5 cases in comparison to aneurysms in adults. This hints to the possibility of more extended artery lesions with aneurysms in children. The intracranial hemorrhage which often leads to a diagnosis makes an immediate neuroradiological check necessary. Hemorrhage or operation were survived by all children. Space occupying hematomas diminish the quality of outcome considerably and therefore must be operated on immediately.

Carotid Artery, Internal↗
PubMedDate not supplied

[Effect of nutritional status on absorption kinetics of vitamin E in mucoviscidosis].

Exocrine pancreatic insufficiency with varying severity must be anticipated in 85-95% of CF patients. It leads to fat maldigestion and malabsorption of the liposoluble vitamins - A, D, E, K - and fecal loss of fat. In general, supplementation with fat-soluble vitamins is recommended in CF patients. In this study an oral vitamin E tolerance test (100 mg/kg bodyweight) was performed in 5 healthy adult volunteers to elaborate a vitamin E absorption kinetics and additionally in CF patients on medication with pancreatic enzymes to evaluate the absorption of vitamin E. 19 CF patients (ages 4 to 19 years) were studied after cessation of any additional vitamin E supplementation for 7 days. Vitamin E serum concentrations were sampled over a 72 hour period. Serum vitamin E determinations were performed with a HPLC-fluorescence technique. The kinetics of Vitamin E in healthy volunteers can be described with an open 2 compartment model. CF patients revealed consistently an altered kinetics of absorption of vitamin E, which was not compatible with this model. Baseline (c*) and maximal serum concentrations (cmax) of vitamin E as well as the area outer the oral absorption curve (AUC) correlated with the nutritional status expressed by bodyweight percentiles in CF patients. The results of this study show that in normal weight CF patients on pancreatic enzymes medication vitamin E depletion is unlikely.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗
PubMedDate not supplied

[Myelosarcoma as the primary manifestation of acute myeloid leukemia].

Myelosarcoma ("Granulocytic sarcoma", "Chloroma") is an extramedullary tumor composed of granulocytic precursor cells and related to myelogenous leukemia. If the tumor precedes acute leukemia diagnosis is difficult and requires special diagnostic techniques. This is documented by the presented 7.5 years old girl with primary myelosarcoma. In spite of early and intensive chemotherapy and radiation the sarcoma soon was followed by acute myelogenous leukemia with skin infiltrations. Cytogenetic classifications may in future lead to the development of a differentiated therapy of myelosarcoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

[Value of the basophil stimulation test in the diagnosis of juvenile bronchial asthma].

The diagnostic value of histamine liberation from basophil granulocytes (BST) was investigated in asthmatic children with house dust, house dust mite or moulds allergy. BST corresponded at about 80% to skin prick test (PRICK) and radio-allergo-sorbens test (RAST) in 178 patients. The relevance of the results of BST, PRICK and RAST for the asthmatic symptoms was proved by bronchial provocation in 77 children. BST corresponded clearly better to the inhalation test than PRICK and RAST. Correspondence of BST in house dust mite allergy amounted to 94%, in house dust allergy to 100%, and in moulds allergy to 77%. Overall correspondence of BST was 92%. Even when RAST and PRICK showed different results correspondence of BST attained 91%. In cases with identical results of BST, PRICK and RAST the bronchial provocation test corresponded in 98%. It is concluded, that if history fits to BST, RAST and PRICK bronchial provocation is no longer essential for the diagnosis of relevant allergens in perennial childhood asthma. This seems to hold also in cases of house dust or house dust mite allergy, where the result of PRICK or RAST is different. Especially in childhood the use of BST in the diagnostic procedure provides the following advantages: risks of bronchial provocation and the necessary hospitalisation can be avoided, cooperation of the patient is not needed, and costs can be reduced.

Allergens↗
PubMedDate not supplied

[Myeloperoxidase deficiency as a cause of recurrent infections].

Myeloperoxidase (MPO) deficiency is a common hereditary leukocyte function defect. A two year old girl with MPO-deficiency suffered from recurrent skin infections. No MPO-activity was detectable in leukocytes of her peripheral blood smears, while NBT reduction and chemotactic activity was normal. The quantitative enzyme determination in leukocyte sonicates confirmed the total MPO-deficiency in the girl's leukocytes and a partial MPO-deficiency in the cells of her mother. The patient leukocytes demonstrated also an impaired chemiluminescence.

Chemotaxis, Leukocyte↗