Late-onset Epstein-Barr virus (EBV)-negative extranodal B-precursor lymphoblastic lymphoma of donor origin after hematopoietic stem cell transplantation (HSCT).
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Biomedical subjects
Publications and source records attributed to E König.
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PURPOSE: This is a prospective analysis of the value of MRI in suspected inner ear malformations. MATERIALS AND METHODS: In 50 patients (43 children and young adults, 7 adults) with suspected inner ear malformation MRI (1.5 T) was performed. In addition, 42 of these patients underwent CT. For the analysis of the inner ear structures, the constructive interference in steady state (CISS) sequence with 0.7 mm slice thickness was used. Functional tests revealed a sensorineural hearing loss or deafness in 82 temporal bones (TB) and a combined hearing loss in 4 TB. The hearing loss was unilateral in 14 patients. MRI and CT findings were compared. RESULTS: Imaging findings were normal in 58 TB. The pathological findings included inner ear malformations (35 TB), inflammatory changes (4 TB), partial obliteration of labyrinth (2 TB) and congenital aural atresia (1 TB). An isolated absence of the cochlear nerve (1 TB) could only be found by MRI. In the remaining cases, an inner ear malformation was diagnosed by MRI and CT with the same confidence but MRI was superior in displaying the fine details. CONCLUSIONS: MRI will become the method of choice in the diagnosis of inner ear malformations.
OBJECTIVE: The aim of this study was to determine the dose-limiting toxicities (DLTs) and maximum tolerated doses (MTDs) of a docetaxel-carboplatin regimen in patients with locally advanced cervical cancer (LACC) or recurrent cervical cancer. The regimen was administered weekly, with a maximum of 12 courses. PATIENTS AND METHODS: Twenty patients were treated with with a total of 145 cycles of weekly carboplatin and docetaxel. The starting dose of docetaxel was 25 mg/m(2) with increments of 5 mg/m(2) until a final dose of 35 mg/m(2) was reached. Dose-escalation of docetaxel was followed by carboplatin at AUC 2, AUC 2.5, and AUC 3, respectively. Defined dose-limiting toxicities were WHO grade (G) 3 hematotoxicity, G4 mucositis, and G2 neurotoxicity. The response status of the patients was assessed using the common ECOG response criteria. RESULTS: Two of four patients developed a DLT at dose level 4. Nonhematological toxicity was generally mild, except for ubiquitous complete alopecia. The MTD was reached at docetaxel 35 mg/m(2) and carboplatin AUC 2 mg/mL.min. The overall response rate was 65% in the entire group of evaluable patients and 77% in patients with primary LACC, with two cases of pathological complete response. CONCLUSION: This dose-dense regimen was well-tolerated and could be administered on an outpatient basis.
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OBJECTIVE: Studying gender differences in fat mass and distribution in a homogeneous group of children. DESIGN: Cross-sectional study. SUBJECTS: 610 children aged 5-7 y in Kiel, Germany. METHODS: Anthropometric measures, bioelectrical impedance analysis (BIA). RESULTS: Although boys had increased body weights (P<0.05), body mass indexes (BMI's) (P<0.001) and waist/hip ratios (WHRs) (P<0.001), the %fat mass as assessed by BIA (P<0.05) was increased in girls. Although the increased BMI in boys was independent of the percentile used, gender differences (that is, lower values for boys than for girls at the same age) in WHR, the sum of four skinfolds and %fat were seen up to the 90th percentile. By contrast, above the 90th percentile there were no differences in skinfold thickness and %fat between boys and girls. Studying 42 BMI-matched pairs (boys and girls) also showed that the %fat estimated by BIA (P<0.001) was increased in girls. Plotting the average of %fat as obtained from skinfold- and BAI-measurements against the difference between data obtained by the use of the two methods shows that BIA %fat overestimates skinfold %fat at low or normal percent fat mass (that is, up to 20%) in both genders. By contrast, at increased fat mass, BIA %fat seems to underestimate skinfold %fat in both genders. CONCLUSIONS: Gender differences in fat mass and fat distribution are obvious in children aged 5-7 y. These differences are independent of gender differences in body weight. However, the nutritional state has an influence and gender differences cannot be detected in overweight and obese children. Our data also suggest that a children-specific formula used to calculate %fat from skinfold measurements is inappropriate.
A prospective, randomized, double-blind and placebo-controlled study was conducted to assess the effectiveness of i.v. administration of 6% hydroxyethyle starch solution (HES) in preventing moderate and severe ovarian hyperstimulation syndrome (OHSS) in patients in an in-vitro fertilization programme. A total of 101 women who had serum oestradiol concentrations >1500 pg/ml and/or more than 10 follicles on day of human chorionic gonadotrophin (HCG) administration were recruited into two groups: HES group (n = 51) received 1000 ml 6% HES; and the placebo group (n = 50) received 1000 ml of sodium chloride 0.9% solution at the time shortly after embryo transfer. Follow-up examinations 7 +/- 1 and 14 +/- 1 days after embryo transfer included transvaginal ultrasound (diameters of each ovary and maximum cysts, number of cysts, ascites), blood tests (serum oestradiol, progesterone, beta-HCG, C-reactive protein, blood count, plasma proteins, electrolytes, kidney function tests) and evaluation of abdominal pain, nausea, diarrhoea, abdominal swelling and weight gain. Only one moderate OHSS developed in the HES group whereas seven moderate-severe cases were observed in the placebo group (P = 0.031). Furthermore, serum oestradiol concentration, leukocyte count, increase in abdominal circumference and weight gain 14 days after embryo transfer were significantly higher in the placebo group. There were no differences between the two groups in terms of age, oestradiol concentration and number of follicles at time of HCG injection. Administration of 6% HES prevents the development of moderate-severe OHSS in risk patients.
PURPOSE: To demonstrate HRCT findings and their therapeutic relevance in suspected congenital hearing disorders. MATERIAL AND METHODS: It was checked in 96 young patients if HRCT findings of the temporal bone could explain functional findings. Furthermore, the therapeutic consequences were noted. RESULTS: Normal CT and normal functional findings were obtained in 49 temporal bones (TB). In conductive hearing loss (41 TB), dysplasias of the conducting apparatus (37 TB) and inflammatory changes (3 TB) were found. Combined hearing loss (18 TB) was clarified completely or partially in half the cases. There were 22 dysplasias of the inner ear, 3 dysplasias of the middle ear, 1 abandoned examination (2 TB), and 55 normal CT findings in senorineural hearing disorders (82 TB). 1 retardate had a malformation of the inner ear and, contralaterally, inflammatory middle ear. In cases of vestibular disorders (24 TB), 14 malformations of the inner ear were detected. An indication for an operation was given in 23 TB. In 22 TB, it was contraindicated. The CT was one preliminary examination to a cochlea implant in 19 patients. The therapy was carried on with hearing devices in the other patients. CONCLUSION: HRCT is an important method in diagnosis and therapeutic planning of suspected malformations of the temporal bone.
The duration of action and the pharmacokinetics of gliquidone (1-cyclohexyl-3-[[4-[2-(3,4-dihydro-7-methoxy-4,4-dimethyl-1, 3-dioxo-2(1H)-isochinolyl)ethyl]phenyl]-sulfonyl]-urea, AR-DF 26 SE, CAS 33342-05-1, Glurenorm, Beglynor) were investigated in 32 patients with non-insulin-dependent (type 2) diabetes mellitus over 16 h. In a single-blinded cross-over design vs. placebo, one 30 mg tablet gliquidone was administered 15 min before breakfast. Concomitant to the measurement of glucose and insulin, the gliquidone plasma levels of 20 subjects were determined by a new specific liquid chromatographic (HPLC) assay method with fluorescence detection, and the pharmacokinetic parameters calculated. Following the gliquidone administration, the mean plasma glucose profiles of the responders were up to 15% lower than with placebo (p < 0.005) between 8 a.m. and 6 p.m., representing a duration of the blood sugar-lowering effect of 8 to 10 h. Insulin values were raised, with peaks over 40% higher, during or shortly after meals. Subsequently, the insulin levels returned to approximately the same levels obtained with placebo during the postprandial phase. Plasma concentrations of gliquidone showed pronounced interindividual variability. The mean maximum concentration in plasma Cmax was 0.65 microgram/ml, (range: 0.12-2.14 micrograms/ml, coefficient of variation (CV): 82%). The median time to reach maximum plasma concentrations tmax was 2.25 h (range: 1.25-4.75 h). The areas under the plasma concentration-time curve from zero time to infinity (AUC0-infinity) and the mean terminal elimination half-lives (t1/2 beta) were computed from those patients (N = 8) who exhibited at least five plasma levels above the limit of quantitation in the terminal log-linear phase using a two-compartment model: the mean AUC0-infinity was 5.1 micrograms.h/ml (range: 1.5-10.1 micrograms.h/ml, CV 56%). The dominant half-life t1/2 alpha derived from therapeutically relevant plasma levels of gliquidone (> 80 ng/ml) was approximately 1.2 h (range: 0.4-3.0 h. CV: 71%) and the mean terminal half-life t1/2 beta was approximately 8 h (range: 5.7-9.4 h, CV: 17%). From the pharmacodynamic behavior as well as from the pharmacokinetic parameters it can be deduced that gliquidone belongs to the class of short-acting sulfonylureas used in antidiabetic therapy.
Klippel-Feil syndrome is characterized by a short-neck and a low occipital hairline due to such deformaties of the cervical spine as aplasia, dysplasia and fusions of the cervical and thoracic vertebrae. Approximately 1/3 of cases have sensorineural deafness, although occasional unilateral or bilateral conductive hearing loss due to middle ear malformations have been described. We have now treated two cases with unilateral middle ear malformations in patients with known Klippel-Feil syndrome. In the first case a completely malformed stapes was found as well as an atypical course of the facial nerve, which was found to be exactly over an absent oval window. In the second case aplasia of the stapes was seen and the oval and round windows were absent. No epitympanum was identifiable and a compact mastoid was found. In addition to audiometry, high-resolution CT was indispensable in diagnosis. However, surgical procedures in cases with unilateral middle ear deformations should be performed only on explicit request of the patient and not before the age of 16.
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Disability statistics recorded so far within the framework of public health services concerning paediatric diseases and diseases of adolescents in the North German Federal Land of Sleswick-Holstein are deficient in many respects. Classification according to individual kinds of disability is lacking in clarity; statistics are not representative of the entire population; they are unsuitable for assessing the performance of public health services; it is by no means evident in what manner the data have been compiled and why; no differentiated information is given on the large group of persons threatened with disability. The article discusses various aspects of classifying disabled persons. It is not clear whether reasonable statistics on this subject will be compiled in future. Paediatricians and doctors in care of adolescents are mainly concerned with advisory and expertising activities in respect of the individual patient.
More than one third of all sensorineural hearing loss and deafness has a genetic basis. Syndromes are already clinically distinguishable from monosymptomatic forms, but even the latter represent a heterogeneous group of diseases, which differ in mode of transmission, audiometric features of the hearing loss and possible progredience of the symptoms. Till now only audiometric and genealogical methods have been available for diagnosis. So far serological and cytogenetical studies have given no results and molecular-genetic investigations have only given results for some very rare syndromes. To help affected families and to prepare molecular-genetic studies we examined all patients at our hospital who suffer from a sensorineural hearing impairment for the onset of a monogeneous mode of transmission. Altogether we could find 39 stricken kins, 15 of them with autosomal-dominant, 16 with autosomal-recessive and 8 with an unclear mode of transmission. We registered anamnestically 1548 persons, from these 171 were suffering from hereditary hearing impairment. We were able to examine 117 of the affected persons. Seven typical audiometric features could be found. Including mode of transmission and possible progredience even 17 forms can be found. This represents a higher number of monosymptomatic forms of hereditary sensorineural hearing loss than described in the literature. It emphasizes that the main obstacle hindering future genotypical diagnosis will be the extreme high heterogenity.
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The genes encoding procyclin, the major glycoprotein expressed on the surface of procyclic forms of Trypanosoma brucei, comprise a multigene family. It has previously been demonstrated that procyclin genes in cloned trypanosome strains from Kenya and Uganda show restriction fragment polymorphisms. A detailed study of the Kenyan strain 227 has revealed that procyclin genes are arranged in tandem at 3 distinct loci (Pro A, B and C) and that the polymorphism is due to the duplication of 1.3 kb in the Pro A locus, which has generated an additional procyclin gene. Northern blot analysis has shown that at least 2 loci are transcribed and that a minimum of 3 procyclin genes are expressed within a cloned line. The transcription of procyclin genes is resistant to 1 mg ml-1 alpha-amanitin, whereas that of the 5' flanking gene in the Pro A locus is sensitive. This observation suggests that the two genes form part of separate transcription units with a promoter between them.
Glyceryl trinitrate (GTN) induced relaxation was investigated in preparations of corresponding femoral arteries and veins from rabbits containing the intact endothelium in comparison to denuded vessels. Either vascular rings (app. 5 mm segments) or helical cut strips were isotonically mounted in organbaths (37 degrees C, Krebs-Henseleit-buffer). Vessels were precontracted with their specific EC-50 of norepinephrine. Cumulative concentration response curves for GTN were evaluated. Arteries and veins were dilated in a concentration dependent manner in the range of 1 nmol/l to 0.1 mmol/l. V.femoralis was the most sensitive vessel with an EC-50 of 10 nmol/l. There was no significant difference in the sensitivity of veins with or without endothelium. In contrast to this, for half-maximal relaxation of A. femoralis 2-3 orders of magnitude higher concentrations were necessary, depending on the presence of endothelium. The concentration ratio artery/vein (A/V) amounted to 800 in normal vessels, 100 in denuded vessels, resp. indicating that for half-maximal relaxation of endothelium containing arteries significant higher concentrations were needed. By preincubation for 60 min with the EC-90 of GTN in arteries, as well as in veins, tolerance could be induced independently of the presence of endothelium. However, veins were more affected than arteries, but tolerant veins were still more sensitive to GTN than tolerant arteries. This was true both for preparations with and without endothelium (A/V = 70 in normal vessels, 20 in denuded vessels, resp.). Moreover, the endothelial dependence of arterial relaxation was attenuated. Treatment of the tolerant vessels with cystein (1 mmol/l) did abolish the GTN-tolerance in veins but not in arteries.(ABSTRACT TRUNCATED AT 250 WORDS)