[Johannes Kathe, 1880-1965].
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Bacteriology↗
Biomedical subjects
W Köhler
Publications and source records attributed to W Köhler.
[Epidemiologic and immunologic considerations of the current measles situation].
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Child↗
[Antibacterial spectrum of nonactin with special consideration of Corynebacteriaceae].
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Anti-Bacterial Agents↗
X-linked adrenomyeloneuropathy associated with 14 novel ALD-gene mutations: no correlation between type of mutation and age of onset.
Adrenomyeloneuropathy (AMN) represents a milder form of X-linked adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder. The disease is characterised by an abnormal accumulation of saturated, very long chain, fatty acids, because of altered peroxisomal beta-oxidation that concomitantly leads to demyelination in the central and peripheral nervous systems. ALD shows a highly variable phenotypic expression and extensive mutation analysis in ALD patients has failed to establish a genotype-phenotype correlation, even in the presence of the same ALD-gene defect. Therefore, we have looked for a relationship between the molecular lesion and the age of onset in 19 patients with a well-classified clinical course of AMN. The nearly complete novel spectrum of ALD gene mutations identified has revealed no obvious correlation between the type of mutation and age of AMN onset in this small series. However, intrafamiliar concordance could be observed with respect to the occurrence of adrenocortical insufficiency. This supports the idea of one (or more) additional gene(s) contributing to the phenotypic expression of ALD.
ATP Binding Cassette Transporter, Subfamily D, Mem↗
[7-week-old infant with a hydrolethalus syndrome: case report, differential diagnosis and literature review].
We report on an 7 weeks old male infant with hydrocephalus internus, dysplastic low-set ears, micrognathia, vitium cordis, hypogenitalism, and polyhydramnios in pregnancy. The variability of the Hydrolethalus syndrome will be demonstrated. Our patient shows, that this syndrome is not a lethal in all cases. We report on an 7 weeks old male infant with hydrocephalus internus, dysplastic low-set ears, micrognathia, vitium cordis, hypogenitalism, and polyhydramnios in pregnancy.
Abnormalities, Multiple↗
[Continuous doxorubicin infusions. A pilot study in young patients].
Eleven young patients with relapsed childhood malignancies received continuous infusions of 5-10 mg/m2/day doxorubicin. 30 infusions were given via a central venous access until signs of mucositis showed up (7-52 days). Other toxicity was low, both bone marrow depression and fever and neutropenia occurred in 7/30 and 5/30 courses of therapy, respectively. No signs of liver or cardiac toxicity were seen in these heavily pretreated patients. In 8/11 patients tumor regression was documented, though only two partial remission were noted. Continuous infusions of doxorubicin are an effective and relatively non-toxic treatment for relapsed childhood malignancies. Since this form of therapy enables good quality of life and medical care on ambulatory basis, a phase II-study of low-dose doxorubicin for relapsed tumors is justified.
Adolescent↗
Velocity and acceleration of height growth using kernel estimation.
A method is introduced for estimating acceleration, velocity and distance of longitudinal growth curves and it is illustrated by analysing human height growth. This approach, called kernel estimation, belongs to the class of smoothing methods and does not assume an a priori fixed functional model, and not even that one and the same model is applicable for all children. The examples presented show that acceleration curves might allow a better quantification of the mid-growth spurt (MS) and a more differentiated analysis of the pubertal spurt (PS). Accelerations are prone to follow random variations present in the data, and parameters defined in terms of acceleration are, therefore, validated by a comparison with parameters defined in terms of velocity. Our non-parametric-curve-fitting approach is also compared with parametric fitting via a model suggested by Preece and Baines (1978).
Body Height↗
An analysis of the mid-growth and adolescent spurts of height based on acceleration.
Height growth between four weeks and 20 years of 45 boys and 45 girls from the Zürich Longitudinal Growth Study (1955-1976) was analysed using kernel estimates. Timings of the mid-growth spurt (MS) and of the pubertal spurt (PS) were determined in an automatic way from the individual acceleration curves, together with height, percentage of height, velocity and acceleration at these ages. The small mid-growth spurt is a consistent phenomenon, peaking at 6.4 years (M,F) in acceleration and at 7.7 years (M) and 7.5 years (F) in velocity. There are no significant sex differences in its intensity. In girls, the PS follows in close succession to the MS; in boys there is a substantial period in between. In addition to the age of peak height velocity, ages of onset, maximal acceleration and end of the PS are defined. Sex differences in timing and size of the pubertal peak previously established were again verified. New results relate to the asymmetry of the PS, which is more pronounced in girls, and to sex differences in intensity and duration of the first rising phase of the PS. After this phase, boys and girls do not differ in timing but only in the intensity of deceleration.
Adolescent↗
Human height growth: correlational and multivariate structure of velocity and acceleration.
In this paper the correlations between parameters quantifying the mid-growth spurt (MS) and the pubertal spurt (PS) of height are explored. These parameters are defined in individual acceleration, velocity, and distance curves, which are obtained via kernel estimates as previously introduced. The MS proves to be a phenomenon largely independent of the PS. Intensity, timing, and duration of the PS are also independent mechanisms of growth and neither of them influences adult height in an appreciable way. Adult height depends mostly on pre-adolescent velocity. Short-, medium- and long-term regulatory mechanisms for explaining height growth are considered. For short-term mechanisms, acceleration plays a crucial role.
Adolescent↗
Comparison of mid-infrared and Raman spectroscopy in the quantitative analysis of serum.
Mid-infrared or Raman spectroscopy together with multivariate data analysis provides a novel approach to clinical laboratory analysis, offering benefits due to its reagent-free nature, the speed of the analysis and the possibility of obtaining a variety of information from one single measurement. We compared mid-infrared and Raman spectra of the sera obtained from 247 blood donors. Partial least squares analysis of the vibrational spectra allowed for the quantification of total protein, cholesterol, high and low density lipoproteins, triglycerides, glucose, urea and uric acid. Glucose (mean concentration: 154 mg/dl) is frequently used as a benchmark for spectroscopic analysis and we achieved a root mean square error of prediction of 14.7 and 17.1 mg/dl for mid-infrared and Raman spectroscopy, respectively. Using the same sample set, comparable sample throughput, and identical mathematical quantification procedures Raman and mid-infrared spectroscopy of serum deliver similar accuracies for the quantification of the analytes under investigation. In our experiments vibrational spectroscopy-based quantification appears to be limited to accuracies in the 0.1 mmol/l range.
Biomarkers↗
[Change in sodium and potassium concentration in human seminal plasma infected experimentally with Ureaplasma urealyticum].
The concentration of sodium and potassium ions was measured by flame photometry in human seminal plasma and human sperm cells as well as in a sperm cell suspension infected experimentally with Ureaplasma urealyticum during incubation as long as 22 hours. The sodium concentration is 86.2 +/- 21.97 mmol/l cells in sperm cells and 120.8 +/- 15.87 mM in seminal plasma. The potassium concentration is 33.2 +/- 16.35 mmol/l cells in sperm cells and 26.7 +/- 4.92 mM in seminal plasma. The sodium concentration decreased considerably in the suspension medium of sperm cells during the first hour of incubation and increased again subsequently. The potassium concentration in the suspension medium changed only little. The sodium concentration in the infected sperm cell suspension was insignificantly increased compared with the mycoplasma-free controls. The potassium concentration was slightly higher in the infected specimens than in the controls. There was no mycoplasma-induced effect on the concentration of sodium and potassium in the sperm suspension after experimental infection.
Humans↗
MR findings in adult-onset adrenoleukodystrophy.
PURPOSE: To describe the MR findings of brain and spinal cord in adult-onset adrenoleukodystrophy. METHODS: One hundred sixty-four adult patients ranging from 19 to 74 years of age (119 men and 45 women) with clinically and biochemically proved adrenoleukodystrophy underwent MR of the brain. In 30 patients the spinal cord also was evaluated with MR. RESULTS: The brain MR findings were abnormal in 54 of 119 males and in 9 of 45 female heterozygotes and consisted of varying degrees of demyelination of the cerebral white matter in 40 patients, corpus callosum in 25 patients, corticospinal tracts in 46 patients, visual tracts in 31 patients, and auditory tracts in 18 patients. The thoracic spinal cord showed diffuse atrophy in 18 of 20 men and in 8 of 10 women. CONCLUSION: It is important to recognize the MR findings of adult-onset adrenoleukodystrophy, because not uncommonly the clinical and MR findings of adrenoleukodystrophy are misdiagnosed as multiple sclerosis, olivopontocerebellar or spinocerebellar atrophy, amyotrophic lateral sclerosis, or dementia. Analysis of the MR findings and correlation of the clinical findings has permitted a tentative subdivision of adult-onset adrenoleukodystrophy population into four subtypes that appear to differ in respect to prognosis and possibly pathogenesis. MR evaluation of the brain in adrenoleukodystrophy also is helpful in patient selection for experimental therapy, which is most effective if offered in the early stage of the disease.
Adrenoleukodystrophy↗
[Prenatal diagnosis of hypoplastic left heart syndrome--indications for abortion or transplantation?].
Early diagnosis of fetal cardiac malformations in center of Perinatal Medicine is now possible with the use of modern high frequency ultrasound technics in combination with the 3-step-program of the DEGUM. The diagnosis of most cardiac malformations can early already be obtained in the 2nd trimester as for example the lethal hypoplastic left ventricle syndrome. We want to discuss the problem of prenatal diagnosis of the hypoplastic left heart syndrome and the possibilities of therapeutic procedures.
Abortion, Eugenic↗