["Karusselstimulation", a new method of electrophrenic long-term nerve stimulation (author's transl)].
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Biomedical subjects
Publications and source records attributed to A Lischka.
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It has been suggested previously that non-enzymatic glycosylation of certain enzymes results in decreased enzymatic activity. In order to examine the role of high blood glucose concentrations (BG) on serum alkaline phosphatase (AP) activity, we determined BG and serum AP in 32 healthy children during an oral glucose tolerance test (OGTT) and in 10 type I diabetic children at 30-min intervals for at least 150 min. A significant negative correlation was noted for BG and AP during the oral glucose load (r = -0.57, p less than 0.01). In diabetics, however, only children with marked BG fluctuations showed this inverse relationship between BG and AP. These observations could be explained by the formation of a Schiff base as the initial step of non-enzymatic glycosylation of AP. This assumption was further confirmed by in vitro experiments, in which AP was incubated with glucose resulting in decreased enzymatic activity. The dialyzable aldimine formed initially is subsequently stabilized as ketoamine after long-term incubation.
The effect of insulin induced hypoglycemia on glucoregulatory hormones and intermediary substrates was studied in four infants (three boys and one girl, ages 12-89 days) with persistent hyperinsulinism secondary to nesidioblastosis (two) or microadenoma of the pancreas (two). During the "fasting test" the following data expressed as mean basal plasma values +/- SEM vs. mean hypoglycemic values +/- SEM were obtained: insulin (57.3 +/- 17.9 vs. 27.5 +/- 10.6 microU/ml), C-peptide (4.9 +/- 1.1 vs. 3.5 +/- 0.9 ng/ml), free fatty acids (0.30 +/- 0.01 vs. 0.32 +/- 0.02 mmol/l), beta-hydroxybutyrate (less than 0.03 vs. 0.05 +/- 0.02 mmol/l), alanine (204.0 +/- 67.5 vs. 228.3 +/- 64.9 mumol/l), lactate (5.3 +/- 0.7 vs. 5.4 +/- 1.1 mg/dl), pyruvate (41.3 +/- 4.8 vs. 19.7 +/- 4.2 mg/dl). These data suggest "inappropriate" elevation of insulin and C-peptide levels, inhibition of lipolysis and lack of gluconeogenic substrates secondary to endogenous HI. An increase of cortisol (6.5 +/- 4.1 vs. 16.3 +/- 5.7 micrograms/dl), adrenaline (0.015 +/- 0.05 vs. 0.25 +/- 0.24 ng/ml) (3 out of 4) and noradrenaline (0.28 +/- 0.06 vs. 0.64 +/- 0.14 ng/ml) was noted, whereas only minute increase was found for glucagon (134.3 +/- 51.7 vs. 177.0 +/- 76.2 pg/ml) and HGH (5.7 +/- 1.1 vs. 7.1 +/- 1.1 ng/ml). Although some stimulation of neonatal glucoregulatory hormones was evident, this was not strong enough for counteracting endogenous HI.
Four infants (three boys and one girl, ages 12-89 days) with persistent hyperinsulinism secondary to nesidioblastosis (two) or microadenoma of the pancreas (two) were treated with cyclic somatostatin (S) as part of the preoperative management until subtotal pancreatectomy was performed within 12 to 35 days. The individual dose dependent response of glucoregulatory hormones to exogenous was evaluated by means of the "somatostatin sensitivity test" (SST). Thereby S was infused in stepwise increasing doses, as dictated by the prevailing blood glucose levels, until normoglycemia was achieved concomitantly with a dextrose infusion at rates of 5 mg/kg/min. This procedure resulted only in a partial suppression of insulin, C-peptide, glucagon, HGH and cortisol, without recurrence of hypoglycemia. Compared to baseline levels, plasma concentrations of insulin decreased by 61%, of C-peptide by 64% and a rise of glucagon by 23% was observed. The SST which can be performed under routine clinical conditions, is a useful procedure for evaluating the individual S-dose necessary to achieve normoglycemia. The risk of total S-induced suppression of hormones, such as IRI, IRCP, HGH, glucagon and cortisol can be omitted.
A five year old boy who had received logopedic treatment for more than two years was seen as an outpatient because of speech retardation. He presented with myopathic face, incomplete closure of both lids and severe weakness of facial muscles, bilateral winging of scapulae and hyperlordosis. Extraocular and pharyngeal muscles were not affected. Motor and sensory nerve fibre conductions and electromyography were within normal limits. CPK was moderately elevated (320 U/I). Muscle biopsy of right deltoid muscle revealed unspecific myopathic changes. The patients brother aged 7 also presented with facial weakness, elevated CPK and neurogenic changes in EMG of deltoid muscle. Both parents were clinically and electrophysiologically unremarkable. Although problems to speak distinctly are usually not the first manifestation, we found in this family facio-scapulo-humeral muscular dystrophy.
In six male patients with anorexia nervosa (a.n.) we describe the anamnestic data, the course of disease and progress in therapy. We also present the results of psychological testing and organic investigations. The mean weight loss at the time of first investigation at the clinic was 70% of ideal body weight. The mean time between first symptoms of a.n. and the first admission at the clinic was 19 months. The psychopathological status showed in all six patients symptoms of obsessive, in two patients additional of depressive behavior. This finding is in contradiction to the result in 61 female patients with a.n., where hysteriformic and depressive symptoms predominated (36). The follow up time reached from 18 to 84 months (mean 49 months). At the time of last control-investigation we found only one patient restored to health, two patients showed a positive progression. In two patient the psychiatric situation was nearly unchanged, one patient committed suicide. The three patients without positive therapeutic effect were three times respectively six times at the inpatient department and discontinued two times or also three times the psychotherapy. In our experience the course of disease in male patients with a.n. seems to be more serious and more resistant to therapy than in female patients.
Female patients with anorexia nervosa (a.n.) are characterized by distinct endocrine features probably due to hypothalamic pituitary dysfunctions. There is only a limited number of case reports available on patients with a.n.; mostly with few data on hormones. In six male patients with a.n. we examined basal and stimulation values of several hormones performing three pituitary function tests. Basal and stimulated values of luteinizing hormone (LH) and of follicle stimulating hormone (FSH) after LHRH were low comparable to results in prepuberal boys. Similarly, testosterone levels in serum were also markedly reduced. By exploring the pituitary-thyroidal axis total T4 was diminished in one patient and at the lower limit in two patients; concentration of free T4 was in the normal range, while five of six subjects had reduced total T3 concentration and two of six patients showed increased reversed T3 levels; TBG concentration was always in the normal range. Basal TSH was normal, while in two patients the TSH stimulation levels after TRH were diminished; in all patients the TSH stimulation levels were found to be delayed. The basal levels of growth hormone were normal, but the growth hormone response after insulin was diminished in four patients. In all six patients basal prolactin (PRL) and PRL concentration after TRH stimulation was in the normal range. The neuroendocrine results in the six patients with a.n. confirm in males a similar hypothalamic-pituitary dysfunction as it is already known for female patients.
Ten to fifteen percent of infants of myasthenic mothers develop neonatal myasthenia independent of the severity of the maternal disease. The antiacetylcholin receptor-protein antibodytiter (AChR-AK) was followed through the first twelve months in a boy who's mother suffered from myasthenia gravis (M.G.) since twelve years. Only unspecific symptoms for an impaired neuromuscular transmission were found, the diagnosis however was confirmed by determination of AChR-AK (alpha-Bungarotoxin binding assay, Lindström 1979). Maternal AChR-AK did not change during the observation period: values were between 120 X 10(-9)mol/l and 140 X 10(-9)mol/l. The neonatal titer on day one was 100 X 10(-9) mol/l, somewhat below the corresponding maternal titer (120 X 10(-9)mol/l). By 7 months the AchR-AK had decreased to the reference value according to the half time for IgG (t/2 = 17 days). In contrast to Keesey and Donaldson we found no relationship between AChR-AK levels and clinical course.
Low birth weight babies and sick full-term babies, who require total parenteral nutrition and repeated intravenous applications of drugs, which irritate peripheral veins, very often need a reliable central venous catheter. The aim of our paper was to study prospectively the efficiency and the complications of peripheral percutaneous Silastic-catheters. Over a period of 9 month we inserted 114 central venous catheters via peripheral veins in 111 premature babies and sick full-term infants at our neonatal intensive care unit. The mean duration of use was 13.7 days, the catheter-induced septicaemia-rate was 3.5%. We never saw serious complications of a central venous catheter, the most common complication was an intravasal central obstruction, but we found no relation between the occurrence-risk of complications, the duration of use and the infusion flow rate.
In a male infant with congenital growth hormone deficiency and genital hypoplasia (micropenis, cryptorchidism and small scrotum) androgen receptors, tissue specific 5 alpha reductase and steroid excretion pattern were determined in order to test the hypothesis of peripheral androgen resistance. A reduced number of cytosolic binding sites (Nmax) was found for the T and DHT receptor in patients foreskin compared to controls (n = 9) of similar age. The specific affinity (Kd) of the cytosolic receptor, however, was normal. Tissue specific 5 alpha reductase determination revealed a Vmax of 3.3 pmol/mg/h (controls 15.8 +/- 1.4). Analysis of urinary steroid excretion pattern revealed decreased levels of T metabolites, indicating impaired T metabolism. We postulate, that genital hypoplasia in patients with congenital GH deficiency is associated with impaired target organ responsiveness to androgen hormones caused by abnormalities within the pathway of intracellular reactions of T utilization.