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Biomedical subjects

W Müller

Publications and source records attributed to W Müller.

At least 1,279 records · Page 71Linked to original sources
PubMedDate not supplied

[Neonatal progeroid syndrome (Wiedemann-Rautenstrauch syndrome): case report and review of the literature].

The neonatal progeroid syndrome represents a complex of symptoms with unknown cause and pathogenesis. At 37 weeks of uncomplicated pregnancy a boy of non-consanguineous turkish parents was delivered by section. The weight of 1740 g and the length of 43 cm were striking, the circumference of the head was still within normal range. Further on the patient showed the diagnostic criteria of Wiedemann-Rautenstrauch syndrome: Beside growth failure and nearly absence of subcutaneous fat, old-looking face with hydrocephalic appearance, prominent scalp veins and sparse scalp hair, large hands and feet with long fingers and toes were found. Neonatal teeth didn't exist. But neonatal teeth are not always present in this syndrome. Among 19 published cases they were also not described in 6 cases (31.6%). Cataract was not found. A skin biopsy taken at the age of four months revealed an extremely thin dermis with pronounced rarefication of elastic material by light microscopy. On the electron microscopical level, no qualitative morphological aberrations or signs of degeneration were found, but normal although very small elastic fibres. With increasing age the appearance was nearly unchanged. In spite of high-caloric nutrition the increase of weight was not sufficient. The clinical symptoms of this patients were compared with the 19 already published cases of Wiedemann-Rautenstrauch syndrome. This shall be a contribution to the exact description of that extremely rare syndrome. We hope to facilitate, that hereby the differential diagnosis in difficult cases will be more easy. Because of heterogeneity it has to discuss if the Wiedemann-Rautenstrauch syndrome really represents a separate genetic entity within the group of premature aging syndromes.

Biopsy↗
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[Local treatment of periarthropathies with the 5-HT3-receptor-antagonist tropisetron].

All substances used in the local treatment for periarthropathies, such as local anesthetics, corticosteroids or botulinum toxin A, possess certain disadvantages. Finding alternatives to these agents in the local treatment of the disease therefore seems desirable. Comparative studies proved a local injection of the 5-HT3 receptor antagonist, tropisetron (Navoban(R)), to be more effective than an injection of the local anesthetic prilocaine in treating periarthropathies of different localizations. A comparison between the local injection of 10 mg of depot dexamethasone combined with 60 mg of lidocaine and 5 mg of tropisetron showed that the two regimens had the same effect. These findings demonstrate that a local injection of 5 mg tropisetron does represent an alternative to the local treatment with corticosteriods plus local anesthetics. However, these results should be corroborated by additional studies.

Acetanilides↗
PubMedDate not supplied

[Transient periventricular echodensities (TPE) in preterm infants under 1500 gramms: an analysis of the outcome of the last 10 years].

BACKGROUND: The aim of our study was to analyze neurodevelopmental outcome in preterm infants with TPE. PATIENTS: 46 preterm infants with a birthweight (BW) < or = 1500 g were included in the study. They all had TPE in parieto-occipital location (some combined with frontal flares). Exclusion criteria were PVL II-IV, intraventricular hemorrhage, asphyxia and isolated frontal TPE. METHOD: We analyzed ultrasound scans (1992 - 2001) of patients with TPE (diameter > 1 cm, visible in both sagittal and coronal planes, duration > 7 days), and neurological follow-up visits (date of demission, corrected age of 4, 8, 12 months, and after that every 2 years until 6 years of age) regarding the neurodevelopmental outcome. We devided the patients in group 1 (duration of TPE 8 - 14 days) and group 2 (duration of TPE > 14 days) and then analyzed the groups regarding neurodevelopmental disorders and factors increasing the risk of occurrence of TPE. RESULTS: From 1992 - 2001 578 children < or = 1500 g were admitted at our department. 48 patients (8,3 %) fulfilled the entry criteria, 2 of them died, so 46 were included in the study. In group 1 (10 patients) the median gestational age (GA) was 28 weeks (range 25 - 31), median BW 1075 g (range 685 - 1430), 6 children were neurologically unimpaired, one developed a motor handicap, and 3 a psychomotor retardation. In group 2 (36 patients) the median GA was 28 weeks (range 25 - 35), median BW 1034,5 g (range 540 - 1470), 24 patients were neurologically unimpaired, 7 developed a motor handicap, and 5 a psychomotor retardation. No statistical difference was found between the groups neither in neurodevelopmental outcome nor factors increasing the risk of the occurrence of TPE. CONCLUSIONS: Obviously, the finding of TPE is of clinical relevance already at a duration of only 7 days regarding later neurological development.

Age Factors↗
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[Inhalation therapy with nitric oxide in pulmonary hypertension: Comparison of preterm infants versus newborn infants].

BACKGROUND: Inhaled nitric oxide (iNO) is used as a vasodilator in pulmonary hypertension (PH) of the newborn infant. PATIENTS AND METHODS: Retrospective analysis of patients, who were treated at our department with iNO in the period from 1994-2001. Response was defined as an increase of the paO (2)/FiO (2) Ratio > or = 20 % and/or a decrease of the oxygenation index (OI) >/= 20 % after 2 h (early response), and consecutively after 24 h (late response). The patients were divided into a) primary persistent pulmonary hypertension of the newbom (PPHN), or b) pulmonary hypertension secondary to meconium aspiration syndrome (MAS), sepsis or congenital diaphragmatic hernia (CDH). RESULTS: Between 1994 and 2001 we treated 47 patients with iNO at our neonatal intensive care unit. We included 16 (35 %) preterm infants (GA 34,5 [25 - 37] weeks, GG 2061 [680 - 3410] g) (Median/Range) and 31 (65 %) newbom (GA 40 [38 - 42] weeks, GG 3510 [2550 - 4560] g). 18 (38 %) patients suffered from primary PPHN, 29 (62 %) from secondary PPHN (14 MAS [30 %], 8 sepsis [17 %], 4 CDH [8 %]). 8 (50 %) preterm and 20 (64 %) term infants showed a positive iNO response after 2 h, again 8 (50 %) preterm and 20 (64%) term infants showed a positive iNO response after 24 h. There was neither a significant difference between term and preterm infants at 2 h, nor at 24 h. Between 2 h and 24 h 10 patients changed in their response to iNO. 5 (18 %) patients with early response showed a significant degradation after 24 h, whereas 5 (26 %) of the patients without early response showed a significant improvement of the oxygenation alter 24 h. Alltogether 13 (72 %) patients with PPHN, 8 (57 %) with MAS, 2 (50 %) with CDH, 4 (50 %) with sepsis showed a positive iNO response after 24 h. In regard to the oxygenation parameters at start of iNO-therapy, the patients with early response did not differ from the patient without response (median OI: 20,0 versus 21,8, median paO (2)/FiO (2) Ratio: 59,3 versus 55,0 mmHg at the start of the iNO therapy). CONCLUSION: In regard to iNO response, there was no significant difference between term and preterm infants. Due to the changing response, a positive iNO-response after 2 h had no predictive value for the further prognosis of the oxygenation situation under iNO therapy.

Age Factors↗
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[An asphyctic newborn infant with sonographically dilated ventricles].

Brain edema causes severe cerebral defects after perinatal asphyxia. In managing children with asphyxia adequate therapy of brain edema is most important for the neurological outcome. Usually estimation of the width of the ventricular system is initially used for sonographical classification or suspicion of brain edema. We present a case with the unusual aspect of wide cerebral liquor system in spite of severe asphyxia and severe brain edema with consecutive cerebral atrophy. In this case brain edema could only be recognised by the use of doppler sonography of the cerebral vessels and was confirmed by brain pressure monitoring. We therefore suggest that sonographic diagnostic workup of newborns with asphyxia must include a doppler-sonography of the cerebral vessels as well as a brain pressure monitoring.

Asphyxia Neonatorum↗
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[Blood propionic acid with hyperammonemic coma].

We report on a mature male newborn who presented clinically on the 2nd day of live with poor feeding and acidotic breathing. Laboratory findings like severe metabolic acidosis, hyperammonemia, hyperglycinemia, ketonuria and elevated urinary excretion of lactate and propionate suggested the presence of organoacidopathia. Propionic acidemia, however could be diagnosed definitively only when the characteristic urinary and blood metabolites were found during the state of a hyperammonemic coma provoked by a fully oral protein regimen. The diagnosis was affirmed by reduced propionate fixation and by reduced propionyl-CoA-carboxylase shown in the patient's skin fibroblasts.

Acidosis↗
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[Postoperative sonographic follow-up of brain tumors in childhood].

Postoperative neurosonography was performed in 69 babies and children after osteoclastic neurosurgery for primary cerebral tumors. Astrocytoma of the posterior fossa (26 cases) and medulloblastoma (25 cases) were frequent histological findings. Early postoperative bed-side examinations revealed reliably complications such as cerebral hemorrhage, edema or hygroma. Postoperative baseline studies were performed and reliability of sonographic criteria for tumor recurrence, such as high echogenicity and dense echotexture, were established. Regressive tissue changes under the usual postoperative cerebral radiation therapy and chemotherapy included transient increase in perifocal edema followed by tumor shrinking and cystic lesions or calcifications. Shunt dynamics were controlled by morphometrical studies of ventricular size. Long-time follow-up examinations were performed in most cases and covered periods of up to 4 years. Sensitivity of neurosonography for tumor recurrence was superior to non-contrast computed tomography; there were no false positive findings as proven by CT and magnetic resonance.

Adolescent↗
PubMedDate not supplied

[Congenital hyperthyroidism caused by diaplacental exchange of thyrotropin receptor antibodies].

We describe the case of a premature baby, extremely small for date whose mother had an untreated hyperthyroidism during pregnancy. Severe symptoms of connatal hyperthyroidism appeared after a latency period of seven days. As the underlying cause we suggest the placental transmission of thyrotropin-receptor-antibodies (TRAb), which were elevated in the mother and the newborn. Main symptoms in the newborn were tachycardia, tachypnea, sweating and diarrhea.

Autoantibodies↗
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[Repeat intertrochanteric osteotomy in the implantation of a cement-free straight total hip endoprosthesis following previous varization osteotomy. A case report].

Severe deformities of the proximal femur can make it impossible to implant a straight-shaft prosthesis. A correctly aligned axis can be achieved by intertrochanteric osteotomy. The case report described here shows that with exact fitting, the Zweymüller type cement-free prosthesis not only results in stable diaphyseal conditions for its own implantation, but is also a suitable means of achieving a stable internal osteosynthesis of two diametaphyseally proximal fragments.

Female↗
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[Prophylaxis of respiratory syncytial virus (RSV) in preterm infants with/without bronchopulmonary dysplasia: hyperimmune globulin (RSV-IGIV) and palivizumab (MEDI-493)].

Respiratory syncytial virus (RSV) causes seasonal epidemics between December and March (April) and remains the main agent that causes severe lower respiratory tract infections in young infants. Children with bronchopulmonary dysplasia up to 24 months of age and preterm infants with a gestational age of 32 weeks and below, who are less than six months of age, are at highest risk for severe RSV infection. RSV-IGIV has been demonstrated to reduce significantly RSV associated hospitalizations, RSV associated hospital days and the incidence of severe RSV lower respiratory tract infections. Monthly infusions during RSV season were safe and well tolerated. Adverse events related to the hyperimmune globulin infusion were generally mild (< 3%) including fluid overload, decreased oxygen saturation and fever. Palivizumab, an intramuscularly administered humanized monoclonal antibody (RSV-glycoprotein-F antibody), will be preferable for the future because of ease of administration and comparable reduction in the risk of hospitalization. RSV-IGIV and palivizumab are both cost expansive and prophylaxis should be limited to high-risk infants.

Antibodies, Monoclonal↗
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[Successful treatment of therapy refractory shock lung in a newborn infant with congenital varicella using extracorporeal membrane oxygenation].

A newborn with congenital varicella complicated by varicella pneumonia was transferred to our hospital on day 16 of life for the consideration of extracorporeal membrane oxygenation (ECMO). The newborn received varicella zoster immunoglobulin 13 hours after birth since the mother developed a varicella exanthema two days before delivery. On day 10 of life the newborn became clinically symptomatic with red macules and pustules. The chest roentgenogram revealed reticular pulmonary infiltrates in the right upper lobe. Antibacterial chemotherapy was initiated. In the following days the cutaneous lesions progressed, and respiratory symptoms like tachypnoea and oxygen dependence occurred. Chest roentgenograms revealed diffuse reticular and patchy pulmonary infiltrates. On day 14 of life antiviral chemotherapy with acyclovir was started. ECMO was initiated in the veno-arterial mode on day 17 due to severe respiratory failure despite maximal conventional assisted ventilation and carried out for 14 days. With the age of 10 weeks he was discharged from the hospital with mild chronic lung disease without oxygen requirements and without neurological handicap. ECMO might be considered as life saving support in newborns with severe congenital varicella, considered to have a high mortality risk.

Acyclovir↗
PubMedDate not supplied

Cellular and vascular reactions in the liver to radio-frequency thermo-ablation with wet needle applicators. Study on juvenile domestic pigs.

UNLABELLED: The aim of this animal experiment was to investigate the cellular and vascular reactions in the liver of juvenile domestic pigs produced by a radio-frequency thermoablation (RFTA) applicator perfused with saline solution. METHODS: A total of 13 coagulation necroses were produced in the liver of 3 anesthetized domestic pigs using RFTA. The pigs were dissected and the coagulations examined. RESULTS: The mean macroscopical length and width of the coagulation zones with a hemorrhagic marginal zone after a 5-minute application time were 34.1 +/- 8 mm (22-46 mm) and 20.8 +/- 4 mm (12 +/- 28 mm) respectively. The sonographically determined diameters correlated significantly (r(length) = 0.741 and r(width) = 0.923). Three areas in the coagulation zones could be histologically distinguished: (1) central necrosis zone, (2) hemorrhagic marginal zone, (3) sublethal damage zone. Large vessels did not show any substantial changes after RFTA. Venous vessels less than 1 mm were completely thermally denatured or destroyed. CONCLUSIONS: Tumors in close proximity to large blood vessels can be treated by RFTA with 'wet electrodes'.

Animals↗
PubMedDate not supplied

[Validation of the Tekscan system for statistic and dynamic pressure measurements of the human femorotibial joint].

In vitro dynamic pressure measurements in the healthy and pathologically altered knee joint help to improve our understanding of the loading pattern on femorotibial surfaces. The aim of the study was to evaluate a piezoresistive pressure measuring system. A human cadaveric knee was mounted in a material-testing machine (Bionix 858) using a specially designed knee-holding device. Axial loading of the knee, flexed at 20o, at 500 N, 1000N and 1500 N was then carried out. For the static investigations, the piezoresistive measuring system (Tekscan), was compared with the FUJI measuring system. In addition, dynamic measurements were also performed with the Tekscan System. With the exception of the lateral compartment at a load of 1500 N, no differences in maximum pressures were observed between the two systems. Nor were there any differences with regard to contact surfaces, either in the medial or lateral compartment (p > 0.05). However, the reproducibility of the data was significantly higher with the Tekscan System (p < 0.01). Dynamic pressure measurements obtained with the knee flexed 20 to 90o showed that the lateral contact area shifted from anterior to posterior, while the medial contact area remained virtually unchanged. The Tekscan System proved to be more reliable than the FUJI System, and permits simultaneous measurements in both compartments. The Tekscan System is suitable for dynamic measurement of the femorotibial joint, and permits measurements to be made under more physiological conditions.

Aged↗
PubMedDate not supplied

Non-invasive estimations of ventricular ejection time and stroke volume: comparison of impedance cardiography and the Portapres 2.

Stroke volume estimates and left ventricular ejection time (1) derived from calibrated finger pulse pressure recordings (Portapres 2, TNO-BMI, Amsterdam) based on the Model Flow method and (2) derived from impedance cardiography were compared in 20 student subjects. The recordings were made for seven conditions: supine rest, standing, mental arithmetic, ergometer exercise 50 W, motor performance test, reading aloud, and walking. Findings indicated that caution is required in assuming concurrent validity for both methods in tracking haemodynamic changes. Within-subject correlations were low or even negligible. Basic discrepancies were evident regarding the estimation of left ventricular ejection time but obviously more sources of measurement error exist which necessitate further research.

Adult↗
PubMedDate not supplied

Estimation of total PSA with a supersensitive PSA-assay during neo-adjuvant-chemotherapy of prostate cancer before radical resection of prostate.

43 patients with untreated and clinically localised prostate carcinoma (cT1-3NoM0) were submitted to neo-adjuvant complete androgen deprivation treatment and radical prostatectomy. Hormonal treatment was given until PSA (supersensitive Immulite 3rd generation assay) reached a value of < 0.1 ng/ml or the nadir value. The resultant mean duration of treatment was 6 months (3-22 months). 93% of our patients reached a PSA value of 0.1 ng/ml or below. The nadir of 6 patients was between 0.1-0.3 ng/ml. In one case it remained above 0.3 ng/ml. 82% had a measurable hypoechoic lesion on initial transrectal ultrasound. 84% of these became smaller, 7.5% remained unchanged and 8.5% increased. Of the patients with cT1-2 tumours 2% had a positive margin and in 28% of the specimens no tumour tissue could be detected at the time of prostatectomy. Of the initial patients with epithelial cells in bone marrow only 14% remained positive after controlled induction and all of them had fewer cells than before. Five patients relapsed showing raising PSA values > 0.1 ng/ml in the follow-up. In this group the mean PSA and the time of nadir was significantly higher (0.15 ng/ml) than the mean PSA at the time of nadir of the rest of the group (0.06 ng/ml). Supersensitive PSA assays like Immulite 3rd generation offer a valuable tool to collect data for clinical evaluation to optimise therapy of prostate carcinomas.

Antineoplastic Agents, Hormonal↗
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IUPAC collaborative trial study of a method to detect genetically modified soy beans and maize in dried powder.

This paper presents results of a collaborative trial study (IUPAC project No. 650/93/97) involving 29 laboratories in 13 countries applying a method for detecting genetically modified organisms (GMOs) in food. The method is based on using the polymerase chain reaction to determine the 35S promotor and the NOS terminator for detection of GMOs. reference materials were produced that were derived from genetically modified soy beans and maize. Correct identification of samples containing 2% GMOs is achievable for both soy beans and maize. For samples containing 0.5% genetically modified soy beans, analysis of the 35S promotor resulted also in a 100% correct classification. However, 3 false-negative results (out of 105 samples analyzed) were reported for analysis of the NOS terminator, which is due to the lower sensitivity of this method. Because of the bigger genomic DNA of maize, the probability of encountering false-negative results for samples containing 0.5% GMOs is greater for maize than for soy beans. For blank samples (0% GMO), only 2 false-positive results for soy beans and one for maize were reported. These results appeared as very weak signals and were most probably due to contamination of laboratory equipment.

Agrobacterium tumefaciens↗
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Prognostic value of the cyclin-dependent kinase inhibitor p27Kip1 in gastric cancer.

In the present study, the expression and prognostic role of p27 were immunohistochemically investigated in 413 curatively resected gastric carcinomas. Strong p27 expression in more than 50% of the tumour cells could be detected in 57.4% (n = 237) whereas 42.6% of the tumours (n = 176) only showed p27 expression in less than 50% of the tumour cells. No significant correlation could be observed between p27 expression and the prognostic parameters pT category, pN category, blood and lymphatic vessel invasion as well as with tumour histology. Concerning other cell cycle associated proteins, p27 expression was inversely correlated with p21 expression, however, there was no correlation either with cyclin D1 and cyclin E or with expression of p53, bcl-2 and tumour cell proliferation. Univariate survival analysis revealed a poorer prognostic outcome for patients with tumours expressing p27 in more than 50% of the tumour cells (p < 0.049). However, by multivariate analysis, this prognostic influence of p27 could not be verified as independent from the known prognostic parameters of the pTNM-system (p < 0.325). The present data on 413 curatively resected gastric carcinomas suggest, that expression of p27, analyzed alone or in combination with multiple cell cycle regulatory proteins, has no prognostic value in gastric cancer.

Adult↗