Biomedical subjects

J Müller

Publications and source records attributed to J Müller.

At least 217 records · Page 12Linked to original sources

PubMed1999

[Torticollis spasmodicus, blepharospasm and hemifacial spasm. Subjective evaluation of therapy by patients].

Injections with botulinum toxin type A (BTX) are considered the first-line treatment for spasmodic torticollis (ST), blepharospasm (BL) and hemifacial spasm (HFS). Because BTX brings only temporary and partial relief, patients frequently try other additional therapies to minimize their symptoms. The subjective rating of all therapies ever tried by patients with ST, BL and HFS was evaluated by using a simple questionnaire. Two hundred questionnaires were considered (112 TS, 54 BL, 34 HFS). BTX was rated subjectively the best therapy in all three diagnostic groups (median: 2 = good effect). Despite Citalopram and physiotherapy (median: 3 = average effect), all other therapies were rated with a median of > or = 4 (= minimal effect). Patients with ST tried 7.7, patients with BL 2.4 and patients with HFS 2.6 different types of therapy. In conclusion, BTX is the most effective treatment for patients with ST, BL and HFS, as rated subjectively. Further evaluation of therapies additional to BTX injections is recommended.

Blepharospasm↗

PubMed1999

Ultrasound-guided puncture of a Dandy-Walker cyst via the lateral and III ventricles.

Since the Dandy-Walker syndrome was first described by Dandy and Blackfan, Taggart and Walker, the many variants of posterior fossa anomalies, the appropriate management of these malformations and the clinical outcome have been the subjects of controversy. Surgery of the posterior fossa with membrane excision was initially the preferred method of treatment. Unfortunately, there was a high rate of complications, and many of the patients treated in this way still needed a shunting system. Ventricular-peritoneal and/or cysto-peritoneal shunting is commonly used to treat symptomatic posterior fossa cysts of Dandy-Walker malformations and hydrocephalus. Cysto-peritoneal shunt implantation only was associated with a high rate of complications, and most patients so treated needed a ventriculo-peritoneal shunt in addition. According to the literature, combined ventriculo-peritoneal and cysto-peritoneal shunting is needed for satisfactory decompression of Dandy-Walker cyst and hydrocephalus in between 16% and 92% of cases. We report on a young patient with a Dandy-Walker malformation who needed drainage of the posterior fossa and a ventricular shunt. We decided to drain the cyst and the supratentorial ventricles via a single, especially prepared, catheter with many perforations. The catheter was inserted under ultrasound guidance. The tube was inserted from the left lateral ventricle through the foramen of Monro into the III ventricle and downwards into the cyst. Intraoperatively, an immediate decrease in the size of the cyst and the supratentorial ventricles was observed. Postoperative MRI confirmed the exact position of the catheter and sufficient drainage of the posterior fossa cyst and the ventricles. Six months later the girl was seen in our outpatient department. Clinical examination showed no neurological deficit, and MRI demonstrated sufficient drainage of the ventricles and the Dandy-Walker malformation, and in addition hypoplasia of the corpus callosum.

Adult↗

PubMed1999

Cardiac recovery in dilated cardiomyopathy by unloading with a left ventricular assist device.

BACKGROUND: Lasting recovery from intractable end-stage dilated cardiomyopathy, which occurs with ventricular unloading, has recently been demonstrated in 5 patients. Here our extended clinical experience with the "weaning" concept is presented. METHODS: In 19 patients (23 to 65 years) with intractable end-stage dilated cardiomyopathy, ventricular assist devices were explanted after support periods of up to 26 months, when repeat off-pump studies had shown either restoration of cardiac function (left ventricular ejection fraction, > 45%) and dimensions (left ventricular internal diameter in diastole, < 55 mm) or partial recovery (left ventricular ejection fraction between 35% and 40%) with serious complications on the device. At the time of device placement left ventricular ejection fraction was below 20% and left ventricular internal diameter in diastole more than 64 mm and bridge-to-transplantation had been planned. RESULTS: Seven patients with persistently restored cardiac function for more than 8 months and 5 patients for less than 5 months after weaning were studied. Five patients with recurrent heart failure died within 4 to 8 months after explantation. Four patients had to be transplanted and 2 died for reasons unrelated to cardiac function. An individual optimal left ventricular ejection fraction and left ventricular internal diameter in diastole was reached before pump removal was actually conducted in all patients. These parameters gradually deteriorated until pump removal. CONCLUSIONS: Lasting recovery can be reached by ventricular unloading in a subset of patients with intractable end-stage dilated cardiomyopathy. Obviously, there is an individual optimum of recovery that cannot be further improved by prolonged unloading.

Adult↗

PubMed1999

Induction of genotoxic effects and modulation of the intracellular calcium level in syrian hamster embryo (SHE) fibroblasts caused by ochratoxin A.

The mycotoxin ochratoxin A (OTA) is a naturally occuring contaminant of food. The genotoxic status of OTA is still controversial because contradictory results were obtained in various microbial and mammalian gene mutation assays. In this study, OTA was investigated to examine its potency to induce micronuclei (MN) in SHE cells. The SHE-micronucleus assay revealed that OTA induces MN in a dose- and time-dependent manner. The results of kinetochore analysis revealed that mainly clastogenic events are involved in OTA genotoxicity. Induction of mitotic disturbances can be closely related to changes of the intracellular calcium concentration ([Ca2+]i). The investigated time course of OTA-induced [Ca2+]i changes revealed that the obtained signal is a short spike signal resembling physiological responses. In the absence of extracellular calcium, a long-lasting signal indicates possible damage to intracellular calcium stores or channels. Our data show that the OTA-induced [Ca2+]i rise is caused by Ca2+ -release from intracellular stores as well as Ca2+ influx from extracellular area. Finally, the influence of the changed intracellular calcium level on the actin cytoskeleton was investigated. Visualization of the actin filaments revealed time- and concentration-dependent effects. Cell shrinkage and depolymerized filaments were observed. We conclude that OTA disrupts actin filaments by a direct irreversible binding to actin.

Actins↗

PubMed1999

A simple method of coagulating endometrium in patients with therapy-resistant, recurring hypermenorrhea.

STUDY OBJECTIVE: To assess the efficacy, safety, and tolerability of hydrothermablation in the treatment of recurrent menorrhagia. DESIGN: Prospective, nonrandomized survey (Canadian Task Force classification II-2). SETTING: Endoscopic center at a university hospital. PATIENTS: Eighteen premenopausal women with recurring menorrhagia resistant to hormone treatment. INTERVENTIONS: Coagulation of the endometrium at 90 degrees C for 10 minutes under hysteroscopic control. MEASUREMENTS AND MAIN RESULTS: During follow-up of least 12 months, nine (50%) of patients had amenorrhea and 17 (94%) had reduction or cessation of pathologic blood flow. One woman underwent hysterectomy because of recurrent dysmenorrhea. CONCLUSION: In this study, hydrothermablation was a safe and effective method of treating recurrent menorrhagia. It does not require extensive training and may help avoid hysterectomy. (J Am Assoc Gynecol Laparosc 6(3):265-268, 1999)

Adult↗

PubMed1999

Major basic protein, but not eosinophil cationic protein or eosinophil protein X, is related to atopy in cystic fibrosis.

Increased eosinophil granule proteins have been described in serum and sputum samples of patients with cystic fibrosis (CF). It has been assumed that eosinophil degranulation is enhanced in atopic subjects - as in asthmatics. Since in CF no differences in eosinophil cationic protein (ECP), eosinophil protein X (EPX), and eosinophil peroxidase between atopic and nonatopic subjects have been detected, we investigated whether major basic protein (MBP) is increased in serum and sputum samples derived from atopic (n = 14) compared with nonatopic CF subjects (n = 26). In CF patients, high mean serum (sputum) levels of ECP 29.7 microg/l (2.7 mg/l), EPX 53.7 microg/l (7.9 mg/l), and MBP 984.6 microg/l but low sputum MBP levels (57.4 microg/l) were measured. In addition, in serum and in sputum samples, a significant correlation between MBP and ECP (P<0.03 and P<0.0001, respectively) or EPX (P<0.05 and P<0.0004, respectively) was detected. By subdivision of the patients into allergic and nonallergic subjects, significant differences were found for serum MBP values only(mean 1382.2 microg/l vs. 770.5 microg/l; P<0.0001), but not for ECP or EPX serum levels or for eosinophil proteins in sputum. Although no differences between atopic and nonatopic CF patients in ECP and EPX were found, serum MBP levels were higher in patients sensitized to inhalant allergens than in nonsensitized subjects. These results indicate differential release of eosinophil granule proteins in peripheral blood from eosinophils, and they also indicate that MBP in serum likely is to be a better discriminator of atopy in CF.

Adolescent↗

PubMed1999

Ultrasound B-mode changes in the uterus and ovaries and Doppler changes in the uterus after total body irradiation and allogeneic bone marrow transplantation in childhood.

Internal genitalia and uterine blood flow were assessed by ultrasound in 12 females 4.0-10.9 years after total body irradiation and allogeneic bone marrow transplantation for childhood leukaemia or lymphoma. Median age of the participants was 12.7 years (range 6.1-17.6) at bone marrow transplantation and 21.5 years (11.6-25.6) at the follow-up study. At follow-up all had entered puberty and 11/12 females had experienced the menarche. Eight females received sex steroid replacement therapy, three had spontaneous pubertal development and one woman experienced symptoms of estrogen deficiency. Median uterine and ovarian volumes were significantly reduced to -2.6 standard deviation scores (SDS) (-6.3 to -0.6), P = 0.002, and -2.6 SDS (-4.8 to -0.5), P = 0.002, respectively, compared with normal controls. Follicles were only detectable in two individuals. Uterine blood flow was impaired, as a systolic blood flow could be measured in 6/9 individuals, and a diastolic blood flow in 1/9 females. Our results indicate that the prescribed dosage of hormone replacement therapy, which was sufficient to induce bleeding and suppress other stigmata of premature menopause, was inadequate to generate normal uterine growth. In order to achieve uterine growth higher doses of hormone replacement therapy may be required. Our results confirm pelvic ultrasound as a reliable tool for investigation of internal female genitalia; however, in an infertility setting further tests are indicated.

Adolescent↗

PubMed1999

Detection of cytogenetic aberrations both in CD90 (Thy-1)-positive and (Thy-1)-negative stem cell (CD34) subfractions of patients with acute and chronic myeloid leukemias.

Acute myeloid leukemia (AML) and chronic myeloid leukemia (CML) are thought to arise from malignant hematopoietic progenitor cells representing early and undifferentiated stem cell clones. In CML there is evidence for a progenitor cell subset free of leukemic clones, depending on the course of the disease. Additionally, it has been suggested that in AML, the early stem cell compartment (CD34+/90+) does not harbor the malignant clone. We analyzed white blood cells from leukemia patients for the presence of aberrant cells in stem cell subfractions. Sixteen patients with CML, six patients with AML, two patients with acute lymphatic leukemia (ALL) and one with chronic myelomonocytic leukemia (CMMOL), all with known cytogenetic abnormalities, were evaluated according to their CD90 (Thy-1)-positive or -negative phenotype. Subsets were sorted on to slides and further characterized by FISH and/or standard cytogenetic testing. The bcr-abl translocation or gross chromosomal abnormalities could be detected in equally high amounts of 92.2% and 89.2% in both stem cell subsets. We conclude, that in progressed AML and CML cells characterized by specific genetic aberrations implicated in the malignant state can be found in the CD34+/CD90+ and CD90- population, thus making CD90 an inappropriate marker to distinguish benign from malignant cells in these leukemias.

Acute Disease↗

PubMed1999

Augmented destruction test with an electromechanical pulsatile total artificial heart.

The total artificial heart (TAH) being developed by these authors successfully completed hydrodynamic and hemolysis studies followed by two acute implantations. Before commencing preclinical studies on any device, documentation of the reliability and durability of each component has to be done. This TAH was submitted to a 4 month destruction test under the most severe driving conditions to detect any weak mechanical components of the system. The maximum dP-dt in the left pumping chamber was chosen of more than 15,000 mm Hg/s, which is almost 6 times higher than that of the normal driving condition. The pump was submersed into a water bath that was maintained at 37 degrees C. The TAH was driven in a left master alternative (LMA) variable rate mode at 8 L/min output flow, 15 mm Hg reload, 100 mm Hg left afterload, and 25 mm Hg right afterload. The outflow, pressures, and temperature inside the TAH were monitored. Several stress concentration areas were detected. The connection between the roller nut and support plate proved to be one of the most stressed regions, and a more reliable fixing procedure was required. This portion was redesigned to offer a durable connection. No malfunctions of the actuator or controller were detected throughout the testing duration. No temperature elevation more than 1 degree C on the center piece of the TAH was demonstrated.

Equipment Failure Analysis↗

PubMed1999

Botulinum toxin A in the management of spastic gait disorders in children and young adults with cerebral palsy: a randomized, double-blind study of "high-dose" versus "low-dose" treatment.

The present study was performed to assess dose-response relationships of local botulinum toxin A (BtxA) treatment in children and teenagers with spastic gait due to cerebral palsy (CP) in a randomized, double-blind study employing a "high-dose" (200 units Botox per leg) and a "low-dose" (100 units Botox per leg) treatment arm in 33 patients with CP. Response parameters included changes in muscle tone assessed by the Ashworth scale at knee joint, range-of-motion (ROM) measurements at knee and ankle joint, objective analysis of longitudinal gait parameters as well as subjective assessments of improvement. Patients in the "high-dose" arm received 40-80 units Botox/muscle versus 20-40 units Botox/muscle in the "low-dose" group. Patients in both treatment arms showed significant improvement of Ashworth score (p<0.001) and ROM (p<0.01), while gait analysis revealed significant increase in gait velocity (p<0.01) and stride-length (p<0.001) over baseline. Subjects in the "high-dose" group showed significantly greater improvement on objective response measurements compared to "low-dose" patients. Also, children aged 7 years or less had greater functional benefit compared to the subgroup of patients older than 7 years. Incidence and severity of side-effects were similar in both treatment groups. The present study demonstrated dose-dependent functional improvement of dynamic deformities and spastic gait pattern in children and young adults with CP treated with local injections of botulinum toxin. A dose of 200 units Botox per leg distributed to 4 or 5 muscle bellies per leg is superior compared to 100 units Botox per leg without significantly affecting the risk of side-effects.

Adolescent↗

PubMed1999

[Selection of a cochlear implant and results of implantation].

BACKGROUND: The cochlear implant is the therapy of choice for completely deaf patients. With this concept speech communication can be restored or developed. METHODS: The selection of the implant system may depend on different factors. As long as speech competence is thought to be of highest importance, a fast stimulation strategy involving a state of the art cochlear implant that provides 1500 Hz for every single electrode is close to the optimum. Further parameters include deep insertion into the cochlea (normally 30 mm) and other technical details. RESULTS: With these conditions fulfilled, about 2/3 of the adult deaf patients will later be able to use a telephone. Children who received implants in due time and do not have other cerebral defects will be partly able to meet with the requirements of normal schools.

Adolescent↗

PubMed1999

Bone mineral content and collagen defects in osteogenesis imperfecta.

Whole-body and spine dual-energy X-ray absorptiometry was done in 63 patients with osteogenesis imperfecta aged 5 to 63 y, and the results were compared with OI types and collagen defects. Bone mineral content (BMC)-for-age, bone area (BA)-for-age, bone mineral density (BMD)-for-age, and BMC-for-BA were reduced, especially in patients with OI III/IV and/or in those with a qualitative collagen defect. BA-for-height was normal. Some patients with OI I and/or a quantitative collagen defect had BMD at or above -2 z-scores. We conclude (i) that both BMC and BMD differ significantly between OI types and collagen defects, (ii) that reduced BMC-for-age in OI patients is due mainly to reduced height ("short bones") and reduced BMC-for-BA ("light bones"), whereas BA-for-height ("bone width") is normal, (iii) that most OI patients have lower than average BMC, but in some mildly affected patients brittleness may exist with only small reductions in BMC.

Absorptiometry, Photon↗

PubMed1999

Secular changes in anthropometric data in cystic fibrosis patients.

The aim of this study was to study the secular changes in anthropometric data over calendar time in patients with cystic fibrosis (CF). Growth curves were constructed for 270 patients based on height and weight registrations from the medical files. Height, body mass index (BMI), magnitude of pubertal peak height velocity (PHV) and age at PHV were analysed for possible secular changes from the 1960s to the 1990s. There was a significant change in height over calendar time in only 1 of 12 age groups. BMI showed a significant increase in 10- and 15-y-old boys and girls and in 5-y-old girls. The magnitude of PHV changed significantly over time, whereas age at PHV was constant. No significant changes in height and age at PHV over calendar time were observed; this was probably due to a selection bias since the oldest patients, who survived to be part of the present investigation, represented milder forms of the disease. The increase in BMI and change in magnitude of PHV over calendar time may reflect the improvement in treatment leading to a better survival and clinical status through puberty. The increase in BMI and change in magnitude of PHV were sufficient to overcome the selection bias from older patients with milder disease.

Adolescent↗

PubMed1999

Comparative analysis of gait in Parkinson's disease, cerebellar ataxia and subcortical arteriosclerotic encephalopathy.

Quantitative gait analysis has been used to elucidate characteristic features of neurological gait disturbances. Although a number of studies compared single patient groups with controls, there are only a few studies comparing gait parameters between patients with different neurological disorders affecting gait. In the present study, gait parameters were compared between control subjects, patients with parkinsonian gait due to idiopathic Parkinson's disease, subjects suffering from cerebellar ataxia and patients with gait disturbance due to subcortical arteriosclerotic encephalopathy. In addition to recording of baseline parameters during preferred walking velocity, subjects were required to vary velocity from very slow to very fast. Values of velocity and stride length from each subject were then used for linear regression analysis. Whereas all patient groups showed slower walking velocity and reduced step length compared with healthy controls when assessed during preferred walking, patients with ataxia and subcortical arteriosclerotic encephalopathy had, in addition, increased variability of amplitude and timing of steps. Regression analysis showed that with changing velocity, subjects with Parkinson's disease changed their stride length in the same proportion as that measured in controls. In contrast, patients with ataxia and subcortical arteriosclerotic encephalopathy had a disproportionate contribution of stride length when velocity was increased. Whereas the findings in patients with Parkinson's disease can be explained as a reduction of force gain, the observations for patients with ataxia and subcortical arteriosclerotic encephalopathy reflect an altered spatiotemporal gait strategy in order to compensate for instability. The similarity of gait disturbance in subcortical arteriosclerotic encephalopathy and cerebellar ataxia suggests common mechanisms.

Adult↗

PubMed1999

Impaired thymopoietic potential of immature CD3(-)CD4(+)CD8(-) T cell precursors from SIV-infected rhesus monkeys.

Immature thymocyte subpopulations were examined for their capacity to differentiate in a newly developed xenogeneic monkey-mouse fetal thymus organ culture (FTOC) system. We provide evidence for impaired precursor function of CD3(-)CD4(+)CD8(-) thymocytes after in vivo infection with SIVmac251 as indicated by a reduced cell number per FTOC and a lower percentage of thymocytes with more mature phenotypes. Addition of recombinant SIV glycoprotein 120 (rgp120) also resulted in a dose-dependent impairment of T cell maturation in FTOC. The data suggest that in patients infected with HIV, T cell maturation and thus replenishment of peripheral pools may be compromised as a result of intrathymic infection or circulating viral gp120.

Animals↗

PubMed1999

Ventricular assistance for recovery of cardiac failure.

Mechanical assist devices have been used increasingly to support patients who await heart transplantation. The initial goal was to provide sufficient circulatory function to keep these patients alive and to allow them to recover from secondary organ dysfunction. A recent observation showed an improvement in native heart performance in some transplant candidates who receive support with mechanical assist devices. Under these conditions, myocardial recovery has been mostly restricted to patients with primary dilated cardiomyopathy and with extended periods of ventricular support. Also, the exact mechanisms that lead to substantial myocardial recovery remain unknown. Several investigations have demonstrated improved myocyte performance, reduced fibrosis, reduced cytokine levels, and reduced autoantibodies during long-term mechanical support; therefore, the observation of cardiac recovery during mechanical support is in accordance with currently discussed models of end-stage heart failure.

Heart Diseases↗

PubMed1999

Radiologic anatomy of ventricular assist devices.

Ventricular assist devices (VAD) allow for long-term circulatory support of patients with end-stage heart failure. With the increasing duration of circulatory support, diagnostic imaging plays an important role in the management of patients on a VAD. The aim of our review was to analyze the radiologic features of different VADs. From 1987 to 1996, 319 patients (mean age 42 years, range 3 to 74 years) were treated with a VAD. A Berlin Heart VAD was implanted in 263 of the patients, the univentricular Baxtor Novacor was implanted in three patients, and the univentricular CI Heartmate was implanted in 19 patients. All patients were studied by serial chest radiographs. In addition, 70 patients underwent computed tomography (CT), and five patients underwent electron beam CT. The Berlin Heart VAD was used as a biventricular support system in 218 patients. In all cases, the position of the wire-directed cannulae was identified on the chest radiographs, while the exact position of the cannula tip could be visualized by CT only. The plastic cannulae of both the Novacor and the Heartmate were not discernible on radiographs, but required CT for evaluation. Computed tomography also resolved the metal components of the pumps. The titanium-made pump housing of the Heartmate caused beam-hardening artefacts that might conceal fluid accumulations in the pump pocket. Computed tomography is the standard of reference for examinations of cannula position, pump position, and pump components of ventricular assist devices.

Adolescent↗

PubMed1999

Purification of the trehalase GMTRE1 from soybean nodules and cloning of its cDNA. GMTRE1 is expressed at a low level in multiple tissues.

Trehalose (alpha-D-glucopyranosyl-1,1-alpha-D-glucopyranoside), a disaccharide widespread among microbes and lower invertebrates, is generally believed to be nonexistent in higher plants. However, the recent discovery of Arabidopsis genes whose products are involved in trehalose synthesis has renewed interest in the possibility of a function of trehalose in higher plants. We previously showed that trehalase, the enzyme that degrades trehalose, is present in nodules of soybean (Glycine max [L.] Merr.), and we characterized the enzyme as an apoplastic glycoprotein. Here we describe the purification of this trehalase to homogeneity and the cloning of a full-length cDNA encoding this enzyme, named GMTRE1 (G. max trehalase 1). The amino acid sequence derived from the open reading frame of GMTRE1 shows strong homology to known trehalases from bacteria, fungi, and animals. GMTRE1 is a single-copy gene and is expressed at a low but constant level in many tissues.

Amino Acid Sequence↗