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T Voigtländer

Publications and source records attributed to T Voigtländer.

At least 19 recordsLinked to original sources
PubMed2005

High prevalence of subclinical hypothyroidism in patients with Anderson-Fabry disease.

Anderson-Fabry disease is a rare lysosomal storage disorder. It results from a deficiency of the lysosomal alpha-galactosidase A and leads to progressive accumulation of globotriaosylceramide in the endothelium and tissue cells of various organs. Some of the typical clinical findings such as tiredness, dry skin, myalgia and arthralgia as well as vague gastrointestinal complaints are also symptoms of hypothyroidism. Therefore, we studied the thyroid function in patients with Anderson-Fabry disease. Thyroid function was studied in 11 patients (6 female, 5 male) with Anderson-Fabry disease by measuring thyroid-stimulating hormone (TSH) and free thyroxine serum levels. Nine patients had chronic kidney disease with stage 1 and two with stage 5. Subclinical hypothyroidism (normal serum free thyroxine concentrations along with elevated serum TSH levels) was found in 4 of 11 patients (36.4%). Subclinical hypothyroidism was observed in both male and female patients as well as in patients with stage 1 and stage 5 kidney disease. Subclinical hypothyroidism is a common finding in patients with Anderson-Fabry disease, showing an excess prevalence as compared to the normal population. The high frequency seems to be relevant regarding the potential consequences of a hypothyroid state.

Adult↗
PubMed2005

Hormonal profile and fertility in patients with Anderson-Fabry disease.

Anderson-Fabry disease is a glycosphingolipid storage disorder with an X-linked recessive inheritance. The alpha-galactosidase A deficiency leads to a progressive accumulation of globotriaosylceramide in the endothelium and tissue cells of various organs. The kidney, heart and brain are predominantly affected. Reports on endocrine function and fertility rates in patients with Anderson-Fabry disease are sparse. In the present study, we assessed ovarian, testicular and adrenal function in a cohort of patients with Anderson-Fabry disease. Plasma follicle-stimulating hormone, luteinizing hormone, prolactin, estradiol, testosterone, sex hormone-binding globulin, somatotropin, insulin-like growth factor-I and serum cortisol were measured in 13 patients (six female and seven male), currently observed in an outpatient clinic. The profile revealed an undisturbed hormonal function and a normal fertility rate in both male and female Anderson-Fabry patients when compared with the corresponding Austrian population.

Adrenal Cortex Hormones↗
PubMed2004

[Noonan's syndrome with an unusual combination of hypertrophic cardiomyopathy, congenital bicuspid aortic valve, coarctation of the aorta and hypoplastic aortic arch].

Noonan's syndrome is characterized by craniofacial anomalies, i. e. ptosis, webbing of the neck and a deep nuchal hairline, as well as skeletal deformities such as short stature, clinodactyly, pectus carinatum and funnel chest and other organ anomalies, mainly cardiac valve disease, less often testicular retention or kidney malformations. Noonan syndrome presents with aspects similar to Ullrich-Turner syndrome, but can be found in both male and female patients. In about one half of the patients with Noonan's syndrome cardiovascular anomalies occur, mostly anomalies of the right heart (mainly valvular pulmonary stenosis). Aortic stenosis and coarctation of the aorta are rarely seen. We report on a patient with four sequential potentially stenosing and stenosed parts of the proximal systemic circulation: hypertrophic cardiomyopathy, bicuspid aortic valve, coarctation of the aorta and a hypoplastic aortic arch as a part of Noonan's syndrome. This patient presents with a unique combination of anomalies, as he also shows a Madelung like deformity of the wrist.

Adult↗
PubMed2004

[Cardio-MRT. The multimodal functional analysis of the future].

Since initial reports in the early 1990s cardiac magnetic resonance imaging (CMR) has matured and is likely to become an established method for routine cardiac diagnostics. The development of faster gradient-echo sequences and stronger magnetic fields has led to improved temporal and spatial resolution. Myocardial viability can be examined by morphological and functional analysis. Contrast enhanced MRI (ceMRI), perfusion measurements and regional wall motion analysis are the major diagnostic tools. The ability to image in arbitrary double oblique planes provides comprehensive visualization of the heart. The introduction of the MR navigator technique allowed for free-breathing motion corrected 3D coronary MR angiography with improved spatial resolution. Using this approach proximal and mid parts of the coronary arteries have been visualized. Subsequently, sensitivity and specificity for the detection of significant coronary stenoses has been evaluated in a multicenter trial demonstrating good sensitivity and specificity for the detection of significant left main and three vessel disease. However, specificity for the detection of single vessel disease was relatively low. Improved motion compensation techniques and novel imaging sequences (SSFP) are currently under investigation to further refine this technique. Despite these promising results coronary MR-angiography is not likely to replace conventional coronary angiography especially with regard to in-plane spatial resolution, coronary collateralization and in-stent restenosis. In contrast, coronary MR-angiography can provide useful morphological informations including functional analysis of the coronary vascular bed. The combination of a conventional cathlab with CMR may provide CMR-guided myocardial interventions. With further improvements in the catheter technology, CMR interventions using real-time imaging guidance will allow to take advantage of the excellent soft tissue contrast of CMR and the simultaneous visualization of the pulmonary, aortic and coronary vessels. CMR is advantageous for screening and follow-up examinations, and it offers comprehensive assessment of cardiac morphology and function in one single examination.

Cardiomyopathies↗
PubMed2004

[Entrapment-syndrome of the popliteal artery: morphological and functional diagnosis using MRT inclusive provocation test].

A 30-year old man with pain in the right calf was submitted. Typical cardiovascular risk factors were excluded. Contrast enhanced MR-angiography at rest showed occlusion of right popliteal artery with collateral vessels due to an abnormal origin of medial head of gastrocnemius muscle at femoral diaphysis. In contrast to a normal MR-angiography at rest the exercise test detected a lateral deviation and a severe functional stenosis of the left popliteal artery. This was caused by an abnormal medial origin of medial head of gastrocnemius muscle at the inner femoral condylus. The patient has been treated with vascular reconstruction combined with myectomy on the right side and with myectomy only on the left side. These findings showed two different types of popliteal artery entrapment syndrome in one patient. MRI is able to detect the different types especially in different stages. Irreversible damages of popliteal artery in early diagnosed stages may thereby be prevented.

Adult↗
PubMed2003

Is left ventricular hypertrabeculation/ noncompaction a cardiac manifestation of Fabry's disease?

BACKGROUND AND OBJECTIVES: Some types of hypertrophic cardiomyopathy are due to cardiac Fabry's disease. Since left ventricular hypertrabeculation/noncompaction (LVHT) is regarded a subtype of hypertrophic cardiomyopathy, we looked for the alpha-galactosidase levels in blood leukocytes of LVHT patients. METHODS: Included were male patients in whom LVHT was diagnosed between June 1995 and September 2002. Echocardiographic criteria for LVHT were 1) >3 trabeculations protruding from the left ventricular wall, apically to the papillary muscles, visible in 1 image plane, and 2) intertrabecular spaces perfused from the ventricular cavity, as visualised on colour Doppler imaging. Trabeculations were defined as structures with the same echogenicity as the myocardium and moving synchronously with the ventricular contractions. Excluded were patients with known neuromuscular disorders. All patients were asked for systemic manifestations of Fabry's disease and blood tests were taken. The alpha-galactosidase-A activity was determined by means of an established fluorometric assay in blood leukocytes. RESULTS: Forty-one patients were invited and 26 accepted the invitation. The remaining patients had died (n=5), lived abroad (n=5) or were unwilling (n=5). Among the 26 patients, aged 28-78 years, who followed the invitation, one had renal failure due to renal shrinkage and one had suffered from a stroke 3 years previously. Leukocyte alpha-galactosidase levels ranged from 70 to 188 nM/mg Prot/h (normal: > or =42 nM/mg Prot/h). In none of the patients was the alpha-galactosidase level reduced. CONCLUSION: LVHT does not seem to be a manifestation of cardiac Fabry's disease. To definitively exclude Fabry's disease, however, endomyocardial biopsy is required.

Adult↗
PubMed2003

Late improvement of regional wall motion after the subacute phase of myocardial infarction treated by acute PTCA in a 6-month follow-up.

BACKGROUND: The aim of this follow-up study was to investigate the late effects of acute coronary angioplasty (PTCA) on regional wall motion after the subacute phase of myocardial infarction (MI). METHODS AND RESULTS: Seventeen patients were investigated initially at a median of 11 days and again at 6 months after acute PTCA for myocardial infarction (< 8 hours after onset of symptoms) by cardiac magnetic resonance imaging. Corresponding short-axis slices encompassing the left ventricle (LV) were acquired using a standard cine MR for regional wall motion analysis and using delayed contrast enhanced magnetic resonance imaging (ceMRI) for infarct size quantification. The infarct size was similar in the subacute phase and the 6 month follow-up (20.8 and 21.9%, respectively; n.s.). Regional wall motion improved significantly in the area of hyperenhancement [percentage wall thickening (PWT) 21.9% and 37.9%, p < 0.05] in contrast to remote normal myocardium (46.4% and 38.4%; n.s.). Regional wall motion was significantly poorer in transmural compared with nontransmural MI in the subacute stage, and a late improvement could only be observed in transmural MI. CONCLUSION: Transmural areas of hyperenhancement displayed significant late long-term improvement of regional wall motion after acute PTCA, possibly related to prolonged stunning compared with nontransmural areas.

Adult↗
PubMed2002

[Long-term follow-up of patients with acute myocardial infarction treated with primary angioplasty or thrombolysis. Results of the MITRA trial].

Long-term follow-up after treatment with primary angioplasty compared to treatment with thrombolysis in patients with acute myocardial infarction (AMI) remains still to be determined. We therefore analyzed the data of the "Maximal Individual Therapy" in Acute Myocardial Infarction (MITRA-1) Registry. Follow-up data for a median of 17 months after discharge were available in 2090 out of 2195 (95%) AMI patients treated with thrombolysis, as well as 293 out of 312 patients (94%) treated with primary angioplasty. There were only small differences in patient characteristics between the two treatment groups. Compared to patients treated with thrombolysis, those treated with primary angioplasty had a higher prevalence of prior myocardial infarction (16.4% versus 12.2%, p = 0.04), longer prehospital delay: 10 minutes (130 minutes versus 120 minutes, p = 0.002), and a longer door-to-treatment time: 45 minutes (p < 0.001). Primary angioplasty patients were more likely to be treated with beta-blockers (primary angioplasty 79.8% versus thrombolysis 66.2%, p < 0.001) or statins (24.5% versus 16.5%, p < 0.001). There was no difference between the treatment groups for total mortality (p = 0.90) nor for the combined endpoint of death or re-infarction (p = 0.85). However, the combined endpoint of death, re-infarction or percutaneous coronary intervention or coronary bypass surgery was significantly lower in the primary angioplasty group (primary angioplasty 25.6% versus thrombolysis 32.3%, univariate odds ratio 0.72, 95% CI: 0.55-0.95, p = 0.02). This result was confirmed by multivariate analysis after adjusting for confounding parameters (multivariate odds ratio: 0.62, 95% CI: 0.42-0.91). The beneficial effect of primary angioplasty compared to thrombolysis achieved during the hospital stay after an AMI is maintained during a 17 month follow-up. AMI patients treated with thrombolysis were more likely to be treated with either percutaneous coronary intervention or coronary bypass surgery after discharge.

Aged↗
PubMed2002

Elevated 14-3-3 protein and axonal loss in immunoglobulin-responsive, idiopathic acute transverse myelitis.

OBJECTIVES: To report the elevation of the 14-3-3 protein and the complete denervation of hand muscles in idiopathic acute transverse myelitis (IATM) of the cervical cord. CASE DESCRIPTION: In a 29-year-old woman with a 2-week history of neck pain and repeated attenuated flus, subacute quadriplegia, hypaesthesia of both arms, a T3 sensory level, and urinary dysfunction occurred. Based upon the clinical findings, the cervical MRIs, and an elevated 14-3-3 protein in the CSF, IATM C4-C7 was diagnosed. Ten, 17, 28 and 61 days after onset, nerve conduction studies revealed complete denervation of the right abductor pollicis brevis and abductor digiti minimi muscles but gradual improvement of the compound muscle action potential of the left abductor pollicis brevis muscle. F-waves of the right median nerve were absent. Tibial somatosensory evoked potentials showed a prolonged central conduction time. Transcranial magnetic stimulation evoked a response in the left but not the right abductor digiti minimi muscle. CONCLUSION: IATM may cause elevation of the 14-3-3 protein and loss of motor axons originating from affected anterior horn cells.

14-3-3 Proteins↗
PubMed2002

Non-invasive magnetic resonance imaging of coronary bypass grafts. comparison of the haste- and navigator techniques with conventional coronary angiography.

OBJECTIVE: The aim was the study was to evaluate the patency of coronary artery bypass grafts with the 3D Navigator and the Haste magnetic resonance imaging techniques. MATERIAL AND METHODS: Thirty-four patients with 82 grafts (18 internal mammarian artery and 64 saphenous venous grafts) were examined at 1.5 T using a phased arrray body coil. A 2D T-2 weighted turbo spin echo sequence (Haste) and a 3D-angiography sequence in navigator technique were used. Reference method was the conventional coronary angiography that was performed within 7 days. RESULTS: With the HASTE sequence 54 of 56 patent and 12 of the 16 occluded grafts were recognized, the sensitivity and specificity was 96 and 75%. The distal anastomosis could be assessed in 28 of 76 cases. With the Haste sequence 54 of the 56 patent and 12 of the 14 occluded grafts were detected correctly. Eight patent and two occluded grafts were judged as not assessable. The sensitivity and specificity was 96 and 85%. Sixty-six percent (50/76) of the distal anastomosis were identified correctly. With both sequences none of the bypass stenosis could be detected. CONCLUSIONS: The patency of coronary bypass grafts can be evaluated noninvasively with a sensitivity of 96% and a specificity of 76%. No inference can be made with respect to the nature of bypass graft stenosis. Better results can be expected with the development of a blood pool contrast medium, an improvement of the spatial resolution and in the combination of flow measurements.

Aged↗
PubMed2002

[Evaluation of myocardial perfusion reserve in patients with CAD using contrast-enhanced MRI: a comparison between semiquantitative and quantitative methods].

OBJECTIVE: Comparison between two semiquantitative methods and a quantitative evaluation of myocardial blood flow (MBF) for assessment of myocardial perfusion reserve (MPR) in patients with CAD. MATERIAL AND METHODS: 9 patients with coronary stenoses > 50 % were examined with an ECG-gated Saturation Recovery Turbo FLASH sequence by using Gd-DTPA as contrast agent (CA). The entive measurements were performed both during rest and hyperemia induced by adenosine. The up-slopes of the signal-time S(t) curves in the myocardium and left ventricular (LV) cavity were evaluated by a linear fit. MPR was calculated from the original up-slopes of the myocardial S(t) curves and from the up-slopes, which were normalized to the up-slopes of the LV S(t) curves, respectively. For quantification of MBF values, the mathematical model MMID 4 was used and MPR was evaluated from the MBF values. RESULTS: With all tested methods, MPR was reduced in myocardial regions subtended by arteries with stenoses >/= 70 % compared with remote regions. With MMID 4 and the normalized up-slope method, differences between severe ischemic and remote regions were statistically significant. CONCLUSION: The up-slope method with normalization and quantification with MMID 4 are more sensitive methods to differentiate between remote and ischemic myocardium than the up-slope method without normalization.

Adult↗
PubMed2002

[Morphologic and functional assessment of vascular abnormalities of the pulmonary vasculature by breath-hold MR techniques].

PURPOSE: To evaluate breath-hold MR techniques for morphologic and functional assessment of vascular abnormalities of the pulmonary vasculature. PATIENTS AND METHODS: 13 patients aged 11 to 60 years with different vascular abnormalities of the pulmonary vasculature (5 patients with 16 arteriovenous malformations, 8 patients with partial anomalous pulmonary venous return) underwent MR imaging at 1.5 T. For morphological assessment, a contrast-enhanced 3D MR angiography (ce-MRA) was performed after a timing run. Segmented cine- and velocity-encoded GRE sequences were used for delineation of associated cardiac septal defects and for determination of systemic left-to-right or intrapulmonary shunt volumes. Selective intra arterial digital subtraction angiography, cardiac catheterization, and the intraoperative situs served as reference standards. RESULTS: Ce-MRA allowed for detection of all vascular abnormalities and for anatomic characterization of 14/16 arteriovenous malformations. Flow measurements in the feeding arteries allowed for determination of intrapulmonary shunt volumes in 4/5 patients. Flow measurements performed in the pulmonary arteries and the ascending aorta enabled determination of systemic left-to-right shunting in patients with anomalous pulmonary venous return. Cine-sequences clearly depicted associated cardiac septal defects. CONCLUSION: Breath-hold MR techniques allow for morphological and functional characterization of vascular anomalies of the pulmonary vasculature. Therefore, they are the non-invasive method of choice for planning further treatment.

Adolescent↗
PubMed2002

[Prospective feasibility study of chest X-ray vs. thoracic MRI in breath-hold technique at an open low-field scanner].

PURPOSE: MR investigations using a breath-hold sequence at an open low-field MR had to be compared to chest X-rays in patients with a wide spectrum of cardio-thoracic pathologies. MATERIAL AND METHODS: 114 patients and three volunteers who actually received a chest X-ray due to different indications underwent triplanar breath-hold (17 - 20 s) True-FISP sequence using a 0.2 T low-field MR (Siemens Magnetom Open, TR/TE/alpha: 7.3/3.5/80 degrees, SD: 10 mm, Pixel: 2.81 x 1.41 mm) a mean of 5.1 (+/- 8.2) days later. RESULTS: Signal-to-noise ratio as basics for pattern recognition was 3.2 in nodule, 5.0 in infiltration, and 12.0 in effusion, and therefore True-FISP is usable for the detection of these findings. MRI demonstrated nodules (89 % vs. 57 %), infiltration (81 % vs. 71 %), pleural effusions (86 % vs. 75 %), pericardial effusions (100 % vs. 21 %) and pulmonary congestion (90 % vs. 80 %) clearly more frequently compared to chest X-ray. DISCUSSION: MRI of the lung has been implemented successfully at an open low-field MR system. Diagnostic safety and accuracy are at least comparable to those of chest X-ray. The lack of superimposition led to a major improvement in the detection of pericardial effusions and nodules, and an increase in identification of infiltration, pleural effusion, and pulmonary congestion.

Adolescent↗
PubMed2001

Primary angioplasty versus intravenous thrombolysis in acute myocardial infarction: can we define subgroups of patients benefiting most from primary angioplasty? Results from the pooled data of the Maximal Individual Therapy in Acute Myocardial Infarction Registry and the Myocardial Infarction Registry.

OBJECTIVES: We sought to determine the effectiveness of primary angioplasty compared with thrombolysis in clinical practice. BACKGROUND: In clinical practice, primary angioplasty for the treatment of acute myocardial infarction (AMI) has not yet been proven more effective than intravenous thrombolysis, nor have subgroups of patients been identified who would perhaps benefit from primary angioplasty. METHODS: The pooled data of two AMI registries--the Maximal Individual TheRapy in Acute myocardial infarction (MITRA) study and the Myocardial Infarction Registry (MIR)--were analyzed. A total of 9,906 lytic-eligible patients with AMI, with a pre-hospital delay of < or =12 h, were treated with either primary angioplasty (n = 1,327) or thrombolysis (n = 8,579). RESULTS: Despite differences in the patients' characteristics and concomitant diseases between the two groups, the prevalence of adverse risk factors was balanced. Univariate analysis of hospital mortality showed a more favorable course for patients treated with primary angioplasty: 6.4% versus 11.3% (odds ratio [OR] 0.54, 95% confidence interval [CI] 0.43 to 0.67). This was confirmed by logistic regression analysis (multivariate OR 0.58, 95% CI 0.44 to 0.77). Primary angioplasty was associated with a lower mortality in all subgroups analyzed. We observed a significant correlation between mortality and absolute risk reduction (r = 0.82, p < 0.0001) in the different subgroups: as mortality increased, there was an increase in absolute benefit of primary angioplasty compared with thrombolysis. CONCLUSIONS: These large registry data showed the effect of primary angioplasty to be more favorable than thrombolysis for the treatment of patients with AMI in clinical practice. This effect was not restricted to special subgroups of patients. As mortality increased, the absolute benefit of primary angioplasty also increased.

Aged↗
PubMed2001

Serotonergic nuclei of the raphe are not affected in human ageing.

Sleep disorders increase with ageing. The serotonergic system has been linked with sleep regulation. In fatal familial insomnia, a prion disease with insomnia as one major clinical feature, we recently observed a disturbance in the serotonergic system as likely substrate of typical symptoms. Using immunohistochemistry for the serotonin synthesizing enzyme, tryptophan hydroxylase, we investigated the serotonergic median raphe nuclei (dorsal raphe nucleus, superior central nucleus, and raphe obscurus nucleus) in brains of an older (n = 12; age range 62-84 years) and a younger group (n = 10; age range 5-29 years). We found no significant difference between age groups in the percentage of neurons able to synthesize serotonin. Other changes might relate to sleep disturbances in the elderly.

Adolescent↗
PubMed2001

Ellis-van Creveld syndrome: a generalized dysplasia of enchondral ossification.

We report a 25-week fetus with lethal Ellis-van Creveld syndrome who was diagnosed prenatally from the US detection of a narrow chest, postaxial polydactyly of the hands, short acro-/mesomelic limbs and a ventricular septal defect. The postnatal radiographic features of the skeleton confirmed the diagnosis. Literature review of the histopathology of the physeal growth plate is contradictory, varying between retardation of the hypertrophic chondrocytes without disorganization and marked disorganization of the proliferating chondrocytes. We investigated numerous sites of the enchondral ossification and observed retardation of the physeal growth plate in all sites and retardation with pronounced disorganization of the physeal growth plate in the upper mesomelic bone segments only. These data support the concept that Ellis-van Creveld syndrome is mainly a generalized disorder of the maturation of enchondral ossification.

Adult↗
PubMed2001

Contrast-enhanced three-dimensional MR angiography of the thoracic aorta: experiences after 118 examinations with a standard dose contrast administration and different injection protocols.

The aim of this study was to test three injection protocols for contrast-enhanced magnetic resonance angiography (MRA) of the thoracic aorta with a standard-dose application. Ninety-three patients with a total of 118 examinations underwent MRA of the thoracic aorta at 1.5 T. There were three injection protocols: in 24 cases, no test bolus was performed and contrast was injected manually; in 14 cases, contrast was injected manually after a test bolus; and in 80 cases, a MR-compatible injector was used after a timing examination. All patients received 20 ml of Gd-DTPA. Quantitative signal-to-noise (SNR) measurements were obtained at different locations in the thoracic aorta, the pulmonary arteries, and the superior vena cava. Two readers in conference retrospectively evaluated each examination with respect to overall image quality and quality of bolus timing. Bolus timing was considered optimal in 70 cases, and either too early or too late in 11 cases. In 37 examinations the bolus was broadened. The SNR measurements of the thoracic aorta revealed that examinations after bolus testing were significantly superior to examinations without a test bolus (p < 0.001). Signal intensity ratios of the aorta and the pulmonary trunk were significantly higher in examinations with an optimal contrast timing (p < 0.001). Magnetic resonance angiograms of the thoracic aorta with a timing run are significantly superior to non-timed examinations with respect to image quality and SNRs. The administration of 20 ml of Gd-DTPA is sufficient for adult patients.

Adolescent↗