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Biomedical subjects

P Günther

Publications and source records attributed to P Günther.

18 recordsLinked to original sources
PubMed2007

[Wilson's disease].

Wilson's disease is a rare autosomal recessive disorder of hepatic copper transport leading to a biliary excretion inhibition of copper. Overload of the metal mainly in liver and basal ganglia leads to hepatic but also to extrapyramidal motor as well as psychiatric clinical symptoms. Diagnosis is based on clinical suspicion, parameters of copper metabolism, ophthalmic examination and a liver biopsy. A radiocopper test is able to identify patients even with inconsistent laboratory results. For initial assessment and follow-up neurophysiological investigation and MRI are recommended additionally. Genetic analysis can be helpful to detect asymptomatic relatives of the index patient. Dependent on the stage of the disease for therapy chelating drugs and zinc are possible but must be given lifelong without longer interruptions. With early diagnosis and consequent treatment the prognosis of Wilson's disease is excellent and usually the need for liver transplantation can be prevented.

Adolescent↗
PubMed2006

[Correlation of clinical aspects as well as genotype and phenotype in Wilson's disease on the basis of epidemiologic, clinical and cranial MRI findings].

Wilson's disease, a rare autosomal recessive disorder of hepatic copper transport, is characterized by a varying pattern of hepatic, neurologic and psychiatric symptoms. Currently, about 250 causative mutations of the ATP 7B gene are known. However, a correlation between genotype and phenotype according to these mutations is not yet clear. To elucidate a possible correlation in this study 39 patients with Wilson's disease were subdivided into three groups according to the underlying mutation in group I for homocygote respectively group II for compound heterocygote mutation in H1069Q and group III for other mutations. Clinical subtype and extent of neurologic disturbance as well as epidemiologic aspects, presence of psychiatric symptoms, results of acustically evoked potentials (Wave III, interpeak latency III-V) and findings of cranial MRI were considered. While psychopathological symptoms, the results of acustically evoked potentials and cranial MRI show a correlation to the clinical subtype of Wilson's disease there was no genotype-phenotype correlation on the basis of the mutation in H1069Q. The qualitative and quantitative pattern of results do not show any significant differences in the three groups of genotype. Thus, the time of treatment onset still has most influence on the extent of clinical manifestation and reversibility of the toxic copper accumulation.

Adenosine Triphosphatases↗
PubMed2006

[Computer-assisted operational planning for pediatric abdominal surgery. 3D-visualized MRI with volume rendering].

Exact surgical planning is necessary for complex operations of pathological changes in anatomical structures of the pediatric abdomen. 3D visualization and computer-assisted operational planning based on CT data are being increasingly used for difficult operations in adults. To minimize radiation exposure and for better soft tissue contrast, sonography and MRI are the preferred diagnostic methods in pediatric patients. Because of manifold difficulties 3D visualization of these MRI data has not been realized so far, even though the field of embryonal malformations and tumors could benefit from this.A newly developed and modified raycasting-based powerful 3D volume rendering software (VG Studio Max 1.2) for the planning of pediatric abdominal surgery is presented. With the help of specifically developed algorithms, a useful surgical planning system is demonstrated. Thanks to the easy handling and high-quality visualization with enormous gain of information, the presented system is now an established part of routine surgical planning.

Abdomen↗
PubMed2006

[Testicular torsion: diagnosis, differential diagnosis, and treatment in children].

Acute scrotum represents an emergency situation although testicular torsion is present in less than 20% of the cases. Sonography has meanwhile become the definitive modality for diagnosis. Its increasing use before surgical intervention has led to technical improvements in ultrasound diagnostics and critical assessment of ultrasound criteria to exclude testicular torsion as well as standardization of examination procedures. Central arterial and venous perfusion shown to be bilaterally equal on Doppler sonography is the most important criterion for excluding torsion. This article discusses other criteria such as the "resistance index," comparison of parenchymal structure of both testes, evidence for spermatic cord torsion, or differences between the sides in perfusion of the testicular parenchyma and highlights the difficulties involved in partial and intermittent testicular torsion. Alternative investigative methods and the significance of sonography in the differential diagnosis of other underlying causes are addressed. In summary, the combination of interpreting B-mode imaging, color Doppler, and power Doppler sonography and analyzing Doppler flow curves after clinical examination results in successful and conclusive evaluation of the testes in cases of acute scrotum in boys.

Child↗
PubMed2005

[Classification of Wilson's disease based on neurophysiological parameters].

In addition to hepatolenticular degeneration in Wilson's disease, sensory and extrapyramidal motoric systems are also disturbed. In this study a classification on the basis of neurophysiological parameters (EAEP, VEP, MSEP, TSEP and MEP) was established according to subclinical disturbances of these pathways in patients with Wilson's disease on long-term treatment. A cluster analysis of latencies of these evoked potentials was performed. The results of cluster analysis revealed three types of electrophysiological profiles: type I with normal latencies and types II and III showing different patterns of prolonged latencies. However, there was no correlation between clinical classification and the results of cluster analysis based on the electrophysiological data. The neurophysiological based classification provides additional information about central manifestations and aids in characterizing the progress of the disease.

Adult↗
PubMed2005

[Tumour necrosis factor receptor-associated periodic syndrome. A rare differential diagnosis of multiple sclerosis].

Tumour necrosis factor receptor-associated periodic syndrome (TRAPS), a rare autosomal dominant disorder, is characterised by recurrent attacks of fever, myalgias, and abdominal pain. However, manifestations in the central nervous system are hardly known. We describe a family in which one of three affected members developed central nervous system symptoms. First diagnosed as multiple sclerosis (MS), the demyelinisation seems to be a feature of TRAPS rather than MS. The syndrome is discussed as a rare differential diagnosis of MS.

Adult↗
PubMed2005

[Childhood kidney tumors -- the relevance of imaging].

Kidney tumors represent 6.2% of malignant tumors in children. History, clinical course and radiological findings are necessary elements in the differential diagnosis of the different renal tumors. In the case of nephroblastoma, chemotherapy is based solely on the radiological diagnosis without prior histology. In therapy-optimizing studies of the Society of Pediatric Oncology and Hematology, preoperative chemotherapy is performed. Therapy monitoring is performed in the course of and after preoperative chemotherapy to verify tumor response. Radiological staging plays a significant role in deciding on further treatment and in operative planning. Three-dimensional visualization of the abdominal situs can assist preoperative planning. In summary, diagnostic imaging in renal tumors in children plays a role in differential diagnosis, staging, monitoring of therapy, and surgical planning.

Abnormalities, Multiple↗
PubMed2004

[3D-visualization by MRI for surgical planning of Wilms tumors].

PURPOSE: To improve surgical planning of kidney tumors in childhood (Wilms tumor, mesoblastic nephroma) after radiologic verification of the presumptive diagnosis with interactive colored 3D-animation in MRI. MATERIALS AND METHODS: In 7 children (1 boy, 6 girls) with a mean age of 3 years (1 month to 11 years), the MRI database (DICOM) was processed with a raycasting-based 3D-volume-rendering software (VG Studio Max 1.1/Volume Graphics). The abdominal MRI-sequences (coronal STIR, coronal T1 TSE, transverse T1/T2 TSE, sagittal T2 TSE, transverse and coronal T1 TSE post contrast) were obtained with a 0.5T unit in 4 - 6 mm slices. Additionally, a phase-contrast-MR-angiography was applied to delineate the large abdominal and retroperitoneal vessels. A notebook was used to demonstrate the 3D-visualization for surgical planning before surgery and during the surgical procedure. RESULTS: In all 7 cases, the surgical approach was influenced by interactive 3D-animation and the information found useful for surgical planning. Above all, the 3D-visualization demonstrates the mass effect of the Wilms tumor and its anatomical relationship to the renal hilum and to the rest of the kidney as well as the topographic relationship of the tumor to the critical vessels. One rupture of the tumor capsule occurred as a surgical complication. For the surgeon, the transformation of the anatomical situation from MRI to the surgical situs has become much easier. CONCLUSION: For surgical planning of Wilms tumors, the 3D-visualization with 3D-animation of the situs helps to transfer important information from the pediatric radiologist to the pediatric surgeon and optimizes the surgical preparation. A reduction of complications is to be expected.

Age Factors↗
PubMed2004

Abdominal tumours in children: 3-D visualisation and surgical planning.

INTRODUCTION: Solid abdominal tumours are of special importance in the field of paediatric surgery. Because of the dangers of cumulative irradiation and improved delineation of soft parts MRI is usually employed in children for diagnostic assessment. Compiling the radiologic information for surgical planning is often difficult by conventional methods. Newly improved and efficient 3-D volume rendering software is now available for visual reconstruction of tumour anatomy utilising segmentation and other special techniques. Because the intraoperative complication rate is close to 20 % as described in the literature, optimal preoperative visualisation and planning would seem imperative. MATERIALS AND METHODS: All children with solid abdominal tumours at Heidelberg University in the year 2002 were included in this study. MR examinations were performed with a 0.5 Tesla magnet using a standard protocol. All MR data were processed with VG Studio Max 1.1, converting the two-dimensional data into three-dimensional data. RESULTS: This report presents 15 cases using this special technique: 7 with abdominal neuroblastoma, 6 with nephroblastoma, 1 ganglioneuroma, and 1 ovarian teratoma. CONCLUSIONS: Our experience shows that a better understanding of the surgical anatomy, particularly regarding the surrounding organs and vasculature, can be helpful in decreasing the incidence of inadvertent intraoperative injuries to these structures.

Abdominal Neoplasms↗
PubMed2002

[Corrective interventions after elbow para-articular fractures in childhood].

Besides supracondylar fracture of the humerus there are several injuries of the elbow joint, which may lead to major disability. In this study 5 cases of corrective procedures are described after elbow fractures. Initially the lesions were overlooked. These were a fracture of the radial condyle, producing a pseudarthrosis, three cases of Monteggia fractures with persisting dislocation of the radial head in young children and a periarticular calcification issuing from an avulsion of the radial epicondyle and the radial capsule in a 13-year-old. All children had marked functional limitation of the elbow joint. The primarily overlooked fractures were corrected early. Various osteosynthesis procedures including movement and distraction extend fixator were employed. Overall, in all patients an almost complete movement of the joint at existing stability could be achieved. Transcondylar and Monteggia fractures should not be overlooked at the initial diagnosis as secondary operations for correction always have a less favorable outcome than the primary one. For the management of ankylosis of the elbow a movement extend fixator after distraction is a useful additional management.

Adolescent↗
PubMed2002

[Cerebral MRI and evoked potentials in Wilson disease. Comparison of findings in patients with neurological follow-up].

Wilson's disease is caused by toxic copper accumulation, which leads predominantly to hepatic and basal ganglia damage. Characteristic findings in MRI and electrophysiologic examinations are described according to the occurrence of neurological symptoms. In the present study, 28 patients suffering from Wilson's disease (neurological type) were investigated. The results of MRI are compared with abnormalities of evoked potentials (BAEP, MSEP, T-VEP, MEP). All patients show hypodensities in the basal ganglial area (putamen and GI. pallidus) regularly combined with atrophy of the cerebrum and cerebellum in MRI. Signal abnormalities in the mesencephalic region (46% occurrence) and Nc. dentatus (36% occurrence) are combined with the other findings in variable patterns. Only slight changes are found in the pontine region. BAEP are disturbed in 71% of all cases and MSEP in 46%. Combined abnormalities of BAEP and MSEP were found in 39%. Pathological values occurred with a lower frequency in T-VEP (36%) and MEP (39%). The comparison of MRI findings with electrophysiological data done separately for each patient reveals no strong correlation between both methods. Individual MRI findings do not correspond with the patterns of disturbed evoked potentials and vice versa. Therefore we conclude that these methods, MRI and electrophysiological evaluation, supplement each other. Magnetic resonance imaging and electrophysiological evaluation should be performed simultaneously for therapy monitoring.

Adult↗
PubMed1997

Repeated administration of tacrine to normal rats: effects on cholinergic, glutamatergic, and GABAergic receptor subtypes in rat brain using receptor autoradiography.

Tacrine, a potent acetylcholinesterase inhibitor, has been reported to improve cognitive function in patients with Alzheimer's disease. The present investigation was conducted to elucidate in vivo any interaction between tacrine-induced cortical cholinergic hyperactivity and glutamatergic and GABAergic neurotransmission, which might influence the therapeutic potential of tacrine. Seven days after a daily dosage of 10 mg/kg tacrine i.p. quantitative receptor autoradiography was performed in coronal sections throughout the brain. Repeated administration of tacrine resulted in decreased binding to high-affinity choline uptake, nicotinic and M2-muscarinic acetylcholine receptor sites in a number of cortical regions, while reductions in M1-muscarinic receptor binding were restricted to the cingulate and entorhinal cortex as well as caudate-putamen. Moreover, tacrine injections decreased cortical AMPA receptor binding throughout the brain, while NMDA, kainate, and GABAA receptor binding remained unchanged. Tacrine administration alters cortical AMPA receptor binding in the opposite direction to that observed in patients with Alzheimer's disease, suggesting that tacrine may exert a reversal in up/down-regulation of cortical glutamate receptor subtypes in Alzheimer patients. However, the drug-induced reductions in cortical high-affinity choline uptake sites as well as in nicotinic and in muscarinic acetylcholine receptor binding might partially counteract the cognition-enhancing effects of tacrine produced by acetylcholinesterase inhibition.

Animals↗
PubMed1978

[Experimental studies on the castration osteoporosis in the rat. II. Behavior of bone minerals].

In Wistar rats the bone mineral content of calcium magnesium, phosphor, sodium and potassium was investigated 10 months after complete ovarectomy. 20 of ovarectomized animals obtained in this time 0,65 mg ethinyloestradiolsulfonate/kg/month orally. The ovarectomy decreased significantly the content of calcium and magnesium of femur, humerus, vertebra thoracis and lumbalis. After therapy with the oestrogen these minerals were increased, but not significantly. These results did not correlate with the degree of histomorphometrically established osteoporosis. Phosphor, sodium and potassium were not changed significantly. The role of oestrogen dosis was discussed.

Animals↗
PubMed1976

The ultrastructure of the interstitial cells in human fetal ovaries.

Steroid-active interstitial cells (stromal lutein cells) in human fetal ovaries of 12, 15 and 22 weeks of gestation were studied by means of electron microscope. The large cells, which are predominantly situated in the vicinity of capillaries, show high structural similarity to Leydig cells (type A) of the fetal testis. Both are characterized by extensively development of smooth endoplasmic reticulum, accumulation of lipids, and numerous mitochondria of the tubular type containing osmiophilic inclusions. Whether the interstitial cell system of the fetal ovary has any specific function in developmental physiology of the genital system cannot be decided on the basis of the present results. Its differentiation may only indicate adequate response of competent mesenchymal cells to gonadotropic stimulation.

Endoplasmic Reticulum↗