[Work in progress: adaptation of electronic medical records to the requirements of a university eye clinic : Individual extensions of the software "FIDUS" at the Department of Ophthalmology of the Saarland University Medical Center UKS]. / Work in Progress: Anpassung der elektronischen Patientenakte an die Anforderungen einer Universitätsaugenklinik : Individuelle Erweiterungen der Software "FIDUS" an der Klinik für Augenheilkunde am Universitätsklinikum des Saarlandes UKS.

BACKGROUND: The implementation of electronic medical records (EMR) in the Department of Ophthalmology at the Saarland University Medical Center (UKS) in January 2016 was a timely response to growing documentation requirements and rapidly increasing electronic diagnostic data. The software system was primarily developed for private practices and cannot therefore meet the different requirements of various clinics out of the box. The purpose of this study was to identify features of the EMR beyond purely paper replacement that can assist in the clinical workflow and whether these features can be implemented in a running system. METHODS: The EMR was specifically individualized with respect to the work processes and documentation requirements of the Department of Ophthalmology at the UKS. In addition to a seamless integration into the hospital information system (HIS) the modifications included changes in the structure and visual presentation of the EMR as well as functional extensions. An internet-based platform was set up to enable a direct exchange of appointments and patient data with specialist practices. RESULTS: Due to the introduction of a so-called ghost list the position of patients within the hospital who are allocated to a physician, e.g. for diagnostics, can be reconstructed at any point in time. The logging of the individual treatment times enables tracking of patient flow within the clinic and a reduction of waiting times. Existing paper documents particularly for the graphic recording of findings, such as sketches, are digitalized eliminating the need to scan documents. The UKS.AUGEN.NETZ is an internet-based portal to facilitate direct organization of appointments with specialist practitioners and for the exchange of digital examination data and medical correspondence. CONCLUSION: The permanent close cooperation between employees of the Department of Ophthalmology at the UKS and the manufacturer of the software enables a continuous optimization of the EMR in a fully operational clinical workflow. In addition, the web-based interface improves the cooperation between the hospital and private practices.

[Per aspera ad astra: implementation of electronic patient records in a university eye hospital : Experience with FIDUS in the Clinic for Ophthalmology at the Saarland University Medical Center UKS]. / Per aspera ad astra: Einführung einer elektronischen Patientenakte an einer Universitätsaugenklinik : Erfahrungen mit "FIDUS" in der Klinik für Augenheilkunde am Universitätsklinikum des Saarlandes UKS.

BACKGROUND: For more than 60 years patient records of the Department of Ophthalmology at the Saarland Medical Center in Homburg were documented on handwritten paper documents and filed in archives. Increasing requirements for medical documentation, exploding volumes of diagnostic data, overcrowded archives and unavailable files when needed, were the essential rationales for the implementation of an electronic patient archive (EPA). METHODS: The EPA has been specifically adapted to the requirements of the Department of Ophthalmology in order to guarantee a standardized unobstructed documentation and patient care across all sections of the department. Seamless integration into the clinic information system (KIS) and network-compatible diagnostic software were essential as well as a timely digitization of existing paper charts. Decisive factors for using FIDUS (Arztservice Wente GmbH, Darmstadt, Germany) as the EPA software were the visual layout of the EPA, user friendliness and reference installations of the software in other ophthalmology departments. RESULTS: After one and a half years of meticulous preparation with specific adaptations to the requirements of our department, the EPA was finally implemented in January 2016 and since then we have been working on improvements. The EPA software retrieves basic patient data from the KIS and diagnostic data from electronic devices via various interfaces. Expenses for printers could be reduced but computer workplaces had to be expanded or newly created. For previous patients paper files are digitized externally and stored in an electronic archive directly accessible from the EPA. DISCUSSION: Successful reorganization and implementation of electronic documentation during clinical routine is feasible with careful preparation and timely involvement of information technology experts, motivated physicians, nurses, research departments and the administration.

Reconstruction mechanisms of tantalum oxide coatings with low concentrations of silver for high temperature tribological applications.

Silver tantalate (AgTaO3) coatings have been found to exhibit outstanding tribological properties at elevated temperatures. To understand the mechanisms involved in the tribological behavior of the Ag-Ta-O system, tantalum oxide coatings with a small content of silver were produced to investigate the metastable nature of this self-lubricating material. The coatings were produced by unbalanced magnetron sputtering, ball-on-disk wear tested at 750 °C, and subsequently characterized by X-ray diffraction, Scanning Auger Nanoprobe, cross-sectional Scanning Electron Microscopy, and Transmission Electron Microscopy. Complementary molecular dynamic simulations were carried out to investigate changes in the chemical and structural properties at the interface due to sliding for films with varying silver content. Both the experimental characterization and the theoretical modeling showed that silver content affects friction and wear, through the role of silver in film reconstruction during sliding. The results suggest that the relative amount of silver may be used to tune film performance for a given application.

Fasting and postprandial liver glycogen content in patients with type 1 diabetes mellitus after successful pancreas-kidney transplantation with systemic venous insulin delivery.

BACKGROUND: In patients with type 1 diabetes mellitus (T1DM), insulin is usually replaced systemically (subcutaneously) and not via the physiological portal route. According to previous studies, the liver's capacity to store glycogen is reduced in T1DM patients, but it remains unclear whether this is due to hyperglycaemia, or whether the route of insulin supply could contribute to this phenomenon. T1DM patients after successful pancreas-kidney transplantation with systemic venous drainage (T1DM-PKT) represent a suitable human model to further investigate this question, because they are normoglycaemic, but their liver receives insulin from the pancreas transplant via the systemic route. MATERIALS AND METHODS: In nine T1DM-PKT, nine controls without diabetes (CON) and seven patients with T1DM (T1DM), liver glycogen content was measured at fasting and after two standardized meals employing (13) C-nuclear-magnetic-resonance-spectroscopy. Circulating glucose and glucoregulatory hormones were measured repeatedly throughout the study day. RESULTS: The mean and fasting concentrations of peripheral plasma glucose, insulin, glucagon and C-peptide were comparable between T1DM-PKT and CON, whereas T1DM were hyperglycaemic and hyperinsulinaemic (P < 0·05 vs T1DM-PKT and CON). Total liver glycogen content at fasting and after breakfast did not differ in the three groups. After lunch, T1DM-PKT and T1DM had a 14% and 21% lower total liver glycogen content than CON (P < 0·02). CONCLUSION: In spite of normalized glycaemic control, postprandial liver glycogen content was reduced in T1DM-PKT with systemic venous drainage. Thus, not even optimized systemic insulin substitution is able to resolve the defect in postprandial liver glycogen storage seen in T1DM patients.

Survival and prognostic factors in non-small cell lung cancer patients with spinal bone metastases: a retrospective analysis of 303 patients.

BACKGROUND AND PURPOSE: For palliative care of spinal bone metastases, stability assessment is of crucial importance. Pathological fractures, instability-related patient immobility and the extent of bone metastasis have been reported to affect patient outcome and these parameters have therefore been used for treatment stratification. We report on stability-dependent fracture and survival rates in over 300 non-small cell lung cancer (NSCLC) patients. MATERIALS AND METHODS: Data from 303 patients with 868 osteolytic metastases treated with radiotherapy (RT) between 2000 and 2012 were evaluated retrospectively. RESULTS: In NSCLC patients with bone metastases only, the retrospective 6- and 12-month overall survival (OS) rates were 76.7 and 47.2%, respectively. In patients with additional non-bone distant metastases, these values were 60.0 and 34.0%, respectively. Survival rates were significantly lower in patients with multiple bone metastases and in those suffering pathological fractures (p=0.017). No significant impact of histological type, location of spinal lesions or treatment regime was detected. Furthermore, stability assessment revealed no influence of vertebral column stability on patient outcome (p=0.739). CONCLUSION: Our analysis demonstrated a correlation between the pathological fractures of bone lesions, the number of bone metastases, additional distant metastases and survival. The results offer a rationale for future prospective investigations.

Accuracy of vertical height measurements on direct digital panoramic radiographs using posterior mandibular implants and metal balls as reference objects.

OBJECTIVES: Conventional panoramic radiography, a widely used radiographic examination tool in implant treatment planning, allows evaluation of the available bone height before inserting posterior mandibular implants. Image distortion and vertical magnification due to projection geometry is well described for rotational panoramic radiographs. To assess the accuracy of vertical height measurements on direct digital panoramic radiographs, implants and metal balls positioned in the posterior mandible were used as radio-opaque reference objects. The reproducibility of the measuring method was assessed by the inter- and intraobserver agreements. METHODS: Direct digital panoramic radiographs, performed using a Kodak 8000C (Eastman Kodak Company, Rochester, NY), of 17 partially edentulous patients (10 females, 7 males, mean age 65 years) were selected from an X-ray database gathered during routine clinical evaluation of implant sites. Proprietary software and a mouse-driven calliper were used to measure the radiological length of 25 implants and 18 metal reference balls, positioned in mandibular posterior segments. The distortion ratio (DR) was calculated by dividing the radiological implant length by the implant's real length and the radiological ball height by the ball's real height. RESULTS: Mean vertical DR was 0.99 for implants and 0.97 for balls, and was unrelated to mandibular sites, side, age, gender or observer. Inter- and intraobserver agreements were acceptable for both reference objects. CONCLUSIONS: Vertical measurements had acceptable accuracy and reproducibility when a software-based calibrated measurement tool was used, confirming that digital panoramic radiography can be reliably utilized to determine the pre-operative implant length in premolar and molar mandibular segments.

Cell specific ultrasound effects are dose and frequency dependent.

Ultrasound is widely used in clinical practice, mostly in diagnostic studies, but increasingly in therapeutic applications as well. This may be the case in acceleration of wound healing or treatment of cancer. Still, little is known about the direct effect of frequency or energy density of the ultrasound upon the cells themselves. We therefore investigated the impact of three different protocols using high, medium and low energy densities at three different frequencies on normal endothelial and epithelial as well as carcinoma cell lines (neuroblastoma and adenocarcinoma cell lines). Proliferation of endothelial and epithelial cell lines was significantly increased depending on the frequency and energy density applied. No influence on actin cytoskeleton formation was seen in these cells after treatment, while a significant decrease in the density of microvilli and the length of filopodia in the epithelial cell line could be noted. The proliferation rate of the carcinoma cell lines was reduced and cells destroyed. Apoptosis was induced in the adenocarcinoma cells after ultrasound exposure. Additionally, the expression of neurofilament was increased in neuroblastoma cells as evidence of beginning differentiation. So, different settings of frequency and energy density in an ultrasonic treatment protocol lead to different impacts on proliferation, morphology and differentiation and might be used to stimulate or inhibit the growth of individual cell types.

Stacking of 2D electron gases in Ge probed at the atomic level and its correlation to low-temperature magnetotransport.

Stacking of two-dimensional electron gases (2DEGs) obtained by δ-doping of Ge and patterned by scanning probe lithography is a promising approach to realize ultrascaled 3D epitaxial circuits, where multiple layers of active electronic components are integrated both vertically and horizontally. We use atom probe tomography and magnetotransport to correlate the real space 3D atomic distribution of dopants in the crystal with the quantum correction to the conductivity observed at low temperatures, probing if closely stacked δ-layers in Ge behave as independent 2DEGs. We find that at a separation of 9 nm the stacked-2DEGs, while interacting, still maintain their individuality in terms of electron transport and show long phase coherence lengths (∼220 nm). Strong vertical electron confinement is crucial to this finding, resulting in an interlayer scattering time much longer (∼1000 × ) than the scattering time within the dopant plane.

Field-directed sputter sharpening for tailored probe materials and atomic-scale lithography.

Fabrication of ultrasharp probes is of interest for many applications, including scanning probe microscopy and electron-stimulated patterning of surfaces. These techniques require reproducible ultrasharp metallic tips, yet the efficient and reproducible fabrication of these consumable items has remained an elusive goal. Here we describe a novel biased-probe field-directed sputter sharpening technique applicable to conductive materials, which produces nanometer and sub-nanometer sharp W, Pt-Ir and W-HfB(2) tips able to perform atomic-scale lithography on Si. Compared with traditional probes fabricated by etching or conventional sputter erosion, field-directed sputter sharpened probes have smaller radii and produce lithographic patterns 18-26% sharper with atomic-scale lithographic fidelity.

[Current state of neoadjuvant therapy of soft tissue sarcoma]. / Aktueller Stand der neoadjuvanten Therapie bei Weichteilsarkomen.

The treatment of soft tissue sarcoma is clinically challenging. Referral to an experienced center with an interdisciplinary team is strongly recommended. Neoadjuvant therapy, including irradiation and chemotherapy, has been applied to improve local control rates, eradicate micrometastases and assess chemosensitivity. However, the role of neoadjuvant therapy remains controversial, especially for systemic therapy, as the only available randomized trial failed to prove a benefit for survival. Nevertheless, on the basis of the current body of literature, neoadjuvant therapy can be considered on an individual basis for patients with high-risk tumors. Whenever possible, patients should be included in a clinical trial.

Insulin resistance is not associated with myocardial steatosis in women.

AIMS/HYPOTHESIS: Insulin resistance, an independent risk-factor for cardiovascular disease, precedes type 2 diabetes and is associated with ectopic lipid accumulation in skeletal muscle and liver. Recent evidence indicates that cardiac steatosis plays a central role in the development of diabetic cardiomyopathy. However, it is not known whether insulin resistance as such in the absence of type 2 diabetes is associated with heart steatosis and/or impaired function. We therefore assessed myocardial steatosis and myocardial function in a sample of women with normal insulin sensitivity, insulin resistance, impaired glucose tolerance (IGT) and type 2 diabetes. METHODS: Magnetic resonance imaging and localised spectroscopy were used to measure left ventricular dynamic variables and myocardial lipid accumulation in interventricular septum of non-diabetic, age- and BMI-matched insulin-sensitive (n = 11, 47 ± 6 years, BMI 25 ± 2 kg/m(2); clamp-like index [CLIX] = 9.7 ± 0.7) and insulin-resistant (n = 10, 48 ± 5 years, 27 ± 4 kg/m(2); CLIX = 4.5 ± 0.4) women with normal glucose tolerance as well as of women with IGT (n = 6, 45 ± 5 years, 28 ± 6 kg/m(2); CLIX = 3.6 ± 1.1) and type 2 diabetes (n = 7, 52 ± 10 years, 27 ± 3 kg/m(2)). RESULTS: Myocardial lipid content was increased in type 2 diabetic women only (insulin-sensitive 0.4 ± 0.2% [means ± SD]; insulin-resistant 0.4 ± 0.1%; IGT 0.5 ± 0.2%; type 2 diabetes 0.7 ± 0.3%; p < 0.05). In insulin-resistant and type 2 diabetic women, stroke volume was lower (-15% and -27%, respectively, vs insulin-sensitive) and heart rate was higher (11% and 14%, respectively, vs insulin-sensitive, p < 0.05). No other differences in systolic and diastolic function were observed between study groups. CONCLUSIONS/INTERPRETATION: In contrast to liver and skeletal muscle, insulin resistance as such is not associated with increased myocardial lipid accumulation.

[Merkel cell carcinoma: a highly aggressive tumor with possible viral etiology]. / Merkel-Zell-Karzinom: Ein hochaggressiver Tumor mit möglicher viraler Genese.

Merkel cell carcinoma (MCC) or neuroendocrine carcinoma of the skin, is a rare and highly aggressive tumor which typically develops in chronically sun-damaged skin in aged or immunosuppressed patients. The clinical course is characterized by early local recurrence and lymphatic metastases. The current discussion on the etiology of MCC is dominated by the recently discovered Merkel cell polyoma virus (MCPyV). Apparently, MCPyV infection takes place early in life and the virus can also be found in healthy tissue. Possibly, a mutation of the viral genome is responsible for the development of the tumor. The 5 year survival rate of patients with primary MCC is only 30-40% after surgical therapy alone but can increase to about 75% after additional adjuvant radiotherapy. In cases with lymphatic or distant metastases various chemotherapy protocols in addition to operative and radiation therapy analogous to those for small cell lung cancer therapy have been found to be effective. Nevertheless, very high recurrence rates are typical in patients with distant metastases. Thus, MCC is regarded as chemosensitive but not chemocurable.Patients with MCC should be treated with an aggressive but individually adapted concept. The consequent integration of radiotherapy into the therapeutic approach can improve the prognosis.

[Radiation therapy of lung carcinoma]. / Strahlentherapie des Bronchialkarzinoms.

At first presentation and primary diagnosis approximately 50% of patients with non-small cell lung carcinoma (NSCLC) and 25% of patients with small cell lung carcinoma (SCLC) have a potentially curable tumor stage. Definitive, adjuvant and neoadjuvant radio- (chemo-)therapy play an important role as part of multimodal treatment approaches. High radiation doses can be achieved in tumor areas with modern radiotherapy planning and treatment techniques without an increase of side-effects. The 3 year overall survival after primary radiotherapy is approximately 50% for patients with NSCLC in stage I and 20% in stage IIIA. Radiotherapy can be used in patients with progressive metastatic disease after insufficient response to systemic therapy with threatening thoracic symptoms and for palliative treatment of cerebral, lymphatic and osseous metastases.

Titanium hydride and hydrogen concentration in acid-etched commercially pure titanium and titanium alloy implants: a comparative analysis of five implant systems.

OBJECTIVES: Acid etching is a popular method to texture the surface of dental implants. During etching, the titanium oxide protective layer is dissolved and small native hydrogen ions diffuse into the unprotected implant surface. They enrich the implant surface with hydrogen and precipitate into titanium hydride (TiH). The aim of this study was to measure the concentration of TiH at the implant surface and the total concentration of Hydrogen at five commercially available implant systems, made of either commercially pure (cp) titanium or titanium alloy. MATERIAL AND METHODS: X-Ray diffraction (XRD) was conducted on each implant system to determine the compounds present at the implant surface. Following a TiH(2)/Ti calibration curve, the concentration of TiH was determined. Concentration of hydrogen in the implants was measured by the inert gas fusion thermal conductivity/infrared detection method. RESULTS: XRD data showed that TiH was present on all cp titanium implants but not on the alloyed implants. TiH concentration varied between 5% and 37%. Hydrogen concentration varied between 43 and 108 ppm, no difference in uptake was found between the cp titanium and alloyed implants. Low solubility of hydrogen in alpha-titanium is responsible for precipitation into TiH. Stronger etching conditions led to higher concentration of TiH2-x. CONCLUSION: High solubility of hydrogen in the beta-phase of the alloy is preventing hydrogen from precipitating into TiH. All implants, even those lacking TiH at the surface, were enriched with hydrogen. In all implants, hydrogen concentration was within the normative limit of 130 ppm.

Persistent arterial stiffness and endothelial dysfunction following successful pancreas-kidney transplantation in Type 1 diabetes.

OBJECTIVE: Successful simultaneous pancreas-kidney transplantation (SPK) in Type 1 diabetic (T1DM) patients results in improved cardiovascular outcome and survival. However, it is doubtful whether the impairment of cardiovascular and endothelial function in T1DM can be completely reversed. METHODS: Pulse-wave velocity, stroke volume, heart rate, serological markers of endothelial dysfunction (soluble intercellular, vascular cell-adhesion molecules, E-selectin, and plasminogen-activator-inhibitor-1) were measured in 10 T1DM patients after SPK with non-diabetic glucose levels, 10 T1DM patients with poor [T1DM>8; glycated haemoglobin (HbA1c)>8%], and 10 with good glucose control (T1DM<7, HbA1c<7%), in 6 non-diabetic patients after kidney transplantation (KT) and 9 non-diabetic control subjects (CON), matching for major anthropometric characteristics. RESULTS: Pulse-wave velocity was increased in SPK (P < 0.02 vs. CON, KT, T1DM<7) and in T1DM>8 (P < 0.02 vs. T1DM<7). Systolic blood pressure was increased in SPK (P < 0.05 vs. CON). Stroke volume was reduced in SPK, T1DM>8 and T1DM<7 and KT (P < 0.01 vs. CON). Heart rate was elevated in SPK and in T1DM>8 (P < 0.0003 vs. CON and T1DM<7). In SPK, soluble intercellular and vascular cell-adhesion molecules were 100% and 44% higher (P < 0.03 vs. CON), respectively, while plasminogen-activator-inhibitor-1 was decreased in SPK (P < 0.02 vs. CON). CONCLUSION: T1DM patients after SPK experience arterial stiffness, a higher heart-rate and blood pressure, reduced stroke volume and serological signs of endothelial dysfunction. Thus, functional and structural cardiovascular alterations as a result of glucotoxicity, uraemia and hypertension in T1DM might not be completely resolved by SPK.

Glucose turnover and intima media thickness of internal carotid artery in type 2 diabetes offspring.

BACKGROUND: First-degree offspring (OFF) of type 2 diabetic (T2DM) patients bear a approximately 40% lifetime risk of developing T2DM. They are insulin resistant and carry a risk of premature atherosclerosis, the extent of which can be estimated by intima media thickness (IMT) of the carotid artery (CA). Thus, this study examines parameters of glucose and lipid metabolism, insulin sensitivity, beta cell function (BCF) and IMT with their interrelationships in middle-aged OFF. MATERIALS AND METHODS: T2DM-OFF (n = 18, 14f/4m, 45.6 +/- 2.1 years, BMI: 26 +/- 1 kg m(-2)) were compared with 18 matching humans without a family history of diabetes (CON; 14f/4m, 44.5 +/- 2.1 years, BMI: 24 +/- 1 kg m(-2); each P > 0.30), all with normal glucose tolerance as tested by three-hour (75 g) oral glucose tolerance tests (OGTT). Two-hour hyperinsulinaemic (40 mU min(-1).m(-2))isoglycaemic clamp tests were performed with simultaneous measurement of endogenous glucose (D-[6,6-(2)H(2)]glucose) production (EGP). IMT [internal (ICA), common CA, and bulb] were measured sonographically. BCF was assessed by Adaptation Index (AI). RESULTS: Before and during OGTT, both groups were similar in plasma glucose, insulin, C-peptide and free fatty acids (FFA), whereas OFF showed ~30% lower (P < 0.03) fasting plasma triglycerides before OGTT. During hyperinsulinaemic clamps, insulin sensitivity was approximately 38% lower (P < 0.03) in OFF who showed higher plasma FFA (44 +/- 9 micromol L(-1)) than CON (26 +/- 3 micromol L(-1), P < 0.05) after 90 min. EGP was similar in both groups. OFF had 38% (P < 0.007) reduced AI. ICA-IMT was approximately 18% higher in OFF (P < 0.002), but did not correlate with insulin sensitivity. CONCLUSION: The data obtained show middle-aged T2DM-OFF with normal glucose tolerance displaying reduced total insulin sensitivity and impaired beta cell function, which relates to impaired insulin-dependent suppression of plasma FFA and increased ICA-IMT.

Characterization of hepatic and brain metabolism in young adults with glycogen storage disease type 1: a magnetic resonance spectroscopy study.

In glycogen storage disease type 1 (GSD1), children present with severe hypoglycemia, whereas the propensity for hypoglycemia may decrease with age in these patients. It was the aim of this study to elucidate the mechanisms for milder hypoglycemia symptoms in young adult GSD1 patients. Four patients with GSD1 [body mass index (BMI) 23.2 +/- 6.3 kg/m, age 21.3 +/- 2.9 yr] and four healthy controls matched for BMI (23.1 +/- 3.0 kg/m) and age (24.0 +/- 3.1 yr) were studied. Combined (1)H/(31)P nuclear magnetic resonance spectroscopy (NMRS) was used to assess brain metabolism. Before and after administration of 1 mg glucagon, endogenous glucose production (EGP) was measured with d-[6,6-(2)H(2)]glucose and hepatic glucose metabolism was examined by (1)H/(13)C/(31)P NMRS. At baseline, GSD1 patients exhibited significantly lower rates of EGP (0.53 +/- 0.04 vs. 1.74 +/- 0.03 mg.kg(-1).min(-1); P < 0.01) but an increased intrahepatic glycogen (502 +/- 89 vs. 236 +/- 11 mmol/l; P = 0.05) and lipid content (16.3 +/- 1.1 vs. 1.4 +/- 0.4%; P < 0.001). After glucagon challenge, EGP did not change in GSD1 patients (0.53 +/- 0.04 vs. 0.59 +/- 0.24 mg.kg(-1).min(-1); P = not significant) but increased in healthy controls (1.74 +/- 0.03 vs. 3.95 +/- 1.34; P < 0.0001). In GSD1 patients, we found an exaggerated increase of intrahepatic phosphomonoesters (0.23 +/- 0.08 vs. 0.86 +/- 0.19 arbitrary units; P < 0.001), whereas inorganic phosphate decreased (0.36 +/- 0.08 vs. -0.43 +/- 0.17 arbitrary units; P < 0.01). Intracerebral ratios of glucose and lactate to creatine were higher in GSD1 patients (P < 0.05 vs. control). Therefore, hepatic defects of glucose metabolism persist in young adult GSD1 patients. Upregulation of the glucose and lactate transport at the blood-brain barrier could be responsible for the amelioration of hypoglycemic symptoms.

Indolent stage IE non-Hodgkin's lymphoma of the orbit: results after primary radiotherapy.

AIMS: Primary non-Hodgkin's lymphoma (NHL) of the orbit is uncommon, representing approximately 8% of extranodal NHLs. Twenty-two patients with indolent stage IE NHL were reviewed retrospectively to analyze the outcome and late effects of primary local radiotherapy. MATERIALS AND METHODS: The median age at first diagnosis was 63.5 years (range 24-82 years). Extranodal mucosa-associated lymphoid tissue lymphoma (n = 15) was the most common histological subtype of NHL, followed by follicular (n = 6) and lymphoplasmacytic lymphoma (n = 1). Radiotherapy was performed using a linear accelerator. The median radiation dose was 40 Gy (range 30-46 Gy). None of the patients received chemotherapy before irradiation. The follow-up period was 62 months (range 8-136 months). RESULTS: A complete response was achieved in all patients. The 5-year local control rate was 100%. Distant relapse occurred in 2 patients, resulting in a 5-year distant relapse-free survival rate of 88%. The 5-year overall survival rate was 89%; there were no lymphoma-related deaths. No serious acute complications (grade 3/4) were observed. Grade 1/2 late effects were documented in 44% of patients. Grade 3 complications (cataract: 2, dryness: 2) were observed in 4 patients (18%). CONCLUSIONS: Indolent early stage orbital NHL can be controlled with local radiotherapy. Morbidity is low. Regular follow-up examinations are necessary to detect rare cases of distant relapse.

[Merkel cell carcinoma--clinically often misjudged]. / Das Merkelzellkarzinom--klinisch oft verkannt.

Merkel cell carcinoma is a rare, rapidly growing, highly malignant dermal tumor which occurs preferentially on light-exposed skin in advanced age. The course of the disease is frequently characterized by the occurrence of lymph node metastases and local recurrences, even in the first year after removal of the primary tumour. The five-year overall survival rate is only about 65 %, despite rigorous therapy. The histological pattern is characterized by trabecular strands of small, uniform cells with large basophilic nuclei and typical neuroendocrine granules. The diagnosis is confirmed immunohistochemically by neuroendocrine and epithelial markers. The excision of the primary tumor is regarded as first-line therapy. Adjuvant radiatiotherapy is almost always indicated and should also include lymph node drainage. In the stage of nodal disease, a combination of excision and radiotherapy is recommended. Adjuvant chemotherapy can be applied in this stage, as in small-cell bronchial carcinoma. The prognostic advantage has, however, not been proven. Despite good response to radiatiotherapy and chemotherapy, with at least prolonged recurrence-free intervals, Merkel cell carcinoma is rarely curable at the distant metastasizing stage. Individually defined, aggressive treatment,including radiatiotherapy, may in future considerably improve the prognosis, especially in the early stages of the disease.