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Background Determination of the Fractional Flow Reserve (FFR) has become part of routine clinical practice. Contemporary clinical use, consequences as well as complications in consecutive, large cohorts have not been thoroughly investigated. We report the results of the prospective Fractional Flow Reserve Fax Registry F (FR2) conducted in Germany. Purpose To systematically analyze indications, procedural parameters, complications and consequences of intracoronary pressure measurements in a large contemporary cohort. Methods Data of 2000 consecutive patients undergoing clinically indicated FFR, iFR or pd/pa measurements in 8 interventional centres in Germany were prospectively collected in a systematic fashion. Data included basic patient characteristics, procedural aspects of intracoronary pressure measurements, associated complications, visual stenosis degree, measurement results and treatment decisions. Results Mean patient age was 68±11 years, 73% of patients were male. Of all patients, 300 patients (15%) had an acute coronary syndrome (STEMI: 9; NSTEMI: 94; unstable angina: 197) and 1002 patients (50%) had undergone previous revascularization. A mean of 1.7±0.9 measurements were performed per patient, for which an average of 1.02 pressure wires were required (more than 1 wire in 64 patients). For all 3373 interrogated lesions, median stenosis degree was 60%. Vasodilator-free measurements were performed in 415/3373 cases (12%, iFR: 346; pd/pa: 69). For vasodilation, i.v. adenosine was used in 396 cases (13%), i.c. adenosine in 2628 cases (87%), and other drugs in 10 cases (0.3%). Measurement was performed before potential revascularization in 3232 cases (96%) and during or following PCI in 141 cases. In 2958 lesions analyzed by FFR, mean FFR was 0.87, with 588 FFR measurements ≤0.80 (19.8%). Median FFR values were higher for i.c than i.v. adenosine administration (0.88 vs. 0.84), but not significantly different after adjustment for stenosis degree. In 735 cases (20.2%), intracoronary pressure measurement was followed by revascularization measures, while in 2637 cases (79.8%), no revascularization or no further revascularization was performed. In 36 out of 117 stenoses visually estimated to be ≥90%, revascularization was deferred following pressure measurement (31%). In 75 out of 2958 lesions analyzed by FFR, revascularization was performed even though FFR was >0.80 (3%). Severe complications (vessel dissection or occlusion) occurred in 5 out of 2000 patients as a consequence of intracoronary pressure measurement, resulting in death of 1 patient. Conclusion In clinical practice, the majority of intracoronary pressure measurements are performed in stenoses of intermediate angiographic severity and revascularization is deferred in approximately 80% of lesions. Vasodilator-free measurements are infrequent and route of adenosine administration has no effect on results. Complication rate is low but not negligible. Acknowledgement/Funding Abbott Vascular

History and admission findings In patients with glucose-6-phosphatase dehydrogenase (G6PD) deficiency (favism) jaundice is usually caused by hemolysis due to stress, infection or following the application of drugs. We report on a 74-year-old Italian with known G6PD deficiency complaining of jaundice, weight loss and abdominal pain. Physical examination revealed jaundice of the eyes. Scrotal examination by palpation and ultrasound showed no abnormalities. Investigations Serum levels of beta-human chorionic gonadotropin and alpha-fetoprotein were within normal limits, total bilirubin was extremely elevated, with predominant direct bilirubin. Abdominal ultrasound showed posthepatic blockage of bile flow with a dilated ductus hepatocholedochus (DHC) in the absence of gallstones. Enlarged, multiple contrast-stained paraaortic and retroperitoneal lymph nodes were detected by endoscopic ultrasound and magnetic resonance imaging. Due to failed endoscopic retrograde cholangiopancreatography, visualization of the biliary tree by percutaneous transhepatic cholangiography (PTC) was performed showing an occlusion of the DHC. Therapy and course After successful stent-implantation by PTC with decompression of the biliary tree, the jaundice disappeared. Computer tomography-guided percutaneous biopsy of a retroperitoneal lymph node was performed for histological evaluation showing a primary extragonadal nonseminomatous germ cell tumor. According to the histology (embryonic carcinoma) and clinical stage of the tumor systemic chemotherapy was initiated including cisplatin, etoposide and ifosfamide. After the first cycle of chemotherapy the patient suffered from pneumonia leading to septic shock. Twenty-seven days after admission, the patient died of multiple organ failure. Conclusion Extragonadal germ-cell tumor presenting as retroperitoneal lymph nodes with obstructive jaundice has to be considered in the differential diagnosis of cholestasis.

HISTORY AND ADMISSION FINDINGS A 39-year-old obese woman underwent endoscopic retrograde cholangiopancreatography with elective endoscopic biliary sphincterotomy (papillotomy) for symptomatic retained stones in the common bile duct which were extracted completely after added lithotripsy. Three hours later the patient developed profound subcutaneous emphysema of the face, neck and chest wall and shortness of breath, but had no abdominal pain. Physical examination revealed bilaterally diminished breath sounds and a distended and hyper-resonant abdomen, but no evidence of peritonitis. The patient was afebrile and hemodynamically stable. INVESTIGATIONS An emergency contrast-enhanced computed tomography (CT) of the chest and abdomen was performed. It demonstrated a bilateral pneumothorax, pneumomediastinum, pneumoperitoneum and pneumoretroperitoneum, in addition to extensive subcutaneous emphysema. There was no evidence of extraluminal leakage of contrast medium or intraperitoneal fluid on the CT. THERAPY AND CLINICAL COURSE Because of the increasing respiratory distress an intercostal drain was placed in the left pneumothorax and broad-spectrum antibiotics were administered. No drain was placed in the right lung. A follow-up CT after three days showed decreasing pneumomediastinum, pneumoperitoneum and pneumoretroperitoneum as well as resolution of the bilateral pneumothorax. The patient made an uneventful recovery and was discharged home seven days after the intervention. CONCLUSION Pneumothorax after endoscopic biliary sphincterotomy is a rare but serious complication that should be kept in mind after postinterventional development of shortness of breath.

We studied the differences in protein composition and immunologic reactivity of two E. coli-derived L-asparaginase (l-Asp) preparations (I and II), Erwinia-Asp (III) and PEG-modified E. coli l-Asp (IV). On gel filtration, each of preparations I-III showed three major peaks at 100, 270 and 460 KD, all with enzyme activity, whereas PEG-Asp showed peaks at 35 and 220 KD. On SDS-PAGE one major subunit could be identified at 32 KD (I and II) or 40 KD (III), whereas PEG-modified l-Asp could only be detected by lowering the polyacrylamide concentration and gave a single band above 200 KD. Using a polyclonal rabbit antibody generated against preparation I, only the E. coli l-Asp preparations (I and II) formed precipitin lines on Ouchterlony double diffusion. After freezing and thawing, preparation IV also reacted with this antibody. In sera from patients treated with preparation I, antibodies (detected by ELISA) reacted with preparations I and II but not with preparations III and IV. These results indicate that Erwinia-Asp (III) and PEG-Asp (IV) are distinct from E. coli preparations (I and II) by molecular weight and immunological behavior. They also provide an experimental rationale for the use of Erwinia-Asp as well as PEG-Asp in E. coli Asp-sensitized patients.

A 61-year-old man was admitted to hospital due to recurrent upper gastrointestinal bleeding. Four weeks ago, he had been treated with epinephrine and endoclips by endoscopy due to an arterial gastrointestinal bleeding. The patient had a history of coronary and peripheral artery disease, diabetes, and an abdominal aortic aneurysm. Urgent endoscopy suggested the presence of an ulcus Dieulafoy but no definitive bleeding source could be seen. Due to ongoing melena an abdominal computer tomography was performed and a primary aortoduodenal fistula was suspected caused by the infrarenal abdominal aortic aneurysm. Laparatomy was undertaken emergently and an aortoduodenal fistula was found in the descending part of the duodenum. Repair of the duodenal rent was performed and the aortic aneurysm was replaced by a Dacron prosthesis. The patient was transferred to the intensive care unit. 4 days after initial admission, he died due to septic shock.

Anamnese und klinischer Befund: Ein 84-jahriger Mann stellte sich mit einer Schwellung des linken Beines unter dem Verdacht einer tiefen Beinvenenthrombose vor. Eine familiare oder klinische Pradisposition fur eine thrombophile Diathese (Operation, Unfall, maligne Grunderkrankung oder Immobilisation) lag nicht vor. Fragen zu Defakations- oder Miktionsbeschwerden, Ruckenschmerzen oder neurologischen Ausfallerscheinungen wurden verneint. Die ansonsten unauffallige klinische Untersuchung zeigte eine schmerzlose einseitige Schwellung des linken Beines. Neurologisch zeigten sich keine Besonderheiten. Untersuchungen: Sonographisch lies sich links eine Flussminderung der tiefen Beinvenen, jedoch keine Thrombose nachweisen. In der abdominellen Computertomographie (CT) stellte sich ein solider prasakraler Tumor mit Kompression der Vena cava inferior dar. Die CT-gesteuerte Biopsie der Raumforderung erbrachte die Diagnose eines Ganglioneuroms. Therapie und Verlauf: Der Patient wurde zunachst mit niedermolekularem Heparin antikoaguliert. Eine Resektion lehnte er ab. Aufgrund des deutlich kompromittierten venosen Abflusses wurde dem Patienten eine Antikoagulation mit Phenprocoumon empfohlen. Folgerungen: Ganglioneurome sind seltene, benigne Tumore aus der Gruppe der Neuroblastome und entspringen dem sympathischen Nervensystem. Das Auftreten bei alteren Menschen ist eine Raritat und kann klinisch eine tiefe Beinvenenthrombose imitieren. Die komplette chirurgische Resektion ist die kurative Therapie. History and admission findings: An 84-year-old man was admitted to our hospital because of a swollen left leg resembling deep venous thrombosis. Clinical conditions favoring thrombosis such as surgery, trauma, malignant tumor or immobilization were not evident. His medical and family history were non-contributory. He denied bowel or bladder dysfunction and did not complain of any back pain, discomfort or neurological symptoms. The enlargement of the leg was painless and unilateral. There were no deficits on physical examination. The neurological status of the patient was unremarkable. Investigations: Ultrasonography was performed because deep vein thrombosis was suspected. Color-coded duplex revealed no thrombus in the veins of the left leg. Both femoral veins were compressible. The flow in the femoral vein was much less than in the other leg. Computed tomography demonstrated a solid lesion located in front of the spine displacing the inferior vena cava. CT-guided biopsy was performed. On histological examination it proved to be diagnostic of a ganglioneuroma. Treatment and course: Initially low molecular weight heparin was administered. Surgical resection was recommended, but the patient opted not to undergo any further treatment at that time. Phenprocoumon was recommended to prevent deep vein thrombosis. Conclusions: Ganglioneuroma is a rare, benign, well-differentiated, slow-growing tumor composed of sympathetic ganglion cells and mature stroma with a good prognosis. Its appearance in elderly persons is extremely rare and may imitate deep vein thrombosis caused by vessel compression. The appropriate treatment is surgical and complete excision is curative.

Ein 16-jahriger Junge wurde zur Abklarung einer seit 8 Jahren bestehenden progredienten Dysphagie stationar eingewiesen. Die Familienanamnese, der korperliche Untersuchungsbefund sowie die Laboruntersuchungen waren unauffallig. Der Osophagusbreischluck zeigte eine Kompression der Speiserohre im oberen Drittel von ausen. In der Magnetresonanzangiographie stellte sich eine aberrante Arteria subclavia dextra dar, die als letztes Gefas dem Aortenbogen unterhalb und dorsal der linken A. subclavia entsprang und als A. lusoria durch ihren retroosophagealen Verlauf eine Kompression verursachte. Aufgrund der symptomatischen Dysphagie bestand die Indikation zur operativen Intervention. Uber eine rechtsseitige supraklavikulare Inzision und linksseitige Thorakotomie wurde die A. lusoria abgangsnah ligiert und eine End-zu-Seit-Anastomose mit der rechten A. carotis communis angelegt. Postoperativ zeigte sich rechts ein kraftiger Radialispuls. Der Patient wurde beschwerdefrei am 7. postoperativen Tag entlassen.

History and admission findings A 23-year-old woman with a 5-year history of ulcerative colitis was admitted to our hospital because of bloody diarrhea. Two years previously she had undergone a hemicolectomy for a right colonic stricture. A recurrence of inflammatory bowel disease was suspected and treatment with prednisolone begun. The symptoms improved gradually, but 7 days later she complained of lower abdominal pain. Physical examination revealed a soft abdomen, but bowel sounds were reduced. Investigations The abdominal X-ray was unremarkable, but ultrasonography revealed moderate ascites and no blood flow in the portal vein on Doppler examination. The spleen was slightly enlarged. Contrast-enhanced abdominal magnetic resonance imaging (MRI) was performed immediately, revealing thrombosis of the portal and mesenteric veins. Treatment and course As there was no suggestion of intestinal necrosis, laparatomy was not considered necessary. Intravenous thrombolytic treatment with urokinase was given continuously (bolus of 250,000 units, followed by 200,000 units per hour), in order to lower the fibrinogen level to 100 - 150 mg/dl, together with unfractionated heparin, maintaining the activated partial thromboplastin time between 60 and 85 seconds. The thrombolytic treatment had to be stopped several times because of bloody diarrhea, but no transfusion was necessary. Two days after the start of thrombolytic treatment the abdominal pain and ascites ceased. Doppler sonography now demonstrated hepatopetal flow in the previously occluded portal vein. 4 days later, MRI revealed that the thrombus in the portal vein had dissolved and the portal vein was fully patent. The mesenteric vein was partially perfused, a residual thrombus extending into the portal vein. Tests for thrombophilia were negative. The thrombolytic therapy was stopped after 112 hours and the patient was treated with oral anticoagulation for 6 months. The patient recovered completely, with no evidence of portal hypertension during the following 6 months. Conclusions Thrombolysis with urokinase, guided by the level of fibrinogen, may be an alternative, semi-invasive treatment option in acute thrombosis of the portal and mesenteric veins.

Utilities today must adapt quickly to complex changes in workforce, infrastructure, technology, and regulations, as well as outside factors of the economy, security issues, and societal trends. Linear, cause-and-effect strategic planning does not work well in today's world. Given these uncertainties, how can utilities develop meaningful and useful strategic plans? Faced with these challenges, a group of 18 leading international utilities, assisted through IWA, has developed new ways of strategic planning. This project, sponsored by AwwaRF and conducted by EMA, has created a dynamic and flexible strategic planning framework. A new framework, coupled with many of these utilities' experiences in strategic planning, is the subject of this paper.

BACKGROUND Silastic cuffed catheters play an increasing role in providing long-term vascular access for hemodialysis. The reasons for this were related to increased patient age and an increased number of patients who had exhausted sites for vascular access. We report our experience with subcutaneously tunnelled cuffed hemodialysis catheters. METHOD From May 1, 2001 to February 28, 2003, 258 consecutive hemodialysis catheters were placed in 203 patients (122 men, mean age 70 +/- 12 years) as access for hemodialysis (1.3 catheters/patient). Catheter implantation was explained to all patients and a protocol consent form was signed. The catheter was implanted via the internal jugular, subclavian or femoral vein and the correct final catheter position was determined fluoroscopically. Short and long-term catheter-associated complications were collected from the time of catheter insertion until a follow up of at least 6 months after implantation. RESULTS The mean duration of implantation procedure was 38 +/- 12 minutes with an initial clinical success rate of 100 % and a periprocedural complication rate of 6 %. The median catheter indwell time was 9 months with a primary patency rate of 72 % at the end of the follow-up. Inadequate flow rate < 200 ml/min was noted at one per 41 patient-months but sufficient blood flow was restored in 50 % of these patients with non- or semi-invasive interventions. Bacteremic episodes occurred at a rate of one episode per 16 patient-months. Catheter removal due to severe exit site infections or bacteremic episodes were necessary at one per 73 patient-months. There were no cases of bacterial endocarditis. CONCLUSION Subcutaneously tunnelled cuffed venous hemodialysis catheters are a safe und highly feasible vascular access with a low complication rate and a long use-life especially for elder patients with limited life expectancy, exhausted sites for vascular access or in case of failing hemodialysis arteriovenous fistulas.

History and admission findings A 53-year-old woman had suffered from progressive lower limb weakness for 8 weeks. She was not able to walk on her own. Two hours after admission endotracheal intubation and mechanical ventilation became necessary because of acute respiratory failure. Investigations Chest radiograph and computerized tomography showed a huge lesion originating from the lower lobe of the left lung with mediastinal infiltration. Histological examination of bronchoscopical-guided biopsy disclosed small cell lung cancer. Electrophysiological studies revealed an incremental response on high-rate stimulation in the repetitive nerve stimulation test. Autoantibodies to P/C-type voltage-gated calcium channels were detected. Further, laboratory test results revealed hyponatremia (100 mmol/l) and elevated ADH (11 pg/ml). Diagnosis, treatment and clinical course These results led to the diagnosis of paraneoplastic Lambert-Eaton myasthenic syndrome (LEMS) with acute respiratory failure caused by small cell lung cancer. Additionally, SIADH (syndrome of inappropriate antidiuretic hormone secretion) was diagnosed as another paraneoplastic disease. Because of a prolonged weaning process resulting from inadequacy of mechanical ventilation, administration of intravenous immunoglobulins and chemotherapy with cisplatin and etoposide were undertaken. This made successful extubation possible after 7 days. In spite of all supportive treatment, clinical deterioration occurred. The patient died 27 days after the small cell lung cancer had been diagnosed. Conclusion The Lambert-Eaton myasthenic syndrome should be considered in cases of unexplained acute respiratory failure and clinical evidence of neoplastic disease.

Every public utility − no matter how big or small − will face many new trends during the next several decades. Factors such as changing regulations, renewable resources, customer demands, infrastructure and finance, technology and training, and “even better practices” will challenge utilities as they try to make their organizations more adaptable to change and sustainable far into the future. Utilities that anticipate these megatrends, plan and prepare strategies to deal with them, will be a more productive and sustainable organization. This paper looks at 10 megatrends, the major trends that are shaping the future of utilities.

HISTORY A 42 year old woman was resuscitated from ventricular fibrillation. 5 months previously she had a syncope. Her nephew had died of sudden cardiac death at the age of 25 years. INVESTIGATIONS There was no evidence for ST segment elevation, myocardial infarction or pulmonary embolism. The ECG showed right precordial T wave inversion. Coronary artery disease was excluded angiographically. Echocardiography and angiography revealed inferior wall akinesia of the right ventricle with normal left ventricular function and chamber size. Ventricular fibrillation could not be reproduced by programmed stimulation of the right ventricle during an electrophysiologic study. Results of endomyocardial biopsy of the right ventricle showed a focal fibrous infiltration of the myocardium. Magnetic resonance imaging confirmed inferior wall abnormalities of the right ventricle without typical fatty infiltration in the right ventricular myocardium. CLINICAL COURSE The patient recovered rapidly without neurologic deficits. Arrhythmogenic right ventricular dysplasia was suspected, and a cardioverter defibrillator (ICD) was implanted. Within 6 months after implantation the ICD memory showed no evidence of ventricular fibrillation. CONCLUSION Arrhythmogenic right ventricular dysplasia is an important cause of ventricular fibrillation with a potential risk of sudden cardiac death in young persons. Concealed arrhythmogenesis as an early manifestation of right ventricular dysplasia is difficult to detect.

Hintergrund: Die Leptospirose ist eine weltweit verbreitete Anthropozoonose. Dabei ist Leptospira pomona als Erreger der Schweinehuterkrankheit (Maladie des jeunes porchers) mit benigner Verlaufsform einer Leptospirose bekannt. Fallbeschreibung: Ein 51-jahriger Landwirt wurde mit hohem Fieber, Ikterus und akutem Nierenversagen stationar aufgenommen. Laborchemisch zeigten sich erhohte Retentions- und Entzundungsparameter, eine Thrombozytopenie, leicht erhohte Transaminasen sowie eine Hyperlipasamie als Zeichen einer Pankreatitis. Eine Woche zuvor waren Muskelschmerzen, Fieber sowie Zephalgien aufgetreten. Unter der Verdachtsdiagnose einer Leptospirose wurde eine Penicillintherapie begonnen. Serologisch lies sich innerhalb von 3 Wochen ein sechsfacher Titeranstieg gegen Leptospira pomona, den Erreger der Schweinehuterkrankheit, nachweisen. Unter intensivmedizinischer Behandlung konnte bei fallenden Retentionsparametern eine Nierenersatztherapie vermieden werden. Komplikationen durch die Thrombozytopenie oder Pankreatitis traten nicht auf. Ein Vorhofflimmern als Zeichen einer kardialen Beteiligung konvertierte, spontan in einen Sinusrhythmus. Der Patient wurde 3 Wochen nach der stationaren Aufnahme entlassen. Schlussfolgerung: Die fruhzeitige Diagnosestellung ist von entscheidender Bedeutung fur die Prognose insbesondere der mit Komplikationen behafteten Verlaufsform einer Leptospirose. Auch die Schweinehuterkrankheit als meistens benigne Verlaufsform der Leptospirose kann in seltenen Fallen zu gravierenden Organmanifestationen mit Nierenversagen, Ikterus und Thrombozytopenie fuhren.

History and admission findings A 67-year-old man had complained of diffuse abdominal pain and constipation for 4 days without indication of any underlying disease. On admission there was no evidence of weight loss, fever or nocturnal sweating. Investigations Physical examination revealed signs of an acute abdomen with high-pitched bowel sounds and diffuse abdominal guarding. The X-ray showed ileus of the small intestine which required emergency laparotomy. An obstructing conglomerate tumour was present in the area of the ileum, ca. 80 cm proximal to the caecum. It was removed by partial resection of the small intestine. Diagnosis Ileus of the small intestine with a low-malignant marginal zone B-cell (non-Hodgkin) lymphoma of MALT type (mucoid-associated lymphoid tissue). Treatment and course Postoperative staging indicated no further manifestation of the lymphoma. As no radical operation in resecting the tumour had been performed, combined radio- and chemotherapy was undertaken. Conclusion Marginal B-cell lymphomas of the small intestine are only rarely seen in central Europe. Despite its usually slow growth this non-Hodgkin lymphoma of low malignancy can produce an acute mechanical ileus without prodromal symptoms. A multimodal therapeutic approach is often employed, but there are no established treatment strategies.

E 50-jahriger Patient wurde mit Vigi lanzminderung, Kopfschmerzen, Brechreiz und febrilen Temperaturen bis 40 °C stationar aufgenommen. Laborchemisch zeigten sich eine Leukozytose von 19 500/μl (Normbereich [NB] 4 500–12 000/μl) sowie eine Erhohung des C-reaktiven Proteins auf 14,6 mg/dl (NB < 0,5 mg/dl). Bei bestehendem Meningismus erfolgte die Punktion von trubem Liquor mit Nachweis einer granulozytaren Pleozytose von 1 305 Zellen/μl (NB < 5 Zellen/μl) mit einem Neutrophilenanteil von 36,5%, einer Eiweiserhohung auf 193 mg/dl (NB < 50 mg/dl) und einem erniedrigten Glucosewert von 12 mg/dl (NB 45–80 mg/dl) bei einem Serumglucosewert von 106 mg/dl (Liquor/Serumglucose-Quotient 0,1). Das Liquorlactat wurde mit 4,6 mmol/l bestimmt (NB < 2,1 mmol/l). In der Gram-Farbung liesen sich grampositive Stabchen nachweisen. Unmittelbar nach Anlegen der Liquorkulturen erfolgte leitliniengerecht eine empirische intravenose Antibiotikatherapie mit Ampicillin (Tagesdosis 15 g) und Ceftriaxon (Tagesdosis 2 g). Bei fehlenden kardiovaskularen Risikofaktoren, unauffalliger kardialer Anamnese und normwertigen Parametern von Troponin I und Kreatinkinase zeigten sich im Oberflachen-EKG (Abbildung 1) T-Wellen-Inversionen in nahezu allen Ableitungen sowie eine Verlangerung der frequenzkorrigierten QT-Zeit (QTc) von 573 ms nach der Bazett-Formel (NB bei Mannern 440 ms). Eine QTc-Zeit-verlangernde Pharmakotherapie (z.B. antibiotische Behandlung mit Gyrasehemmern) bestand weder

Thames Water Utilities Limited operates a complex network of 124 water and 389 wastewater treatment works. Regulatory requirements are driving a massive capital programme of more than ₤2 Billion over 5 years to improve performance of these facilities. Improvements are planned, engineered, and operated by a variety of internal and external groups and individuals. Shareholders are demanding profitability and the Director General is pushing for improvements to customer service. To achieve its objectives, Thames has developed an instrumentation, control and automation (ICA) systems master plan. The plan is based on the utility s water and wastewater functional requirements. The functional approach is applied to the large number of diverse facilities by grouping facilities and processes into classifications. The approach is used throughout the plan. Each process and its associated process control strategy are clearly defined to ensure consistency and the cost-effective implementation of instrumentation and control across Thames. The functional approach was developed for each process control strategy using a series of structured workshops to achieve consensus and company-wide acceptance. Each department of the utility was involved in the workshops (including engineering, operations, maintenance, management systems, research and development, operational science). Top management sponsored the planning effort and committed to making the plan a reality.

With the continuing proliferation of computer hardware and software, many utilities are facing the problem of system incompatibility and an inability to share and exchange information among systems, resulting in much duplication of effort. To avoid this in the future, open system architecture and common industry standards are being developed. A utility communication architecture for electric utilities, based on open system standards, is currently being modified for use by water utilities

A 39-year-old obese woman underwent endoscopic retrograde cholangiopancreatography with elective endoscopic biliary sphincterotomy (papillotomy) for symptomatic retained stones in the common bile duct which were extracted completely after added lithotripsy. Three hours later the patient developed profound subcutaneous emphysema of the face, neck and chest wall and shortness of breath, but had no abdominal pain. Physical examination revealed bilaterally diminished breath sounds and a distended and hyper-resonant abdomen, but no evidence of peritonitis. The patient was afebrile and hemodynamically stable.An emergency contrast-enhanced computed tomography (CT) of the chest and abdomen was performed. It demonstrated a bilateral pneumothorax, pneumomediastinum, pneumoperitoneum and pneumoretroperitoneum, in addition to extensive subcutaneous emphysema. There was no evidence of extraluminal leakage of contrast medium or intraperitoneal fluid on the CT.Because of the increasing respiratory distress an intercostal drain was placed in the left pneumothorax and broad-spectrum antibiotics were administered. No drain was placed in the right lung. A follow-up CT after three days showed decreasing pneumomediastinum, pneumoperitoneum and pneumoretroperitoneum as well as resolution of the bilateral pneumothorax. The patient made an uneventful recovery and was discharged home seven days after the intervention.Pneumothorax after endoscopic biliary sphincterotomy is a rare but serious complication that should be kept in mind after postinterventional development of shortness of breath.

A 61-year-old man was admitted to hospital due to recurrent upper gastrointestinal bleeding. Four weeks ago, he had been treated with epinephrine and endoclips by endoscopy due to an arterial gastrointestinal bleeding. The patient had a history of coronary and peripheral artery disease, diabetes, and an abdominal aortic aneurysm. Urgent endoscopy suggested the presence of an ulcus Dieulafoy but no definitive bleeding source could be seen. Due to ongoing melena an abdominal computer tomography was performed and a primary aortoduodenal fistula was suspected caused by the infrarenal abdominal aortic aneurysm. Laparatomy was undertaken emergently and an aortoduodenal fistula was found in the descending part of the duodenum. Repair of the duodenal rent was performed and the aortic aneurysm was replaced by a Dacron prosthesis. The patient was transferred to the intensive care unit. 4 days after initial admission, he died due to septic shock.

An 84-year-old man was admitted to our hospital because of a swollen left leg resembling deep venous thrombosis. Clinical conditions favoring thrombosis such as surgery, trauma, malignant tumor or immobilization were not evident. His medical and family history were non-contributory. He denied bowel or bladder dysfunction and did not complain of any back pain, discomfort or neurological symptoms. The enlargement of the leg was painless and unilateral. There were no deficits on physical examination. The neurological status of the patient was unremarkable.Ultrasonography was performed because deep vein thrombosis was suspected. Color-coded duplex revealed no thrombus in the veins of the left leg. Both femoral veins were compressible. The flow in the femoral vein was much less than in the other leg. Computed tomography demonstrated a solid lesion located in front of the spine displacing the inferior vena cava. CT-guided biopsy was performed. On histological examination it proved to be diagnostic of a ganglioneuroma.Initially low molecular weight heparin was administered. Surgical resection was recommended, but the patient opted not to undergo any further treatment at that time. Phenprocoumon was recommended to prevent deep vein thrombosis.Ganglioneuroma is a rare, benign, well-differentiated, slow-growing tumor composed of sympathetic ganglion cells and mature stroma with a good prognosis. Its appearance in elderly persons is extremely rare and may imitate deep vein thrombosis caused by vessel compression. The appropriate treatment is surgical and complete excision is curative.

A 16-year-old man with an 8-year history of progressive dysphagia was referred to hospital. There was no specific finding in his family history. Physical examination was unremarkable. Complete blood count, serum electrolytes, and liver and kidney function tests were normal. Barium swallow revealed an extrinsic impression of the upper esophagus posteriorly. Magnetic resonance angiography demonstrated an aberrant origin of the right subclavian artery, leaving the aorta below the left subclavian artery. The artery had a retroesophageal course, causing the esophageal narrowing. Due to the persistence and worsening of the patient's symptoms, resection and reconstructive bypass surgery were recommended. Surgical correction was performed through a combined right supraclavicular incision and left posterolateral thoracotomy. After application of a vascular clamp, the aberrant right subclavian artery was ligated almost at its origin, and an end-to-side anastomosis was made with the right common carotid artery. At the end of the operative procedure, good pulses were palpated in the right radial artery. Postoperatively, the patient tolerated a regular diet without symptoms of dysphagia and was discharged on postoperative day 7.

A 23-year-old woman with a 5-year history of ulcerative colitis was admitted to our hospital because of bloody diarrhea. Two years previously she had undergone a hemicolectomy for a right colonic stricture. A recurrence of inflammatory bowel disease was suspected and treatment with prednisolone begun. The symptoms improved gradually, but 7 days later she complained of lower abdominal pain. Physical examination revealed a soft abdomen, but bowel sounds were reduced.The abdominal X-ray was unremarkable, but ultrasonography revealed moderate ascites and no blood flow in the portal vein on Doppler examination. The spleen was slightly enlarged. Contrast-enhanced abdominal magnetic resonance imaging (MRI) was performed immediately, revealing thrombosis of the portal and mesenteric veins.As there was no suggestion of intestinal necrosis, laparatomy was not considered necessary. Intravenous thrombolytic treatment with urokinase was given continuously (bolus of 250,000 units, followed by 200,000 units per hour), in order to lower the fibrinogen level to 100 - 150 mg/dl, together with unfractionated heparin, maintaining the activated partial thromboplastin time between 60 and 85 seconds. The thrombolytic treatment had to be stopped several times because of bloody diarrhea, but no transfusion was necessary. Two days after the start of thrombolytic treatment the abdominal pain and ascites ceased. Doppler sonography now demonstrated hepatopetal flow in the previously occluded portal vein. 4 days later, MRI revealed that the thrombus in the portal vein had dissolved and the portal vein was fully patent. The mesenteric vein was partially perfused, a residual thrombus extending into the portal vein. Tests for thrombophilia were negative. The thrombolytic therapy was stopped after 112 hours and the patient was treated with oral anticoagulation for 6 months. The patient recovered completely, with no evidence of portal hypertension during the following 6 months.Thrombolysis with urokinase, guided by the level of fibrinogen, may be an alternative, semi-invasive treatment option in acute thrombosis of the portal and mesenteric veins.

Silastic cuffed catheters play an increasing role in providing long-term vascular access for hemodialysis. The reasons for this were related to increased patient age and an increased number of patients who had exhausted sites for vascular access. We report our experience with subcutaneously tunnelled cuffed hemodialysis catheters.From May 1, 2001 to February 28, 2003, 258 consecutive hemodialysis catheters were placed in 203 patients (122 men, mean age 70 +/- 12 years) as access for hemodialysis (1.3 catheters/patient). Catheter implantation was explained to all patients and a protocol consent form was signed. The catheter was implanted via the internal jugular, subclavian or femoral vein and the correct final catheter position was determined fluoroscopically. Short and long-term catheter-associated complications were collected from the time of catheter insertion until a follow up of at least 6 months after implantation.The mean duration of implantation procedure was 38 +/- 12 minutes with an initial clinical success rate of 100 % and a periprocedural complication rate of 6 %. The median catheter indwell time was 9 months with a primary patency rate of 72 % at the end of the follow-up. Inadequate flow rate200 ml/min was noted at one per 41 patient-months but sufficient blood flow was restored in 50 % of these patients with non- or semi-invasive interventions. Bacteremic episodes occurred at a rate of one episode per 16 patient-months. Catheter removal due to severe exit site infections or bacteremic episodes were necessary at one per 73 patient-months. There were no cases of bacterial endocarditis.Subcutaneously tunnelled cuffed venous hemodialysis catheters are a safe und highly feasible vascular access with a low complication rate and a long use-life especially for elder patients with limited life expectancy, exhausted sites for vascular access or in case of failing hemodialysis arteriovenous fistulas.

A 53-year-old woman had suffered from progressive lower limb weakness for 8 weeks. She was not able to walk on her own. Two hours after admission endotracheal intubation and mechanical ventilation became necessary because of acute respiratory failure.Chest radiograph and computerized tomography showed a huge lesion originating from the lower lobe of the left lung with mediastinal infiltration. Histological examination of bronchoscopical-guided biopsy disclosed small cell lung cancer. Electrophysiological studies revealed an incremental response on high-rate stimulation in the repetitive nerve stimulation test. Autoantibodies to P/C-type voltage-gated calcium channels were detected. Further, laboratory test results revealed hyponatremia (100 mmol/l) and elevated ADH (11 pg/ml).These results led to the diagnosis of paraneoplastic Lambert-Eaton myasthenic syndrome (LEMS) with acute respiratory failure caused by small cell lung cancer. Additionally, SIADH (syndrome of inappropriate antidiuretic hormone secretion) was diagnosed as another paraneoplastic disease. Because of a prolonged weaning process resulting from inadequacy of mechanical ventilation, administration of intravenous immunoglobulins and chemotherapy with cisplatin and etoposide were undertaken. This made successful extubation possible after 7 days. In spite of all supportive treatment, clinical deterioration occurred. The patient died 27 days after the small cell lung cancer had been diagnosed.The Lambert-Eaton myasthenic syndrome should be considered in cases of unexplained acute respiratory failure and clinical evidence of neoplastic disease.

The use of internal thoracic arteries is an established method for treating coronary artery disease because of their excellent long-term patency rates. However, these results mainly referred to the left internal thoracic artery (LITA) grafted to the left anterior descending coronary artery (LAD). The aim of this study was to compare the patency rate of the LITA after its placement to the diagonal branch.A total of 302 patients (median age 65 years) with previous arterial revascularization were retrospectively enrolled in the study. We compared LITA grafts to the LAD with those to the diagonal branch and with sequential LITA grafts to both vessels with respect to the patency rate over a median follow-up of 39 months after prior operation. Angiography was performed for recurrent angina.The average occlusion/stenosis rate of saphenous vein and LITA grafts were 43.1% and 14.1%, respectively (p0.0001). Of the 302 patients, 248 had received a single LITA graft to the LAD; 21 patients, a single LITA graft to the diagonal branch; and 33 patients, a sequential LITA graft to both vessels. Thirty-three LITA grafts to the LAD (13.3%), three LITA grafts to the diagonal branch (14.3%), and six sequential LITA grafts to the LAD and the diagonal branch (18.2%) were occluded or stenosed more than 50%, respectively (p = 0.68). Seventy-nine percent of LITA graft stenoses were located at the peripheral anastomosis.Patency of single LITA grafts to the diagonal branch or sequential LITA grafts to the LAD and diagonal branch were comparable to single LITA grafts to the LAD. Most stenoses of LITA grafts were located at peripheral anastomoses.

A 42 year old woman was resuscitated from ventricular fibrillation. 5 months previously she had a syncope. Her nephew had died of sudden cardiac death at the age of 25 years.There was no evidence for ST segment elevation, myocardial infarction or pulmonary embolism. The ECG showed right precordial T wave inversion. Coronary artery disease was excluded angiographically. Echocardiography and angiography revealed inferior wall akinesia of the right ventricle with normal left ventricular function and chamber size. Ventricular fibrillation could not be reproduced by programmed stimulation of the right ventricle during an electrophysiologic study. Results of endomyocardial biopsy of the right ventricle showed a focal fibrous infiltration of the myocardium. Magnetic resonance imaging confirmed inferior wall abnormalities of the right ventricle without typical fatty infiltration in the right ventricular myocardium.The patient recovered rapidly without neurologic deficits. Arrhythmogenic right ventricular dysplasia was suspected, and a cardioverter defibrillator (ICD) was implanted. Within 6 months after implantation the ICD memory showed no evidence of ventricular fibrillation.Arrhythmogenic right ventricular dysplasia is an important cause of ventricular fibrillation with a potential risk of sudden cardiac death in young persons. Concealed arrhythmogenesis as an early manifestation of right ventricular dysplasia is difficult to detect.

Distal showering of atherosclerotic debris or thrombus is a feared event during percutaneous angioplasty of degenerated vein grafts.We report on a 66-year-old male patient with a history of coronary artery disease who was admitted into hospital with an acute coronary syndrome. He had had coronary artery bypass graft surgery in 1995. His chest pain resolved under treatment with aspirin, heparin, betablocker, and nitrate. A diagnostic angiogram demonstrated two critical stenoses of the left anterior descending graft. After a continuous infusion of tirofiban administered for 24 h prior to elective angioplasty, a JR 4SH 6 F guiding catheter (Cordis) was positioned. The lesions were crossed with a 0.014" Galeo F wire (Biotronik). A 4 mm x 16 mm stent graft (Jostent, Jomed) was deployed in the distal stenosis and a 4.5 mm x 32 mm self-expandable stent (Magic Wallstent, Boston Scientific) in the proximal stenosis. Thereafter, the patient suffered from chest pain, and the ECG showed ST elevation from V1 to V5. Angiography revealed total occlusion of the left anterior descending artery. An X-Sizer catheter (EndiCOR Medical, Inc.) was introduced and advanced through the vein graft with slow back-and-forth movements. After several aspirations, blood flow was significantly improved. A modest CK elevation (127 U/l) indicated a rather small loss of myocardial tissue.Percutaneous revascularization of narrowed aortocoronary saphenous vein grafts is associated with increased risk of distal embolization and "no reflow". The X-Sizer catheter system is compatible with commercially available guiding catheters, and capable of retrieving thrombotic debris after macroembolization from degenerated saphenous vein grafts including the native coronary artery.

A 67-year-old man had complained of diffuse abdominal pain and constipation for 4 days without indication of any underlying disease. On admission there was no evidence of weight loss, fever or nocturnal sweating.Physical examination revealed signs of an acute abdomen with high-pitched bowel sounds and diffuse abdominal guarding. The X-ray showed ileus of the small intestine which required emergency laparotomy. An obstructing conglomerate tumour was present in the area of the ileum, ca. 80 cm proximal to the caecum. It was removed by partial resection of the small intestine.Ileus of the small intestine with a low-malignant marginal zone B-cell (non-Hodgkin) lymphoma of MALT type (mucoid-associated lymphoid tissue).Postoperative staging indicated no further manifestation of the lymphoma. As no radical operation in resecting the tumour had been performed, combined radio- and chemotherapy was undertaken.Marginal B-cell lymphomas of the small intestine are only rarely seen in central Europe. Despite its usually slow growth this non-Hodgkin lymphoma of low malignancy can produce an acute mechanical ileus without prodromal symptoms. A multimodal therapeutic approach is often employed, but there are no established treatment strategies.

A 51-year-old man was admitted to the hospital for cardiac catheterization because of a small ventricular septal defect documented by echocardiography. Dyspnea on exertion was the leading symptom. Physical examination revealed only weak and delayed femoral pulses, whereas blood pressure measured …

This paper uses the method of least-squares k-spline approximation to generate time derivatives of sampled- data measurements required to determine values for parameters in models of linear and nonlinear dynamic systems. When identifying the parameter values in a nonlinear system in which signals are corrupted with measurement noise, the method developed in this paper provides more accurate values than does an approximate time-delay filter. In generating the required deriva tives in the presence of noise, the least-squares k- spline approximation yielded higher-order derivatives which were substantially more accurate than the same derivatives obtained from a low-pass filter. In a nonlinear case used as an illustrative example, the technique described in this paper resulted in as much as a 32% improvement in the accuracy of one of the identified model parameter values, and its largest error in any of the six parameters identified was 3.5 percent. The method was particularly effective in counteracting impulse noise caused by bad data points.

The elimination of l-asparaginase (l-Asp) was studied in 8 children treated for acute lymphoblastic leukemia according to the CoALL 82 protocol. The patients received four doses of l-Asp as a single agent during induction chemotherapy. We studied the elimination of l-Asp during the first infusion in 1 child, during the second in 3, during the third in 2 and during the forth in 2 children. Using Western blot technique and an experimental rabbit antibody to l-Asp, we were able to detect a single band at 32 kilodaltons (KD) in the serum of all patients between 4 and 36 h after infusion. The molecular weight remained unchanged and no other bands occurred during the time of observation. The detection limit of this method was calculated to 5 micrograms/ml using radial immunodiffusion. Incubation of l-Asp with pooled normal human serum caused no degradation of the enzyme during 48 h at 37 degrees C. Neither the total enzyme nor fragments were detectable in the urine of the patients collected during 8 h after l-Asp infusion. From these results we conclude that l-Asp is not cleaved by proteases in humans. The enzyme is most probably eliminated by the reticuloendothelial system.

This paper presents a simplified method to identify vehicle suspension spring rates, damping characteristics, and unsprung mass inertia properties. The method presented employs a digital computer algorithm to implement an equation error identification technique. A sequence of four laboraory tests was developed to obtain suspension input and response data required in the equation error technique. To verify the technique a test stand employing an electro-hydraulic position servosystem was constructed and the test sequence was applied to the rear suspension of a 1972 Pontiac Catalina. When the identified parameters were used to simulate the response of the vehicle suspensions to the test input, agreement to within 13% of the actual maximum measured response resulted. /SASI/

The elimination of l-asparaginase (l-Asp) was studied in 8 children treated for acute lymphoblastic leukemia according to the CoALL 82 protocol. The patients received four doses of l-Asp as a single agent during induction chemotherapy. We studied the elimination of l-Asp during the first infusion in 1 child, during the second in 3, during the third in 2 and during the forth in 2 children. Using Western blot technique and an experimental rabbit antibody to l-Asp, we were able to detect a single band at 32 kilodaltons (KD) in the serum of all patients between 4 and 36 h after infusion. The molecular weight remained unchanged and no other bands occurred during the time of observation. The detection limit of this method was calculated to 5 micrograms/ml using radial immunodiffusion. Incubation of l-Asp with pooled normal human serum caused no degradation of the enzyme during 48 h at 37 degrees C. Neither the total enzyme nor fragments were detectable in the urine of the patients collected during 8 h after l-Asp infusion. From these results we conclude that l-Asp is not cleaved by proteases in humans. The enzyme is most probably eliminated by the reticuloendothelial system.