Katharinen Hospital

Unna, Germany

Katharinen Hospital

Unna, Germany
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Yogasundaram H.,University of Alberta | Kim D.,University of Alberta | Oudit O.,City University of New York | Thompson R.B.,University of Alberta | And 2 more authors.
Canadian Journal of Cardiology | Year: 2017

Anderson-Fabry disease (AFD) is an X-linked recessive, multisystem disease of lysosomal storage. A mutation in the gene encoding the hydrolase enzyme α-galactosidase A results in its deficiency, or complete absence of activity. Subsequent progressive intracellular accumulation of glycosphingolipids, predominantly globotriaosylceramide, in various tissues, results in progressive organ dysfunction and failure, most commonly affecting the kidneys, nervous system, skin, eyes, vascular endothelium, and the heart. Cardiac involvement in AFD represents a leading cause of morbidity and mortality. Globotriaosylceramide accumulation affects cardiomyocytes, smooth muscle cells, vascular endothelial cells, and fibroblasts leading to various pathologies including valvular regurgitation, conduction disease and arrhythmias, coronary microvascular dysfunction, and right and left ventricular hypertrophy (LVH) leading to early diastolic dysfunction and late-stage systolic impairment. Diagnosis is on the basis of decreased plasma α-galactosidase activity in men and positive genetic testing in women. Contemporary large-scale screening studies have revealed a prevalence of 1%-5% in patients with unexplained LVH in multiple cohorts. Cardiac magnetic resonance imaging, with its unique tissue characterization capabilities, is the most important imaging modality to assess for cardiomyopathy in patients with AFD. Enzyme replacement therapy is indicated in AFD patients with significant organ involvement, and has been shown to clear sphingolipids from endothelial cells in other organs, as well as to reduce left ventricular mass as early as 6 months after starting treatment. There is increasing evidence that enzyme replacement therapy might be more effective if given at earlier stages of disease, before the development of LVH and myocardial fibrosis. © 2017 Canadian Cardiovascular Society


Feuser K.,University of Hohenheim | Feilhauer K.,Katharinen Hospital | Staib L.,Clinic for Visceral Surgery | Bischoff S.C.,University of Hohenheim | Lorentz A.,University of Hohenheim
Molecular Immunology | Year: 2011

Challenge of human mast cells with both stem cell factor (SCF) and IL-4 enhances antigen-dependent mediator release raising the assumption of intracellular crosstalk between the IL-4, SCF, and Fce{open}RI signaling pathways. Here, we analyzed the intracellular crosstalk of IL-4-, SCF-, and IgE-dependent activation pathways in mucosal mast cells isolated from human intestine. The release of β-hexosaminidase, leukotriene C4, and IL-8, but not IL-6, was strongly enhanced in response to sequential challenge of mast cells with IL-4, SCF and Fce{open}RI cross-linking compared to stimulation by Fce{open}RI cross-linking alone. Previous studies revealed that MAPK and other serine/threonine kinases are involved in mast cell activation processes. Here we found that activation of mast cells by Fce{open}RI cross-linking alone results in phosphorylation of ERK and p38, but not of Akt. Stimulation with SCF alone also induced phosphorylation of ERK and p38, and additionally of Akt. IL-4 priming enhanced activation of ERK, but blocked activation of p38. Activation of p38 was required for IL-6 production explaining the inhibitory effect of IL-4 on IL-6 expression in human mast cells. Moreover, IL-4 priming that anteceded Fce{open}RI cross-linking induced activation of Akt. The combined challenge of mast cells with IL-4, SCF and Fce{open}RI cross-linking substantially up-regulated activation of Akt, whereas blocking of Akt inhibited the pronounced production and release of IL-8 in response to the three mast cell agonists. In summary, our data demonstrate that ERK, p38, and especially Akt play an important role in cross-linking IL-4 priming, SCF signaling, and IgE-dependent activation of mature human mast cells. © 2010 Elsevier Ltd.


PubMed | Red Cross, Sana Hospital Offenbach, University Medical Center Frankfurt Main, University of Cologne and 47 more.
Type: Journal Article | Journal: Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery | Year: 2016

Orthognathic surgery has always been a classical focus of maxillofacial surgery. Since more than 100 years, various surgical techniques for mandibular repositioning have been developed and clinically tested. Since the establishment of plate and screw osteosynthesis, orthognathic surgery became more stable and safe. Nowadays, different surgical methods for mobilising the mandible are existing. This international multicenter analysis (n=51 hospitals) is providing first evidence based data for the current use of different surgical methods. The dominating techniques were Obwegeser/dal Pont (61%) followed by Hunsuck/Epker (37%) and Perthes/Schlssmann (29%). The main osteosynthesis materials were plates (82%), bicortical screws (23.5%), or a combination of both (5.9%). 47% of all centers reported to use several surgical methods at the same time, depending on the anatomical problem and the surgeons preference. This shows that different surgical methods seem to work as comparable, safe, and reliable procedures in everydays clinical practise. On this basis, further prospective studies could evaluate possible advantages for our patients.


Malinoc A.,Albert Ludwigs University of Freiburg | Sullivan M.,Albert Ludwigs University of Freiburg | Wiech T.,Albert Ludwigs University of Freiburg | Schmid K.W.,University of Duisburg - Essen | And 9 more authors.
Endocrine-Related Cancer | Year: 2012

The etiology and pathogenesis of renal cell carcinoma (RCC) are only partially understood. Key findings in hereditary RCC, which may be site specific or a component of a syndrome, have contributed to our current understanding. Important heritable syndromes of RCC are those associated with pheochromocytoma, especially von Hippel-Lindau disease (VHL) associated with germline VHL mutations, and pheochromocytoma and paraganglioma syndrome (PGL) associated with mutations in one of the four genes (SDHA-D) encoding succinate dehydrogenase. A subset of individuals with SDHB and SDHD germline DNA mutations and variants develop RCC. RCC has never been described as a component of SDHC-associated PGL3. The European-American Pheochromocytoma and Paraganglioma Registry comprises 35 registrants with germline SDHC mutations. A new registrant had carotid body tumor (CBT) and his mother had CBT and bilateral RCC. Blood DNA, paragangliomas, and RCCs were analyzed for mutations and loss-of-heterozygosity (LOH) in/flanking SDHC and VHL. The proband with unilateral CBT had a germline SDHC c.3G > A (p.M1I) mutation. His mutation-positive mother had CBT at age 42, clear cell RCC (ccRCC) at age 68, and papillary RCC (pRCC) at age 69. Both paraganglial tumors showed somatic LOH of the SDHC locus. Both ccRCC and pRCC did not have a somatic SDHC mutation but showed LOH for intragenic and flanking markers of the SDHC locus. LOH was also present for the VHL locus. Our findings suggest that RCC is a component of PGL3. Biallelic inactivation of the SDHC gene may represent a new pathway of pathogenesis of syndromic and nonsyndromic RCC, perhaps of both clear cell and papillary histologies. © 2012 Society for Endocrinology Printed in Great Britain.


Gutt C.N.,University of Heidelberg | Encke J.,University of Heidelberg | Koninger J.,University of Heidelberg | Harnoss J.-C.,University of Heidelberg | And 18 more authors.
Annals of Surgery | Year: 2013

Objective: Acute cholecystitis is a common disease, and laparoscopic surgery is the standard of care. Background: Optimal timing of surgery for acute cholecystitis remains controversial: either early surgery shortly after hospital admission or delayed elective surgery after a conservative treatment with antibiotics. Methods: The ACDC ("Acute Cholecystitis-early laparoscopic surgery versus antibiotic therapy and Delayed elective Cholecystectomy") study is a randomized, prospective, open-label, parallel group trial. Patients were randomly assigned to receive immediate surgery within 24 hours of hospital admission (group ILC) or initial antibiotic treatment, followed by delayed laparoscopic cholecystectomy at days 7 to 45 (group DLC). For infection, all patients were treated with moxifloxacin for at least 48 hours. Primary endpoint was occurrence of predefined relevant morbidity within 75 days. Secondary endpoints were as follows: (1) 75-day morbidity using a scoring system; (2) conversion rate; (3) change of antibiotic therapy; (4) mortality; (5) costs; and (6) length of hospital stay. Results: Morbidity rate was significantly lower in group ILC (304 patients) than in group DLC (314 patients): 11.8% versus 34.4%. Conversion rate to open surgery and mortality did not differ significantly between groups. Mean length of hospital stay (5.4 days vs 10.0 days; P < 0.001) and total hospital costs (€2919 vs €4262; P < 0.001) were significantly lower in group ILC. Conclusions: In this large, randomized trial, laparoscopic cholecystectomy within 24 hours of hospital admission was shown to be superior to the conservative approach concerning morbidity and costs. Therefore, we believe that immediate laparoscopic cholecystectomy should become therapy of choice for acute cholecystitis in operable patients. © 2013 Lippincott Williams & Wilkins.


Passauer J.,University Hospital Carl Gustav Carus | Petrov H.,Evangelisches Krankenhaus Konigin Elisabeth Herzberge | Schleser A.,Katharinen Hospital | Leicht J.,Dialysezentrum Schwandorf | Pucalka K.,Dialysezentrum Thornerstr.
Nephrology Dialysis Transplantation | Year: 2010

Background. Dry weight assessment (DWA) is essential to efficient therapy of haemodialysis (HD) patients. However, so far objective methods for DWA have not been applicable to daily routine. Thus, exact fluid management in HD remains difficult and is often based on clinical criteria. The aims of this study were (1) to objectively define pre-and post-dialytic ranges of extracellular volume in a large cohort of HD patients (in whom DWA had been defined according to clinical criteria), (2) to compare the hydration status between diabetic and non-diabetic patients, and (3) to assess a patient subgroup that might benefit from correction of target weight.Methods. We measured fluid overload (FO) prior to a mid-week HD session in 370 randomly selected HD patients (50 with diabetes) from five dialysis centres. A new bioimpedance spectroscopy (BIS) device that implies a validated body composition model was applied. This tool allows correct quantification of extracellular FO or-deficiency in comparison to a healthy reference population (normal range-1.1 to 1.1 L according to the 10th and 90th percentile of measurements). In addition, weight and blood pressure were recorded before and after treatment.Results. Pre-dialytic FO ranged from-0.5 to 4 L and post-dialytic FO from-2.5 to 2 L (10th and 90th percentile of measurements), indicating that on average the hydration status of healthy subjects is considered as the optimal target weight in HD patients. Comparison of FO between diabetic and non-diabetic patients revealed no difference. Based on the consideration that an FO <-1.1 L before and >1.1 L after HD indicates inadequate DWA, we identified 98 (26) patients who might benefit from correction of target body weight.Conclusion. BIS is an interesting, objective method to support clinical DWA. Further studies should be performed to investigate beneficial clinical effects of this approach.


PubMed | Prosper Hospital and Katharinen Hospital
Type: | Journal: Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen | Year: 2016

In rectopexy the use of meshes provides stability by mechanical support as well as by the induction of scar formation; however, one of the problems of conventional methods of mesh rectopexy is that many patients postoperatively suffer from functional disorders, such as fecal incontinence and stool evacuation disorders. One reason is the damage of vegetative nerves following dorsal and lateral mobilization of the rectum, which is required for positioning of the mesh. In 2004 DHoore and Penninckx first described the method of ventral rectopexy, anew technique of mesh rectopexy which allows preservation of the autonomic nerves.Does ventral rectopexy provide advantages regarding functional outcome, complications and recurrence rates?A search was carried out in the databases PubMed and Medline for studies on ventral rectoplexy. Presentation and analysis of the current state of relevant studies relating to ventral rectopexy.Ventral rectopexy is characterized by a low complication rate and good functional results in terms of improvement of incontinence, constipation and stool evacuation disorders. The indications for ventral rectopexy are considered in patients with external prolapse of the rectum. Also in a well-selected patient population internal prolapse, rectocele as well as enterocele accompanied by obstructive defecation syndrome represent relative indications for ventral rectopexy.In order to obtain a valid assessment of the value of this procedure it is crucial to improve the current lack of evidence (level3) by prospective randomized studies that compare ventral rectopexy with other surgical techniques and nonsurgical treatment options.


PubMed | Charité - Medical University of Berlin, Catalan Institution for Research and Advanced Studies, Katharinen Hospital, Comprehensive Heart Failure Center and Klinikum Straubing GmbH
Type: Journal Article | Journal: The American journal of cardiology | Year: 2016

Patients with genetic cardiomyopathy that involves myocardial hypertrophy often develop clinically relevant arrhythmias that increase the risk of sudden death. Consequently, guidelines for medical device therapy were established for hypertrophic cardiomyopathy, but not for conditions with only anecdotal evidence of arrhythmias, like Fabry cardiomyopathy. Patients with Fabry cardiomyopathy progressively develop myocardial fibrosis, and sudden cardiac death occurs regularly. Because 24-hour Holter electrocardiograms (ECGs) might not detect clinically important arrhythmias, we tested an implanted loop recorder for continuous heart rhythm surveillance and determined its impact on therapy. This prospective study included 16 patients (12 men) with advanced Fabry cardiomyopathy, relevant hypertrophy, and replacement fibrosis in loco typico. No patients previously exhibited clinically relevant arrhythmias on Holter ECGs. Patients received an implantable loop recorder and were prospectively followed with telemedicine for a median of 1.2years (range 0.3 to 2.0years). The primary end point was a clinically meaningful event, which required a therapy change, captured with the loop recorder. Patients submitted data regularly (14 11 times per month). During follow-up, 21 events were detected (including 4 asystole, i.e., ECG pauses 3seconds) and 7 bradycardia events; 5 episodes of intermittent atrial fibrillation (>3minutes) and 5 episodes of ventricular tachycardia (3 sustained and 2 nonsustained). Subsequently, as defined in the primary end point, 15 events leaded to a change of therapy. These patients required therapy with a pacemaker or cardioverter-defibrillator implantation and/or anticoagulation therapy for atrial fibrillation. In conclusion, clinically relevant arrhythmias that require further device and/or medical therapy are often missed with Holter ECGs in patients with advanced stage Fabry cardiomyopathy, but they can be detected by telemonitoring withanimplantable loop recorder.


Kirsch M.,University of Greifswald | Henkes H.,Katharinen Hospital
Minimally Invasive Neurosurgery | Year: 2011

Background: True aneurysms of the ophthalmic artery (OA) are extremely rare and most often arise at the origin of this artery. We present the endovascular management of a ruptured intraorbital OA aneurysm and of an associated dural arteriovenous fistula (dAVF). Patient: A patient with a ruptured intraorbital ophthalmic artery aneurysm, associated with a dAVF with cortical drainage presented with acute visual loss and intra- and periorbital hematoma. The aneurysm was treated by endovascular coil occlusion. The dAVF was occluded by transvenous obliteration of the draining basal vein of Rosenthal. Both intraorbital ophthalmic artery aneurysms and their rupture are extremely rare. Conclusion: Transvenous treatment of dAVFs is well feasible even with very far going catheterization, in this case to the origin of the basal vein of Rosenthal. © Georg Thieme Verlag KG Stuttgart · New York.


Schiff J.H.,Katharinen Hospital | Koninger J.,Katharinen Hospital | Teschner J.,Katharinen Hospital | Henn-Beilharz A.,Katharinen Hospital | And 4 more authors.
Anaesthesia | Year: 2013

The use of extracorporeal membrane oxygenation in adults has increased in popularity and importance for the support of patients with cardiac or pulmonary failure. Although it is now quite commonly used in the intensive care unit, its use has rarely been described as a means of support during anaesthesia and surgery. We report the case of a patient who required curative resection of the oesophagus following previous left pneumonectomy where veno-venous extracorporeal membrane oxygenation was required both during surgery and for the first three days postoperatively. We describe the anaesthetic management of this patient who only had a single lung, review other alternatives and discuss why extracorporeal membrane oxygenation was particularly suited to this case. To the best of our knowledge, the anaesthetic literature to date does not contain a case report of this type. © 2013 The Association of Anaesthetists of Great Britain and Ireland.

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