Medizinische Klinik最新文献

Whole-body plethysmography is considered the "gold standard" for intrathoracic gas volume measurements and the measurement of specific airway resistance. These parameters are essential to assess a restrictive impairment, overinflation of the lungs and airflow obstruction.

Background: Thrombotic thrombocytopenic purpura (TTP) is a severe disease with microthrombi in various organs. The idopathic subtype is characterized by reduced ADAMTS13 activity mediated via autoantibodies against this protease. Induction of autoantibodies mechanistically is incompletely understood, but certain drugs can induce anti-ADAMTS13 antibodies through hapten mechanisms.

Case report: A 56-year-old man was admitted from an external hospital because of a rapidly worsening general condition, hemolytic anemia (hemoglobin 4.17 mmol/l, hematocrit 0.19), and thrombocytopenia (22 Gpt/l) for unknown reasons. Additionally, he was found to have an elevated lactate dehydrogenase (45.37 μmol/l/s). 13 days before hospitalization he had received vaccination against H1N1. Laboratory tests revealed an increased total bilirubin (126 μmol/l), and a decreased haptoglobin level (< 0.08 g/l). The blood smear showed 24% fragmentocytes. Direct and indirect Coombs test were negative. TPP was diagnosed based on the clinical presentation and the detection of ADAMTS13 antibodies. Despite daily plasma exchange via plasmapheresis and administration of corticosteroids, there was no significant rise in platelet counts. Immunosuppression with a total of four weekly doses of rituximab (375 mg/m(2) body surface area) was added. Over the next 5 weeks, the platelet count very slowly rose. After a total of 46 sessions, plasmapheresis was ended with complete remission of the disease.

Conclusion: This report emphasizes the immunologic susceptibility of TTP, and suggests the potential, but not proven role of H1N1 vaccination in the pathogenesis of TTP, because no serum before vaccination was available. Severe autoantibody TTP can be successfully treated by administering rituximab in addition to standard treatment with plasmapheresis and corticosteroids.

Non-modified human immunoglobulins (IgG) are standard of care for replacement therapy with primary (inherited) immunodeficiencies, and secondary immunodeficiencies due to multiple myeloma (MM) or chronic lymphocytic leukemia (CLL). Further, they have effectively been used as immunomodulation in neurological autoimmune diseases such as Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and multifocal motor neuropathy (MMN). A variety of IgG preparations for intravenous and subcutaneous use are available. In view of the broad range of indications, data on the utilization of the IgG preparations in everyday clinical care are of high clinical interest. Furthermore, data on the outcomes of IgG therapy outside the setting of controlled clinical trials are needed. Therefore, the SIGNS study (Assessment of Immunoglobulins in a Long-Term Non-Interventional Study) was set up as a non-interventional prospective open-label cohort study and was approved by the ethics committee. Led by an interdisciplinary steering board, hospital- and office-based investigators in 30-40 centers throughout Germany (neurologists, pediatricians, oncologists, other) will document approximately 300 patients, and will follow them for at least 2 years. Patients of both genders and any age are eligible if they have received, or are scheduled for, IgG therapy for primary or severe secondary immunodeficiency or neurological autoimmune diseases, and have provided written informed consent. No exclusion criteria have been defined in order to minimize selection bias. Long-term outcome data will be collected on patient characteristics in the various indications, drug utilization (e.g., treatment and dosing patterns), effectiveness (i.e., number of infections), tolerability, health-related quality of life, and economic variables (number of hospitalizations, sick-leave days, etc.) with the possibility to estimate direct costs. For the neurological autoimmune diseases, detailed data will be gathered, among others, on neurological function, muscular function, physical function (grip strength, INCAT disability scale, etc.) and stabilization or progression of symptoms over time. Data collection in SIGNS is performed using a secure internet site and an MySQL database. A number of quality measures are routinely performed including automated plausibility checks at data entry, queries, and on-site monitoring with source data verification. It is expected that SIGNS will contribute to optimization of therapy in this diverse patient population.

Background and purpose: Unclear extremity complaints are common symptoms of inpatients. In a subset of these patients, a clinical suspicion of deep vein thrombosis (DVT) results; this needs to be quickly and definitively clarified by a vascular physician. The question arose of how often a clinical suspicion of DVT was confirmed in an inpatient population and which alternative diagnoses were able to be made by angiologists.

Patients and methods: In a retrospective analysis, all inpatients in the Angiologic Vascular Diagnostics Center of the University Hospital Halle, Germany, examined in 2007 for a suspicion of DVT were evaluated with respect to the definitively made diagnosis.

Results: In 213 (28.6%) of 745 suspected cases of DVT, a DVT was confirmed. In 532 patients (71.4%), DVT was excluded. In 314 of these patients, 436 alternative diagnoses were recorded in the diagnostic reports of angiologic examinations. In 38.6% (n = 168), other venous causes could be confirmed as the most common alternative diagnosis. There were chronic venous diseases in 28% (n = 122), superficial thrombophlebitis (n = 27), and tumor-related pelvic vein compression (n = 19). 17.4% (n = 76) exhibited lymphedema. In 13.3% (n = 58), a generalized edema was diagnosed. Arthrogenic causes followed with 12.8% (n = 56). Lipedema (5.3%) and hematoma (5%) could be verified as other important differential diagnoses. Rare causes were symptomatic or ruptured Baker's cysts (2.5%), erysipelas (2.5%), abscess, aneurysm, muscle tears, and tumors.

Conclusion: The variety of alternative diagnoses in patients with clinical suspicion of DVT is high. The knowledge and systematic examination of potential, even rare differential diagnoses after exclusion of DVT are part of the repertoire of the vascular physician. Unnecessary and expensive, as well as onerous, diagnostic procedures on the patient can be avoided. Anticoagulation that was begun as a result of the suspicion of DVT can quickly be stopped.

Digital ulcers in systemic sclerosis are painful ischemic necrotic lesions of the acra. Optimal treatment consists of conventional wound management and medication: iloprost infusions promote primary healing of the ulcers, while the dual endothelin receptor antagonist bosentan is used for secondary prophylaxis of new ulcers. The described case illustrates the essential interdisciplinary collaboration for optimal management of these patients.

In clinical practice, the reticulocyte count is the most useful method to estimate red cell life span and red cell production. However, experience of hematologists as well as surveys have shown that counting of reticulocytes is often neglected in the diagnostic work-up of unclassified anemias, and many physicians have difficulties in interpreting the results. Formerly, this was partly due to the low precision of manual counts. Today, this method is largely replaced by flow cytometry, available in almost all larger systems for automated blood count analysis, at low costs and sufficient analytic precision. Interpretation is supported by calculated parameters such as the Reticulocyte Production Index. Here, the authors discuss the limits of the analytic methods and the problems of interpretation in the context of the laboratory profile and the clinical setting.

Background: The Marfan syndrome is a typical rare disease with multiorgan involvement and the need for specialized interdisciplinary medical care. A novel German legal directive according to section sign 116 b of the Social Statutes Book V (116 b SGB V) improves options for reimbursement and thus encourages specialized hospitals to provide ambulatory care for rare diseases such as Marfan syndrome. The authors provide the first economic analysis of section sign 116 b in a German Marfan center.

Methods: The costs were assessed in 184 cases with Marfan syndrome receiving medical care in the Hamburg Marfan Clinic. The authors assessed the financial profit both according to payments received from invoices established according to the 116 b directive [reimbursement (116b)] and from calculations according to section sign 117 SGB V [reimbursement (117)].

Results: A total of 117 patients traveled to the Marfan clinic (64%) < 50 km, 27 patients (15%) between >or= 50 and 100 km. The total costs for ambulatory care were 71,606.28 Euro. The reimbursement (116b) was 55,549.87 Euro and the reimbursement (117) was 11,776.00 Euro.

Conclusion: Many patients accept long distances of traveling to receive specialized ambulatory medical care. However, for optimal patient management specialized centers need to cooperate intensively with local health care providers. The novel legal directive according to section sign 116 b has significantly improved reimbursement for Marfan centers and allows for improving the quality of medical care.

Background: Valid, sustained and safe clinical means of colorectal cancer prevention are still lacking, but they are urgently needed to lower the incidence of colorectal cancer. Dietary factors and phytochemicals such as flavonoids play an important role for prevention.

Methods: A selective search of the literature using PubMed was performed with the following key words: flavonoids, cancer, therapy, colorectal cancer focused on clinical queries. Results of clinical studies including the authors' own were compared.

Results: In vivo and in vitro studies with animals, cell cultures and subcellular components provide ample evidence for antimutagenic and anticarcinogenic effects of flavonoids as shown for multiple biological and molecular endpoints. Isoflavonoids in vitro have been shown to induce proliferation of breast cancer cells. Epidemiologic trials (cohort, case-control and cross-sectional studies) yielded inconsistent results for flavonoid protection. Systematic reviews and meta-analyses support the protective role of tea flavonoids on adenoma incidence. An interventional pilot study with sustained flavonoid supplementation was shown to reduce the rate of neoplasia in patients with resected colorectal cancer.

Conclusion: Selected flavonoids possess antimutagenic and anticarcinogenic properties and could reduce the incidence of colorectal neoplasias as shown in epidemiologic trials. Randomized controlled clinical studies with flavonoid intervention are necessary to provide evidence for their role in colorectal cancer prevention.