[Anesthesia in patients with dopa-responsive dystonia (Segawa syndrome) : Presentation of the pathophysiology, clinical picture and approach based on two case reports]. / Anästhesieführung bei Patienten mit Dopa-responsiver Dystonie (Segawa-Syndrom) : Darstellung der Pathophysiologie, Klinik und Vorgehensweise anhand zweier Fallberichte.

Segawa syndrome (dopa-responsive dystonia [DRD]) is a rare neurometabolic disorder characterized by progressive dystonia, diurnal variation and tremors. It is caused by an enzymatic defect (a mutation of the GTPCH1 gene located on chromosome 14q) in the synthesis of tetrahydrobiopterin, an important substrate for dopamine synthesis. In the case of early correct diagnosis, clinical symptoms are well-controlled by levodopa therapy. The disease has several features which may lead to organ dysfunctions (e.g. torticollis, scoliosis, dysphagia and immobilization), which may be of concern for the anesthesiologist. Presenting two case reports of female patients undergoing elective cesarean section and breast cancer surgery, the main principles of perioperative management are discussed. Either techniques of regional or general anesthesia can be performed safely. Preoperative medication with levodopa should not be interrupted. Pharmacological agents with an antidopaminergic mode of action have to be avoided as well as significant pain and emotional stress situations in the perioperative period. Surgery in an ambulatory setting may not be recommended.

[Complications and emergencies in the recovery room]. / Komplikationen und Notfälle im Aufwachraum.

The recovery room as a central monitoring unit plays a crucial role in the perioperative care of patients. Here, the patient is monitored until the subsequent transfer to a further care ward; however, due to the previous anesthesia and surgery the recovery room also provides a high risk for (the development of) complications. The present article provides an overview of the most frequent complications to be expected in the recovery room (in adults), the diagnostics and the acute treatment.

[Antimicrobial treatment in burn injury patients]. / Antimikrobielle Therapie bei Patienten nach Verbrennungstrauma.

Infections are a great diagnostic and therapeutic challenge in intensive care patients with burn injuries. The major problems are due to bacteria with hospital-acquired multiresistance to antibiotics but fungal and viral infections may also be life-threatening. The main key points addressing pharmacotherapy with antibiotic, antifungal and antiviral agents in this special setting are exact diagnosis, early therapy with suitable drugs, adequate duration of treatment and adequate doses based on pharmacokinetic and pharmacodynamic characteristics of these compounds. The latter parameters are significantly altered in burn patients and show a wide interindividual and intraindividual variation in drug response as a result of the characteristic phases of burn injury. Drug concentration analysis may help to avoid inadequate dosing. In this review the main characteristics of burn injuries and the pharmacology of antibiotics, antifungal and antiviral agents in these patients are presented.

Impact of platelets and fresh frozen plasma in contrast to red cell concentrate on unstimulated and stimulated cytokine release in an in vitro model of transfusion.

Transfusion of blood may contribute to immunomodulation. Leuco-depleted standard blood products are supposed to result in less immunomodulation compared with whole blood. To determine the influence of leuco-depleted blood products on the cytokine response, red blood cell concentrates (RBC), fresh frozen plasma (FFP) and platelet concentrates (PC) were investigated in an in vitro model of blood transfusion. Leuco-depleted standard blood bank RBC, FFP and PC were mixed in vitro with AB0 compatible venous blood from healthy volunteers in ratios of 3:1, 1:1 and 1:3. Specimens were incubated in presence or absence of lipopolysaccharide, 1 mug/ml. After 24 h of incubation cytokine release of tumour necrosis factor (TNF)-alpha and interleukin-10 (IL-10) was measured in cell culture supernatants by means of enzyme-linked immunsorbent assay. Addition of RBC, FFP and PC to venous blood from healthy volunteers led to a significant and dose-dependent increase in spontaneous TNF-alpha and IL-10 release. After endotoxin stimulation, RBC, FFP and PC significantly suppressed the TNF-alpha response, while the stimulated release of IL-10 tended to increase, reaching significance only after high doses of FFP. Addition of leuco-depleted blood products changed the spontaneous and stimulated cytokine response in an in vitro model of transfusion. These data may suggest a possible contribution of transfused FFP and PC to immunomodulation after transfusion similar to RBC.

Diffuse intracranial oligodendroglioma in a cow.

This report describes the clinical, morphological and immunohistochemical findings in an adult cow with cerebral oligodendroglioma. The tumour extended from the metencephalon to the telencephalon, with infiltration of the meninges. Immunohistochemically, the tumour cells lacked expression of a specific tumour antigen, were partially positive for S-100 and tau protein, and were negative for a range of antigens including glial fibrillary acid protein, neuron-specific enolase, myelin basic protein and synaptophysin. This is the first report describing a diffuse, cerebral oligodendroglioma in a cow.

Post-traumatic Intraocular Leiomyosarcoma in a Domestic Bovine Calf (Bos primigenius taurus).

The present report describes a case of a post-traumatic ocular leiomyosarcoma in a calf (Bos primigenius taurus) including clinical, histopathological and immunohistochemical features. Following a traumatic episode, the animal developed opacification of the lens and shrinkage of the eye. Subsequently, the ocular lesion increased in size over a period of 6 weeks and reached up to 16 cm in diameter with infiltration into the cranium. At necropsy examination, the kidneys, liver and spleen exhibited multiple firm white metastases up to 2 cm in diameter. The mass was composed of closely-packed streams and bundles of neoplastic cells, which immunohistochemically expressed vimentin and alpha smooth muscle actin. Based on the anamnestic data, morphological appearance, distribution and immunohistochemical results, a diagnosis of a post-traumatic intraocular leiomyosarcoma was made. The tumour may have originated from cells lining the pectinate ligament of the ciliary body.

Listeria monocytogenes septicaemia and concurrent clostridial infection in an adult alpaca (Lama pacos).

A 10-year-old alpaca with a history of anorexia, weight loss and diarrhoea was humanely destroyed and shown to have a multifocal necrotizing hepatitis, splenitis and colitis, as well as an ulcerative to diphtheroid ileitis. Immunohistochemical examination revealed Listeria monocytogenes antigen in the liver and ileum. In addition, L. monocytogenes and Listeria sp.-specific gene fragments were detected by the polymerase chain reaction. L. monocytogenes was isolated from liver and small intestine and Clostridium perfringens type A with beta(2) toxin was found in the small intestine. It is suggested that the infection with C. perfringens type A facilitated the systemic spread of L. monocytogenes.

[Argatroban: pharmacological properties and anaesthesiological aspects]. / Argatroban : Pharmakologische Eigenschaften und anästhesiologische Aspekte.

Argatroban is a direct, selective and reversible active site thrombin inhibitor derived from L-arginine. It is a representative of a new class of antithrombotic drugs which offer inhibition of clot-bound as well as fluid-phase thrombin. Argatroban is characterised by favourable pharmacokinetics (beta-elimination half-time approximately 40-50 min) undergoing hepatic metabolism and mainly biliary excretion. Renal impairment will not result in altered or delayed elimination. For many years, argatroban has been used in Japan and in the United States and is approved by the FDA for anticoagulation in patients with heparin-induced thrombocytopenia (HIT type II). The ease of monitoring with the activated partial thromboplastin time, lack of induction of antibodies and adequate safety in renal failure patients, make this drug a favourable mode therapy in comparison with other anticoagulants such as lepirudin or heparinoids. Since June 2005 argatroban has been approved in Germany for the treatment of patients with HIT type II. The main characteristics of the drug with special considerations for anaesthesiologists and intensive care physicians are presented in this review.

Proinflammatory cytokine gene expression in whole blood from patients undergoing coronary artery bypass surgery and its modulation by pentoxifylline.

The influence of coronary artery bypass grafting (CABG) on spontaneous and lipopolysaccharide (LPS)-stimulated release of tumor necrosis factor (TNF)-alpha, interleukin (IL)-1beta, IL-6, and IL-10 as well as its modulation by pentoxifylline (PTF) were studied in a prospective, randomized, double-blinded study. 12 patients undergoing elective CABG were randomly assigned to receive either saline or PTF (1 mg/kg as a loading dose followed by 1 mg/kg/h) intraoperatively. Blood samples were obtained (A) preoperatively, (B) 20 min after CABG, and (C) 24 h after CABG. Cytokine plasma levels as well as LPS-stimulated cytokine secretion were measured in a whole blood culture system ex vivo and correlated with mRNA expression in peripheral blood mononuclear cells. In addition, the dose-response characteristics of modulation of the cytokine response by PTF were studied in cultured whole blood in vitro. Plasma IL-6 and IL-10-levels were significantly elevated after CABG, whereas neither TNF-alpha nor IL-1beta were detectable. In contrast to the spontaneous release of IL-6 and IL-10, the expression of all cytokines studied was significantly reduced upon ex vivo LPS stimulation early after CABG. Proinflammatory cytokine response upon LPS stimulation was restored 24 h after CABG for the group mean, however, with substantial interindividual heterogeneity. Therapeutic doses of PTF in vitro attenuated LPS-induced TNF-alpha (-50.5%) and most notably IL-10 (-83.9%) release, whereas IL-1beta was even increased (+45.7%). However, application of PTF during CABG neither inhibited the spontaneous production of IL-10 nor modulated cytokine production ex vivo. These results suggest a biphasic response of stimulated peripheral blood mononuclear cell cytokine gene expression during CABG with an initial tolerance to LPS stimulation. The application of PTF during CABG in doses that are primarily based on its use in occlusive arterial disease do not seem to modulate the release of the cytokines studied.

Mixed agonistic-antagonistic cytokine response in whole blood from patients undergoing abdominal aortic aneurysm repair.

OBJECTIVE: To characterize the impact of abdominal aortic aneurysm repair (AAAR) on spontaneous as well as lipopolysaccharide (LPS)-induced gene expression of pro- and anti-inflammatory cytokines. DESIGN: Prospective, controlled in vivo/ex vivo study. SETTING: University hospital. PATIENTS AND INTERVENTIONS: Whole blood from 14 consecutive patients undergoing AAAR withdrawn prior to surgery (T1), at the end of ischemia (T2), 90 min after declamping (T3) and on the first postoperative day (T4) was cultured in the absence or presence of LPS. Five patients undergoing elective inguinal hernia repair served as controls. MEASUREMENTS AND RESULTS: While tumor necrosis factor (TNF), Interleukin (IL)-1 and IL-10 plasma concentrations did not increase significantly, IL-6 was elevated at each time point, as compared with T1. Despite the spontaneous release of trace amounts of IL-6, the ability of cultured whole blood to mount a cytokine response in vitro to LPS was impaired for all cytokines studied at T2 (TNF-62%, IL-1-51%, IL-6 -20%, IL-10-51%). The stimulated IL-6 response was restored early after declamping (T3: +56 %) and enhanced 1 day after operation (T4: +144%). In contrast, stimulated TNF and IL-1 responses remained depressed at T3 (TNF -48%, IL-1-64%) and T4 (TNF-40%, IL-1-24%). A biphasic pattern was observed for IL-10 with initial depression at T3 (-51%) and restoration at T4 (+40%). Among the different cytokines monitored, only impaired TNF responsiveness at early reperfusion (T3) correlated with the postoperative course, as reflected by APACHE II. Cytokine response to LPS was maintained or even increased during and after surgery in the whole blood from patients undergoing hernia repair. CONCLUSIONS: Despite consistent development of clinical signs of systemic inflammatory response syndrome (SIRS) and spontaneous release of IL-6 abdominal aortic aneurysm repair produces a state of impaired pro-inflammatory cytokine response upon a subsequent in vitro Gram-negative stimulus. This early impairment of TNF responsiveness seems to correlate with an unfavorable postoperative course.

Heparin-associated thrombocytopenia (HAT)--still a diagnostic and therapeutical problem in clinical practice.

Type II of heparin-associated thrombocytopenia (HAT) is well known, but the cardinal symptom, thrombocytopenia, is rarely adequately considered. Serious and potential lethal complications such as pulmonary embolism, cerebral stroke, or limb gangrene are often falsely regarded as insufficient anticoagulation. Guided diagnosis and therapy are of vital importance for the patient's outcome. Based on the experience of patients with HAT Type II treated in the intensive care unit, a diagnostic and therapeutic approach to the cardinal symptom thrombocytopenia is presented. A recently developed heparin-induced platelet activation assay (HIPAA) seems to be a highly sensitive laboratory test. The first therapeutic principle in case of presumed and diagnosed HAT is the cessation of unfractioned or low-molecular-weight heparins. ORG 10172 (Orgaran), a low-sulfated heparinoid with a low cross-reactivity (10%) to heparins, can be regarded as the most effective anticoagulant in patients with HAT Type II.

[Haemostatic disorders in ENT patients.Part 2: Pathophysiology, diagnostics, clinical feature and therapy]. / Störungen der Hämostase bei Patienten in der HNO-Heilkunde : Teil 2: Pathophysiologie,Klinik und Therapie ausgewählter Krankheitsbilder.

In the first part of the medical education article on haemostatic disorders in ENT patients the basic physiology of haemostasis and main diagnostic tools were presented and discussed.The second part presents disorders of the coagulation system,thrombocyte function and blood vessels with special emphasis on clinical practice in ENT surgery. In this context, haemophilia A and B, von Willebrand disease and different forms of thrombocytopenia are of main clinical importance. Some underlying diseases such as malignomas, renal and hepatic insufficiency in combination with drug therapy (e.g.anticoagulants and thrombocyte function inhibitors) play an important role in clinical practice as well.Sepsis and haemorrhage may lead to disseminated intravascular coagulation (DIC).Beside a systematic review, important haemostatic disorders are illustrated with case reports.

[Importance of proximal femoral fractures in elderly patients. Results of a prospective study of 61 patients]. / Die Bedeutung proximaler Femurfrakturen für den alten Menschen. Ergebnisse einer prospektiven Studie an 61 Patienten.

In a prospective study we found good results concerning postoperative complications (21%), perioperative lethality (3.2%) and lethality during hospital stay (6.5%) after implantation of dynamic hip screws (DHS) in patients with per- and subtrochanteric fractures of the femur. The ability to walk, as well as housing and social care postoperatively and follow-up for at least six months after dismissal demonstrates the importance of these fractures for the elderly. One of three patients needs professional care, 21% at home, 13% in nursing homes. Six months after operation the lethality is nearly 20%, i.e. three times higher than in hospital. These are the essential data for all techniques of osteosynthesis used in patients with these fractures, which could only be documented in prospective studies.

[Bronchopneumonia and polyarthritis due to Mycoplasma bovis in a calf]. / Bronchopneumonie und Polyarthritis mit Nachweis von Mycoplasma bovis bei einem Rind.

This case report describes gross lesions and histopathological findings in a 3-months-old calf originating from a feedlot with approximately 400 cattle. In this animal and additional 14 cattle of similar age, which were kept together in the same stable, swollen joints had occurred suddenly. The examination of this calf showed that a severe polyarthritis induced by haematogenous spread of Mycoplasma bovis following bronchopenumonia was present, which was characterised by necrotising lesions of the joint capsules and severe cartilage erosions.

Immunohistochemical and morphometric analysis of intestinal full-thickness biopsy samples from cats with lymphoplasmacytic inflammatory bowel disease.

The distribution and numbers of CD3(+) T lymphocytes, immunoglobulin(+) plasma cells and calprotectin (L1)(+) macrophages was analyzed in full-thickness, formalin-fixed biopsy samples from the small intestine (duodenum, jejunum and ileum) and from the colon from nine cats with clinical signs of inflammatory bowel disease (IBD). All animals had lymphoplasmacytic enteritis or lymphoplasmacytic enterocolitis. Equivalent samples from the same intestinal regions from 12 healthy pet cats served as controls. Labelled cells in the lamina propria were counted by computer-aided morphometry. The different cell types were similarly distributed in both groups, but there were differences in their numbers. There were more CD3(+) T cells in the duodenum and jejunum of cats with IBD; however, the difference was only significant for the duodenum. There were significantly more IgA(+) cells in the duodenal crypt region. There were significantly more IgG(+) cells in the lower jejunal crypt region. Plasma cells expressing IgM were decreased in cats with IBD, but the difference was not significant. L1(+) macrophages were significantly decreased in the lower crypt area of the colon in cats with IBD and there was a trend to decreased L1(+) cells in the upper crypt area of the duodenum and jejunum. Comparison of the results of this study with previous findings on endoscopically-obtained duodenal biopsy samples from cats with IBD revealed some differences. These discrepancies might relate to differences between control cat populations, types of biopsy samples, methodological factors such as different counting techniques and the activity of the disease at the time of sampling.