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104. Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 1996 to 2011.

Author

Som S, Deford CC, Kaiser ML, Terrell DR, Kremer Hovinga JA, Lämmle B, George JN, Vesely SK, Som, Sumit, Deford, Cassandra C, Kaiser, Mandi L, Terrell, Deirdra R, Kremer Hovinga, Johanna A, Lämmle, Bernhard, George, James N, and Vesely, Sara K

Abstract

Background: Plasma exchange (PEX) treatment for patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) has risk for major complications.Study Design and Methods: Data for PEX-related complications have been prospectively collected on all patients enrolled in the Oklahoma TTP-HUS Registry, 1996 to 2011. PEX-related complications have been defined as major or minor and as central venous catheter related or plasma related.Results: During 15 years, 1996 to 2011, 72 (24%) of 302 consecutive patients had major PEX-related complications. Analysis of five consecutive 3-year cohorts demonstrated that there has been a significant trend for decreasing frequency of all PEX-related major complications (p = 0.014) and central venous catheter-related major complications (p = 0.021) but not for the less common plasma-related major complications (p = 0.380). ADAMTS13 activity was measured in 288 (95%) of the 302 patients. Analysis of the 66 patients with ADAMTS13 activity of less than 10% demonstrated a significant trend for decreasing frequency of PEX-related major complications (p = 0.036); the trend for the 222 patients with ADAMTS13 activity of at least 10% was not significant (p = 0.118). The decreased frequency of PEX-related major complications among patients with ADAMTS13 activity of less than 10% may be related to a significant trend for decreasing duration of PEX treatment (p = 0.040) and decreasing frequency of requirement for more than one central venous catheter (p = 0.044). The decreased duration of PEX treatment may be related to increased use of adjunctive treatments: corticosteroids (p < 0.001) and rituximab (p < 0.001).Conclusions: The frequency of PEX-related major complications has decreased from 1996 to 2011, possibly related to increased use of corticosteroids and rituximab and the decreased duration of PEX required to achieve remission. [ABSTRACT FROM AUTHOR]

Published
2012
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115. Tryptase from human mast cells does not activate purified human Hageman Factor.

Author

Alter, S. C., Lämmle, B., Griffin, J. H., and Schwartz, L. B.

Subjects
*PROTEOLYTIC enzymes, *TRYPSIN, *MAST cells, *POLYACRYLAMIDE gel electrophoresis, *MOLECULAR weights
Abstract

The effect of tryptase, a neural protease released from human lung mast cell secretory granules, on purified human Hageman Factor (Factor XII) was examined. No increase in Hageman Factor enzymatic activity was detected after incubation with tryptase at 37°C; activation of Hageman Factor by bovine trypsin served as a positive control. Furthermore, pre-incubation of Hageman Factor with tryptase did not diminish the subsequent activation of Hageman Factor by trypsin. Polyacrylamide gel electrophoresis was also performed to show that incubation with tryptase does not alter the molecular weight of Hageman Factor. Therefore, tryptase neither activates nor destroys human Hageman Factor. [ABSTRACT FROM AUTHOR]

Published
1988
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116. Dural puncture and activated protein C resistance: risk factors for cerebral venous sinus thrombosis.

Author

Wilder-Smith, E, Kothbauer-Margreiter, I, Lämmle, B, Sturzenegger, M, Ozdoba, C, and Hauser, S P

Abstract

Objectives: Dural puncture is regarded a safe procedure when contraindications are carefully excluded and has so far not been recognised as a risk factor for cerebral venous sinus thrombosis (CVST). Five patients are described with CVST after dural puncture in the presence of additional risk factors.Methods: In four out of five patients complete investigations for thrombophilia were performed at least one month after withdrawal of oral anticoagulation.Results: In three out of four patients tested, activated protein C (APC) resistance due to heterozygous coagulation factor V R506Q mutation (factor V Leiden) was found. One patient was using oral contraceptives as a circumstantial risk factor and three had had spinal anaesthesia for surgical procedures. Family history of venous thromboembolism was negative in all patients. Retrospective evaluation of 66 patients with CVST disclosed that dural puncture was the fourth most common risk factor (8%) possibly contributing to thrombosis.Conclusion: Dural puncture may constitute an additional risk factor for CVST especially in patients with APC resistance or surgery. In such patients a thrombophilia screen is indicated. [ABSTRACT FROM AUTHOR]

Published
1997

131. Autoactivation of human plasma prekallikrein.

Author

Tans, G, Rosing, J, Berrettini, M, Lämmle, B, and Griffin, J H

Abstract

Incubation of purified human plasma prekallikrein with sulfatides or dextran sulfate resulted in spontaneous activation of prekallikrein as judged by the appearance of amidolytic activity toward the chromogenic substrate H-D-Pro-Phe-Arg-p-nitroanilide. The time course of generation of amidolytic activity was sigmoidal with an apparent lag phase that was followed by a relatively rapid activation until finally a plateau was reached. Soybean trypsin inhibitor completely blocked prekallikrein activation whereas corn, lima bean, and ovomucoid trypsin inhibitors did not. The Ki of the reversible inhibitor benzamidine for autoactivation (240 microM) was identical to the Ki of benzamidine for kallikrein. Thus, spontaneous prekallikrein activation and kallikrein showed the same specificity for a number of serine protease inhibitors. This indicates that prekallikrein is activated by its own enzymatically active form, kallikrein. Immunoblotting analysis of the time course of activation showed that, concomitant with the appearance of amidolytic activity, prekallikrein was cleaved. However, prekallikrein was not quantitatively converted into two-chain kallikrein since other polypeptide products were visible on the gels. This accounts for the observation that in amidolytic assays not all prekallikrein present in the reaction mixture was measured as active kallikrein. Kinetic analysis showed that prekallikrein activation can be described by a second-order reaction mechanism in which prekallikrein is activated by kallikrein. The apparent second-order rate constant was 2.7 × 10(4) M-1 s-1 (pH 7.2, 50 microM sulfatides, ionic strength I = 0.06, at 37 degrees C). Autocatalytic prekallikrein activation was strongly dependent on the ionic strength, since there was a considerable decrease in the second-order rate constant of the reaction at high salt concentrations. In support of the autoactivation mechanism it was found that increasing the amount of kallikrein initially present in the reaction mixture resulted in a significant reduction of the lag period and a rapid completion of the reaction while the second-order rate constant was not influenced. Our data support a prekallikrein autoactivation mechanism in which surface-bound kallikrein activates surface-bound prekallikrein.

Published
1987
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133. SEVERE OSTEOPOROSIS DUE TO SYSTEMIC MAST CELL DISEASE: SUCCESSFUL TREATMENT WITH INTERFERON ALPHA-2B.

Author

LEHMANN, T., BEYELER, C., LÄMMLE, B., HUNZIKER, T., VOCK, P., OLAH, A. J., DAHINDEN, C., and GERBER, N. J.

Abstract

We describe a 33-yr-old man suffering from severe vertebral osteoporosis and urticaria pigmentosa due to systemic mast cell disease (SMCD). Because i.v. clodronate therapy could not prevent further vertebral fractures, an additional treatment with interferon alpha-2b was initiated. During 24 months of treatment, our patient had no further pain episodes, no new vertebral fractures were discovered, trabecular bone mineral density (BMD) increased significantly and urticarial symptoms improved. Nevertheless, the extent of skin lesions remained unchanged. On histological examination, a remarkable decrease of mast cells was observed in the bone marrow, but not in the skin. Five months after discontinuation of interferon alpha-2b, trabecular BMD decreased and urticarial symptoms deteriorated. These findings illustrate a beneficial effect of interferon alpha-2b on SMCD-induced osteoporosis as well as urticarial symptoms, and raise the question whether this treatment may have a diverse impact on mast cell populations in different tissues. [ABSTRACT FROM PUBLISHER]

Published
1996
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136. Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2

Author

Winkler A, Johanna A. Kremer Hovinga, Bianchi V, Jd, Studt, and Lämmle B

Subjects
ADAM Proteins, Anemia, Hemolytic, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic, Microcirculation, ADAMTS13 Protein, Humans, Metalloendopeptidases, Female, Kidney Diseases, Thrombosis, Middle Aged
Abstract

We present the case of a woman (age: 64 years) with acute thrombotic microangiopathy due to severe acquired ADAMTS-13 (von Willebrand factor-cleaving protease) deficiency. She was successfully treated with plasma exchange therapy and glucocorticosteroids. She relapsed seven months later, and splenectomy led to lasting remission. Pathomechanisms of thrombotic thrombocytopenic purpura, especially the role of ADAMTS-13, are discussed and therapeutic measures outlined.

137. Acquired von Willebrand syndrome in a myeloproliferative disorder. Case 6

Author

Baud P, Tobler A, Lämmle B, and Lorenzo Alberio

Subjects
Male, von Willebrand Diseases, Myeloproliferative Disorders, Aspirin, Anti-Inflammatory Agents, Non-Steroidal, von Willebrand Factor, Humans, Middle Aged
Abstract

We present a woman (age: 57 years) with an excessive bleeding episode under acetylsalicylic acid after bone marrow puncture due to an acquired von Willebrand syndrome (avWS) in the context of a myeloproliferative disorder. The laboratory features showed a high platelet concentration and a qualitative defect of von Willebrand factor (vWF) with a low normal vWF ristocetin cofactor activity, a normal vWF antigen and a decrease of the larger vWF multimers in plasma. The exact mechanism of avWS is still incompletely resolved. Myeloproliferative diseases are one of several underlying disorders that may cause avWS. The diagnosis of the underlying disease is important because its treatment may lead to an improvement of the vWF abnormality. For symptomatic treatment of bleeding, desmopressin, vWF concentrate infusion, intravenous immunoglobulin and/or fibrinolysis inhibitors can be tried.

138. Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory

Author

Jd, Studt, Johanna A. Kremer Hovinga, Alberio L, Bianchi V, and Lämmle B

Subjects
Adult, Aged, 80 and over, Male, Adolescent, Purpura, Thrombotic Thrombocytopenic, ADAMTS13 Protein, Metalloendopeptidases, Middle Aged, ADAM Proteins, Humans, Female, Child, Laboratories, Aged
Abstract

Severe deficiency of von Willebrand factor-cleaving protease (ADAMTS-13) activity (5% of normal) is specific for classical thrombotic thrombocytopenic purpura (TTP), a disorder presenting with thrombocytopenia, microangiopathic haemolytic anaemia and often with organ dysfunction such as neurological symptoms, renal failure, and fever. A certain, though according to several case series, variable percentage of patients with clinically diagnosed TTP and most patients with other forms of thrombotic icroangiopathies (TMA) do not show severe ADAMTS-13 deficiency.We determined ADAMTS-13 activity in 508 plasma samples of 309 patients referred to our laboratory in 2001 and 2002. Plasma samples with ADAMTS-13 activity5% were additionally tested for the presence of inhibitory antibodies. Patients were assigned to ten predefined clinical categories according to information provided in the referral letter (TMA not specified; TMA associated with neoplasia or chemotherapy; TMA following haematopoietic stem cell transplantation; TMA with additional disorder; idiopathic TTP; haemolytic-uraemic syndrome (HUS) not specified; HUS with diarrhoea prodrome; atypical HUS; other haematological disorder; no clinical information available).We detected 50 (16%) patients with severe ADAMTS-13 deficiency. Forty-four (88%) of these patients had been classified as idiopathic TTP, 2 as neoplasia- or chemotherapy-associated, and 4 as non-specified TMA. Among the patients labelled as acute idiopathic TTP, the prevalence of severe ADAMTS-13 deficiency was 63% (44/70). Inhibitory antibodies were found in 31 (62%) patients with ADAMTS-13 activity5%. Of the 44 patients with acute idiopathic TTP, at initial presentation or at relapse, with ADAMTS-13 activity5%, 11 were identified to have (probable) constitutional severe ADAMTS-13 deficiency.Severe ADAMTS-13 deficiency is found in about 60% of patients diagnosed with idiopathic TTP but in none of 111 diagnosed with HUS. Plasma ADAMTS-13 activity5%, however, does not identify all patients clinically diagnosed with TTP. Detection of inhibitory antibodies against ADAMTS-13 helps to differentiate between acquired and constitutional forms of TTP, which may be important for treatment strategies.

140. Acute pancreatitis and thrombotic thrombocytopenic purpura

Author

Ip, Bergmann, Johanna A. Kremer Hovinga, Lämmle B, Hj, Peter, and Schiemann U

Subjects
ADAM Proteins, Pancreatitis, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic, ADAMTS13 Protein, Humans, Female, Middle Aged, Tomography, X-Ray Computed
Abstract

Thrombotic thrombocytopenic purpura (TTP) has multiple clinical manifestations and risk factors, but the events that actually trigger acute episodes of TTP are often unclear. We describe the case of a 56-year-old woman who presented with clinical signs and symptoms of TTP and acute pancreatitis. We discuss whether pancreatitis was due to ischemic pancreatic damage caused by microvascular platelet clumping in the frame of TTP, or whether acute pancreatitis, a disorder that results in an intense systemic inflammatory response, may be a triggering event for acute episodes of TTP.

141. SEVERE OSTEOPOROSIS DUE TO SYSTEMIC MAST CELL DISEASE: SUCCESSFUL TREATMENT WITH INTERFERON ALPHA-2B

Author

LEHMANN, T., BEYELER, C., LÄMMLE, B., HUNZIKER, T., VOCK, P., OLAH, A. J., DAHINDEN, C., GERBER, N. J., LEHMANN, T., BEYELER, C., LÄMMLE, B., HUNZIKER, T., VOCK, P., OLAH, A. J., DAHINDEN, C., and GERBER, N. J.

Abstract

We describe a 33-yr-old man suffering from severe vertebral osteoporosis and urticaria pigmentosa due to systemic mast cell disease (SMCD). Because i.v. clodronate therapy could not prevent further vertebral fractures, an additional treatment with interferon alpha-2b was initiated. During 24 months of treatment, our patient had no further pain episodes, no new vertebral fractures were discovered, trabecular bone mineral density (BMD) increased significantly and urticarial symptoms improved. Nevertheless, the extent of skin lesions remained unchanged. On histological examination, a remarkable decrease of mast cells was observed in the bone marrow, but not in the skin. Five months after discontinuation of interferon alpha-2b, trabecular BMD decreased and urticarial symptoms deteriorated. These findings illustrate a beneficial effect of interferon alpha-2b on SMCD-induced osteoporosis as well as urticarial symptoms, and raise the question whether this treatment may have a diverse impact on mast cell populations in different tissues

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