Severe infusion reactions to fabry enzyme replacement therapy: rechallenge after tracheostomy.

A 34-year-old male patient with Fabry disease (OMIM 301500) commenced enzyme replacement therapy (ERT) with Agalsidase alfa, with positive clinical response. Infusion reactions, initially mild and easily managed, commenced during his 13th infusion, and continued over the next 3 years. Severity of reactions subsequently increased despite very slow infusion, extended prophylactic medication and attempted desensitisation, requiring regular intensive care unit (ICU) admissions.

Pharmacokinetics and safety of Intragam 10 NF, the next generation 10% liquid intravenous immunoglobulin, in patients with primary antibody deficiencies.

Background And Aims: Intragam® 10 NF is the next generation 10% intravenous immunoglobulin with three pathogen reduction steps and a noncarbohydrate stabiliser. This open label, cross-over study in patients with primary immunodeficiency was designed to evaluate whether Intragam 10 NF differed in its pharmacokinetics (PK) compared with Intragam P and to assess Intragam 10 NF safety and tolerability.

Methods: Nineteen primary immunodeficiency patients were administered one cycle of their existing Intragam P dose (0.

B cell-intrinsic signaling through IL-21 receptor and STAT3 is required for establishing long-lived antibody responses in humans.

Engagement of cytokine receptors by specific ligands activate Janus kinase-signal transducer and activator of transcription (STAT) signaling pathways. The exact roles of STATs in human lymphocyte behavior remain incompletely defined. Interleukin (IL)-21 activates STAT1 and STAT3 and has emerged as a potent regulator of B cell differentiation.

General anesthetic allergy testing.

Anaphylaxis in the setting of general anesthesia is a rare but potentially lethal event. The investigation of severe reactions is important for confirming the clinical diagnosis and identifying likely causative agents and safe agents that may be used in the future. Many comprehensive reports have described the testing protocol of individual specialized units, whereas there has been no standardization of testing techniques or formal assessment of these tests' diagnostic accuracy.

Wegener's granulomatosis in pregnancy: a novel approach to management.

Wegener's granulomatosis rarely is diagnosed in women of childbearing age and is, thus, uncommonly encountered in pregnancy. Although conventional treatment with steroids and cyclophosphamide controls disease activity in 90% of patients, the associated teratogenicity of such a regimen warrants careful consideration in pregnancy. We describe successful remission induction with the use of intravenous immunoglobulin and steroids alone in a woman diagnosed with de novo Wegener's granulomatosis during the first trimester of pregnancy.

A new neurosurgical irrigating sucking cutter. Technical note.

A new instrument has been developed for the removal of tumors located in areas difficult to reach. It operates by suctioning and cutting tissue, and is equipped with an irrigating sucker. This device is described and its successful use in clinical practice is summarized.