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Heimall J.,Childrens Hospital of Philadelphia | Chen J.,Grifols | Church J.A.,Clinical Immunology and Allergy | Griffin R.,Grifols | And 2 more authors.
Journal of Clinical Immunology | Year: 2016

Purpose: This phase 4, multicenter, open-labeled, single-sequence, crossover study in pediatric patients (ages 2 to 16) with primary immunodeficiency disease (PID) evaluated the pharmacokinetics, safety, and tolerability for subcutaneously (SC) administered 10 % caprylate/chromatography purified human immune globulin injection (IGIV-C, GAMUNEX®) compared with intravenously (IV) administered IGIV-C. Methods: This study included a screening phase, run-in phase (where required), IV treatment phase, SC treatment phase, and end of study/early termination visit. Eligible patients receiving a stable dose of IGIV-C entered into the IV phase to receive two IV infusions of IGIV-C (200–600 mg/kg per infusion) every 3–4 weeks. The weekly SC dose of IGIV-C was calculated using a conversion factor of 1.37 times the prior IV dose. Results: Twelve subjects between the ages of 2 and 16 years participated in the clinical study with the median age being 11 years old. The adjusted weekly mean AUC0-τ,IV was 216,873.7 h*mg/dL for the IV phase versus a mean AUC0-τ,SC of 230,830.0 h*mg/dL for the SC phase. The mean (range) Ctrough was 997.2 (784–1320) mg/dL in the IV phase and 1325.0 (1077–1690) mg/dL in the SC phase. During the SC phase, 100.0 % of the patients (n = 11) experienced treatment-emergent adverse events (TEAEs) that were local infusion reactions and 9 patients (81.8 %) had TEAEs that were non-infusion site reactions. The majority of TEAEs were mild or moderate in severity. Conclusion: In pediatric patients with PID, SC-administered IGIV-C provides comparable overall serum exposure to total IgG to that produced by IV-administered IGIV-C. We have concluded that weekly SC administration of 10 % IGIV-C based on a dose conversion factor of 1.37 is safe and well-tolerated in pediatric patients with PID. Trial Registration: ClinicalTrials.gov identifier: NCT01465958. https://clinicaltrials.gov/ct2/show/NCT01465958?term=NCT01465958.&rank=1 © 2016, Springer Science+Business Media New York. Source


Le T.A.,SA Pathology | Al Kindi M.,SA Pathology | Al Kindi M.,Sultan Qaboos University | Tan J..-A.,Clinical Immunology and Allergy | And 6 more authors.
Internal Medicine Journal | Year: 2016

Background: IgE-mediated allergy to the wheat protein omega-5-gliadin (O5G) is associated with wheat-dependent exercise-induced anaphylaxis (WDEIA), where exercise acts as a cofactor, triggering anaphylaxis after wheat ingestion. The wider application of O5G-specific IgE (sIgE) testing has revealed that the manifestations of O5G allergy extend beyond WDEIA. Aims: This study documents clinical manifestations in a large series of patients with sIgE to O5G. Methods: A retrospective clinical audit was performed on adult patients with a positive O5G sIgE (>0.35kU/L) between 2007 and 2013 compared with a group who had negative O5G sIgE. Clinical characteristics and skin prick test (SPT) results were examined. Results: Sixty-seven patients were characterised, 26 of whom presented with food-dependent exercise-induced allergy, whilst others presented with exercise-induced symptoms without apparent food association (16/67), idiopathic anaphylaxis (10/67), food-induced allergic symptoms without exercise (10/67) or recurrent acute urticaria (5/67). Specific IgE to O5G had 91% sensitivity and 92% specificity for wheat-related allergic symptoms. SPT had sensitivity of 92% and specificity of 84%. Conclusion: WDEIA is the most common manifestation of O5G allergy, but patients may present with a variety of allergic manifestations, and wheat allergy is not always obvious on history. Non-exercise cofactors or a lack of cofactors were identified in many patients. A distinctive feature of this allergy is that despite regular wheat ingestion, allergic reactions to wheat occur infrequently. Testing for sIgE to O5G should be considered in patients presenting with exercise-induced urticaria/anaphylaxis, idiopathic anaphylaxis and recurrent acute (but not chronic) urticaria. © 2016 Royal Australasian College of Physicians. Source


Fabris M.,Clinic of Rheumatology | Grimaldi F.,Unit of Endocrinology | Villalta D.,Clinical Immunology and Allergy | Picierno A.,Laboratory of Immunopathology and Allergy | And 4 more authors.
Autoimmunity Reviews | Year: 2010

Elevated B-Lymphocyte Stimulator (BLyS) and April (a proliferation-inducing ligand) expressions characterize several autoimmune diseases. We here analysed the possible role of BLyS and April in autoimmune thyroid diseases (AITD), comprising Hashimoto's thyroiditis (HT) and Graves' disease (GD). Seventy-seven patients with AITD and 77 blood donors (HBD) were enrolled in the study. Serum BLyS and April levels were assessed by ELISA. Results indicated a significant upregulation of BLyS in AITD patients (1.12 ± 0.39 ng/ml versus 0.666 ± 0.240 ng/ml in HBD; p < 0.0001), with GD patients presenting higher BLyS levels than HT patients (1.22 ± 0.42 ng/ml versus 1.07 ± 0.38 ng/ml; p = 0.0393). In contrast, April levels were downregulated, but only in HT patients [9.9 ± 36.6 (median 0) ng/ml versus 7.4 ± 22.1 (median 1.16) ng/ml in HBD; p = 0.003; and versus 4.2 ± 5.9 ng/ml (median 0.9) ng/ml in GD; p = 0.0353]. In HT patients, Levo-thyroxine supplementation further increased BLyS and tended to normalize April levels. Neither BLyS nor April did correlate with the levels of the pathognomonic autoantibodies (TPOAb, TgAb, TRAb). Data are preliminary, but, for the first time, we provide the analyses of BLyS and April levels in AITD patients, suggesting new tools for the diagnosis, prognosis and possible therapeutic management of AITD. © 2009 Elsevier B.V. All rights reserved. Source


Ryden A.,University of California at Los Angeles | Bando J.M.,Clinical Immunology and Allergy | Aysola R.S.,Critical Care Medicine Clinical Immunology and Allergy
Seminars in Respiratory and Critical Care Medicine | Year: 2014

Continuous positive airway pressure (CPAP) therapy is the first-line treatment for obstructive sleep apnea (OSA). Although the gold standard for the treatment of OSA, CPAP may not be the optimal modality to treat more complex sleep disordered breathing such as Cheyne-Stokes respirations, opioid-induced central apnea, and complex sleep disordered breathing related to chronic hypoventilation syndromes (obesity-hypoventilation syndrome, restrictive thoracic disease due to neuromuscular or thoracic cage disease, chronic obstructive pulmonary disease). Newer generation auto-adjusting PAP devices are increasingly being used to treat OSA. Advanced positive airway pressure modalities have been developed in an effort to improve treatment of the more complex sleep disordered breathing syndromes including automated servo ventilation and volume-targeted pressure-limited ventilation. This article is intended to provide the clinician reader with a description of newer PAP modalities, a review of evidence-supported indications for use, as well as to provide a framework for managing patients with advanced positive airway pressure therapy. © 2014 by Thieme Medical Publishers, Inc. Source


Whyte A.F.,Human Immunology | Smith W.B.,Clinical Immunology and Allergy | Sinkar S.N.,South Australian Institute of Ophthalmology | Kette F.E.,Clinical Immunology and Allergy | Hissaria P.,Human Immunology
Internal Medicine Journal | Year: 2013

Background: Churg-Strauss syndrome (CSS) is a rare, idiopathic systemic vasculitis. There is emerging evidence of an association between the presence or absence of antineutrophil cytoplasmic antibodies (ANCA) and clinical phenotype. Thromboembolism is an increasingly recognised complication of the disease. Aims: Given the paucity of Australian data, the aim of this study was to examine the clinical and laboratory features of CSS in a single Australian centre. Methods: We performed a retrospective review of all patients who fulfilled the American College of Rheumatology classification criteria for CSS managed at the Department of Immunology, Royal Adelaide Hospital between 2002 and 2008. Results: Nineteen patients were included. All patients had asthma and most had upper airway involvement. Peripheral nerve, musculoskeletal, gastrointestinal and cutaneous involvement was common. Renal and cardiac involvement was uncommon in this series. Histological confirmation was obtained in 15 patients (78.9%). Ten patients (52.6%) were ANCA+, and these were more likely to have musculoskeletal involvement, such as arthralgia or myalgia (odds ratio 57, P = 0.005). Thrombosis was a feature at diagnosis in six patients (31.6%); two of these recurred with relapse. Sixteen patients (84.2%) were followed up; five died, and mean survival was 8.9 years. Conclusions: This is the first Australian study to focus on CSS. Our results demonstrate similar presentation and prognosis of CSS to previous descriptions; however, we noted that musculoskeletal involvement was more common in ANCA+ patients. In our series, thrombosis was a significant complication and we suggest that thromboprophylaxis may be warranted. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians. Source

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