Therapeutic apheresis in kidney AB0 incompatible transplantation.

Desensitization strategies to safely perform ABO incompatible living donor kidney transplantations are various and still evolving. Given the successful outcome of the majority of the approaches, the current trend is focused on a minimization of treatments. Based on this consideration, the evolution at a single Center of the desensitization protocol is herein described. Starting from 2010, 58 AB0 incompatible living donor kidney transplantations were performed at the University-Hospital of Padua. Over the years, the initial desensitization strategy with rituximab single-dose induction, pre-and post-transplant plasmapheresis and CMV-specific immunoglobulin administration has been shifted to a minimized approach, omitting post-transplant antibody removal in 25 cases. The results of such reduction in post-transplant antibody removal did not affect the outcome of AB0-incompatible kidney transplants, with a reduction in costs and hospitalization.

Anxiety and Depression Effects During Drug Provocation Test.

BACKGROUND: Drug provocation test (DPT) represents the gold standard for the diagnosis of drug allergy. A DPT can be performed in a single-blind placebo-controlled manner. In anxiety and depressive disorders, patients need to be evaluated to understand the nature of placebo reactions. OBJECTIVE: The aim of this study was to evaluate the psychological profile of patients with reactions to placebo during a DPT. METHODS: We consecutively enrolled patients with suspected drug allergy undergoing a DPT preceded by the administration of the placebo. All patients underwent the Hospital Anxiety and Depression Scale (HADS), a questionnaire aimed to identify anxiety and depression, before the challenge test. RESULTS: A total of 196 patients were enrolled into this study: 8 (4%) patients resulted positive to the DPT, 60 (30.6%) demonstrated anxiety or depression based on the HADS, and 54 had at least 1 placebo reaction during drug provocation. There were statically significant correlations between the positivity of the HADS and the finding of a placebo reaction (Fisher's exact test: P < .001), and between the latter and a history of severe reactions to drug (Fisher's exact test: P < .001). CONCLUSIONS: There is a significant and strong correlation between the loss of psychic equilibrium and the development of a placebo reaction during a DPT. We suggest the use the HADS or other validated questionnaire in clinical practice before a DPT to evaluate the possible psychiatric components.

Chronic Urticaria Patient Perspective (CUPP): The First Validated Tool for Assessing Quality of Life in Clinical Practice.

BACKGROUND: There is a need for validated tools to assess health-related quality of life (HRQoL) in routine clinical practice. OBJECTIVE: The aim of this study was to validate the Chronic Urticaria Patient Perspective (CUPP) for assessment of patients with chronic urticaria (CU) in clinical practice. METHODS: A provisional CUPP was developed from candidate items identified by following an iterative process in a retrospective analysis of 249 Chronic Urticaria Quality of Life Questionnaire questionnaires. The psychometric properties of the CUPP were then tested on a sample of patients enrolled in 13 Italian centers. RESULTS: The study population in the validation phase comprised 152 patients. The 10-item version of the CUPP showed satisfactory internal consistency (Cronbach's alpha values of 0.76 at visit 1 and 0.90 at visit 2), good criteria, and discriminative and convergent validity. Reliability was assessed in 34 patients with no changes in health (Global Rating Scale = 0 at visit 2) and was satisfactory (CCC [concordance correlation coefficient] = 0.9). Changes in CUPP scores were significantly associated with changes in Urticaria Activity Score (UAS)-Hive count (r = 0.36, P < .001), UAS-Itch severity (r = 0.48, P < .001), and UAS-Total score (r = 0.342, P < .001), all of which indicated good responsiveness. The minimal important difference was 1.5. CONCLUSIONS: CUPP is a simple 10-question tool with good psychometric properties that provides a valid, reliable, and standardized measurement of HRQoL in patients with CU.

Addison's disease: a survey on 633 patients in Padova.

OBJECTIVE: Addison's disease (AD) is a rare endocrine condition. DESIGN: We aimed to evaluate clinical, immunologic, adrenal imaging, and genetic features in 633 Italian patients with AD followed up since 1967. METHODS: Adrenal cortex autoantibodies, presence of other autoimmune and nonautoimmune diseases, nonadrenal autoantibodies, adrenal imaging, and genetic profile for HLA-DRB1 and AIRE were analyzed. RESULTS: A total of 492 (77.7%) patients were found to be affected by autoimmune AD (A-AD), 57 (9%) tuberculous AD, 29 (4.6%) genetic-associated AD, 10 (1.6%) adrenal cancer, six (0.94%) post-surgical AD, four (0.6%) vascular disorder-related AD, three (0.5%) post-infectious AD, and 32 (5.1%) were defined as idiopathic. Adrenal cortex antibodies were detected in the vast majority (88100%) of patients with recent onset A-AD, but in none of those with nonautoimmune AD. Adrenal imaging revealed normal/atrophic glands in all A-AD patients: 88% of patients with A-AD had other clinical or subclinical autoimmune diseases or were positive for nonadrenal autoantibodies. Based on the coexistence of other autoimmune disorders, 65.6% of patients with A-AD were found to have type 2 autoimmune polyendocrine syndrome (APS2), 14.4% have APS1, and 8.5% have APS4. Class II HLA alleles DRB1*03 and DRB1*04 were increased, and DRB1*01, DRB1*07, DRB1*013 were reduced in APS2 patients when compared with controls. Of the patients with APS1, 96% were revealed to have AIRE gene mutations. CONCLUSIONS: A-AD is the most prevalent form of adrenal insufficiency in Italy, and ∼90% of the patients are adrenal autoantibody-positive at the onset. Assessment of patients with A-AD for the presence of other autoimmune diseases should be helpful in monitoring and diagnosing APS types 1, 2, or 4 and improving patients' care.

Quality of life in European patients with Addison's disease: validity of the disease-specific questionnaire AddiQoL.

CONTEXT: Patients with Addison's disease (AD) self-report impairment in specific dimensions on well-being questionnaires. An AD-specific quality-of-life questionnaire (AddiQoL) was developed to aid evaluation of patients. OBJECTIVE: We aimed to translate and determine construct validity, reliability, and concurrent validity of the AddiQoL questionnaire. METHODS: After translation, the final versions were tested in AD patients from Norway (n = 107), Sweden (n = 101), Italy (n = 165), Germany (n = 200), and Poland (n = 50). Construct validity was examined by exploratory factor analysis and Rasch analysis, aiming at unidimensionality and fit to the Rasch model. Reliability was determined by Cronbach's coefficient-α and Person separation index. Longitudinal reliability was tested by differential item functioning in stable patient subgroups. Concurrent validity was examined in Norwegian (n = 101) and Swedish (n = 107) patients. RESULTS: Exploratory factor analysis and Rasch analysis identified six items with poor psychometric properties. The 30 remaining items fitted the Rasch model and proved unidimensional, supported by appropriate item and person fit residuals and a nonsignificant χ(2) probability. Crohnbach's α-coefficient 0.93 and Person separation index 0.86 indicate high reliability. Longitudinal reliability was excellent. Correlation with Short Form-36 and Psychological General Well-Being Index scores was high. A shorter subscale comprising eight items also proved valid and reliable. Testing of AddiQoL-30 in this large patient cohort showed significantly worse scores with increasing age and in women compared with men but no difference between patients with isolated AD and those with concomitant diseases. CONCLUSION: The validation process resulted in a revised 30-item AddiQoL questionnaire and an eight-item AddiQoL short version with good psychometric properties and high reliability.