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Background: The generic 36-item Short-Form Health Survey (SF-36v2) has been used to assess health related quality of life in adult patients with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) even though it has not yet been validated for use in this specific disease.Objective: This study aims to validate the SF-36v2 for use in adult patients with C1-INH-HAE.Results: There was a very low item non-response rate (1-3.4%), with a high ceiling effect in 25/35 items and a low floor effect in 3/35 items. A moderate ceiling effect was observed in 5/8 dimensions of the SF-36v2, whereas no floor effect was noticed in any of the dimensions. Internal consistency was good to excellent with Cronbach's alpha coefficient ranging between 0.82 and 0.93 for the different dimensions. Construct validity was good: seven out of the 8 hypotheses defined on clinical criteria were confirmed, discriminant validity assessment showed significant differences among patients with different C1-INH-HAE severity, convergent validity showed a good correlation among the physical and mental component summaries of the SF-36v2 and the HAE-QoL total score (0.45 and 0.64 respectively, P < 0.001). Test-retest reliability was high with intraclass correlation coefficient varying from 0.758 to 0.962. The minimal clinically important difference was calculated by distribution methods and small differences in the domain scores and in the component summaries scores were shown to be meaningful.  CONCLUSIONS: The psychometric properties of the SF-36v2 show it can be a useful tool to assess HRQoL in adult patients with C1-INH-HAE, although with some content validity limitation.Methods: The psychometric properties of the SF-36v2 were evaluated in an international setting based on responses from 290 adult C1-INH-HAE patients in 11 countries. [ABSTRACT FROM AUTHOR]

The term "swelling" has been used in the old scriptures to illustrate a change of normal figure and, as such, an expression of illness. It should be noted that in ancient times, human diseases were very often regarded a punishment from God. Hence, it is not surprising that one of the oldest tests for infidelity involved swelling as an inflicted punishment. The great Greek physician Hippocrates (377-460 BC), considered one of the most outstanding figures in the history of medicine and "Father of the Western Medicine," already used the term oídēma to describe swelling of organs. It took many centuries later until the first description of angioedema as a distinct medical entity was minted by Quinke in 1882. The historical progression in angioedema research has been characterized by intermittent "leaps" in interest and scientific achievements. As an example, it took 75 years from the accurate description of hereditary angioedema (HAE) by Osler (1888), until a group of researchers headed by Donaldson (1963) disclosed the central role of C1 inhibitor in angioedema pathophysiology. What followed was a result of a collective effort by many researchers and scientific groups who were able to elucidate the intricate connections between the implicated biochemical pathways. Still, scientific progress was hardly translated into effective therapy, and another 45 years had to elapse until the renewed interest in HAE was boosted by studies on the efficacy and safety of novel therapies about 10 years ago. In the twenty-first century, HAE ceased to be an "orphan disease" and its future is far more optimistic. It is better managed now by specialized angioedema centers, harmonized clinical guidelines, educational programs, laboratory services, and continued basic and clinical research. Patient associations worldwide are offering support and guidance, and governments and healthcare systems are gradually addressing patient and family needs.

Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain., Objective: The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting., Methods: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed., Results: Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft version of the HAE-QoL was pilot tested in 332 patients, and accurate data were obtained from 290 patients from 11 countries. The reduction process resulted in a new version with 25 items and 7 dimensions (treatment difficulties, physical functioning and health, disease-related stigma, emotional role and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach's α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0.45 and 0.64, respectively) and with SF-36v2 dimensions (P < .004). HAE-QoL scores discriminated significantly among severity groups (median: asymptomatic 133.5 vs severe 84.0; P < .001); between patients with and without long-term prophylaxis (median: 101 vs 90; P = .001); and between patients with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001)., Conclusions: The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence., (Copyright © 2016 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

There is a gap in the literature on the social information processing (SIP) patterns of adolescents exposed to victimization in school. Therefore, we examine the SIP patterns of young adolescents characterized by their teachers and by their own reports as victims, bullies, bullies/victims, and neither bullies nor victims. The 105 adolescents participating in this study were asked to respond to hypothetical social scenarios in which a protagonist is either rebuffed or provoked by peers. The scenarios were ambiguous in nature and thus could have been processed in different ways. Indeed, distinctive processing patterns were found for each of these groups: victims tended to avoid challenging social situations while expecting others to be purposefully hostile or ignoring; bullies tended to interpret others as purposefully hostile and stated their desire to retaliate; bullies/victims showed patterns more similar to those of the bullies than the victims; and those who were neither victims nor bullies tended to view the same challenging social situations as non hostile and more likely to end well for them. We conclude by discussing the theoretical and practical implications of these findings., (© 2013 Wiley Periodicals, Inc.)

Hereditary angioedema (HAE) is a rare genetic disorder, manifested by recurrent bouts of swelling in various tissues. The biochemical basis leading to HAE manifestations is either a quantitative or qualitative deficiency in plasma C1 esterase inhibitor (C1-INH). Its proposed physiological role is regulation of vascular permeability, by inhibiting certain steps in the complement, coagulation and the fibrinolytic pathways. Recent evidence implies that bradykinin, a plasma kinin, is the main mediator of HAE, Leading to vasodilatation and hyperpermeability of small vessels. Bradykinin receptors have been recently identified, Leading to significant progress in our understanding of the physiologic mechanisms leading to edema. HAE is characterized by edema of the extremities, face, tongue, Larynx, genitalia and severe abdominal pains. Edema of the tongue and Larynx are life-threatening, and fataloutcomes have been described. Diagnosis of HAE is frequently missed, due to the rarity, non-specific symptoms and lack of awareness of physicians to the diagnosis and treatment. In this review, the authors describe the mechanisms of HAE and the clinical approach to diagnosis and management, according to accepted international guidelines. Furthermore, new treatment modalities that have recently been developed are presented: novel molecules targeting bradykinin, either by inhibiting its generation from plasma kininogens or by direct inhibition of its specific receptors on blood vessels, replacement therapy with human recombinant C1-INH produced in transgenic rabbits. HAE is a chronic disabling diseasewith serious consequences for the patients and their families, severely affecting the quality of life. Recently, new clinical guidelines were published and treatment centers, specializing in the management of HAE were established. The authors are convinced that the increased awareness of the medical staff, in addition to new data and novel treatments, will reduce the burden of the disease, improve its grim prognosis and enable a better quality of life for HAE patients.

Hereditary angioedema is a rare genetic disorder, manifested by recurrent edema leading to disfigurement, organ dysfunction and life-threatening respiratory impairment that may become fatal. The hallmark of HAE is C1 esterase inhibitor deficiency, but recent evidence points at bradykinin as the main mediator that causes hyperpermeability of small vasculature, leading to accumulation of edema fluid. Current therapeutic options for HAE are limited, and consist of drugs, replacement therapy, and supportive treatment. In view of many disadvantages of the current therapeutic modalities, new approaches to the treatment of HAE are now being offered. This review summarizes our experience with a new line of medications developed for the treatment of acute exacerbations and prophylaxis of HAE--icatibant: bradykinin receptor antagonist, ecallantide: kallikrein inhibitor, and two C1 INH preparations: Berinert-P, human plasma-derived concentrate, and Rhucin: novel recombinant C1-INH produced in transgenic rabbits. Preliminary results of these studies are encouraging and may bring new hope to the patients with this distressing condition. The exact number of HAE patients in Israel is unknown and because patients are treated individually and comprehensive laboratory assessment is partial, many cases might be missed or not treated according to accepted guidelines. We offer a new specialty center for HAE patients, addressing the medical and psychosocial needs of patients and their families.

Purpose: We evaluated the clinical significance of deoxyribonucleic acid (DNA) ploidy pattern as a predictor of prognosis in patients with testicular seminoma., Materials and Methods: Flow cytometric nuclear DNA analysis was performed on archival specimens from 65 patients with pure seminoma who underwent radical orchiectomy between 1970 and 1992., Results: A total of 42 specimens (65%) exhibited a DNA diploid pattern, while 23 (35%) were DNA aneuploid. Diploidy was manifested in 73% of the stage I tumors versus 31% of stage II cancers (p = 0.004). No correlation was found between ploidy and histological type, size or local extension of the tumor. Tumor progression was observed in 5 patients, exclusively displaying aneuploid histograms (p = 0.0017), and 3 of them subsequently died of the disease., Conclusions: DNA ploidy pattern may provide important prognostic information for patients with testicular seminoma.

In recent years radical retropubic prostatectomy is used with increasing frequency to treat localized cancer of the prostate. We report our experience with 100 consecutive cases of prostate cancer that underwent this operation; there were no cases of mortality and a 7% rate of postoperative complications. More than 90% of the patients are socially continent postoperatively and 20% have retained sexual potency. Ninety-four percent of the patients are free of disease (mean follow-up period 18 months). In our opinion, such results support the use of radical retropubic prostatectomy for the treatment of localized prostate cancer in well-selected patients.