Gastric cancer is the leading cause of death in Italian adult patients with common variable immunodeficiency

An increased prevalence of malignant lymphoma and of gastric cancer has been observed in large cohorts of patients with common variable immunodeficiency (CVID), the most frequently symptomatic primary immunodeficiency. Surveillance strategies for cancers in CVID should be defined based on epidemiological data. Risks and mortality for cancers among 455 Italian patients with CVID were compared to cancer incidence data from the Italian Cancer Registry database. CVID patients showed an increased cancer incidence for all sites combined (Obs = 133, SIR = 2.4; 95%CI = 1.7\u20133.5), due to an excess of non-Hodgkin lymphoma (Obs = 33, SIR = 14.3; 95%CI = 8.4\u201322.6) and of gastric cancer (Obs = 25; SIR = 6.4; 95%CI = 3.2\u201312.5). CVID patients with gastric cancer and lymphoma had a worse survival in comparison to cancer-free CVID (HR: 4.8, 95%CI: 4.2\u201344.4 and HR: 4.2, 95%CI: 2.8\u201344.4). Similar to what observed in other series, CVID-associated lymphomas were more likely to be of B cell origin and often occurred at extra-nodal sites. We collected the largest case-series of gastric cancers in CVID subjects. In contrast to other reports, gastric cancer was the leading cause of death in CVID. Standardized mortality ratio indicated a 10.1-fold excess mortality among CVID patients with gastric cancer. CVID developed gastric cancer 15 years earlier than the normative population, but they had a similar overall survival. Only CVID diagnosed at early stage gastric cancer survived >24 months. Stomach histology from upper endoscopy performed before cancer onset showed areas of atrophic gastritis, intestinal metaplasia or dysplasia. CVID patients might progress rapidly to an advanced cancer stage as shown by patients developing a III-IV stage gastric cancer within 1 year from an endoscopy without signs of dysplasia. Based on high rate of mortality due to gastric cancer in Italian CVID patients, we hereby suggest a strategy aimed at early diagnosis, based on regular upper endoscopy and on Helicobacter pylori infection treatment, recommending an implementation of national guidelines

Insufficient knowledge among psoriasis patients can represent a barrier to participation in decision-making.

Patient participation in treatment decisions can have positive effects on patient satisfaction, compliance and health outcomes. The objectives of this study were to examine attitudes of psoriasis patients regarding participation in treatment decisions and to evaluate the effect of a decision-aid for discussing treatment options. A "quasi experiment" was conducted in a large dermatological hospital in Italy: a questionnaire evaluating the decision-making process and treatment knowledge was self-completed by 231 consecutive psoriasis patients after routine clinical practice and by a second sample of 171 patients exposed to a decision-board. In routine clinical practice 67.9% of patients wanted to be involved in decision-making, 28.4% wanted to leave decisions entirely to the doctor and 3.7% preferred making decisions alone. 17.9% and 25.3% of the control and decision-board group had good knowledge. At multivariate analysis good knowledge increased the likelihood of preferring an active role (risk ratio (RR) = 2.21; 95% confidence interval (CI) 1.3-3.9; p = 0.006). The decision-board only marginally improved patient knowledge and doctor-patient communication. In conclusion, large proportions of psoriasis patients want to participate in decision-making, but insufficient patient knowledge can represent a barrier

Family Burden in Epidermolysis Bullosa is High Independent of Disease Type/Subtype

Epidermolysis bullosa is a rare, inherited group of disorders characterized by blistering of the skin following friction or mechanical trauma. The aim of this study was to assess the family burden of epidermolysis bullosa in children aged 0-7 years. A postal survey was conducted. The perceived severity of the disease was evaluated by the caregivers, using the Patient Global Assessment 5-point scale. The caregiver received the Family Strain Questionnaire and the 12-item General Health Questionnaire to assess the probable presence of depression/anxiety. A single-item analysis was also performed for questions related to the burden of disease. Forty-two families were invited to participate. Data from 28 young patients and their caregivers were analysed (response rate 66.7%). The family burden increased with increasing caregiver's perceived disease severity, with increasing patient's body surface involved, and if parents had depression/anxiety, reaching statistical significance in several Family Strain Questionnaire scales. The family burden due to epidermolysis bullosa is very high independent of disease type/subtype

Health-Related Quality of Life in Patients with CVID Under Different Schedules of Immunoglobulin Administration: Prospective Multicenter Study

We assessed the health-related quality of life (HRQoL) in CVID adults receiving different schedules of immunoglobulin replacement therapy (IgRT) by intravenous (IVIG), subcutaneous (SCIG), and facilitated (fSCIG) preparations. For these patients, IgRT schedule was chosen after a period focused on identifying the most suitable individual option

Health-related quality of life and nail disorders.

Health-related quality of life (HRQoL) measurements in clinical practice ensure that treatment and evaluations focus on the patient rather than the disease. To verify whether these measurements are also relevant in a minor dermatological disease and whether a generic routine assessment is sufficient to capture different aspects of quality of life, we studied patients affected by nail disorders. The Skindex-29, the 12-item General Health Questionnaire and the 36-item Short Form of the Medical Outcomes Study questionnaires were used. A total of 114 patients were enrolled: 31% men aged 48 +/- 15 years and 69% women aged 49 +/- 14 years (mean +/- SD). The mean values of SF-36 scales in nail disorders were higher than those reported for minor skin diseases, and Skindex-29 values were lower. A duration of disease > or =1 year was associated with higher Skindex-29 scores on the symptom and emotions scales. Patients with onychomycosis have higher values on the Skindex-29 social functioning scale compared with other nail disorders. The use of HRQoL measurements in routine clinical activities allows us to depict how nail disorders affect patients and show how relevant some neglected aspects, such as pain or emotional trauma, are and offers us the opportunity to address these issues in a patient-centred approach to treatment

double blind placebo controlled randomized trial on low dose azithromycin prophylaxis in patients with primary antibody deficiencies

Background Lacking protective antibodies, patients with primary antibody deficiencies (PADs) experience frequent respiratory tract infections, leading to chronic pulmonary damage. Macrolide prophylaxis has proved effective in patients with chronic respiratory diseases. Objective We aimed to test the efficacy and safety of orally administered low-dose azithromycin prophylaxis in patients with PADs. Methods We designed a 3-year, double-blind, placebo-controlled, randomized clinical trial to test whether oral azithromycin (250 mg administered once daily 3 times a week for 2 years) would reduce respiratory exacerbations in patients with PADs and chronic infection–related pulmonary diseases. The primary end point was the number of annual respiratory exacerbations. Secondary end points included time to first exacerbation, additional antibiotic courses, number of hospitalizations, and safety. Results Eighty-nine patients received azithromycin (n = 44) or placebo (n = 45). The number of exacerbations was 3.6 (95% CI, 2.5-4.7) per patient-year in the azithromycin arm and 5.2 (95% CI, 4.1-6.4) per patient-year in the placebo arm (P = .02). In the azithromycin group the hazard risk for having an acute exacerbation was 0.5 (95% CI, 0.3-0.9; P = .03), and the hazard risk for hospitalization was 0.5 (95% CI, 0.2-1.1; P = .04). The rate of additional antibiotic treatment per patient-year was 2.3 (95% CI, 2.1-3.4) in the intervention group and 3.6 (95% CI, 2.9-4.3) in the placebo group (P = .004). Haemophilus influenzae and Streptococcus pneumoniae were the prevalent isolates, and they were not susceptible to macrolides in 25% of patients of both arms. Azithromycin's safety profile was comparable with that of placebo. Conclusion The study reached the main outcome centered on the reduction of exacerbation episodes per patient-year, with a consequent reduction in additional courses of antibiotics and risk of hospitalization

Health Related Quality of Life in Common Variable Immunodeficiency

Purpose: To quantify the health related quality of life in primary immunodeficiency patients. Materials and Methods: We used generic health status and general psychological health questionnaires to determine the range of issues that needed to be considered in examining the burden of common variable immunodeficiency (CVID). Results: The health status of patients with CVID was lower than that observed in normal subjects. Overall, Role-Physical and General Health scales correlated with a poorer clinical status. Surprisingly, the duration of disease did not influence health status. Being female, older, General Health Questionnaire-positive and alexithymic proved to be major risk factors associated with a poor health status. Patients with chronic lung disease and chronic diarrhea had the lowest values on the Medical Outcome Study, Short Form SF-36 (SF-36) scales. Disease severity perception was associated with the General Health Questionnaire and alexithymia status. Limitations in daily activities as a result of lower physical health were the major problems facing common variable immunodeficiency patients. Conclusion: Our data underlined the importance of conducting a periodical health related quality of life assessment on patients with primary antibody deficiencies and, moreover, stressed the necessity of providing psychological support to at risk patients. © Yonsei University College of Medicine 2012

I bisogni delle mamme di bambini affetti da malattie dermatologiche

L’analisi dei bisogni delle mamme di bambini affetti da malattie dermatologiche è essenziale prima di qualunque intervento infermieristico dermatologico. La patologia dermatologica del figlio può determinare un cambiamento nelle priorità dei bisogni delle mamme. Obiettivo dello studio è l’individuazione dei bisogni prioritari delle mamme. Metodo. La raccolta dei dati è avvenuta presso l’IDI-IRCCS di Roma, maggio-giugno 2007. A 160 mamme di bambini ricoverati è stato somministrato un questionario sui bisogni ed è stata valutata la gravità percepita della malattia (Patient Global Assessement -PGA-) del figlio. Risultati. I bisogni principali delle mamme sono di avere: più informazioni sulla diagnosi del figlio (59%), maggiore continuità assistenziale (48%), maggiori informazioni sui trattamenti (36%), una maggiore capacità nella gestione dei disturbi (36%) e di ricevere materiale informativo (33%). Le mamme che valutano più grave la malattia del figlio, esprimono sia un maggiore bisogno di continuità assistenziale (63%) che di controllo dei disturbi (56%). Anche se la malattia del figlio non viene percepita come grave, l’informazione sulla diagnosi rimane la prima priorità. Conclusioni. Informare ed educare le mamme è fondamentale per non escluderle dal processo di cura. Gli infermieri possono migliorare le aree critiche di assistenza dermatologica mediante l’identificazione dei bisogni

Living with psoriasis: prevalence of shame, anger, worry, and problems in daily activities and social life.

Psychosocial problems are frequent among patients with psoriasis. The aim of this study was to analyse the prevalence of some specific psychosocial issues. These were evaluated in 936 patients using the emotions and functioning scales of the Skindex-29 questionnaire. The problems most frequently experienced were: shame, anger, worry, difficulties in daily activities and social life. All problems were associated with the severity of psoriasis and with depression or anxiety. Shame, worry and annoyance were more frequent in women than in men, and shame and anger were associated with a low level of education. Impairment in work/hobbies was significantly higher in patients with palmoplantar psoriasis and those with arthro-pathic psoriasis. In conclusion, clinicians could gain important insights about their patients by looking at the single items of a quality of life instrument, to identify patients with high levels of emotional and social problems, in order to improve quality of care