Objective: Hereditary angioedema (HAE) is a rare autosomal dominant disorder characterized by recurrent angioedema attacks, without itching or urticaria. With this study, we aimed to increase the awareness of HAE by presenting the characteristics of these patients who applied to the emergency department and allergy immunology clinic. Material and Methods: A total of 38 patients, 25 (65.8%) female and 13 (34.2%) male, were included. Results: The mean age was 40.90±12.66, mean age at the onset of symptoms was 13.5 (1-56), mean age at HAE diagnosis was 24.61±13.78, and the diagnostic delay was 8.84±8.97 years. Of all cases, 18 (47.4%) were followed-up with Type I HEA and 20 (52.6%) were followed up with Type II HAE. A family history of HAE was present in 89.5% and a family history of death due to HAE was present in 31.6% of the patients. Mean age at diagnosis differed significantly between those with or without a family history of death due to HAE (18.0±7.24 vs. 27.65±15.08 years; p=0.043). The episodes were triggered by stress in 20 (52.6%) patients. The symptoms at first presentation included swelling in extremities in 18 (47.4%) patients. Conclusion: Although HAE is a rare disorder associated with variable clinical presentations complicating the diagnostic process, it may also be associated with mortality. Periodic reporting of clinical experience from centers dealing with HAE patients bears significance not only for increasing awareness among medical professionals and preventing diagnostic delays but also for improving the life quality of patients as well as for decreasing the morbidity and mortality.
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Background/aim: Although allergic diseases are generally considered to be diseases of childhood and youth, the first symptoms of allergic diseases can be seen in old age sometimes. The aim of this study was to determine the prevalence and characteristics of allergic diseases in the elderly population admitted to the allergy unit on an outpatient basis. Materials and methods: The files of the patients who applied to our clinic’s allergy unit during the 8-year period were retrospectively analyzed. The data of patients aged ≥ 65 years were obtained from the files of our allergy unit archive. Results: A total of 1272 patients aged ≥ 65 years old were included in the study. The mean age was 70 years (range: 65–97 years). Most of the patients were female (n = 704, 55.3%). Of the patients, 887 (69.8%) presented with cutaneous symptoms, and urticaria was identified in 500 of them (56.3%). Drug hypersensitivity reactions were detected in 175 (13.7%) patients. A total of 71 (5.6%) patients had asthma, 65 (5.1%) had anaphylaxis, 48 (3.8%) had allergic rhinitis, 24 (1.9%) had hymenoptera venom allergy, and 18 (1.4%) had food allergies. Atopy history (OR = 2.323, 95% CI = 1.590–3.393, p < 0.001) and comorbidity (OR = 1.631, 95% CI = 1.050–2.533, p = 0.029) were found to be risk factors for drug hypersensitivity reactions. Male sex (OR = 3.462, 95% CI = 1.097–10.933, p = 0.034) and atopy history (OR = 14.877, 95% CI = 6.081–36.393, p < 0.001) were found to be risk factors for hymenoptera venom allergy. Conclusion: Diagnosis becomes difficult due to the perception that allergic diseases mainly affect young people. Clinical symptoms are not evident in the elderly and age-related difficulties are encountered in diagnostic tests. There is a need to develop specific guidelines for the diagnosis of allergic diseases in the elderly. Key words: Elderly, allergic diseases, allergic rhinitis, drug hypersensitivity reactions, hymenoptera venom allergy, anaphylaxis
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Objective: Common Variable Immunodeficiency (CVID) is a primary antibody disorder characterized by impaired B cell differentiation.Patients commonly present with acute and chronic sinusitis as well as otitis media, which may lead to hearing loss.Materials and Methods: Thirty-three CVID patients (20 male /13 female) with a mean age of 35 years (range 19-65 years) and 33healthy individuals as a control group were included.Results: Among CVID patients, 17 (51.5%) had conductive hearing loss (CHL) , being unilateral in 4 (12.1%) and bilateral in 13 (39.4%).Unilateral and bilateral sensorineural hearing loss (SNHL) were detected in 2 (6.1%) and 5 (15.2%) respectively. CD4/CD8 cell ratio wassignificantly lower while CD8+ T lymphocyte ratio was significantly higher in those with CHL than in those without it (p=0.045 andp=0.009). Elevated CD8+ T cell ratio was an independent risk factor for CHL (p=0.015). Patients with SNHL were significantly olderthan those without it (p=0.040). CD16-56+ cell count was significantly lower in those with SNHL (p=0.031).Conclusion: CVID patients have an increased occurrence of CHL and SNHL, regardless of the cause. They provide evidence for thenotion that these two types of hearing loss are not unrelated, immune dysregulation also plays a role in the process, and SNHL is notindependent of CHL.
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Background/aim: Common variable immunodeficiency (CVID) is a heterogeneous primary deficiency characterized by hypogammaglobulinemia, recurrent infections, an increased risk of autoimmune disease, malignancy, and chronic inflammation. Proteinuria is one of the most important prognostic factors causing progression in kidney disease. Proteinuria causes tubulotoxicity, activates inflammatory markers that cause fibrosis, and consequently nephropathy progression. The data is scant in the literature regarding the inflammation and nephropathy in CVID. Hence, in the present study, we aimed to investigate the relationship between tubular dysfunction, proteinuria, and inflammation in patients with CVID. Materials and methods: This was a cross-sectional study involving 27 patients with CVID (15 females, 12 males; mean age, 39.88 ± 13.47 years) and 18 control subjects (10 females, 8 males; mean age, 33.83 ± 7.97 years). Patients were evaluated for kidney functions including glomerular filtration rate, fractional excretion of sodium, metabolic acidosis, serum/urine anion gap, 24-h urine proteinuria and, were grouped in terms of proteinuria. Blood samples obtained from the patients with CVID were taken into 2 mL EDTA tube to evaluate peripheral NK cell subgroups according to CD56 and CD16 expression and CD3, CD4, CD 8 expression to determine subtypes T cells. These cells were evaluated by flow cytometry technique. Results: Urinary density, fractional excretion of sodium, proteinuria, and metabolic acidosis are found to be higher in patients with CVID when compared to healthy controls. In the bivariate correlation analysis, proteinuria was positively correlated with age (r = 0.496, p = < 0.001), CD8+T cells percentage (r = 0.427, p = 0.02). Albumin, CRP, and CD8+T cell percentage were found to be independent variables of proteinuria. Conclusion: Increased chronic ongoing inflammation was found to be associated with proteinuria in patients with CVID. Hence, in routine outpatient clinics, proteinuria should not be overlooked in this group of patients. Key words: Chronic inflammation, proteinuria, common variable immunodeficiency
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Fatih ÇÖLKESEN ,
Oğuzhan KILINÇEL ,
Mehmet SÖZEN ,
Eray YILDIZ ,
Şengül BEYAZ ,
Fatma ÇÖLKESEN ,
Gökhan AYTEKİN ,
Mehmet Zahid KOÇAK ,
YAKUP ALSANCAK ,
Murat ARAZ ,
Şevket ARSLAN
Objective: The effect of the COVID-19 pandemic on mental health in the long term is unclear. We evaluated severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)–related transmission fear and mental-health disorders in populations at high risk for COVID-19. Materials and Methods: Healthcare workers and patients with primary immunodeficiency disorders (PIDs), severe asthma, malignancy, cardiovascular disease, hypertension, and diabetes mellitus were included in the study. The hospital anxiety and depression scale (HADS) and Fear of Illness and Virus Evaluation (FIVE) scales were applied during face-to-face interviews. Results: There was a total of 560 participants, 80 per group; 306 (55%) were female. The FIVE and HADS-A scale scores of health care workers were significantly higher than the other groups (p = 0.001 and 0.006). The second-highest scores were in patients with PID. There was no significant difference between the groups in HADS-D scores (p = 0.07). There was a significant positive correlation between FIVE scale scores and anxiety (r = 0.828; p < 0.001) and depression (r = 0.660; p < 0.001). The FIVE scale had significant discriminatory power for anxiety (AUC = 0.870, 95% confidence interval [CI] = 0.836–0.904; p < 0.0001) and depression (area under the curve = 0.760, 95% CI = 0.717–0.803; p < 0.0001). Conclusion: During the COVID-19 pandemic, mental-health disorders may develop in patients with comorbidities, especially healthcare workers. They should be referred to mental-health centers.
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Social distancing, curfew restrictions, travel restrictions and quarantines were imposed in order to reduce the risk of transmission of SARS-CoV-2. These restrictions have caused a severe reduction in admissions to hospitals. Therefore, with this study, we aimed to investigate effect of the COVID-19 pandemic on treatment processes and adherence to therapy of the patients that require to receive omalizumab due to chronic urticaria and to address effect of the pandemic on anxiety level of this patient group. Among the patients who were being followed-up for chronic urticaria and receiving omalizumab for this, files of the patients were examined retrospectively. These patients were applied a mini survey and a validated coronavirus anxiety index. Ninety-eight patients (Female: 65; Male: 33) were recruited in the study. The patients’ 3-month adherence to therapy during March-April-May 2020 was 42.9%. It was the lowest during April (56.1%). The curfew (51%) and the fear of contracting SARS-CoV-2 (50%) were the most common reasons. Although rates of admissions to emergency department, an increase in symptoms of urticaria and need for an additional treatment were higher in the patients with chronic urticaria who were nonadherent to omalizumab therapy, this difference was not statistically significant (p: 0.113, p: 0.216, p: 0.141, respectively). During the pandemic, patients’ adherence to therapy has reduced due to both government-related and patient-related reasons. For patients with chronic urticaria, their adherence to therapy should be ensured by minimizing the risk of transmission of SARS-CoV-2. Furthermore, in order to enhance adherence to therapy of this patient group and make access to health institutions easier, it should be considered for these patients to provide some tolerances and privileges in pandemic-related restrictions.
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Objective: Common Variable Immune Deficiency (CVID) is a heterogeneous immune disorder characterized by impaired and/or inadequate B cell differentiation with hypogammaglobulinemia. It is characterized by frequent and recurrent respiratory infections, autoimmune disorders, chronic lung diseases, granulomatous diseases, and increased risk for lymphoid malignancies.Materials and Methods: The medical records of 47 patients (22 females, 25 males) who had been followed up at our clinic and had sufficient data in their files were retrospectively reviewed. Patients were diagnosed with CVID according to the ESID (European Society for Immunodeficiency) criteria.Results: The median age of the patients was 32 (19-65) years. The most frequent clinical presentation of the patients was with recurrent upper respiratory infections (46%), pneumonia (29.8%), otitis media (23.4%) and chronic sinusitis (17%). During the follow-up period, 17 patients (36.8%) developed autoimmune complications, 14 (29.8%) of whom had autoimmune cytopenia. A total of 26 patients (55.3%) had bronchiectasis confirmed with computed tomography of the thorax. Lymphopenia was detected in 13 patients (27.7%). The median immunoglobulin level at the time of diagnosis was IgG 2.77 (0.33-6.90) g/L, IgM 0.31 (0.06-5.99) g/L, and IgA 0.25 (0.01-5.02) g/L. Conclusion: CVID is very heterogeneous in terms of both clinical and laboratory features. Moreover, it is more common than expected, particularly in adulthood. The centers dealing with CVID should share their experiences in order to increase awareness among physicians.
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Common variable immune deficiency (CVID) is a rare primary immunodeficiency disorderthat is characterized by defective antibody production and inadequate B cell differentiation.While frequently recurrent respiratory tract infections are the most prominent clinical featurein CVID patients, CVID is a heterogeneous immune deficiency disorder that involves manysystems and organs such as lymphoid hyperplasia, autoimmune cytopenia, chronic lungdiseases, granulomatous diseases and susceptibility to malignancy. This may lead to delay indiagnosis and immunoglobulin replacement therapy, not being able to receive antibiotics at theappropriate dose and time, chronic inflammation, and therefore secondary amyloidosis. In thiscase report it is aimed to present a CVID patient with autoimmune complications anddeveloping renal amyloidosis during follow-up.
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Objective: Common variable immunodeficiency (CVID) is a relatively frequent primary immunodeficiency disorder characterized byimpaired B cell differentiation with hypogammaglobulinemia in the European Society for Immunodeficiencies (ESID) registry system.Increased inflammatory cytokines, prolonged and/or recurrent infections, gastrointestinal complications, and anti-inflammatorymedications are risk factors for diminished bone mineral density (BMD) in CVID patients.Materials and Methods: The study group included 32 patients with CVID (19 males and 13 females; mean age: 37.33 ± 13.70 years,40.6% female), who had been followed up on a regular basis for a period of four years. The patients were categorized into two groupsaccording to their BMD: low BMD and normal BMD.Results: Seventeen patients (8 females and 9 males) had normal BMD (mean age 34.94 ± 11.41 years, 47.1% female) and fifteen patients(5 females and 10 males) had low BMD (mean age 40.03 ± 15.87 years, 33.3% female). In the low BMD group, three patients hadosteoporosis and 12 patients had osteopenia. Univariate regression analysis revealed that lymphopenia (odds ratio, OR:6.562, 95%confidence interval, CI: 1.095-39.324, p=0.039) was significantly associated with low BMD. Multivariate regression analysis showed thathigher alkaline phosphatase (ALP) levels (OR:1.017, 95% CI 1.001-1.033, p=0.041), lymphopenia (OR:11.028, 95% CI 1.326-91.709,p=0.026), and lower folic acid levels (OR:1.284, 95% CI 1.007-1.637, p=0.043) were also independent predictors for low BMD.Conclusion: Even with some limitations such as the small number for the study population, a single center experience, and a crosssectional design, we recommend that clinical immunologists should be alert for diminished BMD in CVID patients, especially thosewith low folic acid and high ALP levels and lymphopenia.
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Objective: To evaluate the knowledge levels, sensitivity status, familial latex sensitivity, and attitudes towards the prevention andtreatment of latex allergies of healthcare workers (HCWs) at a tertiary hospital.Materials and Methods: The study was carried out cross-sectionally between December 2012 and March 2013. A total of 566 HCWs ata tertiary hospital were included in the study.Results: The data of a total of 566 [333 (58.8%) female and 233 (41.2%) male] HCWs were analyzed. They consisted of 179 (31.6%)physicians, 48 (8.5%) technicians, 238 (42%) nurses, 48 (8.5%) laboratory technicians and 53 (9.4%) patient care workers. The familyhistory of atopy was significantly higher in female HCWs (24.3%) compared to males (17.2%) (p= 0.041). A significant differencewas identified between the occupational groups in terms of the rate of allergic symptoms after coming into contact with medical latexproducts (nurses 59.7%, doctors 17.6%, technicians 5.7%, laboratory technicians 6.9 %, patient care workers 10.1%; p= 0.001). Latexrelated symptoms were significantly more common in atopic HCWs (52.3%) compared to non-atopic ones (19.4%) (p= 0.001). The rateof latex-food syndrome was significantly more frequent in female HCWs (16%) compared to males (8.9%) (p= 0.038). Symptoms thatdeveloped after contact with medical and non-medical latex products were significantly more common in female HCWs (79.9% and80.5%) compared to the male HCWs (21.1% and 19.5%) (p= 0.001). The rate of non-HCW(s) who shared the same house/room with theHCWs after work and who had allergic symptoms while in the same environment with the HCWs was 18%. The rate of these individualswas reported to be highest among the nurses at 53% and there was a statistically significant difference in terms of occupational groups(physicians 25.5%, technicians 9.8%, laboratory technicians 2.9%, patient care workers 7.8%; p= 0.030). Multivariate regression analysisshowed that personal history of atopy (OR= 28.657, 95% CI= 6.548-125.411, p= 0.001) and the type of gloves used (latex gloves) (OR=8.730, 95% CI= 3.490-21.834, p= 0.001) were independent predictors for latex allergy.Conclusion: In conclusion, latex is not only a cause of occupational allergy but is also an allergen that has the potential to cause allergicsymptoms in people who share the same environment with HCWs. Questionnaires questioning the symptoms associated with past latexallergy may be an important tool for demonstrating latex sensitization in HCWs and managing latex-related reactions.
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