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Objective: Hereditary angioedema (HAE) is an autosomal dominant disorder characterized by recurrent episodes of angioedema without urticaria or pruritus. In this study, we compared the levels of anabolic hormones, such as insulin, insulin-like growth factor, growth hormone, and thyroid hormones (thyroid-stimulating hormone [TSH], triiodothyronine [T3], and thyroxine [T4]), and the levels of hormones that are considered catabolic, such as adrenocorticotrophic hormone (ACTH) and cortisol, between HAE patients and controls. We also discuss the contribution of these hormones to the pathophysiology of HAE. Methods: The study included 18 patients (9 diagnosed with HAE type 1 and 9 with HAE type 2) who were followed in the immunology and allergy clinic between January 2013 and January 2020. The control group comprised 28 age- and gender-matched subjects. Results: The HAE type 1, HAE type 2, and control groups showed no significant differences in insulin, insulin-like growth factor, ACTH, cortisol, TSH, or T4 levels. The C-peptide and T3 levels were significantly different between the groups (p = 0.011 and p = 0.027, respectively) (Table 3). Post-hoc pairwise comparison revealed no significant difference in C-peptide level among the groups, but a significant difference in the T3 level was detected between HAE type 1 patients and controls (p = 0.029) Conclusions: Although no significant differences were observed in other anabolic hormone levels between the controls and HAE patients, T3 levels were significantly lower in type 1 HAE patients. Close monitoring of low T3 levels is required, particularly in patients with type 1 HAE.

Purpose of the study The aim of this study was to investigate the relationship of B cell-mediated immunity with disease severity and mortality in patients with COVID-19. Study design In this retrospective cohort and single-centre study, 208 patients with laboratory-confirmed COVID-19 were recruited. A COVID-19 severity score, ranging from 0 to 10, was used to evaluate associations between various factors. Serum immunoglobulin levels and the number of cells in B lymphocyte subsets were measured and their association with disease severity and mortality in patients with COVID-19 examined. Results The median age of the patients was 50 (35–63) years and 88 (42%) were female. The number of deceased patients was 17. The median COVID-19 severity score was 8 (6–8) in deceased patients and 1 (0–2) in survivors. Deceased patients had significantly lower levels of total B lymphocytes, naive B cells, switched memory B cells, and serum IgA, IgG, IgG1 and IgG2 than recovered patients (all p Conclusion In the present study, the prognosis of patients with COVID-19 was shown to be associated with the B cell subset and serum immunoglobulin levels.

Objective: The aim of this study was to determine the clinical course and treatment outcomes of patients with hereditary angioedema (HAE) after infection with coronavirus disease 2019 (COVID-19). Materials and Methods: Thirty-nine patients with HAE were included in this study. These patients were regularly followed up over phone calls since the first COVID-19 case was seen in our country. Patients were asked to visit the hospital if there was a history of contact with a confirmed COVID-19 patient or if the patient developed clinical symptoms of COVID-19.Results: There were 21 (54%) patients with type I HAE, and 18 (46%) with type II HAE. All patients received treatment for angioedema attacks (C1-inhibitor [C1-INH], icatibant), and seven (20%) received long-term prophylaxis (danazol). Treatment for attacks was continued for all patients during the pandemic. Patients taking danazol were switched to long-term prophylaxis using the C1-INH concentrate. Eleven (28%) patients with HAE developed COVID-19 during this study. Only one patient had severe COVID-19. Six patients (54.5%) were diagnosed with type II HAE, and five (45.5%) were diagnosed with type I HAE. The most common COVID-19 symptoms were fever (7/11; 64%) and myalgia (6/11; 55%). Mild angioedema attacks were experienced by 36% (4/11) of the HAE patients diagnosed with COVID-19. Icatibant was used in all patients.Conclusion: Agents used for HAE block the kallikrein-kinin system and may be useful in the treatment of COVID-19. Considering their beneficial effects on COVID-19, it is recommended that HAE patients should continue the use of agents blocking the kallikrein-kinin system. Keywords: COVID-19, hereditary angioedema, kallikrein-kinin system, bradykinin, C1-INH

BACKGROUND 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 1,272 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 gender (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.

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.

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. Keywords: Asthma, COVID-19, fear of virus transmission, mental health, primary immunodeficiency

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 33 healthy 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 was significantly lower while CD8+ T lymphocyte ratio was significantly higher in those with CHL than in those without it (p=0.045 and p=0.009). Elevated CD8+ T cell ratio was an independent risk factor for CHL (p=0.015). Patients with SNHL were significantly older than 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 the notion that these two types of hearing loss are not unrelated, immune dysregulation also plays a role in the process, and SNHL is not independent of CHL. Keywords: Common variable immune deficiency, conductive hearing loss, sensorineural hearing loss

Objective: Coronavirus Disease 2019 (COVID-19) is characterized by high fever, sudden developing respiratory distress, and radiological findings failing to respond to conventional treatments. The purpose of this study is to identify the association of D-dimer levels and lymphocyte counts with poor prognosis and to predict the clinical course in patients with COVID-19. Methods: A total of 118 hospitalized adult patients diagnosed with COVID-19 were included in the study. According to the National Institutes of Health (NIH) COVID-19 treatment guidelines, patients were divided into two groups with severe disease (n= 26) and non-severe (n= 92) disease. Detected at the time of diagnosis, D-dimer levels and lymphocyte counts were compared between severe and non-severe COVID-19 patient groups. Distinctive performance analysis of these values was performed, and cut-off values were determined. Results: The mean age of patients was 62 ± 7 years (range 42-80 years), and 63 (53.4 %) were female. The lymphocyte count was lower (p

Background: The aim of this study was to examine the clinical course and outcomes of patients with diabetes mellitus (DM) with coronavirus disease-2019 (COVID-19).Methods: This retrospective, single-center study included 185 adult patients diagnosed with COVID-19. All patients were separated into 4 groups. Group 1(n=79): patients with no accompanying disease, Group 2 (n=14): patients with only DM, Group 3 (n=31): patients with comorbid disease(s) including DM, Group 4 (n=61): patients with comorbid disease(s) without DM. Data about COVID-19 management and outcome were obtained from the medical records of the patients. COVID-19 was confirmed by real-time polymerase chain reaction (RT-PCR) from throat swab samples. All patients underwent chest x-rays or chest computed tomography.Results: 185 patients diagnosed with COVID-19 were evaluated. The COVID-19 prognoses of the patients were classified as good, moderate and poor. No statistically significant difference was determined between the groups in terms of COVID-19 prognosis (p>0.05). While the rate of DM patients with a good prognosis was 20.4%, the DM patient rate increased up to 40% among moderate or poor prognosis patients. A statistically significant difference was observed between blood glucose levels and mortality (p: 0.008). Mortality due to COVID-19 pneumonia developed in 15 (8.1%) patients. Mortality increase was mostly encountered in the group with DM and accompanying comorbidities. It was observed that ACEI / ARB use had no effect on mortality.Conclusions: Although the study results do not show a statistically significant effect of DM on the prognosis of COVID-19 patients, the higher rate of DM patients in the group with poor prognosis suggests that it may affect the severity of COVID-19. These results may be useful for clinicians in the management of DM patients with COVID-19.

Upper respiratory tract infections (URTI) one of the most common clinical situations encountered by primary care physicians and internal medicine specialists. Viruses are the most common etiologic cause and the clinical picture is usually self-limiting. However, the symptoms of viral URI are substantially similar to those of major pharyngitis agents such as group A Streptococcus (GAS). When atypical presentations and atypic bacterial infections are suspected, microscopic examination of the throat culture is a frequently used laboratory test. Atypical microorganisms detected as a result of throat culture are evaluated as laboratory error and / or contamination and apossible underlying immunodeficiency conditions are overlooked. The diagnosis of the patients is delayed due to the perception of immune deficiency as childhood diseases and the lack of awareness in this regard. In this case, we aimed to present a patient with recurrent tonsillopharyngitis complaints whose first throat culture results was possitive for a gram (-) microorganism and second throat culture was positive for pseudomanas aerigunosa. As a result of further investigations of the patient's lung, bronchiectasis sequelae and diminished count of switched memory B cells were detected. The patient was diagnosed with common variable immunodeficiency, and her complaints disappeared completely with 400 mg / kg intravenous immunoglobulin replacement. In conclusion, it is very important to evaluate the patients especially in the presence of stimulant signs in terms of immunodeficiency, or to refer them to the Immunology department, to manage the complications.

Aim: In this study, we aimed to determine the epidemiological characteristics of infections detected in a tertiary ICU of our hospital and to evaluate the transmission routes.Materials and Methods: In the period of study, a total of 57 health-care associated infection (HAI) cases were diagnosed. Health-care associated infection diagnoses were evaluated according to the "Centers for Disease Control and Prevention (CDC)" criteria. Results: A total of 1278 patients who were followed up in the ICU for two years were analyzed retrospectively. Health-care associated infections were detected in 57 of these patients. The rate of HAI development among patients hospitalized in the ICU was 5.24%. Pneumonia with specific laboratory findings was the most common infection in patients with HAI (33.3%). Eleven different microorganisms were determined as the pathogens of HAI. The bacteriological profile causing HAI in the study, Gram-negative and Gram-positive pathogens were 87% and 12.9%, respectively. Acinetobacter baumanni was the most common pathogen (51.8%). Conclusion: Each unit should determine its patient profile, flora and resistance patterns by conducting surveillance studies and plan treatment strategies accordingly.Keywords: Health-care associated infection, intensive care unit, surveillance., Amaç: Bu çalışma ile hastanemizin üçüncü basamak bir yoğun bakım ünitesinde (YBÜ) tespit edilen sağlık bakımı ilişkili enfeksiyonların (SBİE) epidemiyolojik özelliklerinin belirlenmesi ve bulaş yolları ile ilgili değerlendirilme yapılması amaçlanmıştır.Gereç ve Yöntem: YBÜ’de iki yıl boyunca takip edilen toplam 1278 hastanın takipleri retrospektif olarak incelendi. SBİE tanısı "Hastalık Kontrol ve Önleme Merkezleri (CDC)" kriterlerine göre değerlendirildi.Bulgular: Takip edilen hastaların 57 tanesinde SBİE tespit edildi ve 54 etken saptandı. Yoğun bakıma yatırılan hastalar arasında SBİE gelişme oranı %5,24 olarak hesaplandı. Bu ünitede; mekanik ventilatör kullanım günü 3483, ventilatör kullanım oranı %51, VİP hızı 0,86, ÜK kullanım günü 6734, ÜK kullanım oranı %100, Kİ-ÜSE hızı 0,89, SVK kullanım günü 4327, SVK kullanımoranı%63, SVKİ-KDE hızı 3,93 olarak belirlendi. SBİE görülen hastalarda en sık spesifik laboratuvar bulguları olan pnömoni tespit edildi (%33,4). Bunu sırasıyla alt solunum yollarının diğer enfeksiyonları (%31,6), SVKİ-KDE (%14), Kİ-ÜSE (%7), VİP (%5,2), klinik olarak tanımlanmış pnömoni (%5,2), yumuşak doku enfeksiyonu (YDE) (%1,8) ve laboratuvar tarafından kanıtlanmış KDE (%1,8) izledi. SBİE etkeni olarak 11 farklı mikroorganizma saptandı. Tüm SBİE patojen dağılımına bakıldığında %87,1 oranında Gram negatif, %12,9 Gram pozitif patojen görüldü. Acinetobacter baumanni en sık saptanan patojendi (%51,9). Bunu sırasıyla Psödomonas aeruginosa (%11,1), Klebsiella pneumoniae (%9,3), Staphylococcus aureus (%5,6), Escherichia coli (%5,6), Stenotrophomonas maltophila (%5,6), Enterococcus faecium (%3,7), Acinetobacter lwoffi (%1,9), Enterococcus faecalis (1,9%), Koagülaz-negatif stafilokok (%1,9) ve Enterobactercloacae (%1,9) izledi.Sonuç: Her ünite kendi hasta profilini, florasını ve bunların direnç paternlerini sürveyans çalışmaları yaparak saptamalı ve tedavi stratejilerini buna göre planlamalıdır.

Objective: Common variable immunodeficiency (CVID) is a relatively frequent primary immunodeficiency disorder characterized by impaired 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-inflammatory medications 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 groups according 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 had osteoporosis 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 that higher 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 those with low folic acid and high ALP levels and lymphopenia.

Objective: Bronchiectasis is a chronic debilitating disease of the airways that results in chronic cough and production of viscous sputum due to dilatation of the airways and bronchial wall thickening. Although bronchiectasis is a common problem in immunodeficiencies and a well-known complication of these disorders, there are a limited number of studies on the frequency of immune abnormalities in adult patients with bronchiectasis. Materials and Methods: The records of adult patients who visited the Pulmonology Outpatient Clinic and whose immune system was assessed due to bronchiectasis at the Immunology Outpatient Clinic until June 2018 were retrospectively evaluated. The data of 84 patients with bronchiectasis (mean age: 48.5 ± 17.9 years, female: 45 [53.6%], male: 39 [46.4%]) were analyzed. Results: Six patients (7.1%) had at least one antibody deficiency. Four patients (4.8%) had low IgG levels, one (1.2%) had low IgA level, and one (1.2%) had low IgM level. Fourteen patients (16.7%) had high IgG (16-22 g/l), 10 (11.9%) high IgA (4.08 - 6.60 g/l) and one (1.2%) high IgM (8.87 g/l). Thirty-five patients (41.7%) had at least one abnormality in the peripheral lymphocyte subset analyses. Conclusion: Adult patients with bronchiectasis may have a variety of immunological abnormalities in addition to hypogammaglobulinemia. Therefore, clinicians should not overlook immunological evaluation in the etiological investigation of bronchiectasis, and patients with immunological abnormalities should be closely monitored.

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.

Background: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency in adults. In addition to renal complications of the disease, there may be an increased likelihood of renal dysfunction due to sucrose in immunoglobulin replacement therapies or other drugs used in treatment. In CVID patients, it is important to monitor patients for renal complications at routine intervals. We compared creatinine-based calculation methods for estimated glomerular filtration rate (eGFR) such as Modification of Diet in Renal Disease (MDRD), Cockcroft-Gault (CG), and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) with 24-hour urine creatinine clearance measurement. We aimed to investigate which calculation method was more reliable and consistent in this patient population. Methods: The records of 14 patients who had clinical follow-up at our hospital were retrospectively reviewed. Patients’ eGFR values were measured by three different methods (CKD-EPI, MDRD , and CG formulas). The 24-hour urinary creatinine clearance of all patients and e-GFR calculated by the formula were compared. Results: The eGFR calculated using the MDRD formula was 122.99±41.22 mL/min/1.73 m2, whereas the eGFR measured using the 24-hour urinary creatinine clearance was 99.64(83.35-156.58) mL/min/1.73 m2. Moreover, eGFR calculated by CKD-EPI formula was 113.83±26.46 mL/min/1.73 m2, while eGFR calculated by CG formula was 133.52±45.35 mL/min/1.73 m2. 24-hour urinary creatinine clearance was positively correlated with MDRD, CKD-EPI and CG formulas (r=0.726, p=0.003, r=0.634, p=0.015, r=0.806, p=0.001, respectively). Conclusions: We found that all creatinine-based formulas used in clinical practice for eGFR measurement correlate with 24-hour urine creatinine clearance in patients with CVID. In addition, we have shown that eGFR calculated with the formula CKD-EPI is more closely related to 24-hour urinary creatinine clearance. Therefore, we believe that the eGFR measurement calculated with CKD-EPI is more useful for nephrological follow-up of patients with CVID. It should be noted that our study has some limitations due to the small number of patients.

The aim of this study is to determine the prevalence of malnutrition in outpatients with common variable immunodeficiency (CVID) and the utility of nutrition screening tools to detect malnutrition in these patients.Fifty outpatients with CVID were included in the study. Nutrition risk for each patient was evaluated using four nutrition screening tools: Malnutrition Screening Tool (MST), Malnutrition Universal Screening Tool (MUST), Short Nutritional Assessment Questionnaire (SNAQ), and Nutritional Risk Screening 2002 (NRS-2002).According to MUST, MST, SNAQ, and NRS-2002, malnutrition risk was determined to be 48% (n = 24), 26% (n = 13), 20% (n = 10), and 20% (n = 10), respectively. Malnutrition was detected in 54% (n = 27) of the patients. It was found that MUST showed a better correlation in detecting malnutrition in outpatients with CVID (κ = 0.482, P = 0.001). MUST has a higher positive and negative predictive value than other nutrition screening tools (79% and 70%, respectively). In the multivariate logistic regression analysis, it was found that low serum immunoglobulin A (IgA) levels at diagnosis increased the risk of malnutrition by ∼15 times, and low CD19The prevalence of malnutrition in patients with CVID was found to be quite high, and there was a strong correlation between malnutrition and low CD19

The prevalence and mortality rates of coronavirus disease 2019 (COVID-19) widely vary among populations. Mucosal immunity is the first barrier to the pathogen's entry into the body. Immunoglobulin A (IgA) is the primary antibody responsible for mucosal immunity. We explored the relationship between selective IgA deficiency (SIgAD) and COVID-19 severity. We included 424 patients (203 women) with COVID-19. Eleven patients had SIgAD. Laboratory data of patients with SIgAD and normal IgA levels were compared. The relationship between SIgAD and severe COVID-19 infection was explored using logistic regression analysis. In the univariate logistic regression analysis, the risk of severe COVID-19 disease in patients with SIgAD was approximately 7.7-fold higher than that in other patients (odds ratio [OR], 7.789; 95% confidence interval [CI], 1.665-36.690, P = 0.008), while it was 4-fold (OR, 4.053; 95% CI, 1.182-13.903, P = 0.026) higher in the multivariate logistic regression analysis. Serum IgA levels were positively correlated with total lymphocyte counts and negatively correlated with C-reactive protein levels, which was a risk factor for severe COVID-19. In patients with SIgAD, the number of severe acute respiratory coronaviruses 2 that pass through mucosal membranes may be increased, leading to complications such as cytokine storm syndrome and acute respiratory distress syndrome.

Background The characteristic features of the immune responses of COVID‐19 patients and how they reflect lung involvement have not been clearly elucidated. Aim The aim of this study was to examine the immune status and the correlations thereof with chest CT scores and lung involvement of patients with COVID‐19. Methods In this retrospective and single‐center study, 72 patients with laboratory‐confirmed COVID‐19 were recruited. The counts of peripheral lymphocyte subsets (CD3+ T cells, CD4+ T cells, CD8+ T cells, CD19+ B cells and CD16+ 56+ NK cells) and those of serum immunoglobulins (IgA, IgG, IgM) were measured and their associations with chest CT scores analysed. Results The proportions of lymphopenia in patients with extensive lung involvement were twice that in the general study population. In the severe disease group, the levels of total lymphocytes, T cells, B cells, NK cells; and serum IgA levels, were significantly lower than in the mild disease group (all P

Angioedema is classified into two major groups: mast cell-mediated (histaminergic) and bradykinin-mediated angioedema. Hereditary angioedema and acquired angioedema are well-defined groups of bradykinin-mediated angioedema. Both hereditary angioedema and acquired angioedema demonstrate a similar clinical presentation and can be challenging to differentiate. The prevalence of autoimmune diseases is higher in patients with HAE and lupus is an autoimmune disease associated with HAE. In this case, we aimed to present a male patient who was monitored with the diagnosis of HAE and developed systemic lupus erythematosus (SLE) in the follow-up and to discuss whether the patient had hereditary angioedema and concomitant lupus, or acquired angioedema secondary to SLE. [Med-Science 2020; 9(2.000): 512-4]

Objective: Common Variable Immunodeficiency (CVID) is a heterogeneous immune disorder characterized by frequent and recurrent upper and lower respiratory tract infections. Although immunoglobulin (Ig) replacement therapy (IgRT) reduces the frequency of infections, complications due to infections of the respiratory tract, particularly bronchiectasis, is still challenging and occur in approximately 20% of patients. Therefore, we aimed to identify independent predictors of bronchiectasis in adult patients with CVID. Materials and Methods: We retrospectively analyzed the hospital records of 45 CVID patients (26 male, 19 female). Additionally, we classified the patients according to the presence of bronchiectasis. Bronciectasis was confirmed by high resolution computed tomography of thorax. Results: Forty-five patients with CVID (F:19 [42.2%], M: 26 [57.8%]) were included in the study (age: 36.52 ± 12.86). Bronchiectasis was observed in 60% of the study group. Univariate regression analysis revealed that IgG levels (OR: 4.889, 95% CI, 0.485-0.959, p: 0.012) and IgA levels (OR: 2.559, 95% CI, 0.097-1.235, p: 0.030) at the time of diagnosis and the percentage of switched memory B cells (OR: 0.848, 95% CI, 0.730-0.984, p: 0.030) were significantly associated with bronchiectasis. Multivariate regression analysis showed that a lower percentage of switched memory B cells (OR: 0.853, 95% CI, 0.733-0.991, p: 0.038) and a low platelet count (OR: 1.000, 95% CI, 1.000-1000, p: 0.026) were also independent predictors of bronchiectasis. Conclusion: Clinicians should be alert for CVID patients with reduced switched memory B cells and low platelet counts in order to detect and treat complications associated with bronchiectasis at an early stage.

Objective: Primary Immune Thrombocytopenia (ITP, idiopathic thrombocytopenic purpura, immune thrombocytopenic purpura) is an acquired thrombocytopenia caused by anti-platelet antibodies. The diagnosis of ITP may be challenging due to the various potential causes of thrombocytopenia, some of which are overlooked. Immunodeficiencies are also a rare cause of ITP. Although autoimmunity and, therefore, ITP are a common complication in primary immunodeficiency (PI) patients, there are not many publications in the literature that examine the frequency of PI in ITP patients and the immune abnormalities in these patients. Materials and Methods: Forty-five patients with ITP (F: 37 [78.7%], M: 10 [21.3%]) were included in the study (age 42.9 ± 15.9). Results: At least one antibody deficiency was detected in 7 patients (14.9%), and following further investigations, 2 patients (4.3%) were diagnosed with CVID, 3 (6.4%) with IgG deficiency, 1 (2.1%) with selective IgA deficiency and 1 (2.1%) with possible IgM deficiency. Immunoglobulin levels were normal while at least one abnormality was detected in 20 patients (42.6%) in peripheral lymphocyte subset analyses. The most common abnormality in this patient group was a reduced percentage of CD4+ T-cells (9 patients, 45% of patients with PLS abnormalities, 19.1% of all patients). CD3+ T-cell rates in 8 patients (17.8%), CD19+ B-cell rates in 6 (12.8%), CD3-CD16+56+ natural killer cell rates in 4 (8.5%), and CD4/CD8 cell ratios in 7 (14.9%) were reduced. In addition, the CD8+ T-cell rate in 8 patients (17%) was above the reference ranges. Conclusion: Adult patients who are diagnosed with ITP may develop a variety of immunological abnormalities in addition to hypogammaglobulinemia. Therefore, clinicians should not overlook immunological evaluation in the etiological investigation of ITP and should closely monitor patients with immunological abnormalities.

Background: Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency in adults. We, therefore, aimed to reveal mortality rates, causes of mortality in CVID patients, as well as demographic and clinical characteristics and differences of survived and dead CVID patientsMethods: The study group included 50 patients [(Female: 23 (46%), Male: 27 (54%)] with CVID, who were followed up on a regular basis for a period of ten years (115.18 ± 80.74 months).Results: Diagnostic delay was 84 (0-360) months, and the mean follow-up time was 115.18 ± 80.74 months. The most common clinical presenting complaints were frequent and recurrent infections and pneumonia. At diagnosis, serum IgG levels were 1.72 (0.33 – 6.90) g/L. The overall survival rate of the patients during the follow-up time was 88%. As a result of univariate Cox regression analysis, platelet count was determined to be an independent risk factor for mortality in CVID patients (Hazard ratio, HR: 0.990, 95% confidence interval, CI: 981-0.999, p: 0.025). When the patients were classified according to mean platelet counts (platelets < 207770/mm3 and platelets > 207770/mm3), the mortality rate in the patient group with platelets < 207770/mm3 was determined to be statistically significantly higher compared to the patient group with platelets > 207770/mm3 (log-rank: 0.013).Conclusions: Clinicians dealing with this patient group should remember that immune dysregulation and low platelet count are independent risk factors for mortality and they should remarkably follow up patients with low platelet count closely.

Amaç: Subkutan alerjen immünoterapisi (SKIT) alerjik rinit / konjonktivit, astım ve venom duyarlılığının tedavisi için hastalık modifiye edici tek terapötik seçenektir. SKIT, deneyimli merkezlerde ve deneyimli hekimlerince uygulandığında oldukça güvenli bir tedavi yöntemi olsa da, enjeksiyonlar sırasında veya sonrasında bazı olumsuz yan etkilere ve lokal veya sistemik reaksiyonlara neden olabilir. Biz bu çalışmada, son 5 yılda, immünoterapi uygulanan yetişkin hastalarımızda gelişen lokal ve sistemik yan etkileri tanımlamayı ve bu yan etkilere neden olan faktörleri ortaya koymayı amaçladık.Gereç ve Yöntemler: 5 yıllık sürede, 119 hastaya (58 kadın, 61 erkek) uygulanan toplam 4413 enjeksiyon analiz edildi.Bulgular: Yaş ortalaması 33.7 ± 12.0 yıl olan toplam 119 hasta çalışmaya dahil edildi (Kadın: 58, % 48; Erkek: 61, % 51.3). 119 hastalık çalışma popülasyonunun, 6'sında (% 5.0) lokal reaksiyonlar, 21'inde (% 17.7) geniş lokal reaksiyon ve 9'unda (% 7.6) sistemik reaksiyon gelişti. Tüm enjeksiyonların % 0.14'ü lokal reaksiyon, % 0.48'i geniş lokal reaksiyon ve % 0.20'si sistemik reaksiyonla ilişkiliydi. Sistemik reaksiyon gelişen dört hastada (% 44.4) epinefrin uygulaması gerekti. Yan etki gelişen veya gelişmeyen hastalar arasında IgE düzeyleri ve eozinofil sayıları açısından anlamlı fark vardı (p = 0.001 ve p = 0.002). Doz artış döneminde ve idame aşamasında gelişen advers reaksiyon oranları arasında anlamlı bir fark vardı (p = 0.025).Sonuç: Klinisyenlerin, SKIT ile ilişkili lokal, geniş lokal ve sistemik reaksiyonlarla ilgili farkındalığı arttırılmalı ve klinisyenler özellikle doz artışı döneminde adverse olaylar açısından daha dikkatli olmalıdır., Aim: Subcutaneous allergen immunotherapy (SCIT) currently represents the only disease-modifying therapeutic option for the treatment of allergic rhinitis/conjunctivitis, asthma, and venom sensitization. Although SCIT represents a fairly safe therapeutic option in the hands of experienced physicians and centers, it may also be associated with certain adverse effects. In this study, we describe the local and systemic adverse effects in our adult patients undergoing immunotherapy over a 5-year period in an effort to define the causative factors.Materials and Methods: A total of 4413 injections administered to 119 patients (58 female, 61 male) were analyzed.Results: A total of 119 patients with a mean age of 33.7 ± 12 years were included (Female: 58, 48%; Male: 61, 51.3%). In the total population of 119 patients, 6 (5%) developed local reactions, 21 (17.7%) developed large local reactions, and 9 (7.6%) had systemic reactions. Of all injections administered throughout the study period, 0.14% were associated with local reactions, 0.48% with large local reactions, and 0.20% with systemic reactions. Four patients with systemic reactions (44.4%) required epinephrine injection. Patients who did or did not develop adverse effects were significantly different with regard to IgE levels and eosinophil counts (p=0.001 and p=0.002). There was a significant difference between the rates of total adverse reactions developing during the build-up or maintenance phase (p=0.025).Conclusion: Clinicians’ awareness regarding the local, large local, and systemic reactions associated with SCIT should be improved, and clinicians should be more careful during the immunotherapy, especially in the build-up phase, for adverse events.

BACKGROUND The adverse effects of COVID-19 pandemic on the mental health of high-risk group patients for morbidity and mortality and its impact on public health in the long term have not been clearly determined. OBJECTIVE To determine the level of COVID-19 related transmission fear and anxiety in healthcare workers and patients with primary immunodeficiency disorder (PID), severe asthma, and the ones with other comorbidities. METHODS The healthcare workers and patients with PID, severe asthma (all patients receiving biological agent treatment), malignancy, cardiovascular disease, hypertension (90% of patients receiving ACEI or ARB therapy), diabetes mellitus (42 % of patients receiving DPP-4 inhibitor therapy) were included in the study. A total of 560 participants, 80 individuals in each group, were provided. The hospital anxiety and depression scale ( HADS ) and Fear of illness and virus evaluation (FIVE ) scales were applied to the groups with face to face interview methods. RESULTS The mean age was 49.30 years and 306 (55 %) were female. The FIVE Scale and HADS-A scale scores of health care workers were significantly higher than other groups' scores (p = 0.001 and 0.006). The second-highest scores belonged to patients with PID. There was no significant difference between the groups for the HADS-D score (p=0.07). The lowest score in all scales was observed in patients with hypertension. CONCLUSIONS This study demonstrated that in the pandemic process, patients with primary immunodeficiency, asthma patients, and other comorbid patients, especially healthcare workers, should be referred to the centers for the detection and treatment of mental health conditions.

BackgroundThe adverse effects of COVID-19 pandemic on the mental health of high-risk group patients for morbidity and mortality and its impact on public health in the long term have not been clearly determined.ObjectiveTo determine the level of COVID-19 related transmission fear and anxiety in healthcare workers and patients with primary immunodeficiency disorder (PID), severe asthma, and the ones with other comorbidities.MethodsThe healthcare workers and patients with PID, severe asthma (all patients receiving biological agent treatment), malignancy, cardiovascular disease, hypertension (90% of patients receiving ACEI or ARB therapy), diabetes mellitus (42 % of patients receiving DPP-4 inhibitor therapy) were included in the study. A total of 560 participants, 80 individuals in each group, were provided. The hospital anxiety and depression scale (HADS) and Fear of illness and virus evaluation (FIVE) scales were applied to the groups with face to face interview methods.ResultsThe mean age was 49.30 ± 13.74 years and 306 (55 %) were female. The FIVE Scale and HADS-A scale scores of health care workers were significantly higher than other groups scores (p = 0.001 and 0.006). The second-highest scores belonged to patients with PID. There was no significant difference between the groups for the HADS-D score (p=0.07). The lowest score in all scales was observed in patients with hypertension.ConclusionsThis study demonstrated that in the pandemic process, patients with primary immunodeficiency, asthma patients, and other comorbid patients, especially healthcare workers, should be referred to the centers for the detection and treatment of mental health conditions.

Common variable immune deficiency (CVID) is a rare primary immunodeficiency disorder that is characterized by defective antibody production and inadequate B cell differentiation. While frequently recurrent respiratory tract infections are the most prominent clinical feature in CVID patients, CVID is a heterogeneous immune deficiency disorder that involves many systems and organs such as lymphoid hyperplasia, autoimmune cytopenia, chronic lung diseases, granulomatous diseases and susceptibility to malignancy. This may lead to delay in diagnosis and immunoglobulin replacement therapy, not being able to receive antibiotics at the appropriate dose and time, chronic inflammation, and therefore secondary amyloidosis. In this case report it is aimed to present a CVID patient with autoimmune complications and developing renal amyloidosis during follow-up., Yaygın değişken immün yetmezlik (YDIY), bozulmuş antikor üretimi ve yetersiz B hücresi farklılaşması ile karakterize nadir görülen bir birincil immün yetmezlik tablosudur. Her ne kadar YDIY hastalarında sık tekrarlayan solunum yolu enfeksiyonları en belirgin klinik durum olsa da; YDIY, lenfoid hiperplazi, otoimmün sitopeni, kronik akciğer hastalıkları, granülomatöz hastalıklar ve maligniteye yatkınlık gibi birçok sistem ve organı etkileyen heterojen bir immün sistem bozukluğudur. Bu durum, tanı ve immünoglobulin replasman tedavisinde gecikmeye, uygun dozda ve zamanda antibiyotik alınamamasına, kronik inflamasyona ve dolayısıyla sekonder amiloidoza yol açabilir. Bu vaka sunumunda, otoimmün komplikasyonla seyreden bir YDIY olgusu ve takip sırasında gelişen renal amiloidoz tablosunun sunulması amaçlanmıştır.

Systemic Mastocytosis (SM) is one of the subtypes of mast cell disorders. Patients with SM suffer from flushing, abdominal cramps and hypotension. It may also cause unexplained and recurrent anaphylactic episodes sometimes. The secretion of mediators can be triggered by various factors. Non-steroidal anti-inflammatory drugs (NSAIDs) are extensively used for their analgesic, antipyretic and anti-inflammatory properties, and they are one of the most commonly prescribed drugs in the world. On the other hand, they may cause a group of adverse reactions, ranging from mild reactions like gastritis to life-threatening allergic reactions like anaphylaxis. In this paper, we report a patient who had multiple severe adverse reactions against NSAIDs and was referred to our clinic to find a safe alternative NSAID. She was eventually diagnosed with Indolent SM (ISM) when she was evaluated with all of her systemic complaints and symptoms.

Hereditary angioedema (HAE) is an autosomal dominant disorder. Considerable rate of mortality determines the importance of disease. In this paper, a patient with HAE who developed severe facial angioedema after the first dose of an antidepressant (Escitalopram) prescribed by a psychiatrist because of diagnosis of depression, was presented and discussed. In the presented case, severe facial angioedema was not found to be clearly associated with a particular cause. There were possibilities such as sporadic/idiopathic attack or depression related attack. However, occurrence of an acute attack soon after a newly started drug had to draw attention to Escitalopram. So, in patients with HAE under an antidepressant treatment, antidepressant related angioedema must be kept in mind when the HAE control deteriorated.

EgeUn###, Primary intestinal lymphangiectasia is a rare disorder which characterized by unpaired small intestinal lymph drainage. There is loss of proteins from dilated lymphatic channels located in the mucosa, submucosa or subserosa which is results with loss of gammaglobulins and lymphocytes, leading to impaired humoral and cellular immunity. Herein, we present a 61-year-old patient with immunodeficiency secondary to Primary intestinal lymphangiectasia (PIL), in whom we could attain effective and stable IgG levels only by subcutaneous IgG replacement rather than intravenous IgG. Our experience suggests that Subcutaneous Immunoglobulin (SCIG) replacement resulted in more stable levels of IgG in the presented patient with PIL. We concluded that SCIG should be the preferred route of immunoglobulin replacement therapy in secondary hypogammaglobulinemia due to protein losing enteropathy, especially in PIL.

Aim: Selective immunoglobulin A (sIgA) deficiency is the most common primary immunodeficiency. Individuals with selective IgA deficiency are generally considered to be asymptomatic. In some patients, autoimmune diseases have been reported. Regulatory T cells (T reg) are a group of cells that play a key role in preventing autoimmunity.Material and Methods: 28 patients with selective IgA deficiency and a control group consisting of 15 individuals of similar age and sex were included in the study.Results: Serum IgA levels were lower in patients with sIgA deficiency (p 0.001). There was no difference between the two groups in term of the rate of Treg cells (p: 0.562 and p: 0.873). There was at least one autoantibody positivity in 19 (67.8%) patients with sIgA deficiency and 4 (26.7%) in the control group. The most common positive autoantibody was ANA in both groups. Serum IgG and IgG1 levels were significantly higher in sIgA deficiency patients with autoantibody than in the other group (p: 0.004 and p: 0.004) The relationship between presence of autoantibodies and Treg cell levels in patients with sIgA deficiency was not statistically significant (p: 0.199). The regression analysis showed that the IgG level (OR: 1.594, 95% CI: 1.096-2.319, p: 0.015) was an independent predictor for the presence of autoantibodies.Conclusion: The findings of our study are important as they are the first evaluation in adult sIgA deficient patients. Further studies are required to shed light on this issue in order to evaluate more patients with sIgA deficiency with autoimmunity.

Hypersecretion of PTHrP is a relatively common cause of malignancy-related hypercalcemia. However, there is only one case report of letrozole induced hypercalcemia. A 52-year-old female patient was referred to our clinic because of the recent discovery of hypercalcemia (11.0 mg/dL). The patient had a history of left breast carcinoma. She had started a course of letrozole (aromatase inhibitor; 2.5 mg dose/day) ten months earlier. Patient’s parathyroid hormone-related protein levels were normal and a bone scintigram revealed no evidence of skeletal metastasis. Other potential causes of high calcium levels were ruled out. We recognized that, when letrozole was taken at one dose daily (2.5 mg), she had recurrent hypercalcemia. Our experience suggests that letrozole may precipitate hypercalcemia in a patient with breast cancer.

Aim: Chronic spontaneous urticaria (CSU) is characterized by recurrent urticaria, angioedema and a combination of both over a period of more than 6 weeks. Anti-immunoglobulin E (IgE) monoclonal antibody (omalizumab) treatment is an effective and safe treatment modality that can be applied in antihistamine-resistant cases in patients with CSU and the urticaria control test is a simple and useful test that evaluates the control level of the disease over the past 4 weeks.Material and Methods: Following appropriate urticaria control tests, 82 test results of 41 patients with CSU (23 females, 18 males) who received subcutaneous anti-IgE treatment at a dose of 300 mg/4 weeks for 6 months were evaluated retrospectively.Results: With each question, there was a statistically significant difference between the mean scores before treatment and at 6 months of treatment (p: 0.001 for question 1, p: 0.001 for question 2, p: 0.001 for question 3, p: 0.001 for question 4). As a result, 95.1% of the patients included in the study achieved a complete and/or partial response to treatment.Conclusion: Omalizumab treatment in patients with chronic spontaneous urticaria (CSU), is an effective and safe treatment modality, independent of the patients’ serum IgE levels, eosinophil counts, thyroid-stimulating hormone levels, C-reactive protein and sedimentation rates, presence or absence of anti-nuclear antibody (ANA) and regardless of whether angioedema is associated with chronic spontaneous urticaria. Furthermore, the urticaria control test is a practical test that can be used to evaluate the efficiency of treatment in CSU patients

We describe herein a case of hypokalemia due to proximal renal tubular acidosis (RTA) and Fanconi's syndrome (FS) and nephrogenic diabetes insipidus with DIC – a rare complication of Sjogren's syndrome (SS) and brucellosis. The interesting feature of this case was the presentation with severe hypokalemia, causing acute flaccid quadriparesis with cardiac arrest which is extremely rare. The patient was a 48-year-old woman who suffered cardiopulmonary arrest an hour after hospitalization. Analysis of a blood sample obtained before her cardiopulmonary arrest yielded surprising results: laboratory investigations showed profound hypokalemia (1.1 mEq/L) with renal K wasting, hyperchloremic metabolic acidosis with normal anion gap, hypophosphatemia with hypouricemia, glucosuria, and proteinuria. A diagnosis of RTA and FS were made. On the seventh day, she looked acutely ill, temperature 38.8 °C and pale, and her physical examination revealed purpuric skin lesions on both legs. The serum antibrucella titration agglutination test was found to be 1 of 160 positive with a nosocomial infection. The clinical and laboratory findings were consistent with disseminated intravascular coagulation (DIC). She was unable to concentrate her urine and so a diagnosis of nephrogenic diabetes insipidus (NDI) was reached. A thorough survey for the cause of FS, RTA and NDI revealed that she had xerophthalmia and xerostomia accompanied by high anti-Ro antibody, positive Schirmer test, confirming the diagnosis of SS.