Relationships between bronchiectasis and time to achieving target trough immunoglobulin G levels in patients with common variable immunodeficiency.

Background: The main treatment of common variable immunodeficiency (CVID) is to maintain immunoglobulin G (IgG) levels within the target range. However, trough IgG levels differ among patients with similar body mass index (BMI) and those receiving the same dose of immunoglobulin replacement therapy (IGRT). A crucial factor that underlies these differences is the presence of extensive bronchiectasis, which is associated with the immunoglobulin salvage pathway. Objective: We compared trough IgG levels in patients with CVID and with and in those without bronchiectasis who had received the same dose of IGRT for 2 years to determine the association of IgG level with infection frequency. Method: This retrospective cohort study included 61 patients with CVID, of whom 21 had bronchiectasis. We reviewed the electronic records for demographic variables, baseline immunoglobulin levels, mean trough IgG levels over 2 years, efficacy levels (trough IgG level - baseline IgG level), the time interval from treatment initiation to achieving the target trough IgG level (700 mg/dL), and the number of infections. Results: The median age of the patients was 39 years (IQR, 27-51), and 29 were women (47.5%). There were no significant differences between the groups in terms of age, age at diagnosis, delay in diagnosis, sex, BMI, IGRT type (subcutaneous or intravenous), and baseline immunoglobulin levels. Trough IgG and efficacy levels were lower (P < 0.001 and P = 0.016, respectively), the time required to achieve the target IgG level was longer in patients with bronchiectasis than in those without bronchiectasis, and this time interval was significantly associated with the infection frequency. Trough IgG and albumin levels were correlated (p = 0.007), with minor differences between the groups (p = 0.04). Conclusion: Bronchiectasis was significantly associated with a longer time to achieve the target IgG levels. These long-term differences between the patients with and those without bronchiectasis have significant clinical implications.

Relationship between autoimmune diseases and serum basal immunoglobulin E levels in patients with common variable immunodeficiency.

Background: Autoimmune diseases can occur at any time in patients with common variable immunodeficiency (CVID). However, the relationship between low immunoglobulin E (IgE) levels and autoimmune diseases in patients with CVID remains poorly understood. Objective: We aimed to determine the relationship between autoimmunity and low IgE in patients with CVID. Methods: This retrospective cohort study was conducted by using data that had been collected from 62 adult patients with CVID between April 2012 and December 2021. Serum basal IgE levels were compared between patients with and patients without autoimmune disease. Results: Overall, 23 of the 62 patients with CVID (37.1%) had at least one autoimmune disease (CVID-O). Autoimmune cytopenias, mainly immune thrombocytopenic purpura, were observed in half of all the patients. Other autoimmune diseases present among the patients included rheumatological diseases, inflammatory bowel diseases, lymphoma, granulomatous lymphocytic interstitial lung disease, autoimmune hepatitis, alopecia, and multiple sclerosis. Serum IgE levels were measured at the time of diagnosis; IgE was undetectable (<2.5 IU/mL) in 82.6% of the patients with CVID-O (n = 19). The median (interquartile range) serum IgE value in the patients with CVID-O was 2 IU/mL (1-16 IU/mL), which was significantly lower than the median serum IgE value in patients with CVID and without autoimmune disease (p < 0.001). Low IgE levels in patients with CVID-O were an independent risk factor for the development of autoimmune disease in patients with CVID (odds ratio 3.081 [95% confidence interval, 1.222-7.771]; p = 0.017). Conclusion: Low serum IgE levels were associated with the development of autoimmune disease in patients with CVID. The monitoring of serum IgE levels in patients with CVID may be useful in the early diagnosis and treatment of autoimmune diseases.

High digit ratio (2D:4D) is associated with attack frequency and severity in hereditary angioedema patients.

BACKGROUND: Hereditary angioedema (HAE) caused by a deficiency or dysfunction of the plasma protease C1-inhibitor is a rare autosomal-dominant disorder. We explored a possible correlation between the ratio of the second and fourth finger lengths (2D:4D) and the frequency of HEA attacks, and whether the ratio might predict laryngeal attack. METHOD: We evaluated 35 HEA patients aged 19 to 66 years; 3 were subsequently excluded. The 2D:4D ratio was calculated by dividing the length of the second finger by that of the fourth finger of both hands. A structured clinical questionnaire exploring HAE course and treatment over the prior year was administered. RESULTS: Of the 32 participants, 56.25 % (n = 18) were female. Of them, those with high 2D:4D ratios suffered significantly more laryngeal attacks than others; 93.3% of patients with high 2D:4D ratios experienced ≥5 attacks annually, significantly more than those with low ratios. Among type 2 HEA patients, 75 % of those experiencing ≥5 attacks annually had high 2D:4D ratios; all patients with low 2D:4D ratios reported <5 attacks annually. These significant effects were found for right-hand 2D:4D ratios and not left-hand 2D:4D ratios. CONCLUSION: The data suggest that intrauterine sex hormone exposure, which affects the 2D:4D ratio, is significantly associated with HEA attack frequency and severity, and laryngeal edema.

Current Statement of Intensive Care Units in Turkey: Data obtained from 67 Centers.

OBJECTIVES: We aimed to obtain information about the characteristics of the ICUs in our country via a point prevalence study. MATERIAL AND METHODS: This cross-sectional study was planned by the Respiratory Failure and Intensive Care Assembly of Turkish Thoracic Society. A questionnaire was prepared and invitations were sent from the association's communication channels to reach the whole country. Data were collected through all participating intensivists between the October 26, 2016 at 08:00 and October 27, 2016 at 08:00. RESULTS: Data were collected from the 67 centers. Overall, 76.1% of the ICUs were managed with a closed system. In total, 35.8% (n=24) of ICUs were levels of care (LOC) 2 and 64.2% (n=43) were LOC 3. The median total numbers of ICU beds, LOC 2, and LOC 3 beds were 12 (8-23), 14 (10-25), and 12 (8-20), respectively. The median number of ventilators was 12 (7-21) and that of ventilators with non-invasive ventilation mode was 11 (6-20). The median numbers of patients per physician during day and night were 3.9 (2.3-8) and 13 (9-23), respectively. The median number of patients per nurse was 2.5 (2-3.1); 88.1% of the nurses were certified by national certification corporation. CONCLUSION: In terms of the number of staff, there is a need for specialist physicians, especially during the night and nurses in our country. It was thought that the number of ICU-certified nurses was comparatively sufficient, yet the target was supposed to be 100% for this rate.

Patient and physician delay in the diagnosis and treatment of non-small cell lung cancer in Turkey.

AIM: The early diagnosis and treatment of lung cancer are important for the prognosis of patients with lung cancer. This study was undertaken to investigate patient and doctor delays in the diagnosis and treatment of NSCLC and the factors affecting these delays. MATERIALS AND METHODS: A total of 1016 patients, including 926 (91.1%) males and 90 (8.9%) females with a mean age of 61.5±10.1 years, were enrolled prospectively in this study between May 2010 and May 2011 from 17 sites in various Turkish provinces. RESULTS: The patient delay was found to be 49.9±96.9 days, doctor delay was found to be 87.7±99.6 days, and total delay was found to be 131.3±135.2 days. The referral delay was found to be 61.6±127.2 days, diagnostic delay was found to be 20.4±44.5 days, and treatment delay was found to be 24.4±54.9 days. When the major factors responsible for these delays were examined, patient delay was found to be more frequent in workers, while referral delay was found to be more frequent in patients living in villages (p<0.05). We determined that referral delay, doctor delay, and total delay increased as the number of doctors who were consulted by patients increased (p<0.05). Additionally, we determined that diagnostic and treatment delays were more frequent at the early tumour stages in NSCLC patients (p<0.05). DISCUSSION: The extended length of patient delay underscores the necessity of educating people about lung cancer. To decrease doctor delay, education is a crucial first step. Additionally, to further reduce the diagnostic and treatment delays of chest specialists, multidisciplinary management and algorithms must be used regularly.