Effect of Modulator Therapies on Nutritional Risk Index in Adults with Cystic Fibrosis: A Prospective Cohort Study.

Background: Modulator therapies improve weight and body mass index (BMI) in cystic fibrosis (CF) patients. We aimed to compare the nutritional risk index (NRI) in adult CF patients receiving modulator (MT) or only non-modulator (conventional) therapies (non-MT). Methods: A single-center prospective cohort study was conducted between June and December 2023. The NRI based on weight gain and albumin was calculated at beginning and end of a 12-week period in both groups. This design was pragmatic, since it was based on individual patient access to MT for 12 weeks. Results: In total, 107 patients were included [mean (SD) age: 23.85 (4.98) years, 54.7% male, 46.7% MT]. In the MT group, mean (SD) weight (kg) and albumin (g/dL) increased significantly [changes: +3.09 (2.74) and +0.17 (0.37); p < 0.001]. In the non-MT group, weight and albumin decreased significantly [changes: -0.99 (1.73) and -0.12 (0.30); p < 0.001]. Compared to the MT group, baseline mean (SD) NRI in the non-MT group was significantly higher [100.65 (11.80) vs. 104.10 (10.10); p = 0.044]. At the end of the 12 weeks, mean (SD) NRI in the MT group was higher than in the non-MT group [104.18 (10.40) vs. 102.58 (12.39); p = 0.145]. In the MT group, the NRI category improved in 22 (44%), and worsened in 3 (6%) patients (p < 0.001). In the non-MT group, the NRI category improved in 2 (3.5%), and worsened in 10 (17.5%) patients (p < 0.001). Conclusions: This is the first study reporting on a positive effect of MT on NRIs, based on weight gain and albumin. Personalized nutrition and routine follow-up of adults with CF based on NRI is recommended prior to MT initiation.

Successful desensitization in a patient with recurrent anaplastic oligodendroglioma presenting with procarbazine-mediated anaphylaxis.

INTRODUCTION: Procarbazine is an oral chemotherapeutic agent used in the treatment of brain malignancies and is associated with hypersensitivity reactions. In case of grade 4 reactions, rechallenge should be avoided, and the agent should be replaced, unless the treatment is curative, in which case the application of a desensitization protocol should be considered. We present a successful case of desensitization in procarbazine anaphylaxis. CASE REPORT: A 53-year-old male patient was diagnosed with recurrent anaplastic oligodendroglioblastoma. The patient received three cycles of procarbazine, lomustine, and vincristine chemotherapy for malignancy recurrence. In the fourth cycle, on the 12th day of procarbazine treatment, the patient developed anaphylaxis. Procarbazine was given together with premedication as part of the 12-step desensitization process, and the fourth cycle was successfully completed. MANAGEMENT AND OUTCOME: Procarbazine hypersensitivity reactions are observed less frequently than reactions to other chemotherapeutics. We presented a case of procarbazine-associated severe anaphylaxis that was able to continue procarbazine chemotherapy with successful desensitization. This case is important in terms of confirming the procarbazine desensitization protocol. DISCUSSION: In literature there is only one protocol developed was successfully applied in one patient with procarbazine anaphylaxis. In the current case, we took this protocol into consideration in the management of our patient. Following the use of this protocol, the patient was able to continue procarbazine chemotherapy successfully. Procarbazine anaphylaxis is rare, and more cases are needed to be reported to confirm the desensitization protocol and when to continue procarbazine treatment.

Comparison of 300 mg versus 600 mg daily maintenance doses of aspirin treatment after desensitization in N-ERD: A three-year multicentre experience.

Background: Aspirin treatment after desensitization (ATAD) is effective in preventing nasal polyps recurrence as well as respiratory symptoms in patients with nonsteroidal anti-inflammatory drug (NSAID)-exacerbated respiratory diseases (N-ERD). However, there is no consensus on effective daily maintenance doses in ATAD. Therefore, we aimed to compare the effects of two different maintenance doses of aspirin on clinical outcomes for 1-3 years of ATAD. Methods: This was a retrospective, multicenter study that involved four tertiary centers. The maintenance doses of daily aspirin were 300 mg in one center and 600 mg in the remaining three. The data of patients who were on ATAD for 1-3 years were included. Study outcomes (nasal surgeries, sinusitis, asthma attacks, hospitalization, oral corticosteroid use, and medication uses) were assessed in a standardized way and recorded from case files. Results: The study initially included 125 subjects, 38 and 87 were receiving 300 and 600 mg daily aspirin for ATAD, respectively. Number of nasal polyp surgeries decreased after 1 -3 years compared with before ATAD in both groups (group 1, baseline: 0.44 ± 0.07 versus first year: 0.08 ± 0.05; p < 0.001 and baseline: 0.44 ± 0.07 versus 3rd year: 0.01 ± 0.01; p < 0.001; and group 2, baseline 0.42 ± 0.03 versus first year: 0.02 ± 0.02; p < 0.001 and baseline: 0.42 ± 0.03 versus 3rd year: 0.07 ± 0.03; p < 0.001). Conclusion: Given the comparable effects of 300 mg and 600 mg aspirin daily as maintenance treatment of ATAD on both asthma and sinonasal outcomes in N-ERD, our results suggest using 300 mg of aspirin daily in ATAD owing to its better safety profile.

Comparison of the clinical outcomes of patients with NSAID-exacerbated respiratory disease receiving aspirin or biologicals.

OBJECTIVE: NSAID-exacerbated respiratory disease (NERD) is characterized by exacerbation of respiratory symptoms after NSAID intake. While research for specific treatment options continues in patients who cannot tolerate or are unresponsive to aspirin treatment after aspirin desensitization (ATAD), biologicals have emerged as a new therapeutic option in NERD patients. The aim of this study was to compare the quality of life, and the sinonasal and respiratory outcomes of NERD patients treated with ATAD or biologicals. METHODS: Patients who have been followed up at a tertiary care allergy center and who have been receiving at least one of ATAD, mepolizumab or omalizumab for at least six months were included. Evaluations were made using sinonasal outcome test (SNOT-22), asthma control test (ACT), short form-36 (SF-36), blood eosinophil counts, need for recurrent functional endoscopic sinus surgeries (FESS), and asthma or rhinitis exacerbations requiring oral corticosteroids (OCS). RESULTS: A total of 59 patients comprised of 35 (59%) females and 24 (41%) males with a mean age of 46.1 (min-max, 20-70) years were included. The baseline blood eosinophil count was higher, and a significant decrease in blood eosinophil counts was observed in the mepolizumab group compared to ATAD group (p = 0.001, p < 0.001, respectively). At follow-up, the rate of recurrent FESS was lower in the group that received mepolizumab (p = 0.02). CONCLUSIONS: In NERD patients, mepolizumab significantly decreased blood eosinophil counts and recurrent FESS. There was no significant difference between the patients receiving ATAD or mepolizumab regarding other clinical parameters.

Community-acquired pneumonia - An EFIM guideline critical appraisal adaptation for internists.

BACKGROUND: In real-life settings, guidelines frequently cannot be followed since many patients are multimorbid and/or elderly or have other complicating conditions which carry an increased risk of drug-drug interactions. This document aimed to adapt recommendations from existing clinical practice guidelines (CPGs) to assist physicians' decision-making processes concerning specific and complex scenarios related to acute CAP. METHODS: The process for the adaptation procedure started with the identification of unsolved clinical questions (PICOs) in patients with CAP and continued with critically appraising the updated existing CPGs and choosing the recommendations, which are most applicable to these specific scenarios. RESULTS: Seventeen CPGs were appraised to address five PICOs. Twenty-seven recommendations were endorsed based on 7 high, 9 moderate, 10 low, and 1 very low-quality evidence. The most valid recommendations applicable to the clinical practice were the following ones: Respiratory virus testing is strongly recommended during periods of increased respiratory virus activity. Assessing the severity with a validated prediction rule to discriminate where to treat the patient is strongly recommended along with reassessing the patient periodically for improvement as expected. In adults with multiple comorbidities, polypharmacy, or advanced age, it is strongly recommended to check for possible drug interactions before starting treatment. Strong graded recommendations exist on antibiotic treatment and its duration. Recommendations on the use of biomarkers such as C-reactive protein or procalcitonin to improve severity assessment are reported. CONCLUSION: This document provides a simple and reliable updated guide for clinical decision-making in the management of complex patients with multimorbidity and CAP in the real-life setting.

Clinical implications of fungal isolation from sputum in adult patients with cystic fibrosis

Background/aim: Cystic fibrosis is an autosomal recessive disease with a defect in mucociliary activity that is characterized by recurrent pulmonary infections. Bacterial agents frequently implicated in airway colonization are Haemophilus influenzae, Staphylococcus spp., and Pseudomonas spp. Fungal isolation from sputum is common in adults. However, growth of fungal agent only in sputum culture in patients with cystic fibrosis is insufficient for the diagnosis of fungal diseases. There is limited data about the clinical significance of fungal isolation in sputum cultures. The aim of the study was to investigate the clinical outcomes andsignificance of fungal isolation from sputum samples in adult CF. Materials and methods: This retrospective study included patients who have been admitted between October 2017 and January 2019 in an adult cystic fibrosis unit. Patients were grouped according to fungal pathogenicity as; fungal disease group, colonization group, and nonisolated group. The data of the last one year, including demographics, clinical data, laboratory, treatment modalities, results of cultured bacteria and fungus from sputum samples, respiratory function parameters, frequency of exacerbation, and hospitalizationwere compared between groups. Results: A total of 330 sputum samples from 88 adult patients with CF were collected. Patients were divided into 3 groups, the fungal disease group (n = 10, 11.4%), colonization group (n = 49, 55.7%), and nonisolated group (n = 29, 32.9%). Presence of pulmonary exacerbation, number of admissions to emergency department, and the number of positive cultures for bacteria from sputum were higher in the fungal disease group (p = 0.03, p = 0.01 and p < 0.001). The fungal disease group had higher rate of antibiotics by parenteral routethan other groups (p = 0.001) whereas lung functions were similar. Use of nutritional supplementation and parenteral antibiotherapy were the factors associated with elevated risk of fungal isolation. Conclusion: Frequent use of parenteral antibiotics and use of nutritional supplementation were found to be independent risk factors for fungal isolation from sputum in adult CF.

Regular Treatment With Aspirin 300 mg/day After Desensitization in Patients With N-ERD: 12-Year Results.

OBJECTIVE: Aspirin desensitization is recommended for patients with nonsteroidal anti-inflammatory drug (NSAID) hypersensitivity, in whom asthma is uncontrolled despite medical treatment, and/or frequent endoscopic sinus surgery (ESS) is required due to nasal polyps. There are few studies in the literature on long-term follow-up of patients undergoing regular aspirin treatment after desensitization. This study aims to evaluate the effect of regular aspirin treatment on respiratory function, symptom control, quality of life, and the number of nasal surgeries required during a period of 12 years. MATERIAL AND METHODS: A total of 18 patients were included in the study in 2006; 11 patients were excluded and 7 patients regularly taking aspirin for 12 years were evaluated. Oral aspirin desensitization was performed at 4-6 weeks following the ESS. Patients receiving 300 mg/day aspirin were followed up in control visits every 3 months. Nasal and respiratory system examinations and pulmonary function test were performed, and all patients responded to the SF-36 Quality of Life scale during each visit. RESULTS: There was no change in respiratory function parameters following the12-year aspirin treatment. There was no statistically significant improvement in the quality of life; however, the need for ESS due to the recurrence of nasal polyps decreased significantly (P = .000). At the 12-year follow-up, all symptom scores improved, but improvement in the postnasal drip score was statistically significant (P = .046). CONCLUSION: Long-term regular treatment with aspirin at a dose of 300 mg/day in patients with N-ERD improved symptom scores, and alleviated the need for ESS due to nasal polyp recurrence.

Cuban Blue Scorpion Venom and Lung Carcinoma: Is it Always True What Does Not Kill Me Makes Me Stronger?

Historically snake, scorpion, and hymenoptera venom have been used to treat various diseases. It is well known that alternative and/or complementary treatment options are widely used, especially among cancer patients. The case we describe here has lung carcinoma and received blue scorpion venom as an alternative therapy. A 86 years old male patient was admitted complaining of dyspnea. Thorax computed tomography (CT) showed a mass lesion and intraparenchymal nodules on the left upper lobe. Percutaneous transthoracic biopsy was performed, and the diagnosis was reported to be an epidermoid carcinoma. After the shared decision, A shared decision with the family and oncology specialists, the patient decided to try Cuban blue scorpion venom oral drops. Initially, it was well tolerated. However on the sixth day of treatment, sudden death occurred. Scorpion venom has been considered as a natural source for cancer therapy. Rhopalurus junceus is an endemic scorpion species from Cuba. Although it belongs to the most dangerous species related to human scorpionism, and no fatal stings have been reported. Currently published articles investigate the composition and in vitro anticancer activity of the venom; there are no controlled clinical trials evaluating toxicity or effectiveness. Uncontrolled use of alternative treatment options may lead to unexpected interactions with concomitant medications.

Screening instrument to identify skin prick test-negative patients with asthma and/or rhinitis.

INTRODUCTION: To the best of our knowledge, there is no prediction tool for determining respiratory allergy in patients with asthma and/or rhinitis. OBJECTIVES: As such, this study aimed to develop an instrument for identifying skin prick test (SPT)-negative patients with asthma and/or rhinitis. METHODS: This cross-sectional study screened 510 consecutive patients diagnosed with asthma and/or rhinitis between November 2009 and May 2011 in Ankara, Turkey. A structured questionnaire was the screening tool that was administered during the initial visit by the treating specialist and compared with a SPT result. All 15 variables were analysed using the logistic regression model to obtain ß values (a score) for each variable. Each variable had a score, and scores were summed up to obtain final score for each patient. RESULTS: The study included 363 patients, of which 165 (45.5%) were SPT positive. The final model included 13 variables. The lowest and highest scores were -21 and 26. A total score of 5.5 had sensitivity of 63% and specificity of 80%. The area under the receiver operating characteristic curve was 0.78. The histogram of the total scores for the study population was bimodal. CONCLUSIONS: We designed a highly specific 13-item instrument for identifying SPT-negative patients. It may be used in middle income countries where allergy testing is expensive, not reimbursed or few allergy clinics are available. We think that our idea is interesting, that needs further research.