J Allergy Clin Immunol Glob. 2025 Jun 24;4(3):100525. doi: 10.1016/j.jacig.2025.100525. eCollection 2025 Aug.

ABSTRACT

Hyper-IgE syndrome (HIES) is a rare and sporadic primary immunodeficiency usually characterized by atopic dermatitis, recurrent skin staphylococcal infections, recurrent pulmonary infections, and elevated IgE levels. Due to the rarity of the syndrome and its nonspecific and wide presentation, the diagnosis is difficult and arises with other diagnoses including other types of primitive chronic granulomatous disease or acquired immunodeficiency, severe atopic dermatitis, or cystic fibrosis or chronic respiratory infection as tuberculosis. To date, there is no gold standard management and treatments aim to relieve symptoms and avoid complications. We report on a 30-year-old female with a long-time misdiagnosed HIES.

PMID:40697947 | PMC:PMC12281938 | DOI:10.1016/j.jacig.2025.100525

Vavilovskii Zhurnal Genet Selektsii. 2025 Jul;29(4):594-599. doi: 10.18699/vjgb-25-62.

ABSTRACT

Pseudomonas aeruginosa is one of the leading causes of nosocomial respiratory tract infections and plays an important role in lower respiratory tract infection in patients with cystic fibrosis (CF). Biofilms, which are organized cell clusters, ensure the survival of microorganisms in unfavorable environmental conditions and contribute to the chronicity of infection and the formation of persistent forms. The aim of this study was to determine the phenotypic ability and genetic potential for biofilm formation in clinical strains of P. aeruginosa persisting in patients with CF against the background of constant intake of antimicrobial drugs. Bacteriological, genetic, and bioinformatic methods were used to characterize five P. aeruginosa strains obtained from patients with CF. Phenotypically, all strains were classified as moderately biofilm-forming, while the biofilm formation coefficient varied from 2.10 to 3.15. Analysis of draft genomes revealed differences in the representation of some genes or individual loci of three of the four known signaling pathways (cAMP/Vfr, Gac/Rsm, and c-di-GMP) that have been described in P. aeruginosa genomes and are related to the regulation of biofilm formation. In addition, differences in the representation of genes such as frzE, tcpE, and rcsC are shown. Of undoubted interest is the analysis of genes such as pppA, icmF, clpV1, trpE, trpG, and stp1, which are used for extended multilocus typing PubMLST and differed in the structure of loci in all analyzed strains. These genes can be used to identify clinical strains of P. aeruginosa and to characterize their biofilm-forming properties. Thus, genes potentially participating in both biofilm formation and regulation have been characterized in the genomes of clinical P. aeruginosa strains that persist for a long time in patients receiving continuous antibiotic therapy. Characterization of the genetic potential for biofilm formation makes it possible to search for reliable genetic markers of this process in order to monitor the evolution of the pathogen as a result of long-term persistence in the host organism.

PMID:40697940 | PMC:PMC12277583 | DOI:10.18699/vjgb-25-62

Front Cell Infect Microbiol. 2025 Jul 8;15:1628481. doi: 10.3389/fcimb.2025.1628481. eCollection 2025.

ABSTRACT

The lungs are constantly exposed to airborne pathogens and depend on robust innate immune surveillance for protection. The cyclic GMP-AMP synthase (cGAS)-stimulator of interferon genes (STING) signaling pathway, a core component of the innate immune system, plays a pivotal role in defending against respiratory infections caused by viruses, bacteria, and mycobacteria, including Mycobacterium tuberculosis. Dysregulation of this pathway has been linked to several chronic lung diseases, such as cystic fibrosis, chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis, and asthma. Upon sensing cytoplasmic DNA, cGAS activates the STING pathway, producing type I interferons and pro-inflammatory cytokines that drive host immune response. However, many pathogens have developed strategies to evade detection or surpass cGAS-STING signaling. This systematic review highlights the molecular mechanisms governing cGAS-STING activation, its interaction with lung pathogens, and its potential as a therapeutic agent in respiratory diseases.

PMID:40697819 | PMC:PMC12279746 | DOI:10.3389/fcimb.2025.1628481

Ann Gastroenterol. 2025 Jul-Aug;38(4):446-452. doi: 10.20524/aog.2025.0983. Epub 2025 Jun 30.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a common life-limiting genetic disease often associated with hepatobiliary complications. Endoscopic retrograde cholangiopancreatography (ERCP), though valuable, carries procedural risks. We assessed the safety of ERCP in CF patients using real-world data.

METHODS: A retrospective cohort study using the TriNetX database (2010-2024) identified adults (≥18 years) with CF who underwent ERCP. Propensity-score matching adjusted for confounders, including age, sex, race, and hospitalization history. The primary outcome was post-ERCP pancreatitis (PEP); secondary outcomes included bleeding and infection. Subgroup analysis evaluated outcomes in patients with choledocholithiasis.

RESULTS: Among 534 matched CF patients (mean age 44.6 years; 48.3% female), rates of PEP (8.3% vs. 4.9%, adjusted odds ratio [aOR] 1.76, 95% confidence interval [CI] 0.937-3.315; P=0.075), bleeding (3.1% vs. 2.1%, aOR 1.52, 95%CI 0.674-3.409; P=0.31), and infection (3.7% vs. 2.4%, aOR 1.55, 95%CI 0.638-3.785; P=0.33) were not significantly different compared to non-CF controls. Subgroup analysis of choledocholithiasis patients similarly showed no significant differences.

CONCLUSIONS: ERCP in CF patients demonstrated comparable adverse event rates to non-CF controls. These findings support the procedural safety of ERCP in this population, though further prospective studies are needed to validate these results and clarify risk by indication.

PMID:40697435 | PMC:PMC12277522 | DOI:10.20524/aog.2025.0983

Biofilm. 2025 Jul 2;10:100301. doi: 10.1016/j.bioflm.2025.100301. eCollection 2025 Dec.

ABSTRACT

Pseudomonas aeruginosa biofilms cause severe infections in the airways of patients suffering from cystic fibrosis (CF) that are difficult to eradicate, even with intensive antibiotic therapy. The main goal of this study was to define the dual transcriptional response associated with the formation of P. aeruginosa biofilms in a polarized lung epithelium monolayer. We analyzed the dual response of healthy and CF epithelium after infection with P. aeruginosa isolates from acute and chronic infections. Our results show that strains of P. aeruginosa isolated from chronic infections specifically increase the expression of secretion systems of type I, III and VI to hijack the host response. Conversely, strains associated with acute illness use ABC transporters to counteract the antimicrobial response. In return, a distinctive expression pattern in the CF epithelium, including a high degree of cytokine secretion and keratinization, is largely observed in acute infections. Our results show that both host and pathogen genomic backgrounds contribute to the outcome of infection and specific transcriptional signatures could be used in the diagnosis, particularly in CF patients.

PMID:40697188 | PMC:PMC12281032 | DOI:10.1016/j.bioflm.2025.100301

Medicine (Baltimore). 2025 Jul 18;104(29):e43414. doi: 10.1097/MD.0000000000043414.

ABSTRACT

BACKGROUND: This study evaluates the efficacy and side effects of different doses of erythromycin in patients with stable non-cystic fibrosis bronchiectasis (NCFB).

METHODS: Altogether, 148 patients diagnosed with stable NCFB were enrolled in this study and randomly assigned to one of the following 4 treatment groups: Group A received erythromycin 250 mg/d; Group B received erythromycin 375 mg/d; Group C received erythromycin 500 mg/d; and Group D received a placebo. Each group consisted of 35 patients. The dataset comprised lung function data and computed tomography, St. George respiratory questionnaire, modified Medical Research Council, bronchiectasis severity index (BSI), and exacerbations, FEV1, age, chronic colonization, extension, dyspnea scores, which were collected before and at 6 months after treatment. These were subjected to a univariate analysis.

RESULTS: Groups C and D exhibited significant differences in forced expiratory volume in 1 second, forced vital capacity, quality of life, and computed tomography Bhalla, BSI, and E-FACED scores (P < .05). Additionally, Groups B and D demonstrated significant differences in forced vital capacity and St. George respiratory questionnaire, BSI, and E-FACED scores (P < .05). Contrarily, Group A exhibited nonsignificant differences.

CONCLUSION: The oral erythromycin regimen at a dose of 500 mg/d can improve the lung function and quality of life of stable NCFB patients.

PMID:40696686 | DOI:10.1097/MD.0000000000043414

Rhinology. 2025 Jul 22. doi: 10.4193/Rhin25.904. Online ahead of print.

ABSTRACT

I would like to welcome you to the August issue of Rhinology. In the latest issue of Rhinology, you will find high quality articles spanning the entire breadth of the field of rhinology. From studies on inflammatory sinus disease to skull base pathology, from medical to surgical treatments, every reader is sure to find studies of interest and applicability to their practice. For the focus of this editorial, I highlight for the reader the article by Le Bon et al., which describes the case of a patient with cystic fibrosis (CF) and chronic rhinosinusitis (CRS) with nasal polyps in whom concomitant NSAID-exacerbated respiratory disease (NERD) was uncovered after the initiation of triple combination CFTR modulatory therapy with elexacaftor-tezacaftor-ivacaftor (ETI).

PMID:40694656 | DOI:10.4193/Rhin25.904

Microbiol Resour Announc. 2025 Jul 22:e0030625. doi: 10.1128/mra.00306-25. Online ahead of print.

ABSTRACT

Burkholderia cepacia complex causes life-threatening respiratory infections. Here, a bacteriophage with activity against B. cenocepacia was isolated from wastewater. It has a genome size of 70,144 bp and has the taxonomic classification Irusalimvirus. It has no genes associated with lysogeny, bacterial resistance, or virulence.

PMID:40693765 | DOI:10.1128/mra.00306-25

Pediatr Pulmonol. 2025 Jul;60(7):e71187. doi: 10.1002/ppul.71187.

ABSTRACT

BACKGROUND: Despite eligibility of over 90% of people with cystic fibrosis (PwCF) for CFTR modulators, 12% of eligible PwCF are not prescribed these therapies. The CF Foundation CF Learning Network (CFLN) convened a quality improvement (QI) innovation lab (iLab) to investigate gaps in modulator use and identify best practices for modulator initiation, management, and side effect screening.

METHODS: Thirty-one CF centers used the Model for Improvement to adapt interventions to local context by Plan-Do-Study-Act cycles and approach aims in two phases: (1) increase documented elexacaftor-tezacaftor-ivacaftor or ivacaftor (ETI/I) use or co-produced deferral by 4% by 5/2023 and (2) increase side effect screening rates for PwCF taking ETI to 80% by 8/2024. Interventions included processes to track eligibility and facilitate safety assessments and tools like screener forms. Centers submitted weekly measures. We used control charts and run charts to analyze progress.

RESULTS: Of 4649 PwCF evaluated from 12/2022 to 5/2023, 98.5% on average had documentation of either ETI/I use (89.3%) or deferral (9.2%). The most common reason for deferral was previous side effects (39.0%). Across 3236 visits from 3/2024 to 8/2024, ETI side effect screening rates improved, with the centerline shifting from 67.1% to 80.8%. Potential side effects were identified in 24.5% of screenings, most commonly mood changes, inattention/brain fog, and weight gain.

CONCLUSIONS: Application of QI methods resulted in processes and tools for enhancing modulator management and improving side effect screening across multiple CFLN centers. Potential side effects, including those not listed in the prescribing information, were commonly reported, highlighting the importance of side effect screening and management by care teams. Interventions can be reproduced by other centers and applied to novel CF therapies.

PMID:40693596 | DOI:10.1002/ppul.71187

Chin Med J (Engl). 2025 Jul 21. doi: 10.1097/CM9.0000000000003712. Online ahead of print.

ABSTRACT

Recent studies have challenged the once prevalent notion that the human lungs are sterile, instead unveiling a dynamic microbial environment that interacts intricately with both the host and external factors. This review describes the distinct microbial compositions between healthy individuals and those with respiratory diseases, as well as discussing the variations in microbial composition across different disease states. We explore the crucial role of the lung microbiome in maintaining respiratory health and describe its implications in various respiratory diseases. We discuss how these microbial differences correlate with the severity and progression of respiratory diseases, including chronic obstructive pulmonary disease, cystic fibrosis, lung cancer, asthma, coronavirus disease 2019, and tuberculosis. Furthermore, we analyze the pathogenic mechanisms of the lung microbiome, as well as the associations between changes in the lung microbiome and systemic effects, including the emerging concepts of the gut-lung axis and brain-lung axis, which highlight the interconnected influence of the microbiota on lung health. This review aims to provide a comprehensive understanding of the profound impact of microbial dynamics on respiratory health and disease, suggesting new avenues for targeted diagnostic and therapeutic strategies.

PMID:40693591 | DOI:10.1097/CM9.0000000000003712

Pediatr Pulmonol. 2025 Jul;60(7):e71214. doi: 10.1002/ppul.71214.

ABSTRACT

INTRODUCTION: Disorders of gut-brain interaction (DGBI), such as irritable bowel syndrome, occur at a higher prevalence in adults with cystic fibrosis (CF), compared to the general population. DGBI are associated with impaired quality of life and significant health-system costs. This study aimed to assess the proportion of cwCF with DGBI, compared to non-CF controls.

METHODS: Validated for the assessment of DGBI, ROME IV surveys were distributed to cwCF and non-CF controls aged 0-18 years as part of the Evaluating the Alimentary Tracts in Health and Disease (EARTH) observational study. CF participants were recruited from the Sydney Children's Hospital (SCH) CF outpatient clinic between 2018 and 2022. Non-CF controls were recruited from outpatient clinics, advertisements, and word-of-mouth.

RESULTS: Forty-four cwCF (female = 22 [50%], median [IQR] age = 7.04 [2.25-11.06] years) and 48 non-CF controls (female = 22 [45.83%], median (IQR) age = 8.04 [3.57-12.77] years) completed baseline surveys. Symptoms consistent with at least one DGBI were observed more frequently in cwCF compared to non-CF controls (36.36% vs. 10.42%%, p = 0.01). Functional abdominal pain was experienced at a higher prevalence in cwCF compared to non-CF controls (13.79% vs. 0%, p = 0.02). No significant differences in the prevalence of other specific disorders were observed between cwCF and non-CF controls.

CONCLUSION: Compared to non-CF, cwCF were significantly more likely to experience functional abdominal pain, and at least one DGBI collectively. Further large-scale studies are needed to validate our findings and ascertain the role of routine screening of DGBI in pediatric CF cohorts.

PMID:40693578 | DOI:10.1002/ppul.71214

Pediatr Pulmonol. 2025 Jul;60(7):e71194. doi: 10.1002/ppul.71194.

ABSTRACT

BACKGROUND: There is a paucity of information on the microbiological profile in Indian children with CF. This study aimed to evaluate the microbial profile of airways in Indian children with CF and to generate antibiotic sensitivity patterns.

METHODS: Children ≤ 18 years old with newly diagnosed CF (Clinical and two positive sweat chloride tests) were included. The respiratory samples were collected at enrollment, follow-up (FU), and during pulmonary exacerbation.

RESULTS: Three hundred and thirteen children were enrolled with a median (IQR) age of 2.8 (0.6, 8) years; 197 (62.9%) were boys. 530 microbiological samples were collected during the study period. At enrollment, Pseudomonas aeruginosa was the most common organism detected in 63 (36.8%) children, followed by Staphylococcus aureus (32, 18.7%), Escherichia coli (23, 13.4%), Klebsiella pneumoniae (12, 7.0%), Acinetobacter baumanii (8, 4.7%), and Haemophilus influenzae (3, 1.7%). During FU, P. aeruginosa was also the most common organism detected in 56 (26.8%) samples, followed by S. aureus (43, 20.5%). The median age of P. aeruginosa infection was 1.3 (0.5, 6.4) years, whereas it was 4.3 (1.5, 12.6) years for S. aureus. Chronic P. aeruginosa infection was noted in 21 (6.7%) children, which was significantly associated with increasing age and lower weight. Antibiotic resistance was observed in 1.8%-76.6% of samples.

CONCLUSIONS: This study explores the first nationwide microbiological profile of the airway in Indian children with CF. P. aeruginosa was the most common isolated organism at enrollment and during FU. This study provides antibiotic sensitivity patterns to common organisms that will help to promote antibiotic stewardship and formulate policies for children with CF in this region.

PMID:40693575 | DOI:10.1002/ppul.71194

Pediatr Pulmonol. 2025 Jul;60(7):e71212. doi: 10.1002/ppul.71212.

ABSTRACT

OBJECTIVE: Targeted gene panels can be used to diagnose and exclude genetic disorders at a lower cost and sometimes shorter turn-around time than exome or genome sequencing. There are limited data on the yield of targeted gene panels for genetically-mediated respiratory disorders.

METHODS: Review of testing results and chart review was conducted for all patients having received one or more targeted gene panels for interstitial lung disease, mucociliary disorders, and/or pulmonary vascular disease during a 5-year period. Targeted gene panels (PulmZoom) were developed by the Johns Hopkins Genomics DNA Diagnostic Laboratory in conjunction with relevant faculty experts. Testing employed next generation sequencing (NGS) with Sanger sequencing to confirm low-quality and/or complex insertion-deletion variants where appropriate.

RESULTS: A total of 416 subjects received testing between January 2019 - December 2023. 4.1% received test results that confirmed or established a genetic diagnosis. Results from 1.9% of tests suggested a potential diagnosis, while 21.6% received uncertain results, and 72.4% received a negative result. Mucociliary subpanels had the highest yield of definitive or potential diagnoses, including cystic fibrosis, which was the most common disease identified, followed by primary ciliary dyskinesia.

CONCLUSIONS: Targeted gene panels can offer assessment of comprehensive lists of genes to aid in the diagnosis of genetically-mediated respiratory disorders. Yield for panels may be increased with selection of suitable patients via subspecialty evaluations through Pulmonology and Genetics. Future research should assess the efficacy of a sequential approach with targeted gene panels and exome analysis versus exome sequencing alone as well as cost/benefit analyses.

PMID:40693558 | DOI:10.1002/ppul.71212

Case Rep Oncol Med. 2025 Jul 14;2025:4443916. doi: 10.1155/crom/4443916. eCollection 2025.

ABSTRACT

Cutaneous squamous cell carcinoma (cSCC) is one of the most prevalent malignancies worldwide, and a subset of patients, particularly immunocompromised individuals, will face heightened risks of metastasis and mortality. This report examines two cases of immunocompromised patients treated with a combination of platinum- and taxane-based chemotherapy together with PD-1 inhibition, having progressed after PD-1 monotherapy. The first patient, a 54-year-old man with HIV and recurrent metastatic cSCC, had rapid progression of disease while undergoing PD-1 inhibition with cemiplimab but later achieved tumor control when treated with pembrolizumab, carboplatin, and paclitaxel. The second patient, a 36-year-old man with cystic fibrosis and a history of lung transplantation, had no signs of response to cemiplimab but experienced a partial response when treated with cemiplimab, carboplatin, and paclitaxel. These cases suggest that combining chemotherapy with PD-1 inhibition may help overcome primary resistance to PD-1 therapy in advanced cSCC.

PMID:40692904 | PMC:PMC12279414 | DOI:10.1155/crom/4443916

Gastro Hep Adv. 2025 Feb 8;4(6):100641. doi: 10.1016/j.gastha.2025.100641. eCollection 2025.

NO ABSTRACT

PMID:40692822 | PMC:PMC12277765 | DOI:10.1016/j.gastha.2025.100641

J Cyst Fibros. 2025 Jul 20:S1569-1993(25)01528-0. doi: 10.1016/j.jcf.2025.07.007. Online ahead of print.

ABSTRACT

BACKGROUND: Gastrointestinal (GI) complications are a common source of morbidity for people with cystic fibrosis (pwCF). The pathobiology of these clinical presentations is not fully understood, but there is evidence that gut dysmotility may be a primary contributor.

METHODS: We studied gut motility in ileum samples from CF (CFTR-/-) and wild-type (WT) swine at birth (P0) and one week of post-natal life (P7) using organ bath assays.

RESULTS: Ileal samples from both WT and CF swine displayed spontaneous peristalsis. CF swine presented with reduced basal amplitude of the peristaltic waves compared to WT swine. Stimulating the ileal samples with increasing concentrations of acetylcholine (ACh) resulted in four main findings: 1) ACh increased the amplitude of smooth muscle contraction in all ileal samples in a dose-dependent manner. 2) At P7, ACh stimulation caused a significant increase in the maximum smooth muscle contraction in the WT but not in the CF samples. 3) Increasing doses of ACh caused fatigue-like contracting decline in smooth muscle from WT samples at both ages, but not in samples from CF swine. 4) ACh stimulation had no effect on the frequency of smooth muscle contraction in either genotype.

CONCLUSIONS: Our results show ileal dysmotility in the CF swine characterized by a decrease in basal peristalsis and weaker smooth muscle contraction. Our data suggest that GI dysmotility would impact chyme transit through the GI tract, which may predispose pwCF to intestinal manifestations associated with the disease.

PMID:40691113 | DOI:10.1016/j.jcf.2025.07.007

Antimicrob Agents Chemother. 2025 Jul 21:e0053425. doi: 10.1128/aac.00534-25. Online ahead of print.

ABSTRACT

Mycobacterium abscessus lung infections remain difficult to treat. Previous studies have shown that the combinations of a carbapenem and a second-generation β-lactamase inhibitor are effective in vitro, in macrophages, and in zebrafish embryos. We report here that imipenem combined with avibactam is also effective in a C3HeB/FeJ mouse model of infection in reducing bacterial burden in organs. The addition of amoxicillin improved the antibacterial activity of this combination in the lungs and kidneys.

PMID:40689756 | DOI:10.1128/aac.00534-25

World Allergy Organ J. 2025 Jul 3;18(8):101082. doi: 10.1016/j.waojou.2025.101082. eCollection 2025 Aug.

ABSTRACT

Although 90% of asthmatic patients suffer from mild and moderate disease, little is known about the burden on health status and quality of life, the long-term trajectory of disease severity, and the socio-economic impact. The Mild Moderated Asthma Network of Italy (MANI) is a real-world, cross-sectional, prospective, observational cohort study designed to explore these issues. Here we aimed to provide an identikit of asthmatic patients receiving treatment according to GINA steps 1-4, and enrolled in the centers of excellence participating in the MANI. Among 679 analyzed patients, 63% were female, and the mean age was 50 ± 16 years. Asthma was mild in 15.8% of patients (GINA steps 1-2) and moderate in 84.2% (GINA steps 3-4). The mean age of asthma diagnosis was 34.3 ± 17.7 years, 50% of patients were suffering from allergic rhinitis, and 13% from nasal polyposis. Mean FEV1% was 91.4 ± 19.4%, predicted with a FEV1/FVC ratio of 74.7 ± 11.9. The mean asthma control test value was 21.2 ± 3.73, and AQLQ score was 5.74 ± 1.07. Among the included patients, 17.2% had at least one asthma exacerbation in the previous year, with 14.2% requiring systemic steroids; 6.2% were referred to an emergency room in the year prior to enrollment; 2.2% required an asthma-related hospitalization; and 0.6% had been admitted to an Intensive Care Unit (ICU). Unscheduled visits were necessary for 3.8% of patients, 6.5% reported ≥5 lost work days due to asthma, and 11.5% declared ≥10 lost days of spare time. About 70% of patients were receiving treatment according to GINA Track 1. Uncontrolled cases constituted 16.7% of patients treated according to GINA steps 1-2, and 26.3% of patients treated according to GINA steps 3-4 were uncontrolled. Compared to patients with mild asthma, those with moderate asthma had more impaired lung function (FEV1% 88.5 ± 18.4 vs 94.4 ± 17.9, p = 0.05; FEV1/FVC 73.0 ± 9.76 vs 79.6 ± 9.56, p > 0.001), exhibited greater need for systemic corticosteroids for treating exacerbations (13.8% vs 2.3%, p = 0.032), and showed greater adherence to therapy (TAI score 50.0 ± 5.66 vs 45.7 ± 8.42, p < 0.001). Overall, mild/moderate asthma exhibited a substantial clinical and care impact. Patients treated with GINA steps 3-4 constituted the vast majority of patients attending specialist centers. A quarter of these patients were uncontrolled, and therefore need re-evaluation or treatment upgrade. Expanding recruitment of the MANI study will allow further phenotyping of these patients.

PMID:40689384 | PMC:PMC12271777 | DOI:10.1016/j.waojou.2025.101082

Med Arch. 2025;79(2):159-163. doi: 10.5455/medarh.2025.79.159-163.

ABSTRACT

BACKGROUND: Children's drawings are considered an important tool for detecting emotions and experiences that a child may be unable or unwilling to express verbally.

OBJECTIVE: This study aimed to assess the emotional state, psychological development, and adjustment mechanisms of children with chronic diseases using projective drawing tests, and to compare their responses with those of healthy children.

METHODS: A cross-sectional design was implemented in one region of Greece, involving 100 children aged 6-12 years. The sample included 50 children with chronic diseases (25 with type 1 diabetes and 25 with cystic fibrosis) and 50 healthy children serving as a control group, selected from pediatric clinics and schools. Data collection took place between January and June 2023. Participants completed three projective tests: the Kinetic Family Drawing Test (KFD), Tree Drawing Test, and House Drawing Test, which assessed emotional expression and psychological functioning.

RESULTS: Children with chronic diseases expressed emotions differently compared to healthy peers. In the KFD, they depicted fewer smiling faces, indicating higher emotional distress and a more negative perception of their family environment. In the tree drawings, symbolic elements such as birds-representing freedom or hope - were more common among children with chronic diseases, while healthy children more often drew roots, suggesting emotional stability. The House Drawing Test revealed no significant differences between the two groups.

CONCLUSION: Projective tests such as the KFD and Tree Drawing Test appear to be effective in identifying emotional issues in children with chronic illnesses, unlike the House Drawing Test. These findings support the integration of such tools into psychological assessments and therapeutic interventions for pediatric chronic disease populations.

PMID:40689283 | PMC:PMC12269769 | DOI:10.5455/medarh.2025.79.159-163

J Clin Transl Endocrinol. 2025 Jul 2;41:100407. doi: 10.1016/j.jcte.2025.100407. eCollection 2025 Sep.

ABSTRACT

BACKGROUND: Elexacaftor-tezacaftor-ivacaftor (ETI) became available for adults with cystic fibrosis (CF) in 2019, but its impact on CF-related diabetes (CFRD) remains unclear.

METHODS: A single-center retrospective cohort study was conducted among adults with CFRD to examine the change in insulin dose-adjusted Hemoglobin A1c (IDAA1c). Linear mixed effects model (LMEM) analysis was used to investigate the change in IDAA1c between baseline and 24 months of follow up, comparing an ETI-treated group to an unexposed group. Baseline values were those documented at treatment initiation for the ETI-treated group and in March 2020 (±3 months) for the unexposed group.

RESULTS: A total of 49 adults were included, 39 were treated with ETI and 10 were not. Median [Interquartile range] time since CFRD diagnosis at baseline was 13 [7-18] and 14 [10-18] years, respectively (p = 0.610). In the ETI-treated group, mean weight increased by a 4.44 kg (95 % Confidence Interval, 95 %CI: 3.08 to 5.79, p < 0.001), insulin total daily dose decreased by 5 units (95 %CI: -9 to 0, p = 0.033), and hemoglobin A1c (%) decreased by 0.65 points (95 %CI: -0.96 to -0.34, p < 0.001). No change was observed in the unexposed group. LMEM analysis found a numerically significant association between ETI and decreased IDAA1c, estimated at -1.14 points (95 %CI: -2.35 to 0.06, p = 0.067) after adjusting for age, sex, time since CFRD diagnosis and the introduction of Metformin.

CONCLUSION: A numerically significant association between ETI and IDAA1c decrease was observed in adults with established CFRD after 24 months of treatment, suggesting ETI contributed to improved glycemic control.

PMID:40688702 | PMC:PMC12273438 | DOI:10.1016/j.jcte.2025.100407