Pulmonary Associates

Post-intubation tracheal stenosis (PITS) is a potentially life-threatening but often underdiagnosed complication of prolonged mechanical ventilation. Its incidence is expected to increase with the global increase in intensive care unit admissions. We report two male patients, aged 60 and 18 years, in whom symptomatic tracheal restenosis developed after surgical repair for PITS. Both patients presented with dyspnea and stridor. Rigid bronchoscopy was performed with balloon dilation and silicone stent placement. In one case, granulation tissue developed as a complication of long-term stenting and was successfully treated with electrocautery. Both patients showed marked clinical improvement and maintained airway patency at 12-month follow-up. These cases highlight the importance of early recognition of PITS and indicate the value of bronchoscopic intervention as an effective salvage strategy when surgical repair is unsuccessful.

<bold>Rationale:</bold> The criteria for positive bronchodilator response (BDR) has been changed to address bias from age, sex and baseline FEV1. Although the same bias could be expected when assessing bronchial hyperreactivity (BHR), this is not applied to bronchial provocation test (BPT). <bold>Aim:</bold> To investigate the impact of using 20% fall in FEV1 from predicted when assessing BHR. <bold>Method:</bold> The PD₂₀ from baseline (PD₂₀BL) and PD₂₀ from predicted (PD₂₀PRED) were calculated on all BPT referrals from January2020 to December 2024. BPT was done using dosimeter protocol. Analysis was performed using GraphPad Prism. <bold>Result:</bold> The analysis of 157 BPTs resulted in 98 positive tests. Using PD₂₀PRED 61% changed in BHR severity classification, Fig.1. Majority of the shift, Table1, was observed within the mild BHR and marked BHR group. Two cases of mild BHR started treatment, however the PD₂₀PRED resulted in a normal BHR. Those who had lower severity results with PD₂₀PRED had FEV₁ >100% of predicted. <bold>Conclusion:</bold> Assessing BHR using PD20PRED could provide better classification, especially in the mild category leading to possible change in therapy. <fig><object-id>erj;66/suppl_69/OA2225/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig> <table-wrap><object-id>erj;66/suppl_69/OA2225/TB1</object-id><object-id>T1</object-id><object-id>TB1</object-id><caption> Table 1: Summary of BHR severity. </caption><table><colgroup><col></col><col></col><col></col></colgroup><tbody><tr><td>Severity</td><td>PD20BL</td><td>PD20PRED</td></tr><tr><td>Marked BHR</td><td>24</td><td>42</td></tr><tr><td>Moderate BHR</td><td>31</td><td>27</td></tr><tr><td>Mild BHR</td><td>42</td><td>26</td></tr><tr><td>Normal</td><td>1</td><td>3</td></tr></tbody></table></table-wrap>

<bold>Aim:</bold> We aimed to compare the clinical and laboratory features of siblings with cystic fibrosis (CF) registered in the Cystic Fibrosis Registry of Turkiye (CFRT-TR) in 2023, both before and after newborn screening (NBS) and modulatory treatment. <bold>Method:</bold> Siblings with two or more CF diagnoses were included. A comparison was made based on clinical and laboratory characteristics before and after NBS (before 2015) and modulatory treatment. <bold>Results:</bold> A total of 240 siblings from 113 families were included (47% female), with a mean age of 9 years. The median age at diagnosis was 0.33 years. Of these, 135 were born before NBS. Among those not receiving modulatory treatment, the mean age of diagnosis for older siblings was (2.97±4.36 years), compared to (1.00±2.29 years) for younger siblings (p<0.001). No significant differences in weight, height, BMI z-scores, colonization status, or FEV1% were found between older and younger siblings (p>0.05), though liver disease was more common in older siblings (p=0.011). Younger siblings diagnosed after NBS were diagnosed earlier (0.59±1.10 years) than older siblings diagnosed before NBS (2.84±4.37 years) (p=0.003). No significant differences in clinical parameters were found between the two groups (p>0.05). No differences were noted in FEV1 changes, growth parameters, colonization status, or complications before and after modulatory treatment between the older and younger siblings. <bold>Conclusions:</bold> Younger siblings were diagnosed earlier with NBS. Having more than two CF siblings did not affect clinical follow-up. Modulatory treatment improved clinical and laboratory outcomes, consistent with existing CF literature.

Salbutamol (Salb) is often used as a bronchodilator when performing lung function tests. In April 2024 Salb was temporarily unavailable in our country, resulting in a switch to formoterol (Form). According to literature a similar bronchodilator response has been seen using Salb 400 µg and Form 24 µg. However, because of the side effects we decided to use 12 µg instead of 24 µg. The aim of the study was to investigate whether 12 µg Form shows a similar response as 400 µg Salb. Methods For measuring the response, bronchodilator recovery (BDR) after methacholine challenge tests (MCT) was used. All tests were performed in 2024. BDR was measured 15 minutes after submitting Salb or Form. The BDR was defined as: (FEV1 after bronchodilation – FEV1 pre provocation) / FEV1 pre provocation) * 100%. The BDR of Salb and the BDR of Form in tests with a fall in FEV1 of at least 11% were compared with an independent t-test. Results Salb and Form groups were similar in age (47.8 resp. 47.4), BMI (30 kg/m2) and % fall in FEV1 (21.4% resp. 19.8%). Both groups had more female than men (62% resp. 54%). The mean BDR of the Salb and Form group were resp. –2,0% and –4,4%, p=0.001. Conclusion The BDR of 12 μg Form is significantly lower than the BDR of 400 μg Salb. This is not clinically relevant for MCT, however this may be a clinical relevant difference with respect to other lung function tests. Further research is needed. <fig><object-id>erj;66/suppl_69/PA641/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

Ataxia telangiectasia AT), an autosomal recessive disorder due to mutations in the ATM gene, results in progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, pulmonary compromise, sensitivity to ionizing radiation, and increased risk of hematologic malignancies. Orthopedic manifestations such as scoliosis, hip displacement, gait abnormalities, and extremity contractures are common, but reports are scarce. This study aimed to evaluate and summarize the orthopedic manifestations associated with AT. A retrospective review was conducted of patients with AT. Reported outcomes included demographics, orthopedic manifestations and operative/nonoperative treatments, radiographic exposure, development of malignancy, and ambulatory status (using the Functional Mobility Scale at 50 m, FMS50). Twenty-four children were included [11 (45.8%) female] with a mean age at diagnosis of 5.5 (SD = 3.5) years. The most common reason for orthopedic consultation was ataxia; 12 (50%) were walkers (FMS50 = 4,5) and 12 nonwalkers (FMS50 = 1,2). Foot deformities were present in 10 (42%) including pes planovalgus (N = 6, 25%), Achilles tendon contracture (N = 1, 4%), hallux valgus (N = 1, 4%; underwent Akin osteotomy), equinovarus (N = 1.4%), and gastrocnemius contracture (N = 1, 4%). Six children (25%) developed scoliosis, and three underwent fusion. Other manifestations included hip flexion contracture (N = 2, 8%), hamstring contracture (N = 2, 8%), torticollis (N = 1, 4%), and osteomyelitis of the ischium (N = 1, 4). This report describes orthopedic manifestations associated with AT, most commonly foot deformities, followed by scoliosis. Since radiographic surveillance was not performed due to radiosensitivity, the frequency of hip displacement in AT could not be ascertained. Orthopedic surgical interventions, where required, were generally successful. Level of evidence: 3.

Dyspnea is a common symptom with varied causes. Pulmonary vascular involvement in Hereditary Hemorrhagic Telangiectasia (HHT) includes pulmonary arteriovenous malformations (PAVMs) and pulmonary hypertension (PH), both which can result in dyspnea. Additionally, dyspnea is a common symptom in individuals with obstructive airway disorders (OADs) such as asthma, bronchiectasis and chronic obstructive pulmonary disease (COPD). These respiratory conditions are not mutually exclusive, individuals with HHT can have pulmonary vascular involvement and a concomitant OAD. However, the likelihood of this co-occurrence is not currently known. We aimed to determine the prevalence of co-occurrence to improve patient diagnosis and management. We conducted a cross-sectional review of individuals seen in the Edmonton HHT Center as of July 2023 with a definite diagnosis of HHT to assess the proportion of patients with a concomitant OAD (asthma, bronchiectasis, or COPD). 132 patient charts were included. 55.3% had at least one identified PAVM and 28.0% had a documented OAD (asthma = 15.9%, COPD = 10.6%, Bronchiectasis = 2.3%). More importantly, 18.9% of individuals had both an OAD and a PAVM. Dyspnea as a symptom of OADs requires specialized assessment and management. PAVMs in HHT also require specialized care, but involve different treatment approaches. It is important to identify individuals who have both HHT and an OAD to improve management which takes both OADs and pulmonary vascular disorders into account when assessing HHT patients with dyspnea. Early recognition creates better precision health and more effective care of individuals with HHT and dyspnea.