Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare systematic neoplastic disease exclusively seen in middle-aged women with an incidence of 5-9 per million. They can occur sporadically or in association with tuberous sclerosis. Histopathological diagnosis is the gold standard. Median transplant-free survival from the time of diagnosis is 23 years. We here by present premenstrual female with history of recurrent dyspnea with differential diagnosis for various interstitial lung disease diagnosed to have LAM. She was managed with bronchodilator therapy and pulmonary rehabilitation as per European respiratory society guidelines. Keywords: lymphangioleiomyomatosis; interstitial lung disease; uniportal VATS; histopathology

The Effect of Long-Acting Dual Bronchodilator Therapy on Exercise Tolerance, Dynamic Hyperinflation and Dead Space during Constant Work Rate Exercise in COPD

Purpose: We investigated whether dual bronchodilator therapy (glycopyrrolate/formoterol fumarate; GFF; Bevespi Aerosphere®) would increase exercise tolerance during a high-intensity constant work rate exercise test (CWRET) and the relative contributions of dead space ventilation (VD/VT) and dynamic hyperinflation (change in inspiratory capacity) to exercise limitation in COPD. Methods: 48 COPD patients (62.9±7.6yrs; 33 male; GOLD spirometry stage 1/2/3/4, n=2/35/11/0) performed a randomized, double blind, placebo (PL) controlled, two period crossover, single-center trial. Gas exchange and inspiratory capacity (IC) were assessed during cycle ergometry at 80% incremental exercise peak work rate. Transcutaneous PCO2(TcPCO2) measurement was used for VD/VTestimation. Results: Baseline post-albuterol FEV1was 1.86±0.58L (63.6%±13.9 predicted). GFF increased FEV1by 0.18±0.21L relative to PL (P<0.001). CWRET endurance time was greater after GFF vs. PL (383±184s vs 328±115s; difference 55±125s; P=0.013; C.I. 20-90s), a 17% increase. IC on GFF was above placebo IC at all time points and fell less with GFF vs. PL (P≤0.0001). Isotime tidal volume (1.54±0.50 vs. 1.47±0.45L; P=0.022) and ventilation (52.9±19.9 vs. 51.0±18.9 L/min; P=0.011) were greater, and respiratory rate was unchanged (34.9±9.2 vs. 35.1±8.0 br/min, P=0.865). Isotime VD/VTdid not differ between groups (GFF 0.28±0.08 vs. PL 0.27±0.09; P=0.926). Conclusions: GFF increased exercise tolerance in COPD patients and the increase was accompanied by attenuated dynamic hyperinflation without altering VD/VT. ClinicalTrials.Gov Identifier: NCT03081156

Effect of perioperative bronchodilator therapy on postoperative pulmonary function among lung cancer patients with COPD

Chronic obstructive pulmonary disease (COPD), an established risk factor for lung cancer, remains largely undiagnosed and untreated before lung cancer surgery. We evaluated the effect of perioperative bronchodilator therapy on lung function changes in COPD patients who underwent surgery for non-small cell lung cancer (NSCLC). From a database including NSCLC patients undergoing lung resection, COPD patients were identified and divided into two groups based on the use of bronchodilator during the pre- and post-operative period. Changes in forced expiratory volume in 1 s (FEV1) and postoperative complications were compared between patients treated with and without bronchodilators. Among 268 COPD patients, 112 (41.8%) received perioperative bronchodilator, and 75% (84/112) were newly diagnosed with COPD before surgery. Declines in FEV1 after surgery were alleviated by perioperative bronchodilator even after adjustments for related confounding factors including surgical extent, surgical approach and preoperative FEV1 (adjusted mean difference in FEV1 decline [95% CI] between perioperative bronchodilator group and no perioperative bronchodilator group; − 161.1 mL [− 240.2, − 82.0], − 179.2 mL [− 252.1, − 106.3], − 128.8 mL [− 193.2, − 64.4] at 1, 4, and 12 months after surgery, respectively). Prevalence of postoperative complications was similar between two groups. Perioperative bronchodilator therapy was effective to preserve lung function, after surgery for NSCLC in COPD patients. An active diagnosis and treatment of COPD are required for surgical candidates of NSCLC.

Features of the clinical course of asthma combined with gastroesophageal reflux disease in children

Among the comorbid conditions in children with asthma a significant place is occupied by lesions of the upper digestive tract+in particular, gastroesophageal reflux disease (GERD). It is known that asthma can provoke the development of reflux, and reflux in turn leads to a worsening of the course of asthma. Scientific studies indicate a better ability to control asthma in children after appropriate treatment for GERD. Purpose — to study the features of the clinical course of asthma combined with gastroesophageal reflux disease in children. Materials and methods. Ninety+nine children aged 6–17 years with asthma of varying severity and level of control were examined. These children which were randomly divided into two groups: group 1–79 children with asthma combined with GERD, and group 2–20 children with asthma without GERD. A questionnaire was administered, an assessment of day and night symptoms of asthma according to the scoring system was conducted, and an ACT test according to age was performed on all examined children. With the consent of the parents, twenty-two children who had complaints characteristic of gastrointestinal lesions, underwent fibroesophagogastroduodenoscopy with biopsy from the proximal and distal esophageal mucosa. Results. In children with asthma combined with GERD, atopic dermatitis is probably more common (in 46.8% of patients), and symptoms of hypersensitive reactions to food are registered (in 74.6% of patients). Children in group 1, in contrast to children in group 2, had a longer and more severe course of asthma and probably more frequent nocturnal symptoms of the disease, which was associated with poorer control of asthma and the need for bronchodilator therapy (8.5±0.5 inhalations per month against 4.5±0.5 inhalations per month, respectively, p<0,05). Peculiarities of GERD in children with asthma were: nonspecific complaints, catarrhal changes of the lower third of the esophagus (reflux esophagitis in 98.7%), focal hyperplasia of the basal layer of the epithelium and intraepithelial eosinophilia up to 5% (9%). Girls and boys of different ages experience significant differences in complaints from the gastrointestinal tract. Conclusions. The presence of GERD has been shown to affect the control of asthma in children, associated with nocturnal symptoms, the need for bronchodilator therapy, more severe and prolonged course of the disease, concomitant atopic dermatitis and food hypersensitivity reactions. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Keywords. asthma, gastroesophageal reflux disease, children.