Lung Transplantation in Patients With Systemic Scleroderma-Description of the First Consecutive Cases in Poland: Case Series Report and a Short Literature Review.

Pulmonary complications of systemic scleroderma (SSc), such as interstitial lung disease and pulmonary hypertension (PH), are responsible for up to 60% of deaths among patients. For many years, most centers considered SSc a contraindication to lung transplantation (LTx); however, recent publications show that appropriately selected SSc candidates for LTx give results comparable to patients with idiopathic PH or idiopathic pulmonary fibrosis. This paper presents the cases of a 60-year-old male patient (patient 1) and a 42-year-old female patient (patient 2) diagnosed with SSc in 2019 and 2013, respectively. In both patients, interstitial-fibrotic changes in the lungs leading to respiratory failure were confirmed by high-resolution computed tomography as well as pulmonary hypertension (WHO group 3), which was also diagnosed during right heart catheterization. In both cases, despite pharmacotherapy, pulmonary fibrosis progressed, leading to severe respiratory failure. The patients were referred for LTx qualification. LTx was possible to consider in patients due to the lack of significant changes in other internal organs. Double LTx was successfully performed in both patients (patient 1-July 19, 2022; patient 2-September 14, 2022). They were discharged from the hospital in good condition on the 22nd and 20th postoperative day, respectively. LTx is a last-chance therapy that saves lives among patients with extreme respiratory failure in the course of SSc. It prolongs and improves the quality of life. The selection of appropriate patients is key to the success of the procedure.

Lung Transplantation in Patients With Pulmonary Hypertension With Extracorporeal Membrane Oxygenation (ECMO) Support: 5-Year Experience.

Lung transplantation (LTx) is the only treatment option of patients (pts) with pulmo-nary hypertension (PH) when pharmacologic treatment is unsatisfactory. ECMO is essential during LTx in every patient with pulmonary arterial hypertension and in most patients with sec-ondary PH. This is a retrospective, single-center study comparing LTx outcomes in patients with and without PH covering a 5-year experience. In the years 2018-2023, 219 LTx were performed, of which 56 (25.6%) with ECMO support, among which PH was diagnosed in 34pts (60.7%) in WHO groups 1,3,4: 19pts, 14pts. and 1pt respectively. The veno-arterial type of ECMO was used in patients with PH as intraoperative support (n = 34; 100%). The early (30-day) and long-term survival (1 year) of patients with and without PH did not differ statistically: 91.2% (95% CI: 82.1%-100%) vs. 77.3% (95% CI: 82.1%-100%)(P = .48) and 53.0% (95% CI: 36.6%-76.7 %) vs. 41.3% (95%CI: 23.1-74.0) (P = .48) respectively and the median hospitalization time from ECMO weaning to dis-charge was also comparable: 31 days (Q1-Q3: 21-40; IQR 20) vs. 28 days (Q1-Q3: 24-42; IQR :18) (P = .99). Patients with or without PH undergoing LTx with ECMO have comparable survival and hospital stay outcomes despite being the most challenging of all lung diseases treated with lung transplantation.

The aspects of local allergic rhinitis in Polish children and adolescents.

Introduction: Local allergic rhinitis (LAR) is one of the endotypes of rhinitis which occurs commonly in different age groups. Aim: To present the occurrence and characteristics of LAR in Polish children and adolescents. Material and methods: In the study protocol, three hundred sixty-one patients aged 5-17 with chronic rhinitis were included from 8 centres in Poland. Medical history and diagnostic procedures were performed with aeroallergens: skin prick tests, allergen-specific serum IgE and nasal provocation tests. In addition to LAR, allergic rhinitis (AR), dual allergic rhinitis (DUAL) and non-allergic rhinitis (NAR) were explored and compared. Results: LAR was confirmed in 21% of patients, systemic allergic rhinitis (SAR) in 43.9%, DUAL in 9.4% and NAR in 33.9% of patients. Based on nasal provocation test (NPT), allergy to HDM prevailed in the LAR group (68%), grass in the SAR group (58%), and grass and HDM in the DUAL group (32% and 64%). Girls were prevalent in the LAR group, and severe rhinitis and asthma were more common than other endotypes (p < 0.05). Conclusions: LAR is a common disease in children and adolescents and is often associated with severe rhinitis and frequently coexists with asthma.

Novel Hybrid Treatment for Pulmonary Arterial Hypertension with or without Eisenmenger Syndrome: Double Lung Transplantation with Simultaneous Endovascular or Classic Surgical Closure of the Patent Ductus Arteriosus (PDA).

Patients with pulmonary arterial hypertension (PAH) become candidates for lung or lung and heart transplantation when the maximum specific therapy is no longer effective. The most difficult challenge is choosing one of the above options in the event of symptoms of right ventricular failure. Here, we present two female patients with PAH: (1) a 21-year-old patient with Eisenmenger syndrome, caused by a congenital defect-patent ductus arteriosus (PDA); and (2) a 39-year-old patient with idiopathic PAH and coexistent PDA. Their common denominator is PDA and the hybrid surgery performed: double lung transplantation with simultaneous PDA closure. The operation was performed after pharmacological bridging (conditioning) to transplantation that lasted for 33 and 70 days, respectively. In both cases, PDA closure effectiveness was 100%. Both patients survived the operation (100%); however, patient no. 1 died on the 2nd postoperative day due to multi-organ failure; while patient no. 2 was discharged home in full health. The authors did not find a similar description of the operation in the available literature and PubMed database. Hence, we propose this new treatment method for its effectiveness and applicability proven in our practice.