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Archivos de Pediatría del Uruguay

Print version ISSN 0004-0584On-line version ISSN 1688-1249

Abstract

AMARO, Verónica et al. Characteristics of children and adolescents with chronic lung diseases referred for assessment by the Uruguayan lung transplant team in 2003 - 2023. Arch. Pediatr. Urug. [online]. 2024, vol.95, n.nspe1, e211.  Epub Sep 01, 2024. ISSN 0004-0584.  https://doi.org/10.31134/ap.95.s1.3.

Introduction:

lung transplantation has been recently consolidated as a therapeutic option for children and adolescents with severe lung disease when other treatments have failed. This procedure provides a good quality of life and additional survival, similar to that of transplantation in adult patients. The general indication for lung transplantation is progressive, potentially fatal respiratory failure due to parenchymal or vascular lung disease. The selection of transplant candidates is complex, requiring specific medical and socio-environmental conditions.

Objectives:

to analyze the clinical characteristics of pediatric patients with chronic lung diseases referred for evaluation by the Uruguayan lung transplant team. To analyze the clinical characteristics and survival of Uruguayan pediatric lung transplant patients.

Material and methods:

descriptive and retrospective study of all patients under 19 years of age referred to the lung transplant team between 2003 and 2023. The data was obtained from the program’s database and through medical records’ review.

Results:

30 patients were included (15 male). The mean age of the referred patients was 13.4 ± 3.6 years. Etiologies were 17/30 (56.6%) cystic fibrosis, 4/30 (13.3%) bronchiolitis obliterans post-bone marrow transplant, 4/30 (13.3%) pulmonary hypertension, 3 /30 (10%) post-infectious bronchiolitis obliterans, and 2/30 (6.6%) diffuse interstitial lung disease.

14 patients were placed on the transplant list, of which 9 were transplanted, 1 remains on the waiting list and 4 died while waiting for the transplant. Of the rest of the patients referred for evaluation: 7 patients are still under evaluation. 2 patients were excluded for psychosocial reasons or medical reasons. 2 patients could not be followed-up and other 5 died during the evaluation.

The etiologies of the pediatric transplant patients were cystic fibrosis (3/9), post-bone marrow transplant bronchiolitis obliterans (3/9), and pulmonary hypertension (3/9). Of the transplanted patients, 5 died (two in the post-operative period, and three died from sepsis between 1-2 years post-transplant). The remaining patients are in post-transplant follow-up for 1 month, 2, 7 and 9 years. No statistically significant differences in conditional survival were found when comparing adult and pediatric patients.

Conclusions:

results in transplanted patients in terms of characteristics and causes of mortality are comparable with those reported internationally. Post-transplant mortality was somewhat higher than in other studies, but we believe that there is bias due to the low number of patients. Given the results above, we propose that transplantation continues to be a valid therapeutic alternative in our country.

The challenges of this treatment include the limited availability of suitable donor organs, the toxicity of immunosuppressive medications necessary to prevent rejection, prevention and treatment of bronchiolitis obliterans, as well as maximizing the growth, development and quality of life of the receivers.

Keywords : Chronic Obstructive Pulmonary Disease; Lung Transplant; Pediatrics.

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