Advances in molecular genetics have enabled improvement of knowledge in pathogenesis and diagnosis of either monogenic or multifactorial neonatal lung diseases. Variants in genes regulating surfactant function and metabolism are implicated in some rare and common respiratory diseases. Congenital surfactant deficiencies are rare diseases due to mutations in genes encoding surfactant proteins and cause significant and often lethal respiratory failure in newborns and interstitial lung disease in older children. Diagnosis is made by molecular analysis and eventually confirmed by histological analysis of lung tissue. A multifactorial contribution, resulting from interaction between multiple genes and environmental factors, has been supposed for respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD). Several potential candidate genes, especially regarding surfactant proteins and cytokines, have been shown in association with these diseases. Genetic variants predisposing to RDS or BPD are usually polymorphisms which are not causative, but can increase susceptibility to the disease. Identification of infants at risk of disease can be useful to provide them individualized therapies. (www.actabiomedica.it).
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Background: Anakinra is an interleukin-1 receptor antagonist (IL-1Ra). Since IL-1 has been shown to play a key role in the etiology of different autoinflammatory diseases, blocking its pathway has become an important therapeutic target, even in neonates.
Aims: We aimed to report our experience in using anakinra to treat specific neonatal inflammatory conditions.
Adv Sci (Weinh)
January 2025
Department of Molecular Biology, Cellular Biology, and Biochemistry, Brown University, Providence, RI, 02912, USA.
Pulmonary hypertension (PH) increases the mortality of preterm infants with bronchopulmonary dysplasia (BPD). There are no curative therapies for this disease. Lung endothelial carnitine palmitoyltransferase 1a (Cpt1a), the rate-limiting enzyme of the carnitine shuttle system, is reduced in a rodent model of BPD.
View Article and Find Full Text PDFBMC Pediatr
January 2025
Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Background: Consequences of lung injury and inflammation in preterm infants with bronchopulmonary dysplasia (BPD) contribute to prolonged oxygen requirements. Home oxygen therapy (HOT) is an alternative way of respiratory support in infant with BPD. However, there is no consensus on weaning guidelines.
View Article and Find Full Text PDFPediatr Surg Int
January 2025
Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa, Nagoya, Aichi, 466-8550, Japan.
Purpose: To analyze the frequency and predictive factors of the development of postoperative pectus excavatum and scoliosis in children who underwent surgery for cystic lung disease.
Methods: This study examined patients who underwent surgery for cystic lung disease (open and thoracoscopic) between July 2000 and December 2018 with a > 3-year follow-up period. Lesion size, surgical outcomes, and subsequent musculoskeletal complications were compared between the open surgery and thoracoscopic surgery groups.
BMJ Open Respir Res
December 2024
Department of Design Sciences, Lund University, Lund, Sweden
Rationale: Preterm infants diagnosed with bronchopulmonary dysplasia (BPD) are thought to have fewer and larger alveoli than their term peers, but it is unclear to what degree this persists later in life.
Objectives: To investigate to what degree the distal airspaces are enlarged in adolescents born preterm and to evaluate the new Airspace Dimension Assessment (AiDA) method in investigating this group.
Methods: We investigated 41 adolescents between 15 and 17 years of age, of whom 25 were born very preterm (a gestational age <31 weeks, with a mean of 26 weeks) and 16 were term-born controls.
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