Objective: To depict the epidemiological features of congenital pulmonary airway malformation (CPAM) in Chinese population.
Methods: Using 2010-2019 data from the Chinese Birth Defects Monitoring Network, we calculated the prevalence rates (PR) and 95% confidence intervals of isolated and nonisolated CPAM according to birth year, infant sex, maternal age and residence area, and examined the secular trends by using Poisson regression models. The perinatal outcomes of affected infants and associated malformations with CPAM were also analyzed.
Results: During this period, a total of 2451 CPAMs were identified among 20,183,999 births, yielding a PR of 1.21, 0.95, and 0.27 per 10,000 live and still births for the overall, isolated and nonisolated CPAM, respectively. Significant upward trends in CPAM PR were observed. The PR varied significantly by infant sex (male vs. female, 1.28/10,000 vs. 1.10/10,000), residence area (urban vs. rural, 1.49/10,000 vs. 0.88/10,000), and by maternal age (<20 years, 0.94/10,000; 20-24 years, 1.04/10,000; 25-29 years, 1.32/10,000; 30-34 years, 1.28/10,000; ≥35 years, 1.05/10,000). Compared with isolated CPAM, much more live births with nonisolated CPAM died in the early neonatal period (5.0% vs. 1.0%). The additional malformations with nonisolated CPAM occurred most frequently in the circulatory system.
Conclusions: The PR of CPAM in this study are comparable to those reported in non-Chinese populations. The increasing trend in prevalence and poor perinatal outcomes of the affected infants indicate an urgent need to strengthen the clinical and public health interventions of CPAM.
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http://dx.doi.org/10.1002/ppul.25785 | DOI Listing |
Eur J Hum Genet
December 2024
National EDS Service, London North West University Healthcare NHS Trust, London, UK.
Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder predominantly caused by pathogenic COL3A1 variants. Characteristic arterial and intestinal fragility and generalised severe tissue friability can lead to clinical events from childhood. We highlight a paucity of literature regarding children diagnosed with vEDS, possibly explained by a restraint in predictive testing, and present data on 63 individuals (23 index cases) with a clinical and genetic diagnosis of vEDS in childhood (<18 years) to address this.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
Department of Anesthesiology, University Children's Hospital, 30-663 Krakow, Poland.
Background: Major aortopulmonary collateral arteries (MAPCAs) are rare remnants of pulmonary circulation embryological development usually associated with complex congenital anomalies of the right ventricular outflow tract and pulmonary arteries. Effective management requires surgical unifocalization of MAPCAs and native pulmonary arteries (NPAs). Traditional imaging may lack the spatial clarity needed for precise surgical planning.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
Laboratory of X-Ray Endovascular and Reconstructive Cardiovascular Surgery, Department of Cardiovascular Surgery, Federal State Budgetary Institution "Research Institute for Complex Issues of Cardiovascular Diseases", Blvd. Named After Academician L.S. Barbarasha, 6, 650002 Kemerovo, Russia.
The Purpose: Evaluation of the short-term and long-term results of a phased correction of the tetralogy of Fallot (ToF) with stenting of the right ventricular outflow tract (RVOT) in comparison with a one-stage total correction (TC) of the defect.
Materials And Methods: Two groups of patients with classical ToF were formed. Group 1 (n = 25; median age = 72 days) was initially represented by children with ToF with a more severe clinical status (median weight = 3.
J Cardiovasc Dev Dis
December 2024
Cardiology Department, Mother and Child Health Institute of Serbia, 11070 Belgrade, Serbia.
Background: The vertical vein (VV) ligation during the total anomalous pulmonary venous return (TAPVR) correction is still controversial. Our study aimed to define the potential risk factors for VV persistence and their percutaneous occlusion.
Methods: The retrospective cohort study included 40 patients (26 males) with TAPVR treated at the tertiary referral center from 2005 to 2024.
Adv Respir Med
December 2024
Department of Thoracic Surgery, Beijing Institute of Respiratory Medicine and Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China.
Background: Recent studies on bronchiectasis have revealed significant structural abnormalities and pathophysiological changes. However, there is limited research focused on pulmonary venous variability and congenital variation. Through our surgical observations, we noted that coarctation of pulmonary veins and atrophied lung volume are relatively common in bronchiectasis patients.
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