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- Doughty, Elizabeth S., et al.
(författare)
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Long-Term Effect of TBX4 Germline Mutation on Pulmonary Clinico-Histopathologic Phenotype
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Ingår i: Pediatric and Developmental Pathology. - 1093-5266.
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Tidskriftsartikel (refereegranskat)abstract
- Tbx4 protein, expressed in mesenchyme of the developing lung, contributes to airway branching and distal lung growth. An association between pediatric onset of pulmonary arterial hypertension (PAH) and genetic variations coding for the T-box transcription factor 4 gene (TBX4) has been increasingly recognized. Tbx4-related PAH onset has a bimodal age distribution, including severe to lethal PAH in newborns and later onset PAH. We present an autopsy study of a 24-year-old male with a heterozygous TBX4 variant, who developed pulmonary arterial hypertension at age 12 years. This unique case highlights the complex pulmonary histopathology leading to lethal cardiopulmonary failure in the setting of TBX4 mutation.
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- Kielt, Matthew, et al.
(författare)
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Classifying multicenter approaches to invasive mechanical ventilation for infants with bronchopulmonary dysplasia using hierarchical clustering analysis
- 2023
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Ingår i: Pediatric Pulmonology. - : John Wiley & Sons. - 8755-6863 .- 1099-0496. ; 58:8, s. 2323-2332
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Tidskriftsartikel (refereegranskat)abstract
- IntroductionEvidence-based ventilation strategies for infants with severe bronchopulmonary dysplasia (BPD) remain unknown. Determining whether contemporary ventilation approaches cluster as specific BPD strategies may better characterize care and enhance the design of clinical trials. The objective of this study was to test the hypothesis that unsupervised, multifactorial clustering analysis of point prevalence ventilator setting data would classify a discrete number of physiology-based approaches to mechanical ventilation in a multicenter cohort of infants with severe BPD.MethodsWe performed a secondary analysis of a multicenter point prevalence study of infants with severe BPD treated with invasive mechanical ventilation. We clustered the cohort by mean airway pressure (MAP), positive end expiratory pressure (PEEP), set respiratory rate, and inspiratory time (Ti) using Ward's hierarchical clustering analysis (HCA).ResultsSeventy-eight patients with severe BPD were included from 14 centers. HCA classified three discrete clusters as determined by an agglomerative coefficient of 0.97. Cluster stability was relatively strong as determined by Jaccard coefficient means of 0.79, 0.85, and 0.77 for clusters 1, 2, and 3, respectively. The median PEEP, MAP, rate, Ti, and PIP differed significantly between clusters for each comparison by Kruskall–Wallis testing (p < 0.0001).ConclusionsIn this study, unsupervised clustering analysis of ventilator setting data identified three discrete approaches to mechanical ventilation in a multicenter cohort of infants with severe BPD. Prospective trials are needed to determine whether these approaches to mechanical ventilation are associated with specific severe BPD clinical phenotypes and differentially modify respiratory outcomes.
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- Lewis, Tamorah R., et al.
(författare)
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Association of Racial Disparities With In-Hospital Outcomes in Severe Bronchopulmonary Dysplasia
- 2022
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Ingår i: JAMA pediatrics. - : American Medical Association (AMA). - 2168-6203 .- 2168-6211. ; 176:9, s. 852-859
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Tidskriftsartikel (refereegranskat)abstract
- Importance: Bronchopulmonary dysplasia (BPD) is the most common serious morbidity of preterm birth. Short-term respiratory outcomes for infants with the most severe forms of BPD are highly variable. The mechanisms that explain this variability remain unknown and may be mediated by racial disparities.Objective: To determine the association of maternal race with death and length of hospital stay in a multicenter cohort of infants with severe BPD.Design, setting, and participants: This multicenter cohort study included preterm infants enrolled in the BPD Collaborative registry from January 1, 2015, to July 19, 2021, involving 8 BPD Collaborative centers located in the US. Included patients were born at less than 32 weeks' gestation, had a diagnosis of severe BPD as defined by the 2001 National Institutes of Health Consensus Criteria, and were born to Black or White mothers.Exposures: Maternal race: Black vs White.Main outcomes and measures: Death and length of hospital stay.Results: Among 834 registry infants (median [IQR] gestational age, 25 [24-27] weeks; 492 male infants [59%]) meeting inclusion criteria, the majority were born to White mothers (558 [67%]). Death was observed infrequently in the study cohort (32 [4%]), but Black maternal race was associated with an increased odds of death (adjusted odds ratio, 2.1; 95% CI, 1.2-3.5) after adjusting for center. Black maternal race was also significantly associated with length of hospital stay (adjusted between-group difference, 10 days; 95% CI, 3-17 days).Conclusions and relevance: In a multicenter severe BPD cohort, study results suggest that infants born to Black mothers had increased likelihood of death and increased length of hospital stay compared with infants born to White mothers. Prospective studies are needed to define the sociodemographic mechanisms underlying disparate health outcomes for Black infants with severe BPD.
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- Marsal, Karel, et al.
(författare)
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Fetal and placental circulation during labor
- 2004
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Ingår i: Fetal and Neonatal Physiology. - 9997628314 - 9997628268 - 0721696546 ; , s. 758-758
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 6. |
- McKinney, Robin L., et al.
(författare)
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Multicenter Experience with Neurally Adjusted Ventilatory Assist in Infants with Severe Bronchopulmonary Dysplasia
- 2021
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Ingår i: American Journal of Perinatology. - : Georg Thieme Verlag KG. - 0735-1631 .- 1098-8785. ; 38, s. E162-E166
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Tidskriftsartikel (refereegranskat)abstract
- Objective The aim of this study is to determine patterns of neurally adjusted ventilatory assist (NAVA) use in ventilator-dependent preterm infants with evolving or established severe bronchopulmonary dysplasia (sBPD) among centers of the BPD Collaborative, including indications for its initiation, discontinuation, and outcomes.Study Design Retrospective review of infants with developing or established sBPD who were placed on NAVA after ≥4 weeks of mechanical ventilation and were ≥ 30 weeks of postmenstrual age (PMA).Results Among the 13 sites of the BPD collaborative, only four centers (31%) used NAVA in the management of infants with evolving or established BPD. A total of 112 patients met inclusion criteria from these four centers. PMA, weight at the start of NAVA and median number of days on NAVA, were different among the four centers. The impact of NAVA therapy was assessed as being successful in 67% of infants, as defined by the ability to achieve respiratory stability at a lower level of ventilator support, including extubation to noninvasive positive pressure ventilation or support with a home ventilator. In total 87% (range: 78–100%) of patients survived until discharge.Conclusion We conclude that NAVA can be used safely and effectively in selective infants with sBPD. Indications and current strategies for the application of NAVA in infants with evolving or established BPD, however, are highly variable between centers. Although this pilot study suggests that NAVA may be successfully used for the management of infants with BPD, sufficient experience and well-designed clinical studies are needed to establish standards of care for defining the role of NAVA in the care of infants with sBPD.
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| 8. |
- Sindelar, Richard, Docent, 1964-, et al.
(författare)
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Established severe BPD: is there a way out? Change of ventilatory paradigms
- 2021
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Ingår i: Pediatric Research. - : Springer Nature. - 0031-3998 .- 1530-0447. ; 90:6, s. 1139-1146
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Tidskriftsartikel (refereegranskat)abstract
- Improved survival of extremely preterm newborn infants has increased the number of infants at risk for developingbronchopulmonary dysplasia (BPD). Despite efforts to prevent BPD, many of these infants still develop severe BPD (sBPD) andrequire long-term invasive mechanical ventilation. The focus of research and clinical management has been on the prevention ofBPD, which has had only modest success. On the other hand, research on the management of the established sBPD patient hasreceived minimal attention even though this condition poses large economic and health problems with extensive morbidities andlate mortality. Patients with sBPD, however, have been shown to respond to treatments focused not only on ventilatory strategiesbut also on multidisciplinary approaches where neurodevelopmental support, growth promoting strategies, and aggressivetreatment of pulmonary hypertension improve their long-term outcomes. In this review we will try to present a physiology-basedventilatory strategy for established sBPD, emphasizing a possible paradigm shift from acute efforts to wean infants at all costs to amore chronic approach of stabilizing the infant. This chronic approach, herein referred to as chronic phase ventilation, aims atallowing active patient engagement, reducing air trapping, and improving ventilation-perfusion matching, while providingsufficient support to optimize late outcomes.
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