Dataset Information

Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

ABSTRACT:

Objective

To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic.

Study design

This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEV₁pp), percent predicted forced vital capacity (FVCpp), and FEV₁/FVC over time.

Results

Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEV₁pp (P < .001), FVCpp (P = .017), and FEV₁/FVC (P = .001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEV₁pp by an average of 11.5% (95% CI, 2.9%-20.1%; P = .010). A history of oxygen use at initial hospital discharge also correlated with decreased FEV₁pp by an average of 8.0% (95% CI, 1.2%-15.0%; P = .023).

Conclusions

In a select cohort of CDH survivors, average pulmonary function declines with age relative to expected population normative values. Those with severe CDH represent a population at risk for worsening pulmonary function test measurements who may benefit from recognition and monitoring for complications.

SUBMITTER: Dao DT

PROVIDER: S-EPMC6917899 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

Dao Duy T DT Hayden Lystra P LP Buchmiller Terry L TL Kharasch Virginia S VS Kamran Ali A Smithers Charles J CJ Rice-Townsend Samuel E SE Zalieckas Jill M JM Becker Ronald R Morash Donna D Studley Mollie M Wilson Jay M JM Sheils Catherine A CA

The Journal of pediatrics 20191106

<h4>Objective</h4>To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic.<h4>Study design</h4>This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEV<sub>1</sub>pp), percent predicted forced vital capacity (FVCpp), and FEV<sub>1</sub>/FVC over ti ...[more]

PMID: 31704056

Similar Datasets

Project description:Congenital diaphragmatic hernia (CDH)-related deaths are the largest contributor to in-hospital neonatal deaths in children with congenital malformations. Morbidity and mortality in CDH are directly related to the development of pulmonary hypertension (PH). Current treatment consists of supportive measures. To date, no pharmacotherapy has been shown to effectively reverse the hallmark finding of pulmonary vascular remodeling that is associated with pulmonary hypertension in CDH (CDH-PH). As such, there is a great need for novel therapies to effectively manage CDH-PH. Our review aims to evaluate emerging therapies, and specifically focuses on those that are still under investigation and not approved for clinical use by the Food and Drug Administration. Therapies were categorized into antenatal pharmacotherapies or antenatal regenerative therapies and assessed on their method of administration, safety profile, the effect on pulmonary vascular pathophysiology, and overall efficacy. In general, emerging antenatal pharmaceutical and regenerative treatments primarily aim to alleviate pulmonary vascular remodeling by restoring normal function and levels of key regulatory factors involved in pulmonary vascular development and/or in promoting angiogenesis. Overall, while these emerging therapies show great promise for the management of CDH-PH, most require further assessment of safety and efficacy in preclinical models before translation into the clinical setting. IMPACT: Emerging antenatal therapies for congenital diaphragmatic hernia-induced pulmonary hypertension (CDH-PH) show promise to effectively mitigate vascular remodeling in preclinical models. Further investigation is needed in preclinical and human studies to evaluate safety and efficacy prior to translation into the clinical arena. This review offers a comprehensive and up-to-date summary of emerging therapies currently under investigation in experimental animal models. There is no cure for CDH-PH. This review explores emerging therapeutic options for the treatment of CDH-PH and evaluates their impact on key molecular pathways and clinical markers of disease to determine efficacy in the preclinical stage.

Dataset Information

Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

Objective

Study design

Results

Conclusions

Publications

Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure