Pulmonary hypoplasia correlates with the length of anhydramnios in patients with early pregnancy renal anhydramnios (EPRA).

Autor: Jelin EB; Division of Pediatric Surgery, Department of Surgery, Johns Hopkins University, Baltimore, MD, USA., Hooper JE; Department of Pathology, Johns Hopkins University, Baltimore, MD, USA., Duregon E; Translational Gerontology Branch, National Institute on Aging (NIA/NIH), Baltimore, MD, USA., Williamson AK; Department of Pathology and Laboratory Medicine, Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA., Olson S; Biostatistics, Epidemiology and Data Management (BEAD) Core, Johns Hopkins School of Medicine, Baltimore, MD, USA., Voegtline K; Biostatistics, Epidemiology and Data Management (BEAD) Core, Johns Hopkins School of Medicine, Baltimore, MD, USA.; Department of Pediatrics, Johns Hopkins University, Baltimore, MD, USA., Jelin AC; Division of Maternal-Fetal Medicine, Department of Gynecology and Obstetrics, John Hopkins University, Baltimore, MD, USA. ajelin1@jhmi.edu.; Department of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA. ajelin1@jhmi.edu.
Jazyk: angličtina
Zdroj: Journal of perinatology : official journal of the California Perinatal Association [J Perinatol] 2021 Aug; Vol. 41 (8), pp. 1924-1929. Date of Electronic Publication: 2021 Jul 06.
DOI: 10.1038/s41372-021-01128-0
Abstrakt: Background: Early pregnancy renal anhydramanios (EPRA) occurs when the fetus is anuric before 22 weeks gestational age (GA) and is considered universally lethal. Serial amnioinfusions have successfully ameliorated the lethal pulmonary hypoplasia associated with EPRA and have resulted in cases of neonatal survival, peritoneal dialysis, and renal transplant.
Objective: We sought to evaluate the lung pathology of untreated fetuses and neonates that had EPRA.
Study Design: This is a retrospective case series of all fetuses and neonates diagnosed with isolated EPRA that underwent autopsy at a single tertiary care center between 1987 and 2018. Autopsy data were correlated with ultrasound findings and GA at delivery. Fetal weights, lung weights, and lung developmental stage were recorded.
Results: Nineteen cases met criteria for analysis and ranged from 16 to 38 weeks GA at termination or birth. The observed-to-expected (O/E) lung-to-body-weight ratio was significantly associated with GA (r = -0.51, p = 0.03), such that as GA increased the O/E ratio decreased. When limited to patients >22 weeks, this relationship strengthened (r = -0.75, p = 0.01). Importantly, overall O/E body weight had no relationship with GA.
Conclusion: This study shows that the degree of pulmonary hypoplasia in EPRA increases with the length of anhydramnios. This suggests that amnioinfusions are likely to be of most benefit the soonest they can feasibly be initiated.
(© 2021. The Author(s), under exclusive licence to Springer Nature America, Inc.)
Databáze: MEDLINE
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