TY - JOUR
T1 - Pulmonary hypoplasia correlates with the length of anhydramnios in patients with early pregnancy renal anhydramnios (EPRA)
AU - Jelin, Eric B.
AU - Hooper, Jody E.
AU - Duregon, Eleonora
AU - Williamson, Alex K.
AU - Olson, Sarah
AU - Voegtline, Kristin
AU - Jelin, Angie C.
N1 - Funding Information:
EBJ received funding from NIH NICHD RO1HD100540-01. ACJ received funding from the NIH NIDDK K23DK119949-02.
Publisher Copyright:
© 2021, The Author(s), under exclusive licence to Springer Nature America, Inc.
PY - 2021/8
Y1 - 2021/8
N2 - 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.
AB - 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.
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U2 - 10.1038/s41372-021-01128-0
DO - 10.1038/s41372-021-01128-0
M3 - Article
C2 - 34230606
AN - SCOPUS:85109870030
VL - 41
SP - 1924
EP - 1929
JO - Journal of Perinatology
JF - Journal of Perinatology
SN - 0743-8346
IS - 8
ER -