Search

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is the most common prenatally-diagnosed lung malformation. This lesion, classified as macrocystic or microcystic, can lead to significant fetal compromise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention. Current evidence suggests that microcystic (but not macrocystic) lesions and those with a cyst volume ratio (CVR) >1.6 are responsive to steroid therapy. The objective of this study was to identify patterns of prenatal steroid administration for the management of CPAMs and to identify characteristics of CPAMs prompting steroid administration. METHODS: An 18-question survey was distributed to obstetricians from the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet), from January to April 2019, to capture antenatal steroid prescribing patterns. RESULTS: Response rates were 28.3% (138/487) for PRCRN and 63.3% (19/30) for NAFTNet. Among PRCRN members, 16.8% administered prenatal steroids, with most (77.2%) doing so for both microcystic and macrocystic CPAMs; corresponding percentages for NAFTNet members were 90.9% and 52.6%. Two thirds (65.6%) of obstetricians who administer steroids do so for a CVR > 1.6, without evidence of mediastinal shift or hydrops fetalis. CONCLUSIONS: There is a lack of consensus among obstetricians as to the CPAM characteristics that should prompt administration of prenatal steroids. Many surveyed obstetricians do not use cyst type or CVR to guide decision-making regarding steroid therapy.

Anhydramnios secondary to anuria before 22 weeks of gestational age and congenital bilateral renal agenesis before 26 weeks of gestational age are collectively referred to as early-pregnancy renal anhydramnios. Early-pregnancy renal anhydramnios occurs in at least 1 in 2000 pregnancies and is considered universally fatal when left untreated because of severe pulmonary hypoplasia precluding ex utero survival The Renal Anhydramnios Fetal Therapy (RAFT) trial is a nonrandomized, nonblinded, multicenter clinical trial designed to assess the efficacy, safety, and feasibility of amnioinfusions for patients with pregnancies complicated by early-pregnancy renal anhydramnios. The primary objective of this study is to determine the proportion of neonates surviving to successful dialysis, defined as use of a dialysis catheter for ≥14 days.A consortium of 9 North American Fetal Therapy Network (NAFTNet) centers was formed, and the RAFT protocol was refined in collaboration with the NAFTNet Scientific Committee. Enrollment in the trial began in April 2020. Participants may elect to receive amnioinfusions or to join the nonintervention observational expectant management group. Eligible pregnant women must be at least 18 years of age with a fetal diagnosis of isolated early-pregnancy renal anhydramnios.In addition to the primary study objective stated above, secondary objectives include (1) to assess maternal safety and feasibility of the serial amnioinfusion intervention (2) to perform an exploratory study of the natural history of untreated early pregnancy renal anhydramnios (3) to examine correlations between prenatal imaging and lung specific factors in amniotic fluid as predictive of the efficacy of serial percutaneous amnioinfusions and (4) to determine short- and long-term outcomes and quality of life in surviving neonates and families enrolled in RAFT IMPLICATIONS: The RAFT trial is the first clinical trial to investigate the efficacy, safety, and feasibility of amnioinfusions to treat the survival-limiting pulmonary hypoplasia associated with anhydramnios. Although the intervention offers an opportunity to treat a condition known to be almost universally fatal in affected neonates, the potential burdens associated with end-stage kidney disease from birth must be acknowledged.gov identifier: NCT03101891.

ImportanceThere is some data to suggest that racial and ethnic minority infants with congenital diaphragmatic hernia (CDH) have poorer clinical outcomes.ObjectiveTo determine what patient- and institutional-level factors are associated with racial and ethnic differences in CDH mortality.Design, Setting, and ParticipantsMulticenter cohort study of 49 US children’s hospitals using the Pediatric Health Information System database from January 1, 2015, to December 31, 2020. Participants were patients with CDH admitted on day of life 0 who underwent surgical repair. Patient race and ethnicity were guardian-reported vs hospital assigned as Black, Hispanic (White or Black), or White. Data were analyzed from August 2021 to March 2022.ExposuresPatient race and ethnicity: (1) White vs Black and (2) White vs Hispanic; and institutional-level diversity (as defined by the percentage of Black and Hispanic patients with CDH at each hospital): (1) 30% or less, (2) 31% to 40%, and (3) more than 40%.Main Outcomes and MeasuresThe primary outcomes were in-hospital and 60-day mortality. The study hypothesized that hospitals managing a more racially and ethnically diverse population of patients with CDH would be associated with lower mortality among Black and Hispanic infants.ResultsAmong 1565 infants, 188 (12%), 306 (20%), and 1071 (68%) were Black, Hispanic, and White, respectively. Compared with White infants, Black infants had significantly lower gestational ages (mean [SD], White: 37.6 [2] weeks vs Black: 36.6 [3] weeks; difference, 1 week; 95% CI for difference, 0.6-1.4; P P 21 = 3.9; P = .05). Black infants had higher 60-day (White: 99 patients [9%] vs Black: 29 patients [15%]; χ21 = 6.7; P = .01) and in-hospital (White: 133 patients [12%] vs Black: 40 patients [21%]; χ21 = 10.6; P = .001) mortality . There were no mortality differences in Hispanic patients compared with White patients. On regression analyses, institutional diversity of 31% to 40% in Black patients (hazard ratio [HR], 0.17; 95% CI, 0.04-0.78; P = .02) and diversity greater than 40% in Hispanic patients (HR, 0.37; 95% CI, 0.15-0.89; P = .03) were associated with lower mortality without altering outcomes in White patients.Conclusions and RelevanceIn this cohort study of 1565 who underwent surgical repair patients with CDH, Black infants had higher 60-day and in-hospital mortality after adjusting for disease severity. Hospitals treating a more racially and ethnically diverse patient population were associated with lower mortality in Black and Hispanic patients.

Postnatal evaluation of prenatally identified congenital lung malformations (CLMs) often includes a chest x-ray (CXR) and neonatal intensive care unit (NICU) admission for observation. With current efforts aimed at prioritizing value and resource utilization, we sought to assess the utility of this practice in infants with known CLMs. We hypothesized that CXR and NICU admission are overused and could be deferred in the majority of cases.Clinical and radiographic data for infants with CLM from 2007 to 2016 were reviewed with IRB approval. Regression models were developed for respiratory support (RS), symptoms within 30 days of discharge (Sx30), and abnormal CXR. Predictors included initial symptoms (IS), birth weight (BW), gestational age (GA), cyst-volume-ratio (CVR) and abnormal CXR. Odds ratios (ORs) and ROC curves were generated for significant predictors (p 0.05).Fifty-eight infants were identified. Eight were excluded because birth or surgery occurred outside of our institution. Another four were excluded for requiring immediate surgery, leaving forty-six for full analysis. All infants underwent initial CXR and NICU admission, and 22 (47.8%) had an abnormal CXR. Higher CVR (OR = 6.69, p = 0.024) and lower BW (OR = 0.27, p = 0.028) both increased the odds of an abnormal CXR. Applying optimal ROC cutoffs for CVR and BW would have safely eliminated 21 of 46 CXRs, increasing CXR sensitivity from 48% to 68%. For RS and Sx30, no variable, including abnormal CXR, significantly predicted outcomes. Twenty-seven infants (59%) had a NICU stay of24 h and only three patients (6.8%) developed Sx30.Both CXR and NICU admission appear to be overused in infants with CLM. CXR result did not predict need for respiratory support or symptoms following discharge, and thus may not aid in the initial evaluation or in the prediction of future care needs. Using CVR and birth weight can guide CXR use and optimize its sensitivity. Need for NICU admission could not be predicted, but a majority of infants spent24 h in the NICU without intervention, suggesting that NICU admission was likely not needed for all infants in this setting.Study of diagnostic test, Level II evidence.

OBJECTIVE To assess feasibility and maternal and infant outcome after fetoscopic tracheal balloon occlusion in patients with severe congenital diaphragmatic hernia. METHODS We conducted a prospective cohort study of fetuses with congenital diaphragmatic hernia and observed/expected lung/head ratio less than 30%. Eligible women had planned fetoscopic tracheal balloon occlusion at 26 0/7-29 6/7 weeks of gestation and balloon removal 4-6 weeks later. Standardized prenatal and postnatal care was at a single institution. Fetoscopic tracheal balloon occlusion details, lung growth, obstetric complications, birth outcome, and infant outcome details until discharge were evaluated. RESULTS Of 57 women screened, 14 (25%) were enrolled between 2015 and 2019. The congenital diaphragmatic hernia was left in 12 (86%); the pre-fetoscopic tracheal balloon occlusion observed/expected lung/head ratio was 23.2% (range 15.8-29.0%). At a median gestational age of 28 5/7 weeks (range 27 3/7-29 6/7), fetoscopic tracheal balloon occlusion was successful in all cases, and balloons remained in situ. Removal was elective in 10 (71%) patients, by ultrasound-guided needle puncture in eight (57%), and occurred at a median of 33 4/7 weeks of gestation (range 32 1/7-34 4/7; median occlusion 34 days, range 17-44). The post-fetoscopic tracheal balloon occlusion observed/expected lung/head ratio increased to a median of 62.8% (44.0-108) and fell to a median of 46.6% (range 30-92) after balloon removal (all Mann Whitney U, P

Background: Neonatal Intensive Care Unit (NICU) graduates are at increased likelihood of hospital readmission when compared to non-NICU admitted infants, resulting in significant financial and emotional burdens. Some early readmissions following NICU discharge may be preventable. Population-based data identifying factors and costs associated with early readmission are lacking. Methods: We conducted a secondary data analysis on a cohort of infants admitted to NICUs included in the IBM MarketScan Research Databases. Infants were privately insured, born from 2011-2017 and discharged from NICUs across all 50 states. We examined rates of 7 and 8-30 …

Objectives Automated red blood cell exchange (RBX) is an important treatment for patients with sickle cell disease (SCD). Although not specifically targeted for removal, platelets (PLTs) are collected along with red blood cells during RBX. We sought to determine whether the pre- and post- RBX PLT counts could be used to derive the post-procedure hemoglobin S% (HgbS%). Methods Using the pre- and post- RBX lab values of 59 SCD patients undergoing 112 RBX procedures over 1 year, we derived mathematical formulas which estimate the post-RBX HgbS% based on the pre-RBX HgbS%, the pre- and post- RBX PLT, and a correction factor. Results For patients with pre-RBX HgbS > 40%, the mathematically derived post-RBX HgbS% was statistically indistinguishable from the measured post-RBX HgbS%. Conclusions Using a simple formula, pre- and post-RBX platelet counts can provide a rapid approximation of the measured post-RBX HgbS% in patients with SCD.

Objectives To determine the concentrations of nicotine and nicotine metabolites in RBC units as a means to estimate the point prevalence of exposure within the healthy donor pool. Methods Segments from 105 RBC units were tested for the presence of nicotine, cotinine, or trans-3ʹ-hydroxycotinine by liquid chromatography–tandem mass spectrometry. Results Of the 20 (19%) units that contained detectable concentrations of nicotine, cotinine, or trans-3ʹ-hydroxycotinine, 19 (18.1%) contained concentrations consistent with the use of a nicotine-containing product within 48 hours of specimen collection. One RBC unit contained nicotine concentrations consistent with passive exposure. Conclusions Chemicals from nicotine-containing products are detectable within the US RBC supply. Further investigation is needed to determine the risks of transfusion-associated exposure to nicotine and other tobacco-associated chemicals among vulnerable patient populations such as neonates.

Objective To facilitate further assessment of transfusion-associated lead exposure by designing a procedure to test packed red blood cells (pRBCs) prepared for transfusion. Study design The relationship between pRBCs and whole blood lead concentration was investigated in 27 samples using a modified clinical assay. Lead concentrations were measured in 100 pRBC units. Results Our sample preparation method demonstrated a correlation between whole blood lead and pRBC lead concentrations ( R 2 = 0.82). In addition, all 100 pRBC units tested had detectable lead levels. The median pRBC lead concentration was 0.8 μg/dL, with an SD of 0.8 μg/dL and a range of 0.2-4.1 μg/dL. In addition, after only a few days of storage, approximately 25% of whole blood lead was found in the supernatant plasma. Conclusion Transfusion of pRBCs is a source of lead exposure. Here we report the quantification of lead concentration in pRBCs. We found a >20-fold range of lead concentrations in the samples tested. Pretransfusion testing of pRBC units according to our proposed approach or donor screening of whole blood lead and selection of below-average units for transfusion to children would diminish an easily overlooked source of pediatric lead exposure.

We tested 220 red blood cell units for the presence of pharmaceuticals; 15 units (6.8%) were confirmed to contain low concentrations of opiates, benzodiazepines, stimulants, or barbiturates. Further study is needed to determine whether these drugs, which are not prohibited in donated blood by current Food and Drug Administration standards, could mediate adverse reactions in children.

OBJECTIVES. For a cohort of extremely premature, ventilated, newborn infants, we determined the power of either serial caretaker intuitions of “die before discharge” or serial illness severity scores to predict the outcomes of death in the NICU or neurologic performance at corrected age of 2 years.METHODS. We identified 268 premature infants who were admitted to our NICU in 1999–2004 and required mechanical ventilation. For each infant on each day of mechanical ventilation, we asked nurses, residents, fellows, and attending physicians the following question: “Do you think this child is going to live to go home or die before hospital discharge?” In addition, we calculated illness severity scores until either death or extubation.RESULTS. A total of 17066 intuition profiles were obtained on 5609 days of mechanical ventilation in the NICU. One hundred (37%) of 268 profiled infants had ≥1 intuition of die before discharge. Only 33 infants (33%) with an intuition of die actually died in the NICU. Of 48 infants with even 1 day of corroborated intuition of die in the NICU, only 7 (14%) were alive with both Mental Developmental Index and Psychomotor Developmental Index scores of >69, and only 2 (4%) were alive with both Mental Developmental Index and Psychomotor Developmental Index Scores of >79 at corrected age of 2 years. On day of life 1, the Score for Neonatal Acute Physiology II value for nonsurvivors (38.2 ± 18.1) was significantly higher than that for survivors (26.3 ± 12.7). However, this difference decreased steadily over time as scores improved for both groups.CONCLUSIONS. Illness severity scores become progressively less helpful over time in distinguishing infants who will either die in the NICU or survive with low Mental Developmental Index/Psychomotor Developmental Index scores. Serial caretaker intuitions of die before discharge also fail to identify prospective nonsurviving infants. However, corroborated intuitions of die before discharge identify a subset of infants whose likelihood of surviving to 2 years with both MDI and PDI >80 is approximately 4%.

Pfaff and colleagues (2008) argue that “blurring of identity” (10) is “the crucial step” (10) that must occur in order for an individual to act in accordance with the “Golden Rule” (10). They conte...