Low Rate of Spontaneous Closure in Premature Infants Discharged with a Patent Ductus Arteriosus: A Multicenter Prospective Study.

OBJECTIVES: To assess the rate of spontaneous closure and the incidence of adverse events in infants discharged home with a patent ductus arteriosus. STUDY DESIGN: In a prospective multicenter study, we enrolled 201 premature infants (gestational age of 23-32 weeks at birth) discharged home with a persistently patent ductus arteriosus (PDA) and followed their PDA status at 6-month intervals through 18 months of age. The primary study outcome was the rate and timing of spontaneous ductal closure. Secondary outcomes included rate of assisted closure and the incidence of serious adverse events. RESULTS: Spontaneous ductal closure occurred in 95 infants (47%) at 12 months and 117 infants (58%) by 18 months. Seventeen infants (8.4%) received assisted closure with surgical ligation or device assisted occlusion. Three infants died (1.5%). Although infants with spontaneous closure had a higher mean birth weight and gestational age compared with infants with a persistent PDA or assisted closure, we did not identify other factors predictive of spontaneous closure. CONCLUSIONS: Spontaneous closure of the PDA occurred in slightly less than one-half of premature infants discharged with a patent ductus by 1 year, lower than prior published reports. The high rate of assisted closure and/or adverse events in this population warrants close surveillance following discharge. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02750228.

Changes in the Diagnosis and Management of Patent Ductus Arteriosus from 2006 to 2015 in United States Neonatal Intensive Care Units.

OBJECTIVE: To identify changes in the diagnosis, pharmacotherapy, and surgical ligation of patent ductus arteriosus (PDAs) in infants born premature and report on temporal changes in mortality and morbidity from a large volume of neonatal intensive care units (NICUs) in the US. STUDY DESIGN: We queried the Pediatrix Clinical Data Warehouse for all inborn infants without major anomalies born between 23 and 30 weeks' gestation from 2006 to 2015 for a diagnosis of PDA, use of indomethacin or ibuprofen, history of ductal ligation, mortality, and major morbidities. RESULTS: There were 829 091 infants entered in the Clinical Data Warehouse; 61 520 infants from 280 NICUs met our inclusion criteria. The diagnosis of PDA declined from 51% to 38% (P < .001), use of indomethacin or ibuprofen decreased from 32% to 18%, and PDA ligation decreased from 8.4% to 2.9% (both P < .001). During the study period, mortality decreased with no increase in any measured morbidity. Of the 163 sites with data for both periods, 128 (79%) showed a decrease in the diagnosis of PDA, and 132 (81%) showed a decrease in the use indomethacin and/or ibuprofen when 2011-2015 was compared with 2006-2010. Of 103 sites with at least 1 PDA ligation, 85 (83%) showed a decrease in PDA ligation in a similar comparison. CONCLUSIONS: In this large population of infants <30 weeks' gestation from 280 NICUs across the US, there were significant decreases in the diagnosis and treatment of the PDA. Although there was no evidence of increased morbidities, it remains uncertain how these changes may directly affect infant outcomes.

A patient with a unique frameshift mutation in GPC3, causing Simpson-Golabi-Behmel syndrome, presenting with craniosynostosis, penoscrotal hypospadias, and a large prostatic utricle.

We present a Hispanic male with the clinical and molecular diagnosis of Simpson-Golabi-Behmel syndrome (SGBS). The patient was born with multiple anomalies not entirely typical of SGBS patients, including penoscrotal hypospadias, a large prostatic utricle, and left coronal craniosynostosis. In addition, he demonstrated endocrine anomalies including a low random cortisol level suspicious for adrenal insufficiency and low testosterone level. To our knowledge, this is the first report of a prostatic utricle in SGBS and the second report of craniosynostosis. The unique disease-causing mutation likely arose de novo in the mother. It is a deletion-insertion that leads to a frameshift at the p.p. S359 [corrected] residue of GPC3 and a premature stop codon after five more amino acids. p. S359 [corrected] is the same residue that is normally cleaved by the Furin convertase, although the significance of this novel mutation with respect to the patient's multiple anomalies is unknown. We present this case as the perinatal course of a patient with unique features of SGBS and a confirmed molecular diagnosis.

Postdischarge growth and development in a predominantly Hispanic, very low birth weight population.

OBJECTIVES: The goals were to assess postdischarge growth and developmental progress of very low birth weight (birth weight: <1500 g) premature infants in a predominantly Hispanic population and to identify predictors for neurodevelopmental impairment at 3 years of age. METHODS: A cohort of 135 very low birth weight infants (gestational age: 23 to 35 weeks) were monitored to 3 years of age. Maternal and neonatal characteristics, anthropometric z scores, and developmental performance (using corrected age until 24 months) were analyzed collectively and according to gestational age groups. Specific criteria for failure to thrive and microcephaly were used. RESULTS: A characteristic pattern of poor weight gain in the first 12 months was followed by accelerated weight gain starting at 18 months, whereas head growth decreased at 18 months, with recovery beginning at 30 months of age. Infants born at gestational age of or=27 weeks achieved catch-up growth by 30 months of age. Mean developmental scores also decreased in infancy, with improvements in motor development emerging at 18 months and cognitive skills at 30 months. Growth z scores, particularly for head growth, correlated with developmental scores. Infants born at gestational age of