Association of timing of congenital diaphragmatic hernia repair with survival and morbidity for patients not requiring extra-corporeal life support.

BACKGROUND: While physiologic stabilization followed by repair has become the accepted paradigm for management of congenital diaphragmatic hernia (CDH), few studies have examined the effect of incremental changes in operative timing on patient outcomes. We hypothesized that later repair would be associated with higher morbidity and mortality. METHODS: Data were queried from the CDH Study Group (CDHSG) from 2007-2020. Patients with chromosomal or cardiac abnormalities and those who were never repaired or required pre-repair extra-corporeal life support (ECLS) were excluded. Time to repair was analyzed both as a continuous variable and by splitting the cohort into top/bottom percentiles. The primary outcome of interest was in-hospital mortality. Secondary outcomes included need for and duration of post-repair ventilatory and nutritional support. RESULTS: A total of 4,104 CDH infants were included. Median time to repair was 4 days (IQR 2-6). On multivariable analysis, high-risk (CDHSG stage C/D) defects and lower birthweight predicted later repair. Overall, in-hospital mortality was 6%. On univariate analysis, there was no difference in the number of days to repair between survivors and non-survivors. On risk-adjusted analysis, single-day changes in day of repair were not associated with increased mortality. Later repair was associated with longer time to reach full oral feeds, increased post-repair ventilator days, and increased need for tube feeds and supplementary oxygen at discharge. CONCLUSIONS: For infants with isolated CDH not requiring pre-operative ECLS, there is no difference in mortality based on timing of repair, but single-day delays in repair are associated with increased post-repair duration of ventilatory and nutritional support.

Smartphone-based prenatal education for parents with preterm birth risk factors.

OBJECTIVE: To develop an educational mobile application (app) for expectant parents diagnosed with risk factors for premature birth. METHODS: Parent and medical advisory panels delineated the vision for the app. The app helps prepare for preterm birth. For pilot testing, obstetricians offered the app between 18-22 weeks gestational age to English speaking parents with risk factors for preterm birth. After 4 weeks of use, each participant completed a questionnaire. The software tracked topics accessed and duration of use. RESULTS: For pilot testing, 31 participants were recruited and 28 completed the questionnaire. After app utilization, participants reported heightened awareness of preterm birth (93%), more discussion of pregnancy or prematurity issues with partner (86%), increased questions at clinic visits (43%), and increased anxiety (21%). Participants reported receiving more prematurity information from the app than from their healthcare providers. The 15 participants for whom tracking data was available accessed the app for an average of 8 h. CONCLUSION: Parents with increased risk for preterm birth may benefit from this mobile app educational program. PRACTICE IMPLICATIONS: If the pregnancy results in preterm birth hospitalization, parents would have built a foundation of knowledge to make informed medical care choices.

Alveolar capillary dysplasia with multiple congenital anomalies and bronchoscopic airway abnormalities.

Alveolar capillary dysplasia is a rare and fatal disease of newborn infants. Here we describe a patient with alveolar capillary dysplasia, multiple congenital anomalies, a novel genetic mutation and previously undocumented airway findings on bronchoscopy. Knowledge of these associations may help diagnose this rare disorder in neonates with hypoxemic respiratory failure.

Variations among US hospitals in counseling practices regarding prematurely born infants.

OBJECTIVE: We studied several counselor-independent elements of prenatal counseling regarding prematurely born infants. Elements studied include: indications to offer counseling, clinical settings in which counseling is offered, personnel assigned to counsel, availability of tools to assist counseling and post-counseling documentation requirements. METHOD: As the study aimed to explore system-based practices and not counselor-based practices, we surveyed Neonatal Intensive Care Unit medical directors. RESULT: Responses were received from 352 hospitals (53%) in 47 states. Analysis was based on responses from the 337 hospitals that routinely counseled women anticipating a premature birth. In 299 (≈ 90%) hospitals, counseling was primarily performed by neonatal professionals. Premature labor was the most common indication to offer counseling; however, in 54 hospitals most counseling was offered before labor and based on maternal risk factors for preterm delivery. In nearly all (99.7%) hospitals information was provided verbally and face-to-face; a third of the hospitals also provided written information. For non-English-speaking Hispanic patients, 208 (62%) of the hospitals had certified hospital-based Spanish interpreters. Five (1%) hospitals provided specialized training to the designated prenatal counselors. The upper gestational age eligible for counseling at all 337 hospitals included 33 weeks; in 134 hospitals, gestational age of <23 weeks was not eligible for counseling. CONCLUSION: Antenatal parental counseling for premature delivery is a widely practiced intervention with substantial system-based variability in execution. Interventions and strategies known to improve overall counseling effectiveness are not commonly utilized. We speculate that guidelines and tool-kits supported by Pediatric and Obstetric professional organizations may help improve system-based practices.

Prenatal counseling beyond the threshold of viability.

OBJECTIVE: It is common clinical practice to counsel parents expecting an early-moderate premature birth. The aim of the current study was to assess maternal knowledge of potential problems of prematurity after counseling. STUDY DESIGN: Prospective study of 49 participants admitted between 23 and 33 weeks gestation with threatened premature birth; a prematurity knowledge questionnaire and the State-Trait Anxiety Inventory were administered after counseling but before delivery. RESULT: Across all gestational-ages, participants were more aware of short-term problems than long-term problems. With increasing gestational age the knowledge of long-term problems decreased (P=0.01). Maternal knowledge was 82% for gestational ages where clear guidelines exist regarding goal of counseling and information that should be provided to the parents. CONCLUSION: Most mothers of early-moderate premature infants are not aware of the potential for long-term problems. Guidelines, which outline the information that should be provided to parents, may improve maternal knowledge after counseling.

Readiness of obstetric professionals to inform parents regarding potential outcome of premature infants.

Parents often regard obstetric professionals as an important source of information regarding prematurity. However, there is no information regarding the readiness of these obstetric professionals to inform expectant parents of the potential outcomes of premature infants. Using a self-report questionnaire, we determined the knowledge of obstetric professionals regarding outcomes of premature infants, and gauged their confidence in providing this information to expectant parents. Some 50% of obstetric professionals reported that they 'struggle to answer parental questions' regarding premature infants. The majority of obstetric professionals correctly identified potential morbidities of prematurity, but compared to neonatal professionals, they were less likely to discuss this information with parents. When they do provide information to parents, obstetric professionals were least likely to discuss neurological morbidities. Our study has identified an important barrier to the effective transfer of neonatal outcomes information to expectant parents. This limitation requires further investigation and intervention.

Effects of Carbicarb and sodium bicarbonate on hypoxic lactic acidosis in newborn pigs.

BACKGROUND: Use of sodium bicarbonate (NaHCO3) may result in intracellular acidosis due to the generation of CO2. Carbicarb, has been reported to be superior to sodium bicarbonate (NaHCO3) because of lesser generation of CO2. The present study was designed to investigate whether Carbicarb or NaHCO3 is superior to normal saline in the treatment of hypoxic lactic acidosis. METHODS: Hypoxia was induced by ventilation with 8% O(2) in 30 piglets with fixed ventilation. When the pH fell to < 7.2, hypoxia was reversed by placing the animals in 21% O2 (experiment 1) or 100% O(2) (experiment 2) and either saline, Carbicarb or NaHCO3 were given. Data were collected for 120 minutes after therapy. RESULTS: In both experiment 1 (severe acidosis, pH < or = 7.1) and 2 (moderate acidosis, pH < or = 7.2) use of Carbicarb and NaHCO3 increased the arterial carbon dioxide tension (pCO2) significantly (p < 0.05). With moderate acidosis: 1) use of alkalinizing agents compared to saline resulted in an initial improvement in arterial pH at 1 minute, but thereafter, the differences were not statistically significant; and 2) there were no differences in hemodynamic variables and plasma lactic acid concentration between the three groups. CONCLUSIONS: The data demonstrate that 1) both Carbicarb and NaHCO3 significantly increase arterial pCO2; and 2) use of either alkalinizing agent in moderate acidosis does not alter the course of acidosis.

New dysmorphic features in Rubinstein-Taybi syndrome.

We report a new case of Rubinstein-Taybi syndrome with a hypoplastic right kidney, persistent pulmonary hypertension, and mitral valve regurgitation. Other pertinent features included broad thumbs, broad big toes, syndactyly of the third and fourth fingers bilaterally, beaked nose, broad columella of the nose, patent ductus arteriosus, and motor and mental retardation. The testes were descended. The 3 month old patient had delayed motor and mental development corresponding to a 1 month old infant.