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• In extremely preterm newborns with respiratory distress, respiratory distress syndrome (RDS) is by far the most common diagnosis. • The development of exogenous surfactant for treatment of RDS is one of the most important advances of newborn medicine. • Despite widespread consensus on the antenatal use of steroids, controversies remain, including the type of steroid, the dose, frequency and timing, and the route of administration. • Application of nasal continuous positive airway pressure (CPAP) started at or soon after preterm birth may be considered an alternative to routine endotracheal intubation with early surfactant administration. • Severe hypercarbia immediately after birth suggests one of the following diagnoses: pulmonary hypoplasia, tension pneumothorax, congenital diaphragmatic hernia, or severe airway obstruction. • When prolonged rupture of membranes occurs at 15 weeks, the incidence of pulmonary hypoplasia is 80%; when it occurs at 19 weeks, the incidence is 50%, and when it occurs after 26 weeks, the incidence is nearly zero. • Pulmonary hemorrhage occurs in 3%–5% of preterm infants needing respiratory support, usually at 1 to 3 days of age.

Gastroschisis is the most common congenital abdominal wall defect. Despite advances in the surgical closure of gastroschisis, consensus is lacking as to which method results in the best patient outcomes. The purpose of this meta-analysis was to compare short-term outcomes associated with primary fascial closure and staged repair with a silo in patients with gastroschisis.We reviewed Medline citations, as well as the Cochrane Database of Systematic Reviews, between January 1, 1996 and June 1, 2012. Articles were identified using the search term "gastroschisis" and [("treatment outcome" or "prognosis") or randomized controlled trials]. Case reports, reviews, letters, abstracts only, non-English abstracts, and studies that did not address at least one of the outcomes of interest were excluded from the meta-analysis. Two independent reviewers identified relevant articles for final inclusion. A standard data collection form created by the authors was used to extract study information, including study design, patient characteristics, and reported patient outcomes. The data were analyzed using standard meta-analytic techniques.Twenty studies were included in the meta-analysis. In the five studies that selected closure method randomly or as a temporal shift in practice, silo was associated with better outcomes, with a significant reduction in ventilator days (p0.0001), time to first feed (p=0.04), and infection rates (p=0.03). When all studies were included, primary closure was associated with improved outcomes.Silo closure is associated with better clinical outcomes in the studies with the least selection bias. Larger prospective studies are needed to definitively determine the best closure technique.

Objective: To evaluate the changes in outcome that occurred during implementation of a comprehensive outreach program to five level I newborn care facilities, by one level III neonatal intensive care unit in rural WA State. Study design: Standardized outreach educational programs were provided. Outcome measures were assessed by collecting monthly perinatal and postnatal data on 61,551 births. Three six-year periods were defined. Period I (1988-1994) was compared with period II (1994-2000) and period III (2000-2006). Results: Comparing the averages for the three periods, there was a statistically significant increase in maternal transports from level I hospitals, and significant decreases in early-onset sepsis, meconium aspiration syndrome, low Apgar scores, major congenital malformations, neonatal and perinatal mortality rates, very low birth weight (VLBW) infant mortality, chronic lung disease and duration of hospital stay (all p< 0.001). Conclusion: A successful regionalization of perinatal service in rural areas can be accomplished by establishing extensive outreach programs. This model can be utilized to improve perinatal, neonatal and VLBW infant outcomes.

The need to teach professionalism during residency has been affirmed by the Accreditation Council for Graduate Medical Education, which will require documentation of education and evaluation of professionalism by 2007. Recently the American Academy of Pediatrics has proposed the following components of professionalism be taught and measured: honesty/integrity, reliability/responsibility, respect for others, compassion/empathy, self-improvement, self-awareness/knowledge of limits, communication/collaboration, and altruism/advocacy. The authors describe a curriculum for introducing the above principles of professionalism into a pediatrics residency that could serve as a model for other programs. The curriculum is taught at an annual five-day retreat for interns, with 11 mandatory sessions devoted to addressing key professionalism issues. The authors also explain how the retreat is evaluated and how the retreat's topics are revisited during the residency, and discuss general issues of teaching and evaluating professionalism.

To estimate regional lung volume during ventilation with liquids (e.g. perfluorochemicals, PFC) we developed a multi-compartment mathematical model of a lung and thorax. The height of the fluid column and the fluid’s density determine alveolar pressure (P a ). The weight of thoracic contents above any given gravitational plane influences pleural pressure (P pl ). Transpulmonary pressure (P tp =P a− P pl ) and compliance of the lung and chest wall permit estimation of volumes. The results indicate the lung inflates almost uniformly during total liquid ventilation despite a substantial vertical P a gradient. Inflation uniformity is due to the offsetting vertical P pl gradient created by the added weight of the PFC and sustained by the relative rigidity of the chest wall. During partial liquid ventilation our model indicates that the combination of uniform P a with a large vertical gradient in P pl leads to a vertical P tp gradient and therefore relative over-inflation of the top of the lung. This effect increases with increasing PFC dose and with lung height.

New or unconventional approaches to assisted ventilation in neonates that may reduce the risk of or ameliorate the severity of bronchopulmonary dysplasia are described and discussed. These approaches include various adjuncts to current-generation mechanical ventilation such as in-line tidal volume measurements, the use of exogenous surfactant, patient-triggered ventilation, and various forms of high-frequency ventilation. The experimental tool of liquid ventilation is also described, and its potential benefits and side effects when applied to the problem of neonatal respiratory failure are discussed.

The outcomes of 54 breech-extracted second twins weighing between 750 and 2000 gm were compared with the outcomes of their siblings and of 43 sets of twins delivered by cesarean section for malpresentation. Vaginally delivered first twins had fewer days of mechanical ventilation and oxygen therapy than their breech-extracted siblings (p = 0.004). There were no significant differences in any measures of neonatal outcome when breech-extracted twins were compared with second twins delivered by cesarean section. We conclude that routine cesarean section is not justified for nonvertex second twins expected to weigh less than 2000 gm.

Bronchopulmonary dysplasia is a chronic, sometimes fatal lung disease, which primarily affects premature infants and often leads to a dependence on mechanical ventilation lasting many months. To identify prognostic factors of mortality at 1 and 2 months of age, the authors reviewed the medical records of the 144 neonates admitted to two neonatal intensive care units in Seattle from January 1, 1986, through December 31, 1988, who required mechanical ventilation throughout the first month of life. Likely predictors of mortality were tested by logistic regression analysis. The calculated mean airway pressure at 30 days of age (MAP30) and the diagnosis of bacterial sepsis at any time during the first month of life (Bact0-30) were statistically significant predictors of mortality (P < .001 and P = .018, respectively) and had the lowest deviance in the regression model. The probability of mortality was estimated by 1/(1 + e-x, where x = -6.510 + 0.4588 (MAP30) + 1.475 (Bact0-30), and where MAP30 is expressed as centimeters of water pressure (1 cm H2O = 0.0978 kPa) and the presence or absence of bacteremia is 1 and 0, respectively. The records of the 57 infants who still required mechanical ventilation at 60 days of age were reanalyzed with clinical data available during the first 2 months of life. Mean airway pressure (MAP60) and the fraction of inspired oxygen (F60) at 60 days of age combined to form the best predictors of mortality, where x = -7.668 + 0.2940 (MAP60) + 5.935 (F60). The occurrence of bacterial sepsis during the first 2 months of life, the degree of hypochloremia, and the duration of chronic sedative use were also significant predictors of survival, even controlling of MAP60 and F60. These regression equations allow more accurate estimation of the likelihood of survival for chronically ventilated infants and may facilitate decisions regarding withdrawal or continuation of life support.

To determine whether multiple doses of bovine surfactant would improve neonatal mortality in very premature neonates, we conducted two multicenter controlled trials under identical protocols; the results were combined for analysis. Four hundred and thirty neonates born between 23 and 29 weeks gestation and weighing 600 to 1250 g at birth were assigned randomly at birth to receive either 100 mg of phospholipids/kg of Survanta, a modified bovine surfactant (n = 210), or a sham air placebo (n = 220) within 15 minutes of birth. Neonates who developed respiratory distress syndrome and required mechanical ventilation with at least 30% oxygen could be given up to three more doses in the first 48 hours after birth. Dosing was performed by investigators not involved in the clinical care of the neonates; nursery staff were kept blinded as to the treatment assignment. Cause of death was determined by a panel of three independent, board-certified neonatologists after blindly reviewing case report forms and autopsy reports. Fewer Survanta-treated neonates died of any cause (11.4% vs 18.8%, P = .031), died of respiratory distress syndrome (1.9% vs 15.6%, P < .001), and either died or developed bronchopulmonary dysplasia due to respiratory distress syndrome (39.5% vs 49.1%, P = .044). The incidence of respiratory distress syndrome was also lower in Survanta-treated neonates (28.0% vs 56.9%, P < .001), and the Survanta-treated neonates' oxygenation and ventilatory status were improved significantly at 72 hours. Survanta-treated neonates were also at lowered risk of developing pulmonary interstitial emphysema (23.3% vs 36.9%, P = .002) and other forms of pulmonary air leaks (9.6% vs 20.8%, P .002). We conclude that multiple doses of Survanta reduce mortality and morbidity due to respiratory distress syndrome during the neonatal period.

We performed a multicenter study to test the hypothesis that tidal liquid ventilation (TLV) would improve cardiopulmonary, lung histomorphological, and inflammatory profiles compared with conventional mechanical gas ventilation (CMV). Sheep were studied using the same volume-controlled, pressure-limited ventilator systems, protocols, and treatment strategies in three independent laboratories. Following baseline measurements, oleic acid lung injury was induced and animals were randomized to 4 hours of CMV or TLV targeted to "best PaO2" and PaCO2 35 to 60 mm Hg. The following were significantly higher (p < 0.01) during TLV than CMV: PaO2, venous oxygen saturation, respiratory compliance, cardiac output, stroke volume, oxygen delivery, ventilatory efficiency index; alveolar area, lung % gas exchange space, and expansion index. The following were lower (p < 0.01) during TLV compared with CMV: inspiratory and expiratory pause pressures, mean airway pressure, minute ventilation, physiologic shunt, plasma lactate, lung interleukin-6, interleukin-8, myeloperoxidase, and composite total injury score. No significant laboratories by treatment group interactions were found. In summary, TLV resulted in improved cardiopulmonary physiology at lower ventilatory requirements with more favorable histological and inflammatory profiles than CMV. As such, TLV offers a feasible ventilatory alternative as a lung protective strategy in this model of acute lung injury.

Treatments available to improve compliance in surfactant-deficient states include exogenous surfactant (ES) and either partial (PLV) or total liquid ventilation (TLV) with perfluorochemical (PFC). Because of the additional air-lung and air-PFC interfaces introduced during PLV compared with TLV, we hypothesized that compliance would be worse during PLV than during TLV. Because surfactant is able to reduce interfacial tension between air and lung as well as between PFC and lung, we further hypothesized that compliance would improve with surfactant treatment before PLV. In excised preterm lamb lungs, we used Survanta for surfactant replacement and perflubron as the PFC. Compliance during PLV was intermediate between TLV and gas inflation, both with and without surfactant. Surfactant improved compliance during PLV, compared with PLV alone. Because of the force-balance equation governing the behavior of immiscible droplets on liquid surfaces, we predict that PFC droplets spread during PLV to cover the alveolar surface in surfactant-deficient lungs during most of lung inflation and deflation but that the PFC would retract into droplets in surfactant-sufficient lungs, except at end inspiration.

In infants with bronchopulmonary dysplasia, the influence of the severity of their pulmonary disease on neurodevelopmental outcome is unknown. Neurodevelopmental outcomes at a mean age of 36 months were assessed in 27 premature subjects who had bronchopulmonary dysplasia. Subjects had a mean birth weight of 940 g (range, 540 to 1690 g) and a mean gestational age of 27 weeks (range, 25 to 31 weeks). The duration of mechanical ventilation ranged from 22 to 128 days, and the duration of requirement of supplemental oxygen ranged from 34 to 1033 days. No significant correlations were found between duration of mechanical ventilation or oxygen therapy and overall neurodevelopmental outcome. In contrast, cranial ultrasound findings of intracranial hemorrhage and/or periventricular echodensity related specifically to poorer cognitive outcome. By age 3 years, severity of bronchopulmonary dysplasia is not a sufficient predictor of neurodevelopmental outcome. Intracranial hemorrhage and periventricular echodensity continue to be important predictors. (AJDC. 1991;145:813-817)

In order to better understand the postnatal sequence of surfactant secretion and establishment of the alveolar macrophage (AM) population in newborn primates, healthy Macaca nemestrina monkeys were sacrificed during fetal life at term gestation (n = 5), or at 2 days (n = 5) or 3-4 weeks (n = 5) after term vaginal delivery. Excised lung tissue and left lung lavage were analyzed for phospholipid (PL) content, surface active material (SAM) extract, PL components, surface activity, pressure-volume characteristics, and AM number. Compared to term fetal animals, 2 day old term newborn monkeys were found to have a several-fold increase in lavage PL and SAM, and this was associated with greater maximal lung volume and drier lungs, but not improved deflation stability. During the subsequent 3-4 weeks of life, a 42% reduction in lung tissue stores of PL and SAM, and an 87% reduction in lavage PL and SAM were noted. Despite these major changes in quantity, there were relatively minor changes in the composition of the PL synthesized and released. The reduced quantity of SAM in the 3-4 week old animals led to a small decline in deflation stability. The several-fold increase in lavage PL and SAM during the first 2 days of life was accompanied by a 33-fold increase in AM; there was an additional 4-fold increase in AM number by 3-4 weeks of age. The abundance of lavage surfactant at 2 days of age may play a role in the influx of AM.

The appearance of phosphatidylglycerol in the tracheal wash of infants with hyaline membrane disease (HMD) has been reported to be associated with clinical signs of recovery. We analyzed lung tissue and bronchoalveolar lavage surfactant in an animal model of HMD to determine whether phosphatidylglycerol or some other component is necessary for recovery. The amount and composition of phospholipid (PL) was determined in the premature Macaca nemestrina monkey (140 days' gestation) during an acute stage of HMD, and in two stages of recovery. These changes were compared to observations made in healthy premature controls (140 days), gestational age-matched fetuses (140 days), and fetuses of 150 days' gestation (term=168 days). The amount of PL and its surfactant composition in lung homogenates of the right lower lobe and in lavage of the excised left lung was determined. Compared to 140-day fetuses, the healthy controls had a several-fold increase in lavage PL and disaturated phosphatidylcholine (DSPC) during the first few days of life (p

Retinopathy of prematurity is a retinal vascular disease that occurs only in premature infants. Because of concern that the occurrence of this potentially blinding disease is increasing, a retrospective chart review was undertaken to compare the incidence of retinopathy of prematurity at the University of Washington during the years 1981 to 1984 with previously published data collected at the same institution during the years 1968 to 1980. During 1981 to 1984, there was an increase in the annual numbers of admissions and survivors weighing 1750 g at birth; the survival rate increased significantly (P < .000001). A trend toward an increased risk for proliferative retinopathy of prematurity (P = .057) during 1981 to 1984 period was noted, but the number of "excess cases" was calculated to be only 6 per year. The remainder of the additional cases, 19 annually, were due to increased survival of infants at risk. Thus, the "second epidemic" of retinopathy of prematurity is largely due to improved survival of low birth weight infants rather than to new iatrogenic factors. In infants with proliferative retinopathy of prematurity, there was a previously unreported association between increased severity of disease and lower birth weight (P = .015). There were four children with severe bilateral visual loss due to retinopathy of prematurity identified during the 1981 to 1984 period, whereas only one bilaterally blind infant was noted during the preceding 12.7 years.

• We reviewed the records of 4778 infants who were admitted to the affilliated intensive care nurseries at the University of Washington In Seattle from Jan 1, 1980, through Dec 31, 1983. We evaluated the outcome for patients with bronchopulmonary dysplasia who required mechanical ventilation and supplemental oxygen for at least six months. Eight (53%) of 15 study patients were alive at 3 years of age. Preselected indexes measured during the first six months of life that were not associated with death before 3 years of age included growth measurements, fraction of inspired oxygen at 6 months of age, mean airway pressure, arterial partial pressure of carbon dioxide, and right ventricular hypertrophy. In contrast, recurrent cyanotic episodes requiring intermittent muscle paralysis or long-term sedation therapy to maintain gas exchange occurred in six of seven nonsurvivors and only one of eight survivors. The survivors have reactive airway disease and recurrent lower respiratory tract infections. ( AJDC 1988;142:721-725)

• Ebstein's anomaly of the tricuspid valve occurs as an isolated defect with other forms of congenital heart disease such as transposition of the great arteries or tetralogy of Fallot or, rarely, in association with extracardiac malformations. Because so little is known about this latter group, we studied four cases clinically, at autopsy, and by means of a retrospective chart review. Major extracardiac changes most often involved the craniofacial region, central nervous system, and limbs. Karyotypes were normal, and no distinctive syndromes or anatomic patterns were identified. Since the timing of Ebstein's anomaly is quite precise, ascertainment of such cases on the basis of cardiac morphology may enhance the understanding of etiology and pathogenesis. Both causes and mechanisms may well be diverse, for the clinical histories and anatomic findings in present and previously reported cases vary considerably. Isolated Ebstein's anomaly may develop from topographically and temporally localized damage. Ebstein's anomaly with extracardiac defects may involve damage during a longer, and perhaps earlier, period. (AJDC. 1989;143:570-572)

Exposure of piglets to 68-72 h of hyperoxia has previously been shown to blunt hypoxic pulmonary vasoconstriction (HPV). We tested the hypothesis that the administration of a scavenger of toxic oxygen radicals during exposure to hyperoxia would preserve HPV in piglets. Newborn piglets were kept in FIO2 greater than 0.90 for 68-72 h and compared to control animals kept in room air. Randomly selected animals from both groups were given the O2 metabolite scavenger, dimethylthiourea (DMTU) (500 mg/kg followed by 250 mg/kg/d), resulting in plasma levels of 2-6 mM. Following the oxygen or room air (RA) exposure period, piglets had pulmonary vascular resistance (PVR) measured following a 20 min exposure to alveolar hypoxia (FIO2 = 0.12). Both groups of RA-exposed animals (DMTU and saline treatment) as well as the hyperoxia-exposed saline group had almost 2-fold increases in PVR during exposure to hypoxia (P less than 0.05). Contrary to expectations, the PVR in the hyperoxia-exposed DMTU group did not rise significantly during hypoxia and the use of DMTU did not restore HPV by increasing PVR to levels greater than the hyperoxia/saline group (P = 0.70). Therefore DMTU does not appear to prevent hyperoxia-induced blunting of HPV in piglets.